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

Growing Pains

Hello Interweb! It has been a while…two months to be exact. I wanted to write about how great Christmas was around here, but life events got in the way of that. Two days after Christmas we hopped on a plane to go to Drew’s wedding. There was no time for an update when a wedding was taking place!

I am inserting a short wedding paragraph here that has nothing to do with the point of this post, it just makes me happy. Drew and Lindsey were married just outside of Austin the first week of January. It was beautiful and warm and outside – in January! I mentioned that, right? Lindsey was a gorgeous bride and Drew was a handsome groom. Lindsey’s parents thought of every detail and the day was just perfect. When their official wedding photos are back, I will post more about this joyous occasion. For now, I’ll just put this picture here because it makes me smile.

Drew and Lindsey Penrod

Back to what actually motivated me to write today. (Although writing about the wedding is way more exciting.) Today I wanted to write about some things I am learning about myself in this role of being a parent of a special needs child.

By nature, I am a peacemaker. I do not like confrontation. I want everyone to get along and like one another. I am the one introducing herself to those I do not know. I am also the person introducing new people to those who they have not met to make sure everyone feels comfortable in a new situation. This may be a result of having been a military kid and moving every two years until college. It could also be the result of being a military spouse and working hard to meet people and make connections before it was time to move again. I suspect it is likely a combination of both those things.

I tell you this because as the parent of a complex child (I think I will go with that description of Zack for now), I have had to stretch myself and learn to be OK with people not always liking me. In order to effectively advocate for my child, I have had to learn that I cannot always please everyone. There are times when I have to confront others for Zack’s sake. It is uncomfortable. It is out of character for me and it requires a level of strength and courage I am still learning to find.

I think I will always care about what people think of me. I try my best to make sure that I am honest with my speech, kind with my words and thoughtful of others. My main job at the moment, however, is to be a voice for Zack so he can get the support he needs to become the person he is called to be.

Almost every night before bed we ask him, “What is the most important thing about you, Zack?” He replies, “That I am a child of God and that I am your son.” Although the question is asked of Zack, it is a daily reminder for me as well. I have been called to be Zack’s Mom. To love and protect him. To operate on less sleep than a new mom — for years on end. To learn as much as I can about the gastrointestinal system. To continue to fight for him over and over again when it would be so much easier to quit. It can be an overwhelming, stressful, lonely, and exhausting job. It stretches me and forces me to grow in ways that make me uncomfortable.

And yet….here I am. Each new battle helps me to grow stronger (and not just because I have to work off my stress by exercising), to learn something new about myself (some not so pleasant things as well), and to keep fighting for Zack. So, bring on the growing pains! But please… not all at once!

Zack after killing it at the wedding. His shirt was tucked in for the ceremony and he did NOT wear that hat until the reception.

Hug your babies!

~ Dawn

Whole Exome Sequencing

Do you remember way back in September when I wrote about Zack having Whole Exome Sequencing done at Walter Reed?  No?  No worries, I can barely remember September either…

Let me refresh your memory.  We were told that though this test looks at over 20,000 genes, positive results are only found 30-40% of the time.  This is true even when the person being tested has significant medical signs and symptoms.  Even so, together with Zack’s doctors, we decided to see if this test could find a reason for Zachary’s issues.

When we heard the results were back in only three months, we tried really hard not to get our hopes up.  Jim was not able to go to the appointment with me this time, but we were able to have him listen in on my cell phone as Zack’s geneticist gave us the results.

Are you sitting down? I really think you need to be for this.  Zack’s testing results came back positive for an anomaly.  Not only that, his doctor told us that he felt the anomaly was the reason for Zack’s intestinal problems!  He seemed pretty excited about that and rightfully so!  Many doctors in several hospitals have been working for many years to try to figure out what on earth is wrong with our boy.

And now for the moment you have all been waiting for…oh wait….I guess only Jim and I have been waiting for this moment.  But I’ll share it with you as well.  Here are the results of Zack’s test:

1.  Causative Variants in Disease Genes Associated with Reported Phenotype:

  • Gene – ACTG2
  • Disease – ACTG2 – Related Disorder
  • De Novo – new to Zack (Jim and I do not have this mutation.)

