Zack Update

In the military you can be sure of a few things. First, you will get to meet a lot of amazing people. Second, you will get to travel to amazing places. Third, either you or the amazing people you meet will be moving somewhere new before too long.

Such is the case with Zack’s current surgeon. Zack has been an extremely fortunate guy. Despite the craziness of his disease, he has had incredibly talented and compassionate people caring for him. His original surgeon left us two years ago and it was hard. Little did we know that his new surgeon would be just as amazing or that it would be this surgeon who finally ordered THE test that found Zack’s diagnosis.

When you are the parent of a child with a difficult and complicated disease, having doctors who research your child, think about him after hours, and are always trying to come up with new and innovative ways to help him, the last thing you want to do is say good-bye. Happily, his surgeon let us know leaving the military does not mean he is done helping Zack. We can always reach out to him and have him consult with Zack’s team at anytime. I cannot even begin to tell you how comforting that is (to me).

As I have said many times before, the rest of Zack’s team here is just as wonderful. If only they could all stay here forever…..darn military. Luckily for us, Zack also has an incredible surgeon in Boston. She has been caring for Zack for the past 5 yeas and has performed half of his surgeries. This allows us to have one provider who will be able to follow him all the way through childhood. While that gives us peace of mind in the craziness that is Zack’s life, it does not make saying good-bye to his other doctors any easier.

Enough about that. Zack had his final appointment with his surgeon to discuss our plan going forward. Zack’s pediatrician joined us for the appointment as well. How cool is that? I told you Zack is a lucky boy. Having them both there makes life easier. It can get complicated having several doctors and keeping straight what each one said so that we can update the others. Having a team meeting like that really simplifies things and maximizes everyone’s time.

Together we were all able to all brainstorm his high output. An unconventional idea was discussed. I think I will wait to see how the idea plays out before telling the world about it. We talked about how great it is that Zack’s kidneys function well, because his intestines are falling down on the job. We discussed his obstructive episodes and made a plan for the next painful one. When that happens, we are going to head to the hospital for a CT scan to see if we can catch it in action.

Zack’s surgeon shared a new surgical procedure with us that might be an option for him in the future. I could explain it to you, but it would probably just cause your eyes to glass over. I will spare you, for now…. We talked about his enteric nervous system being out of whack at the cellular level and how best to manage that. One of the most important things we talked about is that before ACTG2 related disorders were identified, obstructive episodes were treated surgically resulting in loss of bowel. Loss of bowel leads to short gut, which leads to TPN dependency. TPN dependency can be life limiting.

To best help Zack avoid TPN, we need to make sure that nobody resects anymore bowel unless it is dead bowel. This should not be hard, because Jim and I are adamantly opposed to any more surgery unless it is life-threatening. Happily (?), all of Zack’s surgeries to date have been necessary and not exploratory. The best thing his surgeon said is that he is optimistic that a management strategy can be identified now that we have a reason for his intestinal dysfunction.

One of the ways we are planning to do that is by visiting Boston once again. Our plan is to meet with Zack’s surgeon and the short bowel team to see what new strategies they have for controlling Zack’s output. Although Zack does not have short bowel (in fact he has most of his small intestine still intact), his gut acts as though he does. If we can get his output under control, his sodium levels should normalize. This is huge because we currently spend an incredible amount of time each day trying to manage these levels. As his surgeon said….no child should have their own Morton’s Salt container.

I bet you have never seen a child so excited about a salt container before. He has been known to open it up and drink salt from it. I wish I was kidding. When that happens, we know it is time to rescue dose the boy and get his electrolytes back in check. Now that we give him CeraLyte daily, drinking from the salt container happens less frequently.

Whew. I am officially out of words for today. It is hard to believe, I know. Thank you for checking in on Zack!

Hug your babies!

~Dawn

Vacation and Boston

Oh vacation, how I have missed you so.  Our family had a great time visiting family in Connecticut, playing in Bar Harbor, Maine and exploring Boston between doctor appointments.  Here are some of our highlights:

Boating in Connecticut.  Zack’s first time on a fishing/lobster boat and he loved it!  He was a man of many questions and is forever bonded with Captain Don.

IMG_20160813_231343_01Zack helping Captain Don drive the boat.

