Gastroparesis :(

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


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

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

September 23

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

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

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

Sept 23

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

Sept 24

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

Sept 25

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

Sept 26

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

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

Sept 27

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

Sept 28

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

Sept 30

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

October 1

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

October 5/6

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

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

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

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

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

October 7

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

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

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

October 8

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

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

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

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

October 9/10

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

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

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