2.  Zack’s variant in the ACTG2 gene has not been reported previously as a pathogenic variant, nor as a benign variant, to our knowledge. (Zack is the first documented person with this variant….of course…) This variant is not observed in large population cohorts. We interpret (Z’s variant) as a likely pathogenic variant, which is likely consistent with the megacolon, small bowel obstruction, dysmotility, and constipation reported in this individual.

You may be wondering what on earth that really means and so do we.  It has only been about 36 hours since we found out.  We left the doctors office armed with many articles to read, websites to look up, and information on some organizations that deal with gastrointestinal diseases that we had not heard of before.

This is what I can tell you.  This is from the NIH website:

The ACTG2 gene provides instructions for making a protein called gamma (γ)-2 actin, which is part of the actin protein family. Actin proteins are organized into filaments, which are important for the tensing of muscle fibers (muscle contraction) and cell movement. These filaments also help maintain the cytoskeleton, which is the structural framework that determines cell shape and organizes cell contents.

The γ-2 actin protein is found in smooth muscle cells of the urinary and intestinal tracts. Smooth muscles line the internal organs; they contract and relax without being consciously controlled. The γ-2 actin protein is necessary for contraction of the smooth muscles in the bladder and intestines. These contractions empty urine from the bladder and move food through the intestines as part of the digestive process.

You can read more about ACTG2 on the website and look up health conditions commonly found with genetic changes on the gene.  One of the interesting things we read in one article is that periods of severity can wax and wane.  Zack often has periods of intestinal obstructions in his small bowel.  Thankfully, they have been resolving on their own lately.  However, he definitely has periods of more intense pain, prolapsing and obstructing.  We also think this may explain why he had normal motility testing and then a few months later his testing showed complete dysmotility.  A year after that his testing showed zero motility once again.  Soon after that he had his final motility testing which showed that he had a few centimeters in his colon with motility.  Craziness.  It may also explain Zack’s urine retention issues after surgery.  These always result in bladder scans and the threat of catheterization.  We need to discuss these things further with his GI doctor.

Zack’s geneticist said that people usually have one of two reactions to news of genetic anomalies.  They either express great relief or sadness.  I think I experienced some of both, but mostly great relief.  Knowing that Zack’s condition is not “curable” is actually helpful, in my opinion, because it rules out a lot of testing and guessing about next steps.  The sad part about this is that when we told Zack that the doctors found a reason for his intestines misbehaving, he was excited.  He said, “Yay!  Now I can be cured!!”  That was a bit of a tough conversation, but only for like two minutes.

Jim and I will have an appointment with Zack’s GI to discuss plans going forward in mid December.  We have many questions and I am sure his doctor does as well as this is new information for all of us.  Until then we will be researching and reading all we can about ACTG2.

I leave you with a photo of our crazy family over Thanksgiving weekend trying to get that oh.so.elusive Christmas card photo.  Jim’s face reminds me of Steve Martin in Father of the Bride and totally cracks me up.  He says he was just checking to make sure Zack was OK.  The boys and I were laughing like crazy because Zack was being a goofball.  Good times and great memories of our last time together at home before Drew gets married!

Hug your babies!

~ Dawn

World Ostomy Day

Lately, it seems like every day is dedicated to something.   Today, for example, I have learned that it is National Orange Wine Day, National Mad Hatter Day and National Noodle Day, to name a few.  While I think National Days are fun (especially National Chocolate Day….need to look that one up), I want to tell you about a day that we are going to celebrate the heck out of around here.  To add to the excitement, it is not a National Day, it is a WORLD Day.  Today is World Ostomy Day 2018.

I must start by acknowledging that every day is Ostomy Day around here.  Every Day.  Even so, we are happy today is officially designated World Ostomy Day.  It gives us a chance to help people learn more about ostomies and how wonderful they can be.

