I will begin by saying that much of what I speak of I am just learning. We still don't have many answers as test are still pending and probably won't have all the answers until we return to the GI doctor on June 30th. So some of these things I mention are just what has been said to us these past 3 weeks in the hospital. We actually just went for a GI follow up on May 25th. She had a swallow study and a gastric emptying scan done a few months prior to this appointment. We know she didn't do well on the swallow study but was going to find out results of the gastric emptying scan. Annabel has had problems on and off with abdominal distention and almost all her life with severe constipation. She had taken miralax the last three years and Laculose her first two years of life. Last summer she had a bowel resection and this provided good relief for probably 4 or 5 months. Then it seems that things were creeping back to her old ways. More distention, more discomfort and less and less calories. The gagging and retching had stopped after the resection but was becoming a larger problem. Dr. B had given us Zofran and that seemed like the answer for about 5 weeks. Then we noticed in the late afternoon like the dose wasn't holding her over. We have had a few ER visits as of late and every KUB(tummy xray) notes obstruction/ileus.
This brings us to the appt. on May 25th with GI. Annabel had been in much pain the last few days, we were down from 1000 cal. daily to about 400 cal if we were lucky. With this she was uncomfortable. You have to remember I use a pump and when someone tells me that she needs this schedule I am going to do all I can to pump that food into her. It was obvious something wasn't working from feedings, the bowel, to gagging and retching. We have always felt Annabel endures much pain and her pain threshold is very high. So the GI doctor could see upon our arrival that she was very uncomfortable and sent her for an xray. The KUB came back and we were told to return right away to his office. Again it showed obstruction/ileus and he looked back at her xrays. I asked then about the gastric emptying scan done a few months back and what the results were. It showed very delayed emptying and that she had motility issues. He admitted her then and placed her on IV. The diagnosis early on was pseudo-obstruction. It was described to me as all her food goes in, and it just sits there and backs up, very little stooling comes out except very watery smears. Then after days of this watery diarrhea, like 15-20 diapers daily. Each and every time they tried to put her back on feeds her tummy would become very distended. After maybe a week she was placed on TPN/Lipids. She had a PICC line placed to receive this nutrition. Her G button was switched to a GJ button in the hopes that if her stomach wouldn't accept the food then her small intestine would. Your small intestine takes in much small amounts of feeds so therefore she would now need to be fed continuously. The most she was able to take in was 19 mls/per hr. and then they had to discontinue this. She was many days just on TPN letting her tummy rest. Then we would begin again at 5mls. and she was never really able to tolerate more than about 12 hrs. For anyone who has mentioned that she must have lost a lot of weight, the answer is she was back up at discharged to the weight she was admitted with. The TPN has all your nutrition/vitamins/cal and the Lipid she is on also via the PICC line has your fats/calories also. (This is how I understand it, may not be totally right!)
The problems with the PICC line is you have to watch for many things, such as fever, infection, swelling, soreness, redness, etc. The morning after the PICC line she ran a fever above 104 so then you have to do several days worth of blood cultures. This line is placed in her upper right arm and ends just right above her heart. The last few days she had a motility workup. She was put to sleep for the endoscope and the colonoscopy. They also took several biopsies. Then they placed several tubes in her abdomen through her bottom. The next day she was hooked up to this machine that recorded pressures and functions from her colon, stomach, small intestines, bowels, etc. She was given a few meds at different times to stimulate motility, fed her formula to see how her body processed it, then given laxative to see what happened. This is the test that will take a couple weeks to compile all the findings of these two days. They are hoping that this study will indicate if this is a temporary issue (maybe just a bad virus or infection) or if this is a progressive type issue that we will need to deal with. We obviously hope this to be temporary due to the fact that the TPN isn't good for your liver. She does have to have blood drawn weekly and this is a blessing of this PICC line. No sticking her for her draw.
I will relay some of the findings but there is no plan of action/care as of yet. Her colon is very distended, microvilli is small/immature, small intestine is enlarged?, and her cecum is either twisted, malrotated, floating or permanently in the wrong place. Also some of the biopsies in her colon (I think) were in question. I will continue to patiently wait for the end of June for the answers.
The hardest part is that she is hooked to 3 pumps and then we having to vent her g-tube (stomach) 24 hours a day. I should have appreciated how easy it was just to pick up and go. Now I find myself untangling tubes most of the day. With all this new care for her we have two new nurses. They come into our home for 12 hrs. 7 days a week. They are very careful and both so sweet. They will attend school with her also. This will not take away the time her teachers are able to spend with her but will allow them to not do as much of the medical care that is now being required.
Annabel is happy and smiling. She is stressed more than usual and does some different things now. It makes me sad because I know it can't feel good. She is doing lots of hand wringing, seems to not be able to keep her legs as still, several times daily her eyes roll back in her head and she becomes stiff and shakes. She smiles as soon as we say Annabel, stop that!
Kourtni had plans to return to the orphanage for the month of June. Her aunt operates an orphanage there and Kourtni went last year. I was so lucky as was Annabel to have Kourtni here since September. I was able to take part in many things for the twins senior year. That was my goal with having help. Since Annabel will now require nursing, we will no longer be able to have Kourtni. My hope is that when we attend the SOFT conference that she will still see Annabel.
Thank you to all for the prayers and emails concerning Annabel. Life will continue just with a few more tubes and nurses in tow. Thank you to my family, Mel, Derek and Linzy, Tara and Tanya, Carly and Colette for bringing me the things we needed, visiting and playing with Annabel, Tyler for calling via Skype to talk with Annabel and myself, to Michele and Sophia for my Lenny subs and all the home cooked meals, and to Kevin for my turkey/dressing, to Hannah for washing my clothes and ironing Ananbel's gown, riding home with us and to Hannah and Matthew for fetching me food also. To all who called your support was very much appreciated.
Some really good new is that Tyler will be back stateside mid-late July and hopefully home late August. We are ready and I know he is ready to be back. Linzy and Derek's wedding is being held off until Tyler can return and we can all be together as family.