Rett syndrome affects girls and is characterized by progressive motor disablement, loss of acquired skills, seizures and autism. Scoliosis is also a commonly seen as a complication and this study set out to describe its prevalence, classify spinal deformity, and evaluate associations between disability and scoliosis.
29 patients took part in the study, ages ranging from 4 to 33.
The study found that nearly 90% of those who took part had radiographically verified scoliosis, with the median curve being 41° They found that function was poorer in patients with C-shaped curve
Using the Barthel index (10 items measuring daily functioning for activities of daily living and mobility) the function level in females with Rett syndrome showed significant correlation to scoliosis curve type
Spinal deformity and disability in patients with Rett syndrome, Riise R et al, in Developmental Medicine & Child Neurology, 53: 653–657.
Having looked at the Barthel index it seems to me that a more useful study may have been one that focussed on implementation of postural care for these individuals.
see http://www.posturalcareskills.com
Janet
Thanks for the link to the postural care skills site. It’s a good site full of really useful individual stories and helpful advice.
I think your point about focusing on implementation raises for me two important questions – firstly, how the research agenda is set and whether people who may benefit from research findings are as well involved as they could be in setting the priorities. Secondly, how we might improve the ways in which what we already know can be better and more evenly implemented.
john
It would certainly appear that a measure of body symmetry would be appropriate.
We work with a number of girls and women who have a diagnosis of Retts Syndrome. As the person becomes less mobile during the day you see a correlation in terms of their night time movement. This loss of movement during the night leads to habitual lying positions becoming obligatory as gravity has a moulding effect on the body, as described in Hill, S. and Goldsmith, J., ‘Biomechanics and Prevention of Body Shape Distortion’, The Tizard Learning Disability Review, Vol. 15, Issue 2, pgs. 15 – 29, 2010.
When you consider that a child or young person spends an average of three times longer in bed than in school the use of person centred therapeutic positioning at night may well have an important role to play in preventing scoliosis in girls and women with Retts Syndrome.
Thanks for your comment Sarah,
Your point about the ratio of time spent in bed to time spent in school is a helpful reminder to consider person centred approaches to support for sleep and therapeutic positioning.
Link to the Tizard Review article: http://pierprofessional.metapress.com/content/j551q55930328542/
john