July 2018 Update

Time for a Zack update.  I’m tired, so I don’t know how fun this will be to read.

For the past few weeks, Zack’s belly has been hurting.  While Jim was away Zack had two episodes of pretty intense pain that were fairly short in duration.  He was having his usual lack of output during the day along with a swollen stoma and prolapse thrown in to the mix.  Adding to the fun was a lump that randomly appears next to his belly button.

This face is Zack’s pain face.  It kills me.

The lump…sometimes it is on the right, sometimes on the left….

Additionally, Zack has been suffering from some pretty intense anxiety.  He is worried about dying at night.  The worry is so great that it keeps him from falling to sleep.  Seeing him so scared and being unable to calm him is pretty hard.

Last week Zack had a hard day with belly pain, distension and lack of output.  He thought he needed to be hooked up to his feeds because he was soooo hungry.  Turns out he actually had a belly ache.  Once we attached him to his feeds, he began crying in pain. Jim and I felt he was OK and got him ready for bed and then things went a bit wonky.  Zack was so upset about dying, convinced he was low sodium and worried about his belly that he actually asked us to take him to Walter Reed.  Zack NEVER asks to go to the hospital.  Jim and I were still not positive he needed to go but, because Zack is almost always right, off we went at 11:30 at night to the ER.

Zack did well until the staff began discussing getting blood for labs and then angry badger made an impressive return.  Jim says angry wolverine is a more accurate description.  Jim needed to restrain him.  The staff discussed giving him ketamine or ativan to calm him down.  I left the room because it was too upsetting to see him like that.  The staff and doctor were so incredibly kind and tried really hard to help Z feel more comfortable.  After about 45 minutes of fighting, Zack was able to calm himself down and no ketamine or ativan were needed.  He was then able to ask the nurse if she could use a butterfly needle to get his labs and even helped her find the best vein.  All labs and x-rays came back within normal limits.

The doctor said that his belly symptoms were consistent with an intermittent obstruction.  These happen to him most days, but this one was particularly painful and his belly was distended making it a bit more intense than our average day.  As soon as his output started back up he felt much better.  Also typical for him.  So basically, more of the same crazy we have every day with some major anxiety added on top for fun.

The next day, Zack’s GI doctor and I spoke.  We know that Zack has narrowing near his jejunum and another 20 cm in from his ostomy.  We know that surgery would probably fix that issue, but not address the underlying issue – which remains unknown.  Given his history and our desire to NOT have more surgery, he came up with a three-part plan to try to help Zack.  The plan is not found in any medical textbook as we are trying to find things that work specifically for Zack’s “unique” body.  The first idea was to try to simulate the relief Zack seems to get with barium by essentially doing a Miralax cleanout once a week.  We are on our second attempt and the reviews are mixed.  Barium seems to give Zack relief for about a month.  The Miralax lasts about a day.

We did not see any improvement the first week.  Zack’s belly hurt the very next day and there was not any real noticeable difference in his output.  We gave Zack his second dose this past Sunday and still did not notice much of a difference.  Zack’s stoma has been just as swollen and his belly still hurts.  However, although his output was still low today, he actually had output.  Zack also asked us to give him more Miralax to help his belly feel better.  So, maybe there is hope?  We will continue this for a few more weeks.

If the Miralax trial does not do the trick, our next step will be to try to stent open his stoma with a red rubber catheter.  Of course, Zack would need some sort of light sedation for that and well……

How would you like that inside your intestines?

Our third option is to do more scopes and get full thickness biopsies to look once again for eosinophils.  If we find those again, we would do a trial of steroids and if we see improvement in his belly, redo the scopes to try to prove the steroids are helping.  There is much more to all of that, but the gist is to try to figure out if we are actually dealing with eosinophilic ganglionitis. Yes, we have been trying to do that for a few years, but were waiting for Zack to be a bit sicker.  He still is not really technically sick enough, but it is time to try.  Isn’t that nice?

In the meantime, Zack’s anxiety has been getting worse.  As I said before, Zack has become so worried about dying that bedtime is just not happening.  Jim and I found ourselves getting upset with Zack and frustrated with our lack of sleep rather than seeing that our child was/is actually really scared.  Today we met with his psychologist and had a telephone consultation with his developmental pediatrician.  Jim and I feel much better after speaking with the two of them and have a new game plan there as well.  Starting tomorrow we will be switching up some meds and adding some new ones.  We are also working on ways to reassure him, working on ways to help him process his thoughts and feelings, and working on ways to help distract him from those moments which are overwhelming.