Ostomy surgery is a life saving surgery.  It enables stool and urine to be eliminated from the body in a different way because of a malfunction or damage to the digestive or urinary system. There are so many things I could share about ostomies today and I just know that they would captivate you.  However, in an effort to NOT put you to sleep, here is a graphic made by the United Ostomy Associations of America with some fun facts:

For those of you who know Zack and his ongoing intestinal struggles, you may be wondering how on earth we celebrate an ostomy.  Well, sit right back and let me tell you.  See this little guy?  This is Zack without an ostomy.  Look at his belly.  Look at his face.  Does that look like fun?

After being reconnected in Cincinnati and realizing we had major issues on our hands.

This is what “fun” looked like without an ostomy.

This is how he “played” – on the sofa, in small bursts.

Zack without an ostomy – in pain each and every day.

Zack’s colon without an ileostomy.  If you are not an expert in reading x-rays, that dark s-like area taking up his abdomen?  That is not normal.  That ring in his lower pelvis?  Those are staples from his anastomosis.

Sure, we still have lots of issues to deal with even with an ileostomy.  Zack still has days where he does not feel well.  He has different issues to deal with now and some are really tough, but he also good days.  He can go to school, he can play outside, he can run, he learn karate and play tennis and swim and enjoy being a kid.

I asked Zack to pose for some pictures to show how strong he feels with his ostomy.  These are the two he picked.

I wanted to take a picture of him in his black Stealth Belt.  Zack set me straight.  He said, “Mom, this is what my bag looks like.  You can’t see it in the Stealth Belt.”  He is right.  He does not mind showing you what his bag looks like.  It is a part of his body now.  And, he will tell you, it helps him live.  Happy World Ostomy Day!

Hug your babies!

~Dawn

 

 

 

 

 

Best Day Foundation And An Update

August was a month packed with lots of visits to Walter Reed and I wanted to wait until Zack had all of his appointments until I posted an update.  Originally we were just going to have a visit with Zack’s surgeon and a visit with genetics.  At our surgery visit, a referral to neurology was added. To add more excitement to our month, Zachary needed an eye exam.  In addition, Zack’s PCM retired from Ft. Meade and that seemed like the perfect time to transfer Zack’s regular pediatric care to Walter Reed.  This meant adding another appointment to establish care with his new doctor. Actually, we have known this pediatrician for years from Zack’s inpatient days, but she was not officially Zack’s doctor.  The way it all came about is a fun story, but I’ll save that for another time.  We are thrilled to have her supervising Zack’s pediatric care.

As August ended, we knew that we needed to see Zack’s GI and it just made sense to wait to update the blog until all of those things were complete.  Although by doing so, it makes for a long update…

Let me see if I can do a quick(ish) summary….yeah, right…you’ve met me.  Quick is not my thing, but I will try because it is 11:30 p.m.

Not much new happened with Zack’s surgeon other than learning that he was deploying, so we hope that nothing surgical happens in the next few months.  We did talk about some surgical options, but I refuse to think about those until Zack’s body tells us otherwise.

During Zack’s genetics visit, we decided to have whole exome sequencing done.  It is pretty interesting stuff.  If you want to learn more about it you can click on the link above.  The test takes several months to come back and we have been told that it only finds issue about 30 – 40% of the time, but it is one of the best tests available at the moment and much more precise than the last test Zack had 6 years ago.  Jim and I also had our blood drawn so that if abnormalities are found in Zack, they can compare our DNA with his.  We signed lots of papers and had lots of counseling about what this test can and cannot do and the different things we might discover.  Now we just wait.

Neurology really didn’t tell us much, but it did rule out lots of diseases.  The most interesting thing that I learned was that the brain and the skin develop at the same time during gestation.  Zachary has several skin abnormalities which the doctor seemed interested in, but the big diseases that occur together with the brain were ruled out.  We left there learning that the neurologist believes that Zack’s issues are chromosomal.  So, basically nothing new.

Zack’s pediatrician appointment was great because we have never had a pediatrician that truly understood everything going on with Zack.  This doctor really does.  She actually read his entire medical record back to our time in Germany.  That was certainly a time-consuming feat.  We were so thankful that his doctor took the time to do that so that she could really understand Zack and all of his medical concerns.

