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

 

 

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

 

 

Vacation and Boston

Oh vacation, how I have missed you so.  Our family had a great time visiting family in Connecticut, playing in Bar Harbor, Maine and exploring Boston between doctor appointments.  Here are some of our highlights:

Boating in Connecticut.  Zack’s first time on a fishing/lobster boat and he loved it!  He was a man of many questions and is forever bonded with Captain Don.

IMG_20160813_231343_01Zack helping Captain Don drive the boat.

IMG_20160813_162355656Jake and Jim could get used to Zack doing all the work.

IMG_6164Zack and his shark.  Not bad for a first fishing trip!

IMG_20160813_220526112_TOPThe Co-Captains relaxing after a hard day on the water.

Exploring Acadia National Park.  I am not sure how we have so few pictures of this.  Here are Jim and Zack on top of Cadillac Mountain trying to pick up one of the islands in the harbor.IMG_6152

Bar Harbor adventures.  Jim, Zack and I took a 4 hour sea kayaking tour.  See those islands in the picture above?  We paddled to the third and took a break.  Then we went around the third and back into shore.  We saw some porpoises and bald eagles.  Happily, we did not see any of the sharks that also inhabit the harbor because that would have freaked me out.

IMG_6118 IMG_6110We rode the carriage trails in Acadia National Park on bikes.  We ate popovers at Jordan Pond, frolicked on Sand Beach, drove around the entire island and shopped and ate and had a grand old time.  Zack and I also went on a date on Diver Ed’s boat.  Diver Ed scuba dives into the harbor and videos the ocean floor as he goes.  The video is broadcast on board.  Then he comes up with some of the creatures he finds down there and the kids get to touch them all before releasing them back to the ocean.  Zack was so enthralled with the whole thing until the touching part.  He finally worked up the nerve to touch a few things.  Can you tell how hard it was for him to do it?

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After a week of fun, we headed to Boston to see Zack’s doctors.  First we met with Zack’s surgeon.  It was strange to see her in a new hospital, but she was as awesome as ever.

While Zack is currently doing better than he has in years, we still have some concerns.  You may have noticed that I have been talking about how his stoma has been swelling which constricts his output during the day and then goes back to normal when he rests.  His surgeon and doctor here both feel he is prolapsing internally.  One thought is that where his stoma was stitched down internally actually worked this time, but his intestines before that point internally are still trying to get out.  We think they are getting stuck at that point, cutting off the flow.  Thankfully things have been resolving well so far, but it is not supposed to be happening.  We have a plan in place now for IF things go wonky.

Today we met with one of the leading pediatric motility experts in the nation.  He asked us lots of questions.  He said that Zack’s case is interesting because there is a lot going on that does not make sense….yet.  He is hopeful he can figure out what is happening with Zack.  He did say that just because Zack’s small intestine manometry test was normal does not mean his small intestine is functioning normally, just that things are being pushed through.  We talked about Zack’s biopsies that showed eosinophilic ganglionitis, the possibility of autoimmune issues and other things, but he wants to review all biopsy slides, testing, fluoroscopies, manometries, CT Scans…basically everything that has already been done…for himself before giving his opinion.  Jim and I appreciate that and think it is a good idea.  Of course, Zack’s files from both Cincinnati and Walter Reed are so incredibly large that it will take some time for him to get through it all.

The doctor said that there are several drugs we can try to help slow down Zack’s output.  He wanted to talk to his surgeon about a few things first, so we do not have a plan for that yet either.  Even though we left without a plan, Jim and I felt that he was very thorough and we really appreciate that he wants to understand why Zack’s body is behaving this way.  Why does he have secretory diarrhea?  Why doesn’t his colon work?  Is there also a problem with his small bowel?  All questions his other doctors have asked before and have not been able to answer.  Somehow I feel good about his method of investigation and have hope that perhaps he will be able to narrow things down a bit more.  It is good to have hope.

While we were in Boston we were able to connect with some of dear friends that we had not seen since 2011.  We spent a great day at their home catching up.  Why do we wait so long to do these things?  Oh yeah…intestines.  We were also able to see a friend from our time in Minot.  Her daughter has been in the PICU since DECEMBER!  December people!  I am happy to say that her daughter is getting close to getting out of the PICU and to finally going home.  It is amazing to see her faith and strength.  It was a blessing to me to be able to spend the evening with her.

So there you have it, an exceptionally long update.  We are so thankful for our vacation and our time in Boston.  We are thankful that we had a fun time as a family.  We are happy that we were able to come to Boston for a non-urgent visit.  We are happy to have scoped out the area, found a hotel close to the hospital and to know we have friends close by.  While we did not have time to see all of our friends here, we know we will be back! Until then…

IMG_6104Hug your babies!

