One Week Later….

In my last post, I signed off quickly because Zack was headed to the OR for his colonoscopy, biopsies, and manometry catheter placement. The rest of his hospitalization was not fun for him (was any of it fun?). Zack required a lot more mom and dad love and encouragement than he typically does in the hospital. By the time he was discharged on Friday and we made it home, it was time for dinner and we all just crashed.

Let me take you back to last week. Zack woke up in the PACU and he was NOT amused. He was in pain and needed to have a bit of Fentanyl to help calm him down. Even then, he just kept crying about how much his belly hurt. While that is very typical for Zack and his colon, it is still hard to know he is hurting and there is little we can do to help him.

We spoke with his doctor about Zack’s procedure, and he told us that it was pretty tough. He was unable to get his scope past Zack’s descending colon because it was filled with white globules that were like cement. Even with irrigation, he could not get them to move. He said his colon did not look normal (shocker), and there were many areas that bled very easily that he did not expect to see. Those are not his exact words, but my version. He said that given that Zack’s colon had been resting for a number of years now, he would have expected it to look much healthier. Nothing really surprises us when it comes to Zack’s intestines anymore.

After the PACU controlled his pain, he made it back up to the floor. This particular testing protocol called for Zack to eat and drink soon after he returned to his room. Yeah. When your belly hurts, eating is not high on your fun things to do list. We finally got him to drink some juice, and the team called it good. Luckily, he was able to finally settle with Tylenol and didn’t need any other pain meds. Zack told me that he did not want me to take any more pictures of his face because his belly was hurting and he felt crabby. I believed him.


We will follow up with Zack’s doctor on October 5 to get the results of his testing. I have no idea what to expect. To my untrained eyes, it looked like there were some contractions happening in his colon. Unofficially, I heard that there was some motility, but ineffective and not through all areas. I guess we will find out soon.

There are two other things that I want to document here so that I do not forget them. First, when the OR weighed Zack the first day, he had lost almost 4 pounds since his last weight check on August 10th – and he was wearing shoes in the OR. He was weighed at 29.3 kg (64.46 lbs) in the OR and had been 31.3 kg (68.86 lbs) at his well-visit. That was very disappointing because we are working so hard to get Zack to 70 pounds! Second, as I type this a week after discharge, Zack still has some impressive bruising from 2 of his 4 IV placements. I’m surprised how badly he bruised. Since I look back on this blog to help me get procedures, surgeries, and hospitalizations in order, I appreciate you indulging me this extra paragraph.

While all of that was happening, we were also juggling getting our pup, Captain Awesome, to different doctors in both Maryland and Virginia. Cap’s red blood cell count, platelets, and liver values were very concerning. He had an abdominal ultrasound, liver aspirates, and tons of blood work. All of the vets were shocked that they did not see cancer….in our newly turned 3 Golden Retriever. Even though they did not find cancer, we were being prepared to lose our pup. Zack knew Cap was sick, but not how bad things were.

I am happy to report that Cap’s blood work is starting to improve. His platelets are still very out-of-whack, so we have to be gentle with him and try not to let him get hurt. His ears have gone from yellow to almost pinkish again and he is eating. You know a Golden is sick when they do not want to eat. He still has a way to go before we feel confident in his recovery, but we have hope!!

To make life more interesting, three days after we got home, Zack started 7th grade -virtually. Thanks, COVID. Zack loves routine, so getting back into the school groove has been good for him. In true Dawn fashion, I finished painting the new school room/office and putting furniture back into it the night before school started. To be fair, August was a crazy month. I’m glad I did it at all!


I will leave you with some pictures of Zack enjoying his new hobby. One of our good friends introduced Z to fishing during quarantine and he loves it. It is the perfect hobby for this time of isolation. He appears to be quite the fisherman. He has caught (and released) at least one fish each time he has been fishing. Jim and I are just grateful that he wants to be outside.

Until next time….

Hug your babies!

~ Dawn

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

Our Crazy Life

When last I wrote, the world was humming along like normal – people went to work, kids went to school, and toilet paper was available in grocery stores. Oh, the good old days….

There is something about living through a pandemic that made wanting to blog pretty low on my list of things to accomplish. On the top of my list was cleaning all the things, making sure to isolate, keeping Zack safe, and learning how teach special education classes. You know, the normal things each of us are called to do now.

A surprising benefit to isolating was that Zack actually grew taller and remained pretty healthy. He did not have many prolapses, he did not obstruct much, we were better able to regulate his formula intake and electrolyte supplementation, and he didn’t run around expending ridiculous amounts of energy. Even though all of those things were great, not being able to see friends and family or participate in social activities was hard.

