I Never Knew Sleeping Could Be So Dangerous!

I have been meaning to do a post about Becca’s sleeping habits for a long time.  It has definitely been the subject of stress in this house. Brief recap: Back in the beginning of last year Becca had a sleep study that showed she has severe obstructive sleep apnea.  Her apnea is characterized as centralized sleep apnea which, at least in part, means that the part of her brain that is supposed to tell her to breath doesn’t function properly while she’s sleeping.  Pretty much her brain forgets to tell her to breath periodically throughout the whole night.  I know, rude! As a result (from both her apnea and obstruction), her oxygen levels dropped pretty low.  Anyway, since her sleep study results were not so good we decided to get her tonsils and adenoids removed to see if it would help.  That happened last June and turned into a month long stay at the hospital (see this post for details).  Since the surgery, Becca has had a great recovery and has been really healthy.  End of recap.
Oh and here’s a picture of how Becca has been sleeping since her first sleep study:

 
Since she won’t keep tubes in her nose we do blow by (high concentration of oxygen is blown into her tent by her face).  The side flap is usually down.

So, a few months ago we did a repeat sleep study to see if the tonsillectomy helped with her breathing/oxygen levels.  And the results came in….nope.  Not at all.  Her study was still just as bad.   The doctor told me that basically she’s having an apneatic/obstructive event about every two minutes through the night with her oxygen levels dropping low.  It was kind of sad considering all that we went through to get her tonsils/adenoids removed.   

The next step was to see what her oxygen levels are like while sleeping in her magical oxygen tent.  A few weeks ago we rented a pulse oximeter and tested Becca through the night.  And hooray! The oxygen does seem to be helping as her levels stayed good and high.  Whew.  That was a relief.  So after all that, we just keep doing what we have been doing.  But, I’m ok with that.  I'm just so relieved that the oxygen is helping and she doesn’t have to wear anything on her face. 
I’ve had times when I’m very worried about Becca and her unconventional sleeping habits.  As you may or may not know, many girls with Rett pass away in their sleep (without any warning and at any age).  Brian and I both have times where we go to check on Becca and we have a brief moment of sinking terror when we don’t see her chest rise right away.  At least it helps a little to know that the oxygen is helping!

Tonsils, Adenoids, and a Code Blue

This is kind of a delayed update, but it's been hard for me to write about it, and I wanted to wait a while before writing so I could look at it a little more objectively.

About 5 months ago Becca's geneticist Dr. Rope recommended we do a sleep study for Becca. She gets pretty tired during the day, and most days she wakes up between 4 and 6am, so there's definitely room for improvement in her sleep routine. We scheduled the sleep study (that's probably worthy of its own post. We'll never do another sleep study anywhere other than at Primary's. The sleep tech was pretty sure he wouldn't need to wrap the EEG nodes, but Becca went at them most of the night, and fought the nasal cannula as well... it was a blast...) and the big takeaways were that her oxygen levels are a bit low when she sleeps and that her brainwaves are all over the place (no surprise there, pretty typical for Rett girls). We decided to give her blow-by oxygen at the night, and see a ENT about possibly getting her tonsils removed.

The ENT, Dr. Grimmer, thought it might be helpful to remove Becca's tonsils and adenoids.  Her tonsils were enlarged, and while it wasn't a major concern, it might help her breathe a little more easily.  Becca does cough sometimes in the night, and it gets pretty bad when she's sick, so it seems like it'll be a good thing in the long run.

But first we had to make it through the surgery.  June 6th we went in to Primary Children's Hospital for her T&A.  We were really nervous before we went in because Becca had coughed a couple times that morning and had a low-grade fever, but she does just cough randomly sometimes, and the hospital told us to go ahead, so we brought her in for the surgery.  We won't ever take that kind of risk again.

After the surgery they took Becca to the floor for recovery (it was technically an outpatient surgery, but they wanted to keep her overnight to make sure everything was all right).  I wasn't there, but right away Paula knew something was wrong.  Becca wasn't breathing well and was obviously pretty miserable whenever she woke up from getting her tonsils and adenoids removed.

