Diagnoses

I have been meaning to update for over a week and somehow the hours keep ticking away and there just hasn't been a moment.  We are all incredibly exhausted and still trying to recover from the hospital.  That being said, I wanted to fill everyone in on what they found while we were there.

As I had mentioned previously, one of the biggest things that Dr. P had brought up and wanted to look into was the colon function.  Oftentimes if the colon is full it will send signals to the stomach that there is no more room and to therefore not empty any more contents, thus delaying the gastric emptying process.  This is something that no other doctor had either mentioned or looked into before.  The first thing Dr. P wanted to do with Charlie was to clean out her colon by starting a regime of laxatives, which I had mentioned previously.  It has taken several months to find the appropriate dosage for her, the one that gets her bowels moving consistently since she is chronically constipated as a result of all that is going on with her.

When scheduling this last round of tests it was incredibly important that we have her at the right dose and that she was "going" regularly (although forced with laxatives).  It made for, as she said, "the best circumstances instead of the worst."

With all that being said, everything that we had originally thought to be wrong with Charlie we have now to come find out is not quite the case.  Her original diagnoses of Gastroparesis is not what is actually wrong.  Each test she had that definitively proved that she had Gastroparesis was a result of her colon not functioning properly and being too full, therefore sending those signals to the stomach to stop emptying.  What she does have is called "Neuropathic Small Bowel Dysmotility."  It is an incredibly rare motility disorder, especially in children, and one frankly I still don't fully understand.  What I do know is that it is quite the opposite of Gastroparesis but with many of the same symptoms.

In a healthy individual with normal motility the Small Bowel (which is your small intestine) goes through three different phases.  The first phase is the resting phase, which occurs between meals/digestion.  The second phase is the mixing phase where the food is churned up so that it can be digested and pushed down towards the large intestine.  Lastly, there is the recovery/housekeeping phase where remnants are pushed through along with any added secretions.  Charlie's intestines only go through one phase, which is the second phase.  There is never any rest and never any recovery.  In addition, the contractions that she does have in that second phase are arrhythmic and incredibly frequent and strong.  This is true for the contractions in the stomach as well (strong and frequent.)  Therefore, Charlie's small bowel (intestine) is extremely hyperactive, irritable and incredibly inefficient.

For the time being we now need to change the course of treatment.  We have taken her off of the Erythromycin as that was incredibly counterproductive.  It was forcing stomach contractions in a child who was already having forceful contractions.  We have now been prescribed some new medication to try which will work at hopefully slowing down the signals from the nerves and therefore slow down the contractions.  We have decided to give her body a bit of time to bounce back on its own from that last medicine.  She has never had a chance to solely be on the Miralax and we want to know what kind of an effect that will have first.  We will reevaluate her in a few weeks and determine when we need to start her on the new meds.  It is most likely not going to be a matter of "if" but a matter or "when."  Unfortunately, this diagnoses can be a very painful one for Charlie.  The strength of the contractions and the fact that there is no rest is most likely why Charlie feels pain when she eats, and therefore does not want to eat.

The last component to all of this is that the belief now is that the true underlying cause of all of this DOES lie in her colon.  She most likely has a hyperactive stomach and small bowel because she has a hypoactive colon.   For the time being she can be managed on the Miralax and then she could potentially need to go back and have the colonic version of this test repeated in the next year or two to see if they can determine what the underlying cause is in all of this.  Could there be something there that is bigger?  Or something that could be fixed, etc.?

So, there you have it.  We now have a solid diagnoses.  Different from where we started, not sure if it is better or worse, but it is a real answer and we can stop looking and work on moving forward and making her comfortable.  This has been an incredibly exhausting week beyond words, but at the end of the day we can thank our lucky stars that we went through all of this to get to where we are.  How we got lucky enough to find this amazing doctor in our own backyard we will never know.  Our fingers are crossed that now that we know what is truly going on we can stabilize her eating situation, help her put a little weight on, grow a little and help her to stay comfortable.

