Sleep Problems

We've had restless nights.  Many of them.

Two years ago, before we had Brianna's diagnosis, her sleep began to deteriorate.  It went from what I expected from a breastfeeding toddler (nursing every couple hours, but except for frequent wakeups, no major issues falling asleep and staying asleep).  After she got her feeding tube (18 months) I thought maybe she would sleep more deeply.  Nope.  As my pregnancy with Liam progressed, and her sleep deteriorated, I began to wonder HOW I was going to cope with a newborn and a child who needed constant attention through the night.  This was when we began using Mickey Mouse Clubhouse as a way to keep her happy while I could at least doze.  But I knew we couldn't keep it up.  While visiting on the mainland for two months, Brianna was falling asleep at 5am, and waking around 9/10am.  No naps.  This continued when we got home.  At this point, I started using the "Sleep Lady" sleep training technique and we also used melatonin to "reset" her sleep cycle.  I've never been a fan of the idea of sleep training, but I believe that it was necessary, for both of us.  After a few weeks, blessedly, she was able to fall asleep on her own, and typically slept through the night.  About 2 weeks later, Liam was born (and we know how babies sleep!)

In the nearly two years since Liam's birth, Brianna has had fairly good sleep, at least for a child with PDCD.  She easily fell asleep on her own.  A couple times a week we would hear her awake in her room, chattering happily to herself, but she rarely was upset.  We assumed she would just wake up, hang out, and fall back asleep.  A couple times a month, especially if she was unwell with sinus problems, she would wake up upset.  We'd rock her, soothe her, maybe have to spend an hour or so comforting her, but she would fall back asleep without much issue.

Around two months ago it began to change.  Exciting things have been happening.  In February, we changed her Ketogenic diet ratio from 3:1 (fat to protein/carb ratio) to 4:1.  This seemed to have a profound effect on Brianna - almost as drastic as her changes upon starting the ketogenic diet.  Her energy levels increased, and she began to become much more active.  Walking while holding just a hand, or someone holding onto her shirt.  "Chattering" more.  Becoming more interested in what was going around her.  Having a better time at school.  Two months ago, she started exploring the house a lot more.  She began to figure out how to get out of bed and open the door.  This started in the morning, but about 2 weeks ago she figured out how to get out at night (previously if she got out of bed, she'd just crawl to the door and cry...now she stands up, opens the door, and then gets down to crawl and find someone).  She would wake up starting once or twice a week, crying.  I could tell she was having major issues with congestion.  She wasn't alone, about two months ago many people, including myself, started to notice issues with sinuses and whatever was going on with the weather/VOG (volcanic "smog" if you will, from the erupting volcano on the Big Island).  It really was affecting her sleep.  When she would wake up, she couldn't breathe very well, so she wanted to be up, because it felt better.  After a few weeks, what was intermittent had become a nightly thing.  First for an hour or two, then for hours.  We resorted again to letting her watch Mickey Mouse Clubhouse, just so we could function.

This picture is from July 24th, about the time our sleep struggles began.


For the last two weeks, Brianna has been going to bed, after much coercion and repeated attempts to put her in bed, around 9pm.  Then she wakes around midnight, not going back to sleep until anywhere from 3-5 am.  Naps are almost nonexistent (she just comes out of her room).  This has started to impact her in big ways.  We have noticed a lot more chorea (uncontrolled waving arms of the arms and hands).  Also, she was beginning to have "fits" again.  Things would set her off into a seizure-like event (could be an actual seizure, I don't know for sure).  After over a year without seizures, and with her much bigger and more aware of what's going on, it is heartbreaking to watch.  She screams when they happen, and her whole body flails uncontrollably.  Afterwards, I just hold her and comfort her until she gets distracted (by Mickey Mouse, of course).  Her nurse at school told me that yesterday she had about a 2-3 minute seizure.

I am INCREDIBLY fortunate to have a support group on facebook of other PDCD parents.  I truly don't know how we would survive without them.  I tend to be a "suck it up and press on" kind of person, and honestly, a lot of times it doesn't occur to me to ask for help until I'm past the point of coping.  At this point, I desperately told the group what we were going through.  Many offered great advice about dealing with her sinus problems.  And several of the mothers explained the sleep problems they've had with their children.  A popular medication is Clonidine, safe for children with PDCD, and with few side effects (and not addictive).  I wrote a desperate email to her metabolic team, and they agreed that Clonidine was a great next step.

Last night we started the Clonidine.  I would say it's a moderate success.  She went to sleep more quickly than she has in weeks, asleep by 7pm.  She woke up around midnight (her usual wake time of late), and I laid in bed with her.  She was back asleep within an hour.  Also a first in a while.  Now it's five am and all the children are awake sitting on the couch watching Mickey Mouse.  The Vog is hitting our family hard, they've all got hoarse voices and coughs.  I'm praying for the trade winds to help us all feel better.

