Hospital: Day 1

Our original plan was to wake up Tuesday morning, take Levi downtown for his monthly appointment at Children's (which just so happened to be scheduled for that same morning), bring him home, then turn around and take Charlie back to be admitted.  Now everything was off.  I was taking the morning off of work since I already had a sub and was going to take Levi to his appointment.  Judson was feeding Charlie a large breakfast "just in case" and then going to take her to school.  While heading in for the appointment the office manager called with the quote.  Apparently the cost of the test was only $253.  She asked if we wanted to go ahead with it, especially since they were able to get us back on the schedule.  I laughed out loud and said, "For $253?  Um, yeah!"  She then gave me the details, which were that we couldn't be admitted that day and that it would be pushed back to Wednesday.  The doctor would go over everything when I came in with Levi.  I quickly hung up, called Judson, etc.  We were on!

Meanwhile, I had apparently missed another call from the hospital in my mad phone call making.  It was the doctor asking us to bring Charlie in right away.  Of course I was already half way there, in rush hour traffic, and was barely going to be able to make her appointment.  I called Judson back and had him quickly put Charlie in the car to race down there.   The doctor came in to see Levi immediately so Judson was unable to make it in time.  To be honest, I don't even know what we discussed about Levi, it all happened so fast!  She went over what would happen with Charlie over the next few days which was not going to be pleasant.  Insurance was still denying two nights of her hospital stay so they were going to have to do part of the procedure as an outpatient.  We were bringing her in to place the NG tube so that we could take her home and administer the medication for the next day ourselves.

When Charlie and Judson got there the nurse took ALL of us back (including Levi).  Although ideally we would never want Levi to be there for this there was no other option.  I spoke with him about what he was going to see and got out the iPad and turned it up as loud as we could to distract him.  I assured him that Charlie was not going to be in pain that she was just crying because she was a bit scared.  I think he was more curious than anything.  It took several of us to hold her down while they placed the NG tube down her nose and into her stomach.  We then got instructions for how to pull and test stomach juices, flush the tube, and administer the medication, all of which needed to be done every hour until she could be admitted the next morning.

We left there terrified and feeling like maybe we were making the wrong decision.  Before we walked out we checked on how much it would cost to pay out of pocket to stay the night.  We decided to save the thousands of dollars and try to make it work.  Every hour when we would sit there through the thirty minute process, holding her while she was screaming in fear and pain, we would discuss that if we could make it maybe we could use the money that we were "saving" for a vacation.  Luckily, my stepdad and mom came over and played with Levi, helped to hold Charlie, ordered dinner, etc.

Our first major set back came when the medicine was stopping in her stomach and causing it to be distended.  It wasn't going anywhere and she was rock hard.  She also was not "emptying" which was the whole point of this process.  We called the nurses who had us take a break from the meds for an hour which provided some relief.  That night was one of the worst night's "sleep" in a while.  We were all exhausted by the morning.  Charlie hadn't eaten anything solid for 24 hours and she was definitely not feeling well.  Now we got to sit around, continue the meds through the NG tube, and wait for the phone call to bring her in to be admitted.

Insurance IS a Four Letter Word!

Levi went in the next day for his MRI and he was a champ!  Fortunately, it was normal and we proceeded on with his meds as we had been.  About two weeks later I hadn't heard anything about scheduling the hospitalization/testing for Charlie so I called to check in and found out that the orders had not been put through yet.  They said it would be done right away and they would get back to us to confirm.  I heard from them later a few days later and they told me to expect a date in mid September.  Our next appointment for both kids was in mid August.  Charlie had been doing well on this new regimen of meds which was promising and Levi was having a few setbacks which meant that we were needing to cut back on his meds and do some clean outs for him.  Never a dull moment!  We made appointments to return, hoping that the next time Charlie would be in would be for the testing.

