Splints, Other Steps to Ease Hand Stereotypies Common With Rett
People with Rett syndrome are likely to undergo an intervention at some point in their lives to ease their characteristic hand stereotypies, or continuous and purposeless hand movements, a Japanese study reported.
Factors associated with this included age at diagnosis, walking difficulties, and the frequency of hand mouthing. Further studies can help in identifying approaches that work best for patients and caregivers according to their individual needs, its scientists said.
The study, “Comparison of the presence and absence of an intervention to reduce hand stereotypies in individuals with Rett syndrome,” was published in the Journal of Applied Research in Intellectual Disabilities.
Limited hand skills and repetitive, purposeless hand movements — called stereotypies — are characteristic of Rett syndrome. Hand stereotypies have been reported to cause skin injuries and joint contractures (a shortening and tightening associated with deformity) that aggravate feeding and limit self-care.
Interventions to help manage these symptoms, often assistive devices, have been reported to help ease hand stereotypies.
However, it is unclear how many Rett patients have undergone such interventions for stereotypies and which factors promoted their use.
A team of researchers in Japan analyzed the responses to a questionnaire conducted as part of an observational study with families from the Japan Rett Syndrome Association and Rett Syndrome Support Organization in Japan.
The questionnaire, sent to 194 families, focused on general information — such as age, sex, interests and age at diagnosis — and included questions into patients’ behavior, hand function, hand stereotypies, and therapeutic interventions given.
A total of 71 families (36.6%), accounting for 72 Rett patients (one set of twins) with a mean age of 12.8, responded. Patients’ mean age at diagnosis, reported by 69 respondents, was 3.3 years, and most (97.2%) were female.
All patients showed some form of hand stereotypy — with hand mouthing being the most frequent (58.3%), followed by clapping or tapping (40%). Hand squeezing/flicking was the least common (5.6%).
Hand stereotypies appeared at a mean age of 2.5, according to 62 respondents. Their frequency was determined to be continuous (100% of the time) by 25.7% of the 70 respondents to this question, almost continuous or 75% of the time by 35.7%, and half of the time by 20%.
Of 68 Rett syndrome patients, most — 49 or 72.1% — “were currently receiving or had received an intervention to reduce hand stereotypies,” the researchers reported.
The most common intervention was the use of elbow, hand and finger splints. Others included massages, hand holding, and sensory play.
When comparing patients who had undergone an intervention (either currently or in the past) with those who did not, the researchers observed several factors that separated the groups.
Intervention patients were significantly younger at the time of the study (mean age of 11 vs. 14.5 for non-intervention patients) and at diagnosis (2.5 vs. 4.2). They showed a significant decrease in stereotypic hand movements and better locomotion and reaching function than non-intervention patients.
They also exhibited more hand mouthing and clasping/posturing movements than non-intervention patients, whose hand stereotyping was most commonly hand squeezing or flicking movements.
Joint and elbow joint contractures were observed more often in the intervention group, and a significant difference in current problems was evident between groups as well, with 43.7% of intervention respondents reporting current problems compared with 29.6% on those not using an intervention.
Overall, these results show interventions for hand stereotyping being used with a high proportion of Rett patients in Japan. “Interventions for stereotypy-associated problems are important and there are several variables related to whether an intervention is received,” the scientists wrote.
Future studies are needed to “identify which interventions improve the quality of life of participants and caregivers according to their individual needs,” they concluded, noting high levels of stress reported by parents of children with Rett syndrome.