The Rest of the Stay

We are finally home, trying to get some rest.  The last two days at the hospital were definitely better than the first.  It was hard to keep Charlie in bed for the test.  She was still groggy from the anesthetic and hating the fact that she had a tube in her nose and IV's all over her hands.  Overall, I think the pictures can tell how the last two days there unfolded better than I can....

The nurse came in on Thursday morning to connect the sensors and begin the test.  They also ordered an Xray to check again that the sensors were still in place.  Xray came to the room, did it on the spot and we began.  

During the test Charlie got to play doctor with Mr. Potato Head when Grant came by to visit.

Then she got to check on daddy's heart beat.  After all, she had her vital signs checked so often she should have a turn to do it herself.

During the manometry testing (and throughout our stay), she watched the "soothing sounds" video of the underwater scenes which included activity on a coral reef, fish swimming by, dolphins playing, etc. on REPEAT!!! It was hysterical.  She would yell for the "pishies" as soon as it would end so we could start it over.  Thank goodness for this kind of television.  Maybe she has a future as a scuba diver.

Forty-two hours after her last meal she was finally able to eat.  She was actually excited! We ordered her some macaroni and cheese, a milkshake and vanilla pudding.  It was needed for the testing.  She had 20 minutes to get down 400 calories (which is why they had to order calorie rich food.)  She made it in the knick of time.

We even set up her baby doll (who was also equipped with a tube coming out of her nose, an arm splint to cover up all of the IV ports and a pulse ox monitor on her toe) to eat with her.  She fed her some of the meal as well.  It got messy!

 In order to keep her still we let her do some dot art....

and play with her doctor bunny.  By late afternoon the test was over and we got to take the nose tubes out.  We asked that they unhook her IV's for a few hours so we could let her get out and about.  The hospital has a little playground for the patients so we took her outside to play.

 She only lasted a short time but she did love the slide.

 She also loved just being able to walk in the halls not attached to any other devices.

The next morning we had to wake her up and start all over.  They loaded her in a wagon to take her back to Radiology.  She loved rolling by this animal mural in one of the hallways.

This burrito wrap was used to hold her down, which she definitely did not enjoy.  She complained of being hot.  She could not move for several hours.  She did get to watch Elmo, however.  She eventually gave up the fight and fell asleep on the table.

 When she was all done we got to wheel her back up to the room.

Once we got back, her doctor came back by to check her out again.  She was not entirely thrilled that her fish video got interrupted but she still let her check her out.

Before they would let us go home they needed to draw blood to test for some different things, give her a flu shot and remove all of her IV's.  As you can see, she was NOT happy.  

We did get to meet with the doctor and speak extensively about what they found.  We can home from the hospital late yesterday and are now trying to recover from an incredibly emotionally and physically exhausting experience.  Hopefully, this weekend will be filled with lots of rest and sleep for ALL of us.  

Yesterday

We had our pre op appointment for Charlie on Tuesday and it was DISASTROUS!!!  We showed up and they sent us to surgical admitting to have a routine appointment (check vitals, listen to lungs, go over medical history, blood work.)  We met with the nurse first and then we waited FOREVER for a doctor to come in!!!  Finally someone came in and informed us that they had sent us to the wrong place and that we should have gone to the radiology department for the appointment since that is where the procedure was being done.  We needed to head over there and start all over.  While walking over there someone came running after us in the halls yelling at us to stop.  When we did she said there was another mistake.  They actually needed to admit us then!!! She said that there was a mistake in the system and that we were told about the appointment incorrectly.  This was actually the time for us to be admitted and not a pre-op.  We both panicked.  I needed to be back at work and so did Judson.  We needed to get Levi from school, finish packing, etc.  She took us to radiology to find out what was going on and they confirmed the plan.  I frantically called my mom to help.  Then they took us back to the admitting department to call the GI floor to get the bed ready and go over all of the instructions.  There was a lot of back and forth, explanations, etc.  Then they decided to take us back to radiology to go over everything and discuss the next steps and admit us.  When we walked in a lady said, "So today we are going to do your pre-op and then you can come back at 6:00 tomorrow morning." While this is going on a Jonas brothers look alike came in to the waiting room with his guitar to sing "Twinkle Twinkle"  Seriously, straight out of a movie.  If you are having a hard time following, welcome to our world, it was seriously that crazy and hectic!  At that point we were royally confused and pissed off.  No one knew what anyone else was doing.  We called the GI floor ourselves and got on the phone.  They confirmed that they decided we could go home but we needed to be back in the morning.  So, we went with it in hopes that it was actually the right thing to do.  We went ahead and did the pre-op and the blood draw.  She didn't even flinch.  Instead, each time they took a vile she said, "All done!"  Judson and I both left as fast as we could to get back to work so we could wrap up before checking in yesterday......

