Bringing an end to Cincinnati & 2014

Well, three months have gone by since I last posted and the year is suddenly coming to an end.  In some ways it seems like it has flown by - maybe because we have literally been flying the entire last half of the year, but mostly it feels like one of the longest years on record for me.  I will gladly bid 2014 adieu.  

The main reason for not posting is I was instructed by the staff in Cincinnati that I could not publicly post about any results while the trial was ongoing and even afterwards when the data is being analyzed.  I signed a bunch of paperwork at the start of the trial and apparently I agreed to this stipulation in all the fine print - oops!  I must be very careful about any information I divulge so I will be focusing on non-trial related news of which there is plenty. 

What I can say is I know getting Joshua up to the top notch Cincinnati Children's Medical Center was well worth it, regardless of the results of the drug trial.  I would definitely do it again, especially with our super helpful Aunt Karen up there to help us every step of the way.  She was amazingly supportive and made the trips enjoyable with home-cooked meals and lots of sightseeing.  Joshua paid for her services with many hugs and sincere laughs and smiles.  I'm pretty sure Aunt Karen thought she got a pretty good deal! 

As every year of Joshua's existence has been, this one was filled with highs and lows with many bumps in the road.  We made it through some scary episodes, but I feel Joshua is in a much better place now then he was in the beginning of 2014 from a medical standpoint.  He is basically off all maintenance antibiotics and we have a clear plan for weaning from his IV fluids in 2015 with the goal of removing his CVL as soon as it is safe to do so.  Unfortunately, the plan involves a hole in his stomach with the surgical insertion of a g-tube for feeding and rehydration.  I had desperately hoped to avoid this since Joshua has managed to make it so long without one which is unique for his condition.  If we ultimately get to trade the CVL for the g-tube, we will take it since the CVL has so much more infection risk associated with it.  The g-tube will allow us to provide bolus feeds during the day or continuous feeds at night along with the ability to provide extra fluids for rehydration when needed.  While transitioning away from the IV fluids through the CVL, not having a means of giving Joshua extra fluids would mean many, many hospital stays in order to get a peripheral IV - something none of us want since hospital stays are very difficult on the whole family.  So the g-tube is necessary for multiple reasons, I am just having a tough time accepting my baby going through yet another invasive surgery even if it is for the better in the long run.  Hugging him and saying goodbye as he is taken back to the OR by a stranger gets harder every time, no matter if the surgery is considered "routine".

We made it through another terrifying central line infection in April caused by the nasty bacteria Klebsiella.  The fever from this infection brought on a febrile seizure that landed Joshua in the PICU for the first time.  Boy, I sure do hope we never see the inside of those walls again.  After spending one night watching and listening to Joshua's poor little neighbor code seven times, I was exceedingly grateful to leave with nothing worse than a short seizure and sepsis.  I am reminded over and over again every time we are admitted how fortunate we really are and how much worse Joshua's situation could be.  I am also made to remember those things that are important versus others which are not.  If your child was in the hospital and their condition was hanging in the balance - would you worry/stress/fret over that circumstance, detail, or plan?  If the answer is no, then it's really not that important so give yourself and your family a break and don't get your panties in a wad over it!

The real theme of the year in my opinion has been Joshua's developmental delays, rigorous therapy schedule, and the eventual Autism Spectrum diagnosis he was given in October.  And I thought I already had as much worry and anxiety as I could for our little superhero - wrong!  I can't describe how much of a blow this was to me and David.  We know so very little about how to help Joshua with this, but we will learn.  Joshua is currently in Speech therapy, Occupational Therapy, Hippotherapy (horse therapy), Feeding therapy, and will be starting in an ABA program at UGA the second week of January.  He is using sign language consistently and is able to communicate with us pretty effectively even with only one spoken word.  His therapists continue to remark how smart he is and how quickly he picks up on things and I think speech is right around the corner. 

