Managing Prader–Willi syndrome in families: An embodied exploration

Latino Families with a Child with Prader–Willi Syndrome: Exploring Needs for Support

“…Families need to create certain coping mechanisms such as restricting access to food, keeping the individual with PWS occupied, and using a consistent routine, which requires the involvement and collaboration of all family members (Allen, 2011). Such intensive involvement and responsibilities could be challenging for families.…”

“…Among developmental disabilities, PWS has been recognized as the most common of the microdeletion syndromes, attracting the interest of genetic researchers because of its distinctive behavioral phenotype and multifaceted nature. Its medical and genetic implications have been fairly well researched, and there is a wealth of literature documenting the biomedical aspects of PWS (Allen, 2011;Rae-Seebach, 2010). People with PWS face food-related challenges, motor and speech impairments, impaired pubertal development, intellectual disabilities, and cognitive dysfunctions (Cassidy & Driscoll, 2009;Dykens & Roof, 2008;Hodapp, Dykens, & Masino, 1997;Holland & Wong, 1999;Kundert, 2008).…”

“…There is not much documented about caregiving challenges and everyday practices that are so crucial to the well-being of individuals with PWS and their families and these are often overlooked. Consequently, very little research has focused on the psychosocial emotional impact that PWS has on families, and particularly on siblings (Allen, 2011;Rae-Seebach, 2010). Further, even though there has been an increased interest in family systems research, there are only a few studies that mention the significance of sibling relationships in the context of disability (Lardieri, Blacher, & Swanson, 2000).…”

Treino parental para manejo comportamental de crianças com Síndrome de Prader-Willi: impacto sobre a saúde mental e práticas educativas do cuidador

“…The disease incidence is 1: 10,000 to 30,000 births 1.2. The cognitive and behavioral phenotype observed in people with PWS is characterized by frequent episodes of hyperphagia, stealing food, tantrums, lying behavior, mood swings, anxiety, sadness, aggression and auto aggression, stubbornness and repetitive speech, hypersomnia, compulsive behaviors, deficits in cognitive abilities such as attention and executive functions (especially inhibitory control, working memory and self-regulation) and deficits in visuospatial skills [3][4][5][6][7] . Of these described characteristic phenotypes, the problems most difficult to manage are hyperphagia; stubborn behavioral patterns; challenging, aggressive, oppositional and manipulative behaviours; food theft and lying, which become more frequent and severe with increasing age [8][9][10][11] . Parents and caregivers of people with PWS are routinely faced with two types of problems; the control of access to food to avoid and/or minimize other health problems (such as obesity, scoliosis, and cardiovascular and endocrine illnesses) 12 , and family burden, especially on the primary caregiver, due to the difficulties in the management of behavioral problems that start from early childhood 13,14,15 .…”

Feeding, eating and behavioral disturbances in Prader-Willi syndrome and non-syndromal obesity

“…So far, recommended measures for weight control in PWS amount to a strict calory restriction, close supervision, and regular exercise units [12]. Medical attempts to treat hyperphagia have been largely unsuccessful.…”