IMG_20160813_162355656Jake and Jim could get used to Zack doing all the work.

IMG_6164Zack and his shark.  Not bad for a first fishing trip!

IMG_20160813_220526112_TOPThe Co-Captains relaxing after a hard day on the water.

Exploring Acadia National Park.  I am not sure how we have so few pictures of this.  Here are Jim and Zack on top of Cadillac Mountain trying to pick up one of the islands in the harbor.IMG_6152

Bar Harbor adventures.  Jim, Zack and I took a 4 hour sea kayaking tour.  See those islands in the picture above?  We paddled to the third and took a break.  Then we went around the third and back into shore.  We saw some porpoises and bald eagles.  Happily, we did not see any of the sharks that also inhabit the harbor because that would have freaked me out.

IMG_6118 IMG_6110We rode the carriage trails in Acadia National Park on bikes.  We ate popovers at Jordan Pond, frolicked on Sand Beach, drove around the entire island and shopped and ate and had a grand old time.  Zack and I also went on a date on Diver Ed’s boat.  Diver Ed scuba dives into the harbor and videos the ocean floor as he goes.  The video is broadcast on board.  Then he comes up with some of the creatures he finds down there and the kids get to touch them all before releasing them back to the ocean.  Zack was so enthralled with the whole thing until the touching part.  He finally worked up the nerve to touch a few things.  Can you tell how hard it was for him to do it?

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After a week of fun, we headed to Boston to see Zack’s doctors.  First we met with Zack’s surgeon.  It was strange to see her in a new hospital, but she was as awesome as ever.

While Zack is currently doing better than he has in years, we still have some concerns.  You may have noticed that I have been talking about how his stoma has been swelling which constricts his output during the day and then goes back to normal when he rests.  His surgeon and doctor here both feel he is prolapsing internally.  One thought is that where his stoma was stitched down internally actually worked this time, but his intestines before that point internally are still trying to get out.  We think they are getting stuck at that point, cutting off the flow.  Thankfully things have been resolving well so far, but it is not supposed to be happening.  We have a plan in place now for IF things go wonky.

Today we met with one of the leading pediatric motility experts in the nation.  He asked us lots of questions.  He said that Zack’s case is interesting because there is a lot going on that does not make sense….yet.  He is hopeful he can figure out what is happening with Zack.  He did say that just because Zack’s small intestine manometry test was normal does not mean his small intestine is functioning normally, just that things are being pushed through.  We talked about Zack’s biopsies that showed eosinophilic ganglionitis, the possibility of autoimmune issues and other things, but he wants to review all biopsy slides, testing, fluoroscopies, manometries, CT Scans…basically everything that has already been done…for himself before giving his opinion.  Jim and I appreciate that and think it is a good idea.  Of course, Zack’s files from both Cincinnati and Walter Reed are so incredibly large that it will take some time for him to get through it all.

The doctor said that there are several drugs we can try to help slow down Zack’s output.  He wanted to talk to his surgeon about a few things first, so we do not have a plan for that yet either.  Even though we left without a plan, Jim and I felt that he was very thorough and we really appreciate that he wants to understand why Zack’s body is behaving this way.  Why does he have secretory diarrhea?  Why doesn’t his colon work?  Is there also a problem with his small bowel?  All questions his other doctors have asked before and have not been able to answer.  Somehow I feel good about his method of investigation and have hope that perhaps he will be able to narrow things down a bit more.  It is good to have hope.

While we were in Boston we were able to connect with some of dear friends that we had not seen since 2011.  We spent a great day at their home catching up.  Why do we wait so long to do these things?  Oh yeah…intestines.  We were also able to see a friend from our time in Minot.  Her daughter has been in the PICU since DECEMBER!  December people!  I am happy to say that her daughter is getting close to getting out of the PICU and to finally going home.  It is amazing to see her faith and strength.  It was a blessing to me to be able to spend the evening with her.

So there you have it, an exceptionally long update.  We are so thankful for our vacation and our time in Boston.  We are thankful that we had a fun time as a family.  We are happy that we were able to come to Boston for a non-urgent visit.  We are happy to have scoped out the area, found a hotel close to the hospital and to know we have friends close by.  While we did not have time to see all of our friends here, we know we will be back! Until then…

IMG_6104Hug your babies!

~ Dawn