Manometry Testing Take 5

Almost exactly one year ago, Jim and I took Zack to Boston’s Center for Advanced Intestinal Rehabilitation in hopes of learning some new ways to manage Zack’s intestines. We left with a game plan that included a number of recommended tests. Together with his doctors, we have spent the last year getting those tests completed, except for one – manometry testing. Sadly, Walter Reed no longer performs the test, and our insurance would not allow us to have it done in Boston. And that is how we find ourselves here today at Children’s National Medical Center in Washington, D.C. in the middle of Zack’s 5th round of manometry testing.

I am going to be completely honest. I did not want to put Zack through this again. We have done manometry testing at two hospitals with four different results. We know his intestines are wonky. Why put Zack through such an invasive test again? However, people who are way more knowledgeable about intestines explained that getting another snapshot/data point would be helpful in determining a path forward. With much grumbling and complaining on my part, I finally worked through my dislike of this plan and got myself on board because I needed to be 100 percent in or Zack would sense my fear, and I would never be able to sell the plan to him.

Children’s performs the testing a bit differently. Instead of an overnight admission with scopes and catheter placement on day one, and testing and discharge on day two, this admission last four days. On day one, Zack headed to the OR for endoscopy, biopsies, and manometry catheter placement – both antroduodenal and stomal. This means that woke up with a tube placed in his nose, down into his stomach, and into the first part of his small bowel. He also had another tube threaded through his stoma into his small bowel as well. These tubes have sensors placed several centimeters apart that record the strength and coordination of intestinal contractions.

Zack is a very active child. He never sits still. However, having a tube in his nose and one in his stoma changes Zack. He sits extremely still and is pretty sad. The nose tube stayed in place nicely for the duration of the testing and the doctors were able to get some good data. The stoma catheter was more problematic. There is no way to secure it to the internal intestine or to the outside of his body because of his ostomy bag. The doctors were able to rig his bag to accomodate the tube, but it was pretty messy. I would paint you a picture, but there are some things that are just better left unsaid. Let me just say that it was a very long night for Zack, the nursing staff, the resident, and the laundry room.

Children’s testing protocol has antroduodenal and stomal testing lasting 24 hours, so day two was spent finishing the first round of the test. The plan was to finish the test and head back to the OR for a colonoscopy, biopsies, and placement of the colonic manometry sensors. However, there was miscommunication between the teams and anesthesia was not onboard with taking him back to the OR. That meant that Zack had a free evening to investigate the floor, head to the outdoor Healing Garden, and relax. He was not thrilled to learn that he was limited to a clear liquid diet, but he managed to eat 4 packages of jello and a bowl of chicken broth before being NPO again.

One of the things we have been very impressed by this admission is the Child Life Program. Their intake process flagged Zack. I’m sure it was mostly because he is so cute… but also because of the level of his medical anxiety. They coordinated with a special program they have in place that involved the neuropsychology department coming up with a plan to alert everyone involved in Zack’s care about how best to help him through this admission. Overall, it has been a wonderful help. The Child Life staff has advocated for Zack and alerted the staff to his unique needs. They have intervened when Zack needed a moment to process next steps, they have asked for Barney the Golden Retriever therapy dog to come sit with Zack when he was upset and when they felt he just needed a friend, and they have made sure the teams know to talk about medical issues with us outside of Zack’s room. I wish all hospitals had such strong Child Life programs… .


Transport is here to take Zack to the OR. Signing off for now….

Hug your babies!

~ Dawn

Surgery #10 Update – Home

We are home.  The end.

Just kidding.  I am serious about being home though.

Everything I wrote in the last update was true.  Everything that happened in the OR was different from what anyone expected.  When last I updated on the Zachary saga, surgery and GI thought that Zack’s stomach mucosa had prolapsed.  The plan was to take Z to the OR as an add-on late in the day on Friday.

Both GI and surgery planned to work together to figure out how to handle the situation.  The GI team would perform the endoscopy to look into Zack’s belly.  From there, three scenarios were given.  One idea was to get the prolapse to reduce (go back inside) and be done.  The next was to move the g-tube to a new site and be done.  The third was to add-on to moving the g-tube by doing an exploratory laparoscopy to see if the surgeon could tell why Zack keeps intermittently obstructing.  We were prepared for all three scenarios, but hoping for the easiest one.