Zack has also rekindled his love of watching Miracles From Heaven.  It is a movie about a little girl with intestinal issues that are different from Zack, but similar in many ways.  He seems to really like seeing her get an NG tube, a feeding tube and, of course, seeing Dr. Nurko make a cameo apperance.  He wanted to know why God healed her and if He could heal him.  He also started asking me questions tonight about what happens when you die, if you still have a family in heaven and how God heals you in heaven.  While those may seem like heavy questions for an eleven year old, I prefer to see it as a good sign that we may finally be getting to the root of his anxiety.

I have several friends with kids who are chronically ill.  They have reached out to us and I cannot tell you how much that means to us.  While I am sad to learn that their children also suffer from anxiety over medical issues, it is nice to know we are not alone….because in the middle of the night it sure feels lonely.  I hate that kids who are sick struggle with these issues.  AND I am also so grateful that the doctors who care for them take this seriously and are compassionate and kind.

Lest you think all is doom and gloom around here, Zack does have some fun things happening.  His Aunt Carie is in the States for a few weeks and we have some shenanigans planned with her.  Tennis is in full swing (ha!) and Zack has actually been hitting the ball OVER the net this year.

Our neighborhood is full of boys and Zack has been enjoying playing with them and I love watching that.  Captain Awesome is set to start some new training to hopefully learn how to be a support to Zack and Z is thrilled about that.  We have also managed to hit two carnivals this summer.  At the last one, Zack met up with his cousins and that always make him happy.

Zack with Kelsey (in white).  She’s pretty great.

Tonight we made a new summer bucket list and we hope to start marking things off.  High on his list are going to the beach, making slime, going to Hershey Park and playing with Jake.  We still have another month to make all of that happen, right?

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

Pathology Results

Eosinophilic Ganglionitis. Looks strange. Hard to pronounce at first. The reason behind Zack’s discomfort. We have a name. And that’s about all.

I can’t really tell you much about it, although I’ve read every article I can find on the subject. I “kind of” understand what I am reading, for a moment and then I don’t. My brain starts to shut down after reading such big words and trying to figure them out! I know, you thought I knew a lot, you are not wrong. BUT, this one is a bit tricky.

Zack’s GI doctor took time out of his crazy busy day last week to meet with us to go over a new course of action. I appreciate how kind he is to us and how patient he is with our (my) zillions of questions. I feel very confident in what he has described to us and let’s face it, there really are no experts in eosinophilic ganglionitis. I’m hoping our doctor will become the expert because he’s figured out some wonderful way to treat Zack and everyone will flock to him for advice. A girl can dream….

Our plan is to have Zack go under general anesthesia again in the next few weeks for an endoscopy. If you’ve read any of my other posts, you know this is not my idea of a good time, but Jim and I feel it is important. Z’s doctor needs to have a starting point from which to begin treatment and a way to measure the effectiveness of the treatment.

I believe that his entire intestinal tract will be studied. He’ll have a breathing tube and “they” will breathe for him as a scope is placed down his esophagus looking at every part of his upper GI tract. Since he has two stomas, the doctor can look through them both to see his small and large intestines as well as looking from below for evidence of pesky eosinophils. Once we see how much or how little of his GI tract is affected, we can formulate another plan on how to treat Zack. Or his doctor can…you know, because he is the real doctor. I just play one at night.

I have been trying to gain an understanding of the role of eosinophils. What are they and what do they do? I read this from Cincinnati Children’s Hospital:

Eosinophils have many diverse functions

The functions of the eosinophil are varied, some of which are very similar to other white blood cells. They are implicated in numerous inflammatory processes, especially allergic disorders. In addition, eosinophils may have a physiological role in organ formation (e. g. postgestational mammary gland development).

Eosinophilic functions include: movement to inflamed areas, trapping substances, killing cells, antiparasitic and bactericidal activity, participating in immediate allergic reactions, and modulating inflammatory responses.

Eosinophils can be either helpful or harmful

At one extreme, such as in the illness erythema toxicum, eosinophils play the role of a beneficial modulatory element or an innocent bystander. At the other extreme, represented by conditions like Loeffler’s disease and idiopathic hypereosinophilic syndrome, eosinophils are linked with permanent pathologic changes.

That makes sense. Then I read the articles our doctor gave us and it got confusing again. So, this is what I do know:

1. We have a name for what Zack has going on in his intestines. He’s had it for some time, maybe his whole life, now we just know what to call it.

2. It is rare.

3. People don’t really understand it.

4. It can cause pseudo-obstructions like Z had in the hospital.

5. It seems to respond to systemic steroids.

6. We have a doctor we feel extremely comfortable with and whom we know will do all he can to help our guy.

7. My brain could never handle being a doctor.

Zack’s doctor said that after we read the articles, we should send him our questions. I’ve decided that most of my questions are probably unanswerable. Except the part where I ask him to explain eosinophilic ganglionitis to me again, and then again, until my brain finally gets it.