Thursday, August 30, 2012

Sweet Sounds

This past Monday, we went to see Dr. Miller up at Shands in Gainesville. Dr. Miller is Aerin's endocrinologist and is truly a stellar example of how doctors should practice medicine. It is obvious that she has a real heart for her patients, some 500 babies, children and adults with Prader Willi Syndrome. She was thrilled to see how much Aerin had grown and celebrated all of her achievements with us in such a way that we could tell that Aerin was almost as much "hers" as she is "ours". To have a doctor that is so much invested in your child's future is priceless.

We talked about starting solids soon (ack!) and getting going on more aggressive speech therapy since Aerin hadn't started babbling yet. It is one of the strangest features of this syndrome, this near silence. I set my alarm each night to wake up for feedings and have to crank up the baby monitors to hear her shuffling in bed when she wakes up from a nap because she makes no noise otherwise.

Speech delays are a common issue in PWS. Many children develop speech apraxia and remain nonverbal for a long time.  It is another hurdle that we face, not knowing if it will affect Aerin or how severe it might be.

And, as always, the Lord met us at that place of fear, hesitation and uncertainty. The next day she spent "talking" her little heart out! It's so precious to see her trying out her lips and cheeks, working against the low tone that weighs her every muscle down to form sound. When she watches us now, she intently watches our mouths as if to crack the code.

I'm just so proud of her, I could burst!

Wednesday, August 15, 2012

Rock 'n Roll

This week started out really ROCKy. I've just been struggling so much with Aerin's developmental delays that it's really been weighing me down. My head knows that this is par for the course with PWS, but my heart just breaks over and over again for her sweet, frail little body. One of the key features of PWS  is hypotonia, or low muscle tone. I find that this is a really hard concept to understand, especially as it's not as simple as "her muscles are weak". I found this great article on what it means to have low muscle tone and the PT who wrote it was able to describe it within the context of us regular-toned folks:

Muscle tone is the resting state of your muscles.  When a child has low muscle tone it means that they need to put a lot more energy into getting their muscles to turn on to do what they want them to do.  I often try to explain this to parents by describing that feeling when you finally get to sink into the couch or your favorite arm chair and relax and then someone calls you from the other room and you have to rev up the energy to get up.  Think about having to do that every time you move because that’s what it can be like for kiddos who have low tone.

Basically, having low muscle tone means that Aerin has to work at least twice as hard as everyone else to complete even the most basic of movements. Aerin has what is called a "global delay," meaning (among other things) that she is very late in meeting her milestones. She is weakest in her trunk and upper extremities, which is why she still can't lift her head at 4.5 months or raise her arms to swat at or grab at a toy. Her lower body is stronger--in fact, she's got quite a kick and I can't wait until she can swim, she'll knock it out of the park.

It's just so very hard not to compare her to other sweet babies and, at those times, the discouragement is so very tangible it hurts.

But, as always, when there is a question God is prepared with His answer. Our answer came in the form of our physical therapist. We've been seeing her regularly for a couple of weeks now and she is very sweet with Aerin and very encouraging to me. She is very proactive and we are already getting Aerin a pair of hip helpers to address Aerin's severe hip abduction (i.e. her legs splay outward like a frog when she is laying down and this can lead to several problems later, not the least of which is hip displasia). We are going to order a set of bamboo braces to help support Aerin as she learns to push up on her hands while on her tummy. And she is going to start investigating whether or not a spio suit will help give Aerin more proprioceptive feedback all over her body, thus encouraging her muscles to engage and work more. And these are just the various orthoses that are in her plan to help Aerin reach those milestones in a more timely manner. She also has established a thorough program of "exercises" that we do with her each day to help push Aerin towards her full, ever-blossoming potential.

So, we are getting there. Slowly but surely. I have to constantly remember that Aerin is on her own timeline and no one else's. It's not fair to her for me to constantly be comparing her to others when she is marching right along to the beat of her own little drum. The Lord has made a way for my daughter and she will follow it on His and her terms, not mine or the world's.

All of that being Aerin ROLLed over for the first time! We put her on her tummy this morning for some tummy time and she just plain decided that she wasn't gonna have it, so she just popped herself right back over to her back where she was just fine, thank you very much. Yes! She ROLLed!

It's just like my Lord to ROLL away the ROCK I find myself facing. <3


Thursday, August 9, 2012

We're still here!

Well, it has been eons since my last post. I'm starting to think that blogging will be a lot like journaling for me--bejeweled with great intentions that are not often seen through to completion!

We have survived the summer! I remember when we finally came home from the hospital: I was pumping 8 times a day, we were bottle and tube feeding Aerin, running off to countless doctor and therapy appointments, and my two big kids needed TONS of love and attention. I had no idea how we were going to manage with daddy working 12 hour shifts! Well, needless to say, we watched a lot of tv this summer and were very blessed by many friends who hosted all of the playdates that kept the four walls of our house from closing in on us. School started on Wednesday which provides a measure of relief from the craziness for all of us--especially since we are blessed with a wonderful school family and great teachers.

Aerin is doing so well. We haven't used her G-tube for a feeding in 21 days (and prior to that it had only been twice in 14 days--so I am totally counting those two weeks as well)! Honestly, we didn't know what to expect when we brought her home and thought it would be many, many months before we would reach this milestone. The Lord is so faithful!

She is a busy little beaver. Since my last update, she had a baseline EEG that showed some slowing of the brain waves on the back of her brain. This is the same area where she had excess fluid on her brain immediately after birth. Her neurologist opted to do a CT scan to follow up on this and the original MRI done in the NICU. The CT scan showed that Aerin has enlarged first and third ventricles, probably from that excess fluid, and we are going to just watch her carefully over her first year to make sure that it does not progress into hydrocephalus. Typically this is a symptom that is outgrown by the age of one--definitely the outcome we are praying for.

She has physical therapy 2-3 times per week and oral motor therapy every other week. We have been blessed, again, with wonderful therapists. They are all very much in Aerin's corner, pushing her towards her potential and cheering her on. Physical therapy is the hardest right now (for me) since she still has a significant delay in her gross motor skills. Never before have I realized how much I took for granted with K & A. Aerin works so hard to do the most basic things: kick her legs, raise her arms, lift her head. She has definitely gotten stronger, but she still has so far to go and I can't help but feel a little overwhelmed and discouraged at times.

One of the unfortunate side effects of her gross motor delays has been developing plagiocephaly, or flat spots, on her head. We are in the process of adding yet another doctor to our "Team Aerin"--a neurosurgeon who will evaluate her to see if she is a candidate for a helmet to help reshape her head before her skull finally fuses and hardens. In the grand scheme of things, this is relatively minor. But it feels a little like a blow to our forward progress as it is yet another issue we have to tackle.

Having Aerin is like being in an Olympic training program for mental self-discipline. I am constantly having to fix my thoughts on those things which are "...true, and honorable, and right and pure and lovely and admirable." (Philippians 4:8) All of the dark, discouraging and frightening thoughts hover perilously close to my heart and it is a constant battle to keep them at bay. I admit that this was a hard week for that and I have spent more than my fair share of time in tears.

But, I come back to the line of my favorite Matt Redman song (for now anyways):

You're rich in love and you're slow to anger
You're name is great and you're heart is kind
For all your goodness I will keep on singing
10,000 reasons for my heart to find.
(from 10,000 Reasons)

And I know that I always, ALWAYS have 10,000 reasons to rejoice.

On that note, I'll leave you with a little video of my sweet girl and her giggle...