摘要：Introduction. Prader-Willi syndrome (PWS) is a rare genetic disorder characterized bysevere hypotonia during the neonatal period and the first two years of life, the onset ofhyperphagia with a risk of obesity during infancy and adulthood, learning difficulties andbehavioral or severe psychiatric problems. This complex disease has severe consequencesand difficult management issues also for patients’ families. Parents of children with PWSneed appropriate psychoeducational intervention in order to better manage their childrenwith PWS. The purpose of this study was the implementation and evaluation of aPWS psychoeducational parent training program.Methods. The Italian National Center for Rare Diseases implemented a pilot parenttraining program offered to parents of children with PWS. The intervention’s effects wasevaluated using questionnaires comprised of 11 items rated on a 7 point Likert scale.Results. The intervention was offered to 43 parents. The behavior problems management,dietary restrictions, autonomy and relationships were indicated by parents as the prioritytopics which needed to be addressed. Evaluations, immediately post-intervention andafter 6 months, were reported by parents, fulfilling specific questionnaires. 90% of parentsinvolved in the study, appreciated the methodology, 86% felt more informed about PWS,47-62% felt more capable to better approach behaviour’s problems, 20-25% felt betterabout the child’s health situation and future expectations. Feeling more capable to helpthe child autonomy and relationships were reported in 62% and 63% of parents respectively,which decreased significantly (p < 0.05) according to the evaluation 6 months afterthe intervention. Younger age of parents (< 44 years of age) was significantly correlatedwith better understanding on how to help the child’s autonomy (OR: 0.05; CI: 0.04-0.8)and to better collaborate with the child’s teachers (OR: 0.02; CI: 0.001-0.9).Conclusion. Parent training is a promising intervention for parents of children withbehavior’s problems. Interventions with a behaviorally oriented program, addressed toparents of PWS affected children, is a useful tool in increasing their ability to manage theproblems related to the disease.
关键词：Prader-Willi syndrome; rare diseases; parent training; parents; evaluation program
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