Zack was admitted early Friday morning so that he could get ready on the ward instead of in the Ambulatory Procedure Unit (APU).  The APU is a bay style holding area before you go into the pre-op holding area.  This place freaks Zack out and as he is anxious enough about medical stuff.  Being able to be in his own room is just better for the entire hospital.  No, seriously, it’s true…  Since he was being added to the OR schedule, that meant lots of time just waiting around.  This is how Jim and Zack felt about that.
Zack was not happy because the PICC team could not get a vein for his IV.  They decided his body was too cold.  He was wrapped in blankets with eight hot packs tucked inside.  He was definitely warm, but the PICC team was delayed and he was super warm by the time they returned 2.5 hours later.

When they finally took him back, Zack did a really good job of remaining calm.  He was able to remain in his room while he received his versed and wheeled down while he was loopy.  That made taking him back the best experience we have had in years.

The surgery and GI teams scoped him and found an area of “schmutz” (a technical term I assume) on the stomach wall that they were able to clean up.  I guess this was where the g-tube had been rubbing.  When they went to reduce the prolapse they discovered that it was not stomach tissue at all.  It was a keloid.  I read this description of keloids in an article from medicinenet.com

A keloid, sometimes referred to as a keloid scar, is a tough heaped-up scar that rises quite abruptly above the rest of the skin. It usually has a smooth top and a pink or purple color.

The doctors came into our room to tell us they had good news and bad news.  I did not like that!  They told us about the keloid and how it was an easy fix – the good news.  For the life of me, I could not figure out how there could possibly be a down side to that!  The surgeon said that the bad news was that the way they had to fix it was probably going to be something that Zack would not like.  In Dawn Speak, he cut out the area in a big circle shape, then had to cinch the skin closed, apply some ACell wound healing “paper” over it (cool stuff, you should check it out), suture 4 plastic bumper thingy’s (no idea what they are called) to Zack’s skin temporarily and place his g-tube on these.  Got it?

This is a new surgeon to Zack.  The surgeon Zack has had for the last 5 years is leaving the military, sadly.  We have known his new surgeon for some time and have heard great things about him.  However, whenever you work with a new medical team, it takes some time to learn how they work and for them to get to know you.  One thing about Zack is that lots of things that should hurt a lot do not (intestinal things) and things that should not hurt do (skinned knees).

Anyway, Zack does not seemed phased by this new configuration of g-tube hardware.  It only needs to stay in place for 14 days, yay!  However, for those 14 days he is not allowed to participate in PE, recess, karate, PT, ride his bike, run, jump, you know…have fun.  He has only asked for Tylenol a few times and seems to be walking around just fine.  Our biggest issue, so far, is that the wound and tube are covered in Tegaderm with a little hole cut out for the feeds to attach.  The Tegaderm is like a plastic sheet covering the g-tube to protect it.  We like that, however, to attach the tube to the g-tube, you have to actually grasp part of it that is under the plastic.  This makes attaching the tube quite an event as Zack stomach is sensitive to being pushed on at the moment.

Another issue we had at the hospital is that anesthesia caused Zack to be nauseated for the first time ever.  He was sick in the PACU.  He got some Zofran and was sick again on the ward.  The next morning he woke as happy as could be, except that he was asking the surgical resident and nursing staff for sodium.  They did not give him any.  Shortly after that, he started to say his head was hurting, took off his glasses, turned off the TV and asked me to close the blinds.  Then he started telling me that he needed his stomach vented because the IV fluids were filling his stomach too full.  We called the nurse and when she asked him how he felt his said, “Not awesome.”

Zack was given some Zofran and IV Tylenol and still did not feel great.  The therapy dogs came to visit and he pet them and went back to bed.  Zack does not stay in the bed unless he feels pretty bad. The surgeon came in and said that we should give him his sodium pills and start his CeraLyte back up, but keep him on just sips of clears.

Another thing that happened yesterday was that the surgeon was able to see Zack go 5 1/2 hours without output and the nursing staff saw his belly distend.  Zack’s GI knows this happens frequently and we know that there is really nothing we can do about it, but it is weird and frustrating.  The surgeon agrees with everyone else that he is obstructing intermittently. We talked about living with this for the past two years and being able to continue doing so since it has not become an emergent issue.  But we were also able to talk about how that keeps us a bit anxious most of the time because we know it can lead to more serious issues.  We left it at that, but at least he is aware and thinking about it now as well.

Happily, after Zack received all that sodium and a had good night’s sleep he woke up back to normal.  He was thrilled to order a pancake for breakfast and noodles for lunch and could not wait to get home.

He got his wish and his best buddy was here waiting to greet him.  Now for mom and dad to get some rest……

Hug your babies!  But not too tightly if they have just had abdominal surgery….

~ 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.