Parenting stress in families of children with Prader–Willi syndrome

Abstract

Prader–Willi syndrome (PWS) is a neurodevelopmental disorder characterized by multiple endocrine, metabolic, respiratory, cognitive, and behavioral/psychiatric symptoms that may lead to severe emotional strain in their caregivers. In this study, we evaluated parenting stress by the Parenting Stress Index‐short form (PSI/SF) and parent‐reported behavioral symptoms by the Child Behavior Checklist (CBCL/6–18) in families of children with PWS. Sixty‐seven home‐resident PWS patients and their families were recruited in this study. The patients' mean age was 14.9 ± 8.3 years, and 33 (50.8%) were male. High parenting stress was reported by 41.5% families, as determined by high total stress scores of PSI/SF. The patients in high stress families were significantly older than those in low stress families (18.2 ± 8.0 vs. 12.6 ± 7.8 years, p = .007). CBCL/6–18 was used to evaluate the somatic and neuropsychiatric symptoms of PWS patients aged between 6 and 18 in the subgroup of the 35 families. In this subgroup, 37.1% of families reported high parenting stress. High stress families reported a higher T‐score in anxiety/depression, withdrawn behavior, somatic complaints, thought problems, attention problems, and delinquent and aggressive behavior of their children with PWS. After multivariate stepwise logistic regression analysis, the T‐score of somatic complaints was the only factor related to high parenting stress, with an odds ratio of 1.279. Our data demonstrated the high care burden of families with PWS and highlighted the importance of having dedicated medical care for both somatic and neuropsychiatric symptoms.

Exploring the characteristics and most bothersome symptoms in MECP2 duplication syndrome to pave the path toward developing parent‐oriented outcome measures

“…Strati et al showed the altered gut microbiota in Rett syndrome individuals is likely secondary to impairment in MECP2 functioning (Strati et al, 2016 ). Wong et al studied the parental distress in children with Prader Willi syndrome (another neurodevelopmental disorder) and they found that somatic symptoms were the only factor related to high parenting stress (Wong et al, 2021 ). More than one‐third of subjects in our cohort required second‐tier medications to manage constipation (i.e., not responding to dietary modifications or first‐tier medications).…”

Dynamic Changes in the Quantitative Electroencephalographic Spectrum During Attention Tasks in Patients With Prader–Willi Syndrome

“…In this study, we used the behavior task (CPT-3) to demonstrate attention problems in patients with PWS. Although ADHD is a common comorbidity in patients with PWS, the diagnosis discussed in previous studies has mostly relied on parent-reported questionnaires ( Wigren and Hansen, 2005 ; Wong et al, 2021a ). Our study escalates the behavioral evidence of ADHD in patients with PWS and opens the window to study genotype–phenotype correlations between ADHD and specific genetic dysfunction of PWS, which may improve treatment.…”

“…Because attention is an important predictor of intelligence ( Schweizer and Moosbrugger, 2004 ), it is difficult to find a control group of patients with only an intellectual disability and no symptoms of ADHD. In addition, both intellectual disability and attention problems were stressors for families of PWS ( Wong et al, 2021a ); therefore, abnormal CPT-3 performance of patients with PWS indicated their significant functional deficits and highlighted the importance of dedicated medical and social support.…”

“…Hyperphagia and lack of satiety are cardinal symptoms of PWS, which often lead to morbid obesity if the food environment is unsupervised ( Miller et al, 2011 ). Moreover, the characteristic neurobehavioral symptoms of PWS impose severe caregiver burden and stress for families ( Kayadjanian et al, 2018 ; Wong et al, 2021a ), with attention problems being highly prevalent in these patients ( Gross-Tsur et al, 2001 ; Wigren and Hansen, 2005 ; Wong et al, 2021a ). Genetic defects of PWS result in widespread developmental abnormalities of brain structures ( Manning et al, 2018 ) and neurotransmitter systems ( Luck et al, 2016 ; Wu et al, 2020 ), which are strongly associated with the pathogenesis of attention problems ( Faraone et al, 2015 ).…”

Quality of Life in Children with Prader–Willi Syndrome and the Impact of the Disease on the Functioning of Families

“…Parents unequivocally report fear for their child’s future, considering not only the management of further treatment, but also considering social acceptance. There is a relatively small number of researchers investigating the quality of families in which a child has PWS, and examining the impact of the disease on family functioning [ 36 ].…”