On one of my first days back at work from summer we finally got the call.  Charlie would be admitted on September 16th.  She would be in the hospital for two days prior to the procedure in order to place an NG tube and do a  complete clean out.  The procedure/test would take place on Thursday the 18th and then we would most likely get to go home on Friday the 19th.  Once we knew the dates we started making arrangements (subs for school, a place for Levi to stay, pick up and drop off for Levi, taking the week off, the dogs, etc.)  There was so much that went into preparing for that week both mentally and physically.  Then the proverbially, "shit hit the fan!"

The Friday before she was supposed to be admitted (the 12th) we got a call from the hospital saying the insurance company had denied the claim through the pre approval process.  What was so maddening about this was that the only thing approved was the colonoscopy part of the test, which was simply an "add on."  It was not the primary test, nor the main reason we were there.  It was used to help guide in the sensors and put them in place but to also look at the anatomy while there was the chance.  This was not the test that we really needed!  The hospital wanted to let us know what steps they were taking to have the decision reversed.  I asked if I should start panicking and they said not to yet, but that if it didn't eventually get approved we would need to cancel.  Judson began repeatedly calling the insurance company to fight the decision although ultimately we found out that,  of course, we would not have an answer that day and would need to wait until Monday....the day before admission!  I was beside myself.  I was in tears.  We waited months to get to the point where we could get answers and now the insurance company was deciding they just didn't think it was necessary.

So, now we needed to wait for the doctor to make her appeal.  They were setting up a peer review to be completed on Monday.  A doctor from the insurance company and our doctor would make an appointment to speak with each other so that Charlie's doctor could explain why this test was indeed incredibly necessary.  I was still, at this point hopeful but could barely function at the thought of it not working out.  I lost it at preschool pick up and could barely get through the weekend.

Monday morning rolled around and Judson began calling the insurance company.  They said that if they did not make contact with the doctor that day the case would become "delinquent."  The two doctors were stuck in a round of phone tag apparently for days and were left with only a matter of hours to connect.  I began quickly calling the doctor's office and was unable to get through.  Eventually, they called to inform me that the review was set up for 2:00 and we would be hearing shortly after.  To help ease my frustration at this point I asked how often these tests are approved after the peer review process.  The lady said that about 95% of the time they end up being approved, however she has seen them ultimately denied and canceled.  So, I hung up and we waited!

If you were anywhere near me at around 2:30 on Monday afternoon you know how this call went.  The  insurance company decided that the test was not only not medically unnecessary but that it was too experimental.  They were standing behind their initial denial.  The hospital was now informing me that the testing and hospital stay was going to be canceled.  I immediately broke down and wanted answers.  They told me that as the parent I could file an appeal, which honestly wasn't enough.  We had 12 hours at that point.  I asked to immediately speak to the doctor, who would call me back.  At which point, I hung up the phone and called the insurance company hysterically crying.  Once I got someone on the phone I insisted on answers.  The doctor beeped in and I had to click over (quite the whirlwind).  She explained the call, her frustration, I told her of mine, and asked what this meant going forward.  She said that we could keep appealing and hope to be able to do it later down the road.  If we were unable to ultimately go through with the test we could make decisions for her care based on the information that we have.  That, I am really not ok with, and I am not going to let the insurance company get away with!  I asked if anyone had ever paid out of pocket and she said that it had happened once or twice but usually people fight it and appeal and ultimately win, or the other option would be to go through with the test and appeal after the fact.

That got me thinking.  How much was this test?  Certainly not cheap, but what were we talking?  She made a comment about how the other parts were all covered, it was just this test that was denied.  So, I asked for the cost.  She said she would have the office manager call me immediately and we got off the phone.  I then called the insurance company back and "nicely" explained my deep sadness and frustration with what was happening while at the same time getting an explanation of what was covered.  Since the colonoscopy was covered so was the anesthetic, operating room, hospital stay, etc.  I left work at this point with an extremely puffy face telling them that I would see them the next day.  From there I called the office manager to see if a) we could get ourselves rescheduled since everything had already been canceled with the hospital and b) how much money we were talking. The office manager said she would get right back to me.   She called back to say that she could not get any answers for me right then, but that she was working on it.  She was trying to get us on the schedule and find a price.  She was leaving in two minutes for the day but would stay an extra few just in case she heard back from the other departments.  If I didn't hear back from her in the next few minutes I wouldn't hear until the next morning...and I didn't hear.  So, we proceeded as if everything was off.  Until the next morning!