Hopefully the Clonidine will give Brianna the sleep she needs.  Keep her in your prayers, as always!

Finally an update!

What a year. Now that I've figured out a way to blog from my iPad (and iPhone!) I intend to shoot for at least a weekly blog post so everyone can see what my amazing Brie is up to!
We have been so blessed. Brianna has come so far in the last year. This time last year she had totally regressed from the world. She would curl up and scream anytime someone not in her immediate family spoke to her. Random sounds would send her into a fit. It seemed like the only thing that made her happy was watching Mickey Mouse. She was having daily seizures and it felt like we had lost a part of her.
Now, she has blossomed. She is a chatterbox, talking away in her own language. Interacting with people, even strangers. She's crawling around, and trying to walk as much as possible holding on to whatever she can. It has probably been 8 months since she had a seizure. We are truly and deeply blessed. Between the ketogenic diet, all the work school has done with her, and the therapists at Kapiolani Medical Center, she has come such a long way. She is truly a little miracle.

- Posted using BlogPress from my iPad

FINALLY Figured Out

I just wanted to pop in and say that I figured out my problem with the comments. It all had to do with cookies. I guess if I just had googled "I can't post comments to my own blog" I probably would have had it figured weeks ago, but that was just WAAAAAY too obvious. ;)

I plan on blogging this weekend, updating you all to Brianna's progress with school. Thanks for following!

Test Blog

I'm doing a test to see if anyone else is having problems commenting on my blog. Please try to comment, if you are unable to, please let me know (via facebook). Thanks!

Week in Review

It's actually been a really good week for Brianna. We've been adjusting her diet to find the right ration. When we started she was at an 87%, but her team wanted her below 80%. The reason is because when the fat content is above 80% it sends her into the danger zone where she needs more frequent monitoring. And we have to be really careful about what to feed her to make sure the levels don't adjust. So she would essentially have to be tube fed with little to no oral feeds because the diet preparation is so complicated. So we wanted to keep her at a lower ratio. When we had initialized the diet, the effect was obvious. She became more interactive, her energy levels increased, and her SPD (sensory processing disorder) became much less obvious. However, when we lowered the ratio under 80%, her entire demeanor changed. Her seizures returned, her energy plummeted, and she was expressing more sensory issues. So we decided to return to the higher ratio. It wasn't as drastic or obvious as the first time. She has still had a couple seizures, but her energy levels have definitely been higher. And she's so much more engaged and vocal.



On Wednesday she had physical therapy and did beautifully. Her therapist noted that her balance is really good and she's on the verge of standing and learning to walk. Right now our major roadblock is her confidence. She actually stands without support if she's not paying attention, but as soon as she notices, she grabs on to someone or slowly sits down.

We discussed transition with her case worker. On August 30th, she turns three and ages out of early intervention. At that point, her therapy becomes the responsibility of the DOE (department of education). So she will be starting DOE preschool on September 1st.

On Thursday she had speech therapy at Kapiolani Women & Children's medical center. She is working with the augmentative communication specialist there. Brianna did amazingly. They brought out the ipad, which has a lot of great applications for working with augmentative communication. Brianna had a hard time at first, she tends to pull in and have a hard time making her hands do what they need to do. But the therapists did great at helping her and drawing her out. And within 15 minutes she was interacting and making the ipad do what it needed to do. Watching her I realized that she understands a LOT more than what is obvious to us on a normal basis. Being able to use a device to tell us what she wants/needs will open up a whole new world for her. For now, Brant and I are researching which ipad we are going to purchase for her until we decide what kind of permanent device that we will get for her through insurance. We want to make sure it's a device that will last a while since insurance only covers them once every five years. So first we have to get her to understand how to make choices via the device, and how to use her hands to manipulate it.

We're also discussing the possibility of getting her a powered chair. Mobility will always be an issue for her. I have no doubt she will learn to walk, but she will never have the stamina and energy that a healthy child has. It's important for her to feel empowered about her mobility and to be able to move around with the control in her environment that typical children her age would have. Of course, being in a powered chair opens up a whole new world of complications - adapting the vehicle for a lift, deciding when and how to use the chair, and so on.

Thursday, after speech therapy, she had a bit of a rough afternoon. When we got home, she decided she wanted to explore outside, so I let her crawl around on the porch while I cleaned up the area. She was standing behind one of the chairs, holding on to the back, when I think she stepped back and lost her balance on the edge of the porch and fell back - pulling the chair on top of her. She cut her eye open, and we weren't sure how deep it was, so I took her to the ER to see if it needed stitches. It didn't, so we had a fun 2 1/2 hours sitting around waiting for them to clean it up, slap a couple steri-strips on it and send us on her way.



