Sunday, April 13, 2014

We Flexed One HECK of a Muscle!

Yes, this is my annual Muscle Walk post.  

This year was incredible.  Ya'll absolutely blew me away.  Our biggest group, our highest total raised, and our most donors ever.  48 of us showed up to Bridgestone Arena in Nashville,  ready to walk in support of Andrew.  

This whole group of caring, loving hearts showed up for my son.  No one drove less than 45 minutes and some of them drove as many as 7 (and no, they aren't relatives) - all for Little.  I can't articulate to you all how much that meant to me.  We were the second or third largest group there.  Our group was so large, that we couldn't fit in the photo area!  We just squished together at our meeting point and handed my camera to a total stranger.  :)

 I am so touched that all of you came.

Over 100 people donated to our team this year!  Our goal was $2630.01 (a penny more than we raised last year) and we took exactly $4,000 to Muscle Walk.  That's not all, though!  The donations kept coming in even after the walk and our grand total raised was:
That's more than double my original goal!!!!!!!!!!!!!!!!!!!!!!!!  I still cannot believe how much ya'll gave!  Do you know how many hours of research that money will fund?  How many wheelchairs that money can repair?  That money WILL play a part in finding a treatment for Duchenne.  THANK YOU, THANK YOU, THANK YOU!  Out of 15 (or more) teams, we raised the 4th highest amount of money.

 Little Boy was a crankpot for the whole thing.

This itty one is Ian.  Ian is wearing Andrew's original Little's League onesie from our very first Muscle Walk. BTW - I need that back, Desi.

 Linda Decker, our fearless leader!

And finally, he's excited (we were waiting for the elevator so we could we leave).

This will surely be my "year to beat" in both participants and funds.  I know every year can't be like this one, and it will hold a special place in my heart for the rest of my journey with Duchenne.

With so many people on our side and in our corner, I have no doubts about how Little and I will be able to walk this path.  Every night, when I pray with him before bed, I thank God for all of the people who love us and support us and I ask Him to let all of you know how much you are appreciated through my actions.  I fail, miserably and often, but I hope that even when I'm being a snot, you all know just how much I genuinely appreciate you.  I wrote a note of thanks to my congregation that my preacher read from the pulpit.  Since all of Little's League doesn't attend my church, I want to share an amended version of it here.  It's for all of you, not just my family at Barker's Mill.

I want to thank you all from the bottom of my heart.  You're outpouring of love and support for me and my son was never more evident to me than at this year's Muscle Walk.  To the 48 of you who showed up to walk for Andrew, I am humbled and blessed to call you our League.  When the cure for my son is finally discovered, it will be because of you.  Thank you.


Monday, January 6, 2014

Adaptive Equipment Thus Far (and tips on how to procure your own)

Little Bit is in his last few days of the terrible, horrible, no good, very bad threes.  I have my fingers and toes crossed that the fours will be better.  In his short little life thus far, he hasn't needed too many pieces of adaptive equipment.  I thought I'd share them all here, along with how we went about procuring them, in the hopes that it helps someone else out in figuring out how to aide their special needs tot.

***Here is my mandatory disclaimer stating that I am not a physician or physical therapist and am in NO WAY qualified to diagnose or recommend treatment options for anyone's child!  I am simply putting out there what we have used and how it has helped Little for you to use as a reference or inspiration or completely ignore or to use however you see fit.***

1.  Hip Helpers:  spandex shorts with a sewn up seam in the center to tighten up hypotonic hip joints.   He wore these for a few months when he was just about a year old, at the suggestion of his physical therapist.  Andrew sat wide legged, with his legs out in a v and these helped to bring his legs together closer to regular hip distance.  They can also help with w sitting and hip rotation in babies with hip abduction.  I did not go through insurance for these, as they were only $16 per pair.  In this picture, Little is wearing them in a size C.  I still have them, if anyone needs a pair in that size.  Click the link above to make sure your baby needs a C and check with his/her pediatrician or physical therapist to see if these are a good fit for your child and let me know via e-mail ( or facebook (link on the right) or by leaving a comment below.

2.  Cascade 3.5 D.A.F.O:  He got fitted for these when he was about 13 or 14 months old.  We were able to get them through our insurance via primary care physician's referral to a pediatric orthopedist.  Cascade makes many different dafos and the 3.5 is the model that both his PT and his Ortho agreed was best for him due to his hypotonia and supination.   Vocabulary lesson time (HOORAY!);

hypotonia  hy·po·to·ni·a (hī'pō-tō'nē-ə)
 A condition in which there is diminution or loss of muscular tonicity, resulting in stretching of the muscles beyond their normal limits.

supination  su·pi·na·tion

1. rotation of the hand or forearm so that the palmar surface is facing upward (opposed to pronation ).
2. a comparable motion of the foot, consisting of abduction followed by inversion.
3. the position assumed as the result of this rotation.

 He began wearing them every time we wanted to try to get him to walk and  once or twice per day for an hour to stretch.     He no longer walks in them, but we are still using the 3.5s (in a larger size, of course) at home to stretch his calves and feet when they are particularly tight.

The casting process.  Look at that little squishy baby!!!!!

