Field Day

Let me premise this next part by saying I am a chicken.  I hate being pushy.  It is really hard for me, even in situations like this.... The next morning I called the office of the pediatric endocrinologist.  The first opening was in six weeks.  So, I begrudgingly took the appointment and figured that we would just wait.  My associate/ bestie was sitting there flailing her arms and yelling at me!  She told me to not get off the phone and to tell the receptionist the concerns of the other doctor and that they had wanted us to be seen ASAP.  Believe it or not, I succumbed and did it.  Once I said that, everything changed.  She said that she would let the doctor know and that she would have the doctor call that day to talk to us and see if she could fit us in.

That afternoon she called, was as nice as could be, shared the same concerns and said she would fit us in over her lunch break the following Wednesday.  It was at this point that Judson and I decided it was time to call in our reinforcements.  Katie was due virtually any day with Mia, school was about to end, Levi was sensing all of the problems and acting out and management of all of our work schedules, swim lessons, doctors appointments, drop off and pick up, etc. was becoming quite the nightmare.  We called my mother in law who without hesitation got a flight to come out for three weeks to help!  She was scheduled to come in that following Thursday, our last day of school, the day after Katie was due.  Should be great!!!

So, Wednesday rolled around...Field Day at school.  I had arranged to go to the doctor with Judson and my mom during the break between the action.  When I woke up I had several messages on my phone.  Katie was potentially in labor. Not for sure yet, but the contractions were starting.  I went to school and waited, still moving along with our plan, phone in hand.  By the time our appointment rolled around at noon the baby was almost here.  My mom went to the hospital to be with Katie and my awesome aunt came with us.  It was devastating having to make the decision to go with Charlie and possibly miss the birth of my niece.  As I got in the car to leave school I called to check in on Katie's progress.  It was looking more and more like the baby would be there within the hour.  I wasn't going to make it.  I think it was the first time in all of this that I was actually sobbing.  I don't know why it took until then but for some reason this was my breaking point.

The doctor saw us right away.  Fortunately, she spent a lot of time with us and thought that the chances of a tumor were slight.  She ordered a bone age study to be performed and a growth hormone analysis to be done.  Another round of blood work but that we should have some answers.  The second we wrapped up, I bolted.  As fast as I could I ran to my car.  It was literally a scene out of the movie.  I pulled into the valet at the hospital and ran up the stairs, in to the hospital room and made it just in time!!!  Phew....

Georgia arrived the next morning and we could breathe a little easier.  She and Judson took Charlie for all of her bloodwork and X-rays so that I could make it to my last day of school.  We then waited again...as I said, waiting is something we unfortunately are used to at this point.  The bone age study was normal.  Normal, was not what we were looking for.  Normal, means that she is not growing at the right rate!  The hope was that her bones would be behind and that she would be able to catch up.  We were fine with puberty staring later :)  Everything else was fine, growth hormones, etc.  The doctor explained that we could go ahead and order an MRI to explore further or wait three to four months to give the medicine some time to work before we had to do another test like this which would include another general anesthetic.  We decided at this point we had put Charlie through so much that we wanted to wait.  It was summer and we needed a break, Charlie needed a break.  We had our Children's Hospital appointment coming up and would be looking forward to a second opinion and what that doctor would be able to tell us in terms of where to go from here.

Summer began and we had a solid three weeks of no appointments, which was bliss!  We were able to hang out and enjoy time together.  Play with Nana, go strawberry picking, have playdates, play with the new baby, swim and be NORMAL!

Doctor Drama

That afternoon I had left not an extra moment to spare in order to make it from picking up Levi at school to his first swim lesson across town.  I signed him up for a crash course at the beginning of the summer.  When we signed on with this company there were pages of instructions, directions, etc. all of which went over the fact that if you were late, too bad!  So, with ten minutes to get there and Levi not even dressed yet, the phone rang.  I quickly pulled off the road and into the Vallarta parking lot.  I whipped out my paper and pen.  I tried as hard as I could to listen to the doctor over the screams of the two toddlers vying for attention from the back seat.  There may have been a few bribes thrown in and a pacifier and blanket tossed in the back seat.  Bottom line was that he had found that she had a severe arrhythmia of her stomach.  Her stomach made no contractions of its own without food. A typical stomach should contract roughly three times a minute in order to move regular secretions, stomach juices, etc. When food entered the stomach there were slight and weak contractions which is why food does get out, yet not effectively and efficiently.  They were able to narrow the problem down to being neurological (the nerves not firing properly as opposed to the muscle just not working).