When the Best Case Scenario Sucks!

Over the course of the next month the frequency at which Charlie had been pooping was decreasing.  It was becoming evident that this new regimen was not working either.  At best she was going to the bathroom every other day, or when given the stimulant suppositories, which was not ideal.  On the nights that we gave those to her (which was several times each week) we had to chase her around the house to catch her so we could hold her down.  We would then have to go through an agonizing hour or so while she cramped and then went to the bathroom.  It was miserable to watch and I am certain even more miserable for her.  She knew exactly when it was time and would start yelling an hour beforehand, "no pository!"  It was heartbreaking!

In mid July, we went back for our next appointment.  First, we checked in about Levi and all was good with the exception of the fact that he still had not gained an ounce since January.  We reported the differences that we had been seeing since starting the enzyme and she agreed that it had been working, which was awesome.  Now we just needed to work on putting weight on him.  We were tasked with making sure to up his daily fat intake.  It was vital that there was weight gain by the next appointment.  His MRI was scheduled for the next day and we would check in after that.

Next, it was time for Charlie.  My mom has been coming with us to the appointments so that she could stay in the waiting room with one kid while the other is seen and then we can switch them out.  There is nothing like trying to talk to the doctor while the two kiddos are "discussing" who gets to hold the iPad and watch Frozen.  When the doctor came in we told her about how the month had gone and that Charlie had been complaining that her tummy hurt more and more.  She frequently asks us for a bandaid to put on her stomach since it hurts so much.  I expressed frustration and sadness at the fact that no matter what we do she isn't feeling good and we don't seem to be making much progress.  The doctor checked her and while examining her Charlie immediately told her that it hurt.  It was clear that she could feel that Charlie was backing up again.  This is when, for the first time in over a year, she talked long term and laid it all out for us.  As this part of the conversation started we knew we had to get Charlie out of the room so we could listen.

What she said was that it was time to move on to do the next phase of testing that we were hoping to never have to get to.  Back in January she had told us that this was done for children like Charlie who are potentially left with only surgical options.  This test will look to see what, if any, function there is in her colon.  She stated that presently the oral laxatives are not working for Charlie and furthermore it has become psychologically damaging for her to take them (chasing her around the house, etc.)  There isn't much more they can give safely and effectively.  The next option and our best case scenario would be to do something called a Cecostomy.  This could be done if there is a good response to medication and some function during the test.  This would be done through placing a port into the top part of her colon so that we could manually flush her out and inject medication straight into the colon.  If we did this there would be a possibility that one day it could be removed.  If they were to determine that there is no function in the colon then they would need to do a colostomy and either remove the colon or reroute around it so that all waste products can be emptied.

It was then that our hearts dropped.  Judson asked if the test would give us all of the answers and she said that it would not but it would hopefully give us clearer expectations.  I think she could sense that we were "freaking out just a tad."  When we asked if this was really best case scenario she said that there was still a slight possibility that the bowel could be "immature" and have not really woken up yet.  It could one day, but to not get our hopes up.  Even if this was the case we may still need to do the Cecostomy for the meantime until the medication can be better managed orally.