I do not know who coined the phrase, “All good things must come to an end”; however, that has indeed seemed to be true for us. Things were going along pretty well medically until a few weeks ago when Zack got ready for bed and told me he had a stomach cramp. I had noticed that he had not had any output for a few hours and made a mental note. Fast forward to 1:00 AM … the dreaded, “MOM!” rang out and I knew. He was having waves of cramping, minimal output, and was incredibly uncomfortable. Zack started saying he wanted to go to the hospital. We got dressed and ready and then he said that maybe he could make it a bit longer. Mercifully, he was able to fall asleep for a few hours before the pain returned.

When he woke up he was still not feeling well. After texting with his doctors, we decided to bring him in. I will spare you all of the boring in between details, but whatever was causing the 14 hour blockage ended up resolving itself at the emergency room. My theory is that the three bananas he ate at one time caused the issue, but that’s just a theory. Whatever caused the problem, it was definitely the most intense obstruction Zack has had in months. We had packed our bags expecting to stay and were thrilled to be able to come home without being admitted or having surgery!

Zack’s feelings about being in the Emergency Room once he started feeling better. He was ready to get the heck out of there.

You know there has to be more to the story than an obstruction that resolved on its own, right? On Monday, Zack and I were invited to use our friends pool. It was blistering hot and Zack was so excited that he couldn’t wait for me to finish getting ready. He jumped on his electric scooter and headed up the street. I followed along about 5 minutes later. As I rounded the bend in our road, I heard someone crying. It took a moment for me to realize it was Zack. I saw him in lying on the side of the street, on his back, and then I heard him crying, “Help me!” My heart stopped and I ran faster than ever before to get to him. Zack said he thought he broke his arm and he was most certainly correct. His right arm looked like Joe Theismann’s leg injury, but on an arm. (I’ll spare you the pictures. You are welcome.)

Thank goodness for technology and cell phones. It was blistering hot and everyone was inside staying cool. Zack and I were the only people outside and we really needed help. I was able to reach two friends who raced to our rescue. Liz helped me get some ice on his arm and some shade over him as he was on the blistering hot asphalt, sweating like crazy, and in some pretty severe pain. Our friend Sarah rushed to render aid. Interesting side note: Sarah is Dr. Sarah for those who remember Zachary’s blizzard medical emergency. She is Zack’s guardian angel – for real. It was clear that he needed emergency medical attention and a plan was formed. Sarah, Liz, and several neighbors gathered to help Zack while I ran home in to grab our insurance cards.

Zack was incredibly brave as we drove to the closest hospital. He was brave when they tried 3 times to get an IV started. He was brave for the nerve block that was injected directly into his broken arm. He held it together so well….until they told him he needed to be sedated to manipulate his arm back into place. Angry Badger made a small comeback at that point. It’s always hard to try and explain Angry Badger to people who do not know Zack. It’s one of the reasons I am thankful for the hospitals that treat Zack routinely. They understand that medical trauma anxiety is a real thing for kids who have had multiple invasive procedures and surgeries. The fear, anger, and range are real and scary. I warned the medical staff. I am not sure if they actually believed me. They believe me now. Happily, Zack’s anger was short-lived this time, and I was able to let him know how proud I was that he was able to control himself.

The break. That is not his wrist, it’s his forearm.
All fixed.

I was able to be in contact with Zachary’s pediatrician during all of this. Words can never adequately describe the comfort it brings me to know Zack is so well cared for by his medical team. We made a plan for follow-up care because Jim and I knew we wanted Zack to be treated by the orthopaedic clinic at Walter Reed rather than our local hospital. There are many reasons for this, but one of the main reasons is keeping his team involved in all areas of his care makes life so much easier.

Zack is sporting an over the elbow cast and has determined that casts are not as cool as he thought. Because of the type of break in Z’s arm, he will require weekly X-rays for a bit. Happily, it appears that his bones were set well and should heal completely without surgical intervention. Sadly, his cast was a mess and needed to be cut and pasted to make it livable for the next 8 – 10 weeks. The doctor did not want to take the whole thing off and risk moving his bones. The middle part of his cast remained and the hand and elbow portions were cut and replaced. Much to Zack’s delight, his cast is now red.



Next up medically, Zack has his yearly genetics appointment at the beginning of August. He still needs to have his 13 year well-check. And the big one….Zack will be having his manometry testing at Children’s National Medical Center the first week of September. Zack will NOT be thrilled about that. We have purposely not mentioned it to him as it will certainly bring Angry Badger back with a vengeance and nobody wants to see that until absolutely necessary. It was nice to have a few months of quiet.