From here he wanted to prescribe two different medications.  Both with gnarly side effects.  Both I had already researched and was already scared to give her.  He asked me to call in the morning to make an appointment to come in to meet with our regular doctor to go over everything.  We spent that night panicking, freaking out, etc.  We researched, talked to my cousin (a pediatrician), my stepdad, etc.  Bottom line is that one of the drugs is an antibiotic (Erythromycin.)  We would be giving her two doses a day, potentially for the rest of her life.  It would force stomach contractions which is the good news.  The down side is that by giving her an antibiotic it can make her more prone to superbugs such as c. dificil or make the efficacy of the antibiotics less so when they are actually needed down the road for another illness.  The other drug, Reglan (another drug to increase motility and promote contractions) has a black box warning from the FDA.  It can cause uncontrollable muscle movements and ticks, what appears to be Parkinson's like symptoms.  It occurs in a large percentage of people given the drug and oftentimes is irreversible.

I called the next morning to make an appointment.  They made one for us on the next day he was in the office...just my luck it was smack in the middle of our last field trip of the year.  I went home that night and with the help of my mom, stepdad and Judson we typed a list of questions three pages long (no joke!)  We went in prepared!!!!

On the day of the appointment we saw him and it quickly became apparent that Charlie's condition was a bit beyond his scope.  He was unaware that we had even gone through with the test, had not even seen the results, yet had known that we had been prescribed these meds.  Suddenly many of my questions became irrelevant.  We felt as though this group was no longer able to handle her condition.  We had been told all along that her condition was extremely rare, and that there are just not many cases like it, therefore not much research or others to go off of.  They were learning as we went.  It was at this point that we made the decision to move on and seek out a second opinion with a doctor who had experience with her diagnoses regardless of where that took us.  In the meantime, we were deciding to hold off on the meds until we could speak to someone who could explain our options in a way that could put us at ease.

We left the office and immediately called Dr. M.  He spoke with me over the phone and thought that it would be best for all of us to come in so we could sit down and go over it all together as a starting point.  He stayed late after his last patient one day that week so that Judson, my mom and I could sit down with him.  We met with him for over an hour.  He again explained that in all of his years of practice he had never seen anything like this.  He went over the medications and put us at ease.  We agreed to start Charlie on the antibiotic but to hold off on the Reglan in order to see if we could get one to work before putting her on the other.

At the forefront of the next concern was that she was still not growing in height or weight and it was time to delve into that deeper.  For the first time it was suggested that this may be a separate issue and that it was time to look further into the cause.  Could it be a pituitary tumor?  Growth hormone issue, etc?  He suggested we make an immediate appointment with a pediatric endocrinologist as we didn't want to mess around with this any longer potentially risking stunted growth.

We left his office feeling much better.  Called the pharmacy to fill the prescription for the Erythromycin and called the endocrinologist to make an appointment.  At that point we began calling around looking for the most specialized pediatric motility clinics in the country to find that there are really only a handful...seriously about three.  After researching for hours on end we found that the best clinic and doctors are located in Columbus, Ohio.  I called, and they said it would be a few weeks to get in.  We were prepared to travel when suddenly I found that one of their doctors had just left to come to Children's Hospital to start a motility clinic in LA.  Could we have just gotten that lucky?????  Not only that, but her specialty is in gastroparesis.  We called and made an appointment.  It was going to be a wait to get in but at least we were on the books.  Baby steps but it was steps in the right direction!

and then the next test....