The goals for 2015 will be continued early intervention for Mr. Joshua's delays and autism, as well as removal of the CVL after the g-tube is stable.  He will also be starting at the special needs Pre-K in August for 3 hours a day (terribly exciting and frightening at the same time).  I hope for less hospital admissions and more socialization with children his own age.  I pray for patience for me and more fun times with my spouse.  I give thanks for our extended families and dear friends that have carried us through the toughest times and provide us an outlet when we need it most.  May the very best be in store for you and your family in the New Year!

Cincinnati Trips 2-6 (Oops!)

Between the incessant traveling, the constant appointments, the minion always occupying the iPad, and dumb exhaustion, I have neglected to keep up with the weekly blogs posts I envisioned at the beginning of this journey.  I'm shooting for monthly posts at this point.  So before I am called away to mommy duty, let's hit the highlights of the last 5 whirlwind weeks (yeah right, this actually took me 3 days to finish).

Trip #2 on 8/27/14:

This was the first of four visits where Joshua and I traveled alone.  This was a daunting task, but I had learned a few things from the first trip that helped me pack and prepare better for travling without a pack mule (my Dad or husband).  Joshua continued to impress me with his traveling skills and remarkably good behavior on the plane.  

  

This was considered the Baseline visit which included the first dose of Gattex and three yucky blood draws to go along with it.  Our morning started bright and early with an 8 am appointment where he promptly began the first of three pokes in the arm over the next 8 hours.  The process did not go well for the poor guy and he was pretty traumatized after the first round.  Then he was treated to the first injection of Gattex and the clock started ticking down the hour until his next blood draw.  

Aunt Karen (my constant and greatly needed helper) and I were able to escape with Joshua for half an hour to give him a breather before the second round.  We found our way to an outdoor playground that was perfect for distracting the little guy for a bit.  Until a Child Life employee informed us the playground was closed due to the heat and we should have seen the signs.  Well I had seen the signs, but since it's 10 am, in the low 80's, and the playground is in the shade, I thought they were pretty silly.  Because we're from Georgia where you wouldn't step outside for half the year if your qualification for high heat is a balmy 83.  After a stern talking to, I was given "special permission" to stay out there for 20 minutes.  Jeez, these folks take stuff seriously at Cincinnati Children's!

After a relatively uneventful second blood draw, we had a 5 hour window of time to explore the hospital, have a long lunch in the cafeteria, and a nap with Joshua sleeing on top of me in a hospital bed.  Then came the last and most traumatic arm poke before we were allowed to escape to the airport to begin the long trek home armed with a week's supply of Gattex.  

 

 I'm finally home!

Trip #3 on 9/2/2014:

This was a relatively easy visit compared to the last two.  The appointment was at noon instead of 8 am which made for a more leisurely morning and it was only 90 minutes long instead of 4-8 hours.  Most of the appointments will be like this one, thankfully.  I have to bring a urine sample from Joshua and the Input/Output diary I am responsible for keeping daily.  Joshua has vitals measured, including the all important weight gain, a blood sample is collected from his line, a physical exam is performed, and then we are basically free to go after speaking with the doctor, dietitian, nurse, and coordinator to collect the next week's supply of Gattex.

Trip #4 on 9/8/14:

After a year of not having a single reduction in Joshua's TPN and lipid volume, visit 4 brought the first decrease we have been anxiously waiting for!!  Joshua's TPN rate was reduced by 10% - woohoo!  Dr. Cole pointed out that 10% only amounted to 50 some odd mL of fluid, so it wasn't anything to get too excited about.  You bet it is!  It's a step in the right direction and we are very grateful for it.

Trip #5 on 9/15/14:

This visit Joshua had lost a little weight after the reduction in TPN volume so the rate was not reduced any further.  On a positive note, Joshua started reliably drinking a smoothie concoction I make for him with banana, coconut milk, peanut butter, and coconut oil.  I hope I will be able to sneak in other ingredients that my picky little eater normally doesn't approve of!

Trip #6 on 9/22/14:

This week David had the privilege of accompanying us so Aunt Karen was able to hit the beach with family.  This visit was another long appointment with three blood draws spaced out over six hours.  I was really anxious to get past this visit since it signals the halfway point, we get to start going every other week instead of every week, and it's the last long appointment that requires all of these yucky blood draws.  