Zack’s ophthalmology visit was not as wonderful.  Last year Zack was so nervous about getting his eyes dilated that the doctor said he could wait until this year to do it, but that it had to be done.  Zack was even more nervous this year, but we worked on helping him through it and he was ready.  Before his appointment, the Red Cross Dogs made a special trip to see him to help him relax.  When we got to the appointment, Zack was clearly agitated…but ready to get his eyes dilated.  The doctor saw him and said, “Well Zack, I’m OK with waiting until next year to dilate your eyes, but I will leave that decision up to your mom. Just know next year you REALLY have to get it done.”

Um……who does that?  What doctor tells a child something like that and then makes the mom the bad guy.  I was not impressed.  Zack was ready to do it because he knew he had no choice.  Now he thinks he can get out of it again.  I rarely complain about doctors on this blog as it really serves no purpose, but come on!  That was craziness.

Zack and Elsa and Bailey before his eye exam.

Z’s GI appointment today was interesting.  For the past month we have been noticing a downward trend in his weight.  Today we talked about getting more calories in him through his feeds.  He currently receives 1200 calories overnight and we are bumping that up to 1400.  His g-tube, which was too small before his revision in March, is now too big because of his weight loss. That was resized today.  We also discussed seeing if a trial of steroids might help Zack.  He is still dumping (too much output) for some unknown reason, still has belly pain, still prolapses and still has days where his stoma is swollen.  The thinking is that perhaps Zack’s issues are some weird autoimmune response and that a course of steroids (several months) might help calm that down. Because this has been an issue for years, nobody knows just how long it might take.  From what I understand, we will be watching his body for clues to measure its effectiveness.  Steroids come with their own set of side effects, but one of the good ones, for Z, is weight gain.

If the steroids improve his symptoms, we will take him off of them and wait and see what happens.  If his symptoms return, we will do scopes and full thickness biopsies to get a baseline.  Then start the steroids back up for a time and repeat the biopsies to look for improvement.  If they don’t work or if Zack has adverse side effects, we will stop the treatment.

Whew.  Two more updates.  First, Cap has started Medical Assistance Dog training to try to help Zack with his medical trauma anxiety or PTSD.  We are all pretty excited about that.  It is a long process, but Cap seems to really enjoy it and Zack loves it!

Second, Zachary had the opportunity to head to Ocean City, MD last weekend and go SURFING!  The Best Day Foundation hosts events in different cities for children and young adults with disabilities to allow them to try adventure activities that they would normally not be able to.

Zack was paired with some buddies, Angie, Terri and Mason, who helped take him around to different stations.  They were wonderful and so encouraging and loved on Zack and helped him feel incredibly special.  A big team of volunteers worked to help the kids surf and boogie board, play in the sand, meet some super heroes, have lunch and get medals and goodie bags.  The weekend was super rainy but, for that hour and a half, not one drop of rain fell, the sun came out and once each child had several opportunities to get into the water the skies opened back up.  Perfect timing.

I leave you today with our favorite pictures.  Just a little warning….I am posting a ridiculous amount of photos.  My blog also serves as my journal, so I wanted to make sure I could find these pictures easily.   Some pictures are our own and some are from the Best Day Foundation Chesapeake Bay Facebook page taken by Desiree Ortman Photography and Nick Denny Photography.  I’m sure you will be able to tell which are ours.  If you are on FB, head on over to the Best Day page and check out the awesome photos of the event there as well.

Zack all suited up and waiting his turn.

A very happy surfer!

Zack after his first ride being asked if he wanted to go again…heck yeah!

The amazing volunteers who helped Zack catch some waves.

Zack’s buddy, Angie.  She made his day extra special.

Another of Zack’s buddies, Ms. Terri.

First boogie boarding attempt.

Check out the size of those waves.

Zack’s team.

Just a boy enjoying the ocean.

Zack telling us that he really NEEDS a surf board.

Zack’s cheering section.  Dad, Mom, Abby and Dan.

Celebrating with pizza and some of Zack’s favorite people.

And finally, one of Zack’s surfing runs.  The video is a bit blurry, but totally amazing. Thank you to the Best Day Foundation for an amazing experience!

After writing all of this, I came to the conclusion that jamming all of this info into one post was a bit nutso.  If anyone besides my mom actually reads to the end, I will be super impressed.

Hug your babies!