Our doctor called that evening to explain his frustration with the tech that had completed the first test and that UCLA would be redoing the test for us with solid foods.  I then explained to him that it had already been completed and that the tech had told us that the results were abnormal.  He promised that he would look into the system for the report and call us back as soon as he could find them.  So, we waited...and waited....and waited some more.  Two days later there was no word.  At that point Charlie had become incredibly sick.  It was presumed that she had pneumonia as a result of the bronchospasm from the anesthetic she had with the endoscopy the week beforehand.  From bad to worse, right?  A couple more days had passed and Charlie was on the mend.  It was then Levi's All About Me day at school.  Judson left work and met me for his special day.  As Murphy's Law would have it, right in the middle of our share the phone finally rang.  Of course, in all of this Levi had been the one person who had been getting neglected and the one chance we were getting to devote all of our attention to him was being taken by the phone ringing with the doctor calling with the test results.  We had no choice but to take the call, we had waited DAYS and who knew when we would have the chance to get the doctor on the phone again.

I ran out of the classroom, phone in hand to speak to him.  I had been carrying a pad of paper and a pen with me everywhere I had been those days, and yet in this moment it was no where to be found.  He spouted off some numbers.  I sat in the courtyard of the preschool listening as best I could.  All I caught was that she was suffering from a SEVERE delay.  Only 28% of the stomach contents emptied which was far below what would be considered normal.  Before going further he wanted to send us for a test called an "electrogastrogram."  Essentially and EKG of the stomach.  We called to set up the appointment to find out that there is only one woman in LA that does this test and she was completely booked for a month.  The next test opening was the last day of the school year and yet, we had to take the appointment.  Just so happened that that same afternoon our UCLA connection called to check on us.  I told her just that and within hours she got us an appointment for the beginning of the following week.

The night before the test I received a phone call from the doctors office that insurance approval was still pending.  She advised that they could not go through with the test without approval.  I called the company and got nowhere fast.  They suggested calling when they opened in the morning.  We woke up at 5 am our time and began calling.  We pulled on their heartstrings asking for supervisor after supervisor until finally we got someone on the phone who realized we were talking about a baby.  She then looked at her diagnoses and felt badly for us.  She told us she would rush it up to the board for approval and make sure it was the next case seen.  We should have an answer in an hour.  CALL BACK!  So, I went to my 7:30 meeting at work while Judson worked the phones and planned to meet the two of them at the hospital by 8:30.  He kept calling and still nothing.  Call, after call we got the same answer.  "Sir, it takes 3-15 days for approval for any procedure, even with a stat order. " We insisted that this could not be correct.  What about emergencies, or in this case a procedure that had been scheduled to be done immediately with less than 3 days notice.  There must be someone who can do something.  Still, no one who could help us.

Regardless, we decided to head over to the hospital to wait it out there.  Charlie had been fasting since dinner time the night before and we felt it was better to be there so we could start the moment we got approval.  The hours began to tick by.  Everyone in the waiting area became furious for us.  Elderly people waiting for their colonoscopy, others waiting for loved ones, everyone knew our story.  We made fast friends with the staff and patients while at the same time Charlie was becoming listless.  Soon she was throwing up and the situation was becoming more critical.  We needed to make a decision.  Do we reschedule for another day, wait for insurance, etc.  If we rescheduled we would have to wait a month for an appointment that was late in the day (therefore she would have to fast again for the same length of time...not a good option!)

At that point I went down to the financial planning office to find out how much the procedure cost on the off chance insurance denied us, Judson got on the phone again, the doctors office was calling over and over as was the hospital.  The staff at one point suggested we walk her over to the emergency room and insist they admit her so that they could perform the test that way. All doctors, with the exception of the head of pediatric GI were at a conference in Florida and we were left to make a decision.  We made one last attempt and while on the phone Judson got word that the insurance company not only DENIED the claim but had done so over an hour earlier but had failed to tell any one of the numerous people who had called.  The fury we felt to know that some random person sitting at a desk in an office building made this decision was beyond words!!!!

The staff felt so badly for us they didn't know what to do next.  At this point it was after lunch time and they paged the doctor who came running over immediately.  He told us that he knew exactly who we were, not only from our contact but...YES, her case is that rare and that he had already been in the loop and consulting.  He felt that it was imperative that she have this test and that it be done NOW.  He took us back and told us that they would figure out the money situation later.