Yesterday was THE MOST TRAUMATIC yet!  We arrived at the hospital at 6:00 ready to go.  Charlie was in good spirits.  We went to admitting where they handed us the paperwork for the wrong child.  When we informed them that we were here for Charlie and not Tanya they straightened it out and sent us over to radiology to prep Charlie for her procedure.  It was a normal pre-op situation.  Sit and wait.  Vital signs, etc.  Doctors came in to explain everything, sign consent forms, etc.  Eventually it was time and they came to get us. Now let me preface this next part by saying that Charlie has been under general anesthesia several times before.  Each time they have given her a little something to calm her and they would take her from us after saying our goodbyes.  This time they had us take her to the OR.  It was just as you would picture, large sterile room, monitors everywhere, cold, people buzzing everywhere and she was instantly freaked out.  She was clutching me for dear life.  We were trying to calm her down until at one point the anesthesiologist asked me to lie her down.  She was pissed.  Flailing about and screaming.  They put a mask over her face and within seconds she was gone.  I'm not sure what I was thinking they were doing, but I was not mentally prepared to see her being put under.  It was hard to see the eyes rolling back and her gone.  We didn't get to kiss her goodbye and reassure her, etc.  We were asked to leave so they could intubate her, etc. and they would call us when they were done.  So, it began! 

We were called about an hour later to come to the recovery room.  When we got to the door you could hear this blood curdling scream.  She was beyond pissed.  The tubes they had placed were making her miserable, she was in pain.  It was rough.  She kept looking down at her hand and all of her IV's and would start to cry all over again.  At one point about an hour in she looked down at her hand and muttered her first words, "OH NO!"  We couldn't help but laugh.  

Eventually, they moved us up to our room and hooked up all of the sensors to start the testing.  It was pretty evident from the start that there was a problem.  They called in the doctor to come and take a look.  Of the 8 sensors placed from the esophagus down through the intestines, five were not reading.  They called for an X-ray to look at the placement (yay, for more radiation!)  At one point they thought they had it working and let it run for an hour so.  The doctor came back up to check and decided that they were indeed not working.  They needed to send us back down to the OR to have Charlie go back through the procedure again.  The sensors were not set right and they needed to be advanced down through her intestines further.  This would be her second general anesthetic of the day and she hadn't fully woken up from the first one.

They called down to prep the room, called for transport and took us right away.  We got down there and took us back in.  This time I was better prepared to be there when they put her out.  They told me how long it would be until she was unconscious, and then she was out.  This time the recovery room called the second they wheeled her out so we could be there when she opened her eyes.  It made for a much better recovery.  When she woke up they took us back to the room and did another XRAY to confirm everything was in place, which it was.  They then hooked her up to the machines to make sure the sensors were reading.  At this point it was too late to run the tests so we needed to wait to start until today.  It was about 5:00 and she had not had anything to eat or drink since dinner time on Tuesday night.  They decided to start IV fluids to prevent dehydration and run them through the night.  She would only be able to have clear liquids (but only water and juice) so that the tubes and sensors did not get disrupted or displaced again.  

Charlie was rather uncomfortable for the good part of the day yesterday and night last night.  She HATES the tube in her nose and is incredible lethargic.  She never made an attempt to go anywhere, move about her bed, get up, etc..  We sat her up briefly and that was really it.  It was very sad.  Crossing our fingers that today is a better day.  At this point, we are waiting for them to come in and get started for the day.  We will definitely be here for an extra night, which is a bummer on so many levels.  Another night being away from Levi, Judson is now having to miss his most important night of work for the year, and we are emotionally and physically exhausted.  Hoping to be home tomorrow evening! Another update will come soon.

