Kaitlyn Hatchard (Spinal Muscular Atrophy-Type 1)

2011-11-21T15:08:00.000-04:00

Oh my dear...what has it been?? 6 months..has it really been 6 months since I posted last? A friend of mine says that she keeps checking the blog to read Mother's Day, and is sick of seeing the same post! I guess I better get back to my regular updates! So much has happened in the past six months!
Where did we leave off? Oh yeah..hematology. We had an appointment with hemotology back in the spring and they have diagnosed Kaitlyn with a Multifactorial Anemia. Which is a polite way of saying "we do not know what is causing her anemia" (their own words!). They have also used the term Anemia of Chronic Disease, just another way of saying the same thing really. There seem to be multiple factors involved in causing Kaitlyn's anemia. I had them test Kaitlyn's erythropoietin levels to see if she was making enough of this hormone. Erythropoietin is a hormone produced in the kidneys that tells bone marrow to make more red blood cells. Her levels came back very low normal....but just within normal range.

I remember questioning the hemotologist at the time and saying since she was low normal would we not start her on a trial of Erythropoietin replacement, and he said no her levels are normal. Two weeks ago when we were in seeing the nephrologist Kaitlyn was once again very anemic and he asked if we had ever had her Erythropoietin levels checked. I told him we had the test done back in the spring and that her levels came back low normal. He checked the records and noticed that she had been anemic at the time that the test was taken. The nephrologist stated that the Erythropoietin levels should have come back high normal or even higher as her body should have been trying to stimulate red cell production. I am a little perturbed that we didn't start the Erythropoietin therapy back in the spring when I questioned the levels. The nephrologist started Kaitlyn on Erythropoietin that very day and we have since had another transfusion. If the hormone replacement therapy works then hopefully Kaitlyn will not need such frequent transfusions. Erythropoietin is the second hormone related to the kidneys that Kaitlyn does not produce in sufficient amounts. More research needs to be done on SMA children and hormone levels!

Kaitlyn has also started Pamidronate Infusions. Pamidronate is a bi-phosphonate drug that will help Kaitlyn bind the calcium into her bones. It is that drug that will have a twofold effect for Kaitlyn. For one, it will help control her hypercalcemia and it will help increase her bone density. Kaitlyn's serum calcium levels are much more stable now. Her ionized calcium still comes back high, but not near as high as pre Pamidronate. We feel like the drug is working for her. We are currently using a quarter dose of Pamidronate (based on weight) as Kaitlyn had a bad reaction to Pamidronate after the first round.

In the beginning we gave Kaitlyn a half dose of Pamidronate the first day and a half dose the second day. We wanted to be cautious with Kaitlyn and divide the first dose over two days to avoid Kaitlyn dropping her calcium levels too quickly! But Kaitlyn had her own twist! Kaitlyn developed a secondary Fanconi Syndrome. This is where the kidneys shut down and start to spill glucose and amino acids in the urine! Since we believed the Fanconi Syndrome to be secondary to the Pamidronate all we had to do was wait and see if the condition corrected itself in the absence of the medication. Kaitlyn's body slowly recovered from the Fanconi Syndrome. We waited about eight weeks and then decided to try Pamidronate again at a much lower dose. When Fanconi Syndrome is medication/drug induced then there is a level of the drug that should both produce the desired effect medically without causing the Fanconi Syndrome. We have been giving Kaitlyn a 1/4 dose based on weight since the summer. We do the four hour infusions once a month and so far Kaitlyn has had no issues. We recently went up a smidge on her dose as her calcium levels came back high again. Pamidronate one of the few drugs we have to try and control Kaitlyn's hypercalcemia.

We have (a month ago) also started Kaitlyn on a VERY weak vivonex solution running into her J-tube. The gastroenterologist has stated that if we can get even 10% of her diet delivered enterally then it would go a LONG way towards protecting her internal organs (including her liver, kidneys) and the lining of her intestine. For those of you familiar with the AA Diet and vivonex specifically; weare mixing 1 tbs (about 1/8 of a package of vivonex) with 240mls of water to make a .2 cal/ml mix. Kaitlyn gets this very dilute formula at a rate of 10cc per hour for almost 24 hours per day. So far Kaitlyn seems to be tolerating this mixture fairly well. She does have more green bile backing up into her stomach,but she is still straight drianage (and likely always will be!). This means that we leave Kaitlyn's G-button open at all times and allow any stomach fluids and bile to drain from her stomach. Since Kaitlyn's stomach is paralysed completely she does not have the ability to even tolerate her own gastric secretions. Her stomach does not contract to pus these fluids down into the intestine to be digested/used in the appropriate way. If we cap off Kaitlyn's G-tube she is gagging within minutes!!! She will vomit if her tube remains blocked for more than a couple of minutes.

Our hope is that the green bile we see backing up from her small intestine will lighten in time. Before starting the vivonex in her J-tube we were running water at the same rate 10ccs for 24 hours. The drainage at that point was pretty clear, almost all the time. Occasionally there would be a bit of yellowy bile but next to no green ever came out. I'm not sure if we will ever see a lack of green bile while on any sort of stomach feeds. However, she seems to be tolerating the feeds in every other way :) She is not bloated, she does not complain of tummy pain, she is not gagging on a daily basis and her bowels are still working!

Our latest news is of a more "normal" fashion! Kaitlyn is now requiring the use of glasses to correct her vision. She has astigmatism and is near sighted. Right now her prescription is pretty weak but her opthalmologist wanted her to have glasses in the hopes that it may help her Strabismus. She seems to really like her glasses :) Good thing! She looks pretty cute :) We have altered some ear pillows to accomodate her side lying position. So far we have had zero issues with the glasses. They do not mark her face or head at all!

Happy Mother's Day

2011-05-09T00:23:00.000-03:00

Wow, I cannot believe it has been several weeks since I posted. We have another transfusion under our belts. Kaitlyn's hemoglobin got as low as 65 this time in a little less than five weeks :( I think it's time for a referral to hemotology on this issue!

We are also meeting with our Nephrologist this Wednesday to see what we can do about Kaitlyn's hypercalcemia. There are ways to treat hypercalcemia if the cause is identified. Our nephrologist believes that Kaitlyn is probably leching calcium from her bones. This concerns my husband and I a great deal!! Kaitlyn is already osteopaenic. Her bones are brittle and she has now been leeching calcium from them for the past almost six months!!!! We have had bone density scans done for the past three years. Kaitlyn was due for more scans this past January! Our doctors felt that the scans wouldn't be much help? I'm not sure I agree :( I feel that we would have a fairly good comparison from the past three scans. Perhaps it would give us some insight as to the extent of bone degredation (if any) to the calcium being slowly removed from her bones! If she is leeching calcium from her bones then there are ways to prevent this from continuing!

One way is to treat Kaitlyn with bi-phosphonates. One such drug is called Pamidronate which is used by several SMA children for brittle bones. It helps keep calcium in the bones. Currently we are adding ZERO calcium to Kaitlyn's diet!! This concerns me greatly as calcium is good for more than just bones! Our doctors have increased Kaitlyn's sodium and fluid intake trying to get her to "pee" off the excess calcium in her blood. So far this has been working, but it is only a band-aid! I do not believe that she has an indefinite supply of calcium and I also do not want to wait until we break one of her bones before we treat the problem!

We had a rough week two weeks ago. Kaitlyn's J-tube blocked when I was giving her afternoon meds! This happened on Monday afternoon. We couldn't take Kaitlyn in until Monday evening. By then, being Easter Monday, there was nobody around who knew what to do with Kaitlyn. They admitted us so that we could see our surgeon early Tuesday morning. By admitting us they could also administer some IV fluids/meds to replace the fluids Kaitlyn would be missing from her J-tube. Kaitlyn gets about 300cc's per day through her J-tube. Not to mention medications.

Anyway, my story....Tuesday morning arrives and our surgeon comes to tell us the bad news. We haven't a replacement tube in the hospital. Kaitlyn has a GJ Mickey Button. To my knowledge she is the ONLY child using this tube in our children's hospital. Our surgeon had to order TWO new tubes from the States. He had surgery time tentatively booked on Thursday. Rather than discharge us from the hospital, they sent us home on a pass so that we had the IV fluids we needed and IV medications. We went back to the hospital on Thursday in the morning. We were scheduled for afternoon surgery. Our surgeon came up in the afternoon to tell us that the J-tube hadn't arrived, it was stuck in customs at the airport :( We decided to stay overnight and Kaitlyn would have her surgery Friday morning. Kaitlyn had her surgery and everything went well. She lost another tooth when the anesthetist intubated her :( She is now missing both her two front teeth.

This weekend has been quiet. We went "shopping" Saturday. Shopping is in quotations because first you have to have money to shop! LOL Owen needed a new pair of rubber boots and the family wanted to get me a movie I've been wanting for Mother's Day. The kids each picked a toy as well...nothing big. Well, Kaitlyn did spy an Ernie that she NEEDED!!! She said "need Ernie...need Ernie"!! How can we refuse when she asks so sweet!! I hope everyone had a pleasant Mother's Day!!

Kaitlyn and Daddy (with Ernie) having a cuddle in the rocking chair.

Kaitlyn's new toothless smile :)

Kaitlyn having a cuddle with Mama! She doesn't exactly fit in my lap anymore!!

Owen holding his Freckle Face plant! He picked it out and has been doing EVERYTHING with it since we brought it home! He even took it to watch him have a bath and ate with it at the dinner table :P

My WILDMAN!!! Kaitlyn's twin Alexander :)

Hypercalcemia

2011-03-24T09:27:00.001-03:00

Last week Kaitlyn had another blood transfusion. She had blood work done on the Tuesday and her hemoglobin was 71! We went in on Wednesday for a very long day in house. They couldn't admit Kaitlyn as our hospital is very short on rooms right now and is full of RSV! We do NOT want to be anywhere near that stuff. We were given our own room and our own nurse (thanks Linda!!) in the Medical Day Unit. Because it was a Wednesday we also got to see Kaitlyn's pediatrician who does rounds in our hospital! It was so good of her to see us!! She arranged for us to meet with a nephrologist to try and get this hypercalcemia under control.

The nephrologists believe that Kaitlyn may be leeching calcium out of her bones. This can happen when individuals are immobile for entended periods. Right now their main concern is her hypercalcemia! Her serum calcium is at critical levels (3.19) and they have been trying to bring the calcium down slowly. Kaitlyn has been started on additional sodium both in her TPN and in her J-tube. They are also adding additional water to her TPN. Their goal with the sodium is to get Kaitlyn to excrete more calcium in her urine by increasing her total urine output. In almost one week of this treatment Kaitlyn's total serum calcium has dropped a smidge (although she also had a blood transfusion), so they are treating more aggressively this week with more sodium to try and bring the calcium down into a normal range. Once this is accomplished then they will deal with the cause of the hypercalcemia.

If her hypercalcemia is due to leeching calcium out of her bones, then they can treat that by the addition of biphosphonates (ie. pamidronate). In the meantime we will just have to watch her close. I have no idea how weak her bones are at this point, but calcium leeching out of her already weak bones kind of scares me! Biphosphonates kind of scare me too because of their side effects. Luckily we know several SMA Type 1 children who have already had these transfusions (pamidronate) so we will lean on them when the time comes ;)

In less than two weeks Kaitlyn's hemoglobin is already below normal values. Her hemoglobin was 104 yesterday. Normal values are 115-145. I cannot wait to get these acute issues dealt with and see if we can't get her a little more stable! Perhaps then her hemoglobin won't drop so fast! She doesn't feel herself when the hemoglobin drops too low :(

Some recent shots of the kids! Sorry I haven't posted any in so long :(

Kaitlyn is such a Chatty Cathy!!!

2011-03-14T00:05:00.000-03:00

From the time that Kaitlyn was an infant people have been telling us that it is highly unlikely that she would ever speak. Speech is incredibly difficult for an SMA Type 1 child. They have neither the lung strength nor the articulation to make speech. Against all odds our girl has begun her journey with words!! She has had a few simple words since she was about ten months old. For three years now we thought that the few amazing words she could belt out were all that she would ever have. Well since coming home from the hospital in December our girl has developed an incredible desire to speak!! She "talks" almost non-stop now! She is still not talking in full sentences, but her enunciation and vocabulary have definitely grown :)

I never thought that I would see the day that my daughter would be able to tell me verbally how to help her! She has been verbally asking for coughs and/or physio for a while now. The other day she was fussing and fussing. I changed her diaper, smoothed her clothing (checked for uncomfortable wrinkles), readjusted her position and nothing seemed to help. I think I even took her temperature! (Force of habit :P) I got a little frustrated that everything I did wasn't helping! I finally asked her "Kaitlyn what is wrong?" Kaitlyn said, "Ishy (itchy)". Me, "Itchy what?" Kaitlyn, "Itchy head!!!" So I told her to stop crying that I would scratch her head and I did. I couldn't help but be a little speechless, amazed, and a little overwhelmed!!! My girl just communicated something to me in a WAY that I never thought would EVER be possible. As I'm scratching and wondering when I should stop....She says, "that's better!!!" The first thing I did was chuckle and shake my head in wonder and then ran for my cell phone to tell Tim :)

The other night Tim was lifting Kaitlyn's upper body off the bed in a big bear hug. He kind of squeezed her and gently rocked her from side to side. Kaitlyn says "easy" then "too rough!!" LOL We both looked at each other and just laughed. Daddy said, "Sorry Kaitlyn"!! His hug certainly didn't look rough to me, but we are both so thankful that she is finally able to communicate with us even in these simple ways!!!

Kaitlyn has been dealing with anemia. Two weeks ago her hemoglobin was 88 down from 116 two weeks before that! Normal hemoglobin is 115-145 g/L. She has bloodwork again this Tuesday and will likely need another transfusion. She seems to need a transfusion about every six weeks. I want to add that Kaitlyn is not iron deficient. Her serum ferritin is 1368.1! Normal values are 12-120 ng/mL. Serum Ferritin can also be elevated from inflammation within the body.

On top of the anemia Kaitlyn has been dealing with back to back urinary tract infections. Six weeks ago we had to learn how to catheterize Kaitlyn to help empty her bladder. She was going 12+ hours consistently without urinating! We cathed her once in the hospital and got 540 mLs!!! Kaitlyn has had urine retention for several months, but it has only been the past few weeks that she has had difficulty urinating "enough" on her own. She always seemed to urinate well, but NEVER completely emptied her bladder. Now she is having difficulty some days urinating at all. Urine catheters were ordered for every eight hours. We have been placing the catheters every day. Since beginning the catheters we have had back to back UTI's. Last week we started an antibiotic for UTI number three. Friday, Kaitlyn's urine really started to stink. An aweful sulphur smell (rotten eggs). I didn't know what to think. Well last night when I cathed her there was a LOT of sediment in her urine. When I dipped the urine there was a high level of blood in the sample. This is after five days of antibiotics. The bacteria was sensitive to the antibiotic Kaitlyn was taking. We can only assume she has managed to grow a new bacterium resistant to the antibiotic we were using. We started a new antibiotic today and are keeping our fingers crossed that when the sensitivities come back that we are NOT going to need IV antibiotics! The lab said this sample was almost pure puss :(

The third issue we have been having is with elevated serum calcium. Both the ionized calcium and total calcium values are elevated! I have no idea what this means! Her phosphate levels have been normal so our doctors do not think the hypercalcemia is due to the parathyroid. They think her issue is kidney related, but I'm not sure in what way! Our paediatrician is going to consult Kaitlyn's renal doctor who follows her for her high blood pressure. Hopefully he will have some suggestions. Tests we can do, or some sort of treatment! We have taken out ALL oral calcium and Vitamin D3 supplementation ordered by endocrinology. They wanted her to have 800 mg of elemental calcium in addition to the calcium in her TPN. The calcium in her TPN was below normal levels for a typical TPN order. They have also taken that reduced calcium down by an additional 20%. They have been trying to lower her serum calcium, but alas the serum calcium continues to rise! Her total serum calcium was 3.19mmol/L two weeks ago. Normal values are 2.1-2.7 mmol/L. I sure hope they figure this one out soon as her ionized calcium has been high since we were dicharged back in December!

Kaitlyn has routine blood work this Tuesday and will likely need to be admitted to hospital for another transfusion. Our hospital is reportedly full of RSV!! We will NOT have to go through ER or admissions! Our doctors always triage Kaitlyn right from the comfort of her hospital room! They are also great at isolating Kaitlyn from the nasty bugs and we never share a nurse that has an isolated kid!

Please keep Kaitlyn in your thoughts as we try to figure out what is going on inside her little body!

Home for Christmas 2010!

2011-01-07T21:41:00.002-04:00

Oh my Dear!!! It has been FOREVER since I posted! Sorry! We have been a little..err...busy :) We got home December the 17th! Just in time for Christmas! Thanks to some very special people making some holiday magic we had an AWESOME Christmas!!! With one week left until Christmas, Tim and I had no time for shopping :( We cannot begin to thank those involved for all of the help they gave to our family!! I do not even know WHO to thank for some of the gifts we received! All we can say is that our family had the best Christmas ever, and thank you to everyone that helped make our day so special.

So what happened?? I know I for one am still struggling for answers! Why did Kaitlyn's stomach stop working? Why is her small bowel also not functioning properly? Why was her potassium dropping dangerously low when we were trialling feeds? Why did she require TWO blood transfusions while we were trialling feeds? I could handle the respiratory stuff , but this metabolic/blood/digestive issues are WAY over my head to understand! I have spent countless hours on the internet searching, reading and learning trying to understand what is happening to our daughter. Looking for answers or a solution to her troubles!

So far Home TPN has been fairly "easy". We have to be super careful to keep everything clean! Aseptic techniques and sterile feilds are not hard, you just have to follow the rules EVERY TIME! The time it takes to set up IV feeds is actually less than anything we ever did for enteral feeds!! Crazy but true! The hard part of IV feeds is getting some of the supplies we need covered!! We have an insane medical system that pays for everything our child needs when she is hospitalized, but when we want to bring her home those same supplies are not covered!?!?! Some of the medications we have Kaitlyn on work better for her when given through her IV! But those medications are expensive and are not covered when we bring our daughter home. We found aid through Palliative Care from our local Children's Hospital to pay for our IV meds. But this doesn't cover the cost o some of the supplies we need to deliver these meds. This is NO fault of the Palliative service!!! We are extremely greatful for the help they have offered freely to our family! The cost of the supplies AND meds we need, I feel, should be covered. We are doing the "system" a favour by keeping our daughter in our own home. We save the government thousands of dollars per DAY by having Kaitlyn at home! You'd think the least they could do is cover meds and supplies needed to keep her at home! OK that's my rant for the day ;)

We are doing well since coming home. Everyone has adjusted to the new routine really well. Tim is still able to get out to the firehall training sessions every week. The boys are happy and Kaitlyn is thrilled to be home! She thrives in her home setting! She has discovered some new words and is coming out with more every week. She still does not communicate in sentences, but her vocabulary is growing and her responses are becoming more predictable. We have been able to discover that she knows most of her colors, now that she is actually saying the words :) We use a lot of eye gaze to find out what Kaitlyn knows, but unfortunately with eye gaze if she doesn't cooperate then the activity is useless :)

We have had some troublesome blood labs since coming home. Some of Kaitlyn's bi-weekly blood-work has been a little off. Her GI doctors and her pediatrician have been on top of it though and we are hopeful that the changes they have made will help. The other issue unrelated to TPN is that Kaitlyn is anemic again. She is not iron deficient though, so we are not too sure what is going on.

Hopefully next week's blood labs will look better so we can get our girl stabilized and keep her home for a VERY long time :)

Feeding Intolerance or Bacterial Overgrowth in the Small Bowel ???

2010-11-21T08:46:00.000-04:00

So for the past week we have been entertaining the idea that Kaitlyn may have a bacterial overgrowth of the small intestine. It might explain the bloating that we have had, the gas and intolerance in feeds! However, I don't think it explains the gastroparesis and duodenal paralysis, the low to no bowel sounds and the fact that she still has barium throughout her intestine two weeks after surgery!! Our surgeon thinks the bacterial overgrowth may be what's causing the feeding intolerance. He has consulted GI and they have decided that they will treat Kaitlyn for bacterial overgrowth, as there is no way to really accurately test for bacterial overgrowth. The best way to tell if bacteria is causing her feeding intolerance is to treat for bacterial overgrowth and then try to feed her again. GI has recommended an antibiotic to be given through Kaitlyn's J-tube (treat the infection directly!) and has placed her back on Cisapride (motility med.)!

We were also having issues with low grade fevers and off and on fevers! We cultured EVERYTHING!! All cultures came back negative....except low growth yeast from her throat! (Guess that might explain the THICK white quarter sized plugs we have been pulling out on the end of our 12Fr catheters!!! YUCK!! Kaitlyn had a few days before we started treating for bacterial overgrowth that she was feeling miserable. She was not sleeping, she had the fevers, she was whining almost continuously, and sometimes was even crying!! We couldn't find anything wrong. In the meantime we kept watching her haemoglobin levels get worse and worse! She was really anemic!! We decided to do a transfusion of packed red cells! About half way through the transfusion Kaitlyn stopped whining and went to bed really well (she actually went to SLEEP)!! Then the next thing we notice is her fever is gone...and doesn't return!! The next day she was acting like normal Kaitlyn again (just a little tired from missing so much sleep the past few days)!

Because of the fevers and the no sleeping and crying Infectious Disease ordered a CT scan to rule out any inflammation/infection in the small bowel or other organs that we have been unable to see with other diagnostic imaging. The CT scan was clear, but did show us that both hips are dislocated. This is quite common with SMA, particularly the weaker children/adults. No intervetion is typically needed as there is just no muscle there to support the joint! We did have proper x-rays done of her hips to document the bones as they are right now!

Here's the journal of our past week:

November 16

What a rough couple of days! Kaitlyn has been unable to rest...and I mean NO restful sleep for almost three days! She has been crying a LOT, fussy and just NOT herself. Her hemoglobin finally dropped to 68 and our pediatrician called it....she's not making enough new blood! We had to give her a transfusion of red blood last night. What a difference it made in her sleep. She went to bed around 10pm and is still sleeping now at 8am!! I got my Kaitlyn back :) She is now exhibiting some discomfort when we touch her belly and x-rays are clear, ultrasounds are clear, all cultures sent are clear and her tummy is still soft!!! Today we are having a CT scan to rule out anything minute we might be missing!!! I almost hope they find something (something fixable!!!!!!). This "not knowing" what's going on, not understanding why she is not tolerating any feeds is driving me NUTS!! We are also working towards getting the paperwork started for home TPN!! This process apparently is supposed to take several months!!! Owen's Birthday was on Sunday! My big guy is 6 years old now :) He is the bestest of all big brothers and we are so proud of him!!!

November 18

Kaitlyn is a different kid since her blood transfusion two nights ago!! The fussies have stopped, the fevers have stopped, the really high blood pressures have stopped and blood pressure is stabilizing!! Her HR has gone down 30 bpm and she is sleeping peacefully through the night again :) CT scan was clear...no abnormalities. Heamoglobin is up to 117 from 68! Kaitlyn is chronically anemic, we almost never see normal haemoglobin values :)

GI has decided to put Kaitlyn back on Cisapride and begin treating with antifungals/antibiotics for a possible bacterial overgrowth in her small bowel! This is our last effort to get her gut working. In the meantime we have started the process to get home TPN!! Apparently the longest part of the process to get home is the training involved to teach the families how to run the TPN at home!!! Can you see me rolling my eyes folks!!!!! If Kaitlyn's issue is bacterial overgrowth it might not look so good for enteral feeds...but we will be giving it our best shot!

November 19

Kaitlyn was in such a good mood today :) Laughing and playing her funny little games! She had her nurse in stitches..hehehe. Update: Hypertension diagnosis is official! Renin levels came back twice the normal values!! We have doubled her dose of Blood Pressure med and her BP seems to have stabilized over the past couple of days. She will remain on BP meds for the rest of her life. CT scan showed dislocated hips :( We knew the left was out. They do not seem to be causing her any discomfort on a regular basis at this time!

We have started an antibiotic through her J tube to fight the possible bacterial overgrowth! We have also restarted the Cisapride (motility med.) for what GI calls the "what can it hurt....and if it helps even 10%"!! We are giving this stomach one last chance and we are putting everything we've got into it!! She is on half vivonex/half pedialyte mix and is running at 10cc per hour. So far she is looking pretty good! We are definitely getting more by way of residuals, but they are clear (for the most part) and only slightly tinted yellow/green!! Do I dare to say that the belly might actually look a wee bit less distended tonight?? SHHHHH...don't tell her I said that!!!

TPN....UGH....TPN! What a HUGE hassle!!! There is a special TPN board in which a handful of people sit in specialized positions?? One of the main decision makers for the home TPN process is away on sabbatical until January! The nurse specialist (also highly important for the home TPN process) is away on vacation!!! She won't be available for a meeting to even discuss the option of Home TPN until Decemeber 8!!! So if the belly doesn't work, then we are stuck here until after the 8th of December! We are doing everything else we can in the meantime! We will be trained on how to run TPN at home. We will be practising accessing Kaitlyn's port and disconnecting and connecting lines! We have done everything already that we need to know except for the changing of the actual needle in her port!!

G-J Tube Surgery

2010-11-13T23:38:00.017-04:00

Kaitlyn had her G-J tube surgery Nov. 3. She had to be intubated as the surgeon had to place a scope to help him guide the new tube into place. She had to have her G-tube stoma stretched to take a larger G-J tube. Our surgeon then placed the J-tube and advanced the tube well into the small bowel. Everything went so smoothly! The biggest hitch about the surgery itself was that the anesthetist knocked out one of her front teeth :( They took Kaitlyn to PICU after surgery for recovery. This way Tim and I have access to her immediately after surgery....and she is where she needs to be in case of complications. But, like a trooper, she pulled through surgery yet again with flying colors and extubated to her own vent as soon as they were happy with the placement of the J-tube and that there were no perforations, etc. She did so well we were back up to our room in PMU two hours after she was extubated!!

Other than being in a little discomfort from all the air they pumped her full doing the surgery, Kaitlyn's surgical recovery was quick! She was pretty much recovered by the third day post surgery. We started pedialyte at 5 mls per hour through her J-tube and ran that for 24 hours. The next day we tried half pedialyte/half double diluted vivonex and ran that mix at a rate of 5 mls per hour. She seemed to tolerate both the formula mix and the rate really well. We then worked her up to a rate of 15mls per hour and that's when her tummy started to bloat a bit. We held the rate at 15mls for a good 18 hours. We then slowly worked her up to 25mls per hour and that's when we started seeing some bigger residuals coming back in her tummy. We have even seen some creamy residuals very reminiscent of formula backing up into the stomach!!

Since our admission to hospital Kaitlyn has also been diagnosed with high blood pressure and is now on medication for hypertension. We have to check her blood pressure several times a day and dose her with a hypertensive med. if her BP gets too high! She has had pressures as high as 140/100 and continuously over 120/90 until we started blood pressure meds. We have been treating her for high blood pressure anytime her systolic pressure is over 120 up to every six hours. The doctors believe that Kaitlyn has hypertension because she is a chronically vented kid. Chronically ventilated kids can sometimes have hormonal imbalances and in Kaitlyn's case this seems to be true. We are awaiting some test results that should confirm our doctor's suspicions.

