title: Activity and participation in children with neurofibromatosis type 1.
abstract: We describe activity and participation in children and youth with neurofibromatosis type 1 (NF1), and compared an intervention and control group after a strengthening program using the Pediatric Outcomes Data Collection Instrument (PODCI) and the Children's Assessment of Participation and Enjoyment (CAPE). Questionnaires were filled out by parents at baseline, 12-weeks, and 1-year. The intervention group performed a strengthening program twice a week for ten weeks, followed by a 9-month independent program. Thirty-six participants (18 control, 18 intervention) between the ages of 5- and 18-years (mean 10.6 years, SD 4.6 years) were enrolled, and 34 completed the 1-year assessment. There were significant differences between formal and informal participation (p<0.0001) in baseline CAPE scores for the entire cohort. At 12 weeks, PODCI upper extremity function improved in intervention and decreased in controls (p=0.040), while happiness declined in intervention and increased in control (p=0.003). There were no significant differences between control and intervention groups in any of the CAPE or PODCI change scores from baseline to 1-year. Upper extremity function, sport and physical function, comfort/pain and happiness PODCI scores were lower than normative values. The NF1 cohort had low participation in formal active physical and skill-based activities. The companionship and location dimensions suggest participation occurs with family and other relatives in the home or a relative's home and reflects a pattern of social isolation from peers.
authors.affiliation: Shriners Hospitals for Children Salt Lake City, Salt Lake City, UT, United States. Electronic address: bjohnson@uwlax.edu.
authors.forename: Barbara A
authors.initials: BA
authors.lastname: Johnson
authors.affiliation: Department of Pediatrics, University of Utah, Salt Lake City, UT, United States.
authors.forename: Xiaoming
authors.initials: X
authors.lastname: Sheng
authors.affiliation: Shriners Hospitals for Children Salt Lake City, Salt Lake City, UT, United States.
authors.forename: Amber S
authors.initials: AS
authors.lastname: Perry
authors.affiliation: Department of Pediatrics, University of Utah, Salt Lake City, UT, United States.
authors.forename: David A
authors.initials: DA
authors.lastname: Stevenson
keywords: Activity; Children's Assessment of Participation and Enjoyment; Neurofibromatosis type 1; Participation; Pediatric Outcome Data Collection Instrument