Cincinnati Trip #1

Well, we made it home safe and the trip went off without a hitch for the most part.  Joshua handled the traveling so much better than I expected, he is such a trooper!  He was scared of the crowds and the TSA experience to start out with.  We made it through Security with Joshua's IV fluids and medical supplies with surprisingly little trouble and Joshua perked up once we got on the train to the concourse.  We were able to pre-board the plane and Joshua was seated comfortably in a window seat where he promptly fell asleep while everyone boarded the plane and took off.  I couldn't believe he slept through the take off and most of the plane ride!

 

While I was checking into the hotel, I assumed Joshua was crawling on the nearby couches since this is a favorite past time and I could see the top of his head.  When I rounded the corner of the couch, this is what I found:

 

And, boy did he have fun seeing himself in his first full length mirror as soon as we entered our hotel room!  Within a few hours we were being whisked away to Aunt Karen & Uncle Jeff's house for a lovely evening.  Joshua was able to play outside in a backyard and we were able to enjoy a yummy home cooked meal.  It's extra nice to have family close by at our new home away from home!

The next morning started out early since the shuttle to Cincinnati Children's left at 7:30 to make our 8 am appointment.  Crystal, our do-it-all Clinical Coordinator, met as the door and led us through the maze that is CCHMC.  I was supposed to bring a urine sample and had been sent plastic bags that are supposed to stick around his little wee-wee inside the diaper.  Naturally, Joshua wanted nothing to do with this and kept pulling them off before I could get the diaper secured.  He also wasn't complying with supplying a stool sample, that is until the doctor decided to do a rectal exam to collect the sample.  Joshua promptly let one loose thirty seconds later and we finally got the urine sample at the same time, too.  Smart kid.  

Dr. Cole and his staff assured us Joshua is an excellent candidate for Gattex, they fully expect he will be able to tolerate more feeds, his stools will be thicker, and his appetite will naturally increase due to his body being able to absorb more nutrients.  He gets to start the drug next Wednesday, 8/27 when we go back for our next trip since this week's trip was the screening visit.  Joshua had some blood drawn and then had to make it through an EKG before it was time to head back to the airport after a quick lunch in the cafeteria.  

 

Joshua fell asleep on the ride back to the airport and I managed to get him out of the car seat, hold him on my shoulder, and navigate curbside check-in.  The little guy only slept for 5 more minutes after I found a nice comfy counch for him.  Then it was time to chase him around the concourse, riding escalators & elevators, and trying our best to keep him from running in front of every single person in the airport.  

Oh how I wish I could say the flight back was as uneventful as the day before.  In true Joshua style, he pooped twice before the fasten seatbelt sign was off.  The first time the nice gentlemant next to me allowed me to change him right there in the seat.  The second time... well, let's just say the first sign I had was finding poop on the seatbelt - never a good thing.  As I trekked all the way to the back of the plane carrying Joshua and getting poop on my arms, I contemplated how I was going to change his entire outfit in the airplane bathroom.  Ever tried to lay a child down in a lavatory?  Ain't happening!  Thankfully the last row was empty and the sweet man sitting in front of us didn't mind the mess and smell of a head to toe outfit change - socks and all.  After that episode we couldn't make it back to our seats because the beverage cart was blocking our path so after more stepping on toes and knocking people's shoulders with the diaper bag, we made it back to the poop changing row where we stayed (lacking all electronic devices) for the remainder of the flight.  Joshua finally had enough climbing on me like a monkey and passed out in my arms through the landing, disembarking process, and woke up on the train to baggage claim.  

I know from the pictures it looks like he slept a lot, but that was the only time he slowed down enough for me to snap a shot!  For the 36 hours we were gone, he actually only slept about 10 hours total. The other 26 hours my Dad and I were chasing him around unless we were lucky enough to strap him in a seat of some kind.

In other news, we spent all day Wednesday at CHOA for a Hematology visit at the AFLAC clinic and then had to hang around for a few hours until our ultrasound appointment that afternoon.  The ultrasound showed Joshua's blood clot is gone!  But, it also showed a narrowing in the subclavian vein so the hematologist recommended he stay on a prophylactic dose of Lovenox which is one injection a day instead of two.  So that's a little progress! 

