Scoliosis

[tjwhite2]

Hey all MDC1A families!
I am wondering if any of you are dealing with scoliosis with your kids and what you are doing about it.  We have a 7 year old who has significant curves and we are looking into surgery.  Just curious what some of the older kids around have had done to prevent or correct.  Would love any info you have!  So glad to have all of you around to chat with about our kids!  You can reply back or feel free to email me at tjwhite2@frontiernet.net.
Thanks!
Tammy

[Ann]

Hi Tammy,
My son, Jack, doesn't have Merosin-Deficient CMD, he has dystroglycanopathy CMD. But, he did have a significant scoliosis (90+ degree - "C" curve) and had spinal fusion surgery at the age of 8.  He was fused from T-2 to sacrum.  Jack had a brace starting at about age 5, but he never used it successfully because it was to difficult for him to breathe with the brace. I felt like age 8 was young for the surgery, but it has definitely helped him.  Jack is wheelchair bound and ventilator dependent - the surgery helped with his sitting and his overall health. 

Ann

[anne]

Dear Tammy,

The goal is to wait with spinal surgery until after puberty hits to allow for spinal growth.

This is not always possible.

One of the orthopedic surgeons who worked on the CMD Care Guidelines, due to be published later this year, is extremely knowledgeable in scoliosis repair in CMD. He is in Toronto at Sick Kids.  We have helped arrange a second opinion on a child with CMD in the US.  Given that many orthopedic surgeons in the US have not repaired scoliosis in CMD they may not be aware of some of the key issues which he can address (approach, timing, dealing with limited mouth opening, dealing with breathing issues, where to lock in/fixate the rod).

One of the issues with scoliosis surgery in CMD- regardless of subtype is the large degree of blood loss in the operating room due to the extremely fibrotic and scarred paraspinous muscles (muscles on either side of the spinal column).  Anticipation is key, as is good surgical technique to "go after each bleeding vessel", which is something that may or may not be familiar and in the orthopedic surgeon's mind.

This like all decisions in CMD requires finding a doctor who is experienced with spinal surgey in muscular dystrophy, who is willing to engage in a dialogue about the "best time" and possibly getting a second opinion.

Best,
Anne

[tjwhite2]

Thank you so much for your replies!  Anne, we would love to get in contact with the doctor from Toronto and get him connected with our orthopedic surgeon.  They are going to schedule Isabelle's surgery to put a rod in for late July as her curve is now at 64 degrees in her brace.  Would really love as much info we can get!  Thanks so much for all you do!!!
Tammy

[tjwhite2]

Update on Isabelle,
We did go ahead and have a growth rod put in. She was at 85 degrees pre-surgery and is now at 45 degrees post surgery.  Please feel free to look at our caring bridge site as she is currently in the hospital recuperating.  If you read the journal, it shows her journey.
Thanks!
Tammy White
www.caringbridge.org/visit/isabellewhite