They hooked her up to the machine and started right away.  Once the test was started he went over every bit of her medical history.  The best part was that we knew who he was from our friends, and knew that he was supposed to be great.  We had contemplated weeks ago switching to him as our doctor at UCLA and now we got to have his opinion.  He sat with us for a while and went over the test and what it could show.  He told us that no matter who was in town he was the one who interpreted the results and that he would be the one calling us to make a plan.  We felt a bit of relief having had him in the room with us and knowing that he was now part of our "team."

Charlie was a trooper.  She made it through like a champ.  Once the first portion of the test was over and we were able to get some food in her she made a comeback and got her smile back.  We are constantly impressed with how this little girl always looks and acts so normal.  No one would ever know by looking at her that she is sick.  We spent the afternoon waiting by the phone for his call, which came later that day....

Our Next Steps...Gastric Emptying Scan

From that point we were told to schedule a "Gastric Emptying Scan."  This is a nuclear medicine test that is performed in the hospital.  They would have Charlie eat a meal that had been laced with radioactive material and then through a series of timed "photos" be able to determine how quickly food is moving out of her stomach.  I immediately called to schedule the appointment hoping to get it done as soon as possible.  The scheduler at UCLA told me that the orders needed to be approved by the radiologist and that until that point we could not schedule an appointment, and that even then there was a two week wait at minimum.  As much as I hate being pushy, I called later that day and again the next.  They assured me each time that the doctor would get to it soon.

It just so happened that while we were waiting to hear back I was having a conversation with a friend about what had been going on with Charlie.  It turned out that she knew some higher ups in the UCLA system and was able to get us in contact with them. The next morning I was contacted and had an appointment for first thing the following morning!  Such a relief!!!!  She was such a godsend in this entire process.

Our doctor had explained exactly what foods to pack for her and so we did, we came prepared.  Upon arrival the tech hurriedly took us back and REPEATEDLY explained that he was very busy but that he was doing everyone a favor and fitting us in.  He then told us that he only needed her sippy cup of milk and that it was not necessary to use the solid food that the doctor told us to bring.  We questioned him, got his answer which made sense at the time and went ahead.  She drank her milk and we did the test.  She screamed and kicked.  It took an act of congress to hold her down and strap her to the table at each 15 minute interval.  It was painful to see.  We found that singing the "Wheels on the Bus" was our only chance of keeping her calm. At the end of the test he told us that it looked to him like the results were normal....her stomach emptied a little over 50% of the milk.  We were SHOCKED!!!  Not possible.  We were going to see the severity of the problem, not IF there was a problem.  We left there dumbfounded and immediately called Dr. M. and our UCLA GI doctor.  Dr. M got on the phone right away and was just as surprised.  He said he would contact the other doctor to see if they could go over the report and develop a plan.  Of course it was a Friday afternoon and we were left wondering what on Earth was happening all weekend.

Monday morning rolled around and as the kids were filing in the classroom door, my phone rang.  It was the tech from UCLA calling to explain that our doctor had called him very upset.  He had performed the test incorrectly and therefore it needed to be redone!!!  (Shocker, I know!) So, we were right from the start, the test was invalid because of the use of only liquids and no solids, plus the fact that the amount she ingested was not even measured.  He said he needed to talk to our doctor but would call me back within 10 minutes.  Sure enough he did.  He wanted us to come back in right away.  Only problem was that the test had to be completed on an empty stomach.  I ran as fast as I could to daycare to stop them from feeding Charlie only to find her taking her last bite of breakfast.  So, we had to wait four hours before we could start the test.  I was of course unprepared to come with a meal at all, let alone one that was appropriate for this test.  I had to quickly leave work and run all over town trying to find food to make her that they could lace with the radioactive materials.  My awesome teaching partner then met me in the kitchen where we made five different varieties of sandwiches just to make sure we would have one that was right.  I frantically called my mom to meet me at the hospital so I didn't have to be there alone with Charlie (the thought of having to go through strapping her down every 15 minutes and holding her still by myself was daunting.)  Judson was in a meeting and couldn't leave this time.

