I love that it has been awhile since I have updated. Frequent posts usually mean something is up and not in a good way. While Zack still has a lot going on, most of it is seems pretty mundane and not at all interesting to write about. Over the past few weeks, Zack has had a few new things going on, so the time is right to update once again.
On Monday, Zack’s surgeon in Boston reached out to us to let us know that the team there (GI, surgeon and pathology) had gone over all of Zack’s previous testing and biopsies and everything looked “pretty normal.” Given that nobody can figure out what caused Zack’s colon to stop working or why he has such high output, this really was not surprising. We are still waiting to hear what Boston’s pathology department thought of his eosinophilic ganglionitis and hypertrophic nerve cell slides. Those are really the only ones we have ever had come back questionable.
Last week we met with Zack’s surgeon at Walter Reed for a routine follow-up. Since he recently returned from a six-month deployment, we wanted him to actually lay eyes on Zack and bring him up-to-date on everything we learned in Boston. Zack’s surgeons do actually talk when necessary, but going over the emergency game plan we came up with in person made me feel much better.
One of the things that both GI doctors and both surgeons seem to agree upon is doing a trial of high dose steroids with Zack. I have no idea what constitutes a high dose, but for sure more than the 5 mg he was on in 2015.
I was able to tell them that because Zack’s colon worked, then had zero motility, then worked in small areas and then did not work at all again, I am reluctant to try another reattachment of the entire colon. Happily, that is not the intent of this trial. The majority of his colon is definitely done. Because he has had and continues to have such weird issues with his ileostomy that are hard to manage well, the best option would be to reattach his small bowel to his rectum. As I have said many times, his high output makes this a bad idea because it would likely leave him incontinent. Nobody wants that.
The current ideal scenario would be to find a small portion of the colon that actually works to help absorb fluid and save that to help make reattachment possible. I am not sure how this would be tested. Even if the steroid course helped some portion of Zack’s colon function again, he cannot be on steroids forever. We still would not know the cause of the colon failure and how to keep it from happening again. So many questions and things I do not yet understand.
I believe (again…not sure I am correct) one of the questions the doctors have is if Zack’s issues are related to an autoimmune issue and if they do not try the steroid dose, are we risking long term harm to the rest of Zack’s intestines? Not having a diagnosis makes things tricky.
To try and tackle the high output and low sodium issue, Zack began a new drug called Cholestyramine. It is a drug typically used to lower cholesterol levels. It is also used in people with high output ileostomies and chronic diarrhea from gallbladder removal to help slow things down. It works by binding bile acid to help keep water in the intestines.
At first, Zack had a day or two of thickened output, but the volume remained high. The past few days his output has been back to the normal watery consistency. It is possible that it is keeping his sodium levels up, but we have not had labs to confirm this yet. I am basing this soley on the decrease in the number of times Zack is attempting to raid the pantry in search of the Morton’s salt container. Hey, I have a hypothesis, I am making observations and soon I will have actual sodium measurements to support my hypothesis. It’s all part of the scientific method….I could be a scientist in addition to a doctor…you never know!
Zack’s stoma continues to be worrisome. It continues to swell during the day restricting output. On Monday, Zack had zero output for over eight hours. His school nurse said he was off that day. His behavior in school was not stellar either. Tuesday he told us that he felt so much better and that his pain had been up the day before, he just did not want to tell anyone. Tuesday he had high output and today I heard that he did not have any output before lunch. I will be interested to see how the rest of the school day goes and what his stoma looks like when he gets home.
Zack is used so used to living with some degree of constant pain, that it is just part of how we do life. I often wonder, though, if on the days where he is extra crabby if pain might be a reason. Honesty in rating his pain level to others is not one of his strengths, even when he is really sick. Admitting he does not feel well usually leads to a decrease in fun and an increase in doctor visits and he is not having that!
I talked to his surgeon here about this craziness – the swelling, lack of output and pain. I asked him if I had permission NOT to worry about it since Z’s stoma is not technically prolapsing. He said I still need to worry. I already knew that, but I was hoping that he would tell me I was being ridiculous and to chill out. The dreams I have in life are really pretty simple…..
Given all that, things are still millions of times better this year than the previous three years. Zack went Trick-or-Treating and walked the entire neighborhood for the first time ever! He participated in his school fun run and was able to run/walk 4 miles! The biggest news is that he went two months without an actual doctor visit. Granted, I still communicate via text with his GI most weeks and Z still has frequent labs, but not having an actual appointment is huge.
Because things are better around here, I was actually able to go and visit our oldest son in Texas. Drew is in his second year of graduate school at the University of Texas in Austin. He has been there over a year and this is the first time I have been able to travel to see him! It was a wonderful weekend and, as a bonus, I was able to spend time with family and friends in Houston, San Antonio and Waco.
Our family is also getting excited about another exciting development. Because Zack is doing better, we are getting a puppy in January! We intend to train this new pup to become a therapy dog with the hope that he will be a comforting companion for Zack both when he is sick and when he is well AND that as he gets older we can bring him to the hospital to visit other children who need some love as well. We are all very excited, especially Zack. He cannot wait to meet the puppy and has already picked out a name – Captain Awesome Penrod. We many need to work on that!
Hug your babies!