The Parent Manual

It has been quite an interesting week for Meagan.  What started off as a seemingly simple issue snowballed into a much larger problem.  And from that we have finally started to have several answers available to us to help make a long term plan for some aspects of Meagan's care.

Let's rewind a little bit - Meagan has been having random pain episodes.  She will throw her head back, and fuss or cry, and be overall just miserable.  It happened here and there really for the last several months, but I never noticed any increase.  Likewise, I never noticed any significant change in her mood or her progress with therapies, so for the most part, these episodes sat on the back burner.

A few weeks ago, I noticed they started happening a little more than I had remembered.  What got my attention was not necessarily the frequency, but, the intensity.  She reacted like someone had just poked her with 1000 pins, and would cry, moan or scream in pain. She also would hit or scratch at the back of her head or punch herself in the sides of the head.   Along with this behavior, she has increasingly been wanting to be upside down.  She wants to hang her head over the back of my arm, or wiggle herself up her nap nanny to sleep with her head hanging halfway over the top of it.

For Meagan, these behaviors were new and also bothering her.  Now that said, she wasn't screaming 24/7... if that were the case we would have been at the ER ages ago.  But, those who know Meagan, know that unless she is hungry, she is an incredibly happy person.  She can sit contently for hours and just take in her surroundings.  She is blissfully and innocently content almost all of the time.  In recent weeks, her contentedness seemed to be a little less common.  Times she wasn't upset, she wasn't necessarily happy either - there were a lot of blank stares, or just sitting and looking out with very tired eyes.  She was sleeping a bit more and more frequently during the days.  And at night she was starting to wake up startled - I assumed with a nightmare or something like that.  To the average person, probably nothing looked "that" wrong with Meagan. But I knew something was wrong.

The first stop we made, of course, was the Pediatrician.  If it's any sort of "normal" or "simple" problem, we jump for joy.  Give me flu, ear infections, strep....anything along the common illness spectrum is better than something going on inside her brain.  I emailed Dr. R (Neurosurgeon) to give him a heads up on her behavior but told him we were going to see the Pediatrician first.  We saw Dr. B on a Thursday.  Surprisingly, the doctor found an infection in her right ear.  Her 5th since December, so after we left, we also called and made an appointment with a pedatric ENT to talk about tubes.  I left thinking we were in the clear.  I figured as soon as the medicine was in her for a few days we would be back to our good old typical Meagan.

The weekend came and went and while she still had happy times, she was still also having the fits.  Hitting. Crying. Wincing her eyes, as if in pain.  I was confused because she had been on the antibiotic plenty of time by then, so when Monday came around, back we went to the Pediatrician. I also emailed Dr. R to let him know she was still having episodes and we were out of ideas if all checked out ok with Dr. B.  I walked in to the pediatric office expecting her ears to be much worse.  Dr. B said her ears actually looked better.  We left and chalked it up to residual irritation after having her ears hurting.  Then came Wednesday.

This pretty much describes our Wednesday

Wednesday started out like any other day.  We got up, sent the older kids off to school, and started about our day.  Then I heard Meagan taking short breaths like she was in pain. Following that was her head thrashing, hitting, and crying.  I picked up my phone and emailed Dr. R to tell him she was still acting out.  I also told him we would go to the Pediatrician one more time to double check her ears but I didn't think that was the culprit.  Dr. B did a 'once over' on Meagan, and all looked ok.  Her ears looked even better than on Monday.  There was no 'easy' answer as to what was causing her episodes of discomfort.  In my gut I knew it was probably brain or GI related and Dr. B agreed.  Within about 15 minutes of leaving the Pediatrician, I got a callback from Dr. R's office.  They advised to head to Children's, get Meagan a CT, and then come over to the office to see Dr. R and discuss.

We drove down to Children's hospital.  Of course being unplanned, I had the girls with me. When we got back to CT, I realized I wouldn't be able to be with Meagan because of the other girls being there.  So we stood outside the door while Meagan got her CT done.  The first CT I haven't been in since she was born! I could hear Meagan through the door and out into the hallway. Hearing her made me want to go in there and be at her side.

