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

 

 

Doctor Visits, Puppy Updates, and Zack!

Hello Blogosphere!

It has been so long since I have updated that I feared I might have forgotten my password.  In reality, I have been struggling with if I want to continue to update this site while Zack is doing well.  It takes time and brain power and there is just so much one can talk about when life is humming along.  But then I remembered that the reason I started writing in the first place was not just because of Zack’s colon.  Rather, I started writing to share our experiences of working through life with a special needs child and everything that entails.  My hope was that someone might stumble across this blog and find something helpful in their own journey.  Zack’s colon just turned out to be an attention hog and shut out the other areas that make our life unique. Even though things are a bit calmer on the colon front at the moment, I still have things I want to share about navigating through this crazy special needs world.  So, for now, I have decided to type on…..

When I last posted at the end of September, Zack was getting ready to have some doctor appointments.  His first visit was to his Developmental Pediatrician.  Jim and I always have a lot of questions for this particular doctor.  I mean, she is the expert and we crave information. Information is power, right?  Except when there are no clear-cut answers.  Just like with his colon.  I just cannot let that be. It’s a character flaw I suppose.  Or just another way I am learning that in somethings only faith can give me peace.

At this particular visit, we discussed once again how Zack’s uniqueness is most likely linked to his chromosomal abnormality.  Because there are so many aspects of his body that work differently than his typically developing peers, this makes sense to us.  We also talked about the dreaded questions.  What will Zack’s life look like when he is no longer a child?  Will he be able to graduate from high school with a diploma or a certificate?  Will he be able to live independently?  Will he be able to handle bills?  Will he be able to have a family?  Support a family?  How do we plan for all of these different scenarios?

The answers to those questions are not ones I want to share here.  I do not believe in sharing predictions about my son’s future at the tender age of ten.  Rather, I want to allow him to grow on his own and let his body and brain (and God) decide how his story will play out.  But I am still a planner and a realist, so we talked about ways to help Zack develop into the best Zack he can be.  We discussed ways to help him become more independent and some important life skills that we need to work on in the next few years.  We talked about services that are available to families with kids who have special needs and how to apply for them.

One of those services is a grant program called LISS or Low Intensity Support Services.  This program provides funding to children with severe chronic disabilities, both developmental and physical, that are likely to continue indefinitely.  We were able to apply, but sadly were not chosen to receive the grant.  The selection process is completely random, by design, and occurs twice a year.  We plan to apply again in the spring and are crossing our fingers that we will eventually be selected.  The grant can be used for things like respite care (Oh, to dare to dream..), social skill opportunities (karate), medical equipment and other things.  We have been eligible for this grant all along and did not know it!  The important thing is we know about it now and that is why I am sharing it here.  Maybe someone else can benefit from this information as well.  It may just be a Maryland program.  I briefly researched this (like looked at one google search page) and could only find information about our state, but I’m betting other states have a similar program.

When Jim retired from the Air Force, we lost our respite care benefit.  We have wonderful health care overall.  I feel guilty even complaining about respite care.  However, Zack’s care is a bit tricky and not many people are comfortable caring for kids with ostomies and g-tubes.  Even family members who love our kiddo have to team up to help us as it can be overwhelming for one person to figure things out.  If we are lucky enough to eventually be selected for this grant, Jim and I might even be able to leave Zack for more than a few hours. Life goals…..

Zack also had a check-up with his GI doctor and things went really well.  Zack really likes going to Walter Reed and seeing his favorite doctors, nurses and social worker.  We learned that Zack’s weight has plateaued this year.  While that is not ideal, he has grown two inches in height which indicates he is doing better.  Jim and I are once again grateful for Zack’s g-tube as we can play with the amount of formula we give him overnight to encourage more weight gain.