Finally, and not at all related to Zack, Jim and I celebrated our 30th anniversary in between all of this crazy. Our celebratory trip was cancelled due to COVID and I was grumpy about it. Our 25th anniversary celebration was cancelled because Zack was discharged from the hospital that day. I just could not shake the self-pity. It was not a good look. (What can I say? I’m human.) Jim and our neighbors hatched a plan and we had a really nice celebration right on our own deck. Decorations, wine, and champagne courtesy of our friends. Dinner from a local restaurant. Zack socially distanced outside with neighbors. Zoom call with all of our boys. It was a great night of celebrating this crazy life we lead together.

Beautiful night.
Happy Anniversary!


I hope that pandemic life has been quiet for you. I hope that you have been getting lots of home improvement projects completed, new hobbies started, and that you have been safe and healthy. I can’t leave without a public service announcement: Wear your masks! If not for yourselves, do it for kids like Zack who need the extra protection.

Hug your babies!

~ Dawn



Happy New Year!

In my blog life, no news is good news – medically. Zack has had a bit of testing since November and all of it resulted in good news. I like that. Let me catch you up…

In December, Zachary had a big ultrasound appointment in radiology to check for a hernia and to check on some other internal organs. Happily, his kidneys, bladder, spleen, and liver all looked great. No hernia was found – also great news. The radiologist did say that he could see that Zack’s abdominal wall had some areas of thining which could account for the bulging we see next to his incision line when he is obstructing. But it is not a hernia that needs to be surgically repaired. Yay!

Zack also had another procedure in radiology later that month. Zack has a surgeon-made mucous fistula at the bottom of his scar, above his pubic bone. This mucous fistula is tiny. It is about the same size as the point on a pen. His surgeon created it back in 2015 to help give us access to his colon. His GI doctor and the radiologist were not sure if it would be possible to access it at all and wanted to try it in the fluoroscopy suite. Zack told the doctors that he did not need any pain medicine because he was a warrior. Big brother Jake was there to help distract Zack and he didn’t even need to do anything. Zack allowed them to put a little, tiny, NICU catheter into the fistula. Then the radiologist pushed some contrast to see if it would go in and it did! Zack’s GI told the radiologist that he only wanted a small amount of contrast placed since Z usually retains things in his colon.

There was much rejoicing in the room that we had access AND that Zack was amazingly calm. He did not seem to be in any pain, he was happy as we left, and he was asking for food – all good things. About an hour later, all of that changed. Zack started moaning in pain. When we got home, he wanted me to “vent his stomach” to make the pain stop. He couldn’t understand why that would not help his colon feel better. Soon after, he began crying in pain and asking me to make the pain stop. He continued to be in distress until he put himself in bed at 4:00 and fell asleep. When Z falls asleep, something is definitely not right. We woke him up to see Santa come down our street on a fire truck. But Jim had to carry him outside, and he went right back to bed. I am happy to report that he felt much better in the morning. However, the last time we tried to access his colon, the same thing happened – intense pain and crying. Not sure what that is about and haven’t really unpacked that with his doctor yet.



Zack quickly put that behind him as his brothers and sister-in-law were here for Christmas, and there was much fun to be had. We crammed a lot into a few days and it lifted all of our spirits.


This past week, Zack attended Outdoor School. Where we live, all 6th graders go to camp for a week to learn about our environment, the Chesapeake Bay watershed, native plants and animals, and lots of other things. It is a week that almost all children look forward to, and it is lots of fun. Unfortunately, Zack was unable to spend the night. The nursing staff was more than prepared to take care of Zack overnight. However, because of all the care he needs overnight and the fact that the kids sleep in cabins, the staff was concerned that his care would be disruptive to the other campers. Jim and I completely agreed with this assessment. I was able to drop Zack off each morning at 8:00 AM and pick him up at 9:00 PM. Zack was very sad that he was not able to spend the night. The principal worked hard to turn a no into a win for Zack (she’s amazing). She allowed him to be assigned a cabin with the other boys, bring his camping gear, and have cabin time during the day. The only thing he did not do was sleep in the cabin.



The past two school years were incredibly hard for Zack. His doctors told his elementary school that he just needed some wins. Outdoor school worked hard to give him a win and I am so thankful. His middle school has also worked hard to give Zack some wins this year. Zack has been happy to go to school, and he is excited about learning again. There are no words to explain how Jim and I feel about that, but an amazing sense of relief would be close.

