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!!