Physiological adaptation after a 12-week physical activity program for patients with Prader–Willi syndrome: two case reports

Abstract

BackgroundPhysical activity programs are a powerful tool against several diseases including obesity and their comorbidities. Prader–Willi syndrome is the most common genetic disease associated with obesity, and brings with it behavioral and emotional problems that need complex management. Research into the effect of physical activity programs on Prader–Willi syndrome is limited and it is frequently argued that if a physical activity program is too complex, the participants are more likely to drop out. Therefore, in this study, we assessed the physiological adaptation effect of a physical activity program with increasing complexity and load, in a boy and a girl with Prader–Willi syndrome by assessing changes in lipid profile, body composition, and physical fitness parameters.Case presentationCase 1 was an 11-year-old girl, mixed race (brown), with an intelligence quotient of 68, 52.0 % body fat, and a body mass index of 45.3 kg/m2. The Prader–Willi syndrome diagnosis was made when she was 5-years old and was found to be due to an imprinting genomic defect. Case 2 was a 14-year-old boy, mixed race (brown), with an intelligence quotient of 74, 48.8 % body fat, and a body mass index of 37.3 kg/m2. The diagnosis was made when he was 10-years old and was found to be caused by gene deletion. Both participants presented physical characteristics and behavior problems typical of Prader–Willi syndrome. Case 2 presented high blood pressure, high cholesterol and sleep apnea and had to use continuous positive airway pressure to sleep. Both participants were assessed for 12 weeks (three times a week) using a physical activity program designed to improve strength and muscle hypertrophy. The work load was progressively adjusted as necessary and new exercises were added to the program. Prior to the program, the participants’ parents received instructions about managing problem behavior and advice about nutrition.ConclusionsAfter physical activity program several health markers assessed by biological tests and parental report had improved in both participants. The participants positively accepted the adaptations made to the physical activity program during the study. More studies are necessary to assess the benefits of physical activity in the Prader–Willi syndrome population.

Hand strength and dexterity in patients with Prader–Willi syndrome: a pilot intervention study

“…Our training program was not long term and was of a lower intensity than previous study programs. 13 – 15 , 18 , 40 To accommodate the PWS Association timetable, we provided one 2-hour treatment per week for 12 weeks, which might have been insufficient to achieve significant hand muscle strength and dexterity improvements. Future studies should consider the use of higher intensity and higher frequency muscle strength training, such as once per day for 12 weeks, or the addition of simple, effective, and feasible home-based training programs.…”

“…Following previous studies 14 , 15 and in line with the timetable of the PWS Association, a 12-week training program was conducted with a small group of six participants. To ensure compliance and safety, participants were supervised and guided by three trained and certified occupational therapists.…”

“… 7 , 12 Moreover, physical activity in patients with PWS has a substantial influence on the growth of lean body mass, 13 gross motor ability and general physical function, such as body composition, muscle strength, fitness levels, motor quality, and exercise tolerance; the use of GH treatment and physical training to improve these factors has been investigated. 14 – 19 …”

Increasing physical activity in adult women with Prader–Willi syndrome: A transferability study

“…Participation in exercise sessions was excellent, as well as the satisfaction from both participants and their families. Studies that implemented home‐based exercise training programmes, either supervised or non‐supervised, usually reported high participation in exercise sessions compared to programmes based outside the home (Amaro et al, ; Capodaglio et al, ; Rubin, Wilson, Dumont‐Driscoll, et al, ). Instructors involved in this programme reported they had to adapt to the participants’ psychological and cognitive disorders and physical limitations associated with PWS, and sometimes to patients’ fatigue and lack of motivation.…”

“…Due to the specific impairment of executive functions and psychopathological features in this syndrome, integration of an exercise programme as part of daily routine along with individual interaction with health professionals might be especially important (Chevalere et al, ). Two case reports suggested that a home‐based exercise training programme supervised by a PA instructor was feasible and well‐accepted by children with PWS, although mixed findings were reported on changes in body mass and body composition, habitual PA and physical fitness (Amaro et al, ). A recent controlled study conducted in youth with PWS reported that a home‐based exercise training programme facilitated by parents improved muscle strength and gross motor skill proficiency and decreased body mass in subjects who experienced an increase in objectively measured MVPA (Rubin, Wilson, Castner, & Dumont‐Driscoll, ; Rubin, Wilson, Dumont‐Driscoll, et al, ).…”