Zack also just turned 13! He is quick to tell us that he is a teenager now and needs more sleep. If only that were true…… We look forward to consistently sleeping through the night one day, but that day has not yet arrived. He had a family pizza party this past weekend, and the celebration will continue as he and Jim tackle the AT trail for a few days on a Penrod traditional father/son 13th birthday trip – dates to be determined. They will just go overnight so that Zack can get all of his medicine, but it will be fun anyway.


Today we went to Children’s National Medical Center in Washington, D.C. to meet with a new GI team. I did NOT want to go – for a number of reasons. However, Boston recommended some testing that Walter Reed is not able to do, and our insurance denied our request to have it done in Boston – don’t get me started on insurance right now. Happily, our insurance will still cover Z’s surgeon in Boston or I would have cried many tears.

When we arrived, Zack was given a different doctor than we had expected, but this turned out to be a blessing. The doctor he saw was young, energetic, and excited to learn about Zack’s case. He asked a lot of questions and was extremely thorough. We do not have any dates for the testing or admission yet. We expect it will take a few months because the doctor wants to review all of Zack’s biopsies, scans, labs, surgical notes… basically everything, before proceeding so that he has a good understanding of everything that has been done and explored to date. He told us that he had written a paper on eosinophilic ganglionitis recently. That amazed us because there is very little literature on eosinophilic ganglionitits. Nobody really knows if Z has this or not. It is something that still needs to be studied further, but we were impressed that this guy knew more than just the name of it. He feels similarly to Boston, that the ACTG-2 finding makes chronic intestinal pseudo-obstruction the likely diagnosis, but he wants to rule out eosinophilic ganglionitis or see if it is a co-diagnosis. This is what Zack’s GI at Walter Reed has been working towards as well, so we were happy to hear that.

Wow are my fingers tired! I apologize for not having a smooth transition for this closing. Words are escaping me at the moment. That will teach me to wait so long between updates. It was pretty nice not to be on the computer, though. I’ll update as we get more information. Until then, Happy New Year!

Hug your babies!

~ Dawn

Testing

I feel like it’s been ages since I’ve written anything on this blog. It crashed. I had to get it restored. It took a long time. It was a pain and painful. I realized once again that I need help in computer related areas of my life. I wish computers were a thing when I was growing up. I remember being in school and a teacher saying that one day everyone would have a computer in their house. I remember thinking that sounded far-fetched, but look at us now. I almost feel like I’m living in an episode of the Jetsons. I just wish I had the amazing dog walking sidewalk and Jane Jetson’s maid.

Welcome to my brain. Focus, Dawn.

Yesterday Zack had a stomagram. It was part of the recommendations Boston sent to our doctors here as next steps to rule some things out. Zack had been working hard in therapy to get ready for the test and boy could we tell! Thanks, Dr. Caitlin!

Zack’s GI and his pediatrician worked hard to make things low stress for him as well. And guess what? Zack was a ROCK STAR! I believe his GI doctor’s exact words were, “Who is this child?” I think Angry Badger left the country because there was no procrastinating, no fussing, no crying — just a compliant and happy child. I do not want to overthink it. I’m just so incredibly grateful to everyone who has worked so hard to help Zack overcome his incredible fear and anxiety. Even if it only lasts for this one day, I’m so proud of Zack.

The other amazing thing that happened was that his pediatrician arranged for immunology to come down to the fluoroscopy suite to give Zack and his GI doctor their flu shots together. When I say that I believe Zack has the best medical team, I am not exaggerating. Shots normally freak Zack out and require two people to administer them. Not this time. Zack stuck his arm out and barely flinched when the needle went into his arm. Seriously, I am so amazed. Zack did have a little bit of medication to help take the edge off his anxiety, but he really shocked us all with how laid back and compliant he was.

Because I am pretty sure my family will want to know what stomagram is, I thought I’d give you my description of what happens. Zack’s stomagram was a dye test that was performed by inserting a catheter into Zack’s stoma and injecting contrast into his intestines. While this was happening, x-rays were taken in real time (like a video) to watch how the fluid flowed. The purpose of this specific test for Zack was to look for strictures and areas of dilation in the intestines. It can be a bit uncomfortable, but Zack seemed oblivious. Unbelievable…

It appears that the stricture we saw previously is no longer there. This is awesome news because I believe (I’m never quite sure if I’m right) that this means we are one step closer to being able to try some new medicines to try and control Zack’s output. One medication that has been mentioned is octreotide. I don’t know the name of the other one, but Zack’s doctor believes it may be an orphan drug? And for all I know, there may be many more to try. However, the presence of strictures makes the likelihood of those medications obstructing Zack much higher and nobody wants that.

