Coping with Prader-Willi syndrome

• Published in: Journal of the American Dietetic Association 2002-04, Vol.102 (4), p.537-542
• Main Authors: GOLDBERG, DENA L, GARRETT, CHRISTY L, RIPER, CYNTHIA VAN, WARZAK, WILLIAM J
• Format: Article
• Language: English
• Subjects: Adaptation, Psychological; Adolescent; Adult; Behavior modification; Behavior Therapy; Biological and medical sciences; Body Weight - physiology; Child; Childhood obesity; Company business management; Diet; Diet Surveys; Diet, Reducing; Exercise; Families & family life; Female; Food and nutrition; Health aspects; Humans; Male; Management; Medical disorders; Medical sciences; Mentally disabled children; Metabolic diseases; Obesity; Obesity - diet therapy; Obesity - etiology; Obesity - prevention & control; Obesity in children; Parent-Child Relations; Parents & parenting; Patient Compliance; Prader-Willi syndrome; Prader-Willi Syndrome - complications; Prader-Willi Syndrome - diet therapy; Prader-Willi Syndrome - psychology; Stomach, duodenum, intestine, rectum, anus; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the digestive system; Treatment Outcome; Weight control
• ISSN: 0002-8223; 2212-2672; 1878-3570; 2212-2680
• DOI: 10.1016/S0002-8223(02)90123-9

Weight- and behavior-control issues are major concerns for parents of a child with Prader-Willi syndrome. However, limited information is available on how families implement the necessary dietary restrictions and the effects of the strategies. This study identified the advice a group of families received regarding weight management, the nutrition concerns they faced and how they coped with these concerns, and the effectiveness of their coping strategies. A 2-step survey methodology was used. Survey 1 identified the strategies parents used to cope with the feeding issues typically presented by children with Prader-Willi syndrome. Survey 2 evaluated the frequency with which these coping strategies were used and their effectiveness. Respondents also provided information on why strategies were not effective. Surveys were mailed to 496 parents/guardians of children (aged 25 years or younger) with Prader-Willi syndrome. A total of 293 (64%) responded. Advice given to families centered on general weight management and dietary guidance. Difficulties centered around coping with food-related behaviors. Coping strategies varied; what worked for 1 family did not necessarily work for another. Participants indicated a desire to share experiences and a need for specific strategies to cope with feeding-behavior difficulties. A few basic behavior-management strategies, including successful use of incentives, responding to misbehavior, rewarding compliance with an exercise program, and modifying the behavior management when indicated, are briefly reviewed. J Am Diet Assoc. 2002;102:537–542.