So, there we were.... We left completely panicked.  The test, which requires a four day hospital stay would be scheduled and we would need to wait months to find out what we were looking at.  My mom was with us so of course we told her and the rest of my family and Judson told his family.  I had my book club that night and told them when they asked how everything was going and then I really didn't discuss it with anyone for weeks, with the exception of the most amazing friend that I met through a group with children who have similar issues.  About three weeks later I called my boss and told him that I needed to fill him in and he couldn't have been nicer.  Even then I told him that no one knew anything about it and that I wasn't ready to discuss it but that I wanted him to know so that we could plan ahead.  Eventually I began telling people and now it has become easier and easier to talk about.  The fear in the beginning of the unknown was so scary (still is.)  So, now here we are waiting for the hospital stay which will be here before we know it!

Another Round of Testing

That following week we took her in for the anorectal manomentry test.  This looked at the function in the rectum, etc.  She was a champ and the results were basically normal which was a big sigh of relief.  This told us now that the problems she is facing do indeed lie higher up in the colon.  The doctor again tweaked the medication and said that we would need to keep a very careful eye on her for the foreseeable future in order to avoid another impaction.  This would mean getting checked every other week for a while.  We would avoid further testing until the medication proved to be unsuccessful and we would need to look into other measures.

At our next appointment we took both kids so that Levi could start his clinical trial now that he has been diagnosed with Malabsorption of Sucrose.  While there, they took cheek swabs from both kids as they decided they wanted to look into whether or not there was a genetic connection between what was going on with both children.  She also ordered an MRI of Levi's spinal cord to look for potential nerve damage as a reason for the issues that he is having and ordered the same anorectal manometry test that Charlie had just had to see what his function was like.  At this same appointment the doctor decided that the Miralax was no longer working for Charlie and that we would take her off of it and just give her the suppositories and the mineral oil.  Hopefully we would get more production through that.  The plan was to check back again in two weeks with both kids.

About two weeks later we took Levi for the manometry testing.  It was quite a different experience than with Charlie.  Levi panicked when we took him into the room.  We had a Child Life Specialist with us who showed him pictures and brought toys, but it did nothing to lessen his anxiety.  It took four of us to hold him down in order to start the test.  He was terrified and uncomfortable and there was no amount of convincing him that it was going to be over quickly.  What we found in the test was that he had appropriate function but was confusing signals when it was time to go to the bathroom (e.g. would push out when he was trying to hold in and vice versa.)  We are hoping that with age and development this will get better.  In the meantime, we got the prescription for the enzyme he will now be taking when he eats in order to be able to absorb sucrose.  It is a lot of work but we noticed a difference right away.

At the same appointment we checked in about Charlie who had not been having the success we had been hoping for after cutting back on the Miralax.  We got new instructions for her, bringing the Miralax back into the regimen and would meet back soon to check on both of the kids....

The Clean Out

It' been a few months since the last update so I will pick up where I left off...

We started the big clean out on Easter morning.  She quickly started going to the bathroom and within jut a few hours it became apparent that it was not going to work.  Based on my limited knowledge and what I had known about how backed up she was, her poops cleared way too quickly!  By Monday she was miserable.  She wasn't allowed anything but clear liquids, she didn't feel good, and was having clear diarrhea constantly.  I called the doctor to check in once we had finished the regimen.  I explained my concerns that although she had been going frequently there was virtually no stool and that therefore it hadn't worked.  The nurse agreed and said that they would get back in touch about what to do next.  Within a few hours they called back and said the doctor had ordered an XRAY to be done immediately to confirm everyone's suspicions.  I grabbed Charlie from daycare and we quickly drove downtown to the hospital. Charlie knows what is up when we walk into the hospital and I was by myself.  She threw a complete fit.  For the first time, she wiggled so much during the XRAY they had to repeat it which means even more exposure to radiation.