Zack will also have an abdominal ultrasound in a few days to rule out a hernia. Ultrasounds are easy for Zack, so I am not worried about him freaking out over that. I think that will be the last test for now. Zack’s doctor(s) and I are still debating the need for more manometry testing. Some people with the ACTG-2 gene mutation can have waxing and waning symptoms. BUT even if his colon had some action, there is no way we would ever reconnect Zack given how sick he became last time we tried it. In addition, his last two manometry tests showed very little and no motility. On the other hand, I understand that the more information we get on Zack’s intestines the more we will be able to understand his specific disease. I’ll save that discussion for another day.

Now that we are back home, I have questions. Is a stricture able to resolve on its own? Could that really have been a snapshot of Zack’s intermittent obstructions? He also has a stricture higher up closer to his stomach, but this test did not go up that far. Do we care about that one? Is it small enough not to worry about? You would think these questions would occur to me when Zack’s doctor is around, but they don’t.

Zack was actually tired on our drive home and almost fell asleep. That literally never happens unless he is really sick. I’m guessing that the little bit of medication he had on board helped with that situation. It was a good day all around. I love good news. More than that, I love that we were able to see Zack feel comfortable in a medical situation for the first time in years.

Hug your babies!

~Dawn

Boston

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!

~Dawn




Gastroenteritis

On Monday, Jim and I both came down with a stomach bug that knocked us out. As ileostomy parents, nothing strikes more fear into our hearts than a stomach bug. Except obstructions, and prolapses….maybe it would be more accurate to say that stomach bugs are one of the things that strike fear into our hearts. Stomach bugs usually mean a trip to the ER for fluids and sometimes an admission. We tried really hard to keep our germs away from Zack……..

Sadly, Zack came down with what we believe is the same virus on Tuesday. He wasn’t throwing up, but his output was out of control. Zack’s output is always higher than normal for an ileostomy. He averages between 1,200 – 1,500 out daily. This is higher than it used to be for some reason. He goes above 2,000 once or twice a month. Last month he hit an all time high of 2,850. It could have been March….I don’t know, I am tired. At any rate, recently he hit 2,850 ml of output in one day and that was pretty darn high. We took him in too make sure his serum sodium was doing okay and we were pleasantly surprised to learn it was 138! Anything 135 or higher is considered normal.
Zack should be the poster child for CeraLyte. It has literally saved his life several times over. Anyway, he was still given fluids to help offset his losses and we were on our way.

On Tuesday, Zack hit 4,000 ml of output in a 24 hour period. That is the same as 2 – 2 liter soda bottles of soda. That’s a gallon of poop out of his skinny little body! We were in contact with his doctors and Dr. Sarah, our neighbor, came down to check him out. His heart rate was 108, his blood pressure was 105/56, his eyes were sunken, he was sitting quietly on the sofa and generally not acting Zack-like. My mama radar was not happy, so we all decided to take him in to be seen and to get IV fluids.

Zack’s serum sodium on Tuesday was 133. That is considered low, but with the volume of fluid flowing out of his tiny body it felt like a great number. His urine sodium level was undetectable, per usual. Another ratio was also off, possibly his anion gap ratio for those of you medical people. I am not really sure, but they could tell he was dehydrated and wanted that ratio to be back in line. Two boluses of fluid later, his serum sodium was back up to 136, the ratio was better, and we all felt comfortable bringing him home with the stipulation we see his doctor in the morning. I mean, by that point it was 4 AM and technically already morning, but whatever.

We got home and crawled into bed at 5 AM. By 6:30 AM, Zack’s output was already 1175. The rest of yesterday was spent on the sofa. ZP actually took two naps and took himself up to his room and went to sleep for another 1/2 hour. Um. Have you met Zack? He does not just sit. He never naps unless he is really sick. His eyes looked bad. Everything was making me worried. At 5 PM I decided to stick him in the shower to see if that might help him feel better. Here is how he looked then:

Jim and I were both concerned. However, by some miracle, he felt so much better after that. He had some spunk and even got in trouble for something silly. We were feeling much better and convinced his illness was on its way out.

You know there is more to this story, right? Overnight Zack had 550 out at 2 AM. At 6:35 he had another 725 and by 9:30 this morning he was at 1950 ml of output. Here is the crazy thing about Zack. He is not acting overly sick. He’s still on the sofa, but has some spunk back. His eyes look a bit better. Here’s a picture from this morning. But the output……

Jim and I were so conflicted about the best course of action. We texted his GI, but he’s got a life, a job, and other patients, so we are still waiting to hear from him. His pediatrician and I came up with a plan for now. Given Zack’s dislike of hospitals, we are going to hang out here for a bit, continue rescue dosing with CeraLyte, measuring everything that comes out of his body (including urine) and everything that goes into his mouth. If it becomes too much for us to handle, we can admit him and have the doctors take over the monitoring for us. My threshold for that is how quickly his output gets to 3,000 ml out and/or he becomes lethargic.

