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. 2015 Feb;30(2):281-91.
doi: 10.1007/s00467-014-2921-5. Epub 2014 Aug 27.

That eagle covering me: transitioning and connected autonomy for emerging adults with cystinosis

Maya Doyle  1 , Allison Werner-Lin
Affiliations

That eagle covering me: transitioning and connected autonomy for emerging adults with cystinosis

Maya Doyle et al. Pediatr Nephrol. 2015 Feb.

Abstract

Background: Rare diseases pose transitioning challenges owing to limited provider expertise and changing healthcare systems. The timeframe and developmental changes of emerging adulthood overlap with the transition of patients with cystinosis from pediatric to adult-oriented healthcare.

Methods: This study utilized techniques of qualitative grounded theory to explore the experiences of adults aged 18-47 with cystinosis, and their parents, with a focus on the transition to adulthood and adult-oriented care. Forty-six individuals from 21 families were recruited online and at cystinosis conferences to participate in focus groups and/or individual interviews. The constant comparative method was used to conduct both line-by-line and focused coding of verbatim transcripts.

Results: The following elements were reported to be critical to the transition to adulthood and adult-oriented care: gaining skills and responsibility for disease management, progressing toward autonomy while remaining connected to caregivers, and having strong communication with and between providers.

Conclusions: Data analysis identified behaviors and relationships that support and/or threaten autonomy and treatment adherence. Participants described institutional, relational, and practical barriers to transition. Suggestions for improving transitioning include: identifying patient/family strengths and improving pediatric-adult provider partnerships and communication. Further research is needed into the experience of patients before and after transition to adult-oriented care.

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Figures

Fig. 1
Fig. 1
Timelines for adult participants with cystinosis: time to diagnosis, first and second transplant, transplant to present, and death, as of 2012. Note: after taking part in two focus groups, participant P047 died in 2012, after qualitative data collection had ceased. The parents of P34 contributed information, participating in a focus group and family interview, the year following the participant’s death.
See this image and copyright information in PMC

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