~Dawn

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

 

Fluoroscopy Results

I started typing this post on June 28 (it has taken me forever)…which was the 5th anniversary of the day Zack came home from the hospital after his first two abdominal surgeries.  It is funny to me the things my brain decides are important and need to be stored forever, like this date, and the things it decides are not, like how convert fahrenheit to celsius.  This is not totally random as I just returned from visiting my sister in Egypt where the temperature was 45 ° celsius and all my brain could figure out was that it was over 100 ° fahrenheit.  I’m so totally off topic….

On the anniversary of this occasion, I thought it might be fun to see what Zack looked like on this date five years ago.  Here he is on the drive home from the hospital.

Such a crazy scary time in our lives and the beginning of our journey into medical oddities.  It was fitting then that, on this day five years later, we found ourselves meeting with Zack’s surgeon to talk about his crazy intestines.

If you are following Zack’s story, you will remember that he had a fluoroscopy test done the last day of May.  We got the results from Zack’s GI doctor mid June, but I was away.  Our GI’s advice was to have Zack evaluated by the surgeon for consideration of correcting the narrowing seen on the imaging and to get him back on their radar…and so we did.

Zack’s surgeon and I talked for quite a long time about quite a few things.  He confirmed that there are indeed two areas of narrowing in Zack’s small bowel.  One is very near his stomach and I totally forgot to ask where the other one is.  Strange for me, I know.  For some reason, I was completely freaked out that there was a narrowing by his stomach.  I could not figure out how they would fix that and allow it to heal and still be able to feed Zack.  All that worrying for nothing (I need to quit with the worrying) as the surgeon said it is in a spot that is able to be fixed.  The issue is figuring out what our threshold for fixing it is.  I told him that Jim and I do not want to fix it until we have to and so that is our plan, for now.

One of the things Z’s surgeon said is that the narrowing could be from adhesions.  In all of Zack’s previous surgeries, he has had little to no adhesions.  His surgeons have all been surprised by this given the child has had 10 abdominal surgeries at this point.  However, it would not be surprising if this is the case now.  His surgeon said that the next time Zack is opened up, it is not going to be a little surgery.  I did not ask many questions about that because I did not want to think about it yet.  When your child has wonky things going on, sometimes it is best to try to just deal with today.  If you know me, please stop laughing now.  I realize I am almost certainly the worst at this, but sometimes I CAN actually do it.  Usually when thinking about it overwhelms me.

We also discussed how long it took for the barium to come out of Zack’s ostomy.  Although the radiologist suggested it was slow motility, it was actually showing Zack intermittently obstructing.  Z’s GI here and his surgeon in Boston have said this all along, but now we have proof.  Actually, Jim and I see proof quite often when Z goes 8-9 hours with no output, has belly pain and a distended tummy.  Proof means nothing though when nobody can say why it is happening or what the heck to do about it.  Since it has been resolving on its own, we do not need to do anything…but it is still not normal.

This not normal stuff is what has been bothering me for a long time.  I spoke to his surgeon about when we began this journey and how it was to give his colon a rest with the hope that it would begin to function properly again.  Instead, it stopped functioning altogether.  That was “fine” because a person can live without their colon.  At that time we also knew that his small bowel was functioning properly.

Since that time his small bowel has been having issues. First with the obstructions which were mechanical in nature, so fixable and not expected to be related to bowel function.  Then the prolapsing began and it was intense and severe.  Up to nine inches of intestines out daily from November 2013 until June 2014 with a few weeks breaks with revisions.  Pretty significant prolapsing again after each subsequent surgery until July 2016.  Since that time Zack has “only” had on average 3-4 prolapses a month, but prolapsing intestines are not normal.  Lately (the last few years), he has been having these intermittent obstructions and belly pain.

I went over all of this with his surgeon and told him my fear that his once seemingly normal small bowel is now having issues.  He was sympathetic and agreed that Zack’s small bowel is indeed having issues.  I told him I was worried that what happened with his colon might happen with his small bowel.  He said, “Don’t go home and google this, but that can happen.”  He also said that it would be extremely rare.  I have tried not to think too much about that, but I’m thinking about it right now! We talked about this for a bit and about the fact that there are lots of “heavy hitting things” we have not tried yet up to and including small bowel transplants.  He was in no way suggesting that Zack needs a small bowel transplant, but just the fact that we were talking about small bowel transplants as an extreme option for my child was surreal.