We got there, they did the test.  We kept Charlie busy by playing on the iPad while we waited it out.  She was much better about going in the machine this time, and actually smiled while we sang.  When the test was done we had the results we were "looking for."  Funny to say it that way.  It was almost peace of mind to be back to a place of knowing what was going on.  He told us then that the results were definitely abnormal but that the doctor would call us to go over everything.  Then the waiting game began again!!!!

Part III...the Endoscopy

Then we got to wait...something we have become very accustomed to.  At this point we all believed that there were significant chances of an obstruction around the pylorus...something that the two ultrasounds she had would not have seen.  Dr. M referred us to the pediatric UCLA group for an immediate endoscopy.  We needed to have a consultation with the doctor before the procedure.  Upon meeting him we found that he agreed.  There must be something there such as a web, growth, etc.  We would certainly find it and hopefully be able to correct it with surgery.  Although no one wants their baby to have surgery, I actually looked at it as best case scenario....we certainly did not want to be dealing with the alternative.  The procedure was scheduled and we were optimistic.

We arrived to the hospital early and got to play games in the preop room.  There was a lovely social worker who came by and chatted with us and brought toys for Charlie.  Soon they came and started the IV's, then the sedatives.  Boy, was she loopy.  Ultimately, they took our still smiling baby in their arms and carried her back to the operating room where they do the procedure.  She was placed under a full general anesthetic and intubated.  We knew that going in.  We were also told that the procedure would take no more than an hour and that they would be out to get us as soon as they wheeled her out so we could be there when she woke up.  We sat there nervously waiting, watching the hands on the clock tick by.  Close to an hour and a half later and we still hadn't heard anything.  I KNEW something was up.  Finally, they came out and said she was out but we couldn't go back yet, the doctor wanted to talk to us.

He met with us and said all went well on HIS end but that he didn't find anything, nothing, nada, zilch!!!  My heart sank.  I knew at that moment we had something on our hands that was going to be life altering.  He sat with us for nearly 30 minutes explaining that he was diagnosing her with GASTROPARESIS.  In lay terms, gastro = stomach and paresis = paralysis.  He talked to us about all of our options both long term and short term....she most likely would never grow out of this, would need to be medicated for the long haul, may one day need nutrition from a feeding tube and that gastric pacing (a pace maker for the stomach) may be an option as she gets older as they currently are not used in young children let alone toddlers.  A lot to take in...most of which I'm not sure how I remember.  He explained that he had more tests that needed to be run in order to determine what our best course of action would be for now.  At that point they took us back to see her.

The anesthesiologist explained that she had experienced a bronchospasm (a problem with the airway) while being intubated.  They had to treat this at the start of the procedure which is what took so long.  Poor thing was out of it but happy to be with us.  We took her home for some good cuddling and lots of napping while awaiting the call to schedule the next test to determine how poorly her stomach was actually working and emptying anything that actually entered it.  Unfortunately, this was only the beginning of tons of tests, procedures, doctors visits, etc. that would take us through the next several months.

How it all Began Part II

We were thrilled to be able to meet Dr. M (our first hero in this entire process.)  He came recommended from several places.  He just so happens to practice as a general pediatrician but specializes in GI issues.  At our first visit we spent well over an hour going over every bit of Charlie's medical history.  He seemed concerned by what we were describing and ultimately sent us for a battery of tests.  At this point we were convinced Charlie had Celiac Disease.  She seemed to have the symptoms, possible familial link, etc.  We half held out hope that it could be that simple and half hoped that it was something else because the thought of getting all gluten out of the house seemed a bit daunting, although great for the waistline.   