I had bragged to David about what an angel Joshua had been on basically all of the previous flights, so he decided to turn into a Gremlin on the flight out just to prove me wrong.  David was sitting in the row in front of us and pretended not to know who the screaming child was.  All of our fellow passengers and crew members were immensely relieved when the monster finally passed out.  The unfortunate lady sitting with me and Joshua told me at the end of the flight what a good Mom I was - if you only knew what I was thinking while the little dude was fussing his brains out, you probably wouldn't compliment me.  ;)

The day picked up once we got to the hotel and were able to grab a bite to eat before playing outside!

  

    

Then the evening got really interesting, as I took a spill stepping off a small step while trying to take pictures of Josh.  I rolled my ankle before deciding it would be better to fall into the grass rather than tear my ankle up.  I writhed around in the grass for 15 minutes moaning "Oh God" while Joshua decided I must want to wrestle and crawled all over me.  Much better than him taking off for the road that was 30 yards away, fat chance of me being able to keep up with him given my current state.  

I managed to grab a picture in mid-air as I tumbled to the ground.  The next day after being on my feet for 16 hours, my ankle was in pretty rough shape and I had to stuff hospital gloves in the back of my shoes to keep them from rubbing skin off my heels.  Am I "styling and profiling" you ask?  Nah, more like "limping and blistering".  

The long appointment went surprisingly better than the last one, Joshua didn't make a peep during the first two blood draws.  The only tears came when he was awakened from a 20 minute nap to a tourniquet being tied around his arm before the final poke and even those were very brief.  He is such a trooper!  

  

Somehow we managed to make it to the famed Cincinnati Zoo for a few hours before our flight.  I tried to ignore the resistance from my ankle as we walked around trying to show Joshua all of the animals.  I think he noticed about 4.  

 

The great news from this visit is Joshua had gained a significant amount of weight - yippee!  I believe this is because he recently started accepting his peanut butter and banana smoothies.  Now the dietian wants me to add raw spinach to it and see if he will still accept it.  The jury is still out on that.  Because of his rate of weight gain, the doctor approved cutting out four days of his lipids so now he will just require lipids three days a week.  That is so awesome because it's the lipids that cause eventual liver damage and it reduces the time it takes me to prepare his IV fluids almost in half for four nights a week.  More great progress!

In non-Cincinnati related news, Joshua has been seen the last two weeks in a row and will be seen again next week at the Marcus Autism Center.  The first appointment was a consultation with a nurse practitioner to decide if he should proceed to the pre-test with the psychologist.  We had the pre-test with the psychologist this week.  This was after being told to my utter amazement that the next available appointment was 8/28/2015.  Polite persistence paid off and I was called back that afternoon with a cancellation for this week.  I can't imagine having this autism evaluation hanging over our heads for the next year, I am just ready to know one way or the other whether these Marcus people think he falls on the spectrum so we can get him additional services if he needs them.  Joshua's 4 hour evaluation is on Monday at 9, but only a helicopter would be able to get us through Decatur traffic to make it there on time.  We should have results that afternoon. 

And to end this post with a positve twist, check out www.onlineathens.com on Sunday to see an article in the Athens Banner Herald about Joshua and his paintings!  We fit in a session with a reporter and photographer this week and it will run on the front page on Sunday (underneath UGA football coverage, of course).  I will post a link when available.  The link to Joshua's paintings is:  http://growingupfolden.blogspot.com/p/artwork-by-joshua.html

Sending love and hugs to all the Joshua supporters out there!!

xoxo

Joshua & his Mama

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!!

Joshua post surgery

May was another busy month for this little superhero.  Joshua had an exploratory abdominal surgery on 5/6 to look for adhesions and/or strictures based on an abnormal X-ray study in April that showed a loop of dilated bowel and the chronic belly pain he has been living with for over a year.  I was prepared for the worst - a mass of adhesions and multiple strictures requiring additional loss of bowel plus the tough recovery from the intensive STEP procedure to surgically lengthen the dilated loop of bowel.  I anticipated a 2-3 week hospital stay.  However, once the surgeon got in there she discovered one adhesion, no strictures, and only a small section of dilated bowel that was not worth performing the STEP procedure on. 