On Friday, she stayed at home with dad while the boys and I went to the beach with friends. Brianna had therapy at noon, and we wouldn't be back in time. It's good she didn't go because it was a fiasco of a day (though we did have fun at the beach), though that's a story for the family blog. =) Her dad said therapy went great, she was really interactive and excited. It's really amazing to see the positive changes in her.

Today was a little rough. She had a seizure this morning - which usually affects her mood and energy afterwards. I made a bad decision to take her in the stroller with her brothers and walk over to the arts and crafts fair about 1/2 mile away. It was a windy day and a lot of people. She was getting a little upset, so I tried to put her on the horse for a pony ride. She actually rode really well, I didn't even have to support her. But she was really upset and cried the whole time. I think she would have loved it if it hadn't been a bad day. She proceeded to continue to melt down, so I had her dad come pick her up and take her home. Where she was fine. *sigh* Bad mom.

So now she's taking a nap, and hopefully she'll wake up feeling better!



Here you can see she's figured out how to use the remote and what it's for, even if she doesn't know which buttons to push. She wasn't a fan of Kai Lan and wanted to turn it to Mickey. Smart girl!!!

Best's Disease

So we had gone to a pediatric opthamologists because a few of Brianna's therapists have asked about her vision. On the first visit, the opthamologist, Dr. Young, said her vision appears to be fine. However, he noticed some unusual "cysts" on her retina. He asked about any vision issues that run in the family, at the time I was unaware of any. So we wanted to arrange further exploration of her retinas, which will require sedation. So we wanted to coordinate that with an MRI due and have the pediatric ophtomologist at Tripler take care of it. And he wanted to see us back in a month to see if anything had changed.

Upon further investigation I found that there is a history of vision issues in our family, specifically Best's disease. We went back last week and I mentioned that to Dr. Young. And he said that he believed what he saw resembled Best's, but he's never seen a child Brianna's age present with it. There's a good chance that we may have to go to the mainland (again) to investigate it further, as there are no retina specialists on Hawaii. Typically Best's disease starts between 3-15. Stages one and two aren't visible on the retina, but stage three appears to be like an egg yolk. Stage four, where vision may be effected, appears to look like scrambled eggs. It appears Brianna would be at stage three, which she is incredibly young to have progressed so far - in both eyes.

Best's disease may or may not effect vision. There's no real way to tell if it will. Typically, if it does, vision isn't affected until individuals are in their 40s. And it typically still allows those who have the disease to still read and drive.

Best's disease appears to be unrelated to her PDCD. She seems to have drawn some short sticks with "unrelated" disorders, since this Best's and her craniosynostosis weren't directly related to the PDCD. However, I wonder if maybe the PDCD accelerated the rate of the progression of her disease.

I'm praying hard that it won't effect her vision. It seems so cruel that with all she's suffered, that her ability to see would be yanked away as well. I try so hard not to be angry, I know that she doesn't miss what she's never had, so it's not as hard for me to accept that she doesn't walk or talk, or do things that "normal" kids do. But she sees, and she loves observing the world. And to have it taken away from her, with her having no ability to understand why, truly would break my heart.

I do try to be like Job, and not question what God "allows" to happen in our lives. He knows a greater plan and his brush strokes a greater picture than I can ever imagine. But there are times that, like David, I wonder why God allows things to happen. My faith isn't shaken, but sometimes my resolve is.

So I continue to pray for my Brianna, this precious little light. And I also pray that God will continue to give me strength. To bear me up and give me the tools and the wisdom to know how to help my little girl on her journey of life, and to also provide the love and attention to my incredible little men.

Thank you again for sharing our journey. And please continue to pray for our family. God bless.

Ketogenic Diet

We started Brianna on the ketogenic diet last week. It's a diet typically used for seizure disorders not controlled by medication. However, since her body doesn't process carbohydrates, the theory behind using the ketogenic diet for PDCD management is that it isn't flooding the system with carbohydrates that can't be processed. We started on Thursday, so today is day four.

Despite the fact that she has a wicked sinus infection that is requiring antibiotics (she'd been fighting it for over three weeks), she is making amazing progress. A month ago she was making progress, but her energy levels were incredibly low. She seemed constantly tired. We were really noticing an increase in autistic-type behaviors. More stimming, more sensory problems. She would curl up in the fetal position anytime we were around other people. Even people she had previously been comfortable with.

Four days after we started the diet and today at the opthomology office she was crawling around "jabbering" to people. Her energy levels have definately increased. This weekend at a party she was actually comfortable being around people - quite a few people. Amazing steps for her. I was so afraid that we had lost a part of her. She used to be quite smiley and social towards people and that had just seemed to disappear. Now it's back.

Tonight I was watching "Extreme Makeover: Home Edition." Everytime the group on the tv would applaud, Brianna would pause in playing with her toys and clap. I don't know why, but it just brought tears to my eyes. It just seemed so "normal".

I love this little girl so darn much.