~ Dawn

Home For Now

When we checked into Walter Reed last Wednesday, Zack could not walk from the parking garage into the building.  He was so weak that he rode in a wheelchair to see his doctor and then to be admitted onto the ward.  He was so pale that his feet were a freaky version of white and his tummy hurt all of the time.  I am happy to report that after his “tune-up” our boy has regained some of his spunk.  I like spunk.

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Zack’s iron levels are improving.  There are several different markers for how your body uses iron.  I do not know what they all mean, but I do know that the number that shows how hard his body is working on making new red blood cells is up.  That is good.  He has pink lips again, his ears almost have some color again and his feet are less freaky looking.  All good things.  Even better, his energy level is returning.  When we left the hospital yesterday, Zack walked on his own all the way to the car.  That makes me pretty happy and Zack noticed it as well.  In fact, Zack commented that his doctors must be crying happy tears because he feels so much better.  Amen.

One of the issues Zack has struggled with his entire life is eating well.  Through the years, we have done a pretty good job of getting enough calories into our guy.  For a few years he even tracked along the 50% curve on the growth chart.  Sadly, for the past few years he has been dropping on the chart and is currently below the 5th%.  Since surgery, Zack has had a lot of pain with eating as well and this has led to long periods of Z not eating.  The decision to give Zack and NG tube was bit hard emotionally for me and for Jim. How will Zack feel about having a tube in his nose?  Is there anything else that might happen that we do not know about?  If there is something else, do we really want to know?  We are getting a little tired here…But then we regrouped.  Just needed a moment to process.

Obviously, we wanted to do what was best for Zack.  His doctor explained that his poor nutrition could be contributing to his belly pain.  Poor nutrition hinders healing, energy levels and a host of other things.  Zack was just not getting better, so we knew we needed to have the NG tube placed.  Surprisingly, after the initial yuck of placing the tube, Zack has done really well with it.  His belly pain has dropped significantly as well.  He is currently receiving all of his calories through the pump, but is allowed a few bites of graham crackers and jello and to drink his precious G2 (lower sugar Gatorade).  Beginning next week, we will introduce full liquids as well.  I am unclear about how long Zack will be on his feeding tube, but I am thrilled he is doing well with it.

As Zack and I drove home, Jim was home getting Zack’s new Kangaroo Joey feeding pump set up.  Sadly, the guy who dropped it off did not know how it worked, so we spent a bit of time figuring it out.  That was a bit frustrating as he was supposed to be able to explain it all to us.  Happily, it is much like the one we used in the hospital.  It took a little doing, but after calling and talking to our nurses, talking to customer service and searching Google, we were up and running.  Zack loves that he has the option of wearing his pump in a backpack so that he can do all the fun things he likes to do and not be tied to an IV pole.  It is actually pretty handy.

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Z getting ready to check out the neighborhood for the first time in 10 days.

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 Wearing his snazzy backpack.

So now what?  Well, Zack seems to be having an issue getting stool out.  Over the past two weeks it has become evident that he is going several days without any output and then has a day where he becomes very uncomfortable and distended and then it all bursts forth.  Real life people, real life…When this happens he is in considerable pain, vomits and bleeds from his bottom.  We know that this is most likely from his stricture at the anastomosis (reattachment) site between his rectum and descending colon and that the stricture needs to be fixed.

Obviously, we would like to have this problem addressed as soon as possible.  After much discussion between our doctors here and Z’s surgeon in Cincinnati, we have decided to head back to Ohio in the near future.  To fix the stricture, Zack will go to the OR where he will be scoped before the procedure to see how the anastomosis (reattachment) looks.  His surgeon will then dilate the stricture with some sort of balloon.  Once that is done, she will scope him again to make sure that there are no complications and that is it.  It is not surgery, but it is performed in the OR.  Usually, this procedure is a same day procedure, but because Zack’s body has been difficult lately, his surgeon wants to admit him overnight just for observation.

Zack’s surgeon will be out-of-town next week.  Jim has a business trip the following week, so we are trying to decide if he will cancel his trip or if we can wait a few days longer to travel to Cincinnati.  We should have it all planned out by the beginning of the week and look forward to solving yet another issue for our guy.

One lingering concern is whether or not Zack is having motility issues as well.  When we went to Cincinnati for motility testing, all the tests came back normal.  That was before he was reattached.  Now that his intestinal tract is put back together, there is some question as to whether or not his motility is actually normal. It does not feel as though it is…but that is a problem for another day.

Today we are happy because Zack is home to help celebrate his big brother’s birthday.  He was most upset yesterday when his tummy was not feeling well.  He was crying because he needed to get home to his big brother for his birthday.  He told everyone he was feeling awesome, even though he clearly was not, because he did NOT want to stay another moment in the hospital.  He got his wish and is so excited to be home to celebrate Jake’s birthday.   I think he is most looking forward to giving birthday spankings and presents.  He’s all about the presents.