Here's a bit of a journal of the past few days:

November 3

Surgery was a little late today. Kaitlyn did great. Hopefully when we begin feeds in a couple of days the formula will flow in the right direction! She has some discomfort tonight, but overall not too bad. Her tummy is still pretty swollen. They knocked out one of her front teeth intubating her....so her mouth is also a little sore!! Poor girl...and she had such a beautiful smile :(

November 6

Kaitlyn has fully recovered from surgery. We started feeds yesterday. Pedialyte running at 5mls per hour. Today we are running half pedialyte/half dd vivonex, also starting at 5 mls per hour. The directions from surgery are to go up by five every four hours! I think Tim and I may decide to up feeds a little more slowly... :) So far she seems to be tolerating the rate! Kaitlyn is still having issues with high blood pressure! She is now having to go on meds for hypertension.

November 8

We have started feeds. We have made it to 25 mls/hour of half vivonex-half pedialyte. But there is a lot of residual in her tummy! Not too sure if this is just "normal" for Kaitlyn, but it seems to me that we could run into problems if we are constantly dumping residuals from her tummy!! Perhaps we are just too anxious... to get home. Kaitlyn's tummy is nice and soft with each new increase, but her tummy is definitely bloated, compaired to her normal! She is not fussy, but she is flushing a lot the past few days. However, she is also on a number of new meds :/ Hopefully everything will sort itself out as we continue feeds!

November 13

Got to see our surgeon in a tuxedo tonight!!! I have no idea what function he was called out to to come consult our daughter....but THAT's dedication and true caring!! Kaitlyn is once again on full TPN! Yesterday and today she has been running a fever and ALL cultures are negative!! We cannot find infection anywhere...no reason for the fever! We did an x-ray of her abdomin this morning and her J-tube is in the right place, there is no abno...rmality to be found. However, since placing the j-tube we have been having a lot of bile back up into the stomach. When I say bile I do not mean lightly tinted greenish yellow secretions!!!! I mean thick dark brown-green, sometimes greenish-yellow, and sometimes VERY suspicious of creamy formula-like appearance!!

Yesterday and through last night Kaitlyn was getting more and more aggitated. She has not been sleeping well the past three days! Yesterday she was awake at 5:30 am, had a half hour nap in the afternoon and then stayed awake until 9:30pm! She then woke up at 12:00am until 2:00am and then woke up for the day at 6:00am!!! She was miserable ALL day today. She fussed and whimpered and even cried tears until this afternoon when she FINALLY had a three hour nap. We turned her feeds off at 9:00am this morning and they have been off since. Her tummy is quite distended and sore to the touch. Our surgeon is now thinking that she may have a bacterial overgrowth in her small bowel causing the bloating and discomfort. He wants to consult GI tomorrow and see what they think. The only other thing he is thinking is that this could just be an intolerance to gut feeds. Want to hear something crazy??? Kaitlyn's bowel still has barium dye in there from ten days ago when we had surgery!!

Bacteremia X 2 and GJ Surgery

2010-11-02T07:34:00.002-03:00

Oh my dear! I didn't realize how long it has been since our last post! You guys must be bursting for news! I have been answering e-mails for those impatient for updates ;) Are we still in hospital you ask?? Yes, we are still here, with no discharge in sight yet! Kaitlyn is still on full TPN and is still NPO (nothing by mouth...or in Kaitlyn's case nothing going into her stomach!) not even oral meds! She has also not been getting even pedialyte through her tube, even though we know she can tolerate very small rates, as she does not need the extra fluids right now. Kaitlyn is getting over her second bout with bacteremia! We stopped the meds for the first round of bacteremia (infection in the blood) and we were waiting 48 hours to repeat the blood culture to make sure that we got all of the infection! Kaitlyn didn't even make it to 36 hours before spiking a fever and getting really sick agian! Bacteremia is NO fun! It slams one hard, especially one as weak as Kaitlyn.

SO besides the second bacteremia what's been going on....we left off with Kaitlyn needing an NJ tube placed....

Here's a bit of a journal from the past week and a half:

October 20

Soooooo....we sent down a requisition today for Radiology to place an NJ tube in Kaitlyn and the radiologist sent up a refusal post hast!!!! The radiologist on duty currently is the radiologist that perforated Kaitlyn's bowel almost two years ago exactly! He is outright refusing to place an NJ tube in Kaitlyn even thou...gh our surgeon has recommended the proceedure! The radiologist thinks she is at too high a risk of erosion or perforation of the bowel!! The poor guy is scarred for life because of what happened with Kaitlyn :( Our surgeon is on vacation until Monday, so I guess we will form Plan C at that point! Oh well, it gives us the weekend to go up on Kaitlyn's feeds very slowly and see if she can feed through her tummy! She's on IV Pantoprazole and IV Odansetron.

October 22

A full week on Cisapride.....and where are we... We are able to run pedialyte at 20 mls with zero residuals!! At 20 mls per hour of pedialyte plus her maintenance in TPN she was getting REALLY drooly. We decided to give her some formula yesterday. We double diluted some vivonex and mixed that half-and-half with pedialyte. This makes a 0.2 cal/ml solution!! We turned her rate back to 10mls per hour thinking she would have a harder time digesting the food versus pedialyte.....and her g...ut shut down again!!! UGH!! We had to leave her pump turned off all night last night. This morning we started our day with a fresh batch of half-n-half and started her rate at 2mls per hour. we upped her rate every couple of hours to 6mls per hour. Then we left her at 6mls for three or four hours, and then upped her to 8mls and she stopped digesting. This time there was a lot of green bile coming back. It seems like the Cisapride has worked to increase motility, but whether or not she will ever be able to tolerate rates sufficient to feed???? We have officially been at this for a month now!! We are ready to go home!

Placing the NJ to test the lower bowel would be our preference (and truely the request of our surgeon). However, we will have to speak to our surgeon on Monday and re-evaluate our plan. He may be willing to place the J tube without the NJ. We just need to get home and are trying to work the best possible outcome for Kaitlyn at the same time.

October 24

Fourth day on half double-diluted formula and half pedialyte. We have been able to work Kaitlyn up to a rate of 7mls per hour. Every time we increase her rate to 8 mls per hour she backs up! Our surgeon and pediatrician will be back tomorrow. We will have to devise Plan C. In the meantime we have been dealing with a nasty thrush outbreak :( Kaitlyn's poor little mouth and throat are full of it! I have been suctioning a lot of thick white goop out of her throat! Poor girl has been asking for more vent time too.......no wonder! We have been using nystatin for the past three days and she is finally seeming a bit better today. Boy was she uncomfortable the past two days!! We have restarted her acidophillus today as her tummy is also bloated. It has been a month since she's had anything in her gut to digest! Now she is on her second round of IV antibiotics and her gut is probably stipped of any beneficial bacteria :( Hopefully the acidophillus will help if she can tolerate the additional fluid. Right now she is getting her acidophillus and her Cisapride orally and all other meds are being done IV! We are trying to give her gut the best chance we can!!! Just so ya's know....Enoxaparin injections SUCK!!! I will try to rememberto take some pictures of Kaitlyn's poor little legs when she wakes up from her nap. They are all bruised and ugly looking :( On the bright side....her port now all of a sudden gives blood back!!! It has been suggested that we have the Enoxaparin to thank for dissolving clots/fibrin from around the port allowing blood return :) This is very good news as it only took a few days for the blood to return. This could be an easy treatment for future clots/fibrin in her port!!

October 27

Kaitlyn's stomach has been completely shut down for the past two days :( She got a little distended and uncomfortable and then her tummy just decided to shut down again. For the past two days green/yellow bile has been accumulating in her tummy and she has gaggy spells that force us to aspirate her tummy and toss the bile!!! (AKA vomitting!!!). I know this story sounds all too familiar to some of you :( So what upset me today.....Our surgeon comes by today and I tell him our concerns about the bile/discomfort and intolerance to feeds. I told him we were frustrated about the green in her stomach (as it should NOT be there!!!!) and was wondering if there was another study or something we should be doing to figure out why her stomach keeps shutting down??? He said he wanted to do an upper GI with barium to see if there was any bowel blockage/adhesion (from scar tissue) or any other "reason" why food might not make it through the gut! I reminded him of the dye test that we had already done and he said that he would review that study as well as our x-rays to determine if there was ANYTHING they could attribute to blockage (partial or otherwise!).

Our surgeon comes back and tells us that he has reviewed our dye test and x-rays as well as talked to collegues and radiologists about Kaitlyn's condition. They seem to feel that because she is older now and more "robust"(????) and because they didn't see ANY scar tissue/adhesions/blockages to be concerned about they feel that she could tolerate having another GJ tube being placed. He is really concerned about putting her through another surgery, especially one that would be such high risk. I totally see his point....but on the other hand I am confused as to why he was so against having us place another GJ tube and now thinks that it is the lesser risk??? I hope he comes by tomorrow and explains everything to us!! It kind of makes me a little mad to be honest! If he had reviewed all the tests and done some consults before we discussed surgery in the beginning we could have placed a GJ tube by now and been closer to getting home!! He has also tentatively reserved surgery for Monday morning in case we need it!

We stopped the Vancomycin today for the blood infection. 48-72 hours should be enough to tell if the infection will come back. Let's hope this is the LAST blood infection we ever have!! She was pretty sick! After 48 hours we will repeat the blood cultures to make sure the infection is completely gone!

The only other thing going on the past two days has been a high blood pressure. We have had readings like 135/84, 124/88, 135/102, 140-102, 140-88 (normal blood pressure for a child Kaitlyn's age/height 92/52).....I guess I'm wondering if anyone else has seen this??? During these blood pressures Kaitlyn has been completely comfortable...either sleeping through the entire reading or awake and happy to talk to the "visiting" nurse :)

HR has been back to normal tonight and sats are perfect!! I hope this means tomorrow will be a better day for my girl :) Another interesting point....Kaitlyn had a routine needle change in her port today and the "fluke" blood return is still there!!! It appears as though the Enoxaparin injections have given us back our blood return in our port when two rounds of the TPA didn't work!!! :) I am happy as it is an easy thing for us to try the next time that Kaitlyn's port fails blood return!

October 29

ID doctors believe that the offending bacteria is in her line. There is not much hope to save this line. However, Kaitlyn has been rendered inaccessable for PICC lines and we have already had tw...o cut downs in her left arm. Both legs have been ruined by past blood clots, resulting in too many collaterals for line placement. We have only her right arm left for venous blood draws and emergency IV lines!! We are saving the jugulars for new lines! Right now we are waiting for Monday to place a GJ tube. We are hoping to get Kaitlyn up and running with the GJ as right now her only source of nutrition is TPN. Once she is on full feeds, then she will have had a good run on IV antibiotics. When we are able to stop the TPN it will make it much less likely that the bacteria will return to the blood. If it doesn't come back then we will leave the port and pray that the infection doesn't return if it's not being fed (with the TPN). If we stop the IV antibiotic and the infection returns then we will remove the port and place another when the time is right...ASAP.

November 1

No surgery today :( Our doctors forgot to hold the enoxaparin (Blood "thinner"). It needs to be held for 24 hours before surgery! Also Kaitlyn's pre-op blood work came back with haemoglobin of 75! Normal haemoglobin is 115-145. She would have had a blood transfusion before surgery. Lets hope the sample was just diluted with the extra fluids she has been getting. We have tentative surgery now for Wed.

Well that pretty much catches us up to date for now! Will try to do another post in a few days.

Gastroparesis and Bacteremia!!!

2010-10-20T01:54:00.002-03:00

What's been going on you ask??? Well, we have been battling to get home. Slogging through the many obstacles that keep jumping in our path! First Kaitlyn's tummy slowed considerably. Then it stopped altogether. Then we ended up in hospital. Then Kaitlyn got pneumonia (likely from aspiration--due to feeds backing up in her stomach, causing emesis). Then we started a motility med. Then she got bacteremia (blood infection--likely from her port!). Then we retrialled the motility med.!!! Had to start subcutaneous enoxaparain injections. See what I mean!! Crazy! Who would have thought that one little tummy could cause such horrific trouble :(

I think the worst part of being in hospital (besides fearing what will happen to Kaitlyn) is not being with our boys! It sucks not being able to be in two places at once. I have seen very little of my boys over the past month! They keep asking when we are coming home :( If it weren't for Grandparents we could not do this!!! Tim's dad has been up with our boys from the beginning and he will never know how much we appreciate his help! All the grandparents have had their turns taking care of Owen and Alex. Our boys are lucky to have grandparents that are willing and able to care for them when Tim and I are trying to balance always having one of us at the hospital, Tim's work, and each of us trying to spend precious moments with our boys. We cannot thank the grandparents enough, especially Grampy, for helping us to care for our boys!!! You are making it possible for Tim and I to focus on getting Kaitlyn healthy and back home where we all belong. Without you we could not make the important daily decisions that we are being asked to make, decisions that could seriously affect the outcome of this hospitalisation for Kaitlyn. Thank You!!

Here's a bit of a journal of our past few days. This should bring everyone up to speed that I am not in contact with on a daily basis! For those of you sending notes and e-mails...we appreciate your support, prayers, thoughts, suggestions and concerns for our daughter. I don't know what we would do without our friends and our SMA extended family!

October 12

Increase in feeds.....FAIL! Kaitlyn did really well on feeds yesterday only to stop tolerating the same rate overnight. She backed up 40mls in her belly through the night and had some coughing/gagging fits :( We drained off the 40mls for fear she would have a huge vomit. The color of the fluid was mossy green....bile! This should not be in the stomach. We turned her feeds off and this morning I drained off another 35 mls (also green) that had accumulated :( Stomach feeds have been discontinued. Kaitlyn is also anemic (not sure yet what is causing this) and she now has a fever again!!! We ......are awaiting chest x-rays and will likely end up back on antibiotics later today :( She seems to be refluxing quite a bit so aspiration is suspected. Our surgeon told us that her nissen likely isn't working since her stomach is paralysed! The nissen uses the contractions of the stomach to tighten making it functional! Good to know!!

October 13

What next?? I mean seriously!! Kaitlyn has Bacteremia, an infection in her blood!! All we know is it's a gram positive rod that grew in her blood culture yesterday. We did the culture because of the fever and how sick she looked yesterday morning....and because her chest didn't sound "that bad". The bacteria in the blood culture is not expected to be a contaminant as it grew in only six hours. We are waiting on sensitivities, but in the meantime have contacted Infectious Disease and they started her on antibiotics last night. Kaitl...yn is feeling sick. She is still gaggy and we are having to put her tube to straight drain. This means all oral meds have been stopped. The only good part if this is that it might not be a complete fail on her tummy!! This Sepsis could have caused her tummy to stop working again. We will have to put her tummy through another trial with the Cisapride once she is healthy again.

October 15

Kaitlyn has been responding well to the antibiotics for this blood infection. The sensitivities are back and she is growing Bacillus bacteria and Infectious Disease thinks it is from the port! TPN makes a great medium for bacteria to feed on causing infection! ID says the port shouldn't have to come out as long as she ...continues to get better!! We will do a two week course of antibiotics. Today she has tolerated having her tube elevated :) She is still NPO, but at least she seems to be fine with her own gastric juices now. She has been so nauseous over the past couple of days we had to put her on Zofran (antiemetic). We are ...going to leave her on Zofran until she starts tolerating a bit of pedialyte. We are hoping to start her cisapride again tonight in preparation for another stomach trial. Another issue we have been having since admission is getting her anticoagulated. Kaitlyn does not seem to be responding to or absorbing her coumadin. We have had her on twice her normal dose without any effect on her INR. This puts Kaitlyn at risk of developing blood clots due to her port! The issue now is we have been having some trouble getting her potassium to remain within normal levels. It dropped fairly low a couple of days ago and we are not sure why. We have had to give her a bolus over several hours of IV potassium and we have now increased the potassium in her TPN. This increases her anticoagulation needs!! We are now going to have to start giving Kaitlyn enoxaparin injections to "thin" her blood. Hopefully we will only have to do this for a few days!! These injections are painful!! We place insuflons so she doesn't have to feel each poke, but unfortunately the enoxaparin itself burns when it is injected :(

October 16

Kaitlyn is doing better yesterday and today. Since starting the Zoflan (for nausea and vomitting) she has been much more herself. The poor girl has been nauseous since we were admitted and her gut stopped working. When she got sick with this blood infection it got much worse! Poor thing was gagging about once an hour and getting no sleep! Yesterday I elevated her g-tube to see if she could tolerate her own stomach fluids. We did have her tummy draining. She tolerated this well and we have now restarted her Cisapride (motility med) and pedialyte is once again running at 1 ml... per hour :) Baby steps!!! By Tuesday or Wednesday, provided she remains well, we will trial her tummy on increased rates (Pedialyte only). If she doesn't tolerate the pedialyte then there will be no need to go any further with her tummy. At that point we will call the motility med a fail and move on to plan B.

Plan B is placing an NJ tube. Kaitlyn is no longer a candidate for a GJ tube as she has scar tissue in her bowel. (Scar tissue from past major bowel sugery to repair a lacerated and perforated bowel from a GJ tube replacement that went very wrong!!) Our surgeon has told us that he is not as concerned about placing the NJ temporarily as it is a much soft more flexible type of tubing. After placing the NJ tube, if needed, we will see if Kaitlyn can tolerate jejunal feeds. If she has no issues with jejunal feeds and can tolerate a high enough rate to provide enteral nutrition then we will schedule a surgery (post hast!!!) for a jejunostomy! Once the jejunostomy is placed we should be able to go home fairly quickly!! Within a week of surgery :)

October 19

Kaitlyn has had a visit from her pediatrician, had an hour of Speech Therapy, a visit from Scout the Therapy Dog, and is now enjoying a visit from the Child Life Specialist who is making a counting book with Kaitlyn :) And it's only 11:00am....Busy Girl!!! Today is day four back on the Cisapride (motility med.). It is time to start challanging Kaitlyn's tummy to except higher rates of unflavoured pedialyte! We have only worked her up very slowly (1ml per day) to 5mls and her stomach contents are already getting a bit yellow/green. However, she seems to be tolerating the rate. We will continue to increase her rate at closer intervals throughout today and tomorrow and see how she does. If her tummy isn't digesting even pedialyte by Thursday then we are scheduled to place an NJ tube! The NJ tube will tell us if Kaitlyn can tolerate feeds/rates in her lower GI. If she can tolerate NJ feeds then we will schedule a surgery for next week and place a jejunostomy tube. Then we can work on getting her home ASAP!

Note: We have been asked why we don't place a GJ tube. ....Kaitlyn HAD a GJ tube when she was a year old. It was a fantastic option for us! We fed Kaitlyn into her Jejunum, bypassed the stomach and had decreased risk of aspiration. Kaitlyn had her longest stretches out of hospital when she had her GJ tube too! However, when Kaitlyn had her GJ tube for almost a year the J-portion of the tube was being routinely replaced and the radiologist lacerated and perforated her bowel. Kaitlyn had to endure seven hours of bowel surgery including a nissen/fundiplocation. The nissen would allow us to feed Kaitlyn into her stomach with reduced risk of aspiration of stomach contents. Kaitlyn now has scar tissue in her intestine that makes ever having another GJ tube high risk.

So we have also been asked: What makes an NJ tube safe when the GJ is not! Well the NJ tubing that is used is much softer/pliable than the rigid GJ tubing. There is less risk associated with placing an NJ tube than there is placing a GJ tube for Kaitlyn. The NJ tubing is much less likely to perforate or erode the scar tissue in Kaitlyn's bowel! The other reason is that the NJ tube is temporary/short term and would only be used long enough to see if Kaitlyn's intestine is affected by her dismotility! Our surgeon is refusing to place a Jejunostomy unless we can prove that Kaitlyn's intestine is still functioning. He has our best interests at heart!!

That's it for now :) Hopefully it won't be as long before my next post! Take care

Gastroparesis :(

2010-10-11T01:09:00.000-03:00

Many of you that follow Kaitlyn from day to day have already been updated on this current hospital stay. I have been meaning to update our blog for the few people outside of our SMA community that also follow our daughter. We have had several people ask about her condition. It is easier to write it all down here than it is to write individual letters!! For those of you that have already been updated...skip to the end for the past couple of days :)

Gastroparesis...gastroparesis...it's never going to sound good no matter how many times we say the word. Kaitlyn has been diagnosed with gastroparesis. This is a fancy term that means Kaitlyn's stomach has gone on Holidays and we have no idea when it will return. It means her tummy is paralysed. A tummy that is paralysed cannot contract to help push food along the digestive tract. This means that whatever we put in Kaitlyn's stomach just sits there for a very long time. She is unable to take in any nutrition orally (through her G-tube). Kaitlyn has been receiving IV nutrition called TPN (total parenteral nutrition). This is a kind of "food" that can be infused directly into Kaitlyn's veins. It will provide her with all of the nutrition she needs until we can figure out what to do about this paralysed tummy.

Let me go back a bit and fill you in on what's been going on. Here's a little timeline for you from the beginning until today:

September 23

Kaitlyn has been dealing with really poor digestion for the past couple of weeks. Up until yesterday I was getting about 40-45 mls per hour into her. Last night I could barely get 25 mls per hour into her and this morning she had a tummy full. I had to turn her feeds off. When I checked her tummy at 10am one hour past her normal feeding time...she still had ALL the food from the morning! (We know this because we aspirate Kaitlyn's tummy before addig more volume. Otherwise Kaitlyn vomits). To top this all off, Owen brought home a cold from the first week of school. Now Kaitlyn has it. Yesterday morning she aspirated secretions. Today she is really junky with an off/on fever. We are heading into the hospital.

Sept. 23 (From our local Children's Hospital)

OK...so we are settled into our room. TPN is starting in a few minutes, as her gut has now completely shut down :( I am hoping a day or two rest on TPN and then we can begin feeds again REALLLLYYYYY SLOOOWWWLY. Her chest is getting worse as the evening begins, but blood work and chest x-rays taken this afternoon look pretty good, so we will see where this goes. So far the digestion is by far the worst thing happening! We are just watching the chest right now, no antibiotics yet

Sept 23

We are on complete tummy rest. She is on TPN and all oral meds have been DCed for now. We gave her ranitidine this evening to help her moss colored stomach contents and she spit that up!! I guess she's trying to give us a hint! NOTHING IN MY TUMMY....PLEASE. Right now she is really dehydrated. She is so dry right now that she went from sats in the eighties to sats in the mid-upper ninties. Her tongue is sticking to the catheters :( Kaitlyn's Pediatrician has upped her maintenance (the resident had it set at a very conservative rate). Hopefully she can begin to rehydrate now. We are waiting to decide on antibiotics for her chest when we see how she looks tomorrow morning. I suspect antibiotics will be the order of the day, although my girl has surprised me a time or two in the past ;P Our goal is to restart feeds over the next couple of days and just go very slowly!!

Sept 24

Kaitlyn had a rough night. She was so dehydrated that her RR was in the fifties and her HR was 160's-170's!! She also started with fevers which may have been the dehydration or may have been her worsening chest? This morning she is a little better hydrated (they have been hydrating her slowly due to the severity of her chest). Her RR and HR look better and she seems a bit more comfortable. She is sill having fevers though. A radiographer has reviewed Kaitlyn's chest x-ray from yesterday and the conclusion is that they can't rule out upper right pneumonia and based on her symptoms an upper right pneumonia fits. We have decided to treat her with antibiotics to be safe! We are using IV antibiotics as she doesn't seem to be tolerating ANYTHING orally. We are continuing total tummy rest for the next couple of days. Hopefully once we get this respiratory virus/pneumonia clearing we can begin weaning her back onto stomach feeds.

Sept 25

Kaitlyn is a little more stable respiratory wise this morning. It's amazing how much junk will come out when you are better hydrated! Her sats are still lower than normal, but at least we are in the nineties! Her tummy is still not working! She is not even digesting her own stomach enzymes/fluid. We have had to put her tummy to straight drain this morning as she had a really gaggy spell that scared me. She had over 35 mLs of yuck in there! She tends to vomit when her tummy is bothered!! (Yes, she does have a nissen :/) Her fever broke over night and so far has not come back :) I guess the antibiotics are doing something. Hopefully in a day or two we can start to think of trying her on a little pedialyte?? Once we get her back on feeds we can get out of here! We can do IV antibiotics at home :)

Sept 26

OK...so last night Kaitlyn was really fussy. When I went out to the nurses' station to see what we could do, the nurses were like, "is that Kaitlyn we hear hollering"?? Yup! We ended up doing an x-ray of Kaitlyn's abdomin. Everything looks good. There are no signs of blockages or any anomaly. So since there was nothing on the x-ray, she has bowel sounds, her bowels are moving, and her tummy is soft, I thought well perhaps she is just hungry. She is getting nutrition through her port, but her tummy is still empty. She could still feel yucky with nothing in her belly. We decided to try her on 5 mLs of pedialyte. Well, she seemed to tolerate that so we set her pump on 10 mLs per hour and she stopped fussing and digested the 10 mLs per hour just fine all night long :) This morning she is back to not tolerating the pedialyte. But she is tolerating her tube being elevated (no longer straight drain). I think this is going to be a longer process than I had originally hoped.

We have put her back on her Glycopyrrolate through her G-tube. The alternative is to give it to her through her IV, but then we have to turn her TPN off each time we give it as the two are not compatible. We also need to be able to give her the coumadin tonight!! This will be the fourth night without it, and if she cannot tolerate the oral dose tonight then we will have to inject her with Enoxaparin :( I really don't want to have to start sub-Q Enoxaparin injections. Please keep your fingers, toes and eyes crossed that she wll continue to tolerate at least a teeny bit of pedialyte in her system!! Oh yeah....her chest sounds a bit better today. Fewer marbles rolling around (to steal a phrase from another SMA parent) :)

Sept 27

Kaitlyn had a much better day today respiratory wise. She actually spent a few nonfussing hours awake!! She watched some Dora and tried to talk a bit :) Her chest is still junky, but nowhere near what it was! I think the antibiotics have definitely helped. This morning we decided to try her on pedialyte very slowly!! I started her at 2 mLs per hour. Might seem kind of slow, but I REALLY wanted her to accept the rate! After a couple of hours it was obvious that she was going to be able to tolerate more so I bumped her up to 5 mLs per hour in the afternoon. This evening her rate went up to 10mLs per hour and she stopped digesting once again! GRRR! I guess it is back to a snails pace again tomorrow, if not tonight. We have started Domperidone (motility drug) in an attempt to help her digest her feeds. I know it takes a few days to get into your system, so I will hope it starts helping soon. I still want to be out of here by the end of the week! Hopefully Kaitlyn's tummy cooperates :)

Sept 28

No big changes today :( Tummy is still not working! ...And I so wanted to be home by the end of the week! Tomorrow is Wednesday, so even if she starts tolerating consistent volumes with slow increases of ONLY pedialyte it will take AT LEAST three more days to get out of here. The doctors want to see her digesting at least half her normal rate consistantly of only pedialyte before we start adding diluted vivonex. Then building her up to a rate that nutritionally and from a hydration stand point will be manageable... from home......you get the picture. PLEASE, PLEASE, PLEASE let the domperidone (motility med.) do the trick! We are likely consulting gastroenterology tomorrow.

Sept 30

Kaitlyn's tummy is still on Holidays :( We cannot seem to get even 2-3 mls per hour into her around the clock! She seems to be tolerating us putting three of her oral meds down her tube. I question how much is actually being absorbed when the same volume we put in is still there three hours later!! I have been questioning about going home on TPN! In Nova Scotia going home on TPN simply is not done....at least not very often ;) However, this morning I find out that our nursing agency doesn't even cover patients receiving TPN!!! In other words before we could go home we have to find a new agency and all new nurses! Not to mention getting Gastroenterology to sign off on the TPN so that we could go home at all!!! Please let Kaitlyn's tummy start working!!!

