These resources include the best available evidence about the course of Rett syndrome and its management as well as practical information about family associations, specialist clinical centres and links to relevant websites.
Ager S, Downs J, Fyfe S, Leonard H. Parental experiences of scoliosis management in Rett syndrome. Disability and Rehabilitation. 2009;31(23):1917-24. Download snapshot, see journal abstract.
Scoliosis is the most common orthopedic issue for girls and women with Rett syndrome. In this study, we investigated what parents thought about the different ways scoliosis can be managed and what parents thought they needed to help them better manage their daughter's scoliosis.
What we did
We developed a questionnaire about scoliosis and its management in Rett syndrome and invited parents with a daughter with Rett syndrome to participate in this study through the listserv Rettnet and postings to Rett syndrome associations around the world. Families from Australia, the US and Europe participated in this study by completing the questionnaire online. Using the information provided, we looked at parents' perceptions on the effectiveness and side effects of different management options and information provided to them.
What we found
Of the 180 families who were involved in this study, 135 (75.4%) girls had developed scoliosis. We found that most of these girls had received one or more treatments for their scoliosis. These included bracing (40%) , physiotherapy (30%), alternative therapies (13%) or surgery (27%).
Parents felt that bracing and physiotherapy did not prevent progression of scoliosis. Bracing did help ability to sit for some girls but was sometimes problematic causing less mobility or was uncomfortable. Physiotherapy was perceived as helping with spinal flexibility, mobility, posture and comfort.
Of the families whose daughters had had surgery, all but one parent felt that it had improved their daughter's scoliosis. Nearly a third felt that their daughter's mobility had improved but almost a half felt that was unchanged and 20% felt that it had worsened. Even though parents were satisfied overall with surgery as a treatment for scoliosis, there was a considerable emotional strain associated with the surgery, especially for parents of girls who were more mobile or older. Families who lived in urban or rural locations were similarly satisfied with the management of their daughter's scoliosis.
About half of families were satisfied with the information about scoliosis they received.
What it means
It is important that we understand parents' views on this topic because they are important partners in the management of their daughter's scoliosis. Our results suggest that there is room for improvement in providing more information to parents about for the management of scoliosis in Rett syndrome.