‘Uptime’ Physical Activities Aid Life Quality and Walking Abilities in Rett Patients, Study Finds

“Uptime” activities, such as standing and walking, that promote physical activity and work against a sedentary lifestyle can improve walking capacity and life quality in girls and women with Rett syndrome, a study reports.

The study, “Feasibility and Effectiveness of an Individualized 12-Week “Uptime” Participation (U-PART) Intervention in Girls and Women With Rett Syndrome,” was published in the journal Physical Therapy.

Rett syndrome is a rare genetic disorder that affects females almost exclusively and is characterized by developmental and intellectual disabilities. The disease is also associated with motor impairments, with some two thirds of the patients requiring assistance to walk or struggling to be able to walk independently.

Daily physical activity is low in most Rett patients, a problem that tends to worsen as they age.

“Reducing sedentary time and enhancing ‘uptime’ activities such as standing and walking could be an important focus for interventions … in RTT [Rett syndrome],” the investigators wrote.

Researchers with the Health Sciences Center at Lund University in Sweden and colleagues evaluated the feasibility and the effects of a 12-week uptime participation (U-PART) intervention on the quality of life of girls and women with Rett syndrome.

The intervention was a personalized program in which each participant was encouraged to take part in enjoyable activities at home, school, a day center or other settings. Feasibility was assessed using a study-specific questionnaire.

“During the intervention, all participants had access to 1 or more items of equipment (like switch-adapted aids) and 6 participants used a large aid (like a walker) and these aids were perceived as helpful to facilitate ‘uptime’,” the researchers reported.

Its primary goal was to evaluate the amount of time these people spent each day in sedentary activities and the number of steps they took. Secondary goals included assessing patients’ gross motor skills, walking capacity, and quality of life.

All these goals were evaluated twice at the study’s start or baseline (with an interval of six weeks between evaluations), immediately after the 12-week uptime intervention, and at a final follow-up 12 weeks after the program.

Sixteen people (eight who lived with their parents and eight in a residential home) were selected to for the intervention. From these, two were lost to follow-up and 14 were included in the final analyses. A total of 10 patients were ambulatory.

Answers to the questionnaire showed the uptime intervention was considered feasible by caregivers. Both caregivers and patients expressed positive feelings toward the program’s effectiveness, while two caregivers expressed a negative opinion due to its time constraints.

The U-PART intervention led to positive effects that ranged from small to medium in intensity.

When goals were assessed immediately after the intervention, ambulatory patients’ daily sedentary time was reduced by 4% and the number of daily steps taken rose by 689. But this increase in daily steps was not maintained 12 weeks later, at follow-up.

Ambulatory patients’ walking capacity increased by 18.8 meters and their quality of life, as assessed by the Quality of Life Inventory–Disability (QI-Disability), by 2.75 points compared to baseline. (QI-Disability is a 32-item questionnaire developed for children with intellectual disabilities.)

U-PART’s benefits in terms of lesser daily sedentary time and improved walking capacity in patients were still evident 12 weeks after the intervention.

“The U-PART intervention provided a systematic framework for the promotion of uptime within the natural environment of the participants that could be embedded within the individual’s existing support systems,” the researchers wrote.

“Other groups of children and adults with multiple disabilities might also benefit from this kind of intervention, however larger scale studies must be conducted to determine the health-related effects and cost-effectiveness,” they added.