The Last Six Weeks

My goal when initially sitting down and starting this blog was to get everyone up to date on what has been happening with Charlie.  We are so often asked what has been happening, what is new, what are the next steps and how is she doing?  Oftentimes, it is hard to answer without the entire backstory.  Judson and I decided that writing this blog together would be the best way to inform everyone.  Although I began writing a month ago, the story really began when our "fishing expedition" began back in the beginning of April.  We knew then that THIS week was going to be a big week and it was my goal to have the blog up to date by now.  So, this post will be the last to fill everyone in on past events and from here on out it will all be current.  That being said, the last six weeks has been quite the roller coaster.  Something, we have come to find is quite typical when you have a baby with Gastroparesis.

The real problems began when she caught a cold which developed into what the pediatrician believed to be Viral Croup.  Now, ordinarily I would not have thought much about the Croup (granted, certainly not fun...Levi had it and we panicked) but we had been there and done that and knew what to do.  The problem was that Charlie who ordinarily does not want to eat or drink took that to a whole new level now that she did not feel well.  What we found was that we could barely get even the slightest bit of anything down her.  We were lucky to get her to drink three ounces in a 24 hour period.  We were on the verge of going to the ER on several occasions as she was getting dehydrated.  Just as we were making the call she would take a few sips, buying a few hours.  This went on for DAYS!!! The doctor told us that this was a sign of things to come: whenever she gets sick we will always need to be careful.  We also needed to stop the Miralax which threw everything regarding her pooping situation way off track.  The doctor mentioned that a low dose of antibiotics would normally be given to help the situation.  The silver lining to it all was that we didn't have to put her on any antibiotics since she is already on them daily :)

We did, however, make it through and did so with flying colors I might add.  Once she was better she actually began eating like a champ.  Like nothing we had ever seen her do before.  She had been eating two to three times what she would normally eat.  It was quite a severe turnaround.  We got the Miralax up and running and found the right dosage and dare I say, everything was as good as it has ever been.

Two weeks later we went in for our next appointment with Dr. P, the motility specialist at CHLA.  Charlie had gained an entire pound since the last appointment.  I must say that we were in shock!  It was awesome.  She explained that oftentimes after being sick and dehydrated that these little bodies can respond in just this way, putting on this large amount of weight.  She confirmed that the whole "disease" is very much a roller coaster and that it could change and turn at any moment and to not necessarily expect the weight to stay on or the eating habits to stick around for long.

The rest of the appointment was spent discussing our upcoming manometry testing.  This Wednesday, Charlie will be admitted to CHLA for a very specialized motility test.  They will use cameras, sensors, etc. to watch Charlie's stomach and upper parts of her small intestine from the inside.  Our hope is that we will find out more about what is going on.  Is she taking the right medication, dosage, could there be another underlying cause, etc?  They will also repeat the Gastric Emptying Scan as an inpatient, we will meet with a nutritionist and HOPEFULLY leave with a lot more answers.  Unfortunately, Charlie will need to go under another general anesthetic while placing all of the tubes, lines, cameras, etc. which will then run up and out through her nose (yep, should definitely be fun trying to manage that situation.)  To add insult to injury we will need to keep an incredibly active 21 month old little girl as motionless as possible for the duration of this test. This becomes increasingly risky because of the previous bronchospasm.  Our fingers are crossed that this one will be smooth sailing.  If all goes well, our hope is that we will only need to be admitted for two days.  If however, they need to collect additional data or there is another problem that arises that could get extended.  We will be updating everyone on the blog throughout our stay!!!!

Firestorm

One new task that we were given at that last appointment was to document every bit of food that Charlie put into her mouth.  We were also to log every time she went to the bathroom, including the details...definitely a fun job!  This would help us to truly count calories and recognize a pattern between the pooping situation and food intake.  If we did not notice a change we would need to increase the amount of laxatives we were giving her.  We were leaving shortly to head out to Idaho and her appetite had never really "returned."

