Purpose: Medical advances have allowed for the transmutation of cystinosis, a rare disease, from fatal in childhood, to chronic and manageable into adulthood. Arnett's theory of Emerging Adulthood suggests that the ages of 18 to 30 are a time of exploration, instability, and possibility. How do individuals with cystinosis, and their families, navigate this time in their lives?
Methods: Adult men and women living with cystinosis, and parents of adults with cystinosis, were recruited online via advocacy organizations. Focus groups and/or semi-structured interviews were conducted with: 9 women (ages 18-39) and 12 men (ages 18-47) with cystinosis, and 12 mothers and 12 fathers. The study utilized a classic Grounded Theory approach.
Findings: Adults with cystinosis, and their families, recognize the reprieve that changing medicine has provided. Indicators of recognizing the reprieve include: passage of time; outliving prognostication; finding a fit; and living in/valuing the now. Participants live with cystinosis in three contexts which contribute to the transition to adulthood and adult-oriented care: individual, family, and community. Families utilize several strategies to support overall survival, treatment adherence, self-management, and independence. Individuals and families negotiated connected autonomy, allowing for identity exploration and independence from parents and providers, with their continued support. Progression of illness and a demanding medication regimen underlie much (but not all) decision-making. Participants described themselves as "incomplete adults". "Being in-between" childhood and adulthood was reflected in descriptions of the institutional and practical barriers to transition from pediatric to adult-oriented care.
Conclusions: This research provides deeper understanding of how individuals and families live with cystinosis, from childhood through adolescence, emerging adulthood, and adulthood. The challenges and benefits of healthcare transition and illness self-management during emerging adulthood were identified and can be connected more clearly to Arnett's domains of emerging adulthood. The conceptualization of recognizing the reprieve is a new one, which suggests how individuals and families value medical advances and how they find a fit for illness within their lives. Direction for further study includes the experience of other rare/chronic diseases in emerging adults, as well as the experience of being a member of a rare/chronic disease community.
|Commitee:||Kaskel, Frederick, Videka, Lynn, Werner-Lin, Allison|
|School:||New York University|
|School Location:||United States -- New York|
|Source:||DAI-A 74/10(E), Dissertation Abstracts International|
|Subjects:||Social work, Medicine, Individual & family studies|
|Keywords:||Chronic illness, Cystinosis, Emerging adulthood, Health social work, Rare disease, Transitioning|
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