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

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

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!!!

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!

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 :)