We immediately went downstairs to the blood lab.  It took three adults holding her down to be able to get all of the blood that they needed.  I swear it looked like it was three times what they took from me in the prenatal blood work (a freaking TON.) The phlebotomists had also decided that there were so many tests being done and so much blood that was needed that they could not safely do it all in one day.  GREAT!!!  So, then we waited.  Most results came back normal, with the exception of the fact that she was anemic and that she was lacking in some other areas...signals of poor liver function.  We were still waiting on that Celiac Panel, certain that was it.  Two weeks later, and after several calls to find out if it had come in we found out it was negative.  This was honestly the first big blow.  What could it be??? 

Dr. M called and explained that all appeared "fairly" normal with the exception of those few results, from the blood work.  We would potentially not be able to come up with a GI issue and that we may need to move on to an endocrinologist.  He did want to order one last test to explore further in order to make sure that we were looking at everything.

We quickly got an appointment and took Charlie in for an Upper GI and small bowel follow through.  The whole way there we were talking and wondering how on Earth they would get a child who doesn't eat, to drink barium!!!!

Yet, they did.  The radiologist asked for me to go and Judson to stay (something we continually find...mom leaves for x-ray, dad stays behind.)   She tipped up the cup and literally pounded two sippy cups full of barium.  Judson first helped strap her down to the table and then went behind the screen to watch the images tick by.  A test that was supposed to take several hours barely lasted 15 minutes.

All that barium that she drank just pooled in her stomach.  You could see it distend from the outside, you could see her reflux on the screen and most importantly you could see that NOTHING was draining out of her stomach and into her intestines.  It looked like there was a plug in there.  For 15 long minutes not a drop.  They tilted and turned the table in every direction to let gravity do its work only to eventually see an itty bitty trickle!!!  The radiologist stopped the test, there was no need to go further, they couldn't go further.  We were beginning to have some answers.

How it all began...

We know so many people care and are curious about what has been happening that we decided to start this blog as a way to keep everyone in the loop.  

About three weeks after Charlie was born we were becoming concerned with the MASSIVE amounts of spit up that would literally fly across the room.  She was seemingly happy one minute and then there would be projectile vomit the next.  After enduring this for a week I succumbed and called the pediatrician who had us come right on over to take a look.  Upon examination the first thought was that she had a condition called "pyloric stenosis" or a constriction on the pylorus (where the stomach empties in to the intestine).  She sent us for an emergency ultrasound and prepared us for what would most likely be imminent surgery.  Of course, there was nothing.  The pediatrician chalked it up to a virus.  Weeks later and still having intermittent problems, Charlie was finally diagnosed with reflux.  She slept in a nap nanny, was put on baby Zantac and seemed to be doing much better.  However, she slept for very long periods of time, never seemed hungry and when she did eat it was for incredibly short periods of time....what little baby eats for three to five minutes and doesn't want to eat again until forced 5-6 hours later?  I guess I should have known then that something was wrong.

Needless to say, Charlie never put on much weight.  Although she was born in the 75th percentile and maintained that for the first 6 months of life she began to fall off the growth charts once solid food was introduced.  Once I started weaning her off of breast milk the weight gain slowed to a virtual stop.  As soon as she was on all solids and cows milk she stopped gaining weight all together and fell off the charts, she stopped growing in height as well.  Our baby who was once in the 75th percentile was now considered "failure to thrive."

Not only that, she also began having violent vomiting episodes. Sometimes they would last several days.   Quite often the food was completely undigested from meals eaten HOURS beforehand.  We also started noticing that she had an extremely distended stomach.  She literally looked like she was nine months pregnant with a rock hard belly every time she ate.

Armed with this information, I asked the pediatrician for advice.  Her initial thoughts were that she must have a milk protein allergy so she sent us to the most expensive allergist in town (the one who of course does not accept insurance).  After throwing away almost $1000 on allergy testing I was told that Charlie was allergic to virtually every food any human could possibly consume.  She needed to steer clear of dairy, eggs, wheat, soy, chicken, turkey, fish, berries, tomato, oats, etc.  So, here we have a kid who can't gain weight, yet we can't feed her anything!!!  I bought into it VERY briefly until I decided this was not the answer...something else was wrong and from there we took the advice of everyone else around us (and not that of our pediatrician) and found a pediatric gastroenterologist.

This is where the true journey began!!!!