Worn...but Thankful

One of my current favorite songs is Worn, by the band "Tenth Avenue North".  It is from their current album "The Struggle".  The title of this album, and the song Worn, hits so close to home.  In an interview with a band, Mike, the band leader, talked about a rough patch of time he and his wife had with their two young daughters.  Most parents can identify with the frequent wakings of babies, and how exhausting it is going through that period when it feels like the sleepless nights will never end.

Brianna's night-wakings have lasted for the entirety of her life (which will be five years in August).  There have been points where she only slept for about 3-4 hours total in a night (the worst stretch was about 2 months before Liam was born).  But generally speaking, there aren't many nights where she sleeps a good eight hours.  Most nights are like tonight.  She wakes up crying because of her congestion, not being able to breathe freely, around 230 am.  I sit with her for a while, usually giving her another dose of sleep medication, often a saline rinse, and just offering her some company.  She's usually appeased by sitting in her spot - the comfy recliner, watching Mickey Mouse Clubhouse.  She'll usually conk out there once the sleep meds kick in.  

Some nights are more difficult.  On those nights she is virtually inconsolable, requiring 30-45 minutes of soothing while she sobs hysterically.  Eventually she calms down, but on nights like that she's often up for hours.  Fortunately tonights waking appears to be a better night.  On the rougher nights I'm bouncing between consoling her and seeing to Liam, who still usually wakes at least once a night.

When her sinuses are clear, she goes to bed easily and sleeps through the night.  Unfortunately, most of the time, she struggles with congestion.  It has gotten noticeably worse the last year.  But she is scheduled for adnoid removal and ear tube placement surgery on March 5th.  We are hopeful that this surgery will clear up most of her sinus difficulties and allow her the sleep she (and we!) so desperately need.

But the thankful part!  Oh, THAT is the exciting part!  Brianna is walking!  She started taking a few short steps in October, before we left for Disneyworld (I can't believe I never did a blog post on our PDCD retreat trip!  Soon to come, I promise!)

Upon our return from Disneyworld at the end of November, she began to blossom.  She became more adventurous in walking between the furniture in our house.  And after Christmas, she positively took off.  Literally!  She now walks everywhere.  She walks into her classroom at school from the car.  She walks around the house.  She walks to the playground.  She walks in the backyard.  And yesterday, she was walking at the beach!  And not just walking, in the last few days she has figured out how to stand up unassisted!  She no longer needs to crawl to something to hold onto to stand!  God is working amazing miracles in Brianna's life.  Certainly due the work of her therapists, her own determination, but absolutely to the many prayer warriors who remember her routinely.  To you we offer our sincere appreciation!

The walking seems to have helped Brianna bloom.  She has a newfound confidence and has started coming out of her shell.  She babbles more, she seeks out other people.  She especially likes to be nearby other children (especially girls a few years older than her) when they are playing.  The autism part of her diagnosis is not as obvious these days.  We are truly blessed.

So even though we are often Worn, we are so Thankful.  We have experienced many challenges and struggles in the last 4 1/2 years of Brianna's life.  But we have also experienced great joy.  Pride in seeing her blossom.  She is unrecognizable from the tiny girl 2 1/2 years ago who couldn't crawl, who would scream if anyone not related to her talked to her, who was overwhelmed by everything in her world.  To this amazing little girl who is happy and attacking life with every ounce of energy she can muster.  What a blessing she is.

Reality Check

It's been a very long couple months.  Much going on.

Brianna has been battling sinus issues, for, well, forever.  It's been a wall meets head ongoing battle of what's going on.  Congestion due to reflux?  Congestion due to allergies?  It was frustrating, partially my own fault for not being persistent as to finding out what was going on (because from my perspective, it was something that was just always there until it was something more, and when you're dealing with a life-threatening mitochondrial disorder, the constant congestion doesn't seem like a big deal until it's a full-blown infection).