Unfortunately, they don't stop wee ones from tipping over.
Finding shoes to fit over these suckers can be difficult and expensive.  I think Little was in a size 4 shoe in these pictures but those New Balance were a size 6 double wide.  I got them at Sears.  You could also go the route of the Hatchback Shoe, which is a special order shoe that has a hinge in the middle.  The shoe bends open in the center so you can slide your AFO or DAFO in then pop the backside of the shoe up over it.  Pro - easy to get on over the orthotic.  Con:  ugly and expensive.  Unless of course, Hatchback wants to send me a pair for free to try out and review - then they're stylish and affordable!  ;)
3.   Potty Rail: 

Built for him by his Pappy.  I wanted a seat he could sit down on and raise himself off of without help and without falling.  This rail has been the perfect solution for him.  If your child has weak thighs or balance issues and is unable to use the adaptive potty seats that fit over a regular toilet (Little is unable to walk up the ladder type steps and then turn himself around on the skinny platform on the medical potty seats available), you might consider something like this.  Inexpensive and easy to make!  His school even had one made just like it to accommodate him in the bathroom located in his classroom. 
4.  Tiger Helmet:   to show off fierceness 
Kidding, of course.
5.  Baby Bjorn:  because they are an AMAZING company with an equally amazing product.  You can read about how we got our grubbies on this one by checking out the post I wrote at the time - ERGOmaniac
When Little outgrows it, I will be passing it along to another family in need.  I haven't decided how I'll choose - but I'm thinking some kind of contest.
6.  Handcuffs:  because, well, toddlers are jerks.

 Again, kidding.  Don't call CPS.

 7.  Special Tomato Height Right Chair:  This is a chair we use in the cafeteria at school. It took the place of old ratty booster seat that was tethered to a scoop back chair.  The Tomato allows Little to sit at the lunch table with the rest of his class and not feel like he is in a "baby seat" or high chair.  The padding all comes off if I need to wash it and the foot plate and seat are adjustable.  I was able to get this 100% paid for by my insurance through a referral from his PCM.  The referrals necessary to his insurance company were to a medical supply company (Pennyrile Home Medical in our case) who came out to the house and took his measurements, recommended products, and wrote their own referral paperwork.  That paperwork and a second referral from our PCM written out for this specific chair were sent into our insurance company who then approved the order.  After word of the approval, the medical store then places the order for the chair.  It only took about two weeks for it to arrive.

8. Ottobock Kiwi Pediatric Mobility Stroller:  This was procured the exact same way the Special Tomato Height Right Chair was.  Keep in mind that the sun canopy and under carriage storage bag are both considered unnecessary and optional, so many times insurance will not cover them. 
Andrew has outgrown our umbrella stroller, which doesn't have enough support for him anyway.  We went to BabiesRUs and tried out a few of their displays and he was too wide or long for all of them!  Little is TINY - so I'm not sure what the deal was there.  The ones I liked all had oval or rectangular style push bars instead of handles, so I was excited to see that option on most of the pediatric strollers.  I like this particular one because it looks like exactly what it is - a stroller.  Some of the other styles just looked so...medical.  We'll get to that one day, but for now, I wanted one that looked like a really nice stroller and that's exactly what I got. 

It is super smooth to push; even Andrew has no trouble with it. I think my favorite feature is the directional push bar - I can push him in the usual forward facing direction, or if I get backed into a corner or he falls asleep, with one quick push of a button, I can flip the handle bar over the seat to push him in the opposite direction (seat facing me). That will definitely come in handy exiting crowded elevators where there is no room to turn the stroller around! Plus if he is asleep, I can pull down the sun canopy to block people out and keep him facing me for privacy.

The front wheels swivel or I can lock them for going over gravel/cobblestone. There is a zippered pouch on the back plus an undercarriage bag. The only thing it is missing is a cup holder (which I've already added) and unfortunately it takes up my entire trunk some have to remember to leave it at home when I grocery shop. Anything requiring the stroller and luggage or purchases or anything else will mean I'll have to use my husband's car, which means switching over the car seat.

But I am so SO grateful and excited to have it!!!

9.  Cascade 4.0 D.A.F.O.:  this is the one we'll be getting shortly (as in the very next time we go to his PCM).  Andrew's rotation has switched from supination to pronation as his ankle muscles weaken. 