Today we visited an allergist in Athens for Joshua's initial skin testing.  He passed all the skin challenges, and combined with his previous blood testing, we can now confidently rule out anaphylactic allergies as a problem for Joshua - woohoo!  The not so good news is he definitely suffers from allergic collitis which is a tougher to diagnose condition.  We now have a referral to a specialist at Scottish Rite and the advice to see the Allergy Clinic in Cincinnati which is apparently top notch.  One more thing to add to our to do list while we're traveling to CCHMC.

Until next week and Trip #2...

Joshua's Short Gut Journey to Cincinnati

As we prepare to embark on a 16 week journey to enroll Joshua in the Gattex drug trial at the Cincinnati Children's Hospital Medical Center, I cannot help but reflect on what has brought us to this seemingly extreme option.  I am quite nervous about traveling with Joshua taking 11 round trip flights between now and Christmas.  I also hope to provide an explanation for why we are subjecting fellow travelers to Joshua's antics if any of them happen to make it to the blog (please forgive his ear-piercing squeals and frequent fussing at non-favorite cartoons; I promise to have plenty of snacks and every portable electronic device I own fully charged before takeoff.).

So here we go, a look back at the last two years since sweet Joshua joined our family and changed our lives forever.

March 19, 2012:
We received devastating news at our 18 week ultrasound.  The news it is a boy was completely overshadowed by the unfortunate diagnosis we received, a relatively rare condition called gastroschisis.  This is a type of abdominal wall defect where the abdomen fails to close leaving a small hole that allows internal organs to be outside the baby's body floating in the amniotic fluid.  There is a range of severity for the condition which cannot be fully known until the baby is born.

July 31, 2012:
After a relatively uneventful pregnancy given the circumstances of our son's condition, Joshua David Folden was born via semi emergency c-section at 37 weeks after his heart rate became erratic and the inducement process made things worse. It was a bizarre and frightening experience, but I knew the end result was worth it - our baby would soon join us!  Joshua was born at 10:37 pm weighing 6 lbs. 4 oz. and 18" long.  Hearing him cry as he was pulled from my womb was the most anticipated, amazing sound I can imagine (even as I turned my head and vomited profusely from the anesthesia).  After all the worries, concerns, tears, and sleepless nights fearing for my little boy... he is here and he is alive!  My goals after finding out about his condition were accomplished: he made it past 37 weeks and weighed more than 6 lbs.  I know have given him the best shot I can at a healthy outcome - now the rest will be left to God, Joshua, and the talented medical staff at Children's Healthcare of Atlanta.  

August, 2012:

Joshua was immediately whisked to CHOA Egleston after birth for abdominal repair surgery and recovery for gastroschisis.  He had a primary closure at the bedside and was recovering more quickly than anticipated.  He proved what a little fighter he is and our hearts melted as we were first allowed to hold him.

Joshua moved right along with having the breathing tube removed, gut rest to allow the bowel to heal, and once allowed - continued to steadily increase his milk intake.  So much so, that we were being prepared to be discharged on August 31st.  Then tragedy struck the night before that set off a devastating 48 hour cascade of events that will forever affect our lives.

September, 2012:
To keep it brief, Joshua suffered an extremely rare intestinal malrotation with volvulus that resulted in blood flow being cut off to sections of his bowel which then turned gangrenous.  Even after emergency surgery was performed and the gangrenous tissue that used to be half of Joshua's small bowel was removed, he was gravely ill and barely escaped with his life.  We were told by the neonatologist he had a 50/50 shot of making it through the night.

In true Joshua form, he battled through it and another surgery the next day to place an ostomy in his jejunum (second portion of the small intestine).  Joshua spent 5 weeks with an ostomy before having the takedown surgery on 10/4/2012 - another day that is cemented in my head forever.

