Sunday, November 21, 2010
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:
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!!!
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!
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!!
Saturday, November 13, 2010
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:
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 :(
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.
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!
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!!
Tuesday, November 2, 2010
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:
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.
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.
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!!
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!
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.
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.
Wednesday, October 20, 2010
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!
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!!
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.
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 :(
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 :)
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
Monday, October 11, 2010
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:
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.
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!!
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) :)
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!!
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.
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!
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!!
Thursday, September 16, 2010
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)
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 :)
Tuesday, September 14, 2010
Wednesday, July 28, 2010
Monday, June 14, 2010
Saturday, June 5, 2010
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 :)
Thursday, March 4, 2010
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!!
Monday, January 25, 2010
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
Jacquelyn d'Eon - Fiddle
Matthew Stanton - Guitar/vocals
Cecile Martell - Guitar/vocals
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 :)