When Zack talked with his surgeon, his surgeon asked me if I felt his speech was less clear.  I told him that I had noticed that since I returned from my trip, but thought it might just have been because I had been away from Zack for a few days.  That got him asking about Zack’s speech, his school year, and a couple of other things.  Then we talked about what genetic testing we have had done so far.  Zack’s surgeon thinks that whatever is going on with Zack is going on cross systems in his body.  His Kennedy Krieger doctor has been saying this all along.  He said that genetic testing has improved a lot in the 6 years since Z’s last test and suggested we do a much more involved test.  We have a referral in for the geneticist to begin that process in the near future.

We also discussed having Zack more closely followed for now.  Those narrowings in his intestines are still problematic.  Both his GI and surgeon want us to bring him in for an urgent fluoroscopy if his pain returns and we have instructions to have a low threshold for waiting to come in for that.  His surgeon will be out of the country next month.  However, he has left instructions that he is to be contacted directly if Zack comes in to the ED.  I am hopeful that will not happen.  We will see him again once he returns at the beginning of August.

Jim and Zack both reported that Z’s belly did pretty well while I was gone.  It hurt quite a bit today. I told Zack that maybe I should go back on vacation so that his belly would stop hurting.  But seriously, we have noticed that Zack has not prolapsed since his fluoroscopy and that his belly pain has been less as well.  I told both doctors that Jim and I have noticed a difference in pain and prolapsing after these tests.  Although it does not make sense, it could be that the barium is opening up a partial obstruction. It is out of the box thinking and clearly I am not a doctor, but somehow he feels better for a short time after having fluoroscopy studies.

Whew.  That is a lot of information.  Thank you for sticking with me through this long and involved post.  Sometimes writing this blog is a great way for me to brain dump all of the things I just learned.  It helps me process them, although whether I process them accurately remains to be seen.  It is immensely helpful to me, however, when I look back on my posts.  I am able to remember details that would have been forever lost in my brain and replaced by random dates and phone numbers and songs from the 80’s.  It also helps keep my family happy and updated and off my back….just kidding…mostly.

I leave you with a few pictures of Zack’s summer so far.  We are looking forward to a relaxing summer of playing outside, days at the pool and quiet intestines!

Zack and his pup.

Brown belt promotion.

Hug your babies!

~Dawn

 

 

Capernaum Prom

He may only be eleven years old, but tonight Zack attended his first ever prom!  Crazy, I know…and super fun.

For the past year Zack has been attending a Young Life Club at our church called Capernaum.  Taken directly from the younglife.org web page: Young Life Capernaum gives young people with intellectual and developmental disabilities the chance to experience fun and adventure, to develop fulfilling friendships and to challenge their limits while building self-esteem through club, camp and other exciting activities. Without a doubt, club night is Zack’s favorite night of the month.

When Zack began attending Capernaum, we had no idea about prom.  We began hearing about it as the year progressed and thought it would be a fun thing to do.  We also imagined that it would be similar to most of the other meetings of the year with a little dancing mixed in…..WRONG!  This was a full-out PROM people.  The rest of this post will just be videos and pictures of Zack’s amazing night with a few comments thrown in to make things more interesting!

While I CAN tie a decent tie, my skills are a bit rusty now that our older boys are grown.  Jim was away on business this week, so our neighbor kindly offered to help make sure Zack had a good-looking knot.  Thanks, Bill!Zachary’s Grand Entrance!  This was how all of the participants were announced.  Zack definitely felt like a VIP tonight.  Zack was escorted by his buddy, Dan.  Or as Zack likes to call him, “My life-long best friend.”

The amazing Elizabeth, Capernaum Leader and dear friend.  I am still in awe thinking about all of the work that went into this night!

