Sleep Updates

Wow! I can't believe it's been a few weeks since I've last updated!  Time flies when you're having fun (or when you're just really, really busy!).  Last Wednesday, Dr. G finally called to give us the report of Rebekah's most recent sleep study.  

Dr. G started the phone call by saying, "I'm not really sure what to tell you because Rebekah did not respond to anything like I thought she would."  Yikes!  We have a really complicated little girl!

 Cute little sweetheart!

So the CPAP trial was a complete failure.  From what I could tell, Rebekah was hyperventilating because of the pressure of the air blowing in her face.  She was absolutely terrified because she couldn't exhale.  Once they switched Rebekah to a BiPAP, she did relax and was able to rest better.  *Translation:  She slept in 15-20 minute intervals over the next 5 hours.*  Finally around 5am they took off the "elephant" mask and put her on a nasal cannula for the remaining two hours of the study.  Finally, we were able to get more than 15 minutes of sleep at a time!  :)

Bekah loved playing in the rain the other day.

So the final results of the sleep study are....inconclusive.  Rebekah had absolutely no apnea events on the BiPAP, which is great!  However, her oxygen was very unstable and she had multiple episodes of desatting, which disrupted her sleep.  On the flip side, Rebekah did well on the high-flow oxygen setting at 2 liters per minute (LPM), and her oxygen level remained at 98% for the entire time.  She had no obstructive apnea events, although she did have an average of 10 central apnea events an hour, the longest being 16 seconds.  So the big question is which is better, the BiPAP or high-flow oxygen?!  (And for the record, I asked about combining the two.  It's a no-go.  The pressure would be too great, according to Dr. G).

 We've been enjoying warm weather and outside playtime!

Dr. G was very confused and puzzled by Rebekah's results.  Based on Rebekah's central events in the first sleep study, he was fairly confident that Rebekah's central events were caused by a drop in her carbon dioxide (CO2) levels.  However, when he put Rebekah on the high-flow cannula, her oxygen levels and CO2 levels both went up to the normal range, and the central events continued to occur.  Now he is not at all sure why the central events are occurring.

After a 30 minute discussion on the pros and cons of both options, we decided to continue with just the nasal cannula for now, but increase Rebekah's rate to 2LPM.  She has tolerated it well, although she does seem to be fairly restless at night still.  We are going to wait until we have the results of Rebekah's MRI, which is scheduled for March 28, and go from there.  It is possible that there could by a physical abnormality in the shape or structure of her skull that is putting pressure on areas that are causing her central apnea.  We certainly hope this isn't the case, but it is possible.  After the MRI, we will be better able to make decisions about where to go with Rebekah's apnea treatment.

Mommy, I stuck!

So that's pretty much the sleep updates for now.  Treatment for sleep apnea is certainly not a sprint! It's a full on marathon, and we are trying to be patient as we see what works and what doesn't work for our little sweetheart.  We would certainly appreciate your prayers for the upcoming MRI.  It will be done at the hospital in Greenville, and Rebekah will be sedated and intubated.  The good news is that it will be a light sedation, so Rebekah should wake up within a few minutes of the procedure.  We are praying for a good, clear scan that will show any areas that could be potential problems.

Hopefully in the next day or two I will be able to update about Rebekah's vocal chord scope.  I thought about adding it in with this post, but it deserves a post all on it's own.  As I said before, Rebekah is one complicated little girl!  :)

Nancy

Sleep Study Results Part 2

Now where did I leave off?  :)  There were just a few other things from Rebekah's sleep study results that I wanted to share (mostly just so I can look back and reference the information one day if I need to).

One good observation that Dr. G made is that Rebekah's carbon dioxide (CO2) levels are on the low side of normal.  Actually, in some cases, they were below normal.  Part of the sleep study is measuring the amount of CO2 that the body expels each time we breathe out.  For a lot of kids (maybe adults too?) with central apnea, they retain too much CO2, so their levels are quite high.  This can lead to more "events."  Honestly, Dr. G told me at our initial consultation that he anticipated finding Rebekah's CO2 levels to be high, and he gave me a couple of treatment options for that.  He was quite surprised at how low the levels were, and showed me that the lower the CO2 levels were, the more events Rebekah had.  So, one of the goals Dr. G has is to actually increase the amount of CO2 that Rebekah retains, in the hope that it will decrease the number of events that she is having.  One of the ways Dr. G wants to increase the CO2 is by increasing the amount of oxygen Rebekah is on overnight.  Rebekah has been on 0.5 liters of oxygen for a long time now - well over a year, and that is a good therapeutic level for her pulmonary hypertension.  However, Dr. G wants to "pin her to the ceiling" (his words, not mine) with oxygen in the hopes that the more oxygen going in, the more her CO2 levels will rise and hopefully the less apnea we will see.  It sure is worth a try!  So during Rebekah's next sleep study they will be trying her out on 1.5 or 2.0 liters of oxygen to see if that makes any measurable difference in the number of apnea events that we see.

Baby in a Box!  :)

Even though it appears that the central apnea is entirely, or at least in part, related to the CO2 levels in Rebekah's body, Dr. G is still recommending an MRI to rule out the possibility of any possible cranial or spinal malformations.  One in particular is Chiari (pronounced key-ARE-ee) Malformation, in which a piece of the skull is abnormally shaped and presses into the base of the brain or the brain stem.  Among other things, this could cause the type of central sleep apnea that we see in Rebekah.  Dr. G really doesn't think that we are going to find anything abnormal in the MRI, but with Rebekah's complicated medical history, he doesn't want to assume that the apnea is related to something else, without ruling out some brain and/or spine abnormalities.

Rebekah was given a small 

Hello Kitty suitcase on Sunday.