After a couple hours she had what Paula thought might have been a seizure.  For the previous few weeks sometimes Becca's arms and legs had started twitching rhythmically for a few minutes at a time. She was very in there when it would happen, but we weren't sure if they were seizures or not.  Paula took the opportunity to ask the staff if our neurologist could come see what was going on and confirm whether or not it was seizure activity.  Dr. Benedict wasn't available, but another neurologist named Dr. Lloyd checked in and called it a seizure (incidentally, after some follow-up EEGs we now know they weren't seizures, and it sounds like this kind of thing may be common for Rett girls, there just wasn't anybody around with any experience in Rett cases).  They gave her a huge dose of versed (or ativan, I can't remember which) and the twitching stopped but she got all messed up.  Her eyes weren't lined up anymore and she would either sleep or stare off at nothing -- and she still was not breathing well.

The breathing was the biggest concern for us.  Becca's always had a hard time with breathing, and when she gets sick at all or coughs a lot she stops swallowing and stuff builds up in the back of her throat and exacerbates the problem.  She had blow-by oxygen but her breaths were just really short and quick, and her oxygen saturation levels were all over the place (anywhere from 80% to 50%).  We asked a couple times about it, but none of the nurses were concerned with the situation and said it wasn't a big deal after a throat surgery, so we assumed it was fine.  They gave us a suction tool to help clear her out, but told us we shouldn't suction much or it would make things worse -- even though it was pretty clear she was collecting a lot of gunk back there.

This, by the way, was pretty much the end of our implicit trust in nursing staff.  We assume now that nurses won't know the right things to do for Becca, and then they can sometimes pleasantly prove us wrong and earn our trust.  If we'd known then what we know now we probably wouldn't have ended up in the PICU.  Hm... Actually, I guess if we'd known then what we know now we might have pushed them to take Becca directly to the PICU for recovery instead of just going to the floor.  We're a lot more confident now than we used to be that we know what's best for Becca, so I guess at least there's that.  It seems like maybe that's something a Rett parent just needs to learn eventually.

Anyway. I sent Paula home to get some sleep (she was 5 months pregnant, and Becca wasn't coughing quite as much anymore) and settled in for the night. At about 10:30pm Becca's breathing got a lot worse.  She started coughing pretty bad, and then she started coughing worse (this is when the nurse came in), and then she stopped inhaling, and then her coughs got really weak, almost non-existant.  Her oxygen saturation dropped to 20% and the nurse hit the emergency button.  The PICU staff got there within 30 seconds and right away started working on opening Becca's airways. Becca's lips were blue and her skin had lost its color almost completely.  She looked dead other than the faint spasms in her chest. Her oxygen got down to 12% before starting to slowly climb up again.  About half an hour later the respiratory technician finally had her up to 85% oxygen, and we decided that was enough to move her down to the PICU.

As you can imagine, I was pretty much a wreck at this point. I actually think I did really well until somebody said something about "life support" and for some reason that was the thing that threw me. I managed to get a call out to Paula and speak enough that she knew to get there right away. They wheeled Becca down to the PICU and got her all checked in.  She couldn't breathe at all on her own, but at least she was stable.  We found out later one of her lungs had completely collapsed, and the other was significantly obstructed.  The event was classified as "acute respiratory arrest".

I think this is enough for one post. Maybe I'll do the PICU experience in another post.

Melatonin Helps

So Becca's been taking a melatonin pill every night an hour before bed for the last two months, and I think it's safe to say that it's definitely been helping her. It was getting to the point where every night she'd cry for at least an hour, sometimes up to three, when we put her down for bed. But she wasn't sleeping in any longer -- and it kind of seemed like she was even waking up earlier -- so we were getting worried. I mean, it's not like she was mouthing off when we asked her to help clean up her toys or anything, but was having a lot harder time being happy.