October 1

Bad news!! We have to remain in hospital for at least another two weeks, unless Kaitlyn's tummy starts working on it's own in the meantime!!! They won't allow us to go home on TPN unless she requires the TPN longterm! The gastroenterologist feels that Kaitlyn has gastroparesis :( They are going to request a modified dye test to make sure that there are not any other blockages/barriers preventing fluids from leaving the stomach. This will confirm the gastroenterologists suspicions that we are dealing with gastroparesis (paralysed stomach). The gastroenterologist has recommended that Kaitlyn be put on a med. called Cisapride (instead of domperidone). It is a controlled med. that we have to go through Health Canada to get! We should be able to start this med. by the middle of next week. We then have to wait at least a week before determining whether or not it will do the trick to get Kaitlyn's tummy working again! I am hoping her tummy decides to wake up on it's own!!! If it doesn't work we will have some tough decisions to make. Longterm TPN (AT HOME!!!) or a fixed J-tube (which there are no guarantees would solve our issues).

October 5/6

Two nights ago Kaitlyn had a bunch of pedialyte back up in her tummy. I turned the pump off as it was obvious that she wasn't digesting. A few hours later I checked her residuals and twenty mls of green fluid comes out her tube (bile). This shouldn't happen unless there is a blockage in the bowel. So Sunday we had more abdominal x-rays and the radiologist didn't see anything. This morning Kaitlyn woke up with a slightly swollen tummy (and I mean slightly) and she fussed a bit when we touched her tummy. Our doctors got concerned. The resident then came in and told me that there was an area on Kaitlyn's abdominal x-rays that they couldn't rule out a partial-blockage.

So we got bumped to the front of the line for a dye test this morning. Our doctors were concerned about a blockage that may need surgical intervention. They injected a radioactive dye into Kaitlyn's stomach (through her g-tube) and watched her abdomin under flouroscopy. Flouroscopy is like a live x-ray. You could see the dye enter the stomach...fill the stomach...and then....nothing! They waited a few minutes and then they tipped her on her right side (to facilitate drainage..as the tummy drains better while lying on your right side). Still nothing!! We had to turn her as far over on her right side as we could get her and almost tip her towards her belly before her tummy started to drain. When I say "drain"...I mean on the radiograph you could see a whisp of contrast leave the stomach (like a gray fog) and it rolled into the duodenim and very slowly started to dye the small intestine. It was seriously a trickle and took forever to stain the small intestine enough for them to send her upstairs and wait for the rest of the bowel to stain!!! We had to leave her completely on her right side the entire time. She then had another abdominal x-ray to view the rest of the bowel.

So what did the dye test show?? ...Nothing! There is no obstruction or partial blockage. Truely this is a blessing as a blockage would most likely have meant bowel surgery! In fact we had a surgery consult just before going to do the dye test. I really didn't want to have to put her through another surgery, especially bowel surgery again! In some ways this is still frustrating as we just want/need a reason why all this is happening! The dye test did confirm that she definitely does have gastroparesis. Her tummy did not move, it is completely paralysed.

So where do we go from here? Our plan right now is to try the Cisapride. We have to go through Health Canada for this med. It is a controlled med. in Canada. There can be serious side effects to the heart. We have taken all the necessary precautions though and Kaitlyn does not match ANY of the risk factors. Our heart specialist had to sign off for this med. Kaitlyn will be monitored VERY closely and will have heart follow ups after she starts this medication. This is our last hope before choosing to do something more drastic.

Our choices if the Cisapride doesn't work are to leave Kaitlyn on TPN for the rest of her life. And yes, this can be done. It might not be optimal, and definitely isn't preferred. However, if she is followed closely it can be done. Our other option is to elect to put her through yet another surgery and place a fixed J tube. I am not in favour of a fixed J tube solely because I have read too much about them being uncomfortable at the least. Sometimes they are painful and there is no guarantee that if we did place the tube that her bowel is still functioning well enough to take in enough nutrition even through a J tube! Then we will have put her through another surgery for nothing :( This is the most frustrated and helpless I think we have felt on this entire journey that is SMA!!

October 7

So the new drug we were waiting for was denied!! That's right...the call came in yesterday that the new drug we were requesting release from Health Canada was denied! They wanted us to try some other motility meds. first before they would consider releasing the Cisapride. Our gastroenterologist wrote them back and told them why the "other" motility meds. wouldn't work and that he felt that the Cisapride (in his expert opinion) was the only med. that would work! Today the new med. was approved and it only put our schedule behind by a day! Thank goodness we have some good doctors here willing to go the extra mile for our girl!! Kaitlyn got the first dose of Cisapride tonight so we will see how it goes.

Kaitlyn's sats are a little lower today and HR is a little high. We had an episode last night that I am at a loss of words to explain??? She woke up whining (that high pitched there's something wrong whine). When I went to check on her she had a really high HR (170's) and there seemed like a little froth at her mouth. When I suctioned her (highly unusual for Kaitlyn during the night when on the vent) I got a TON of stuff out of her mouth!! Once I suctioned her mouth her HR went down almost to normal and she seemed to settle back to sleep. I didn't know what to think...did she vomit??? ....did she reflux??? ....had she perhaps aspirated some secretions??? I still don't know what to think!! However, all day today her HR was elavated and her sats were lower than the have been for the past three days. She had increased secretions today that were white and thicker than anything we pulled out of her in more than three days :( She is still on IV antibiotics from the aspiration pneumonia she is getting over. We may have to treat her for a few more days.

We have a chest x-ray scheduled for tomorrow morning. We wanted a healthy XR before discontinuing the IV meds. I guess we will have to see if something shows up! I really hope the Cisapride works to get her tummy moving. We are having a surgery consult tomorrow for a jejunostomy.

October 8

Tim came to the hospital early today. We have a meeting with our surgeon today to discuss a jejunostomy. This is a surgically placed tube (like a gastrotomy or G-tube) that is placed directly into the small intestine. This way we could feed Kaitlyn's intestine and by-pass her sleeping tummy! Our pediatrician came in on her day off to be party to the discussion :) We are blessed with wonderful, dedicated doctors!!!

Our surgeon explained in great detail ( : P Thanks Dr. B) all the different proceedures and types of J-tubes that "could" be placed; and then he explained the type of tube placement that he would choose for Kaitlyn. He recommends and indeed will ONLY place a Roux-en-Y Jejunostomy for Kaitlyn. I won't get into details to explain how this particular surgery is done, but state the name of the type of proceedure in case anyone is curious to do some research :P I would also be happy to answer any private e-mails to the best of my ability!

However, our surgeon won't even touch Kaitlyn until we have trialled all methods to get stomach feeds restarted! We have to trial Cisapride for a week. If the stomach is unable to tolerate feeds after trialling Cisapride then we will have an NJ tube placed. An NJ or naso-jejunal tube is a tube that is placed through the nose, threaded dwn through the stomach and into the small intestine. Our surgeon feels that this tube should be very safe for Kaitlyn even in light of her past bowel sugery and resulting scar tissue!! The NJ tubing is apparently a much softer more pliable tubing than the rigid GJ tubing! He WILL NOT place a jejunostomy in Kaitlyn unless we can prove that she can even tolerate intestinal feeds!! He always has our daughter's best interest at heart! If Kaitlyn can tolerate intestinal feeds and her paralysis appears localized to her stomach then he will place the Roux-en-Y jejunostomy.

If Kaitlyn is not a candidate for a jejunostomy then she will have to go home on TPN for the rest of her life. This would not be the best option for our family or for Kaitlyn!

October 9/10

We are into days 2 and 3 of Cisapride and I am happy to say that we have more bowel sounds and Kaitlyn's tummy is doing some growling that it wasn't doing before!! I sure hope this is a sign her tummy is waking up. We increased her feeds (unflavoured pedialyte) from 3mls to 4 mls on the 9th. On the 10th we increased her feeds from 4mls to 5mls. Five mls was the amount her tummy would shut down on before the Cisapride! She tolerated the 5mls all afternoon on the 10th. She was backed up a bit this evening, so we turned her off for a couple of hours to catch up. At bedtime we turned the pump back on at 5mls per hour and so far she seems to be tolerating the rate :) Fingers and toes tightly crossed we may have a minor breakthrough here folks!!

Kaitlyn had a repeat EKG follow-up today, standard testing for Cisapride use. Her heart will be followed closely during our initial use of this new medication. So far everything looks good :)

That's it for now....I'll keep you all updated!!

SMA Blog Party! What is SMA?

2010-09-16T23:11:00.004-03:00

What is SMA?

SMA stands for Spinal Muscular Atrophy. A disease my husband and I had never heard of until our daughter was diagnosed more than three years ago. Spinal Muscular Atrophy Type 1 (SMA1) is a genetic disorder that occurs in 1 in 4 children born to parents who both carry a recessive gene for it. (1 person in 40 carries such a gene.) There are five main types of SMA, type 1 being the most severe and the most common. SMA affects one in every 6,000 live births. This disease is degenerative and affects the motor nerves, resulting in muscle wasting and weakness. Children born with the disorder gradually lose strength in their nervous systems and become paralysed. The paralysis includes the chest and therefore Type 1 children die from repiratory related illness, usually by the age of two. There is no known cure or long-term treatment.

After learning Kaitlyn’s diagnosis we went home and researched anything we could find on the internet about SMA. The information was overwhelming for us and there were no options given to us from our local hospital about approaches we could take in caring for our daughter. There are really three options when caring for a type 1 child:
-Let nature take it’s course
-Using NIV approach (the method we chose for our daughter)
-Tracheotomy

Kaitlyn has been through a great deal in her short life. She has become dependant on machines to help her breathe, to keep her lungs cleaned out, to feed her, to monitor her 24/7, and to help her move. She has had four surgeries and was intubated for each. One intubation was for major bowel surgery to repair a torn and perforated bowel from a misplaced J-tube. Kaitlyn has never been intubated for an illness (a bit of a rarity for Type 1 children). She has a port-a-cath, an internal catheter, placed for venous access. She has a blood disorder that causes her blood vessels to form clots. She has two holes in her heart (also a bit rare, so we are told).

And in light of all this.....she laughs. Kaitlyn laughs because she is happy, because she is smart enough to know she has a reason....a right to be here! She is precocious, she is determined and she is strong. We adore how strong she is and how she never gives up (AKA stubborn). She is a joy and we are enjoying each and every moment with her!

But what would Kaitlyn wish for....what would she most want, if she could tell us? I think she would ask us for a cure. A cure for this horrible disease! She would ask each of us to do what we can to spread the word about SMA, raise awareness, and help fund a cure for SMA. Well guess what folks...Here's an easy way to help Kaitlyn in our quest for a cure!! Go to: http://www.voteforsma.com/ and vote for the Gwendolyn Strong Foundation in the Jimmie Johnson Helmet of Hope Contest as they are trying to win $20,000 towards a CURE for SMA. Please vote every day until the 29th of September. Ask your friends to join us in voting and feel free to join our blog party and tell your readers about SMA. You can talk about Kaitlyn :)

Thanks for your time, and happy voting :)

Kaitlyn Goes to Pre-school

2010-09-14T20:51:00.008-03:00

Kaitlyn had her first day of pre-school today :) The entire day was packed with excitement! We started Kaitlyn's day early to finish her respiratory care before we had to leave the house. Tim did Kaitlyn's care while I got both boys ready for school and caught the live bird flying around in our living room. .....Wait a minute...did I say a live bird??? YES!!! I walked into the living room to see what had the cats all in a fit! Who was staring me back from the back of my couch but a real live bird!!! The poor thing was crashing into our big picture window in our living room and freaking out as our two cats were staring it down. The poor thing MUST have made it's way down our chimney! I have no idea HOW, but no doors had been open before I discovered him. Anyway, I got a towel and trapped him in the window. Then boys both wanted to see him up close and then I took him outside and he promptly flew away. He paused for a breather in a tree not too far from our deck, but was otherwise unharmed :) It's a miracle the poor thing didn't get hurt!

We made it to pre-school on time and Kaitlyn enjoyed most of her day. She liked the free play time first thing in the morning. I layed her on the floor with a pile of toys and a few of the kids came right over and started playing with her. A couple of girls in particular played for extended periods of time :) Kaitlyn can already say one of their names and was asking for her tonight after supper. She kept saying the little girl's name and then saying please!! Then she called for the little girl.....Oh Iya! Oh Iya! We played with playdough, built block towers, played with dinosaurs (STOMP, STOMP, STOMP...good times:), sat in circle time, painted a picture during group time, and "ate" a snack! Kaitlyn got to "eat" cucumber slices with the rest of the kids :) Then the worst part of the day was watching the rest of the kids line up single file to go out through a door in the back of the classroom that leads to their inaccessable playground :( We got to go outside and around the building to watch the kids play, but could not participate. I think we will have to use this time for something more constructive for Kaitlyn, as it was quite sad.

The plan is to attend pre-school two days a week for now and see how it goes. It is tiring and risky being exposed to so many germs, but to see our girl's face when she got to do what the rest of the kids were doing....priceless :) I am not sure if we will attend for the entire winter or not. I guess we will have to see how the winter unfolds.

We are Driving!

2010-07-28T21:53:00.008-03:00

Kaitlyn's wheelchair has finally arrived! We have a custom pink bubble-gum paint job...custom seating...and very sensitive fibre-optic drive controls :) This has been and is going to be a learning process for all involved. Kaitlyn has been such a trooper through all of this!

Kaitlyn had her first try with the fibre-optic drive controls last week! Like a dummy, I forgot my camera :P I took some photos with my phone camera, but alas I have Vista and Vista does not like my phone :( LOL We had training session #2 today. We are having some minor issues trying to find exactly the right place to put the fibre-optic drive controls so that Kaitlyn can access the lights easily! She has so little movement left...even in her little fingers. With the fibre-optic lights all Kaitlyn has to do is interrupt the light beam with her finger and the chair will move until she moves her finger away from the light. She is doing well recognising that when she interrupts the light, the chair moves :) A little too well...LOL! Her OT was trying to get her to stop on command, and she would ask Kaitlyn to stop. Kaitlyn of course is looking all around and ignores the OT. The OT then corrects Kaitlyn by lifting her finger off of the drive control and Kaitlyn proceeds to holler at her :) As soon as we let her finger go you have to jump out of the way :) Move it or loose it!!!

MDA Walk 2010

2010-06-14T00:15:00.006-03:00

Yesterday was our Walk for the Muscular Dystrophy Association here in Halifax. It was a beautiful sunny day. It was rather hot though :( Kaitlyn gets a little fussy in the heat. She made it through the day though and seemed to enjoy the Walk for the most part :)

There were a lot of people at the walk this year. Most individuals/teams either met or exceeded their goals for fundraising. We are thrilled to be able to give back. MDA has been very supportive to Kaitlyn. They have provided many peices of medical equipment over the past three years. Thanks MDA Canada!!

Here are a few photos of our Walk. Sparky the firedog was at our event :) Sparky paid Kaitlyn a special visit after the walk! She was thrilled...she adores Sparky!!

EHS & Sparky the Fire Dog

2010-06-05T22:21:00.010-03:00

We are in the process of updating Kaitlyn's Special Patient Card with Emergency Health Services. A special patient card is a protocol we set in place so that Ambulance drivers know what to do in the event that we should have an emergency with Kaitlyn and need their services. In setting up this new protocol we have had the pleasure of meeting an EHS supervisor who has taken an interest in Kaitlyn. He has requested that our hospital set up an information day where they can learn more about Kaitlyn's condition :) They have also asked the firefighters at our local fire department to attend. The firefighters are our first responders :)

As a result of our meeting with the EHS supervisor, our local fire chief payed Kaitlyn a visit a couple of weeks ago. He brought us a reflective house number; much more visible signage for EHS and anyone else needing to find our house! They even took the time to find us a sign post, pounded it into the ground and hung the sign! The firefighters also arranged to have our road sign moved. The sign was originally placed on a telephone pole about 15-20 feet from the main road. Thank you very much to our local Fire department!

When the fire chief visited he said that he would send Sparky the Fire Dog over the next weekend to see Kaitlyn :) So today Sparky the Fire Dog walked into a very happy little girl's room!!! She was beaming from ear to ear :) She absolutely LOVES dogs and to see this great big "dog" in her bedroom tickled her :) Sparky gave Kaitlyn a stuffed dalmation dog and a firehat :) She LOVES both! Kaitlyn took the dalmation dog to bed with her :)

The Fire Chief also brought several firefighters and a firetruck over. They opened up all the compartments on the firetruck and let Kaitlyn, Alexander and Owen check out anything they wanted :) This was so nice of them!! Our children don't get to go out as much as we may like so they were thrilled to have a visit from a firetruck and Sparky. They will talk about this for days now :) Thank you again to our local Fire Department! You guys are great!

This is how Sparky arrived for his visit :)

Kaitlyn and Sparky :)

Kaitlyn and her loot :)

She likes big trucks after all...LOL

Sparky was so nice to Kaitlyn

She even tried to wave to him :)

Alex loved the firetruck too!

Firefighter Alex...hehehe

Kaitlyn took "Sparky" to bed :) (She calls her dalmation Sparky)

Ready...Set...Drive :)

2010-03-04T00:56:00.009-04:00

Sorry I haven't written in a while. I wanted to update everyone on Kaitlyn's fundraiser for her power chair!! The fundraiser was a success! We were able to reach our goal. Kaitlyn WILL have her wheels! Our next fun....driving :) Look out brothers, walls, kittens....here she comes! We are thrilled for her to say the least :)

The chair will take a couple of months to build! We are having them build specialized vent trays to carry all of Kaitlyn's machines. She is getting an Invacare base. The vent trays are being built from scratch. Her seating is also being custom built :) It will fit her like a glove. We will of course have to replace the seating more often this way, but it is much more comfortable and supportive for Kaitlyn. She will use fibre optics for drive controls. This will take Kaitlyn longer to figure out than a joystick, but ANY mobility is better than no mobility!!! She will eventually figure it out and drive like a pro!!

I cannot wait to take pictures and video for ALL who have supported us! Speaking of which I want to thank everyone for your heartfelt support! People are extremely generous when it comes to the needs of a child! We had people attend our fundraiser that won the fifty/fifty draw and donate their winnings back to our cause.....people that could have used the money themselves!!! This overwhelming support for our daughter is NOT unnoticed!! We are just so grateful to our entire community for once again pulling together and reaffirming once again why we are so proud to be part of such a small town :) We may have a city address but our hearts are rural rooted!!!

We have also been so proud of our SMA community!! You guys are above and beyond! People facing the exact same struggles as us and they scrape together a donation for our cause!!!! This kind of act is unheard of in other "communities". We are so very greatful for your support. Our daughter would NOT be alive today if it were not for the SMA families and what they have already shared with us! Then this.....it's just so overwhelming for us! We don't know where to begin to say THANK YOU! The thanks will be in our daughter's face when she figures out the freedom the power chair has to offer!!

On another note, we have been battling a string of nasty lung infections! Bacterial pneumonias, back to back, and NOT fun!!! Kaitlyn colonizes pseudomonas (two strains...oral antibiotic resistant!), Stapholococcus Aureus, and Stenotrophomonas Maltophilia! The infections almost always hit in the same way! She starts with increased secretions, developing to a level that she becomes bi-pap dependant to control them. She almost always has fever (high 38's to 42)! Secretions are always at some point green or greenish! Then her lungs get "junky" (wet)! This can sometimes develop over a couple of days or sometimes happens as fast as 12-18 hours!!

She was sick just two weeks ago! This past illness hit her fast! She woke up a Monday morning with slight increase in secretions. She had a fever (39.4) by late morning. By afternoon she was bi-pap dependant! By evening we could finally hear the junk rattling around in her chest...up to that point she had been clear! We decided to keep her home and call the pediatrician in the morning. I had planned to get up with her through the night! When Kaitlyn gets junk in her lungs she needs chest physiotherapy (chest clapping/CPT) and cough-assist every two to three hours around the clock!! After her 2:00am treatment she was maintaining sats of 82-84 on 6 litres of O2 through her bi-pap (ventilator), with increased settings!! We decided to wake the boys and take her in to the ER!

As always she grew the same things in her cultures. She had white cell counts of 27.9 (normal 6.0-15.0)!!! Her platelet count was 107 (normal 150-400)!!! Crazy! This typically indicates severe infection! There has never been any indication of bladder or blood infection. Sometimes her ear (right-the one she lays on) looks infected.

Our question to the experts is can this happen with aspiration (that fast)?? Or does it sound more like bacterial infection in the lungs?? We are wondering if there is anything we can do to prevent these pneumonias!! It is so hard on our girl being sick!! She has been on antibiotics since last September...almost constantly!! Our longest stretch is 20 days!!

We hope everyone else has had a healthier winter so far than us! We are very impatiently awaiting spring and warmer weather!!

Kaitlyn being her cheeky self :)

My boys playing baby!! They pull their shirts down over their knees and waddle around the house =D

Kaitlyn sitting in her floor sitter talking to Owen!

Help Kaitlyn Get Her Wheels!

2010-01-25T14:37:00.015-04:00

We have now officially been fighting for a mobilised wheel chair for our daughter for two whole years. When Kaitlyn was 15 months we started questioning about a power chair as we knew that it could sometimes take several months to get our own chair. We figured that by the time that Kaitlyn was 20-22 months (2 years old maximum) she would have her power chair and she would learn to be mobile. Children diagnosed with conditions that do not allow them to walk should be given some form of mobility around the same age that they would "normally" be starting to walk. It has been shown and printed in articles that children as young as 18-19 months are old enough to learn how to drive a power chair!

We put Kaitlyn in a power chair at around 18 months, and although the chair was a mismatched set of parts she totally GOT how to make that chair move! The parts were borrowed from a local wheelchair dealer and our local hospital. We never could get the drive controls right as we didn't have proper seating. I'll try to explain. Imagine that Kaitlyn has less tone than a newborn baby and is weaker too! She needs full body support from the top of her head to the tip of her toes in order to maximize all the strength she can muster!! Without full body support she cannot use her limited movement to operate drive controls on a power chair.

After several "trials" with mismatched parts it became more and more obvious that Kaitlyn needed a proper chair (seating, hand/arm positioning, drive controls, chair functions!!!). Something that could provide her with the support she needs and the features that would make learning to drive possible! As Kaitlyn's condition is degenerative she has lost a LOT of function in two short years :( This has made our task even more challenging! As a result, upping the price of a power chair.

Kaitlyn's new power chair will have power seating functions (tilt, recline) as she will need to adjust her own chair for comfort! She will drive her chair with fibre optic switches as we could not convince our OT that Kaitlyn would be able to drive with a mini proportional joystick. She needs to be able to move a body part in all four quadrants (left, right, forward, backward). We were unable to demonstrate this with the type of mini-joystick provided for two trials! The good thing is that Kaitlyn's chair will be able to support different types of drive controls, so her chair will adapt to her needs down the road :) This chair will also come equipped with vent trays so that we can lug cough-assist, vent, suction, feed pump and oximeter wherever we go!!

Wheel chairs can often be funded through various funding programs. However, since Kaitlyn's chair requires such an elaborate set-up and sensitive controls the price tag is much higher than most chairs. We have managed to raise the majority of the cost of the chair with the help of our local children's hospital. The major funding support comes from PC Children's Charity in the sum of $20,000!!! To all of you who have supported the Superstore when they have their yearly Charity drives (when they ask us at the checkout if we would like to donate 1-2 dollars to their Children's Charity) I thank you from the bottom of our hearts. Now you know where that money goes!!! Right back into the community :)

We were told in January that we need to raise the remaining funds needed for Kaitlyn's chair. The Children's Charity funds must be used before the end of six months from the time it's awarded. We got an extension, but that still only gives us until mid-March! As such, this fundraiser means a great deal to us as it is our last chance to get Kaitlyn her wheels!!

My mother and sister have been organising this fundraiser. They are working tirelessly to make sure that this event is a success! They have decided to have the event in Annapolia Royal at the local Firehall. The date is the 21 st of February, 2010 starting at 1:00pm. They have arranged for local musicians:

Fender Benders - Country/Rock

Janis Sheridan-vocals

Jacquelyn d'Eon - Fiddle

Matthew Stanton - Guitar/vocals

Cecile Martell - Guitar/vocals

And more...

We will also be auctioning off a few items, selling tickets on some items and a bake sale, among other surprises :)

Please come and join us for an afternoon of fun! Kaitlyn and I cannot attend the fundraiser in person, but we are hoping to be able to attend via Skype! We will be able to see everyone and everyone will see us through the web camera on my laptop :) If you cannot make the benefit and would like to make a donation you can mail it to "Kaitlyn Hatchard c/o Kathy Horton PO Box 316, Bridgetown NS, B0S1C0" Any check donations may be made payable to Tim or Kimberley Hatchard. "WE NOW HAVE A PAY PAL ACCOUNT!!" Just go to the link on the top right of this page :)

The New Year Catch Up!

2010-01-08T21:49:00.008-04:00

Oh my goodness!!! I think I left this post too long :) OOooppss! Where to start!

Christmas was great!! We were all very briefly healthy :) All three kids "got" Christmas this year. They were all excited to receive gifts from Santa. Owen kept saying, "I have to be good, 'cause Santa's watchin'....right Mom"? HEHEHE Alex was just excited :) And Christmas Eve we were getting Kaitlyn ready for bed and we were telling her that she had to go to bed and go to sleep so that Santa would visit! She said, "Santa....present....doll-doll"! Let's just say, Santa delivered :O) She is doing really well with her verbal speech these days.

Kaitlyn finished her last round of antibiotics a couple of days before Christmas. This is our longest stay off of antibiotics since early September (for recurrent chest infections/bacterial pneumonia). Currently we are dealing with a head cold. Sniffles and coughing all around, but Kaitlyn is handling it like a pro! So far no chest infections (fingers and toes crossed please people)!

Kaitlyn finally got her much needed floor sitter. Our local children's hospital made her chair. It is molded specifically for Kaitlyn and we have adapted the chair to meet her every need. When Kaitlyn wears her body brace she is supported from head to toe in this chair :) The floor sitter also has a clear plastic tray. Kaitlyn LOVES to sit up in this chair! She cannot tolerate long periods, but I think she digs the view :)

Kaitlyn was approved for RSV shots again this year!! YAY!!!! This is huge for us. RSV is an incredibly dangerous virus for any respiratory compromised individual. For someone like a child with SMA it can be deadly! (Seriously) RSV shots are not a guarantee that one will not get the virus but it does provide protection! Kaitlyn had RSV last year while receiving the shots but her symptoms of the virus were far less than that of any of the rest of our family. The rest of us were really sick. I don't EVER recall being that sick!!! We are convinced that the reason Kaitlyn faired so well with the RSV was because of the RSV shots! She gets two shots (a divided dose) one needle in each of her legs every month from the beginning of December until the beginning of April! This is the worst of the RSV season for our region. This is not fun...but necessary!

