Parental experiences of scoliosis management in Rett syndrome

Background. Scoliosis is the most common orthopaedic complication of Rett syndrome. Parents of affected individuals are vital partners in the clinical management of scoliosis and this study explored parental experiences of various aspects of different management options. Methods. Publicly available Rettnet postings informed the development of an online questionnaire about scoliosis and its management in Rett syndrome. Parents of subjects who met the criteria for Rett syndrome participated in a survey using this questionnaire. Results. One hundred and eighty families participated in this study with scoliosis having developed in 135 (75.4%) of subjects. Eighty-four (62.2% of subjects with scoliosis) had received specific treatment for scoliosis while 51 (37.8%) had not. Surgery was perceived as improving the scoliosis in the majority of subjects but had considerable emotional effects for families of subjects who were less severely affected (p = 0.055) or older (p = 0.063). Physiotherapy and bracing were perceived as not reducing the progression of the curve, but physiotherapy was frequently reported to be beneficial to the subject's quality of life and bracing was frequently associated with side effects such as decreased mobility and problems with pressure. Only half of respondents felt that information about scoliosis provided by clinicians was adequate. Conclusions. The perspectives of parents provided useful insights into the complexities of decision-making regarding scoliosis treatment in Rett syndrome. The provision of scoliosis information by clinicians should be more family-centred.