It was quite a shock to receive basically positive news from the surgeon and a bit of a disappointment that we did not find the smoking gun we have been trying to identify for so long.  I was insanely hopeful that Joshua's chronic pain would be resolved after the surgery and this has not been the case, unfortunately.  I am also concerned that the dilated loop of bowel seen in the X-ray is still present, but only balloons up when Joshua ingests something according to the radiologist reading the X-ray.  Since Joshua was not allowed to eat for 12 hours prior to surgery, the questionable loop of bowel was not dilated like it gets after he eats when the surgeon went in.  That is, the bowel loop is still getting dilated every day after food consumption for a reason we are still not aware of.  Another disappointment from the surgery is Joshua is no longer eligible to participate in the drug trial we were preparing to enter him in.  The trial would have had us traveling to Cincinnati 11 times in 16 weeks which would be exhausting, but he would have been fully evaluated by a new GI team and been given access to a very promising drug that stimulates bowel adaptation a full two years prior to FDA approval.  I was very optimistic the drug trial was going to be the push Joshua needed to get him over the hump with starting to reduce IV fluid intake.  Sadly, this option was eliminated since the trial is in its last phase and will not accept patients that have had surgery in the last 30 days so Joshua will have to wait two whole years before receiving the new medicine (if he even needs it by then). 

If a stricture isn't causing the dilation, the next most likely cause that hasn't been tested for yet is motility issues.  Abnormal motility is difficult and invasive to test for and our current GI team has zero experience with motility testing.  Luckily an esteemed motility specialist from Cincinnati Children's Hospital, Dr. Garza, moved to Scottish Rite recently and we were able to get an appointment with him earlier this week.  I had high hopes that Dr. Garza would be the second opinion we needed to get Joshua back on track with reducing his IV fluid intake (we've been stalled on this since last September), give us new insight into the chronic pain issue, and evaluate his candidacy for further motility testing.  The news we got is positive, but does not help us with the current issues all that much.  Dr. Garza does not believe Joshua would benefit from the invasive motility testing at this time.  Apparently most kids grow out of motility issues so even if Joshua received an abnormal result, the doctor would not change anything about his treatment plan which makes the 3 day inpatient testing seem pretty useless.  We did get a few suggestions for new meds to try and the affirmation from Dr. Garza that we are doing everything we can for Joshua.

Our next clinic appointment where our regular GI doc will discuss Dr. Garza's findings with me is on 6/20.  At this point, I think our team is basically going to lead me to the conclusion that Joshua is just going to have to live with the pain that wakes him up several times a night and hopefully he will eventually grow out of it.  This is not the answer I am looking for and would be completely unacceptable to an adult going through a similar experience that could actually communicate to a doctor how unpleasant every night is. 

Joshua had what I am terming another "allergic event" on 5/20 that had him vomiting profusely.  There were a few bloody streaks in his vomit and stool which caused our team to want him checked out in the ER.  His white blood cell count was very high, but he didn't have a fever, so I insisted on bring him home even though the wheels were in motion to admit us for a day or two.  Based on two similar experiences in April, I knew he would recover from the vomiting and there was no need to keep him hospitalized - he recovers much better at home anyway.  Now we have to figure out what is causing these allergic events and why.  The first two were in response to a probiotic, this last one was after eating steak (which he has had before).  I haven't convinced the doctors, yet, that these episodes are allergic in origin because his symptoms aren't what they expect.  I think their running theory is three separate short-lived stomach viruses that set in exactly 2 hours after eating a non-standard food item which neither David or I catch despite being covered in vomit and poop.  Seems pretty far fetched to me.

