"I'm scared of being like mum": The experience of adolescents living in families with huntington disease

Abstract

Background: The debilitating and very visible motor effects of the incurable, progressive, and fatal neurodegenerative condition Huntington disease (HD) are accompanied by more insidious cognitive, behavioural and personality disturbance. The usual age of HD onset exposes children and adolescents to the natural history of the condition as it affects a parent. This group of young people has been largely overlooked in most research, which has concentrated upon the experiences of affected individuals and their partners. Objective: This study explores the psychosocial context of young people living in families affected by HD, to better understand their experiences and the specific challenges they face. Method: Ten young people from five unrelated families affected by HD separately participated in semi-structured individual interviews. At the time of interview, nine were less than 18 years of age, and none had requested a predictive genetic test. Results: The young people demonstrated a depth of insight in their descriptions of complex and often painful family circumstances. In addition to the tasks and challenges associated with typical adolescent development, young people from families affected by Huntington disease recognize that they face greater responsibilities and stresses. Conclusion: This study highlights areas of unmet needs for young people living in families affected by HD. Best practice HD care should include consideration of the needs of young people in the family, and offer developmentally appropriate HD education, prospective orientation to genetic services, and psychological and social support.