My mind is blown that our new self-imposed threshold for output is 3,000 ml. His doctors have taught us to trust our instincts and we have been doing pretty well with that. We now go by symptoms more than output, whereas before we went by mostly by output. One fear his doctors have expressed is that Zack may become so used to being low sodium that he will not realize when he is in trouble. Happily, everyone believes this current craziness is due to gastroenteritis and will eventually pass.

Another thing we discussed is that Zack’s daily outputs are continuing to become higher. His output has always stumped his doctors. What Jim and I do not know is if this means his disease is worsening or if it is an incidental finding. I mentioned in my last post that we are hoping to head to Boston to see a doctor who works with kids who have short bowel syndrome. That is still our plan. We are just waiting for the insurance referral to be authorized, the doctor in Boston to have an available appointment, and the stars to align. Until then, we are hoping this stomach bugs passes quickly and that we are able to manage his sodium levels well.

Hug your babies!

~ Dawn



Special Olympics 2019

Last week Zack participated in his last Special Olympics as an elementary school student. It was a fun day and this post is a photo journal of his day. Enjoy!

Aunt Becky and Ronnie drove up from Virginia to surprise Zack. They are the best!

Zack entering the stadium. He was just a little excited.

Zack’s classmates and teachers made signs for him that I have been instructed to keep “forever and ever.”

Zack’s coach, Mr. Tozer, making a Freedom Elementary School sign for the parade of athletes when we realized ours was missing. Mr. Tozer has coached Zack every year that he has competed. We will miss the way Mr. Tozer encourages Zack both at Special Olympics and at school.

This picture was taken from the Carroll County Times website of Zack and his entourage walking in the opening ceremony parade. We are grateful for all who came to support him this year.

Tennis ball throw. Mr. Tozer said that Zack threw 10 FEET!! farther than last year. Zack was more excited about his gold medal.

Gold Medal for Zack!

Zack’s 100 meter dash. He was pretty tired at the end, but kept on going!

100 meter dash medal ceremony.

Long jump. Zack was excited to see his Aunt Amy working as a score keeper at this event.

And now a few pictures of most of the people who came to cheer for Zack. I did not get one of his teacher, Mrs. Jensen, who has taught Zack for the past six years. I also missed getting a photo of Jim and my high school teachers for Germany, Mr. and Mrs. Manuel, as well as Mr. Reid and Ms. Elizabeth! Thank you all for cheering for Z!

Dan, Abby and Ms. Randi.

Ms. Karen! We had fun seeing you and cheering for Everett!

Ms. Cindy and Ms. Sarah came to cheer on their day off! We love them.

Ms. Carrie!

Some of Zack’s 5th grade classmates. They were the best encouragers!

Aunt Becky, Ronnie and Grandma or as they like to be called, “Team Zack.” It was extra special to have them there since Dad had to be away on business.

Zack had a wonderful day. If you have never seen or volunteered at a Special Olympics event, I encourage you to check it out. I promise you that it will be the best thing you have seen in a long time. I leave you with a picture of Zack from behind. I think this shirt says it all!

Hug your babies!

~ Dawn

Learning Disabilities

When Zack’s intesines allow us to, Jim and I try to tackle some of the other complicated issues he faces. Lately we have spent an incredible amount of time thinking about Zack and his learning challenges. I thought I would spend some time highlighting a few of his learning disabilities to help our friends and family understand some of the things he deals with every day in addition to his medical concerns.

Zachary showed signs of dyslexia from an early age. He was late learning to speak, had a lot of trouble learning to say his alphabet, could not recognize his letters or numbers, had trouble learning the sounds letters make (still struggles with this), and could not rhyme.

Zachary was not officially diagnosed with dyslexia until second grade when he had neuropsychological testing to help us figure out what learning disabilities we were dealing with as he was falling farther behind in school. This is very typical for kids with dyslexia as many children not diagnosed until third or fourth grade. This delay in diagnosis is significant because early intervention in a multisensory, sequential, structured literacy program used with fidelity is crucial.

Early Intervention Is Critical: When intervention is delayed, it takes four times as long to intervene in fourth grade as it does in the late kindergarten because of brain development and because of the increase in content for students to learn as they grow older.”
National Institute of Child Health and Human Development

In addition to dyslexia, Zachary was also diagnosed with dysgraphia. Dysgraphia is a specific learning disability that affects all aspects of writing. Zack has issues with his fine motor coordination leading to illegible handwriting, inconsistent spacing, poor spelling, and difficulties in the entire writing process including composing sentences and paragraphs.