Kaitlyn has been approved for an LTV 1150 ventilator. This ventilator has the ability to deliver higher pressures than what Kaitlyn's current bi-pap (ventilator) can provide. It is said that the vent provides better ventilation for our SMA children. I guess we will see. The machine is a little heavier and slightly bigger than what we have now, but we'll adapt. We like that it has an internal battery for transport and for when Kaitlyn is sick. It will come with it's own stand to make it easier to move from room to room :) Our goal is to keep Kaitlyn's current bi-pap to have as a back-up vent in case the new LTV should ever fail :(

Speaking of failing back-ups!!!! Kaitlyn's newest cough-assist is broken. Apparently there is a broken spring inside that needs replacing. The replacement is simple, but the cough-assist has to go to the States to be fixed!!! This means that we are without a back-up cough-assist for several weeks. A few months ago our hospital only had one cough-assist. I think they may have a second now, but they couldn't lend us one should Kaitlyn's primary cough-assist fail :( Our home care company that takes care of our home equipment needs doesn't even have a cough-assist so we cannot get one from them!!! I really hope our secondary cough-assist machine makes it to and from the States safely and quickly!!!

I think that's everything for now ;)

Kaitlyn sitting in her floor sitter. All ready for bed :)

Kaitlyn sporting piggys and having fun sitting up :)

Christmas morning :)

Owen discovering Christmas gifts ;) Don't touch....have to wait for sissy!!

Alex discovering gifts! Sissy's almost ready!!!

Kaitln with some of her loot :) Happy girl. Want to know what she is smiling at....???

Kaitlyn's favourite Christmas present.....Owen's toy dinosaur that walks and roars :) :)

Kaitlyn with more gifts all cozy in her bed :)

Ear Pillows

2009-11-26T09:40:00.004-04:00

About a year ago now Kaitlyn started having continuous problems with breakdown on both ears from having to constantly lay on them. Even off-loading by rolling her from side to side didn't work. We would get raw skin that would lead to infections even though we kept the skin as clean and dry as humanly possible! At one point our infections were so bad we had to see plastic surgery as we thought that the infection might be in the cartilage of the ear!!

When our pediatrician told us how serious the infection could be if it were in the cartilage of the ear we knew we had to find a way to get Kaitlyn's ears up off of the surface she was laying on! I thought that if Kaitlyn were to lay on a pillow with a hole in the center then she could still be sidelying while having her ear completely suspended (zero pressure)! (Sidelying for a child with SMA type 1 is essential as they cannot swallow. It allows them to pool secretions in their cheek and even spit out secretions so that they do not aspirate.)

I like to use flannel fabrics when making my pillows. I find flannel absorbs moisture quickly and washes really well. The flannel is also soft and I find it irritates Kaitlyn's skin less. I started with a circle of fabric slightly larger than Kaitlyn's head. For us at that time a dinner plate worked great as a template for marking my fabric. Any circle shape the right size will work to make a pattern for cutting out your fabric. When cutting out my circle I always fold my fabric in half with the right side in. This way I cut out both sides of the pillow at the same time. I then take these two circles with the right side still in, and I sew around the outside with a sewing machine.

I then fold the circle shape in half and cut out a small semi-circle in the middle. The hole in the center of the pillow does not need to be any larger than your child's ear! The smaller the hole the easier it will be to position your child on the pillow when it is finished. I always find that if I cut out the hole to fit Kaitlyn's ear then the finished product will be slightly larger than her ear.

I then turn the fabric right side out and begin sewing up the ear hole. For Kaitlyn's pillows I like to turn the fabric in and sew a nice smooth edge (seamless). This way there is no edging to rub against her ears. Once I have the hole about two-thirds closed I then stuff the pillow with a hypoallergenic polyfoam stuffing. I like to use a proper pillow stuffing as it holds it's shape quite nicely through continuous washings! Once the pillow is stuffed full I sew up the rest of the ear hole. I stuff my pillows full but not too firm!

I have had several families ask me recently how I make Kaitlyn's ear pillows so I thought that I should just post how I make them :) I hope this helps any families interested in making their own pillows! I know for us Kaitlyn is on an ear pillow 24/7 now!!

PICU and Another Lengthy Hospital Stay

2009-11-23T23:49:00.004-04:00

Remember when Kaitlyn was sick with a pnuemonia back in September?? Well it would seem that we never really ever got that cleared up!!

Kaitlyn was on ciprofloxacin (broad spectrum antibiotic) back in September for an illness that seemed bacterial in nature. We know Kaitlyn colonizes pseudomonas in her lungs so cipro is always our drug of choice when she gets an infection. Well back in September cipro didn't work to get rid of the infection. Kaitlyn ended up hospitalised on IV antibiotics. The IV antibiotics were given for two weeks (one week at home) and then another eight days on two broad spectrum oral antibiotics!

After about a week Kaitlyn started with increased secretions again. So we put her back on oral ciprofloxacin. She seemed to respond nicely to the antibiotic this time and we breathed a sigh of relief! However we did run cultures also and the cultures grew heavy growth psuedomonas and heavy growth Staph. Aureus among other bugs. However since Kaitlyn seemed to be getting better we assumed that the buggies weren't infectious but rather just colonized in the lung :(

We finished a ten day course of antibiotic at home. After which Kaitlyn was healthy for two whole days! She went from healthy and happy sitting up and acting normal to wet and junky lungs in twelve hours!! We chose not to expose Kaitlyn to all the sick kids flooding the ER late at night. I stayed up and did therapies every three hours all night last Monday night. In the morning we took Kaitlyn to ER. It would seem that she didn't appreciate being moved! She crashed down into the seventies a couple of times in ER and was having a rough time coming back up! We increased her vent settings and bled some O2 through her bi-pap (it took both to bring her up to 90% O2). Typically we don't like the O2 through the bi-pap, but Kaitlyn's PCO2 level was fine and our goal was to discontinue the O2 as soon as possible!! They accessed Kaitlyn's port and began IV antibiotics right away. I'm glad that they did. She was running a high fever and we believed that she probably had something bacterial as nobody else in our house was sick.

Because Kaitlyn was a little unstable she made the nurses and ER doc a little nervous I think. They decided to send her to PICU. We have not been to PICU for an illness in over a year and a half. As it turns out PICU was an absolute nightmare!!! H1N1 EVERYWHERE!! And me without my vaccine yet...EEEPPPPS! Needless to say I was washing my hands like crazy! As luck would have it, Kaitlyn responded well to treatments and we got rid of the O2 through the bi-pap the next day. We did cough her with O2 for a day longer. X-rays showed a pneumonia in Kaitlyn's upper right lung. She also had some infiltrates throughout the lungs (areas looking mucousy/wet).

After spending one night in PICU we were able to move Kaitlyn to regular floor. This way she could be more closely followed by doctors and specialists that know our daughter best! Also Kaitlyn's pediatrician works in our children's hospital. This is great for us, as any time we are hospitalised we are admitted under our pediatrician's name :) Not a soul knows Kaitlyn like her pediatrician, and while she is not an SMA doctor we love her as she is willing to work with us and listen to our input!! As you know this is huge for our SMA families....just having someone who will listen to and try some of our unconventional ideas :P

Since last Tuesday we have been waiting for Kaitlyn to show some signs of improvement! Her fever is gone, she is not needing O2 in any form and her bi-pap settings are back to normal. But...she is still junky! We took an x-ray yesterday that showed the pneumonia in the upper right lobe has cleared (there's no consolidation anymore). But there are infiltrates throughout the lungs and appears in particular to have spread into the lower right lung! URG!!! In a consult with Infectious Disease yesterday, they confirmed that Kaitlyn is on the right antibiotics to cover the bugs she has...so why is she still sick?? Today we have to redo cultures and they also want to redo her H1N1 test and add some plasma tests (?). You know I almost hope that they find something viral!!! Crazy how we used to wish for Kaitlyn NOT to get a virus! Throw in a couple of super bugs and all of a sudden we are praying for the viral CRAP!!

Hoping my next post is from home...

New Ramp!!

2009-11-11T22:57:00.009-04:00

We bought our new home and moved in back in August. The first thing we knew that we had to somehow make happen was wheelchair accessibility for our new abode. For three months now Kaitlyn's Wheelchair/medical stroller has had to live outside in our van :( Banished alongside the stroller was our back-up cough-assist and true sine inverter (needed to run said device). This equipment is very expensive and difficult to procure. With snow and cold weather threatening to arrive we have been worried about the equipment being stored in our van.

Tim spoke to our Social Worker at our children's hospital about needing a ramp and asked if we could get any help building. Our social worker wanted to know if we needed help building or funding supplies. We of course said that we would be happy with whatever help we could get! Tim and I were hoping for some guidance and expertise in how to build an accessible ramp. A few short weeks later our social worker told us that we would have a couple of contractors come out to assess our needs for a ramp. We had high hopes that the pair would give us some expert advice on how/where to build our new addition.

Then two weeks ago we had a call from the contractor saying that we would be receiving supplies to build a ramp and they would be out shortly to begin the build. Before the call we had no clue that any arrangements had been made for a ramp to be built!! We knew that our social worker had been looking into getting some funding for a ramp, but we had no idea that the funding had been found!

Our supplies arrived a couple of days later and the contractors the day after! They were wonderful! They were even kind and patient with our boys, who just HAD to be out there observing their every move :) The contractors were here for three days and finished our new ramp :)

It is so hard for us to put into words how we feel about having this ramp built for us! Unless you have a child like Kaitlyn and truly understand ALL that she entails each and every day, then it would be hard for you to realize just how much a simple ramp can do to make our lives that much easier! Kaitlyn has frequent appointments at our local children's hospital, not to mention the very few family outings we get together. Taking her out of the house is a huge undertaking!

Before the ramp we had to carry supplies out to our van and load up Kaitlyn's stroller piece by piece! This took several trips back and forth to the house lugging everything out by hand. When Kaitlyn travels out of the house we have to take ALL of her equipment with us. She is a vent dependant child with an extremely unstable airway! The last step was to carry Kaitlyn out in my arms, climb into our non-accessible mini-van, work my way around her stroller and get her settled without breaking any of her limbs! Kaitlyn is a big girl and carrying her in tight spaces is becoming increasingly difficult!!

With the ramp we can load up Kaitlyn's stroller, supplies, machinery and angel baby :) Then all we have to do is stroll her out, down the ramp and into the van! This is just so much easier. Especially in the bad weather!! It is also so much safer both for Kaitlyn's equipment and for Kaitlyn :) With the ramp, an outing of a few minutes can be done without a second thought, when without the ramp it wasn't even considered. This is a huge plus for a little girl whose first words in the morning are "go, go, go"!

Supplies for our new ramp :)

Supplies unloaded....ready for the contractors!!

First snowfall of the winter season covering what was accomplished Day 1 of the build.

Our finished ramp!!! :O)

Some recent pics of the kids:

Feeling Better Again

2009-11-02T23:35:00.003-04:00

Kaitlyn has been feeling much better the past two days! We are back to normal bi-pap times and secretions are much more manageable. I can finally carry her around on my shoulder again and boy is she taking advantage!!!

Kaitlyn goes to get her TLSO tomorrow. A TLSO is a body brace (like a rigid corset) that hugs Kaitlyn's torso so that she will be able to sit up without slumping all over!! It should help make it easier for Kaitlyn to breathe and make positioning her easier. It will help us keep Kaitlyn's back straight to avoid or prolong any surgeries to correct displacement in the spine! Currently Kaitlyn has NO noticeable curvature of her spine :) Our OT/PT is finally convinced that she needs the additional support when they tried her in the new floor sitter they are having made for her! She just collapsed into the seating...they have never dealt with a child with such low tone before! We are all learning as we go.

We are also trialing a couple of options for drive controls for a powerchair tomorrow. One is the mini proportional joystick. Kaitlyn hasn't done extremely well with this joystick :( They have left it so long that now she has lost almost all function in her little hands :((( The second option we are trialling is fibre optics :) The fibre optics are little pinpoint sensors that can be placed anywhere that Kaitlyn has predictable movement. The biggest problem with driving a powerchair like this is that it could take a LOT longer to learn.

The biggest problem by far is not having a proper chair for Kaitlyn to trial this sensitive equipment. They are having a really hard time figuring out how to position Kaitlyn's hands to give her the best function to be able to access the drive controls! Wish us luck that we can figure this all out. I am getting really tired of listening to Kaitlyn cry everytime Alex or Owen gets up and leaves the room and she cannot follow!

Increased secretions, slow digestion....You know the drill!

2009-10-25T07:17:00.003-03:00

I was hoping our next post would be something exciting, something nice....something "normal"!! Kaitlyn has had increased secretions for the past week and a half almost two weeks. It started out like a head cold. Increased secretions were almost entirely from the nose. She has had no fever and has not been bi-pap dependant. Her spirits have been good and she has been acting normal, just extra junky.

Well yesterday it finally happened, she stopped digesting her feeds! Friday Kaitlyn was fine, digesting fine and acting fine except for the increased secretions. Then yesterday morning I gave Kaitlyn her first morning bolus and no issues. Then I fed her again and when I went to feed her a third time she still had all of her last feed in her stomach! I knew right away what was happening! Everytime Kaitlyn gets sick now it stops or severely slows her digestion. It seems as time goes on that it is taking less and less provocation for her tummy to shut down :(

The first thing I always do when digestion slows is I take her formula back to a base mix. Kaitlyn's normal formula contains fruit juice, fruits, supplements and her powdered formula. When she is sick and is digesting poorly we take out all of her fruit juice and fruits. We then add extra pedialyte and this usually helps her food move through her system better. So yesterday I remixed Kaitlyn's food and fed that to her. She still wasn't digesting!!! After we put Kaitlyn to bed Tim went out to get more pedialyte and we gave her straight pedialyte.....finally!! The pedialyte started moving through her. At this point she was pretty dry.

We then gave her pedialyte and mixed in some of her bland formula. This seemed to work! Hopefully we can keep her gut working in the right direction. Thanks to the suggestions from some of the other SMA families I have a couple of things I can try if she stops digesting again!

Her cold does seem to be getting a little worse, but she is not presenting any differently than the rest of us. We'll see how she does over the next day and decide whether or not to take her and have some cultures done. She is nowhere's near needing to be admitted unless she needs fluids!

Home Again

2009-09-23T22:20:00.010-03:00

Sorry for the long time between posts!! Kaitlyn got home last Friday the 18th of September. So what happened.....

Kaitlyn's sputum cultures (mucus from her lungs) came back from the lab positive with both Pseudomonas and Stapholococcus Aureus bacterias! Both of these buggies are serious on their own. Not to mention hard to treat. Kaitlyn colonizes pseudomonas. This is not something that anyone with a normal respiratory system would be susceptible to growing in the lungs. Kaitlyn grows two strains that are both now resistant to the oral antibiotic that we usually use to treat pseudo at home. This medcation is really the only type of oral antibiotic effective against pseudo!!! GREAT! The Staph buggy also turned out to be a fairly resistant strain. It was the Staph that Infectious Disease figures was the cause of Kaitlyn's illness this time around. I think this is the longest illness we have dealt with so far!

In hospital Kaitlyn was placed on two IV antibiotics to get rid of the buggies. After about two days I started asking about getting things in place so that we could take Kaitlyn home and administer the IV meds at home. At first they were telling me that there was no way that we could go home with IV antibiotics....I raised my eyebows and said that I knew for a fact that people DID go home on IV meds and fluids and even TPN when needed!!!! I was only asking for the same rights!

When we are in hospital we do ALL of Kaitlyn's care. The nurses are not familiar with Kaitlyn's equipment, including her cough-assist, and especially her bi-pap. These things are not extremely complicated peices of equipment, but we would have to teach their use twice a day with shift changes and then it takes TIME to get familiar with Kaitlyn specific techniques. We usually spend a good two weeks to train our home nurses to care for Kaitlyn when she is healthy!! It's a whole new ball game when Kaitlyn is sick!!! The "rules" change...she becomes a LOT trickier to deal with. That said...we find it soooo much easier to be at home when Kaitlyn is sick but relatively stable!!! It disrupts our whole family when we have to go to hospital :o(

In the end they agreed to let us home on IV antibiotics as long as our regular nursing service could arrange to deliver the meds. I did learn how to administer the meds myself, but did not take it upon myself as they took away all of our regular service. Tim and I had been working around the clock for three weeks at this point and the last thing I needed was more work!!!

IV antibiotics were finished this Wednesday and Kaitlyn has been switched to two oral antibiotics. One covers her pseudomonas somewhat and the other is knocking the stuffing out of the Staph buggy :o) Our goal in leaving her on antibiotics (hefty antibiotics) is to completely knock out the Staph buggy!!!! The last thing we need is for her to colonize such a NASTY resistant bug!!! ID (infectious disease) had a hard time finding antibiotics that would cover all of the bacteria that Kaitlyn was growing in her lungs. She was even resistant to the newer generation antibiotics that they wanted to put her on!!

So now we are trying to get back into our "normal" routine. It's tough for all involved, especially since Tim and I are still trying to catch up on lost sleep! We are just so glad to be back home! Here are some recent pictures of the kids.

Kaitlyn is REALLY enjoying these kittens! She loves it when they come lay down with her. This little black and white kitty LOVES laying beside her and doesn't even budge or wake up when we turn the suction machine on!!!

Alexander loves the kitty too! This little kitten doesn't mind when they pick him up and cuddle him....he doesn't have enough sense to run :o) But seriously the kids are soooo good to him.

My boys outside in their PJ's playing in our yard one weekend. Tim and I didn't have time to fight with them about getting dressed....so out they went. They'd live in PJ's if we let them ;)

I love my diapers!! This is how I found Alex one morning, laying on top of Kaitlyn's diapers. He looked like he was sad that he no longer needs them :o)

This is Owen on his first day of school.

In Hospital

2009-09-12T11:26:00.007-03:00

Where to begin....

Kaitlyn had her Port-a-cath surgery August 27th and like I said everything went just fine! She came home and had a slight increase in secretions which is normal after being intubated. After about 3-4 days she is usually back to her baseline. By the Monday (four days) post surgery Kaitlyn's secretions were on the rise and not getting any better. She also started with a bit of a cough on Monday. By Tuesday she was bi-pap dependant, but not because she was desatting. She would go into fits of continuous coughing every time we took her off of bi-pap. Secretions were still on the increase and we thought that perhaps she was getting a cold. By Wednesday post surgery Kaitlyn spiked a fever of forty. Considering she just had her port placed we worried about infection in her blood so we brought Kaitlyn into ER to have them run some cultures and figure out what was wrong.

The cultures from ER all came back negative and in the meantime we had taken Kaitlyn back home to care for her. So from Wednesday until the following Monday (1 week since onset of symptoms) she still had fever that would spike to forty every time we took her Tylenol away and she was still obviously junky and sick! On Monday (Labour Day) we took Kaitlyn into ER again as she obviously wasn't getting any better. She had even come down with a new issue of not digesting her feeds very well. Kaitlyn was started on Ciprofloxacin as it is the antibiotic of choice due to her colonization of a particularly nasty bacteria pseudomonas.

After starting an antibiotic Kaitlyn usually responds very well to treatment. Within a couple of days we can witness a remarkable change in her symptoms. This time after five days of antibiotic treatment in our home she still wasn't getting better. She was only digesting about 1/3 her normal volume of fluids. We also had to take Kaitlyn's normal formula down to a base mix, which means that she was getting a lot fewer calories and less of all other nutrients. We did this as Kaitlyn won't digest her feeds otherwise. So we brought Kaitlyn back into ER yesterday (Friday) as we really felt that she needed IV antibiotics and IV fluids.

Our stay in ER was really ridiculous! They kept us there for a total of six hours! SO much for her protocol!!! Five hours after waiting around ER the attending pediatrician from the floor told us that they would admit us for the night, but that they didn't want to change any of her meds. and they just wanted to observe her through the night!!! I told the physician that we've BEEN observing her or the past five days at home and she is not getting any better. I said that if I wanted to observe her for one more night then I would have kept her at home!!! There was a resident shadowing the physician and I thought his eyes were going to pop out....hehehehe!! Long story short they called Kaitlyn's pediatrician who is in charge of one of the teams at our hospital and whom Kaitlyn is always admitted under!! She told them to start the IV meds. but left the fluids up to them. So IV meds were started Friday night and fluids were not.

This morning (Saturday) Kaitlyn is about the same. Because they didn't start the fluids like we had wanted she was pretty dry...so she sounded great when the nurses listened to her. This as we know can be dangerous as if she gets too dry we may not be able to mobilise the junk in her chest to be able to get it out! When Kaitlyn's pediatrician came in to see her this morning she wanted her started on IV fluids. They don't want to give her much, just enough to keep her better hydrated so her secretions won't be so thick!

Overall I think that we needed to be in hospital and now that we are here she will be followed properly so that we can figure out how to keep her from getting sick because of pseudomonas. We may have to start some sort of preventative regimen in order to keep her healthy! Oral cipro alone doesn't seem to be working for her anymore. I'm glad that she will be followed by Infectious Disease from now on...we have been asking for this for some time now! Infectious Disease wants to leave her on IV cipro until they can figure out exactly what it is Kaitlyn seems to be culturing and then hopefully we can check for sensitivities and get her on one or more antibiotics that will knock those buggies out of her!!! Infectious Disease also wants to run a host of other tests (not the standard run of the mill). I am much happier today knowing that they are finally going to DO something to help.

I have observed today that Kaitlyn's tidal volumes are pretty low (60-80) depending on her leak!! That's pretty low for a child her weight. I have also noticed that she is paradoxical breathing while on her vent, and this shouldn't be the case. So far her blood gases are good, but we may have to increase her pressure settings to make her more comfortable so that she isn't having to fight so hard to breathe!!! No wonder she has been sleeping almost 24/7 at home! I don't know how long she has been breathing this way either...I hope not long. I always wonder how much I fail to pick up on when we are home...as I have more responsibilities at home and more distractions. When we come into hospital Kaitlyn is my number one concern as I know my boys are being WELL cared for (Thanks to Grammy and Grampy!!!).

I'll keep everyone posted on how we make out! Right now we are where we need to be!!

Port-a cath....Check!

2009-08-30T20:58:00.006-03:00

Kaitlyn had her port-a-cath placed this past Thursday! She made out fantastic!! Her surgery took about two hours and she was taken straight to PICU afterwards. We wre allowed into PICU before they extubated her. I wanted to be the first to assess her before they pulled the intubation tube. She was starting to wake up, looked good and lungs sounded clear so we went ahead and pulled the tube! We put her right on bi-pap and she maintained sats of 100% (perfect). Overall this surgery has been one of our easiest :o) She had a bit of discomfort for the first couple of days from the incision in her chest and the cut down on her neck, but nothing that couldn't be managed with a little bit of tylenol.

Because Kaitlyn has a history of blood clots (in both groins...both venous and arterial!!) she will need to remain on anticoagulation meds for as long as she has an indwelling catheter! It seems as though this may be the toughest part of placing the port for Kaitlyn. The haemotologist has her on warfarin to extend her clotting time to prevent clots forming around her indwelling catheter. For the first 24 hours warfarin acts as a coagulant instead of an anticoagulant!!! So we had to have Kaitlyn back on Enoxaparin injections. We were allowed to bring her home Friday night, but only on a pass. They couldn't discharge her properly as they needed to check her INR levels Saturday and the labs aren't open on th weekend for outpatients. When they checked her INR levels (to see what her clotting rates were) her level was 8!!!!! They want her level to read between 1.5 and 2!! Crazy. She had to have a dose of oral vitamin K through her G-tube and we had to stop both the Enoxiparin and the warfarin for Saturday night. Then we got to come home Saturday night again on pass from the hospital as we had to take Kaitlyn back into the hospital to have her levels checked again Sunday morning! Kaitlyn's INR level was 1.6 Sunday morning which is exactly where we want her.

This also means that we were finally discharged from hospital and Kaitlyn is back on the warfarin. We have to have her levels rechecked on Tuesday and I'm hoping that her levels are fine by then too. Once she is regulated on the warfarin she will only need once a month checks to make sure that her levels are fine. Her levels shouldn't change too much from day to day as Kaitlyn's diet never changes. The only time we will have to have her levels checked more often would be when she is ill or when she is taking an antibiotic.

Anyway we are thrilled with how she has done so far and are hoping she continues to do well with this port! It sure will make things easier for her when she needs venous access!

Settled!!

2009-08-22T22:29:00.008-03:00

We are almost completely moved into our new home!! The kids are loving it :o) The boys are spending time running in and out of the house, and Kaitlyn is even getting outside more. Just not on days that are too hot! We still need to figure out some way to build a ramp!! We cannot bring her medical stroller into the house! This is a huge pain as it is a good spot for her to sit sometimes :o(

Our two new additions are settling in just fine :P They are quite the household terrors now! In case anyone missed it...we now have two new kittens in our house! Owen and Kaitlyn are smitten!!! Alex just wants to put the sleeper hold on them :P Poor Kittens!

Kaitlyn is scheduled for surgery for 8am on the 27th of August! We only decided this the beginning of August, and as always our hospital made room for our girl!! They seem to really love her there :o) Our surgeon is the best and we have full confidence in him. He won't take her into surgery before making sure that he has a particular team in place for surgery, anaesthesia, and PICU!!! We love him! Kaitlyn is having a Port placed. A port-a-cath is a tiny medical appliance that is placed under the skin. A catheter connects the port to a vein. The port then sits just under the skin and can be palpated and used for central venous access. What does this mean for Kaitlyn? It means that Kaitlyn would never need to feel another needle poke! A blessing!! The skin over the port can be frozen with a gel; then a needle would access her port without her ever needing to feel another poke.

This will save us a lot of worry!! We will never have to worry about blood work ever again. We will finally be able to have Kaitlyn's blood drawn for genetic testing for a blood disorder!! (As she develops blood clots). It will be a huge relief when the port is placed and it is all said and done.

Our two new arrivals!! Introducing Blacker (or Blackie) and Spider!!

Owen learning to play with his new Kitty :o)

Spider is a little more timid...or is it SMART!! hehehe

Looks like Owen is doing a good job. Kitten is Smitten :o)

Kaitlyn in her new home.

Owen showing Kaitlyn Blackie!

Spider is coming around :o)

The Lawn Mower Parade :oD Around and around and around!!! They just wouldn't stop that night!!!!!!!!!!

Our New Home

2009-08-09T23:34:00.005-03:00

We spent the weekend days at our new home!! YAY! The kids ALL love it there! On Saturday I think we were at our house for a total of about twenty minutes before there was a knock at the door. A neighbour boy (eight years old) had heard tell we were moving in and had children :o)Then as I'm walking from kitchen to back deck to check on the kids....what do I see leaning against my house peering into my kitchen?? A big golden retriever dog...wagging her tail and obviously very used to my new home!! She didn't seem to mind that I didn't look/smell like the old owners!! She was very friendly and (like all goldens) lo ved the attention from the kids :o) When we bought our house we had no idea that the price included a neighbour boy and another neighbour's dog!!! Both kid and dog spent the entire afternoon with us :o)

It's funny how negatives about a potential house can take a backseat to all the positives you are focusing on. There are a few things about this new house that will need to be upgraded...some fairly soon and others will have to wait a couple to a few years! The kitchen sink is aweful!!! Pretty grungy looking and very scratched up. One of those sinks you look at and cringe at the thought of having to do dishes in it...not to mention Kaitlyn's things!!! The main bathroom needs to be redone (but this will have to wait). It is serviceable, but the tub does have a couple of chips in it :o( There is a carpet in the master bedroom that I somehow hadn't noticed when we viewed the house!! And the carpet is REALLY grungy!! It's one of those carpets, it's so aweful that we might have to rip it up and walk on the plywood boards for now until we can afford some decent flooring (NOT CARPET!!!!!). The finished rooms in the basement need upgrading, but can wait. there are carpets in the living room and dining room that I would eventually like removed.