The doctor called the next day and had reported what I instinctively had known.  The clean out did NOTHING!  There was no movement, and no change, and now the situation was dangerous and borderline emergency.  We spoke about whether or not to bring her in immediately to take her into an operating room to do a manual disimpaction or whether we wanted to wait what would be 48 hours for one last ditch effort with a new laxative.  We decided to wait, fingers crossed that this would work.  Charlie was to take 4 ounces of mineral oil a day for two days.  If she wouldn't drink it or we didn't get any results we were to call immediately and go in for the procedure.  Thank goodness for daycare and her wonderful teachers who tried everything under the sun and with a straw and a shot glass we got her to drink up!  Fortunately, she went enough to buy us some time and we avoided the manual disimpaction.  We kept on the mineral oil, the miralax, and the suppositories, so she would keep going.

We went in after the weekend and the doctor checked her out and confirmed that the meds finally did their job.  Now, it was finally time for the next big test in an attempt for some more answers.

Update Part 2

Fortunately, we already had an appointment scheduled with the doctor last Monday for Levi so it was easy to add her in and at a time that worked.

***As a side note since I know I briefly mentioned it a few months back, Levi has been having some GI issues, really since birth but that we have become aware of as we have gained more and more knowledge through the process with Charlie.  In having a conversation with the doctor at one point, we found out that there is a strong genetic component just as there is with something like asthma.  Therefore, if you have one child with motility issues there is a HIGH likelihood that any other children would as well. (No there are no other children!)  Instead of really starting anywhere else we took him straight to her.  She was able to pinpoint his condition fairly quickly and changed his diet in order to confirm her thinking.  This last appointment he was diagnosed with Malabsorption of Sucrose and Fecal Retention.  Something that is also extremely rare.  There is an enzyme that is believed to help with this and they want to start him on it.  There is a researcher at the hospital that has been waiting for a patient with this exact diagnoses so that they can study the condition and the effects of the enzyme.  For the past few months Levi has been on a strict diet that has not allowed him to have any fruit and a VERY limited amount of vegetables.  We can now start those back up in anticipation of this clinical trial and the work with the researcher.  Fingers crossed!***

 

His first piece of fruit in months!

Told the doctor that he couldn't wait to tell his teachers :)

Back to Charlie....I begged my mom to come so that we could focus on talking to the doctor not switching "Frozen" and "Planes" on for the kids every few seconds.  The doctor spent WELL over an hour with us just talking about Charlie.  She showed us the XRAY from Wednesday where she was backed up as high as you could see.  The return of symptoms and the fact that the Miralax wasn't doing what was anticipated was concerning to all in the room.  The hope with the Miralax is that it would break everything up in the colon and increase the frequency or amount that she was going, and it was doing neither.  She mentioned that the course of three capfuls over the weekend probably cleared her out significantly and that now she needed to start her on a stimulant laxative (something we had previously been trying to avoid.)  She had a new regimen of two capfuls of Miralax and a dose of Senna (Ex Lax) each day.  She also wanted to order new testing to be done immediately to look into what was causing this.  We have always known that Charlie's underlying issues were lower down in her GI tract but before now she was managed well (or at least we thought she was) on the Miralax.  Now that she is not, we are forced into getting a true diagnoses all the way down as it could be an incredibly different issue than what is happening in her stomach and small intestine.  After we were in the hospital in October the doctor had mentioned not doing any tests while she was being managed and now we are at the place where it has become completely necessary.  Yuck!

This first test is the same test that was performed when she was in the hospital in October, however it is in the very bottom part of the GI tract...as low as you can go.  It will check the function of the rectum.  Fortunately, it can be done as an outpatient.  We also discussed doing the colonic version of the test, but holding off until this one is complete and until she can gather a bit more information and try a few more medications since that one will require a longer hospital stay than the first.  She then sent us down for a final Xray to check to make sure the three capfuls cleaned her out and we were on our way.  We planned to check in at the end of the week with an email about how the new medication was going and with pictures.  Yep, pictures!

