05/14/2024
Prader-Willi Awareness Fact #14
Scoliosis (curvature of the spine) is another challenge which affects individuals with PWS. Children with PWS have an incidence of developing scoliosis at rates between 40 - 90%. It is strongly recommended that the child be seen regularly by an orthopedic doctor, and that yearly spinal x-rays be taken as soon as the child is able to sit independently. [Note: The 'bend over and touch toes" screening which done in schools or by pediatricians is generally not sufficient for monitoring scoliosis in children with PWS.]
If the curve progresses to more than 25 degrees, the first step taken is to have the child wear a brace. The child may wear the brace for all or just part of every day (my own daughter is currently wearing a brace 18 hours a day). The child does become accustomed to wearing the brace but there are comfort issues especially in hot weather, and having to wear the brace does limit physical activity. The child may also eventually undergo casting, which is exactly what it sounds like: wearing a cast around the torso all the time to attempt to limit the progression of the curve.
If the curve progresses to an increasing degree, respiratory function is compromised because the curve forces the body into a posture that affects lung function. At this point, spinal surgery is the next step; the individual undergoes spinal fusion surgery to basically freeze the spine into a permanent upright position. This surgery is delayed as long a possible to give the individual's growth plates a chance to close.
Gabby has scoliosis. According to the last x-ray, her spine went in an 's' shape. She has a brace she is supposed to wear.