In the backyard I found a wonderful pot-bellied stove :o) I really like these little stoves. They are great for outdoor social gatherings and just fun for the family on summer nights! The kids are going to love lighting it up for the first time :o)

Here's Kaitlyn just waking up from her first nap in our new home :o)

Gee.....Wonder what woke her up?? hehehe Introducing the neighbour boy :o)

Kaitlyn's new pet!! She LOVES this dog!! Wonder why??

The view of the ocean!

The neat little pot-bellied stove nestled in our backyard. Can't wait to light it up!

Week-end Getaway!

2009-08-03T21:14:00.011-03:00

Tim and I have been toying with the idea of taking Kaitlyn to our hometown in Annapolis Royal for some time now. Both Tim's and my families are from and still live there. Kaitlyn and I have not been home for almost two years! She was in and out of the hospital sooo much that it just didn't seem safe enough to travel that far from our Children's Hospital. Not that now seems so much safer, but Kaitlyn has been very stable and all of her equipment is 100% portable now! We even have back-ups for most of our machines. (Back-up ventilation next!!) When my sister announced she was getting married this summer (29th of August) we had sort of toyed with the idea of attending her wedding. Then Kaitlyn got a tentative surgery date for the 27th of August. Anyway, when this long weekend arrived...we just decided to take off!

Travelling with an extremely fragile child....nerve wracking! We had a good weekend, don't get me wrong, but it is a little unsettling being so far from the one hospital that sort of GETS our daughter. It was Natal Day weekend in our hometown. Our town celebrates every year with reenactments at the Fort Anne Historical Site, a small parade, Sunset ceremonies, fireworks, Crowning of a Queen Annapolis Royal, pancake breakfast at the local firehall, etc. It's a busy weekend and while we didn't attend half the functions...we had a really good time with our family.

Kaitlyn's favourite part of the weekend was our visits to my mother's home where my youngest sister has turned the place into quite the farm!!! They have goats (at least three), two cows, five horses, chickens (complete with rooster), three dogs, and two cats. Kaitlyn is an animal LOVER!!! She beams for dogs and was particularly taken with the goats and cows too :o) And no bad reactions from the allergy department. But then like a lot of allergies exposure is key to reaction and Kaitlyn has never been exposed before. However, it's not likely that we will be repeating a trip like this again anytime soon!!! It was a LOT of work! We are also on alert for the next couple of days. Kaitlyn was exposed to sooo much this weekend that was completely outside of her normal environment. I guess we'll just have to wait and see and hope we kept her safe!

Here are some pics. of our weekend:

This is Kaitlyn on her way to Nanny's! She was so excited she talked for the first hour of our trip. Then.....she finally gave in and conked out for an hour long nap :o)

Kaitlyn in the middle of Nanny's kitchen table having a bi-pap break as she wouldn't take a nap! She's chewing on a popsicle stick....not of the blue variety this time folks ;o) She is laying on her first sheep skin! My great Uncle bought this for our girl...we hope to make her stroller and van travel more comfortable.

Alexander having his popsicle in the middle of Grampy's new chair!!! Please don't make a mess buddy!!!

Owen with his shadow ;o) My Mom's dog LOVES Owen!!

Kaitlyn giving the horses some lovin'. She was talking the ENTIRE time :o) The girl is a nut over horses!

And again....horse lovin'!

Time for some Cow lovin'!!! This was great fun :o) The baby cows just wanted to suck on her hands!!

One of the baby goats!! They wouldn't hold still for a picture...I snapped this one quick!!!

This is Kaitlyn earlier today on her way to the Parade. She really loves her new sheep skin!!

Here we are waiting for the parade! Kaitlyn sat up for the entire parade and was loving all the excitement!

Kaitlyn watching the parade :o) She really like it!! Want to know what her favourite thing was....scroll down!!

The TRACTORS!!!!! YUP ;o) Not sure why, but she was even saying "tractor...tractor".

Alexander after the parade with some of his loot! A sucker and his beads!! Settled in for the long haul back home :o)

Kaitlyn barely made it four minutes after we got her packed into the van and on her bi-pap ;o) She too got lots of loot. A balloon, foam lobster (what else in Nova Scotia??), and two sets of beads! She had sooo much fun :o)

Proud New Homeowners!!

2009-07-26T22:42:00.008-03:00

We are now the new owners of a four bedroom bungalow! It is a lovely large home with an ocean view! We move in mid-August and are so happy to be leaving our moldy apartment!! We have a lot of work to do before we can move into our new home. We have to build a ramp for Kaitlyn. Right now her medical stroller/wheelchair can not even get into the house!!! Then we need to build a BIG fence around the perimeter of the property. These things are going to cost us a pretty penny!!! But hopefully we will manage to pay for it all. I have no idea how the ramp and fence are going to happen with only Tim. Hopefully he can scrounge up a couple of buddies to give him a hand. It might not be so bad if he had actually ever built anything before, but he is hoping to get some advice and learn as he goes! We also need a chimney insert to run a woodstove...but that may have to wait until next winter! I think we have a smaller woodstove that would get us started.

Kaitlyn and the boys are going to LOVE this new home. They will be able, once we build a fence, to go outside whenever they like :o) Even Kaitlyn LOVES the outdoors. I can see us spending a great deal of time outside! Guess I better start knitting the kids some mittens. Owen likes homemade mittens the best :o)

Kaitlyn has been doing really well. I guess she just needed a full month of antibiotics to help her get the psuedomonas under control. Since she colonizes pseudo. we will never really ever get rid of it! But hopefully we will continue to be able to control it when it flares up! We did learn that she has some resistant pseudomonas. This is a really good piece of information for us to have. Now we know that if we treat with antibiotics and nothing is happening, then we have to take her in to the hospital ASAP!! Hopefully it will be a good long time again before we have another flare up.

The kids have been asking and asking for a kitten!! We would never have considered it in our apartment, but Tim did tell the boys that if we ever moved into a house..... Well, we are getting two kittens mid-August! They will be a nice "welcome to the new house" surprise for the boys!! They are going to be absolutely thrilled!! I cannot wait to see their little faces when they see them. Our boys are really very good with animals, especially babies! Even though they are so rough and tumble with each other. Alexander absolutely adores anything feline!!! He IS a cat on a regular basis :o)

I'll let everyone know how the ramp, fence and the packing go :o) The next time I post we may be in our new home!

Pseudomonas...Strep Throat...House Hunting!

2009-07-17T00:16:00.009-03:00

Wow I cannot believe it has been almost a month since my last post. Several people have asked how we are doing. Well after Kaitlyn got sick we did a two week course of antibiotics. Then Kaitlyn was off antibiotics for two days and started up with green thick secretions and wet lungs again. We took her into ER for a sputum sample. The ER doctor, not really knowing Kaitlyn, and none of our regular doctors being available advised us to put her back on her antibiotic. Two days later we found out that Kaitlyn had cultured peudomonas yet again. When our pediatrician called we found out that Kaitlyn now colonizes resistant strains of pseudomonas. Resistant to the antibiotic we have been trying to treat with. Since Kaitlyn seems symptomatically better while on the antibiotic our doctors have recommended finishing this second two week round of antibiotic and then take her off and see how she does! If she gets sick again with the "green stuff" then we will be forced to try a new med. Not too sure what that is going to be but our Ped. has assured me that she and our pulmo. have a plan!!! It's my hope that when we stop this fourth week of hefty broad spectrum antibiotic that the ugly green monster stays away!!!

Tim and I are just getting over strep throat. I've had a sore throat for almst a month, which isn't uncommon for me. I get sore throats with allergies, weather change, dry air, environmental changes and every virus that goes through he house! But I had about three days of unbearable sore throat and it felt like strep...I used to get strep throat a lot in my younger days. Anyway, after a few days I started to feel better but Tim got worse. He ended up with a sinus infection!! Not fun!

We are still searching for a house! We really need to get out of this apartment! We have found a home that we really like and are in negotiations to buy this house. There are some issues, as I'm sure there always are!! But I'm still hopeful that everything will work out. The place is a little farther outside of the city than we might prefer to be, but not so far that we feel that we are putting Kaitlyn at any risk. It's not too far from the children's hospital. It may make our nursing a little tricky...not too sure how many of Kaitlyn's current nurses will be able to make the trip out there. Who knows the move may bring some new faces.

I don't want to rant about the house we have found that we like in case we don't get it. But I would like to say that it is one of the best we have veiwed as far as meeting most of our needs. We wanted a home with MOST of the living space all on one level. Not easy to do in a small city! People tend to build up...not out! This home is a largish bungalow with a large yard. There are four nice sized bedrooms on the main floor. A huge kitchen, and a big living room. There's a dining room off the kitchen and a nice sized bathroom. The hallways aren't even cramped!! I could totally see a power chair zipping around this house ;O) The basement is partially finished. One bedroom, family room and half bath. With a large unfinished rec room (can you say toy room folks!!!!!). That's enough...you'll have to stay tuned to see if we get the house ;O)

A recent pic of our pseudomonas (bacteria) filled girl! She's always happy, even when sick!

My silly inseparable boys :o)

Friends of ours invite us over evry so often. We are always so thankful for their hospitality and enjoy very much each and every visit! I have very few snap shots of Kaitlyn and I together!

These are Kaitlyn's new "shoes" as Alexander calls them! They are orthotics to keep Kaitlyn's feet from dropping and will hopefully help to prevent contractures.

Under the Weather

2009-06-23T01:02:00.006-03:00

Kaitlyn has been under the weather the past few days. I haven't had much time to post about it as Tim and I have been busy doing therapies around the clock. She started with increased secretions and a bit of a temp. last Thursday evening. By Friday morning she was pretty goupy and had a temperature. Secretions were pretty bright green (reminiscent of our pseudomonas days!). Anyway with the color of the secretions, how fast this all came on, and the fact that nobody else was sick made up our minds to start her on an antibiotic. We did send in a sputum sample for culture, but haven't heard back yet. Hopefully I'll remember to call and get our ped. to find out tomorrow!! We started her on Cipro. which is a pretty broad spectrum (pseudo-killing) antibiotic. Hopefully it'll do the trick!

She still has wet lungs even today! This is just sort of hanging on! I was hoping that she would be all better by Wednesday as we have a neurology clinic. But even if she makes a remarkable recovery overnight, there will still be residual increased secretions on Wednesday. I guess she will have to do her first clinic bi-pap dependant!!! What a long day that will be for her. Thank goodness we are fully mobile for powering all of her equipment!

My boys are coming home tomorrow. They've been having an extended weekend with the grandparents so Tim and I could focus on Kaitlyn. Perhaps once they're home Kaitlyn will get better faster....she really dislikes missing all the fun (can't "run" through the house chasing boys while attached to a machine and moving around too much makes you choke!!!).

I cannot believe how big my girl is getting. I remember the days when she would sleep at the bottom of our bed (when sick) and there was still enough room for Mommy and Daddy. Not anymore!!! Daddy gets to sleep on the couch now!! SHHHH...She's sleeping!

This one is of Kaitlyn holding her magic wand that Lexx and Ayva sent her from Alberta. She LOVES it, and particularly loves when the air from the bi-pap mask blows the wand making the little sparkles flutter! She is also making good use of her new Z-flo positioners that Karah sent me from Kentucky!!! Thanks Karah they are keeping my feet in a better position while I wait for my new AFO's!!

Having a taste of Daddy's salad. She fussed when Daddy tried to give her anything but greens!! She licks and licks and licks....saying, mmmmmmmmm!

Alex never has a nap anymore, except on rare occasions when he wants to be held and then "conks" out. Look how big he is getting!

2009 Halifax Walk for Muscular Dystrophy

2009-06-14T20:37:00.010-03:00

We went to the 2009 Halifax Walk for Muscular Dystrophy yesterday (Saturday June, 13). We have been wanting to go to an MDA function now for quite some time. It always seemed like there wasn't the opportunity in the past or Kaitlyn was sick. This time everyone was healthy, we had a good day to go, and we all had a great time.

It's nice to be able to show our support to such a wonderful organisation. Kaitlyn's picture was in the 2009 brochure for the Walk this year. It was a lovely honour and we were glad to be able to join them. Tim was able to raise a little money through sponsors for the Walk. Friends of ours donated both monetarily and of their time for the occasion. We were glad for the company! Thanks Dave, Amanda, Ryan and Emily! Tim and I would like to thank everyone else who sponsored our family for the Walk. It means a lot to us to be able to give back a little of the money that has been provided for Kaitlyn by MDA to buy essential medical equipment! As always we are grateful for the support!!

The boys and Kaitlyn had a good time. The Walk was led by the 33rd Halifax Pipes and Drums. They are a marching band and the kids LOVED the loud music! The band led us down the Halifax boardwalk along the waterfront. After the walk the kids all played at a playground on the waterfront. Fun was had by all!

When we got home we noticed something awful! Kaitlyn has sunburn!!! I felt awful. She has such fair sensitive skin. We put sunscreen on the kids, but I meant to pack the sunscreen and put on a nice thick coat just after we got there. Well the sunscreen got left at home! Poor Kaitlyn paid the price. We didn't have the canopy up on her stroller because Kaitlyn likes to see EVERYTHING and the weather was overcast most of the afternoon so we really didn't think anything about sunburns!!! The kids were in fleece coats most of the day too. It was nice, but not hot!

Some pictures from our day:

The kids on the board walk.

33rd Halifax Pipes and Drums

Kaitlyn getting her T-shirt for the walk.

Kaitlyn telling me something...she talked continuously while we were out. She was in a reall good mood. The breeze was blowing and she just LOVES the wind!!

33rd Halifax Pipes and Drums lining up for the Walk.

Photo opportunity after the walk!! SAD but I think this may be the very first family photo we have ever had taken!!!

Kaitlyn's sunburn!!!

No News Is Good News!

2009-06-04T21:58:00.008-03:00

Oh my goodness! I didn't realize how long it has been since a posted! OOPS! But the good news is that there really hasn't been much to post about! No news is good news.

So we had a head cold go through the house two weeks ago. Alexander started with a runny nose after getting back from the grandparents' the week before. I thought that it could be just allergies, but then the sniffles didn't seem to go away. Then he got pretty stuffy. When Owen woke up one morning with a swollen and red eye and a runny nose....well then i knew that we were dealing with a cold. Try as we might, it is almost inevitable that Kaitlyn is going to get the cold once it is in the house.

When Kaitlyn gets a cold it always seems to hit her really fast. This time was no different. Kaitlyn was presented with increased secretions one afternoon. Increased secretions make Kaitlyn choke as she has no mechanism to protect her own airway. Kaitlyn lacks the ability to use her tongue to gather secretions and prepare them for swallowing. She also has a highly unpredictable swallow. Sometimes she manages to gulp saliva down the esophagus and sometimes she aspirates saliva into her trachea. This causes her to choke and sputter gasping for air, as she also cannot cough!! Imagine what it's like when you swallow something when it goes down the wrong pipe...we've all done it! Now imagine that you cannot cough that aspirant back up. Not only does it hurt like a beast, but you cannot breathe either. That's what it's like for Kaitlyn. We have to sit next to her when she is sick and suction her fairly constantly. If we didn't she would choke! I have a point to my little story.

The morning after presenting with increased secretions Kaitlyn gets up and secretions are still increased and are now thicker (common with a cold), secretions were colored, she had a fever, and she couldn't stop gagging and choking. So she became bi-pap dependant. Her sats were perfect but she could not tolerate even a very few minutes without choking/gagging. Kaitlyn gags on thicker secretions. Our concern at this stage is that if she aspirates enough saliva she could get much sicker! She could even develop pneumonia or end up hospitalised. So by placing her on bi-pap she is then able to spit out secretions making her a whole lot more comfortable and easier to manage. We are protecting her airway by putting her on her bi-pap. Kaitlyn is old enough to "get" this now! She smiles at us when we ask if she wants bi-pap; she is agreeing that she wants the additional support and security that her bi-pap can provide. We also increase Kaitlyn's breathing treatments to help clear her airway. After three days of pretty continual bi-pap she was able to come off and slowly we were able to reduce her treatments to her normal routine.

Last week we went to the hospital for an overnight stay. Kaitlyn's pediatrician and the RT department wanted to monitor Kaitlyn's O2 sats overnight and have her blood gases checked in the morning. Her O2 sats didn't budge from her normal 99-100% all night! Heart rate was nice and low too....she slept like a baby ;O) I'm so glad that our children love sleep, and that Kaitlyn can go to sleep anywhere as long as she has her bi-pap, her guys (the stuffed animals she sleeps with), and of course her MaMa!!

We've gotten outside on a few occasions just around our building. But at least it's outside! Kaitlyn LOVES the outdoors. She could spend the whole day with the wind blowing in her face. She squeals and squeals!! It's pretty funny.

Now for some recent pics!! Having fun with Daddy!!

Don't ask me what Owen was doing!! Eat or be eaten!?!

Alexander's fake cry face!! It looked even more convincing a split second before this was taken! You can see the beginnings of a smile in his eyes!

One of Kaitlyn's special little grins! This one reminds me of Leah!!

This was Kaitlyn's first B4SMA babies blanket. Kaitlyn was only just diagnosed when she got this blanket.

This is Kaitlyn's NEW B4SMA blanket!! It's HUGE!! Kaitlyn LOVES it! Thanks so much MJ :O)

Fresh Air and an Ambu-bag

2009-05-13T21:54:00.007-03:00

Things have been so quiet around here lately that I haven't had anything to write about. This is a good thing in the world of SMA. Today was a beautiful day out! Sunny and warm. I cannot take all three children out by myself (not with Kaitlyn requiring constant monitoring), but we did all go out on the deck to get some fresh air and sunshine! Kaitlyn and the boys had a blast. The boys antics amused Kaitlyn and she kept saying, "cars...cars...cars". We live in an apartment next to a highway...lots of traffic!

Kaitlyn's RT from Vital Aire was by this morning to drop us off an ambu-bag. We've never had one in the house, and while I have seen ambu-bags used many times before, I have never actually picked one up myself. Learning how to use an ambu-bag isn't hard, and we now have a little more freedom, and an extra tool to help if we need it in an emergency.

This is a picture of Kaitlyn relaxing in the fresh air. She was sitting up in her big girl chair, which she has really outgrown!!! Her shoulders are up past the back of the chair! But she did fantastic. She sat up for an entire 30 mins. without choking, and although I suctioned her several times I didn't get anything worth while.

My cool cat, with her purple sunglasses and killer long blonde hair.

Alexander discussing "big trucks" with his sister...."Did you see that yellow cement truck Kaitlyn?"....."Did you see that blue dump truck Kaitlyn?" Followed by big kisses!!

Back-up Cough-Assist!

2009-05-05T23:26:00.014-03:00

Remember when I said that we were fighting for a back-up cough-assist?? Probably not, it was so long ago!!! Well yesterday we finally got our new cough-assist!! This is HUGE for us. Another small battle won. We didn't even really have to fight very hard. We requested, gave valid reasons why we needed this essential piece of equipment, and have a very wonderful social worker to help advocate for us....and the rest is history!

The machine was funded by the government and we are very grateful to have it. Without the cough-assist our daughter might still be alive but NOT without a tracheotomy (a tube placed through her neck into her lungs and attached to a ventilator 24/7). Not that I have ANYTHING against a trach. but for now Non-invasive ventilation and protocols are working very well for us. This secondary machine means that if something happens to one of Kaitlyn's cough-assists then we will ALWAYS have a back-up. Our children's hospital has only ONE cough-assist and NO plans to get another. The machine is so poorly understood!

We use Kaitlyn's cough-assist several times a day! Three times a day we do CPT (chest physiotherapy) followed by cough-assist. We also cough Kaitlyn before we place her on bi-pap. So that's 5 times a day we use the cough-assist on schedule. When she has increased secretions from a cold or flu, then we have to cough-her more often. I have had to cough Kaitlyn every few minutes for several hours in a row to help her get through a bad cold. When she is sick she is chained at the hip to this machine (so to speak). We also use the cough-assist anytime that Kaitlyn chokes on her own saliva, or throws a plug (this happens quite frequently).

The second cough-assist will also make our lives a lot easier as we can leave one machine always loaded on Kaitlyn's medical stroller/wheelchair. This will cut our packing to leave the house in half...maybe more!! The cough-assist barely fits on the stroller, it's a pretty tight squeeze. Loading and unloading the machine takes a lot of time.

This is a picture of Kaitlyn's cough-assist. It's a rather large machine (11.5 X 11 X 16.5 inches and weighing in at 24 lbs).

This is Kaitlyn's Medical Stroller/wheelchair. The cough-assist is the BIG grey box-like thing underneath Kaitlyn's seat towards the front of the stroller.

A well loaded down stroller. Cough-assist, inverter, gel cell battery, oximeter, bi-pap, DVD player, and feed pump. Suction we either carry or hang from the handle bars! (phew!)

A picture of Owen and Kaitlyn this afternoon. He just absolutely adores this baby girl. They were laying together watching Diego DVD (yes...that's right...I said Diego!!! It's the ONLY other acceptable programming allowed on Kaitlyn's DVD player!)

A Day Out!

2009-05-04T14:24:00.011-03:00

This weekend we went to a friends house for a BBQ lunch. Saturday was over-cast and rainy, but Sunday turned out to be a decent day! There were still some scattered clouds, but it was warm and reminiscent of summer days yet to come.

Because our friends live in their own home (not an apartment like us) they have a room devoted to toys (:O) like, like, like!) and a backyard! Our boys and Kaitlyn had a blast. All of our children just love the outdoors. Kaitlyn really loves the breeze blowing through her hair, she laughs and laughs! She also enjoys the swing set. I hold her and swing and she just loves it!

It was very nice of our friends to invite us over and to have us for lunch. When it was time to go Owen didn't want to leave, so our friends offered to keep Owen longer. Needless to say Owen was thrilled. He LOVES the outdoors and didn't want to come home yet. They offered to keep Alex too, but Tim and I thought that would be a little much yet. Alexander is only two, and while I think that he would be alright without Tim or I there, he is definitely a little more work!! Owen is a pretty self sufficient little guy.

Here are some pics of our day:

Kaitlyn in the playroom watching the boys. She was laughing just before I snapped the shot.

Alexander having a quick break.

Owen pressing all of his "controls" LOL

The swings are a huge hit!

This picture is dark, but Kaitlyn was loving the outdoors!!! I just wish we could get her out more often!

Owen and his ride-ons!!

Alexander, wasn't unhappy just squinting over the sun...I guess I should have remembered their sunglasses!

Owen and his best bud raking leaves! They were having so much fun!

Our friends' youngest! She was having a real blast playing in those leaves :o) Isn't she adorable!

Eyes Like an Angel

2009-04-30T22:35:00.009-03:00

This is a photo my cousin Sharon took when she came for a visit recently. After playing with the photo this is the copy she sent me. I fell in love with it and had to share!! Thanks Sharon!!

Kaitlyn just fell in love with her cousin! I haven't seen Sharon for a few years, our families sort of lost touch :o( Kaitlyn usually won't let people get too close, strangers make her nervous. However, the first thing Sharon asked when she walked in the door (I had Kaitlyn on my shoulder) was..."can I hold her"? I hesitated for a second but asked her to sit down and I'd hand Kaitlyn to her. This was the result...a number of minutes with both Kaitlyn and Sharon smiling and talking. In fact Kaitlyn wouldn't be quiet the entire time Sharon held her. She seemed to enjoy every moment...and she didn't choke!!!

Talk, talk, talk two chatty girls! I think fun was had by all!

Kaitlyn was checking out her cousin Owen!!! Yes Owen!! Sharon and I both named our first-borns Owen. It's so funny that we would pick the same name! Needless to say our families had lost touch for awhile!

Sharon and I! Can you see the family resemblance?

The boys all had fun too! Sharon's Owen is the same age as my twins. Three Little Monkeys Jumping on The Bed.....er trampoline!!!

The Many Faces of Owen and More.....

2009-04-27T23:05:00.011-03:00

Alexander is such a good little guy. After getting a start on some of my morning chores I come out to find Alexander just sitting in the "cumfty" Spiderman chair just playing with his die-cast cars! He plays with die-cast cars, one in each hand, ALL day long!

Trying the floor out, but not willing to give up the chair! If his bum leaves the chair it might get snatched! (p.s. the red socks are in the garbage tonight...we are poor, but not destitute)

Kaitlyn playing with piggy and waiting for brother to appear around the corner!

Do Ya think she LOVES her brother!?!

The "Many Faces of Owen"! Owen is an entertainer by heart. He wanted me to take his picture while making all sort of funny faces. It was our entertainment while waiting to go for the mail. It's really sad, but living in an apartment building we don't have a backyard to send the kids out to break up the monotony. So trips down to the lobby to fetch the mail have become a family outing...if you will! It's the highlight of the day, even Kaitlyn has started fussing when we don't get our act together and get out the door quick enough to suit her!! So Owen's antics were a welcome diversion!!

In the elevator on the way to get the mail. The boys always have to bring something to ride on. Tonight it was their Bopper balls.

Kaitlyn gets to carry the mail back. She's the only one that won't wreck it or loose it on the way back home. The only issue becomes...how big of a fit will she throw when we take her letter from her when we get home!?! The girl likes her letters!!

Raising Fund's for Muscular Dystrophy

2009-04-27T08:53:00.005-03:00

Kaitlyn's Grandmother has organized a fundraiser in honour of all the support Muscular Dystrophy Canada has shown our daughter in helping to get the equipment she needs to live a happier and better quality life. Muscular Dystrophy has been so supportive in helping to find funding to buy a few pieces of equipment for Kaitlyn. We have always been very grateful to their organization and this is one way for us to show our support in return.

Some of the things MDA has helped us get for Kaitlyn are her cough-assist device, her Enteralite Infinity feeding pump (with accessories), and a corner seat (specialized seating-supportive from head to toe). Thanks MDA!

This is only a pic of the poster that will be distributed around our home town, where the concert will be held. MDA supplied the posters for the event.

Ventilation

2009-04-24T00:51:00.005-03:00

You can't have a conversation about Spinal Muscular Atrophy without the topic of ventilation making it's way to the surface. Lately we have been questioning Kaitlyn's ventilation and whether or not she is being ventilated properly, or rather if there may be more things we could be doing to optimize her ventilation. Sometimes it can be hard to find a pulmonologist who supports, or even understands, non-invasive ventilation (NIV). As Kaitlyn's parents we are first in line to advocate for her every step of the way. Ventilation is no different.

The very first appointment we had with a pulmonologist we asked about cough-assist and bi-pap. We had read that they only way for children with SMA (specifically the weaker type 1) to survive and have any quality of life was to use these machines. The only other options would be to let nature take its course and our daughter would not have lived very long, or have an operation for invasive ventilation (a tracheotomy). We were told that NIV and cough-assist wasn't done on babies as young as Kaitlyn was at the time of her diagnosis (5 months). When in fact it is, and some babies use NIV from birth. We did eventually start an NIV protocol, but not until Kaitlyn was 10 months old!

We finally asked a bunch of questions during Kaitlyn's more recent hospital stay. (Goodness knows we were there long enough to ask several people LOTS of questions.) The result was a meeting with Kaitlyn's pediatrician, the RT's at our children's hospital, and the RT from our DME company to discuss Kaitlyn's ventilation. The result of this meeting is that Kaitlyn will now be properly followed for her ventilation needs.

We have had to increase Kaitlyn's pressures on her bi-pap again. She is now 20/3. The left side of her chest has taken a little dip over the past couple of months and she had started peridoxal breathing again. Peridoxal breathing means that as she breaths in her diaphragm expands and her chest falls, and on the exhale the chest rises. Typically this means that pressures need to be increased to help blow open the lungs, expanding the chest, so that Kaitlyn won't have to use her diaphragm to breath. The idea behind the bi-pap is that Kaitlyn allows it to do most of the work while she sleeps so that she can get some true rest and not have to work so hard to breathe.