Thank you to everyone who helped us over the last week and a half.  We appreciate all of your visits, meals and calls.  Thank you to everyone who helped shuttle Jake to and fro and to our awesome friends who put together a birthday surprise for Jake at his Youth Group overnighter.  My heart was happy to know that Jake’s birthday was extra special.  You guys make me smile.

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Um.  Yum!  Chocolate cake!

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My 17-year-old.  Wow.

When we get more information about our trip to Cincinnati, we will update our blog.  Until then, I am hoping for a few uneventful days.  It is about time.

Hug your babies!

~ Dawn

 

Surgery Time….Again

Tomorrow marks one week since Zack was discharged from Johns Hopkins after his crazy stoma prolapse incident.  Yes, let’s call it an incident.  Since then he has been doing really, really well.

zwendys

Thumbs up for getting food in his belly after school today…or maybe just into his cheeks.

Even so, the time has come to do something about this stubborn stoma.  Zack cannot continue to go on like this and honestly, neither can we.  I mean, we could, but it is no longer the best option for our son.

After much discussion with both Zack’s GI here and his surgeon in Cincinnati, we have decided it is time to go ahead with the reconnection surgery.  The results of Z’s week-long GI testing in July showed normal motility and normal anatomy.  The only abnormal finding was that he was not able to empty his colon after the contrast enema.  Like at all.  Not one bit.  Nada. Zero.  Nothing came back out after that procedure.  Normally, the fluid they use for the test just runs back out.  That was concerning because his colon has been diverted by the stoma for over 14 months and he was still able to hold all that fluid in his body.

Zack’s testing also showed redundant bowel loops in his colon.  Think floppy colon…at least that is how Z’s surgeon explained it or maybe just how I understood it.  The redundant bowel loops combined with Zack’s withholding ability makes things a little interesting.

To overcome this, he will have his sigmoid colon removed along with part of his rectum.  By removing the redundancy in his colon, his stool will have more of a straight shot out of his body.  By removing part of the rectum, he should not be able to hold as much stool in anymore.  An important point, Zack is not willfully holding his stool.  He has no conscious control over it.  He likely never learned to stool properly and really has to relearn the behavior.  Fun times.  Man, it is hard to type this because I understand it when I am listening to the doctor, but I am not sure if people can understand my explanation.

After the sigmoid colon and part of the rectum are removed, Zack’s surgeon will attach his remaining colon to the remaining rectum.  She said they use staples for this at Cincinnati Children’s and that they have had great results. Leaking from the attachment site is a complication from this particular surgery and they have not had any incidences of leakage in over 4 years at Cincinnati Children’s.  Once they attach that segment, air is pushed through while one doctor looks from below and one from above to make sure all looks good.  If they have any concerns at all, they will close Zack up and leave his ostomy to allow that section to heal.  If it all looks good, they will then head up to his ileostomy.

The second part of the surgery will be reattaching the small and large intestines.  At this point, the doctor will assess if she can use all of the small bowel or if some will need to be removed.  It is probable that the part that keeps on prolapsing will have to be resected.  Zack has already had a little bit removed once before (15 cm) .  This should not affect his body in any negative way.  Once this has been done, she can then close his abdomen up.  He will also have botox injected into his sphincters to make his first bowel movements less painful.  Sounds easy, right?

After surgery, Zack will be on IV fluids until his body begins to produce stool.  Typically, this averages 4 to 5 days.  The first stool will be very watery and full of mucous (I hope you were not eating while reading this…).  Once this happens, Zack will be able to begin eating.  Eating real food should cause the stool to begin to form.  At this point, Zack will begin taking large doses of laxatives.  Are you jealous yet?

We expect Zack to be inpatient for 5-7 days.  Once he is released, we will stay another week or so to help regulate his laxative dose.  This involves having x-rays to make sure he is emptying properly.  We expect to be in Ohio for a total of two weeks if all goes according to plan.  We are due for that one of these days….

My eyes are now burning because thinking about all of this makes me tired.  We thought we had more time before Zack would have this surgery.  When we left Cincinnati, we thought he was done with surgery until next summer.  We thought Zack would be able to have a break from surgery and hospitals and pain.  I think all of us will just be glad to get this over with and move on to the next phase.  I originally thought the next phase was going to be easy, but it sounds like it will have its own challenges.  Hopefully, those challenges will be able to be managed on an outpatient basis and without more surgery.

We will update our blog once we get to Cincinnati.  Prayers welcomed and appreciated.  As always, hug your babies!

~Dawn