While in Idaho her appetite continued to deteriorate.  She ate so little we found ourselves in the pharmacy for the first time needing to buy Pediasure to supplement and ensure that she was getting SOME calories each day.  We also decided it was time to increase her laxatives.  As many of you have heard by now our trip coincided with the wildfires in Sun Valley.  Not only did the fires burn to within yards of the house but we were there with the kids.  After a long night of evacuations and drama we found ourselves in a diner for breakfast awaiting our flight home.  Charlie SCARFED down an entire pancake.  Everyone at the table had their mouths on the floor.  It was like she couldn't eat fast enough.  And then, within moments of finishing she started hitting her stomaching and yelling "OW, OW!"  Well, we did it!  We let her overeat.  A portion that any toddler would have no problem with was incredibly painful, and for the first time she was verbalizing the pain she was feeling.  We tried to get home on a flight that day but due to the fires and smoke we had to drive....For 15+ hours!!!!! Charlie was still sick and in pain and wouldn't eat, we ran out of her medicine because we should have been home and the poor thing moaned the entire drive.  Poor baby!!!

After getting home, it was time to call the doctor.  We were not seeing the desired results from the Miralax.  She wasn't eating and we were getting nervous about her overall caloric intake and what her output "looked like."  They called back and ordered an abdominal X-ray to be performed at the next appointment.

We took her in and they performed the X-ray.  It was incredibly sad.  Charlie is beginning to dread the entrance to the hospital.  She knows what a person in a white coat means and is fighting back when we need to strap her down.  Every wiggle means more radiation.  The X-ray ended up providing loads of information, however.

They found that on twice the normal daily dose of Miralax her bowels are still completely backed up.  They needed to once again change her dosage for the following month.  She was able to gain back the weight she had lost the previous month but nothing more.  The doctor was very impressed, if I don't say so myself, with my excel spreadsheets and would be sharing them with the nutritionist who she also wanted us to meet with.  There was definitely a correlation between her caloric intake and her output.  For the first time the possible need for a feeding tube was brought up.  Charlie has gained no weight over all in over three months and if we can not get her to gain several pounds by the end of spring then we are looking at a feeding tube.  Just having to have that conversation was rather difficult.

From this point we left awaiting the call to schedule her third gastric emptying scan and the manometry testing.  I was to keep at my excel spreadsheets and work hard at making sure that the pooping situation was controlled and consistent (hard work for sure.)  September was upon us, we were back to work and to school and life was moving ahead quickly....

July kicked our butts!

We now had a plan for Charlie.  Her 18 month check up was in the next few days and we were able to update Dr. M on what happened at Children's Hospital.  Besides these issues everything else looked good.  She got her shots and we were on our way.  He told us that she may possibly have a fever in the next few days as a result of the vaccines, etc. but nothing to worry about.  The following week we were headed to Georgia to visit Judson's family.  Two days before leaving she developed a decent fever.  She seemed as though she wasn't feeling 100% but there were no real symptoms.  We chalked it up to the shots and were not entirely concerned.  The fever broke and she appeared fine.  We had a playdate before we left and I saved packing until the last possible second.  With laundry and kids being awake I just never got to it.  If you know me, you know this is so unlike me!  I woke up the morning of our flight and went in to get her up.  I was limited on time, Judson was at work, I still needed to pack for the kids, do dishes, laundry, take care of all the stuff for the dogs, etc.  I walked in her room to find her covered in the craziest rash you have ever seen.  Would they even let me fly with her?  I called the doctor's office to see what we should do.  They paged him and we waited.  I finally called back and they said that we could be seen in a half hour.  Funny, because we live in the valley and the office is in Santa Monica.  It was 9:00 and I needed to get two kids who were in PJs out the door, in the car and to Santa Monica in morning rush hour on the 405 in 30 minutes.  If I didn't make it I would have to wait over an hour for his next opening and risk not making it back here in enough time to meet Judson to make it to the airport for our flight.  I think the traffic Gods were with me that morning.  I bribed Levi, got the kids in the car and made it.  My mom happened to be in Santa Monica and she met me in the parking lot and helped me unload the kids.  We ran in and made it just in time.