While we were in with Zack’s doctor, a new GI fellow popped in to say hello.  Zack did not recognize her and, in true Z fashion, asked if we knew her.  It was a bit embarrassing, but she was very gracious.  She told Zack that she used to help take care of him on the ward as a resident and that he inspired her to go into Gastroenterology as her specialty.  I guess she really said that my pictures of his 9″ prolapses inspired her, but we will just go with Zack’s cuteness inspiring her.  Hearing that was such a blessing.  Knowing that Zack’s illness helped someone decide to want to help children like Zack…..it was very humbling.  Hopefully one day Zack will understand what that means as well.  Who knows, maybe she will be the one to finally figure his intestines out.

Several other great things came from our visit that day.  First, Zack was able to get his blood work done in the office with minimal fuss.  He was apprehensive and a bit uncooperative at first but, once he remembered that he would be rewarded for doing a good job, he held it together and even helped the nurse find his “good vein”.  This is a huge improvement and something we have been working on weekly at therapy for over a year.  Secondly, because we are managing so well at home we do not need to go back for 6 months!  Six months people!  I do not think this child has gone six months without being seen for GI issues since he was two years old!  It may even be longer than that!

November was a wonderful anomaly around here.  Zack did not prolapse once.  He did not have any obstructive episodes and needed minimal pain medicine.  This must be what “normal” ostomies are like.  It was delightful.  For the past two days, however, Zack is once again asking for Motrin and Tylenol for belly pain, not eating his breakfast or lunch and going back to his belly baseline.  Thankfully, the obstructive episodes and prolapses have not yet returned.  Fingers crossed….but we expect they will be back soon as well.

Now for the fun stuff.  Because Zack has managed to stay out of the hospital for quite some time now, we decided we needed a little more excitement in our lives and added a new family member.  I introduce to you, Captain Awesome Penrod, our new Golden Retriever puppy.

He’s a big, smushy, furry ball of love AND teeth.  Let’s not forget about those puppy teeth.  I hope that it is obvious that the small blonde one named the pup.  We could not talk him out of it and then the name just seemed to fit – both the dog and Zack.  We call him Cap and our vision for him is to train him to be a therapy dog and to one day be able to take him to visit children in the hospital.  Therapy dogs make/made such a difference for Zack and we would love to give back in that way.  He’s a pretty mellow pup, so we are hopeful.

We are also hopeful that Cap will help Zack with several things.  First, we hope that Cap will help Zack with his anxiety.  We have found a few people who are willing to help train Cap to recognize when Z’s anxiety is ramping up and to help calm him.  Of course, Cap will need to be much older than three months to start that training.  Second, we hope that Cap will be a good companion for Zack.  Third, we hope that our pup will help nurture Zack’s sense of responsibility as he learns to care for Cap.  No pressure, pup, but we expect great things from you!

In addition to the excitement of our new puppy, we also have a new celebrity in the house.  Zack made his TV debut tonight.  Take a look!

As the Christmas Season rapidly approaches and all of my cards are sent and all of my shopping is done (in my dreams), I am stuck by how lucky we are to be in this season of relative good health.  It has been a long road of illness for Zack and I am so incredibly thankful to have a Christmas where we can all relax and not worry about hospitals.

Although this year is a good one in our home, I cannot help but stop and think of some special friends that are struggling this year.  I think of our friend, Jim, who went to heaven just a few days ago after a long medical illness and his family who will have their first Christmas without him.  I think of our friend, Emmy, who just went through her second bone marrow transplant and is hoping to be able to be home for Christmas.  I think of our friend, Ryan, who is kicking cancer’s butt and just entered the land of ileostomies.  I think of my friend’s daughter whose family is trying so hard to find answers to her intestinal issues.  I think of our friend, Kayra, who lives in Turkey and has been sick for so very many years and just wants to be a little boy.  I think of my friend, Stephanie, who is missing her beautiful boy, Logan, as the one year anniversary of his passing is quickly approaching. I think of one of Zack’s doctors whose son passed away.  While Christmas is and should be a joyful remembrance of Jesus’ birth, my heart is heavy with prayers for my friends and I am reminded that, for some, Christmas can be a hard time of year.

Wherever this Christmas Season finds you, I hope that you will find the time to slow down and treasure all that is important to you and that you find time to hug your babies!.

~ Dawn