Article

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.

Langue : ANGLAIS