A current sample of Zack’s handwriting.

And because Zack does everything differently, he was also diagnosed with dyscalculia. Dyscalculia is a specific learning disability in math. Kids with dyscalulia struggle to learn math facts, have trouble identifying symbols and using them correctly, and a whole host of other things. Zack struggles with it all.

You may be wondering how on earth Zack tackles learning . That is a good question. Learning is incredibly taxing for Zack. He spends the majority of his day with special education services. He has been working extremely hard at reading. No, seriously…..it is extremely hard and we are really proud of his determination. After years of intervention, he is beginning to understand how to read and can even read almost at grade level.

While he is beginning to understand how to read, spelling is another thing altogether. Not being able to is spell is frustrating for him. Zack is fortunate that his school has provided him with assistive technology in the form of a computer with word prediction software. The technology is great, but Zack still struggles sounding out words. This makes using word prediction software difficult. Zack is learning to sound out words when he can see them on paper, but trying to spell something from memory has been agonizingly slow and this ability is needed to use the word prediction software effectively.

Zack is becoming quite adept at using his computer in other ways. So much so that he was able to fix a problem with the church check-in program a few weeks ago when all of the adults were having issues. He is also learning basic coding through a program called bitsbox that he works on with his dad. He needs lots of help with the numbers, but he understands the concept. I am getting sidetracked. Welcome to my brain.

Dysgraphia makes the physical act of writing incredibly hard. His fine motor skills are weak. He can write his name and other words by hand, but it is laborious and frustrating for him. In addition to stuggling with the physical act of writing, dysgraphia causes issues with language processing, visual-spatial issues, spelling issues (double whammy), grammar issues and problems organizing written language.

To help Zack with his dysgraphia, Zack has several accommodations. He is able to use his computer, but trying to get his thoughts from his head onto paper, even with typing, is daunting. One of his accommodations is a human scribe. This has helped Zack begin to form sentences and basic paragraphs, but he needs heavy adult support.

Dyscalculia is the third of the “D” learning disabilities that Zack faces. Needless to say, math is not Zachary’s favorite subject. Adding and subtracting numbers without a calculator is nearly impossible for him. I am sure you can guess how multiplication and division facts are going. At one time, he knew some of his multiplication tables, but not anymore.

Jim and I have not given up on working with Zack on his math facts. Zack learns best with lots and lots and lots of repetition. Not just with math, but in every area of learning. And so we work on these skills at home in hopes of helping solidifying them – one day.

In addition to all of these, Zack also has ADHD. Zack’s ADHD is not typical in that it does not respond to medication as it should. This causes Zack to have to work incredibly hard to focus in school. It takes more energy for Zack to do his school work than a typically developing child. He needs frequent breaks because his brain cannot focus for long periods of time. This makes his other learning disabilities even more challenging to address.

Add to these things Zack’s medical issues and his anxiety and his learning profile is extremely complicated. And frustrating. And exhausting – not just for Zack, but for everyone who helps him and for us.

Many days when Zack gets home from school he melts down. He tends to save most of his frustration for home, although lately some of his frustration is being seen at school. This makes me sad because outside of school Zack has a very kind and happy heart. The other day Zack came home upset about getting in trouble at school. I found him petting Cap in the dog kennel singing one of his favorite songs, Even If, by Mercy Me. He felt better after that. (I am so grateful for our furry service dog in training.) I tried to be inconspicuous and let him have his time alone, but it was such a precious moment that I took this video:

Interestingly, this song was written by Bart Millard, the lead singer of Mercy Me, about his son’s life long battle with diabetes. He says of the song,

“My son, Sam, is 15 years old, and he’s been a diabetic since he was 2. When you’re a parent of a child with any kind of chronic illness, these things don’t go away. You have a lot of good days, but some days you feel like you’re losing bad. I was in the midst of one of those bad days when ‘Even If’ was written.”

Although Zack was sad about school on this day, I find his love of this song appropriate. Heaven knows I have been known to sing it when Zack is not feeling well. Some of the lyrics:

They say it only takes a little faith/ To move a mountain/ Well good thing/ A little faith is all I have, right now/ But God, when You choose/ To leave mountains unmovable/ Oh give me the strength to be able to sing/ It is well with my soul. … I know You’re able and I know You can/ Save through the fire with Your mighty hand/ But even if You don’t/ My hope is You alone

Jim and I have spent a lot of time telling Zack about his beautiful brain and how it learns differently. We let him know that we realize he has to work much harder than his classmates, but that he IS capable of learning and that we are here to help him. We also talk a lot about how working through hard things makes you stronger.