For now, David and I are reveling in the fact that Joshua has been feeling well during the day and has continued to steadily eat and drink more in the last few weeks.  We have had a few good weeks in a row and that doesn't happen very often in recent times.  Joshua enjoyed a nice trip to Lake Hartwell over Memorial Day weekend.  And we are spending as much time as possible in his favorite place - OUTSIDE!

I like the box as much as my new water table!

A bad, bad month

Line infection.  Febrile seizure.  Blood clot.  Severe vomiting.  Stomach virus.  Autism Spectrum Disorder.  Dilated bowel loop.  Stricture.  Barium enema.  Exploratory laparotomy.  Resection.  STEP procedure. 

If keywords could sum up our miserable month of April, this would be our list.  How could one little guy deal with all of these things in a single month?  It boggles the mind.  My only answer is he's extraordinary.  The last four are still ahead of us starting with the barium enema tomorrow morning. 

How does one frightened/worried/anxious/exhausted mom keep going despite karma throwing a big steaming pile of sh*t on her beloved son?  I'm still trying to figure that one out.

The condensed version of the April saga is:

Week 1 - we get results back from the endoscopy and gut culture.  Surprisingly, all results are negative for bacterial overgrowth and allergic inflammation.  The increased eosinophilic activity and mild blunting of the villi seen last September is no loner present - a positive sign finally!  Sadly, the positive news ends there.  Joshua attended GI clinic on Friday, had a lab draw from his line by a CHOA tech, then came down with a fever which turned out to be a line infection the next day.

Week 2 - we spent all week in the hospital as Joshua experienced his first febrile seizure and first stay in the PICU.  He battled back and fought off the line infection before it became systemic.  Unfortunately, a blood clot at the tip of the line was found during an ultrasound so he had to start being treated with twice daily Lovenox injections.  Joshua went through a gastric emptying study to try and pinpoint the source of his chronic belly pain.  This is the first step in motility testing.

Week 3 - this week was a little more laid back except I was learning the routine with adding three times a day IV antibiotics and twice daily Lovenox injections to all of the other care he requires.  We managed to fit in a vision screening and speech therapy and I was even able to make it to work two days!

Week 4 - here is where the train really starts going off the track.  Joshua beings violently vomiting on Monday two hours after I gave him a probiotic.  At first I thought it as the medicine, but then he continued retching and dry heaving long after it was out of his system and diarrhea set in the next day.  On Tuesday, Joshua and I went to the Emory Developmental Progress Clinic and our GI clinic to have labs drawn.  At the DPC, Joshua was referred to the Marcus Autism Center to have an autism evaluation.  This was not a surprise to me, although it was most unwelcome, because our speech and occupational therapists have both brought up concerns about a sensory processing disorder or autism.  I have not been focused on this partly because I haven't had time to and partly because I refuse to process the information.  To think we could get our sweet baby through all of these medical obstacles and his life could still be limited by another type of condition is crushing and I just can't accept it yet.  But, that's on the backburner for now.  At the GI clinic, our doctor and nurse practitioner examined Joshua and thought it likely he had a stomach virus unrelated to the probiotic.  After days of unrelenting diarrhea, Joshua and I went back to CHOA last Friday for the upper GI with small bowel follow through X-ray study we rescheduled so we could be discharged Week 2.  The study revealed a sinister situation - a loop of dilated bowel upstream of the point his intestines were surgically reconnected.  This is devastating news since it will require an exploratory laparotomy which will very likely result in another resection so Joshua will lose even more bowel if a stricture or narrowing is found.  Our surgeon is planning on speaking with us tomorrow to formulate a course of action after she has time to review the results of the barium enema.  I expect Joshua will be scheduled for surgery within the next few weeks. 