October, 2012:
The ostomy takedown surgery revealed strictured, useless tissue below the ostomy down to the colon and Joshua ended up losing the rest of his ileum and the all important ileocecal valve that separates the small bowel from the colon.  He now officially has Short Bowel Syndrome and a host of long term problems that come with loss of half of your GI tract.

In the short term, however, we battled a truly frightening and hideous MRSA wound infection after the last surgery that once again, almost took Joshua's life.  His heart rate was sustained in the 240-250 range for hours during one particularly bad episode and I literally thought his heart would give out.  It took over a month of daily cutting and silver nitrating the wound with no anesthesia to eradicate the MRSA.  Joshua was on isolation precaution for months which meant he couldn't leave the room and anyone that came in had to be masked, gloved, and gowned.

November, 2012.
After a miserable six weeks since the last surgery, we finally start to make progress with feeding.  As we reach the end of the month, doctors actually start mentioning the big D word - discharge!

December 11, 2012.
The day has finally come!  After 131 days and nights in the NICU, we can take our baby home for the first time.  I only have to learn how to prepare and administer his IV fluids every night, keep up with his complex medication schedule, pump all the breastmilk he needs, change the sterile dressing over the PICC line, observe him for sign of infection, malabsorption, or any other sign of distress and the list goes on.  At the time I thought we were on the downhill slope of battling Short Bowel Syndrome.  Oh, how naive I was!  My mind was protecting me, I guess.  If I had any clue how difficult our struggle would continue to be once we got Joshua home, I don't think I could have handled it.

January 2013 - present
I could write a novel about the difficulties and triumphs Joshua has been through, overcome, and continues to struggle with.  Short Bowel Syndrome is a complex condition and missing your ileocecal valve leads to chronic issues with bacterial overgrowth and other problems.  Although the doctors initially were confident Joshua would have his Central Venous Line (CVL) removed and be off all IV fluids by the age of 2, we did not meet that marker and are quite far from it since his rate of IV fluids has not decreased in almost a year.  Joshua has struggled with food allergies (some yet undiagnosed, I suspect), bacterial overgrowth, life-threatening central line and bloodstream infections one that caused a febrile seizure, line breaks, a blood clot requiring twice a day injections for 6 months, an additional abdominal surgery for lysis of adhesions, multiple colonoscopies, liver damage from the IV fluids, developmental and speech delays from all the time spent in the hospital, and the worst - chronic severe belly pain that wakes him up many times a night and prevents him from resting normally.

I believe we have exhausted our options in Atlanta since we have been going to the only GI clinic dedicated to Short Gut in the state and have sought out the specialists in the area.  The time has come to take what some may consider an extreme step, enrolling Joshua in an experimental drug trial requiring 11 round trips from Atlanta to Cincinnati in 16 weeks.  The drug is called Gattex, it has been approved for use in adults for a decade, and it is supposed to increase growth of the mucosal lining in the intestine which should help Joshua's bowel absorb more food via mouth and decrease his dependence on IV fluids through his Central Venous Line.  We will also have the opportunity to start fresh with a new world renowned team of doctors.  I am so hopeful this is the step we need to get Joshua progressing in the right direction and find a resolution to this chronic belly pain.

Many people have asked about the travel costs associated with the trial.  The pharmaceutical company thankfully covers two airline tickets and one hotel room for each trip.  We will invariably incur costs on things like transportation to the airport, parking, extra baggage fees for all of Joshua's medical supplies, a third ticket for the times Dad can come, and other things that always pop up during travel.  Not to mention me missing more work, which hardly seems possible given my very limited hours during the last two years.  We have no other good option at this point so we have to make it work.  My Dad and David's Mom have each generously volunteered to accompany me on one of the first two trips since David is unable to go due to his school schedule.

We have been incredibly fortunate to be supported by wonderful family, friends, and employers during this life altering journey which has made maintaining a semblance of the life we used to know possible.  We are immensely grateful and know we will continue to feel the outpouring of love and support as we take on this latest challenge for Joshua!  I will do my best to update regularly about our traveling journeys and, hopefully, all the progress Joshua is making on the new drug!!