This meeting allowed my husband and I to ask some questions and raise some concerns. Kaitlyn is dependant on Bi-pap, of this there is NO question. All the medical professionals involved would have to agree. She may only use it nocturnally (except when she has the common cold where she uses it 24/7) but without it she would get too tired to breathe after only a couple of days. She might not even make it through the first night as children with SMA have such low tone that they breathe too shallowly in deep sleep to ventilate their own lungs and they could even stop breathing! So our concern is at what point is she a candidate for a secondary vent? Our DME company told us that if something were to happen to Kaitlyn's bi-pap that they might not have the same unit to replace it with, and they may not have a machine capable of performing all of the things Kaitlyn's bi-pap does now. Then we would be without a bi-pap until ours got fixed!! This is really scary!

The next issue is if we are considering a secondary ventilation machine do we go with another bi-pap or do we switch Kaitlyn to a vent (LTV or some other similarly capable unit). A vent has far more options for ventilation than a bi-pap. It can handle higher pressures, it can be volume cycled rather than pressure, it has a lot more alarms, and can be used for invasive ventilation should the need ever arise! During illness the vent could be huge for Kaitlyn! It would allow her to remain home long after the bi-pap stopped working for her. The bi-pap only handles a maximum pressure of 30, of which Kaitlyn is using 20. When sick, if we use similar pressure increases as colds she has had just this past winter, then we would have to go as high as 26-27. This doesn't leave us a whole lot of pressure for emergencies!

It's unlikely that a vent will be approved for funding. Vents are much more expensive than bi-pap. We have also recently been approved for funding for a secondary cough-assist. I am ever hopeful that the cost of a vent would be weighed against the cost of a two week stay in PICU!! It's in everyone's best interest that we get the equipment Kaitlyn needs so that we can continue to manage her at home. So this is what we will be wishing for the next few months. (These things take time).

Otherwise, Kaitlyn has been fine. She has been making some advances in the way of speech. She is experimenting with a lot of new sounds. She uses a lot more words throughout the day now. Still no sentences or even close to it, but she is at least mixing some real words with the nonverbal communication that she has perfected!! We've NEVER had any trouble understanding exactly what she wants (her nonverbal communication is superb)...probably why she has gone so long without speech!! Yesterday she was a train..."CHOO-CHOO". Pretty funny ;o)

Today Kaitlyn "decided" that she was going to wear her new Dora PJ shirt all day!!! There was no way that shirt was coming off this morning without a WHOLE lot of grief! LOLOL We compromised on the pants. Doesn't she look proud of herself!?!

My Goofy boys acting all silly. They were blowing rude noises into my leather couch...which they thought was incredibly funny!!!

The weather has been so nice the past few days. Sunny and warmer! We have been going for walks after baths before bedtime. Just around the outskirts of our building, but it's better than nothing. This is Kaitlyn all clean and waiting to go outside.

Those of you that follow me on FB may have read that Alexander fell this week and split his chin wide open. He was racing up and down the hallway with Owen. They were each pushing a large truck, racing and making tons of noise! I was yelling at them to stop when I heard....CRASH! Then Alexander comes out of the hallway crying with a big split in his chin. Poor guy, it was too deep for dissolvable stitches, so he has six stitches that need to be taken out. More than likely he will be left with a permanent scar. Sorry for the picture quality, he wouldn't hold still!! (The stitches are blue).

Ports and Plastic Surgery

2009-04-16T23:14:00.004-03:00

We've had two more appointments in the past two days. And we are now followed by yet another discipline!! UGH! Our appointment Wednesday was with our general surgeon. Hands down a fantastic man! I really like him and he has pulled our little one through four surgeries now, one of which was a seven hour sugery he performed entirely himself to repair Kaitlyn's poor torn and lacerated bowel last summer, and he did her nissen then as well. That was the most exhausting surgery I want to have to go through until someday she needs something done about the curvature of her spine (which right now is fine). We went to see him about placing a port-a-cath in Kaitlyn. A port-a-cath is a tiny medical appliance that is placed under the skin. A catheter connects the port to a vein. The port then sits just under the skin and can be palpated and used for central venous access.

This is an example of a central venous access device.

What does this mean for Kaitlyn? It means that Kaitlyn would never need to feel another needle poke! A blessing!! The skin over the port can be frozen with a gel then a needle would access her port without her ever needing to feel another poke. Our surgeon told us that Kaitlyn does have other options for access, but they aren't good. He said he can still do cut downs in her right arm and he still has her external and internal jugular veins to use. He also has left her subclavian veins but doesn't feel as comfortable using these as there is a risk of puncturing Kaitlyn's lungs. He has avoided the jugular veins in the past saving them for future port lines he figured she may one day need. You see once these sites are accessed via cut downs they can never be accessed again, or at least usually cannot, the scarring prevents it!!

So what are our choices...not good! Kaitlyn's right leg is inaccessable for any lines. She has had a total of four veinous pokes and one arterial poke! She also has extensive collaterals in her right leg from past blood clots. Her left leg has been accessed a total of three times and has also had blood clots but we were able to save the leg for future access with four months of twice a day blood thinner injections. However the attempt to place a central line in the left leg this past hospital stay probably means that the left leg is inaccessable now too. Kaitlyn's left arm is inaccessable for any future lines! This leaves us Kaitlyn's right arm, and she would have to endure cut downs in order to gain any access. You see Kaitlyn has also been rendered inaccessable for PICC lines! CRAZY! This kid has the worst access of any other kid I know! We also have her external and internal jugulars which oce accessed cannot be accessed again! So the only option that makes a whole lot of sense is to have a port surgically placed so that we have permanent access to Kaitlyn's central venous system.

SO you might be wondering what's the big deal, why wouldn't you elect to save your daughter from future stress, pain, discomfort, physical and emotional trauma?? Well it's extremely risky choosing ANY surgery for a nueromuscular child. It also really sucks that we have to take our perfectly healthy daughter in and put her through more surgery. Ports also come with their own list of risks. And then we have Kaitlyn's tendency to clot. The last thing we need is for her to form a blood clot around this device and break off that close to her heart!!! Sooo....our next appointment is with haemotology for a discussion about blood clot control. Once we are reassured that we have a plan in place to deal with potential clotting then I feel like we will probably choose to place a port.

Our appointment today was with a plastic surgeon about the breakdown on Kaitlyn's right ear. She has had this breakdown since December and while we have been attempting to treat this condition, until just recently we weren't having a lot of luck. It wasn't until I made Kaitlyn some ear pillows that she really started to heal. Now she never goes anywhere without one of her little pillows! The surgeon didn't have any further suggestions except to keep off-loading on that side :oP But he did think that our idea for the pillows was the best solution for keeping pressure off of her ears. YAY!

These are the ear pillows that I made for Kaitlyn. They help keep all the pressure off of her ears.

Lazy Weekend

2009-04-13T23:39:00.004-03:00

Not too much to report from the last few days. Everything's been pretty quiet! The kids had a good Easter weekend. The boys went down to the Valley (two hours away) to visit with Grammy and Grampy. They had a blast! They love going there as they get to go outside and get to do activities that they wouldn't get to do at home. Daddy brought them home on Saturday so that they wouldn't miss the Easter Bunny gifts Sunday morning.

The Easter Bunny found them all. Kaitlyn got mostly clothes and a couple of teeny stuffed bunnies and a stuffed pink chick! This girl is nuts about stuffed animals, but they have to be animated by someone!! But the resulting giggles are worth the extra work to help her play! Owen and Alex got some CARS DVD character cars (they are nuts about die-cast cars, especially Alexander), some chocolate and goodies, new hats, Owen got a truck and Alex got a Bopper Ball. Everyone was quite happy with their gifts.

This is an older pic. I've had it for a few days, but I had to post it as it was such an AWWWW moment! These boys are glued at the hip. I hope they are always this close! Owen was showing Alexander some of his favourite things on the computer. Owen says, "do you want to see this little Buddy?" He's an AWESOME big brother....well, most of the time!

Kaitlyn likes to watch the boys playing on the computer. She can play her own games but it is getting so that she likes watching them play because she cannot play the same games as them! It's so sad sometimes as I feel like she's being left behind. The games out there for single switch are obviously not very challenging!! She gets too bored. But communication devices are extremely expensive and I am not aware of any funding porgrams that will cover communication devices.

Kaitlyn enjoying a taste of Daddy's bacon Easter Sunday. She thought it was pretty funny to bite down hard so that Daddy couldn't pull the bacon back out of her mouth. Good thing she didn't bite a peice off!

I gave Kaitlyn my popscicle stick, she wanted it REALLY bad! LOL So I let her chew on it, which she thought was pretty funny.

Even more hysterical was when she bit down on the stick and it bopped bunny in the chin flipping his head up and down. Too Funny! She got the closest to an outright giggle I have seen in a while.

Growing!!

2009-04-06T13:58:00.005-03:00

Where does the time go!?! I had to give the boys a hair cut the other day. Everytime I have to cut their hair I feel like I age them several months each! Don't you find when they get hair cuts that they look older?? Tried to take some pics for all to see.

Attempt number one at a nice pic! BRATS!!

They of course thought it was funny to vex Mama! They were sharing a laugh!

I think this was the sixth shot....not too bad! LOL

We measured how tall the kids were the other night. Owen is almost 44 inches tall and four years old. Alexander is 35 1/2 inches tall and 2 years. Kaitlyn we have to try and stretch out her contractures in her knees to get something even close to her full height!!! She measures 37 inches tall and 2 years!!! She's always been bigger than Alexander but WOW! I cannot believe how tall she is! Kaitlyn doesn't get haircuts, but i thought that I would take some pics to document her growth as well.

One for the camera!!

Look how crazy long my hair is!! Check out those natural highlights! Bet you're jealous!!

She's getting to be quite an armful for Daddy!

Back to Baseline

2009-03-31T00:17:00.007-03:00

Everything's been pretty quiet around here. Kaitlyn is back to her normal baseline!! Still having slightly slower digestion, but nothing serious. The rest of the family is finally healthy again. I like when everything is "normal" around here. Now we just have to try and keep the kids healthy until the end of the cold and flu season, which should be soon!! Come on summer! We have had people enter our home and tell us at the end of their visit that they have had illness in their home!! Our own families don't enter our house when there is a sick family member living under their roof or if they know that they have been exposed to illness!! This is just an example of how people don't understand what it means for us when Kaitlyn gets sick. It disrupts our whole family dynamic!! Not to mention the fear, pain and suffering that poor Kaitlyn is put through everytime she gets sick. Especially when she has to endure a visit to the hospital. Don't get me wrong, our children's hospital is hands down a wonderful place and Kaitlyn would not be alive without them, but some visits are definitely easier than others.

We've been taking our normal walks down to the lobby in our apartment building to get the mail. It's a family event and one the kids enjoy, a lot of weeks it is their only outing outside of our apartment....no kidding! Some pics of the outing.

Kaitlyn waiting to go for our walk.

Getting ready to go out the door.

The door opens...Kaitlyn gets excited!

Silly boys all grins too!

New favourite thing to do...crawl through the table in the lobby!

Home Sweet Home

2009-03-27T21:26:00.003-03:00

We're home! We got back home Thursday afternoon. Kaitlyn is doing really well. Spent the last two days off of bi-pap except to sleep. She is pretty tired. Still taking 2 1/2 to 3 hour naps in the day. This morning she decided 6AM was wakey, wakey time for MaMa!! I made her watch Dora until 7AM when I started her normal morning treatment. I know bad MaMa, but I really want...no need...for her to get back into her normal daily schedule!!!

Kaitlyn is tolerating her feeds pretty well, but she's not back to normal yet! When I feed her with her pump she does pretty well. Today I tried to bous her 30 mLs and she cried when she saw me coming with the syringe!?! I'm soo confused. Hopefully she'll start tolerating full feeds at regular concentration pretty soon.

Kaitlyn's left arm is pretty tender. It's the arm her surgeon had to make the cut downs in to place the peripheral PICC line that then blew!!! Hopefully her arm will feel better in a few more days, especially once the incisions heal fully!

RSV Day 11-12

2009-03-24T22:48:00.005-03:00

Sorry for no post for days 11-12. I sort of got caught up in my own illness!! So what happened Monday....we went down on Kaitlyn's Bi-pap settings. She had been holding 98% and above the entire night Sunday night. Only one jump down to home settings! We moved Kaitlyn's treatments from Q3 to Q4 and she has tolerated this just fine. She is still getting the odd pluggy thing up but overall is doing fantastic! Our goal was to also up her feeds and see how she tolerated the change. We went from 10mLs an hour up to 12mLs then fifteen, then up by 5 mLs every four hours.

So far Kaitlyn has tolerated rate increases without any issues. She seems to be digesting everything just fine now. I really think that she just needed the break from eating. Kaitlyn lost her PICC line in her arm Tuesday morning. Her arm was very sore and swollen. I cannot believe that she blew four lines this hospital stay!! Craziness! I guess we'll be having a lot of discussions now about ports (central veinous access). It would seem with Miss Kaitlyn that we haven't much choice anymore! We have tried just about every type of line they can place, and she chooses to blow them all except the central line in the groin. This is the line we requested upon our arrival in hospital the very first day. They didn't place the line....for whatever reason!!! And then a week later when they decided to try she had been undernourished and under hydrated for a whole week.....so no wonder they couldn't place the line! They won't do a jugular poke in this hospital without a secure airway (intubation)....so this was of course never an option for us! Our pediatrician has included in Kaitlyn's protocol...MUST FIND CENTRAL ACCESS STRAIGHT AWAY! Do you think next time they'll listen to us!?! I sure hope so.

RSV Day 10

2009-03-23T10:45:00.003-03:00

Not much to report about day ten. I had been fighting a stomach bug and finally had to call Tim and tell him to come in. There was no way that I was going to be able to stand and do cough-assist when I was running to throw up!! So I went home early Sunday morning and Tim stayed with Kaitlyn. I stayed in bed almost the entire day Sunday. Tim got sick through the night Sunday. I got back to the hospital this morning early(Monday). I'm NOT 100% but at least I'm not throwing up anymore, and poor Tim needed a break. Hopefully he'll go home and spend the day in bed!!

Kaitlyn made out fine yesterday. He bi-pap settings went down. She's almost back to her home settings. Her sats are also much better. Our goal for today is to stretch her treatments from Q3 to Q4 and see how she tolerates the change. Her lungs sound much better, but she is still throwing the odd plug! We have also decided to try upping her feeds again. SLOWLY! She's getting 10mLs and hour now, we will up that to 12mLs and then up to 15mLs depending on how she tolerates. Kaitlyn's pediatrician is back today(Monday) so finally we should have some consistency to her management and hopefully get out of the hospital by the end of the week! Provided we can keep this stomach bug from her!! Keep your fingers crossed that Kaitlyn doesn't get anything new over the remainder of our stay!

RSV Day 9

2009-03-21T07:32:00.003-03:00

Ok, day 9 sucked!! (Sorry, but it did!) So the Intensivist was supposed to come up and place a central line this morning, and in walks the resident whom we knew was working in PICU (where the intensivist is in charge). The resident tells us that Kaitlyn is scheduled in surgery, OR, at 2:00pm. We fought, of course, to get the Intensivist to come to us...but it was surgery or nothing!! So the they wanted us to transport Kaitlyn to the OR without bi-pap, as they have no way to power Kaitlyn's equipment for transport. We said..."UMMM NOPE!!!" She is currently ventilator dependant, panics and can't breathe without the vent right now! To put her through that would be insane! We told them that we had back up power for our equipment and if they would leave everything alone once we had it hooked up, then we would arrange to power Kaitlyn's equipment for the time they needed to have her in OR! I am so thankful now that we went with the more expensive equipment when we were purchasing back up battery power! She was able to have uninterrupted running of her vent for the entire time in OR!

Then they told us that we weren't allowed to go into surgery with Kaitlyn. The resident said that that just isn't done and it is NOT allowed! We told her that indeed it is allowed we have been offered in the past to go in the OR with our daughter! She just said, well it's not allowed this time. We said that she couldn't go into OR with an unprotected airway, unless somebody who knows her goes along!!! They finally agreed to let Kaitlyn's chest physiotherapist go in with her to suction and monitor her airway needs! She would also be there for moral support, a familiar face so that Kaitlyn wouldn't panic. We told them NO ANESTHETICS, freezing YES, but nothing that could compromise her respiratory system!! They agreed!

The plan was to place a femoral central line (called a cook line). This is the type of central line that Kaitlyn has always had. The intensivist couldn't get the cook into the vein, it just collapsed! If they had done it a week ago when we first asked them to they might have had more success!! Kaitlyn is under hydrated right now, making any vein tricky to access. They had to call in Kaitlyn's general surgeon to do what they call a cut down. They cut and pull the flesh away to expose veins and then place a PICC line (a really small catheter) into the vein and advance the line up the vein to a more central location within the body. This places the catheter into even larger vessels for less chance that the line will blow or cause any issues! Our surgeon did the cut down in Kaitlyn's left arm. The first cut he made exposed a vein that blew when he accessed it. Then he had to do a second cut down, also in her left arm, this vein he got threaded but could not advance the line all the way centrally. He could only get the line threaded up just past Kaitlyn's shoulder, but he told us that the line was enough central that it would meet all of our needs for this stay! He also told us that Kaitlyn's left arm is now inaccessible for future lines...not really sure why. I will have to have him clarify this for me next week when he's around!

Respiratorily Kaitlyn has been fairly stable today. Got out three BIG plugs, the most awful looking things you'd ever want to see! Great big gobs of stuff! RSV creates really thick, stringy secretions, I had no idea how yucky this virus was. Until you actually see it for yourself, it's hard to imagine! Anyway, we were getting lots of stuff up especially in the morning. Kaitlyn has a vaso-vagal response. It's when the heart rate drops suddenly when we are suctioning. It only goes down 20-30 beats per minute, but I have only ever seen her do this after surgeries when her body has been really stressed out. (Remember this was before Kaitlyn even went to the OR...and she didn't have any general anesthetics or anything that would typically have caused a vaso-vagal response).

RSV Day 8

2009-03-20T00:07:00.006-03:00

Kaitlyn had another desat over night last night. She was bringing up lots of thick gunk during the 4:00am physio, as a result she gagged and spit up!! I suctioned as best I could, then coughed her to try and get all the formula out of her lungs. It was obvious that she had aspirated some despite my best efforts. She had to have 10 L of O2 added to her bi-pap just to keep her sats above high 80's!! We were able to wean her down to 6 L in only a few minutes with a few more rounds of cough-assist!

She had chest x-rays that showed she still had the infiltrates in the upper left lobe of her lungs, and a new area of what looked like either aspirant or incipient pneumonia in the upper right lobe of the lungs. X-rays are funny things as they lag behind the actual symptoms. They are also open to interpretation, but the main thin is that Kaitlyn doesn't seem to have any areas of pneumonia in her lungs. This is really good because it means that we have been successful in mobilizing whatever is in her lungs. We are not giving the mucus and secretions a chance to settle and become a full blown pneumonia!

Bloodwork still looks good. We asked again for a central line today. Nobody wants to place a central line in Kaitlyn. The surgeons are usually the ones to place the line, but they won't touch our girl, as she is too difficult a case. The Intensivist didn't want to place a central line, she wanted to see if a PICC line could be placed. A PICC line goes into a vein and is a long catheter that is threaded up into the vein until it meets the larger internal vessels. This type of line would be less likely to clot, and more likly not to blow!! There is only ONE doctor in our entire Children's Hospital that has the necessary skill to place these lines. If she's on vacation nobody gets a PICC line....seriously!! She came up to see Kaitlyn, at the request of the Intensivist. She has rendered Kaitlyn inaccessible for a PICC line. She is basically saying that there is no vein on Kaitlyn's entire body that she could access to thread a catheter to a major blood vessel. This is extremely scary!! If our child needed veinous access in an emergency...they might not be able to get it!!!!

They are of course begging us to reconsider some sort of more permanent veinous access. It seems like he older she gets the more tricky she gets to place lines, and the more fragile her veins become...so she blows the lines (this literally means that the fluid being delivered leaks out of the blood vessel into the surrounding tissues)!! This is why Kaitlyn has had two swollen feet and a swollen hand this hospital stay...from the three "blown" peripheral lines! Yes that's right Kaitlyn has blown her third peripheral line...no more TPN until tomorrow. The issue with some sort of more permanent line is that they always require care, which means that Kaitlyn requires more invasive type care! But the trade off is that she would have permanent veinous access. So...why haven't we done this??? Seems simple, place the access line..."POOF" magical veinous access!! (Sorr for the sarcasm ;oD )

There are two main forms of permanent lines, what they call a Port-a-cath or a Broviac line. The broviac line is typically the easiest to place but it is an external line. This means that wherever on Kaitlyn's body that the decided to access a vein, Kaitlyn would have extending outside of her body an external IV connection line! This line has a higher chance of being infected, and you have the worry of it getting pulled, and you have to keep it dry. This means that we would have to use a surgical dressing every time that we wanted to give our daughter a bath. To us anything we choose for Kaitlyn that further limits her "normal" activities just is NOT worth any advantage it may provide. The other option is the Port. It is like a PICC line that is inserted under the skin threaded up to a larger vessel and has a balloon that sits just under the skin. Any time we would need veinous access we just poke the balloon with a needle and BAM veinous access. The problem with a port is that it needs to be surgically placed, which in the past has never seemed worth it to put Kaitlyn through. We always had other options, but we are running out of access the older Kaitlyn gets.

The other issue is that Kaitlyn has a blood disorder and with ANY sort of permanent line she would have to go on blood thinners for the rest of her life. So now, not only are we dealing with permanent veinous access, which comes with it's own set of risks, now we have a whole new set of risks related to the blood thinners. There is relatively low risk with the heparin based drug we have had to have Kaitlyn on in the past. But with that drug it needs to be delivered via a subcutaneous injection two times a day, and the drug is not cheap. While drug costs are usually not our first concern, when we have to pay out of pocket for all of Kaitlyn's drugs that certainly has to factor into any decision we make based on whether to enter into a commitment where she needs to be placed on a preventative medication!

Since Kaitlyn has been rendered inaccessible for a PICC line the Intensivist said that she would be up to place a central line through the large blood vessel in Kaitlyn's groin. This is where she has had her central lines in the past....but it is also where she has had her blood clots (veinous and arterial!) in the past. We have had a consult with haematology and they have said that once the line is placed we will have to start a blood thinner to keep Kaitlyn's blood from forming clots around the central veinous line! The blood thinner should only need to be continued for a short time after the line is removed. The intensivist couldn't make it up today, as it turned out, but she has promised to be up tomorrow morning before noon to place the line.

RSV Day 7

2009-03-18T23:09:00.007-03:00

Last night Kaitlyn blew her second peripheral line!!! This girl does not appreciate the additional hardware! It really sucked because there was a perfectly good bag of TPN hanging from an IV pole mocking me. So as can be expected over-night with only her reduced rate enteral feeds Kaitlyn stopped moving secretions and her sats went back down. The resident came in this morning and I actually had to argue with him about secretion management. I said that I felt that her lower sats were a direct result of her not being properly hydrated, to which he sort of shrugs his shoulders and gives me the eye roll. To which I say..no really. He still doesn't think that I am making any sense until I explain to him that if Kaitlyn is dry that the thicker secretions in her lungs are not being mobilized, her airways are compromised and her sats are therefore lower than normal!!! When she is well hydrated then the thicker mucus has something to slide up on and when we do a treatment more comes up! Therefore higher sats!

Then he talks about lowering her bi-pap settings to her home settings...to which I said, not until her sats actually improve instead of getting worse!! He agreed ;oP Then he tells me that her chest x-rays that we had done yesterday showed infiltrates mostly in the upper left lobe of Kaitlyn's lungs. This would be consistent with a virus like RSV. At least there wasn't any indication of pneumonia, so for now we are keeping things moving! Then, almost in the same breath, he tells me that we should move the treatments to every four hours instead of every three and that way we cold get more food into her (as we wouldn't have to turn the feeds off so often just to do a treatment). What could I say...I have to get food into her somehow!

Kaitlyn tolerated her first treatment this morning really well. I got a bit of gunk out of her nose, but overall not too productive. So I turn her feeds back on and the PT (chest PT) says that she will be back at two for the next physio. I'm thinking to myself that's too long between treatments, but I also need to feed my daughter. Since her chest sounded pretty good I was willing to try it and see how Kaitlyn tolerated the decrease in CPT. About a half hour before she was due for physio she started sputtering. Tim was here so he coughed her and then started physio. She was pretty crunchy sounding in the lungs, but I wasn't worried. PT would be in soon and Tim know how to manage Kaitlyn, so I headed home. I called to see how she had made out with her physio as she worried me a little with how wet she had sounded before I left compared with how she sounded this morning! Tim told me that she had crashed! She was on even more of an increase with bi-pap settings, needing 5L of O2 to maintain sats. A central line was outright refused as Kaitlyn has a blood clotting disorder. The only type of central line that was offered was a jugular line under general anesthetic (knowing full well that we would never choose this in her current weakened state and respiratory condition!!!!). So a THIRD peripheral line was placed and all we can do is hope that she doesn't blow this!

Tim called back a couple of hours later to say that Kaitlyn had been weaned off of the supplemental O2, but that the Bi-pap settings could not be reduced. Her lungs were still wet sounding but not as bad as earlier. She had her new IV and PPN had yet again been started!

Owen and I were playing on the spare bed tonight before supper. He likes to jump on me and rough-house. He smashed the back of his head into my nose...I heard what seemed like a really loud crunch, saw stars, saw black, then felt really sore with a bit of a head-ache. My nose is a bit swollen and there's a bit of a reddish bump!!

Tim calls me tonight and says that Alexander had vomited twice since he got home. I ask you...what else could go wrong!!! Please keep us in your thoughts.

RSV Day 6

2009-03-16T15:22:00.006-03:00

So Kaitlyn woke up this morning with much better sats. After they placed the peripheral line (IV) and Kaitlyn got her PPN she was much better hydrated. We began getting more stuff up out of her lungs and upper airways. After about three more treatments her sats were in the higher 90's (95-97) almost the entire afternoon.

Kaitlyn's IV blew around 9:00am this morning and had to be removed. This meant no more PPN and no more additional hydration. Kaitlyn's dietician was in this morning and decided after several calculations that she really should get some nutritional support. The pediatrician on the floor didn't want to put Kaitlyn through another poke if the line was only going to blow that fast again, but what choice do we have? I cannot let her go a single day more without proper nutrition. The doctors agreed. Another request went out to get the best person in the hospital to place the new IV! The line was placed later this afternoon and after the second treatment today Kaitlyn began to run lower sats again!! Man! This girl likes to be hydrated!! I cannot believe that a few hours on low rate feeds alone could make such a difference!

After fluids were started through the new line Kaitlyn did a little better through her afternoon therapies. By this evening her sats were back up again and we are again getting a bit up. Without the PPN Kaitlyn's mouth gets sticky and NOTHING is mobilized!

No other significant changes! Still on Q3 treatments, only using regular saline nebs, and trying to just let her rest between treatments!!