He tested her for all kinds of things and then looked at her chart and did the math.  She was having a rare reaction to the MMR shot.  The shot was a measles like rash.  Nothing contagious or harmful just uncomfortable for her.  Because her immune system is a bit more compromised from the gastroparesis she was more prone to this.  We ran home, I threw clothes in suitcases and we took off.  We made it to the airport and then became THAT family on the plane.  Charlie was out of control, screaming the entire flight.  It was our own personal hell!

She was out of sorts for several days, not eating, drinking, etc.  When the rash was finally gone the gastroparesis symptoms where there in full force.  One thing we have found is that they really come in waves.  This was a particularly bad stretch for her.  We had a hard time managing everything while on vacation.  Judson's sister who is in college was our superhero.  She dealt with more than any 21 year old should.  In the matter of 24 hours she was thrown up on and pooped on and she took it like a champ.  Super Aunt for sure!!!

By the time we had made it back to LA Charlie had a pretty difficulty month and it was time to see the doctor again.  She had lost a half pound over the course of the month.  Not good by any means, actually rather concerning to us AND the doctor.  The miralax was difficult to manage and we were not getting the desired results. At this point we were going backwards.  The eating was awful, weight gain was negative and colon function was clearly very poor.  The doctor was going to be ordering some new tests to look in to this.  One of which we had done before (twice before), the Gastric Emptying Scan.  She wants to see what the change is once we have her on the right dose of the Miralax and the second will require a hospital stay.  It is a more invasive test that will take a larger more in depth look at what is going on inside (more to come on that.)

We left that appointment (in the beginning of August) feeling like we are now back to amping things up again.  Our quiet and calm times were over, back to frequent appointments and visits, tests, and now a hospital stay in the near future.

CHLA

July rolled around and I have to admit we were super excited for our appointment.  Kind of nervous to find out if we were actually barking up the right tree, doing the right things or was this all a bunch of hoopla for nothing and that she was actually fine.  We left plenty of time to get down there and met my cousin in the cafeteria for lunch.  He walked us up the the GI department and as we signed in they called us back.  They weighed Charlie, measured her, etc. and then took us back to our room.  Not a shock, but she still was not gaining weight.  The doctor came back virtually instantly.  Let's just say Charlie was not on her best behavior that day.  Lots of screaming and wiggling and paper ripping ensued.

We were able to go over the entire story.  Everything from birth until then.  Of course, we had all of the records from St. Johns but nothing from UCLA was ever sent even though we had requested them twice and even called to check on them.  We were able to tell her the results we knew and understood.  We talked about what we noticed, symptoms, etc.

She then mentioned something we had never heard before which was that oftentimes people who suffer from one type of motility disorder have problems with other functions.  Therefore, it would be likely that Charlie is actually having problems with her intestines and colon as well as her stomach.  We focused largely on her colon function at this appointment.  She explained that if the colon is not working properly and emptying as it should it can not send the proper signals to the stomach to empty starting a vicious cycle.  This only leads to increased slowing and delayed emptying.  If we could manage her colon function it could potentially increase the amount of food that could leave her stomach.  So, we needed to fix some of the issues in her colon, which at this appointment we learned were actually issues, something we did not previously realize.  We were to put her on an adult dosage of Miralax and monitor her closely.  We would be reordering all records from UCLA and meet back in a month to go over the progress we had made while on the Miralax.  Dr. P made no hesitation in completely agreeing with the other doctors.  Charlie DEFINTELY has gastroparesis.  We left feeling anxious, relieved and definitely overwhelmed.  The diagnoses was finally confirmed by someone who knows what they are doing.

We finally feel like we are in the right hands and with one of the few experts in this field.  Someone who could actually explain what is happening.  At the same time she had to tell us that this is incredibly rare, there is not much information out there about it, etc.  We don't know what life will be like for her.  Will it ebb and flow, get better, worse, etc.?  At that point,
we had a plan of action for the short term and were looking forward to an upcoming trip and hopefully some results from the new meds.

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!!!!