In January there was a Dyslexia Advocacy Day in Annapolis, Maryland. The purpose of this particular Dyslexia Advocacy Day was to talk to legislators here in Maryland about the Ready to Read Act of 2019 and to gain their support for this legislation by telling our personal stories with dyslexia. Zack heard me talking about going and asked if he could come and speak. Sadly, he came down with a fever and, well, that was the end of that dream…for now.

Even though we were unable to attend, Zack had me write his story in his words to share with our State Delegates. I do not know if we will ever hear back from any of the delegates, but I love that he is learning to find his voice and speak about the things that are important to him. And that, I believe, will be a skill that will help him accomplish great things.

Hug your babies!

~Dawn



Zack’s 12!

Zack turned twelve last month. Twelve. That is crazy to me. Since our other boys are 25 and 21, I suppose I should have seen this coming.

This past week Zack had his yearly checkup. It was his first real checkup with his new pediatrician, so we had a lot to discuss. Zack has a lot of unknowns in his life, but one thing that always brings me peace is the amazing medical team that cares for him.

For some reason, Zack’s body does not feel the need to gain weight right now. We noticed at an appointment in January that he had lost a bit of weight despite an increase in calories from his overnight feeds. At the appointment this week his weight was down a little bit more. This downward trend means that he is barely hanging on to the weight chart. If he loses any more, he will officially be off the chart. Our goal for Zack is to get to the tenth percentile.

Zack’s doctor and his GI will be talking to discuss what our next steps are for gaining weight. I am not sure if we will be changing formulas, mixing his current formula differently to increase the concentration and calories or keeping things the same and getting more calories through daytime formula feeds in addition to his overnight feeds.

High ileostomy outputs continue to plague Zack’s body. During his visit he was on day two of outputs over 2,000 (ish)ml/day, but was doing fine. He looked good and had energy. The day after his visit he woke with belly pain, a headache and lethargy. We rescue dosed him and kept him home from school. I needed to head to the grocery store and took him with me. He was so low energy that he needed to rest in the shopping cart. I’m sure people thought he was sick with something contagious. I would never take him out if that were the case. We needed food. Zack was not contagious. Life goes on, even when you have a chronic illness. After a day of rescue dosing the heck out of him, I am happy to say that he is feeling much better.

This is low sodium Zack. Those are his sodium chloride pills. When he doesn’t get off the sofa and needs to lick sodium, chances are it is going to be a long day at the Penrod house.

Back to Z’s doctor visit…..Happily, he has been consistently tracking in the fifth percentile for height. His doctor said that continued growth in height is a good indication of overall health, so that was reassuring.

We discussed the results of Zack’s new genetic testing and the implications of some of them. Specifically, we revisited the bladder issues kids with ACTG2 gene issues can face. We had discussed having a bladder scan with Z’s GI in December, but just never got around to scheduling one. His PCM agreed that it would be good to get a baseline scan done. Evidently, bladder scans are not the most effective way to see what we need, but are a good starting point. Zack will be having a bladder/renal scan once I finally call to schedule one. I am assuming his appointment will be in the next week or two.

Given the need to discuss all areas of Zack, his appointment was fairly long. We were able to talk about his difficulties in school and go over reports and grades. We were able to discuss his anxiety and what we are working on in that area. As we were talking, it was a reminder to me that any one of those areas by themselves can be overwhelming. Zack has multiple areas of concern and sometimes more than one is in need of attention. It was a good reminder that there is a reason for the difficulty of this season in Zack’s life and that Jim and I are doing a good job of holding it together — most of the time.

Zack was a good patient (yay) and polite and answered the doctor’s questions (mostly) and that was amazing! He was still nervous all the way to his appointment and asked, “This isn’t inpatient, right?” and “This is all awake, right?” But he did not fight getting his vitals taken or his examination and that is a HUGE step for him.

When his appointment was over, Zack had some friends waiting in the lobby. Here are some photos to show how happy he was to see them:

Zack and his buddy Elsa.
Zack and Aslan. He loves him.

Elsa’s human, Pat, and Aslan’s human, Jen, are the some of the most wonderful people ever. They know how much Zack loves these dogs and how much Zack dislikes the hospital. Even though it was not one of their regular days to visit Walter Reed, they made a special trip just for him. How do you thank someone for loving your child that much? I cannot ever begin to tell you just how much it means to us. There are definitely blessings in each day, even the hard ones.

Hug your babies.

~ Dawn