RSV Day 5

2009-03-16T06:26:00.004-03:00

Kaitlyn had a worse night Saturday night. This is to be expected RSV usually gets worse before it gets better. Sats were in the low 90's high 80's most of the beginning part of the night. She did level out as the night went on and climbed up to the low 90's early Sunday morning. We pushed on with the Q3 treatments. We still weren't getting a whole lot out of her. I think she was starting to get not "dehydrated" but definitely on the dry side!! She got a new diaper at 4:00am and it was still bone dry at 9:00am!!!

Sunday morning during rounds I again brought up my concerns about Kaitlyn's nutrition/hydration!! She was still getting only half, if that, of her daily requirement for feeds. For an SMA child this is unacceptable!! The SMA 1 child has about 5-10% of the normal muscle mass! When we think about how critical muscle is for storing nutrients and acting as a buffer for when the body is in need, then it makes more sense why we as parents "freak-out" when our children are even slightly starved! We have spent a great deal of time and energy researching-networking trying to perfect her diet. If she doesn't get the nutrition she needs EVERY 24 hours then her body could potentially waste muscle (break down muscle for nutrients). This makes Kaitlyn weaker and the strength once lost does not usually return.

I was also concerned about Kaitlyn's ventilation. We usually check Kaitlyn's blood CO2 levels to see that she is getting enough air exchange.....breathing in enough O2 and expelling enough CO2. We also wanted to know what her electrolytes were as this can often give us an idea of hydration. None of the normal blood work had been done!

By mid-afternoon Kaitlyn had both her blood work done and a peripheral line placed. The blood work was all normal, and the IV allowed us to begin giving Kaitlyn PPN a dilute form of TPN (an intravenous form of nutrition)! Finally! So far so good. More wet diapers, and her secretions are thinner and we are getting stuff up now!

Respiratory Syncytial Virus

2009-03-14T21:47:00.004-03:00

RSV! One of the most dreaded respiratory viruses that our SMA children can have! Kaitlyn has just been diagnosed with RSV! We did EVERYTHING in our power to prevent this from happening. We are diligent hand washers, Owen has to remove all clothing at the front door when he comes home from school, we are careful when out in public to teach our children not to touch things (elevator buttons and such), etc. etc. etc. RSV is nasty. It's super contagious and is not like the typical cold virus where once you have a particular virus you can't catch it again. RSV can be contracted over and over and over again. That's what makes it particularly nasty in my books!!!

We brought Kaitlyn to hospital over issues with digestion. We have only been able to get half of her normal feeds into her for four days now. We thought that if we brought her in that she could be looked at and we could figure out how to get more feeds into her, even if it meant TPN for a few days so that she wouldn't be compromised nutritionally. They of course had NO idea what to do with her!! It's kind of funny watching medical professionals stumped, kind of scary too! They decided to have radiographs done of her abdomen, particularly the placement of her G-tube and a look at the bowel. The x-rays have shown a partial blockage of the bowel, which is why she isn't tolerating full feeds. We are currently giving her a bland mix of her regular formula and pedialyte to see if she digests it any better. It did seem to be working, but Tim thought tonight that she was reverting a bit. I think perhaps she was just too tired. Time will tell.

Back to the RSV. So far, because they tested for RSV very early in her "cold" (as we hadn't brought her to hospital for her cold symptoms) she is managing very well. She has had a few dips into the low nineties, but overall doing fairly well. But this is only day four since the onset of ANY symptoms! Typically days 5-7 are the worst, this is when the virus seems to peak. A couple of doctors today were even saying that it could be as much as ten days before we see a real spike in symptoms! We are in for a long haul regardless! This is not something that any SMA child is going to shake off in record time. Kaitlyn is the second child that I personally know of that has both received the RSV vaccination and still gotten RSV! Not too sure what's going to happen next year! She'll probably be denied the shots since she got the virus anyway.

As always (but especially now), keep our little one in your thoughts! We are trying to be optimistic that since we caught it so early that we can stay on top of things. Kaitlyn is on Q3 treatments and we are doing our best to keep those airways open!!

Day 5 of Second Cold 2009

2009-03-12T21:35:00.004-03:00

We are officially a sickhouse. I think it's safe to say that everyone in the house is sick to some extent. Owen and Alexander have been sick since Sunday. I came down with a sore throat and no other symptoms. I thought that it was just allergies or sensitivity to environmental factors. I get sore throats A LOT! So I didn't think much of it until yesterday when my nose started to run. Today I'm full of all sorts of thick mucous (gross huh!). Tim is sick with a bit of a cough and he says he's tired, but overall pretty good.

Alexander is the WORST! Poor little guy is absolutely miserable. He and Owen have awful crunchy coughs and runny noses. Owen says his throat is sore, and Alexander keeps holding his head and saying ow..ow! Alexander got up this morning, drank some milk, coughed up a lung and went back to sleep for three hours. He got up was awake through lunch, but wouldn't eat lunch, then went back to sleep for an hour! Alexander had a fever this morning too! 39.1 when he woke up! Since then we have been keeping Tylenol into him. Throughout the day he has been running 38.1-38.3. Alexander LOVES CPT, so we did lots of CPT today! It's good for him! By tonight he had perked up a bit, hoping tomorrow is a better day for him. We'll continue with the CPT because it seems to make him feel better.

Owen is still coughing and is still pretty sick, but at least he is eating and drinking a bit. He hasn't been as tired as Alexander, but he has still been pretty tired. Owen hasn't had a "fever" yet. He is running a little higher than normal, but not technically a fever. He should be on the mend, although he still has a nasty cough and runny nose!

Kaitlyn: ahhhhh Kaitlyn. Well, lets see. She woke up Tuesday night fussing. Usually when this happens she wants to be rolled over or has gas in her tummy that needs to be manually vented. (We leave her tummy vented all night long, but sometimes air still gets trapped!) So I rolled her on her back. I tried manually venting her stomach, but no air would come out. Kaitlyn gave her face like she had secretions or something she needed suctioning. So I suctioned her mouth and didn't get anything really, was reaching to turn off the suction machine when she spit-up!!! Kaitlyn has a nissen!!! How can she spit-up!

Anyway, back in with the catheter I went I suctioned until I got the worst out. Then I whipped the bi-pap off. If there were formula in the airway I didn't want it getting pushed any further into the lungs! I then aspirated her stomach.....I wasn't going to risk having any more food in there until I could figure out what caused her to spit-up. While waiting for her tummy to drain I continued suctioning. The amount of formula in her stomach was astonishing. Her sats were only 1-2 % lower than normal but she was just starting to dip a bit when I felt that her stomach was done draining. I coughed her several times and she came back up pretty fast. When I was done she was holding 96-97%. After CPT and more coughs I put her back on Bi-pap. She had 120mLs drain out of her stomach (which I had to dump), there was 10 mLs left in her stomach plus however much she spit-up!! Kaitlyn sleeps with a rate of 60mLs an hour. For some reason she stopped digesting her food!

Wednesday we had a hard time getting food into her. She wasn't tolerating a very high rate at all. I think that I got most of her day's formula into her, but I had to leave out her solids. Last night we started her out at a rate of fifty, but she wasn't digesting fast enough, so we had to bump her down to 40 mLs an hour. Today she wasn't processing very fast AT ALL. I think I might have gotten about 40 mLs an hour into her, that's about half the rate that she would typically get with solids. Now it's been two days without solid foods. Not a huge loss in calories, but still worrisome when I don't know how long this is going to continue. Also worrisome because of the loss in fluids. Today Kaitlyn also got up with increased secretions. There is some thicker stuff coming out of her nose today. Overall she doesn't look too bad, except for the digestion. Her sats are 1-2 % lower than normal. She is dipping into the low nineties during coughs and when she cries (something she only does when something's up). Tonight she is sleeping comfortably and we have her rate down to 40 mLs an hour. Hopefully she'll have a good night.

Well wishes going out to Lizzy's family and Mary's family. We hope everyone is well soon! Keep us updated folks, we'll be thinking of you all!

Day 2 of Cold Two

2009-03-09T07:40:00.007-03:00

Well morning two is definitely worse than yesterday! The boys are both mouth breathers today and NOT happy about it. Alexander's fever is back but is still low grade. They are both coughing more, nice crunchy wet coughs!! LOVELY! HA!

Alexander can't keep his hands away from his nose. He is constantly fussing over it! He's only two and cannot figure out how to blow it effectively. I keep thinking, "how am I ever going to wash his hands enough to keep the germs down so that Kaitlyn won't catch this cold?" I'm realizing that I won't keep the hands washed!! I'm going to have to wash myself EXTREMELY often, clean surfaces like crazy, and "kill" the first boy that touches Kaitlyn while sick!! It may sound really sad to someone who isn't immersed in the SMA world, but our boys know that they cannot touch Sissy when they are sick!! Even Alex!!! They seem to understand that she is different and just accept that they cannot touch her! Life gets sooo complicated when we have to go to the hospital!

I'm really hoping that Kaitlyn doesn't catch this cold. While it isn't at this stage a chest cold, it is causing thick secretions in both boy's noses and a crunchy wet cough. Something that could easily be FAR worse in their sister! We're keeping our fingers and toes crossed that Kaitlyn doesn't catch this cold!! I guess we'll know today or tomorrow whether or not we've dodged this one or not.

Second Cold of 2009

2009-03-08T20:37:00.004-03:00

Yesterday we had such a beautiful day with friends at their home. Their little guy has been quite ill! He had RSV with pneumonia, couldn't eat or drink much and lost a bit of weight. He was just starting to feel a little better, eating and drinking again, when he got a stomach bug. The poor little guy was vomiting for days. He is much better now, and has been told by his pediatrician to stay home (not go to school and stay out of crowds). His immune system probably isn't up to snuff quite yet. We went to visit them yesterday. Everybody was well and my boys were soooo excited to finally get to see their buddy!! Owen sneezed once or twice while we were there, but I thought nothing of it as Owen has allergies, he often sneezes in a strange environment.

That night the boys were sneezing a bit and Owen coughed once or twice, but again I still didn't think much of it. After the boys had their bath, they looked like they had been drug behind wild horses. They were beat!! Poor Alex could barely keep his eyes open. At 7:30pm Owen says, "I think I'll go to bed now"! He was the first one in bed, usually the last because he is the oldest. I thought HMMMMM they must be really tired! I carried Alexander down to bed next and he felt warm. Our kids almost always feel warm, they run hot! But I decided to check his temp. as we are paranoid about illness in our house! To my surprise he had a bit of a fever. Low grade, but still a fever.

This morning I woke up to the sound of Owen coughing! My first thought, oh NO here we go again! My second thought, Oh crap we were over to our friends home the day before!! But we had no way of knowing! I feel just awful. Owen's nose is all stuffed up and I think all the coughing is from the drainage down the back of his nose. The cough is wet, but not in his chest!! His voice is also scratchy, but he says that he hasn't a sore throat. Alexander had a fever this morning 37.9 degrees, stuffy nose, wheeze, and a bit of a cough! We spent the day wiping noses, hand cleaning/sanitizing, trying to keep Alex from crying (as he gets wheezy and chokes), and sooking two sick boys.

So far Kaitlyn hasn't shown any signs of cold!! Hopefully she will not! The boys have runny noses, are sooky and tired, but at least they aren't that sick. When the cold is in the head...it's a good cold!!! We are hoping this cold blows over as fast as the last one did! And we are also REALLY hoping that he boys didn't pass on their cold to their buddy! I would feel just awful if he got sick because of my boys.

Another Small Battle Won!

2009-03-05T22:25:00.005-04:00

Finally!! A while back I remember having said that we were looking into getting Kaitlyn a second cough-assist. I cannot even begin to imagine what would happen if something were to go wrong with Kaitlyn's machine! We use it so much, and it's not uncommon for her to need to cough at some point through the odd night! In an emergency...it NEEDS to work!

NIV isn't very popular in Nova Scotia, and I personally know of no other person using an NIV protocol in my own province. Our hospital has ONE cough-assist which they use sporadically with limited ability and therefore limited success. They of course blame the effectiveness of the machine!! The cough-assist at the hospital is kept locked in an office of PT. So if we had an emergency and needed the machine off hours we would have to wait for the on call PT to answer a page, call back, get into the hospital, bring us the machine, and then I'm sure argue with us about how it should be employed!! I'm not sure that Kaitlyn would ever live through an aspiration without a working cough-assist used at a moments notice!

Anyway, our social worker called and told us that our request for a second cough-assist was approved. Our first cough-assist came from MDA. They have been awfully good to us!! This new machine is coming from the government. It is such a relief to know that soon we will have a back-up! We have never been taught how to use an ambubag. Something that I think we will remedy very soon, but Kaitlyn has never tolerated this very well. This could be because any time one was introduced she was already pretty sick and scared. I'm thinking that if Tim and I learn how to use an ambubag that perhaps Kaitlyn could learn to cooperate somewhat, at least until we could get to a cough-assist!

Thought that I would share the good news with the ones that support us the most! Take care everyone....Spring is coming!!

This is Kaitlyn sitting in her corner seat! I finally remembered that the supports in the back come apart and can be lengthened to add a little room for growth. The chair is at it's maximum allowance, but it will work for a short time more. She loved sitting up getting a new perspective. She tolerated this position for a half an hour before getting a little tired and chokey. I didn't push her. I thought that was really good for not sitting up for months!!

Laughing at, what else, Dora!!!

She got a kick out of the tray. She fingered a little unicorn and her disney fairy!

Pediatrician Appointment, RSV Shots and G-button Change

2009-03-03T20:12:00.007-04:00

Kaitlyn had a 9:00am ped. appointment today. It was a regular well visit check up. I was not lovin' the early appointment, but we do love being the first appointment of the day!! We tend to miss more of the sickos this way! Kaitlyn weighs 12.6 kg and is 36 inches long. She is now 27 months. She's in the fiftieth percentile for weight which is really heavier than a child with SMA should be. Typically these children are kept in the 20th percentile or close to it. But she's between the 75th and 90th percentile for height. We put Kaitlyn on the AA diet last year in March and since then we have not changed a single thing except to add calcium, Vitamin D3, and 30mLs of prune juice to keep her regular. We have just been waiting for Kaitlyn to grow into her mix. So far no plateau! She just keeps growing and growing and growing!! We asked about reducing calories but our ped. isn't comfortable with this because she says that Kaitlyn is so healthy, and is growing like a weed and she hates to take calories away and make her hungry! Especially since she IS growing so fast, and doesn't show any signs of slowing down.

Kaitlyn has break down on her ear that we have to go see plastic surgery about!! Usually we get very superficial breakdown every few months and it takes a few days to heal. This current breakdown is not very big, but has never really healed up in over two months now. It looks really good most days then will puff up a bit and ooze for a day. We have off-loaded as much as possible, but Kaitlyn obviously prefers that side of her head and can still turn her head to lay on that preferred side. Our ped. gave us a stronger antibiotic cream to try in the meantime. But she wants us to see plastic surgery to be on the safe side, she wants to rule out that there isn't infection in the cartilage! I have made Kaitlyn three pillows now that have a hole in the center for her ear. This way she isn't putting any pressure on that sore ear, but can still have her way about what ear she wants to lay on ;o)

The last big thing we discussed with the ped. was Kaitlyn's Bi-pap settings. Now I know that typically this would be the job of the pulmo. but we have a little resistance in this department! We lost our original pulmo. that got us started on NIV. The pulmo. left now has the work-load of three pulmonologists, and no time for things he has to do a lot of research on. Contacting an SMA expert doesn't happen that much up here, especially when said experts are in the States!! Kaitlyn is still on the same settings she was put on when she first started using Bi-pap. While she has always done really well, she is getting bigger, and it only stands to reason that as she gets older the settings would change to allow her lungs to develop in as normal a manner as the machine can provide. What we have noticed the past few months is a general increase in her respiratory rate, while on bi-pap. Like she is trying to take in more air, but just getting it in the form of faster breaths. Kaitlyn used to spend at least a bit of every night in deep sleep riding the machine. Not anymore. Her RR on bi-pap is well into the 50's most of the night. She has NO issues with her lungs, all is clear, and she still seems well rested throughout the day. But she does tire really easy. It takes a lot out of her when we have a day of appointments.

Here are her current settings in case any REAL experts are reading (MOMS!):

Resmed VPAP III ST-A

Mode: ST
IPAP: 18
EPAP: 3
RR: 30 (back-up rate)
Rise Time: 150mS
IPAP Max: 1.00s
Max I:E- 1:1
IPAP Min: 0.4s
Trigger: high
Cycle: high

After the ped. appointment we headed over to the Children's Hospital for Kaitlyn's RSV shots. One in each leg. She doesn't mind this too much. A quick cry and then it's all done until the next month. Only one more round to go and then we are done until next year!!! Hopefully we will continue to get the shots! Our pediatrician is totally behind us on this front, so hopefully we will get approval again next year. These shots are really expensive, but a PICU stay for two-three weeks is WAAAYYYY more costly!!

In the hallway on our way to RSV shots we ran into our General Surgeon. He is a super wonderful man, who has gotten Kaitlyn through three surgeries so far and we really trust. We asked him if he had time to change out Kaitlyn's G-button. She's had the same button since last summer when she had her surgery. We kept asking if we shouldn't change the button, and everyone kept telling us to change it when it acts sticky or if it clogs. It never did these things. What we were noticing was blood flashing back into her extension more and more often, like there was irritation there somewhere. I suspected the G-button, but Tim and I were really hesitant to change it at home. We have two replacement buttons at home ready to use, but we KNOW Kaitlyn. She likes to be difficult!! (Drama Queen!!)

Anyway, our surgeon said to have him paged as soon as we were done with RSV shots and he would arrange to change the button out. He came to us...and he changed the button out himself...in a three piece suit!! VERY nice man!! BUT it wasn't a classic pull the old one out, put the new one in....OF COURSE!! He deflated the balloon on the g-tube, inside Kaitlyn's stomach, then he gave a pull. It budged but gave some resistance. He pulled harder and Kaitlyn really fussed. Then he gave a good tug and out the tube came with a bit of tissue, blood and yucky stuff around the portion of the tube that had been inside her stomach! The tube had been in way too long! But we didn't know any better (we do now!).

The tissue on the inside of Kaitlyn's stomach had grown around the tube a bit. The site bled for a minute after the old tube came out. But once the tube was out Kaitlyn didn't seem bothered too much by the site. Next the surgeon tried to put the new tube in! HA! Wouldn't go in! Tim and I are sooo lucky that we didn't try to change this tube out at home. Our surgeon had to run and get a guidewire and what he called a "stiffener" to help thread the new tube into her stomach! (The G-button is a very soft silicon material that I'm sure must be very comfortable once in, but not very sturdy for a tight push!) The "stiffener" didn't work, the site was too tight. He then had to stretch the opening and try again! The new tube went in the second time, but not without a pretty good push! He wants to do the next tube change in three months time. We say, "Gladly"!

Since coming home Kaitlyn has been a little fussy about feeds, but so far everything is fine. She went to bed well and seems comfortable.

Kaitlyn all ready to go to her appointments this morning! She always looks forward to trips out of the house. She LOVES going in the van. We always take all of her equiptment. Did I say before how much I LOVE this Max EasyS stroller!!!! Onboard....cough-assist, bi-pap, oximeter, feed pump with food, inverter, gel cell battery, mini DVD player, suction machine, and nestled on top....Kaitlyn!! We carry emergency O2 in the van.

This is the inconvenient way in which we have to load Kaitlyn's stroller. Fully loaded with equipment, Kaitlyn's stroller is very heavy....130-140 lbs !! It takes both Tim and I to push/pull this stroller up the ramps! Not ideal! But worth it to get Kaitlyn out of the house!

Waiting for an End to Cold/Flu Season!

2009-02-27T20:43:00.006-04:00

Sorry I haven't posted in awhile. I guess I'm just lazy! It's those winter blahs that hit this time of year. I'm really getting ready for some nicer weather and the END of cold and flu season!! Friends of ours have been sooo sick, the whole family! Especially their four year old son, who also happens to be Owen's best bud! They go to the same pre-school and Owen really misses him and wants him to come over and play! The poor little guy had RSV (with pneumonia!) and then got hit with a stomach flu. He's lost some weight and has had a hard time keeping enough fluids and food in his belly. It has occupied my thoughts for days now!! We really hope that the little guy and his family will be on the mend soon!!

Everyone here has been well. No news is good news...right! Kaitlyn has had some odd secretions, by times seeming thicker than normal, other times seeming pretty dry, and then sometimes I look over and she's drooling! CRAZY! She's just keeping us on our toes. In spite of all this she is fine and not acting sick or like there is anything particularly wrong. In fact, Tim and I have both commented recently that she seems to be more communicative lately. She's even making an effort to say two to three word sentences. My favourite....."I laaaaAAA Dora"!!! (LaaaAAA is love in Kaitlyn speak)

Today I asked her if I could lay her down that my arms and back were sore and she shook her head no!! LOL I've never seen her do this....and she was all grins while doing it, and looking like she knew EXACTLY what she meant. Raising her eyebrows with an impish grin like she was challenging me to disagree!! I asked her several times and got the same answer every time. She has been wanting to be held A LOT lately. I think that she likes the change in perspective and she seems to tolerate her secretions really well when somebody is holding her. We place her in a sitting position in our lap and hold her head in our hand. This also allows her the freedom to turn her head from side to side, which is how she was able to turn her head from side to side to tell me NO.

I think sitting up more may be encouraging her to communicate, but unfortunately she is so heavy and cannot help support any of her weight. We haven't any specialized seating that we can put her in. Kaitlyn needs full body support in order to sit up. We would need specialized seating with a lot of support, a TLSO(body brace) and some sort of specialized head and/or neck support. Of course these things cost money. Currently we are still trying to get her into a power chair. It's been a year and counting! This would be huge, did I say huge, for Kaitlyn. She really needs to be able to follow her brothers, make some of her own choices, get up off of the floor and start living a little!!

This is Kaitlyn sitting up playing a computer game. She tolerates this really well some days. But we cannot do it for long, only a few minute at a time, as her body is not supported properly.

Petition Video

2009-02-22T21:08:00.001-04:00

A college student from Michigan recently made a video to promote PetitionToCureSMA.com. This student is unrelated to the SMA world but was inspired by our children and their fight for a cure! There really are some truely remarkable and selfless people left in this world. This is a fine example!! Watch close and you will see our girl......perhaps she and her friends will inspire you to sign the PetitionToCureSMA.com if you haven't already!

Blanket Pulls

2009-02-16T23:36:00.001-04:00

This is one of Kaitlyn's favorite things to do!! She loves it...listen close you will hear her laugh!! I think she would do this all day long if she could convince someone to keep it up!!

Valentines and a Mirror!

2009-02-15T00:51:00.007-04:00

Happy Valentine's Day everyone! Sorry for not posting in so long, but not too much has been happening around here lately. Tim and I are pretty much over our head colds. Friends of ours came down with similar symptoms as us only ten-fold! They've been pretty sick. Their youngest had croup and the oldest a fever for a week. They took him into ER and he has pneumonia! Poor little guy. We sure hope they start feeling better soon! There are also a number of SMA children in hospital sick right now. A discouraging number of them with RSV. Thank goodness for our RSV shots every month! While the shots are no guarantee they are better than nothing! We are hoping that these families are reunited very soon. It's harder than you can imagine to be separated from ones loved ones and they from you! Especially in light of the fight that takes place within the hospital walls to save the life of your loved one!

Enough about hospitals and illness. Tim took the boys down to the grandparents for the weekend. It's a two hour drive, which was blizzard part way today. They get to play with grampy and go outside! They always come back with stories to tell. Kaitlyn and I painted a couple of pictures and read a couple of books. She adored both activities! She loves all the undivided attention but you can really tell that she misses all the activity and commotion when the boys are gone. We went for a couple of short walks today with her on my shoulder. This is her favourite spot to be, but she's really getting too heavy (and big) for this now! But boy does she enjoy going for walks. She sings the entire time :D

Kaitlyn has a little mirror that she LOVES to use to see areas of the room behind her. She can turn her head a bit but the movement is really hard for her and takes a lot out of her. This is the face she gets when she can hear somebody coming but can't yet see them. She was watching in the mirror for Owen! Scroll down and see what he does that she LOVES so much!!!!

Owen is such an amazing little guy!! He totally gets Kaitlyn and how to make her happy! It's a special gift he has, and a special kind of love for his little sister!

First Cold 2009 Update

2009-02-07T07:15:00.007-04:00

Oh my! What a week! Tim and I have been hit pretty hard with this cold. Thank goodness it has all been in our head and throat!! The kids are pretty much all better, except when they get tired...then they have a few melt downs. Kaitlyn just has some residual secretions, but is otherwise her same old self.

Kaitlyn is turning into quite the bossy two year old! She is getting more and more particular about how she wants things. If you don't hand her the right sticker, doll, small toy....meltdown! And whatever you do...Don't take away anything she has in her hand!!! She throws a fit when we take something out of her hand...I think she thinks that we are snatching! Like anyone has ever snatched things from her!! Her brothers are VERY good to her...they've never made a point of snatching anything. They are usually the ones that give her things.

The funniest thing she has had meltdown over recently was a receipt. Yes you heard right..a receipt from the future shop I believe! Owen was out in the livingroom with a receipt (of all things!?! It's not like they are lacking in the toy department!!). He was throwing it up in the air and it was spiralling down to the floor. Kaitlyn thought this was just the funniest thing. And of course Brother is the "Apple of her Eye". She absolutely LOVES Owen! Anyway, sometimes he would blow it and it would make noise and stream across the space where she was laying. :O) Good times were being had by all!!! But then the fatal mistake! Owen placed the receipt in her hand!! She threw a FIT when he took it out of her hand to continue the festivities!! It was close to bedtime and we couldn't risk her getting all choked up! There were big crocodile tears, runny nose...the whole deal!! Once she had the receipt back in her hand she was fine. Then Daddy comes out to put her in bed...repositions her and the receipt falls out of her hand. He starts looking for what's wrong..checks her g-tube, checks her limbs, suctions...pauses thinking! :o) <-- ME! I said she wants that receipt laying there! He looks at me,....no really. He puts the receipt back in her hand...then :oD. Laugh! She took the receipt to bed with her. The morning nurse thought that it was pretty funny that she had a receipt in her bed the next morning!

Hoping for a quite weekend for us and all our SMA buddies! Sending healing thoughts for Jacob, Vivianne, Lauren and Kyle, Hayden, Van, and Mary! Get well soon ALL!

Kaitlyn and Daddy having a discussion on the couch! I wonder who was lecturing whom?? LOL She looks upset but she was just concentrating on what she was "telling" Daddy ;O)

Here's one for the Camera!! She's still a camera Piggy!

First Cold of the New Year

2009-02-03T20:39:00.004-04:00

Well we have our first cold of the New Year! I thought that we were going to get away with just a low grade fever and a couple of sneezes. Owen is still fine. He had a bit of a dry cough today, no fever and is otherwise fine. Alexander's fever was gone this morning and he was acting much more himself today, but he too has a bit of a dry cough. Alexander has a bit of a runny nose and is quite tired too. Kaitlyn needed a full treatment last night at 1:00am and had a low grade fever. She woke up this morning with increased secretions, lungs are clear, but the low grade fever was still there! We got lots out of her during her morning treatment. She's been fine all day. Hasn't needed any extra bi-pap time, no major desats, just lots of suctioning and an extra treatment through the day. She's still in a good mood too! Probably because I had to sit next to her the entire day so that I could suction. She loves it when she has my undivided attention! And she loves hollering at Daddy for his! LOL!

Hopefully this is the worst that we will see! She has been good tonight...no alarms!! Always a good night when things are quiet. Sats are great but heart rate is slightly increased probably from the low grade fever.