The kids were treated to limo rides.  I am pretty sure my son thought he was in heaven.  And really, tonight was a like a little taste of heaven on earth.  It has been a really hard year for Zack, in many different ways, so being loved on and treated with such kindness was just what this kiddo needed.  At bedtime he asked if he will still be a VIP tomorrow.  I told him he will always be a VIP to me.  He asked if that meant I would buy him a limo…

Zack, Dan and their buddy Jake.

Zack with sweet Abby.  We love her.

The face of a child in awe and wonder.  I mean, come on!  That is pure joy right there.  I hope he has this look on his face often over the course of his life.  Like his flower?  They thought of everything….goodness knows his mama did not think of that!

Photo booth with his BFF.

What dance would be complete without a conga line?

Zack busting out his moves with his buddies who graduated from high school just last night.  Can I just take a moment to tell you how amazing these guys (and girls) are?  Their hearts for people with disabilities is something beautiful to see.  The way they love on these kids, enjoy hanging out with them and encouraging them is truly remarkable.  I cannot even begin to describe how it blesses us.

Zack only allowed me to dance with him twice tonight.  And so it begins….those teenage years are almost here.  I will take what I can get, even if dancing means I hold his 60 pound body and jump around while he sings.  I am pretty sure that counts as an extra workout, right?  Happily, he did agree to take a picture with me and ended the night giving me great big hugs.  I guess I will keep him around a little bit longer.

And there you have it, Zachary’s first ever prom.  It was definitely a night to remember.  Thank you to all those who worked so hard to make it such a special night!

Hug your babies!

~ Dawn

Fluoroscopy and Zack’s Intestines

Over the past two weeks, Zack’s intestines have been a real pain in the gut.  He had been having some intense belly pain and little output.  He did not have distention or vomiting, so I was not overly concerned when it started.  But then he started missing school.  Three different days over the past two weeks.  The pain kept him home and on the sofa or on the floor – see the picture below:

As you may know, Zack does not nap and he does not sit still…unless he feels pretty lousy.  The other concerning thing about these pain episodes was the sheer volume of output that occurred once it resolved.  Seriously.  When Zack loses fluid that quickly his sodium levels tank fast.  He looked terrible.  Luckily, his doctors have armed us with rescue doses of his electrolyte replacement and we know to pump it into that little body of his.  This seems to work, but it still takes a lot out of our guy.

The last time this happened, Zachary’s school nurse said he really didn’t have any bowel sounds or very, very faint ones.  He was very uncomfortable, so we reached out to his GI doctor about our concerns.  He decided that Zack should have a baseline fluoroscopy to see what was happening.  Our plan is to have an urgent fluoroscopy the next time he has that intense pain.  The problem is that we live a little more than an hour away from Walter Reed in normal traffic.  Getting him there while the obstruction is in full swing could be hard since, so far, they seem to resolve on their own.  If we can see what is happening in the middle of the intense pain and compare it to his baseline fluoroscopy, maybe we will glean some new information.

Today Zack had his baseline fluoroscopy.  He has had many of these done in the past, and we have never before seen anything that helps explain his pain.  When we went in this morning, I prepared Zack that this would likely be our outcome once again.  He asked the radiologist to, “Please find the reason my belly hurts.”  A tall order for sure.

Surprisingly, we actually found some issues today.  There was some bowel dilation shortly after the stomach with a transition zone or narrowing of the intestines right after that.  For awhile we thought there might be two spots, but I think it was decided that we were looking at the same spot from different angles.  I’m not really clear on that.  After that spot, the barium made it through the intestines appropriately until the end by Zack’s stoma where it pooled for an extraordinarily long time.  It took five hours and thirty-five minutes for the barium to come out of Zachary’s stoma.  The radiologist said she had not seen it take that long to come out of a patient since she was a fellow.  I did not ask her how long ago that was…it seemed inappropriate.

The radiologist felt that the pooling of the barium in the spot near the opening of the stoma was most likely due to dysmotility.  I do not think that is the case – and, as you know, I spent many years in medical school….  It feels wrong to disagree with a doctor, but let me tell you why I do.  First, I cannot let myself even entertain the thought that Zack’s small bowel has poor motility now.  I just can’t.  Second, Zachary’s ileostomy has so much out everyday and usually so quickly that this does not even make sense.  If his motility was slow, I do not think we would have this volume everyday and struggle so much with low sodium issues.

