Family-based Intervention for Youth With Prader-Willi Syndrome: The Active Play at Home Study

Compared to other children, those with disability have additional challenges to being
physically active. Prader-Willi Syndrome (PWS) is a genetic form of childhood obesity that
is characterized by hypotonia, growth hormone deficiency, behavioral, and cognitive
disability. In children, the low prevalence of PWS (1 in 10,000 to 15,000 live births) makes
group-based physical activity (PA) interventions impossible. In contrast, the home
environment presents a natural venue to establish a PA routine for this population. The
present high prevalence of non-syndromal childhood obesity (one in four) and high physical
inactivity rates, make alternative approaches to increasing PA in this population an area of
high interest. Specifically, approaches that involve the family have been identified as
possible areas where further research is needed. Therefore, we have developed a 24-week
home-based physical activity that could be suitable for children and adolescents ages 8-15
with PWS as well as obese children without PWS ages 8-11 years. It is hypothesized that: 1)
an age-appropriate 24-week home-based PA intervention will increase PA levels in youth with
PWS and without the syndrome but with obesity; 2) motor proficiency, central sensory
reception and integration, and body composition will significantly improve in youth with and
without PWS following completion of the home-based PA intervention and 3) physical activity
self-efficacy and quality of life will increase significantly in youth with and without PWS
who complete the home-based PA intervention. The study participants are 115 youth ages 8-15
y (45 with PWS and 70 without PWS but categorized as obese). The study utilizes a parallel
design with the wait-listed control group receiving the intervention after serving as
control. Participants are expected to complete the PA curriculum 4 days a week for six
months including playground games 2 days a week and interactive console games 2 days a week.
Parents are trained at baseline and then provided with a PA curriculum (Active Play at Home)
and equipment to guide their implementation of the program at home. Tips related to
scheduling and coping with barriers to daily program implementation are also included.
Throughout, parents are contacted by phone once a week (weeks 1-4) and then every other week
to provide support in between visits. Measurements of children and parent dyads are assessed
at baseline, at 12-weeks of receiving the intervention and at the end (week 24) of the
intervention or control periods. PA intervention compliance is monitored using mail-in daily
self-report checklists.

Inclusion Criteria:

- Having Prader-Willi Syndrome and being between ages 8-15 years. PWS status will be
documented by appropriate molecular and cytogenetic testing (i.e., chromosomes,
florescence in situ hybridization [FISH] 15, DNA methylation, DNA polymorphism
studies)

- Being obese and between ages 8-11 years. Obesity is defined as having a body fat
percentage greater than the 95th percentile (McCarthy, H. D., Cole, T. J., Fry, T.,
Jebb, S. A., & Prentice, A. M. (2006). Body fat reference curves for children.
International Journal of Obesity (Lond), 30(4), 598-602).

Exclusion Criteria:

- Obese children without Prader-Willi Syndrome currently on lipid-lowering medication,
diabetes medications, or blood pressure medications.

- Being pregnant