After the CT we walked across the street to Dr R's office.  Meagan was in another fit, but I was kind of glad. Meagan has this strange 'bond' with Dr. R.  Even when she has been the most upset or the most in pain, she always will smile at him.  At the very least, she will stop her fussing to look at him or listen to him.  Dr. R had never really seen her this upset.  Typically, when one of my kids is really sick, the minute we get to the doctor they are smiling and I look like an over-worried parent.  But not this time.  She would not smile or interact at all with Dr. R.  He was immediately worried about her.  Besides our family, Dr. R probably knows Meagan the best.  He knows her tendencies and her moods, and it was obvious to him something was really hurting. He suggested to me that we admit her that day and see what was going on. He had a suspicion but would need some tests to be sure. I agreed. Dr. R likes the path of least resistance   He won't open up a child or inconvenience a family unless absolutely necessary...so when he says it's time for admission and tests, I know he is right.   He also said while we were there, he could consult with GI and have them come see us too.  I was all for that since we had been given such a runaround by GI lately.  If Dr. R could 'help' them along, by all means.

While they drew up admission papers, I ran home.  Brian made his way home too and I dropped the other girls off with him.  I packed a bag, got a few items for Meagan, and then the two of us were off to Children's.  We checked in, got our room, and settled in for the night.

Meagan was still pretty upset that first night.  She was so restless. Moaning. Crying. Sleeping.  Not herself.  The nurse came in to see us and said that Dr. R had given instructions to leave Meagan alone that night. No checks. No blood. No IVs.  Just to let her rest and do the IV in the morning before her MRI.  He did, however, want her on monitors to watch respiratory rates (which turned out to be a good thing).   All night her alarms went off for breathing.  She never stopped, but her respiratory rates were really low and would stay low. When she was asleep they would hover around 10 or 11 (typically a child her age should average 20-35 per minute).  Occasionally she would jolt like she did at home and her breaths would shoot back up to 20.  Other times, the nurses or myself could get them back up by rubbing on her back, or stimulating her somehow.  It definitely worried me and sparked my interest as to why this was happening.  Morning finally came and we were set up for MRI.

Meagan's IV was placed (of coures by the IV team as she's such a hard stick)  and we headed down to MRI.  I walked back with Meagan and laid her on the table.  They gave her the sedative through her IV and in about a split second her eyes rolled back and she was out.  They told me it would be close to two hours for the scan.  This was honestly the first time I didn't feel badly about sedating her.  She had such a rough time recently, had been sleeping horribly, and was not happy that I was glad for a little while she was able to get some rest.  After finally getting myself something to eat and drink, I went back to the sedation room.  Meagan was carried back in after her scan.  She looked so peaceful. It took her a good while to wake up and finally the sedation doctor had to come in and help her along. After a lot of back rubbing, talking, singing, and rubbing her head, Meagan's eyes finally started to roll around.  Once she was stable enough we were taken back to our hospital room.

Dr. R was in surgeries all day but was able to call me that afternoon.  He said that as he suspected, he did find something on her MRI and would go over details with me later that day.  He said surgery for the issue wouldn't be done on this particular admission, but that it is something that would need to be fixed.  In the meantime, he had two more surgeries so I was to consult with GI and start everything with them.

Later that day, Dr. R and the PA finally came by to discuss Meagan's MRI.  They told me that Meagan has a Chiari Malformation at the base of her brain, which is what he had suspected based on her behavior and my descriptions of her latest episodes.  (You can read about Chiari here and here.)  In short, a Chiari Malformation is is a neurological disorder where part of the brain, the cerebellum (or more specifically the cerebellar tonsils), descends out of the skull into the spinal area. This results in compression of parts of the brain and spinal cord.  The way Dr. R explained Meagan's Chiari to me was by telling me to think of an open wine bottle.  Then imagine trying to cork that bottle - but instead of using one cork, you had to use two.  My first thought was "ow!!" I think I would have screaming episodes too.

  There can be several indicators of Chiari such as head and neck pain, balance issues, poor weight gain, and breathing issues.  A lot of the things Meagan has struggled with lately with are also symptoms of Chiari.  Dr. R said he feels she does need the decompression surgery (You can read about the decompression surgery here).  He is hesitant at this time however because it's an extremely vascular area (ie: a ton of blood vessels)..and that along with Meagan's tiny 15 pound body make it a risky surgery at this time.  He said it will need to be done, but, working with GI, let's try to do what we can to get weight on her first.  As she grows, the area will become easier to navigate.  Of course this has to be held in a delicate balance with her symptoms.  Since she obviously was already experiencing head and neck pain, and feeding difficulties, it isn't a surgery we want to put off forever. Besides the obvious, there is also a risk to her shunt with the surgery.  Meagan is so incredibly sensitive to any pressure changes, and this surgery would open up a new fluid area - thereby changing the pressure of the fluid.  There is a chance it could cause malfunction in her shunt. This is a big issue we are mulling over because it's only been in the last few months we have finally been able to find a shunt setting Meagan is not only happy with, but a setting with which she can thrive.  So there is definitely a lot to think about regarding the decompression surgery and what benefits will outweigh what risks.