Becca usually goes down for bed at 8pm, and she wakes up anywhere from 5am to 7am. I don't remember how many hours of sleep 3 year-olds are supposed to have, but from what I hear that's really good for a Rett girl. Sometimes she wakes up quietly, and sometimes she wakes up and yells I think out of boredom. Paula doesn't get up with that early, she goes into her bedroom (she still fits in her crib pretty well... no idea what we're going to do when we need to move her to a bigger bed), pulls out her pillow and gives her a toy instead, and that usually keeps her happy for an hour or so.

Anyway. Becca's neurologist had mentioned melatonin before if we ever needed it, so we called her up to find out what kind of dosage she should have. It's not a prescribed thing, but it's not like you can find it in every grocery store, so Paula had to go to Rite Aid to find it. She found a liquid one and a pill one (sublingual was the term on the bottle... apparently that means under the tongue as opposed to less-than-capable at speaking even one language, like it sounded to me originally), and wasn't totally sure what to get. She ended up asking the pharmacist to double-check that she remembered the dosage and everything, and he politely told her that melatonin isn't for kids, they shouldn't need it. She tried to explain the situation, but I think the guy judged her anyway. So we got the pills and figured we'd give it a go.

Becca doesn't keep things sublingual, so much. She's actually pretty good at rolling things around in her mouth -- way better at that than actually swallowing them, as it turns out. But she does swallow these pills pretty well, so that's cool. We just have to remember to give them to her every night an hour before bed, since it doesn't really help that much unless you give it a little time to do its thing. We weren't sure if it would actually do any good, but it seems to be working for Becca. Now she's falling asleep in a blessedly normal ten minutes or so, and waking up a happy girl once more. And thanks to the Keppra, once she's gone she stays a comatose noodle for most of the night... you couldn't wake her up if you wanted to.

Rough Week

I'm having a rough week with Becca. She has been shaking more this week. She has been a lot weaker this week. I put her in her gait trainer and she just goes limp and gets mad. But I do it every day anyway. I set the timer and we muddle through until at least ten minutes. I know it's good for her and I know that she'll get back to being ok with it and maybe even start enjoying it again. I'm sure it's hard for her to get back into the swing of things after being back from vacation.

I can't help but cry out of frustration and discouragement when Becca has a hard week like this. I'm exhausted. I think of all the things Becca misses out on. More than that I think of all the things I miss out on (mostly with not being able to communicate with her). Things are just so hard for her! A couple weeks ago I gave Katie a bath in the real bath for the first time and she just sat there. I didn't have to hold her up or anything and she wasn't scared and shaky. I was in awe! Is this what most kids do? I have so many of these experiences as Katie has learned things so easily. Mostly I cry because I'm discouraged. I'm not able to take care for Becca as well as I think she deserves. And man, it's just discouraging to work so hard at skills just to lose all our progress in one day. Makes you feel like it's pointless to work on things! But well, life goes on. I love sweet Becca more than I can say. I would do anything for her.

On a more positive note, Becca did quite well on our trip out to Colorado. She had a wonderful time with her Grandma, Grandpa and aunts and uncle. She loves her family so much. She really loves people in general, but it does seem like she favors those who she's most comfortable with. Lately sleeping has been harder for Becca. Well, sleeping hasn't been so bad once she gets there. It's falling asleep that is really hard for her sometimes. At times it can take her more than 3 hours to fall asleep. One day in Colorado Becca decided she didn't want to take a nap. She assured me that she didn't need a nap and that she would be a perfect angel for the rest of the day (I'm sure that's what she was saying with her little mischievous smile). Here is what she was like in the evening right before we were supposed to go out to dinner...

She sure makes me laugh.

Becca loves watching other kids play and it was fun for her to watch her sister and cousin get into trouble. She loves her sister and cousin!

Becca had a wonderful Christmas! She was healthy and happy. I couldn't have asked for a better Christmas present. I hope you all had a wonderful holiday season!