“Over time it just becomes easier…”: parents of people with Angelman syndrome and Prader–Willi syndrome speak about their carer role

Abstract

Purpose: This study investigated two of the stresses experienced by parents caring for offspring with Angelman syndrome (AS) and Prader-Willi syndrome (PWS) in Western Australia, and identified their coping strategies. Method: Parents of 19 offspring with AS and PWS participated in the Family Stress and Coping Interview which provides a stress level score, and discussion of stressors and coping methods associated with 24 life situations, two of which are reported. All text was examined using directed content analysis. Results: Family carers (14/19) reported high stress associated with the initial diagnosis of AS or PWS in their offspring; and finding time for themselves. Stressors identified included lack of quality information about the disorder, time constraints, and physical and emotional tiredness. Parents adopted a variety of coping strategies, including learning about the disorder, accepting the situation, seeking instrumental and social supports, and dealing with problems. Conclusions: No specific coping strategy was associated with reduced stress. However, parents felt that accurate and timely information during the diagnostic period helped. Parents used family and community support although there were difficulties accessing respite care. Government agencies, service providers, family members, and peer support associations should provide practical and emotional support to assist parents of offspring with AS and PWS, and indeed any form of intellectual disability, across the lifespan. Implications for Rehabilitation  Long-term caring for offspring with AS or PWS can involve considerable stress for parents.  Stress has been associated with poorer health outcomes for parental carers.  Parents need a variety of practical and emotional supports to cope with stress, including timely access to information.

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

“…Excessive food‐seeking behaviours have been negatively correlated with maternal life satisfaction ( n = 123; r = −0.25, P = 0.001) and are associated with higher levels of family stress . Greater availability of food outside the home environment and difficulties in accessing timely respite with carers who have an adequate understanding of PWS may also restrict social activities . Interviews with mothers of children with PWS ( n = 8) exploring parental stress and coping identified concerns related to meeting their own personal needs and difficulties co‐ordinating education, work placement and other supports from service agencies for their child .…”

“…Greater availability of food outside the home environment and difficulties in accessing timely respite with carers who have an adequate understanding of PWS may also restrict social activities . Interviews with mothers of children with PWS ( n = 8) exploring parental stress and coping identified concerns related to meeting their own personal needs and difficulties co‐ordinating education, work placement and other supports from service agencies for their child . Siblings of children with PWS may also experience substantial stress, and necessary supports for them have not been explored …”

“…There have been limited Australian studies of PWS . This gap is particularly relevant for Australian families with the rollout of the National Disability Insurance Scheme where caregivers must provide evidence of therapeutic benefit from supports and services to receive funding for their child.…”

“…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.…”

Historias de vida de familias con hijos e hijas con síndrome de Angelman

“…A pesar de que, según Griffith et al (2011), las familias de niños y niñas con síndromes genéticos raros han sido el foco de escasos estudios, existen varios que subrayan que su crianza puede resultar abrumadora y producir altos niveles de estrés psicológico percibido (Griffith et al, 2011;Miodrag y Peters, 2015;Thomson, Glasson, Roberts y Bittles, 2017). Wulffaert et al (2010) muestran que en general las madres con un niño o niña con SA perciben un alto estrés en la crianza.…”