We left for our vacation/retreat (more on that later?) in the beginning of November.  Back in October Brianna was treated for a bad sinus infection.  At that time, random "fits" as we like to call them starting increasing in frequency.  Not really seizures, but something similar.  Certainly nothing she had control over.  I took it as a sign of something overall not going well with her system.  She took antibiotics for the infection, then after that course was done battled a horrible diaper rash (due to the antibiotics) which finally cleared up two days before our journey.  Not much of a reprieve for her poor system.  Fly to California, spend two nights, fly to Disneyworld for a week, back to California for several nights, drive to Nevada (four hour drive) for several nights, back to California for Thanksgiving weekend and fly back to Hawaii.  It was grueling for my healthy system, and was just too much for poor Brianna.

The whole ordeal just sucked everything out of her.  She was a trooper on the trip, though definitely not at 100%.  We got home and she just started shutting down.  It culminated with several trips to the clinic for sinus/ear infection that wouldn't go away.

Last Friday (November 30th), we were at the clinic and she was at her worst.  Lethargic, her coloring was very pale, and she was quite listless.  Not her usually chirpy self at all.  Her metabolic nurse took one look at her and with tears in her eyes said we were admitting her.  That was the lowest point, fortunately.  She got three injections of antibiotics, was definitely on the mend and on her way home the next day (Saturday), and now, a week later, is back to herself, full steam ahead.  Melting everyone's hearts as usual.

The reality check came in with our appointment with the developmental pediatrician yesterday.  I made the appointment hoping for direction on what I should do about expectations for her.  Mostly about how we deal with training her/discipline, how we go about that.  While her increased mobility and starting to walk is AMAZING, it also brings in a host of other concerns.

It was an illuminating appointment, I got quite a few recommendations and directions for resources.  But I also received a very large pill to swallow.  It was nothing that was particularly surprising - I live with my daughter, I love her, I take care of her every need.  I know her limitations, I know what makes her different and unique.  But hearing the diagnosis, hearing the worlds stated out loud, it's something else.

Mental retardation.

Pervasive Developmental Disorder - Not Otherwise Specified.  That's autism.  And not the functional kind.

I know that Brianna is in her own little world.  I know her eye contact is selective and brief.  I know that while she is four years old, she is more at the level of a 12-18 month old, and doesn't even interact with those around her like a toddler would.

But hearing the words.  Knowing what that means, not just now, but ten years from now, that's something else.  It's like a silence rushes over you, and you hear a very loud, a very decisive door slamming.

I know the platitudes: a diagnosis doesn't define her, she's amazing just the way she is, so on and so forth.

But unless you've looked at your child, hearing the words that make it very clear that your child not only will never live a normal life, but they will need constant and continuous care for the rest of their lives - even when you knew that, having someone look you in the eye and tell you that's what you're dealing with, unless you've been there, you can't even begin to possibly imagine.

A door shuts for your child.  But a door shuts for you as well.  You have one of two futures to look forward to: there will be no empty nest - you will be responsible for taking care of an adult who will be like a small child.  There will be no traveling, no enjoying your grandchildren and sending them home, at least not without the constant burden of untrusting your helpless child/adult to someone else's care while you go enjoy yourself - something impossible to truly do.  There will be no rest from this.

Unless, the unthinkable other future, which is that this precious soul, this forever child, won't be there to think about, to take care of.

You can't possibly imagine the guilt and burden of dealing with one of those two things.  With wondering which one you prefer and then feeling like a horrible person for even wishing that you could have "normal".  Of wondering if something happens to you if you will burden/trust your child's siblings to take care of them, or leave them to an institution.

All of these things, swirling around, and crashing down after a very matter-of-fact detail crunching appointment, where you acknowledge that you have to think about things that no parent ever thinks that they will have to consider.  I was the cool collected mom, dealing with the horrible facts with calm assurance.  And I came home, I clutched my precious little girl, with the most beautiful smile on her face and not a care in the world, and I cried into her hair and I wished it could be different.  Then I looked in her eyes, as she looked at me with her adoring eyes and complete trust, and I thanked God for this precious child.

There are very few people who can understand how you can thank God and scream "WHY?!" at him in the same thought.  But this is the life we have.  A life of profound thanks, and frustration, and beauty, and sorrow.  And an exhausted look towards the heavens asking God for the strength to make it through another day.

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!!!