Life with TPN

I cannot believe we have been home for almost two years with TPN therapy! For those of you who think that your children/family members cannot survive on long term TPN...it's simply not true! My daughter, with all of her diagnoses is still thriving. She is living life and enjoying almost every day. The ONLY reason she is enjoying life is because we are able to be at home on Home TPN! We are very busy now, but I don't think Kaitlyn has ever been so stable :)

Sure we still have bad days. Kaitlyn is still chronically anaemic. Her doctors have diagnosed her with multifactorial anaemia. This basically means that there are multiple factors which cause Kaitlyn to be chronically anaemic. Some have also called Kaitlyn's anaemia: Anaemia of Chronic Disease. Not sure what to think. We have also, interestingly, found that Kaitlyn has reduced production of the hormone Erythropoietin. EPO (or Erythropoietin) is a hormone responsible for telling bone marrow to produce red blood cells. No wonder she is chronically anaemic! So what can we do about this? We have been replacing the Erythopoietin with a weekly shot. It is a sub-Q shot (subcutaneous injection) that totals 1/2 ml of EPO hormone :( Kaitlyn does not like this needle. Even freezing the site with Emla does not take away all of the burn with the larger volume! We freeze the site with Emla, cool the area with an ice pack for a few minutes, and then use BUZZY the Bee to help with the pain. All of this seems to make it easier for Kaitlyn :) She is such a trooper!

Our other biggest battle has been with Kaitlyn's Hypercalcaemia! She has been quite ill with this condition from time to time. Anaemia makes her feel bad, but hypercalcaemia makes her feel nauseous and adds to her dismotility issue. She will also start napping through her bad days, which is how we know she is feeling really not herself!

Kaitlyn's hypercalcaemia is caused by her bones leeching calcium into her blood stream! This is a continuous event! High levels of calcium in the blood stream is NOT normal and like any other electrolyte/vitamin imbalance can be very harmful! Our doctors feel that Kaitlyn's hypercalcaemia is linked to her SMA or lack of mobility. Curious to me that in the HUNDREDS (yes hundreds!!) of SMA families that we know that not another single family is dealing with this issue. I am inclined to believe that Kaitlyn's hypercalcaemia is NOT related to her "original" diagnosis of SMA (Spinal Muscular Atrophy).

We have been attempting to control Kaitlyn's hypercalcaemia with a biphosphonate. The biphosphonate we have chosen is Pamidronate. Pamidronate is well known in the SMA community as it is typically used to increase bone density. Weak bones are VERY common in SMA and many children with Spinal Muscular Atrophy suffer from dislocated, broken or fractured bones. As weak as Kaitlyn's bones were I am glad to say that her bones to our knowledge were never broken or fractured!

We have been doing pamidronate infusions for the past year. We did do a bone density scan (dexa scan) at the beginning of the pamidronate infusions. We were scheduled for a scan four weeks ago but the bone density machine was broken. I am not too sure what to expect from pamidronate in terms of bone density. I mean if you were constantly leeching calcium into the blood stream and pamidronate locks the calcium back into the bone....and we are not adding even a normal RDI for calcium....then I do not see how we could possibly be strengthening Kaitlyn's bones! I guess the dexa scan will tell.

Kaitlyn has also been diagnosed with bronzing. It is a condition where her skin appears to be bronzed, or tanned. Everyone comments on how Kaitlyn has a beautiful tan....like we would take our paralysed daughter out into the sun and let her burn!! URGH!!! Of course not!

Our next HUGE adventure! A larger van! We have been looking for vans in our price range in Canada for the past few years! Transporting Kaitlyn in our current van with our current ramp system (suitcase ramps 75lbs plus!!) is NOT possible for much longer! We have finally found a van that we are hoping will be perfect! We cannot afford this van,  but where there's a will there's a way!!! It will need a couple of modifications to make it safe for Kaitlyn to travel, but when done it will be better than what we have now.

Thanks for reading folks and I hope to make many more posts in the near furture!


Some pictures of our new way of life ;)

Kaitlyn's port....her life-line if you will:

How the lines are connected...making moving her around even more of a challenge ;)

Kaitlyn's IV pole is getting a wee bit crowded ;)

IV meds as her gut simply cannot absorb meds very well anymore.

It is critically important that we keep everything super clean now!

Our new way of administering medications.

A recent pic of our happy girl :) This one was before the bronzing diagnosis.

This picture was snapped only about a week ago. You can really see the bronzing in this pic.

Gastroparesis and Bacteremia!!!

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
 

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

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.