She is in love!

It will likely be late spring or early summer before the MRI is scheduled.  Dr. G wants Rebekah to be comfortable with her CPAP machine for a while, so that she can use it when she comes out of the sedation.  Yes, this MRI will involve sedation and intubation.  We would love to be able to schedule it when she is in Charleston for a heart cath or vocal cord scope.  We'll just have to see how the next couple of months play out and which doctors want to see her when.  We were told June or July for her next cath date, so we could potentially make it work if everyone can coordinate together.  What a blessing it would be to only have Rebekah intubated once and do both the cath and MRI at the same time!

I think that's everything I can remember from the sleep study.  I hope it's everything that we covered!  Rebekah has her first speech therapy on Thursday, so we would appreciate prayers that it goes well.  She should have had her first session last week, but we had to cancel due to sick brothers.  Hopefully everyone stays well between now and Thursday!

Please continue to pray for our little buddies at MUSC.  As far as I have heard, there have not been any significant changes today.  Especially remember their parents and families in prayer.  The days are agonizingly long when you are waiting, hoping for some sign of improvement.

Nancy

Sleep Study Results

So, we were up bright and early this morning for Rebekah's appointment with Dr. G this morning.  If you haven't personally experienced Dr. G, he is a super energetic morning person with severe ADHD!!  I'm just kidding - I really have no idea if he has ADHD, or even if he is a morning person, but I can tell you that he has so much energy that he practically bounces from room to room.  He also changes topics with the speed of a ping pong ball.  :)  It was great, but my foggy sleep-deprived brain actually had to work to keep up with him! All kidding aside, he is very, very knowledgeable in his field, and super nice.  We really like him.

Now for the results.  Dr. G walked in with at least a 1" thick stack of papers from Rebekah's sleep study, sat down and said, "Wow, where do I even begin?"  Hmmmm.  Not the words we really wanted to hear to start off the appointment!  I'll start with the easy stuff first and then some of the more complicated.

First Rebekah has both obstructive sleep apnea and central sleep apnea.  The "events" (lingo for apnea episodes) average about 8 times an hour (for obstructive) and about 9 times an our (for central).  So, as you can see, she does not have one predominate type of apnea.  She's an equal opportunity kind of girl!  :)  If she only had one or the other of the types of apnea at the frequency above, she would fall somewhere in the "moderate" apnea range.  However, looking at the big picture, she has an average of 17 events per hour, which puts her into the "severe" apnea category.  And if that wasn't enough, she also has something called periodic limb movement disorder (PLMD), which basically means that she involuntarily moves her legs (and arms) all night.  Yes, I know; I've slept with her before.  Trust me, she thrashes!  :)

What is the next step, you ask?  The first thing we had done today was to have bloodwork done to test Rebekah's iron levels.  Apparently there are some links between low iron and PLMD in people who have heart problems.  Given Rebekah's heart history, we felt that was a good place to start.  If Rebekah's iron is low, she will begin taking iron supplements.  If not, there are some medications that can help control the thrashing so that she can rest more at night.

As far as the apnea goes, the name of the game is slow and steady.  We aren't going to pinpoint, diagnose and treat every issue in the next two weeks.  In fact, I'll be happy if six months from now we are on top of Rebekah's sleep issues!  For now we are beginning with another sleep study (oh, goody!) two weeks from tonight.  Rebekah will be evaluated using a CPAP (continuous positive airway pressure) machine which will forcefully blow air into Rebekah's nose to keep her airways open.  It will still be up to Rebekah's body to "breathe" the air in, but the machine will not allow her airways to collapse.  Big prayer request over the next two weeks is that Rebekah gets used to the mask before we use it.  It is a small, triangular shaped mask that fits perfectly over her little nose.  There are velcro straps that attach it to a small mesh cap that fits around the back of her head.  The cap and velcro help hold the mask in place.  During the sleep study, the technicians will be making many adjustments to the CPAP machine to try to get the optimal settings for Rebekah.  If the CPAP works well, great!  If not, we will be rescheduled for a trial on the BiPap (Bi-level positive airway pressure).  The BiPap would actually measure Rebekah's breathing and if she went for so many seconds without a breath, it would force a breath into her.  We will just need to see where Rebekah's needs fall and what type of treatment works best for her.

One of the pieces of good news we learned today is that Rebekah's EEG (measures brain activity) that was done during her initial sleep study did not have any red flags on it.  We haven't gotten the official neurologist's report yet, but Dr. G said if there was a huge "red flag" that he would be able to see it on the report.  We are very thankful for that good news!!

There is a little more information from the sleep study, but I am honestly exhausted and ready for bed.  I'm losing focus of what I'm typing!  :)  I will try to finish the rest of the update tomorrow, but in the meantime I will leave you with the first good video we have gotten of Rebekah since her vocal cord paralysis.  Since some of her words are difficult to hear and understand, I will post a translation below the video.  Enjoy!!

Jesus loves me ...I know

For the Bible tells me

Taggie!

Hey, shoes!

Hey, necklace!

No

Mommy, can you help me?

I need a (something that I don't understand)

Bye!

Deep and wide

Notice that Rebekah has her priorities - accessories, shoes and jewelry!  :)  I love that it so perfectly shows Rebekah's personality, even if it is hard to hear her voice.  If you really want to hear the difference, go back to this video where Rebekah sings part of Jesus Loves Me.  The difference is really amazing.  We have really gotten used to Rebekah's "new" normal voice, and every time I listen to the older video, I am just amazed at what her voice used to be.  Wow.

Please continue to pray for all of our little friends at MUSC.  There have been no significant changes today, and I know their families appreciate the prayer!

Nancy