This week Jim, Zack, and I made another trip to Boston Children’s Hospital. Zack is not currently “sick”, but he is still having lots of issues with high outputs and electrolytes. Zack’s surgeon in Boston and our doctors in Maryland felt it would be worth driving up to see the Center for Advanced Intestinal Rehabilitation to see if they had any new ideas that might help us control Zack’s output.
Our first day here was spent meeting with Zack’s surgeon. While we love the care Zack receives at Walter Reed, the nature of a military hospital is that doctors leave, get deployed, and retire. Zack’s surgeon in Boston has known Zack for five of the six years he has had an ostomy and has performed four of his ten surgeries. It is important to us that she remain involved in Zack’s care as she knows his history extremely well, thinks about him when we are in Maryland, and talks about his wonky intestines to her colleagues to try and find new ways to help him.
We discussed several things with her. We talked about Zack’s weight and the issues we have been having keeping that up. Even though his weight is not great, he has grown taller and that made her pretty happy. We discussed her thoughts on Zack’s high output and some theories as to what might be causing them. We also talked about Zack’s intermittent obstructions and what to do about them.
After our visit with Zack’s surgeon, we headed to the lab where he had about ten vials of blood drawn for his visit today. In a most awesome turn of events, Zack did not fuss during his blood draw. He was a bit vocal about making sure they were going to use a butterfly needle, but did not fight getting the blood draw. Jim and I were able to breathe easier after that.
Today we met with the team from the Center for Advanced Intestinal Rehabilitation. This team usually works with children with short gut. Short gut means that the patients have very little intestine left in their body. Zachary has a lot of intestine left, but his output is similar to children with short gut.
Our visit was very interesting. In anticipation of our visit, the team gathered a lot of information from Walter Reed and Cincinnati and reviewed it. When we arrived, we met with one of their nurse practitioners and one of their dietitians and went over Zack’s history in detail. It took about an hour and a half to go over all of this. If nothing else, this team has a very complete picture of Z’s history.
Once we completed that, the doctor came in and we discussed lots of things. We talked about possible causes for Zack’s intermittent obstructions and needing to take a closer look at them before starting any new medication to try and slow his output down. The fear is that trying to slow Zack’s output down if he indeed has two narrowed areas in his intestines would really obstruct him. To do this, Zack would need to have another endoscopy/colonoscopy, a procedure called a stomagram (putting dye through his ostomy to see how it moves through the intestine), and an abdominal ultrasound to rule out a peristomal hernia. All of these tests can be done at Walter Reed. IF these narrow areas are thought to be contributing to Zack’s outputs, they will need to be fixed surgically. Don’t tell Zack.
Side note: When we got to this part of the discussion, Zack started getting angry. However, when asked if he wanted to leave, he said yes! He was then able to leave under control and hang out with staff in the waiting room. It appeared he had a great time.
The doctor also suggested redoing manometry of just the small bowel to see if this could be a reason for Zack’s high output. Jim and I are not sure how we feel about that. Historically, manometry and Zack do not go well together, but we are willing to discuss it further. The doctor said that he would want to run this by his colleagues before making the decision to move forward with this test.
Another suggestion was to look into changing Zack’s formula. He has been on his current formula for several years. It has a high osmotic concentration which could be increasing his output some. Changing to a formula with a lower osmotic concentration may be beneficial.
We also discussed Zack’s ACTG-2 genetic finding. The doctor felt confident that Zachary’s main issue is chronic intestinal pseudo-obstruction. Because of this diagnosis, he feels that Zack really should be seen back in the GI clinic that specializes pseudo obstruction/motility as they might have some other thoughts. Zack met with them three years ago, but at that time he did not have the ACTG-2 finding. Evidently, this diagnosis changes some things about managing his case and the pseudo obstruction clinic might be better able to handle these things.
One good thing that we learned is that chronic intestinal pseudo obstruction varies in severity. Zachary is lucky in that his version of this disease is not the most severe type. However, as the doctor said today, it isn’t an easy version either.
During our time in Boston we were also able to reconnect with friends from our time at the Ronald McDonald House in Cincinnati. Their son just had an incredibly tough surgery and we were able to see them in the ICU. Zack and Kayra are now connected on Roblox and have spent quite a few hours playing online together already. (Don’t judge. Electronics are life savers during hospital stays/appointments.) Seeing our friends was one of the best parts of our trip.
And so the mystery continues…..but at least we have a plan to investigate more things and new eyes on Zack’s case.
Hug your babies!