High levels of caregiver burden in Prader-Willi syndrome

Abstract

ObjectivesPrader-Willi syndrome (PWS) is a rare genetic neurodevelopmental disorder that is characterized by hyperphagia, developmental delay, incomplete sexual development, mild-to-moderate intellectual disability, and a variety of challenging behavioral and psychiatric symptoms. The characteristics of PWS can be difficult for caregivers to cope with and are likely to cause significant and long- term caregiver burden. The current study examined burden in 142 caregivers of children and adults with PWS living in the US using the Zarit Burden Interview (ZBI). The study aimed to measure the level of burden in caregivers of individuals with PWS, to explore the impact of PWS on caregiver quality of life, and to assess ZBI as an indicator of that impact.ResultsCaregivers participating in this study were predominantly mothers, 30–59 years old, non-Hispanic Whites, married or in a relationship, with an annual household income slightly distributed towards higher income. Nearly 90% of the caregiver`s children with PWS lived at home. Caregivers experienced high caregiver burden with an average ZBI score of 44.4 ± 15.4. ZBI scores were highest for caregivers of teenage and young adult individuals with PWS (49.2 ± 14.6 and 49.2 ± 14.1, respectively), while those caring for older adults (>30) and the youngest age group had lower scores (38.6 ±10.5 and 34.8 ±12.5, respectively). Caregivers reported that caring for a person with PWS negatively impacted their romantic relationship, ability to work, sleep, and mood. Whereas we did not find strong correlations between family income or level of help the caregiver receives and ZBI scores, the results showed significant correlations and a linear relationship between ZBI scores and caregiver depressed mood, feelings of anxiety, negative romantic relationship impact, as well as sleep and work disruption.ConclusionsOur study reveals that PWS incurs high caregiver burden and impacts many aspects of the lives of caregiver. We identified the ZBI as a good predictor of that impact. Our findings draw attention to the critical unmet need for support for caregivers of individuals with PWS.

The Optimalization of Family Coping n Caring for Mental Retardation Children through Family Psychoeducation in Jombang

“…The anxiety felt by the family in caring for children with mental retardation was related to the dependence, development, and future of mental retardation children. This was consistent with study which stated that families with mental retardation children felt anxious about the children's future [11] and felt fear related in caring for children with mental retardation when the family has been died [6]. Through this session, families were taught how to overcome anxiety with relaxation techniques or deep breathing.…”

“…Parents who have children with mental retardation experience depression about the uncertainty of the child's future and the length of time of child will depend on parents [6].…”

Current and emerging therapies for managing hyperphagia and obesity in Prader‐Willi syndrome: A narrative review

“…The life‐threatening drive for food in PWS is a source of stress not only for individuals with PWS but also for their caregivers. When compared with caregivers of individuals with disabilities, those who care for adolescents and young adults with PWS have reported the highest level of caregiver burden due to the need to strictly control patients' food intake . Patients may engage in relentless food‐seeking behaviours, such as stealing, foraging, and food hoarding; hence, interventions often emphasize restricting food access with behavioural modifications …”

Requirements for improving health and well‐being of children with Prader‐Willi syndrome and their families

“…Delays in diagnosis and accessing ongoing treatments and supports leads to substantial parental stress in many rare genetic disorders . A high prevalence of child sleep disturbances leads to insufficient and disrupted sleep for caregivers, compounding the management of daytime challenging behaviours . Caregiver demands in PWS also include intensive supervision due to the need to limit access to food .…”

“…Appetite dysregulation, most notably hyperphagia, and the difficult behaviours exhibited by individuals with PWS necessitate high levels of supervision, environmental management and behavioural interventions from caregivers each day. Ongoing vigilance and management can lead to higher levels of distress and reduced quality of life for caregivers of individuals with PWS compared to caregivers of individuals with other chronic congenital conditions or the general population . This review will describe PWS, focusing on appetite dysregulation, respiratory function and sleep disordered breathing and challenging behaviours, and will explore their implications for child and family well‐being and quality of life.…”