Overall, it was an incredibly discouraging appointment but we left with a plan.  I called the office the next morning and made an appointment for the test.  It would have been in two weeks....BUT...On Thursday as I was pulling out of the garage at school my phone rang.  It was the doctor wanting to discuss the results from her Xray on Monday (I will admit that I never thought she would be calling to give us the results.)  I knew right away just from the fact she was calling, and that it wasn't a nurse, that this was not going to be good.  She shared that the Xray had virtually no change.  Something that there certainly should have been on that much Miralax.  She basically did a bowel prep clean out and there was nothing.  She said that everything we saw over the weekend was just the miralax itself pushing through and bringing a little bit with it.  Yikes!!! Not good!!! She said that now we needed to take further action and do a "TRUE" colon clean out.  A nurse would be contacting us right away with instructions and we would go from there.  This would be pushing back any tests as it is vital to get her cleaned out before there are any problems from that.  We would speak at the beginning of the week to report how it went and reschedule the test.

So, we waited and at 5:30 on Friday as the nurses were leaving for the holiday weekend we got our instructions.  We have decided to wait to start the clean out until Easter morning.  We want her to be able to go to our family brunch, do the egg hunt, etc. before she is miserable.  Judson will stay home with her Monday and we will go from there.  They cautioned us that it could take more than once.  Fingers crossed this works so we don't have to do it using other methods!!!!

Update Part 1

A little over a month ago we had Charlie's routine appointment with her motility specialist at CHLA.  At this appointment we reported how the previous three months had been going with Charlie.  Overall, she had been doing really well.  Let's just say her poop still tended to smell a bit on the rough side and she did throw up a few days beforehand.  This peeked the interest of the doctor.  She mentioned working towards predicting when Charlie was going to throw up so that we could avoid having it happen at all.  Sounded good to me!  The smell was likely a sign of a bacterial build up in her colon and thus making her throw up and have other potential problems.  She wanted to up her dose of Miralax to two capfuls a day to try to clear this out.  We would do this regime for a week and then go back down to our once a day dosage.  Perfect timing because we were scheduled to leave on vacation in just over a week.

Everything went well and we left for our cruise....this was the beginning of a very nightmarish past few weeks.  Once we left, Charlie was thrown a GI curve ball.  Her condition has been hard to manage ever since.  While we were gone her poop was all over the map (constipated or diarrhea) and she spent a morning throwing up.  Certainly fun while we were all cramped in a cabin on a cruise ship.  When we got off the ship we called the doctor as she had instructed to do if the smell was back (as it was) and if she threw up again.  The doctor wanted us to up her Miralax to two capfuls a day again for a week and call her back.  And so we did.  We got the same results.

The morning I went back to work from spring break I went in to wake her up to find her asleep in dried vomit.  It was undigested food from the night before.  For the past few weeks her stomach has been as distended as it has ever been.  She hasn't been eating well at all, and now we are fairly concerned. I called and left a message for the doctor and heard back last Wednesday afternoon.  They wanted us to come right over to do a KUB XRAY.  They wanted a clear picture of her entire stomach to see what was going on.  While sitting in the assistant director's office at school I finally started crying.  There was a lot going on.  I was overwhelmed at what we could possibly be facing again, there was a classroom of kids waiting for me to teach math, the need to grab her and run out to drive downtown for an X-ray, figuring out getting Levi from school, worrying about the continued exposure to radiation, etc.

I took a deep breathe, walked in to my classroom, taught my math class, and we left for the X-ray.  After our last stay, she has become wary of the hospital and tends to freak out a bit when we go.  When we walked into the X-ray room she asked to go home.  She was a trooper though and when we were done Charlie yelled, "I did it!" Then we got to wait for results.  They called that Friday morning to tell us that the X-ray showed that she was incredibly backed up (on two capfuls on Miralax, mind you) and that we needed to increase her Miralax to 3 capfuls a day for the weekend.  The doctor also wanted to see her on Monday.  Let's just say it was incredibly challenging to go anywhere last weekend, but at least we would be able to sit down with the doctor and go over everything and ask our questions, talk about a plan, etc.