Kaitlyn Paying with Her Brothers Monday.

Results from Round Two Serial Casts!

2009-02-02T18:48:00.009-04:00

I got to see early what round two of serial casting has done to Kaitlyn's heels! The casts got wet last night when I gave Kaitlyn her bath. Tim wasn't going to bath her, but she needed one, so I dunked her and got her casts wet :O( I guess I'm not used to controlling those long limbs!

Round two of serial casts has left her heels with breakdown again!!! Both heels look bad this time. Is it even worth it?! Well, her ankles are pretty much contracture free and Kaitlyn really doesn't seem to mind the casts at all, or the breakdown for that matter. She's a brave little girl. I still think the breakdown is preferable to surgery to cut her tendons. The surgeon told us that cutting the tendons is painful, at least when the tendons are actually cut!! I don't want to be the one to cause her more pain. I think that I would rather let her ankles contract than to put her willingly through more pain and suffering.

Owen's fever didn't amount to much. It was gone on Sunday. Kaitlyn had a great day Sunday, no real issues at all. Today Kaitlyn has had increased secretions. I've had to sit next to her with suction ready all day. Secretions got a little worse after her nap this afternoon and increased a little before bed. She's sleeping soundly now. The alarms have only gone off twice so far. Her lungs are clear so hopefully she will get a good sleep tonight and feel better tomorrow. She hasn't had any fever yet.

Alexander on the other hand has a fever tonight!! He was feeling absolutely miserable before bed. He fell asleep in my arms after supper (he NEVER does this)! When he was all ready for bed he was playing nicely on the floor with a toy. He got so sleepy he pitched forward onto his nose on the laminate flooring. He cried and any other time I would have busted my gut laughing but the poor little guy was so pathetic!!

Warning: I've attached some pics of Kaitlyn's heels. Remember, just like last time, there is some breakdown and they don't look too pretty but she isn't bothered by them at all. If you mind this sort of thing you might not want to look!

Breakdown on Kaitlyn's Left Heel.

Breakdown on Kaitlyn's Right Heel.

Gain from First Casting...Left Foot.

Gain From Second Casting...Left Foot!

Gain From First Casting...Right Foot.

Gain From Second Casting...Right Foot!

I'm sooo glad that we took photos the last time!!! It really makes the world of difference to not only know that we've had some gain, but actually see the difference!! I thought that the last gain was huge! I'm very pleased with how the casting has worked. Except of course for the breakdown on the heels. Something that we would obviously like to avoid, but at least she isn't in any REAL pain!

Crazy Times!

2009-01-31T22:27:00.008-04:00

Kaitlyn's been a little "off" for two weeks now! Two Sundays ago Kaitlyn had what can only be described as a plug. A plug is when secretions pool in the lungs and form a ball of mucus. This happens when there is decreased airflow to the lungs and inefficient secretion removal (ie. coughing). The plug blocks the airways into the lung causing atelectasis or even collapse of the lung.

Since the plugging Kaitlyn has had little spells of desatting, mostly through the night and first thing in the morning. Almost like she still has some areas of minor collapse...atelectasis. She has normal lung sounds and no fever, but we have been watching her close.

A few days ago she started with watery stools...literally! We took her back to a base formula diluted with extra pedialyte and lots of water. We took out all of her juices and fruit. After a couple of days she seemed to improve so we began very slowly adding back in her juices and fruit. Then she went to the other extreme and no stools at all for four days! CRAZINESS!

The last three or four nights she has been awake several times throughout the night. This is tough on Tim and I as he has to get up and work the next day, and I have to get up and be mentally alert and physically able to handle all of Kaitlyn's and the boy's needs. Tough, tough, tough! Yawn!!! Oh and Alexander has taken to late night strolls in the dark and needs to be taken back to bed!! I know....any other time I'd be LMAO too!

Today Owen is running a fever. Tonight it was 39.1 poor little guy! His voice sounds a little off but he is only complaining of a sore stomach from time to time. No other symptoms other than the fever. He put himself to bed tonight at 7:30pm. However he was up at 11:45pm to sit on the couch with Tim and I for a few minutes. Pathetic!!! Says his throat is really sore tonight, and sneezed all over me several times! We gave him some tylenol and he went back to bed without a fight, but the poor little guy looks pretty sick.

Now we just have to hope that whatever he has Kaitlyn already had!!

Kaitlyn thought MaMa was pretty funny the other night. As I recall she was just laughing at me for no reason. I think this was the last night before she started getting fussy day and night.

Owen and Alexander got new dolphin and shark flashlights from NaNa. They were having fun in the big box Daddy gave them that came with his new exercise bicycle.

The end of what little sleep we get!!

2009-01-30T22:18:00.001-04:00

Owen was so proud the other day! I watched Alexander climb into his crib as I was making the spare bed in his room. I groaned because this meant that he was going to proceed to fuss the entire time that I was making the bed (a job that I hate in the first place). As I finished pulling up a blanket I heard Alexander feet running on the carpet...I turned and thought that I was seeing things. At first I thought that my brain was playing tricks on me and that Alex really hadn't climbed into the crib. Then I asked, "Owen did you get him out?" Owen said, "no....I teached him how to climb out....see!!!!"

He was sooo proud that he had done this....liberated his brother! I groaned as I new it was the end of what little sleep we do get. Last night Alexander did well, no crying out through the night, but this morning Tim asked me, "did you get Alex up?" I said, "no"....then he asked my Mother (who had spent the night) "did you get Alex up?" Followed by a no. Then Tim asked Owen, "did you help Alex out his his crib this morning?" To which Owen replied, "no....he helped himself!"

Round two of serial casts

2009-01-28T10:40:00.007-04:00

We went to see an orthopaedic surgeon yesterday about Kaitlyn's ankle contractures. Not knowing SMA he was pleasantly surprised at what we accomplished with only one casting so far, and even more pleased that we have been able to maintain what we gained for the past two weeks without the use of splints or AFO's. This he found encouraging and has decided to support our request to do serial casting instead of tendon release surgery. Although he did make his big speech about how the casting may have to be repeated and depending on how often, then we may still have to consider surgery at some point to cut the tendons. For now we are happy to avoid anything related to more surgery, more anesthetic (even if it is "only" a local), more scalpels, more stitches, more pain and discomfort!! Enough is enough, and this Kid has been through the wringer enough for the past two years of her life. It's time for a break and to minimize as much as possible everything that we have to do for her to keep her comfortable. It's time she started enjoying some of the life we have worked so hard to give her.

On a lighter note the surgeon said that he thought Kaitlyn's heels were healed enough to recast, so we had round two of serial casts placed yesterday. That's what I love about our children's hospital they will bend over backwards for us. If they can squeeze us in for a casting and save us a trip back in another day then they will! Even when it was a spur of the moment decision. We didn't want to overdo this casting as Kaitlyn had skin breakdown from the last casts. So they decided to take each foot only as far as they could without a lot of force. They still managed to get minus 5 on each foot. When we started with the last casts we had minus 30 one foot and minus 25 the other. So I thought minus 5 after a two week break was pretty good.

Kaitlyn hasn't minded round two of serial casting any more than she minded the first round. This is good, I'm glad that she is not in any discomfort! She still moves her legs about and swings them back and forth in her slings. She can even lift the casts off of the bed to squeeze her legs open and shut. She should not be able to do this without casts let alone with! SHHHH don't tell her!

We decided to leave off the proper casting sock that is usually the first line of defense in any casting. The socks are ribbed and very thin. But we think that the ribbing may have irritated Kaitlyn's skin instead of aiding skin breakdown. It may have increased the friction associated with the normal sheering force inside the cast. This time we went with an extra thick layer of fluffy cotton wrap, followed by a much thinner layer to hold this all together, and then lastly the casting medium itself. Her casts haven't got the nice smooth edge that they had the last time, as they roll the sock back and make a nice edge with a normal casting. But hopefully we have traded our nice smooth edge for not as much or no breakdown!

A pic of Kaitlyn sporting her nice new casts. Boy her legs look really chubby in this photo!

More Screaming and Camera Hogging!!

2009-01-20T23:54:00.001-04:00

Kaitlyn loves to scream! She has recently taken it up as a hobby!! She also is a BIG camera hog lately. Notice how she fusses, looks ugly, then brightens when I turn the camera back to her!!! Too funny...too cute! She's one smart, manipulative cookie. Even with her disabilities she still has spunk!!!

Something's A-Foot

2009-01-20T21:49:00.003-04:00

Let's start with Sunday. Kaitlyn started her day like any other day. Absolutely nothing was wrong....besides our obvious battle. When I got her up from her afternoon nap I decided to give her a saline neb and some coughs as I hadn't done an afternoon treatment yet. I gave her the neb but I didn't do it in-line with the bi-pap, which is the way that we usually do nebs (however we have done nebs without bi-pap in the past). I was suctioning her frequently as the saline makes her drool and we never want her to choke and aspirate secretions. One time I went to suction her and she got a funny look on her face and started to sort of look like she was choking. I grabbed the neb mask off of her face an started suctioning some more, but her sats were dropping and fast!! Suctioning had NO impact. So I turned on the cough-assist and tried to cough her. As I delivered a breath it was making no impact on her sats and she couldn't produce a cough! I personnally have never had this happen before!! I tried coughing her once more but again no cough. It was like she had no air in her lungs to produce the cough. So then I turned off the exsufflation and just did several breaths of 2 second insufflation (we currently do not have an ambu-bag in the house. Something that I think we may have to remedy!). The whole time her sats were still dropping. She hit 70's....dropped into 60's....and then we had O2 hooked up to cough assist. I kept giving her breaths with the cough-assist for another minute and watched as her sats very slowly creeped up closer to 90's!!! We then put her on Bi-pap with 3 litres of O2 and over the next hour, with a few semi-successful coughs, her sats were back to normal. The really odd thing is that she was pretty dry. We weren't getting much out of her. Very odd!!! There wasn't any junk in her lungs, the only thing that I noticed was very reduced air flow in both lungs.

The really odd thing is that 1 1/2 hours after putting her on Bi-pap she was back to room air laying on the master bed begging to taste some supper!! I'm thinking this is CRAZY! Sunday night she was fine and her sats were normal and her lung sounds were perfect! We figured that she must have plugged (mucus in the lung blocking some of the larger airways and collapsing the lung, or a portion of the lung). We thought that we must have gotten the plug out, even though we didn't suction anything much out. Sunday night she slept through the night. Monday morning she got up like nothing had happened! She did dip a little with the first round of coughs and I did get a fair amount of stuff out of her. Then she did fine through physio and more coughs and stared her day the same as normal. She spent the whole morning out with the boys, off of bi-pap sats 98-100. I was careful to do all her treatments really well to get out any risidual junk. She did have a three hour nap in the afternoon. Something that she hasn't done for a few months now. But other than that she was "normal", no lung sounds, no increased secretions and no decreased air flow that I could detect.

Monday night she went to bed and she was a little junky (wet sounding in the lungs). I had to cough her at 10pm at 1am and again at 6am. After the 6am coughs I didn't hear any more junk!?! It's like it disappeared with the sunshine! I thought that whatever had been in there that I must have gotten out. Then she had her morning breathing treatment at 8am and started her day just as normal as always! Sats 98-100 all day...room air...no Bi-pap! Tonight we put her to bed again sounding junky. Am going to bed soon and will cough her again and then throughout the night as needed. Tomorrow we will keep up with the treatments and hope that whatever she has goes away without a hitch!!! We may end up going for x-rays at the very least before the end of the week. But we will avoid a hospital stay at all costs!!!

Kaitlyn watching TV the other morning. Before serial casting we could never have positioned her feet even close to this. Of course she was rolling her ankles about, so it's hard to position her now until we get her into AFO's!!

Daddy reading books to the boys. It's hard to read to both boys at once without battles over who gets to turn the pages or lift the flaps. Tim was doing a good job at disarming arguments!

Second Casting Appointment

2009-01-15T23:19:00.004-04:00

We took Kaitlyn's first set of casts off Wednesday night before her bath. They are the soft form of casts...NOT plaster casts. Hers were made of fibreglass. They are very light-weight. Anyway, we were really hoping that she didn't have any breakdown, but alas! She has pretty severe bruising on both her heels. More on the right side. I'll put some pics at the bottom, but don't look if this sort of thing bothers you!!! Let me tell you that the bruising doesn't bother her at all! I can rub my finger across the breakdown and she doesn't even wince. Let me remind everyone too that Kaitlyn has perfect sensation.

She is such a trooper. She didn't mind having the casts at all. She could still lift her knees to center and squeeze them open and shut. She could still use her slings and is probably what saved her heels from completely breaking through! We have decided not to recast today, but rather wait until next week and recast then, if her heels look better.

So fa the gain from even one casting was HUGE! SOOOO worth having it done. She went from a -25 degrees on the right foot to zero, and she went from -30 degrees on the left foot to a -7!!!! Zero degrees is a 90 degree bend, the negative side of this is when the toes point away from the body. I thought this was pretty good. The other awesome part is that her feet are in better alignment and her toes are no longer curled down and to the side. I’m really happy so far!!!

We should be able to maintain what we've gained with proper physio over the next week, and hopefully we'll be able to recast again next week. If everything goes as well with the second casting then it may only take one more casting. Then we get temporary splints while we have AFO's made. (Ankle foot orthosis).

Kaitlyn's Right Foot

Kaitlyn's Left Foot

Copy Cat!

2009-01-14T14:13:00.005-04:00

We have a serious case of the Copy Cats going on here now-a-days!

Sleep

2009-01-14T12:49:00.003-04:00

Well we got a lot more sleep last night. No low grade fever, no waking up every hour, no fussy baby....Could it really have been that her feet were too hot!! When I thought about the socks the other morning and I checked her toes they were like little ovens. The casts are extremely well padded adding to the heat. I mean I have heard that some children have difficulty with temperature regulation....but really!! I didn't even have to give pain killers. She just slept through the night. I cannot believe how sensitive she is to just about everything! Not to mention dramatic!!

Needless to say we will be doing everything just the same tonight! Do I dare hope for two good nights in a row!!

No Sleep!

2009-01-13T22:40:00.003-04:00

The last two nights have been really rough! Alexander has been calling out several times a night needing someone to come in and tell him there aren't any shadows on his ceiling!! His newest THING! Kaitlyn has been up pretty much on the hour needing to be rolled over, repositioned and nurtured back to sleep. She almost never does this unless she's sick...except this time she isn't sick! She has been running very low grade fevers. I say "very" because her temp. has only been 37.5 degrees Celsius, which isn't very high but it's up for Kaitlyn. HMMMM! Now to try and figure out what's up.

She recently had her first set of serial casts placed. Her lungs are crystal clear, as confirmed by her Chest Physio-therapist (who at our hospital do all respiratory treatments, including cough-assist....not the RT's). She is teething also, which is the most likely culprit, although Kaitlyn doesn't usually run fevers when cutting teeth. Perhaps the combo of both the casting and the teeth? Just keeping a close eye on her for the next few days. The other smaller factor, maybe not so small, is that Tim has been putting socks on her feet over top of the casts. We haven't been doing this through the day, but rather leaving her little toes out to air ;O) It might seem like a small thing to have warm toes, but for a little kid who cannot kick the covers off, and cannot communicate what's wrong...this could very likely be the problem. We'll see how tonight goes!!

Kaitlyn's first set of casts come off tomorrow night at home so that she can get a good full body bath and a bit of a break. Then she will be recasted Thursday. I really hate all these trips into the hospital. At least we don't have to go near the cafeteria, food courts, or anywhere near the floors!

Wish me luck that we can get a little more sleep tonight!

Serial Casts

2009-01-11T20:32:00.006-04:00

We decided to do serial casting on Kaitlyn's legs. This was not an easy decision for us. We asked when she was only a year old for AFO's (ankle foot orthosis) to help prevent contractures in her ankles. Contractures are sometimes painful and they don't allow proper alignment of the legs. She cannot wear shoes and would never be able to stand in a stander if her ankles remain contracted.

We were in to see PT a few weeks ago to have Kaitlyn's legs casted so a mold could be built to make Kaitlyn a pair of AFO's. Well they called us back and said that they didn't feel that AFO's were going to do anything for her. They also felt that her feet were too far out of neutral position (the bottoms of her feet were starting to turn up while the toes were pointing down). PT figured that she would never be able to stay in a pair of AFO's. Their suggestion was to put Kaitlyn through another surgery to have the tendons released.

Tim and I had a hard time with this decision for some reason! I think that we were both afraid to have to put her through ANYTHING else too invasive! But in the end we both felt that surgery might be a little in the extreme if serial casting could be tried first. PT is really doubtful that serial casts will give us the stretch that we need, but we just couldn't justify doing surgery before we tried. They have humoured us and the first set of casts were placed last Thursday. She will go in next Thursday to have the next set placed. We know some children with SMA that have had serial casting done in the past and have had good success with it! Sometimes it has to be redone in time as even with regular stretching the tendons can still get tight. This is a risk we would rather take than to have to put that poor girl through any more surgeries or pain!

While serial casting can be painful it hasn't been for Kaitlyn so far. Sometimes she fusses a bit at them, but more like she just wants them off or that she's tired of the new game we're playing! She is still doing all the same things she did before we had them put on. She can still swing her legs around in her slings. We still bath her, but we have to be extra careful not to get the casts wet. We tie her casts together (this makes them too heavy for her to lift or slide) then we place her legs up on a low stool in the tub. She can't make her frog legs anymore and squeeze her legs open and shut and lift her hip off of the bottom of the tub. But at least she can still get a nice warm bath, move her arms a bit and splash! The warm water is so good for her too before bed. it really helps get her lungs nice and clear before going to sleep.

On another note it would seem that taking Kaitlyn off of the Enoxaparin injections for her blood clotting condition has also gotten rid of her increased thickened secretions that she's had all Fall. I read something about Enoxaparin causing hayfever symptoms. This is SOOO nice for us. She doesn't need constant suctioning like she did. She can spit out most of the saliva now. She still requires constant observation and frequent suctioning, but not like before. When we have something to sit her in she should tolerate sitting up for longer periods now. She is back to her old games of bouncing on the edge of the bed on somebody's shoulder (this never gets old!!!), from the edge of the bed we then rock back and fall onto the bed (followed by hahahahaha), blanket pulls through the house (huge grins), and lots of other rough-housing games. She hasn't been able to partake in these activities as she would choke on saliva and we were forced to run for the cough assist, and/or more suctioning. We had to stop playing all her favorite games. The girls a rough-neck, and I have NO doubt would have been a severe tom boy!!!

After rough-housing on the bed. Kaitlyn's cheek is red from being on a shoulder. She gets red easy! I cannot believe how big Kaitlyn is getting!! It seems like she should look a lot smaller next to her big brother!

Enjoy this pic!!!! I don't often get all three still at once. This took a bit of doing to keep Alexander in one place long enough to capture a pic!

Turns out he just wanted to kiss his Sissy!! AWE! The boys can't go anywhere near her without stopping for a quick snuggle or mooch!

Sling Play with Serial Casts

2009-01-09T22:23:00.001-04:00

Kaitlyn with her first set of serial casts. They aren't holding her back! She's still enjoying her slings, watching Dora, playing with switch operated toys and interacting with her brothers!

Owen Playing Tickle Game with Kaitlyn

2009-01-03T16:31:00.001-04:00

Kaitlyn Laughing

2009-01-03T16:29:00.001-04:00

Powder Fun

2009-01-02T21:53:00.005-04:00

My boys were having some fun with powder the other day. I just had to post this. Thought everyone could use a good laugh! I didn't scold them as there were two adults in the house and they were not being watched. The picture quality is perfect, by the way. There really was powder from floor to ceiling and wall to wall!!

New Year!

2009-01-01T20:36:00.005-04:00

Well this year started out much better than last.

Lets talk about last year. Last year we spent the New Year in hospital. Kaitlyn was having some serious digestive issues. Last January Kaitlyn had a G-J tube. This was a fantastic option for us. However, last January when I would begin her J-tube feeds after a brief fast about twenty minutes later it would start pouring out her G-tube. I can't even begin to tell you how wrong this is, thins should never move backwards through the gut! After a couple of hospital stays for digestive issues, we decided to switch her formula. We put Kaitlyn on an Amino Acid diet, Pediatric Vivonex. This has proven to be one of the most significant things we have done in caring for our daughter.

In May Kaitlyn had been having some issues with increased secretions, over and above her normal range. We put her on an antibiotic when we noticed a slight change in the color of those secretions. We were hoping to avoid any bacterial infection, secondary to whatever it was she had virally. About a week later she still had the secretions and the color was getting more and more green. Then we had a cold go through the house and one day Kaitlyn was fine and the next she was very ill. We ended up in PICU with a very sick little girl. She had caught our cold and we then found out that she also had a bad case of pseudomonas!! Pseudomonas is a particularly nasty bacterium that loves living in the lungs, but can also colonize other areas of the body as well. Kaitlyn had pseudomonas in her lungs, urine, and her g-tube stoma.

She narrowly avoided intubation, but we were able to avoid it by following Dr. Bach's NIV Protocols. We were also able to show our hospital how this protocol works. A very nice feeling to say told you so to the experts! They needed to see this protocol in action! We also taught them a thing or two about using the cough-assist, including bleeding O2 through the cough-assist while coughing to reduce stress on the patient. Another patient (paralysis) in the PICU benefited from this knowledge while we were there! Some hefty antibiotics and two weeks later we went home.

At the end of July Kaitlyn's J-tube blocked. We had to go to emergency to try to get Kaitlyn's J-tube replaced. This is supposed to be a simple take out the old tube and insert a new. It needs to be done in radiography under fluoroscopy and takes about twenty minutes! Well we got to the hospital around 1:30pm and they managed to delay EVERYTHING! It is part of Kaitlyn's emergency protocol at the hospital that they are not to withhold nutrition!!! They couldn't place the J-tube that day (still not sure about that one!). The general surgeon placing the central line couldn't get the femoral vein in Kaitlyn's right leg. They had to call an intensivist down to place a central line in her left groin. By the time that this was all done it was too late to order TPN....GRRRR! That's why we said order it when we first arrived! We had no way to feed our daughter! She would have to fast until TPN could be ordered up the next day. They promised to have a J-tube into her before she would need the TPN. We said order it ANYWAY!

That night Kaitlyn's right leg got really cold, and there were no pulses. Her nurse didn't notice, I DID! They scheduled us for early morning ultrasound, before the J-tube placement so that once the J-tube was placed we could go home! We found out the next morning that Kaitlyn had pretty severe blood clotting in her right leg. Both veinous and arterial clots. There were extensive collaterals formed, so this clotting was believed to be from the central line needed back in May. We then went to Radiography for the new J-tube.

In placing the new tube the radiographer lacerated and perforated Kaitlyn's bowel. He somehow managed to make a gash in her intestine and then poke the j-tube out through the intestine wall!! Kaitlyn had to go for immediate emergency surgery. I literally had only a few minutes to process all of this and make some pretty important decisions for my daughter's well being. Our general surgeon came up to see Kaitlyn and I before surgery. He explained what had happened and what he was going to have to do to repair the damage done. He then told me that we were going to have to consider another way to feed our daughter!!! This I was not expecting! He said that once the bowel has had this type of extensive damage that we would never want to push a tube down through the bowel ever again. We had two options; place a fixed J-tube or perform a nissen/fundiplocation.

The first thing I did was call Tim. He was at home taking care of the boys. I had to tell him over the phone what had happened. He thought that it would be several hours before the surgery would be ready to take her. I told him that they were taking her in twenty minutes and that we needed to make a decision NOW! What a thing to have to tell him when he's at home, no way to get in to see Kaitlyn, and no time to think on it! Thankfully I like research and had read quite a bit about both procedures (just in case- always trying to think ahead). I knew the pro's and con's about both procedures. I told Tim what I thought and what our surgeon had said in answer to some of my questions about both and we decided to go with the nissen/fundiplocation. The surgeries were to be attempted laporiscopically so as to avoid unnecessary pain and healing time!

Seven hours later our daughter pulled through. 36 hours later she was off the vent. In a few days she was back home and we are so glad to have her nissen. This is NEVER the way that I would have chosen to have had it done, but something I wish we had had done LONG ago! She has been so much healthier since the nissen. We discovered another clot, this time in Kaitlyn's left leg, as a result of the central line from this stay. We decided to treat the clots. She had to go home with twice a day subcutaneous injections, which we were able to stop early December.

This fall and winter so far have been really good. Other than head colds that keep going through the house. We have had two aspirations since September. These aspirations were due to secretions and happened at night while we were trying to get some sleep. But we have managed everything at home.

We are hopeful that the year 2009 will be a much better year for us. We are much better prepared to care for Kaitlyn at home this year and have learned so much from 2008! We also have much more confidence in our hospital to be able to help us should we need them. They have also come a long way. There is new research and hopeful trials being started this year. The most hopeful thing by far is the Stem Cell research ongoing.

We are thinking of all the sick children this New Year and hope that they make it home with their families very soon.

Christmas Blessings

2008-12-27T14:41:00.007-04:00

We have just enjoyed our third Christmas with our beautiful daughter. She is now two years old! Something that we thought we'd never see! We have so much to be thankful for, and to have spent another wonderful Christmas with her....well we hope we can enjoy a few more just like this one! The kids all had a wonderful Christmas thanks to all the special things that were done for us this Holiday Season!! We very much appreciate all the help we had to make this such a wonderful Christmas!

We had Secret Santa drop off two large gift bags full of toys for the kids. This definitely rounded out their Christmas "loot". I never feel that our children are lacking in material things, but having lots to play with when you are housebound sure does help. It keeps MaMa from pulling her hair out when they have lots of variety!

Kaitlyn had a box of goodies arrive from Kyle and Friends (http://www.kyleandfriends.org/). She got a new Microlight Switch and computer interface, a new bi-pap (ventilator) mask, and a bunch of toys! She has been REALLY enjoying the computer interface and microlight switch. Now she can play games on the computer! This is going to open up her world and help her have more independence and make some choices for herself for a change! People sometimes get confused that because our SMA children can't get up and walk and do the "Normal" sorts of things that children do that they must therefore not be right or somehow mentally impaired!! The exact opposite is true! Our children are extremely bright and often are labelled with above average intelligence! I know our little girl, even though non-verbal for the most part, has absolutely no difficulty getting her point across EXACTLY what she wants, how she wants it and when she wants it!!!!! And get it right please!!

Then when we thought our Christmas was over and good times had by all, we received a Christmas card yesterday from the South Range Christian Church with a gift for Kaitlyn!! It's hard to believe how one baby girl can touch the lives of so many perfect strangers. We have only just found out that Kaitlyn has been on their prayer list for awhile now, and we had no idea so many wonderful people were rooting for our special little girl. She has been beating the odds, living past her life expectancy, thriving, and living and loving life. If these people could meet our little one, they would see a baby full of life and hope, and teaching all those around her how to love and how to have hope. We strive every day not to extend the life of our daughter and selfishly keep her here with us (although we want this as well) but rather we work so hard to improve the QUALITY of Kaitlyn's life. I don't think that people realize that every kindness, big or small, has an impact on the quality of life we can provide for our daughter and that this is what we are so thankful for every day. It's what keeps us going. So even though it is not enough, we say Thank You to the South Range Christian Church.

I finally got a new camera for Christmas. My husband kept saying there was nothing wrong with the old one, and then it finally went completely caput! It served us well, we got it when we were married five years ago and took over three thousand photos. My new camera is very nice so the pictures and video will continue...yeah!