Meagan after a pain episode. My sweet girl!

 After going over all the information, Dr. R said he was sending Meagan home on an apnea monitor. I was a little taken aback because I did not expect it.   He said because of the relationship her brain stem has with the Chiari, and based on her monitor numbers at the hospital, he wouldn't be comfortable with her sleeping without one. Once it was all explained to me, I completely agreed.  In hindsight I began to wonder if some of her fatigue was because she would awaken suddenly.  And I also wondered if those sudden awakenings weren't nightmares, but were in fact times her body woke up quickly to take a faster breath.  It was all pretty scary to think about! I can tell you I haven't slept much since we have been home because of this, but I know we will adapt.  She will be on the monitor until she has the surgery.

Later that night, the respiratory therapist came by and set up her monitor.  We signed all the rental agreements for the equipment and once we had our stuff packed, we were free to go.  Dr. R said we need to keep a good log of Meagan's episodes and behavior, and also how she is doing with eating and weight gain.  We will see Dr. R again on May 1st to sit down and go over all our data, the rest of her scan, and go from there with a surgery date.

Other findings on her MRI threw out a few red flags to me:

- Pituitary "something"...not sure what but there is either a small mass near it or it is severely overcrowded (which would make more sense) - I'm interested to see if this is impacting her weight gain at all
- Thickened Hypothalamus -   Also her hypothalamus looked extremely thick and she will need another scan of that particular area to see more details

 In some ways we got a lot more answers than we were hoping for.  It is a lot of information to swallow at one time and I'm looking forward to going over more of the details with her Neurologist, especially regarding the pituitary cyst.


I had a little phone conference today with Meagan's PT to run everything by her.  She was in agreement that the Chiari needs to be fixed.  She said she thinks with Meagan's extreme sensitivity to positional change, the Chiari can really be holding her back physically.  And it may explain why when we have worked her really hard at a session, she isn't just tired, but is completely miserable and upset for days afterwards, throwing back her head.  It's also a very strange limbo to be in though.  The Chiari can impact eating and weight gain - yet she needs to gain weight for the surgery.  So do we try to stuff her with what we can in hopes of gaining weight and in hopes that the Chiari surgery removes some of those roadblocks? or do we put in a g-tube so she can surely gain weight before surgery even if eating may improve afterwards? Does she need to see an Endocrinologist now due to the pituitary cyst? Will that have to be drained or removed?  Is the Hypothalamus issue why Meagan has so much trouble with her temperature? Is the Chiari pain or dizziness keeping her from wanting to tackle that next step to sit independently?

Since we have been home today Meagan has had good and bad moments.  She had one pain fit earlier. Crying. Holding her head. Pushing her head backwards over her nap nanny.  But, she has also had content moments.  She is obviously not quite herself yet 100% and something tells me we will see days like Wednesday come and go until we fix the Chiari issue.  On top of that her right eye is a bit droopy and closed and she just acts like she doesn't feel well overall.  I'm praying it's not the case, but it seems like she could be starting to get sick on top of everything else.

Feeling a little off tonight

Meagan's new sidekick at night - apnea monitor

What I've realized this week is that even a solid answer leaves questions to be asked.  Questions about what the best path is, and what all these new findings mean for Meagan now and for Meagan later.  The one thing I can take away from this whole week is that mom knows.  It has completely validated to me our decisions to walk away from doctors who choose not to listen to me or those who claimed to know Meagan better than I do.  I'm no doctor, by far.... however there is just something in us as mothers to know when something is off.  From the first dividing cell when Meagan began to grow to the current moment of snuggling her at night, I know my child better than anyone.  I feel thankful and blessed that Meagan has such a wonderful Neurosurgeon - but even more relieved that he listens to me.

We went into this week thinking we were dealing with ears, and came out with a laundry list of new brain issues, new appointments, and new plans. More risky surgeries and new problems definitely stink.  However, my goals for Meagan are long term - I want her to be as happy, comfortable and independent as possible. To get there, she's going to have to go through some hard things.  It isn't easy being Meagan sometimes. I saw it in her eyes this week. But we knew things like this could come up. Unlike my typical kids, Meagan came with a parent manual in a way.  Parents always joke about their kids coming with instructions.  Well, Meagan kind of did.   We knew of her condition, we knew the risks, the potential problems associated with it, and the probability of the unexpected. Meagan's parent manual may occasionally throw us a few curveballs we didn't read about, but we'll get through it.  A few typos along the way don't dictate who Meagan is. God does. She does. They are the author. And I won't let her forget that.

So exhausted...but still my gorgeous girl