My theory is the same as his surgeon from Boston and possibly his GI here.  I should ask him. I know we all agree he is intermittently obstructing.  I think his stoma is prolapsing internally.  Meaning, I think it is getting stuck on itself near the opening and preventing output from coming out from time to time.  His stoma was pretty swollen once the barium went in and was trying to prolapse, but did not.  It goes along with my theory and I want it to be right.  So there.

So what does all of this mean?  I actually have no idea.  I know that according to our visit today, it appears we have two separate issues going on.  I know that nobody wants to operate on Zack unless absolutely necessary, but the dilation and narrowing of the intestine seem like a potential surgical issue.  When Zack and I left today, the GI team was meeting in radiology for their weekly review of all the pediatric procedures completed there this week.  I know Zack’s GI will be reviewing the results.  I have no idea what to think or what to expect.  My guess is that as long as these things continue to resolve on their own, we will wait and see.  But really, I know nothing. Welcome to my world.

As always, though, my hope is that Zack’s doctors will help turn this face:

Back into this face:

Just to clarify (and to keep from freaking anyone out), at the moment he is fine.  I will update with more information when we figure it out.

Hug your babies!

~ Dawn

 

 

Special Olympics 2018

Before I tell you all about Zack’s amazing day, a health update.  This last month has been rough on Zack.  His belly has hurt since his surgery and for no discernible reason.  He has been consuming a LOT of Tylenol and Motrin.  A lot.  The good news is that those medicines help with his pain and he has not needed to be seen for anything stronger.

He will tell you that his g-tube still hurts.  I am sure that is true, but nothing is visibly wrong with it.  Sadly, we think his keloid is growing back and that stinks.  In addition, he prolapsed 4 times last week alone.  Prolapsing hurts and gets in the way of things Zack likes to do.  He does not like that.  Last Friday we had an impromptu neighborhood picnic and he did not want to miss it.  He was in pain in the shower and mad about missing fun, so he took the handheld shower head and pushed his intestines back into his body.  Because why not?  That did not feel super great, so I don’t think he will do that again.  I hope….

All of the belly pain has meant he has not been eating much.  Because Zack’s doctor increased his feeds, he has not been losing weight which is good.  But he is not gaining weight at the moment like we had hoped.  Thank goodness for feeding tubes.

Enough of the medical stuff….

Zack woke up this morning at 6:20 a.m. ready to get on the school bus, which arrives at 8:27.  Why?  Today was the Special Olympics and it is one of Zack’s favorite days of the year.

Getting greeted by cheerleaders.

This year, several of Zack’s classmates and their homeroom teacher came along to be his cheering section.  As usual, they made some awesome signs, offered lots of encouragement, and were super kind to brave the extremely frosty morning. It was 38 balmy degrees and windy as all get out at 9:30 a.m. when they all arrived.

Mrs. Solomon – Zack’s Homeroom Teacher

In addition to Zack’s classmates, Zack had several other people in his cheering section.  Mr. Tozer, his coach, has been helping Zack for the past three years.  He really knows how to motivate Zack to do his best.

Mr. Tozer coaching Zack before his tennis ball throw.

Zack’s grandma and cousin, Kelsey, were there today as well.  Kelsey has such a kind and compassionate heart for Zack.  She also helped me corral him and that is a true gift given the amount of energy Z had today.  Grandma is one of Zack’s biggest supporters and comes to the Special Olympics every year.

 

 

 

 

 

 

 

Zack also had his buddy, Dan, there to cheer him on.  I’ve written about Dan before.  This young man truly has a gift with children and especially kids with special needs.  Zack lights up when Dan is around and is always asking when he can see him next. Zack also says that Dan is his best friend because he listens to him and is kind and likes to play Lego. His parents have raised an exceptional young man!
Some other fun pictures:

Zack and his buddy, Jake, getting their tennis throw medals.

Long jump

Zack’s long jump video

Medals

100 Meter Dash

Home with his adoring fans.

And that, my friends, is all the typing these fingers can handle for one day.  Have a wonderful weekend!

Hug your babies!

~ Dawn