Scoliosis

I can still remember our trip to Philly and the FPWR conference like it was yesterday.  It was our first time taking a very LONG road trip as a family of three.  The weekend was filled with instant hope and encouragement.  I knew that everyone I met that weekend "got" us and understood what we were going through along our journey with PWS.

During this weekend, we got the awesome opportunity to hear, Dr. Harold vanBosse present with an amazing PT, Janice Agarwal.  It was the first time I learned more about scoliosis related to PWS, and instantly became concerned about Ellie.  I knew we needed to call her pediatrician, and request a baseline X-ray as soon as we returned home.

"Children with Prader-Willi Syndrome have an incidence of developing scoliosis at rates between 40 -90%.  Approximately15% of children with Prader Willi Syndrome will develop severe or significant curves, requiring bracing or surgery" (Reprinted from The Gathered View (ISSN 1077-9965), published bimonthly by the Prader-Willi Syndrome Association USA).  Dr. vanBosse discussed that there were two peak ages that scoliosis could be presented in children with PWS - under 4 years old, and over 10 years old.  Younger children can be at risk due to their hypotonia (low muscle tone).  Dr. vanBosse recommended children with PWS have their first X-ray when they are able to sit up independently, typically around their first birthday.  Ellie was 10 months...to the pediatrician we went once we returned home.

Our pediatrician was awesome about listening to our request and what we learned at the conference.  She ordered an X-ray right away.  It ended up that we were able to have the initial X-ray done in the office right away.  I stressed to her that it had to be an X-ray where Ellie was sitting up.  Many medical professionals choose to lay children with PWS down to X-ray, and their curves can go undetected.  David and I were able to see the X-ray come up as soon as they took it, and immediately I saw it...a curve.  My heart sank.

We were referred to an orthopedist and, ultimately Shiner's Hospital for Children.  What an amazing place. They go above and beyond there, and took great care of us.  Ellie had three X-rays. Her first curve was 17 degrees to the left.  Her second curve was 12 degrees to the right.  I don't even remember the last degree of the curve, but all I remember was the doctor said Ellie doesn't have scoliosis, but rather had postural issues related to her hypotonia.  We will continue to be followed by Shriner's, and continue to monitor her back. She does many exercises to strengthen her core, abdominal muscles, and back muscles.  Hopefully, we can enroll her in hippotherapy soon, (therapy riding horses) which has been proven to be very effective in helping build core strength and combat scoliosis.  We were elated that Ellie didn't have scoliosis, but I couldn't help but think about our many friends that have children dealing with this.  Their children undergo surgery (which is so scary with PWS) and have back casts placed every 2 months.  It is such a hard transition because many of these children are learning to crawl, walk, and run...it feels like a set back, I'm sure.  But, you will see, these children that have PWS have a common thread...they are the epitome of bravery, strength, and determination.  They don't let anything get them down.  Scoliosis doesn't stop them from crawling, walking, running, or being active.  And, they do all of this with a smile on their face.  Inspiring...

This is one of Ellie's good buddies, Haven.  She goes the Shriner's Children's Hospital for scoliosis.  This picture was just taken after she got her new cast and is getting some love from the therapy dogs.  Look at this strong, happy girl!