The aim of the study is to evaluate the feasibility and health-related effects of an individualized 12wk 'uptime' participation intervention in girls and women with Rett syndrome. Girls and women above 5 years of age with Rett syndrome and a confirmed MECP2 mutation will be included. Each individual program focuses on participation in enjoyable activities to promote 'uptime' in home, school/day center and community settings. Primary outcomes are sedentary time and daily steps. Secondary outcomes are gross motor skills, walking capacity, quality of life and participation-level goals. Outcomes are evaluated on four occasions: at baseline and after a 6-week interval, immediately following the 12-week intervention program and 12 weeks after the intervention program.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
OTHER
Masking
NONE
Enrollment
14
The intervention are designed for each individual and are consistent with the model for participation-based interventions. The interventions are goal-oriented (meaningful and family/client-selected goals), family/client-centered (promoted self-determination and decision making), collaborative (partnership between caregivers), strength-based (built on participant and caregiver strengths) and ecological (natural environment and real-life experiences). Design of the intervention comprised three steps: 1) Preparation period (6 weeks); 2) Intervention period (12 weeks); 3) Follow-up period (12 weeks)
Center for Rett syndrome, Department of Paediatrics and Adolescents Medicine
Copenhagen, København Ø, Denmark
ActivPAL
Change in sedentary time
Time frame: Assessed four times; twice at baseline; once after the 12 week intervention and once after a further 12 week follow-up
Stepwatch Activity Monitor
Change in daily step count
Time frame: Assessed four times; twice at baseline; once after the 12 week intervention and once after a further 12 week follow-up
Rett Syndrome Gross Motor Scale
Change in gross motor skills
Time frame: Assessed four times; twice at baseline; once after the 12 week intervention and once after a further 12 week follow-up
2 minute walk test
Change in walking capacity
Time frame: Assessed four times; twice at baseline; once after the 12 week intervention and once after a further 12 week follow-up
Quality of Life Inventory - Disability, questionnaire
Change in quality of life. The Quality of Life Inventory-Disability was used to assess quality of life. This 32 item-questionnaire was developed for children 5-18 years of age with ID. Responses are used to calculate an overall score and six subscale scores (social interaction, positive emotions, physical health, negative emotions, leisure/outdoors and independence), each scored on a 0-100 scale with higher scores representing better quality of life.
Time frame: Assessed four times; twice at baseline; once after the 12 week intervention and once after a further 12 week follow-up
Goal attainment scaling
Change in participation level goals. Goal attainment scaling is a validated method for evaluating achievement of individual goals. Individual goals were assessed on a five-point rating scale ranging from -2 to +2. Baseline was set at -2, the expected level after the intervention at zero and the most favorable outcome at +2. The goals were graded in relation to frequency or duration of an activity.
Time frame: Assessed twice; at baseline and after the 12 week intervention
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