1. rotation of the hand or forearm so that the surface of the palm is facing downward or toward the back (opposed to supination ).
2. a comparable motion of the foot consisting of abduction followed by eversion.
3. the position assumed as the result of this rotation.
4. any similar motion of the limbs or feet of animals.
He will wear these while at school or whenever he will be doing any kind of extended walking.  I have no idea what size shoe he'll have to wear over them, but I can bet it'll be another double wide.   We'll be headed over the brace shop for yet another fitting in a few weeks.  He's an old pro at them by now. 
10.  Physical Therapy and Occupational Therapy:  really, I should have listed these first since he's been doing them since he was 13 months old.  We started off with our states free birth to three, early intervention program called Kentucky 1st Steps.  His PCM put in a referral and KY 1st did the rest.  They came out to my home for every evaluation and session from the time he was 13 months to 3 years old.  At 3, 1st Steps turns you over to the school system for services.  Andrew has been in preschool since Jan 2013, a few days after his third birthday.  He receives 30 minutes of PT and 15 minutes of OT each month.  That is not enough for him, but it is all the state can provide.  Because of that, our incredible therapists kept him on as a private client (again, try your insurance - the worst they can do is deny you.  You'll never know unless you submit a referral).  He now receives PT twice per week for 45 min each time and OT once per week for an hour on top of what the school can do for him. All four of his therapists are on the same page - I even brought his private PT to his IEP meeting at the school last month.  The referrals constantly have to be renewed, and evaluations resubmitted, but every bit of paperwork and waiting room time is worth it to have the services he needs.
And that's it so far!  I think we're incredibly fortunate in that we haven't needed a whole lot yet and that we've been able to get everything we have needed pretty easily.  I know how blessed we are to have this amazing insurance plan through the Army and I also know that we will not have it forever.  In fact, it may be coming to an end pretty soon, but that's a blog post for another time.  I hope I've helped some of you out a little bit, or given you a bit of  insight for what services and equipment you might consider for your son or daughter in the future.  I am always on the lookout for new products to make Little's life a bit easier ( I choose to be proactive) and as I come across them, I will continue to pass the info on to you.
I hope you all had a wonderful holiday season and I wish you a year full of blessings!  Put your faith in Him and God WILL provide!  Proverbs 3:5- "Trust in the LORD with all thy heart, and lean not on your own understanding: in all your ways acknowledge Him, and He shall direct your paths."
*thank you, Pintrest for the sweet tree/car idea*

Wednesday, November 13, 2013

Gratitude in the Face of Disappointment

It's November; that means it's time for FB's annual 30 Days of Gratitude status updates.  This is the second year I've joined in.  I think it's good to take a minute and express your thankfulness about the blessings in your life.  We have SO much to be thankful for.  God has been so good to us.  

So today makes my second post in a row about derailed hope in the Duchenne community.  Last time, I wrote about how GSK's phase III trial on the exon skipping drug, Drisapersen, failed to meet it's endpoints.  Well today, I write to let you know that the competing pharmaceutical trial, Sarepta's exon skipping drug eteplirsen, was just denied accelerated approval from the FDA.  In a nutshell, the FDA thinks the trials were too small and the data not strong enough to move forward on.  They've asked Sarepta to regroup and try a new Phase III trial with a larger group of boys and place half of them on a placebo.  Sigh.  Duchenne is such a rare disease, that it may not be possible to find that large a number of boys who qualify.  The second problem is that no one will want their child to be in the placebo group.  So we've really been punched in the ribs on this one.  It will take years longer now to get this drug out to all the boys who need it.  Years that we don't have.  This is a rapidly progressively deteriorating disease.......the math doesn't add up. 

  The FDA basically just told us all that most of our boys are going to die or have muscles that are too wasted to be helped by the time this drug is available (if it is ever available).  So in the wake of all these setbacks and heartaches, what am I thankful for today?  THE FDA.

Yep, those guys.  They have an incredibly difficult job, seeking out which drugs to give the green light to and which ones to send back to the lab.  I don't envy the FDA for a minute.  I applaud them for doing the best they can with the data they are given.  I may not always agree with them, but I don't work there.  I don't have a full grasp of what approving a drug entails.  What I do know is that the FDA hasn't denied eteplirsen because they hate people with muscle disease, they've denied it because it didn't meet the criteria they judge new therapies by.  This is not a forever denial, it is a "not now, go back and try again and give us new data first" denial.    Unfortunately, with Duchenne, it means more boys will pass and more boys will decline.  This delay may quite possibly cause Little to progress too far to benefit from the therapy by the time his formula is ready.  And that sucks.  Big fat hairy donkey balls.  But if regrouping and reformatting the trials are what ultimately lead to drug therapies that save future DMD kids - well then. 

I was told 2 years ago that my son would never get better, that he'd die before he was legally able to buy his first beer.  I've come to terms with that.  I don't want it - but I understand that's the way this genetic condition plays out.  I've accepted that my son will not lead the life I had envisioned for him while I was pregnant.  The realist in me takes whatever glimmer of light is out there with a grain of salt.  Yes, when things looked like they were working out for Andrew to be one who received a drug therapy formatted to his mutation, I got excited.  I praised God.  But my prayers have always said,  "God, if it be Your will, then....."  I have always known that these drugs might not come in time to save my son.  If they ever do, I will dance with the joy of a thousand angels in my heart, but I will not survive this journey if I don't stay grounded and humbled.  And today, with the news that none of those drugs have Little's name stamped on them at the moment, I still praise God.  He has given me so much.  He gave me Andrew.  He is letting me keep him right now, when He could have allowed a miscarriage or a stillbirth to "ease" my life and heartache - save ME from a life with Duchenne.  But instead He allowed my son to come to be.  He is allowing me to love Andrew for as long as he is on this earth.  How blessed am I?

And when the time is right, the formulas are right, and the data is there - the FDA will approve a therapy for Duchenne.  I know something is coming, even if it is not in my son's lifetime.  And I am grateful.