I have known since the radiologist showed me the dilated section of bowel on Friday that surgery was imminent, but it still hit me hard when I spoke to our GI doc yesterday afternoon and he said the words I never wanted to hear again - Joshua needs surgical intervention. The tears finally came today when the nurse called to schedule the barium enema tomorrow.  This is just the beginning of the painful, uncomfortable things Joshua will be forced to endure over the next weeks.  But, let's not forget he endures pain every single day.  This is just the latest in a lifetime of injustices for the little fellow.  I have no idea what to expect in terms of recovery since we don't know what the surgeon will encounter when she gets in there.  I anticipate we will be talking about weeks of recovery - not days.  You might recall he contracted a MRSA wound infection after his last resection that almost killed him.  The infection lingered for over a month and it was hell.  Surely his luck will change this time around, right?  One would think so, but it hasn't happened yet. 

A slightly positive aspect of this terrible situation is the surgeon can perform a STEP procedure, which is a surgical lengthening of the small bowel, while she is already in there.  This is a surgery I thought was only available in places like Boston and Pittsburgh, but now I understand is an option for us at CHOA.  This is a difficult and invasive surgery, however, it may give him a chance to recover the length of bowel he loses from a potential resection so the net result could be no functional loss of bowel. 

I hope to get a better idea tomorrow of what is in store for us during the month of May.  I have amazingly low expectations.

Another test behind us

Joshua is doing well after his endoscopy and colonoscopy this morning.  He recovered from anesthesia ready to roll!  He was (unsteadily) roaming the halls of Day Surgery within minutes of waking up.  The doc said most everything looked normal for a short gut kid from the naked eye, but the biopsies and cultures collected today will tell the real story.  Those results should be in next week.  We have another clinic appointment scheduled next Friday afternoon to discuss all of the results and potential steps moving forward. 

The doctor did see a "miniscule superficial erosion" in the distal portion of the duodenal wall consistent with inflammation of the mucosal lining due to an allergic response or bacteria - that's the million dollar question.  He also found an area of lymphonodular hyperplasia in the duodenal bulb which basically means his lymph nodes are overly active in this spot.  This is apparently not uncommon in short gut kids because what little intestines they have are forced to work extra hard and this is a constant source of irritation which stimulates the lymph node activity.  Both areas were biopsied so we will know more from the histology reports next week. 

The real thrill and surprise for me this morning was learning the doctor was able to collect an aspirate of gastric fluid to perform a gut culture.  I have been fighting to get the gut culture protocol reinstated at CHOA for months because this test is helpful in identifying the specific types of bacteria causing overgrowth problems.  This would allow us to tailor Joshua's antibiotic regimen to the bacteria affecting him and not play antibiotic roulette using a standard protocol for all short gut kids.  It would also help us rule on whether bacterial overgrowth or an allergic response is the bigger culprit in terms of the inflammation and malabsorption Joshua suffers from and allow us to pinpoint a proper treatment plan.  I learned through my support group that gut cultures are routine at premier children's hospitals in Boston, Pittsburgh, and many others.  I was floored when our doctor told me this test was not an option at CHOA - the best children's hospital in the southeast!  I eventually figured out it used to be a protocol 8 years ago, but the one employee in the lab that read the complex culture results left and they just dropped the test altogether rather than training a new employee.  Unacceptable!  I hounded our GI doc for months about it and stalled the endoscopy until this additional test was included.  Our doctor went to bat for us cutting through some significant red tape and now the gut culture test will be available to any GI patient that needs it at CHOA.  I am pretty proud I was able to instigate this hospital policy change to benefit Joshua and future GI patients!  I am nervous and anxious to learn the results next week.  The gut culture cannot always be collected if enough fluid is not present, luckily our doctor was able to get a good specimen.  Growing out and reading the culture can also be tricky because there are typically multiple types of bacteria and you have to figure out which specific type is overgrowing by counting colonies.  The test is not perfect, but I know dozens of families that have benefited from it and having more information is always better.

I already know our doctor wants to order a gastric emptying test later this month to see how well Joshua's stomach contents are emptying into his duodenum because of his recent vomiting.  This involves Joshua eating radioactively labeled food (it better include bananas) and having a series of x-rays taken over the next few hours.  Sounds like a fun-filled afternoon for Mom & Josh!

Thank you to everyone for your extra thoughts and prayers this week for our little warrior!  He continues to amaze us every day with his strength, bravery, and sweet spirit!!