A coordinated transition model for patients with cystinosis: from pediatrics to adult care

Nefrologia. 2016 Nov-Dec;36(6):616-630. doi: 10.1016/j.nefro.2016.05.012. Epub 2016 Aug 30.
[Article in English, Spanish]

Abstract

Introduction: Improved outcome and longer life-expectancy in patients with cystinosis, and disease complexity itself, justify planning a guided-transition of affected patients from Pediatrics to adult medicine. The aims of the process are to guarantee the continuum of care and patient empowerment, moving from guardian-care to self-care.

Methods: review of articles, expert opinion and anonymous surveys of patients, relatives and patient advocacy groups.

Results: elaboration a new document to support and coordinate the transition of patients with cystinosis providing specific proposals in a variety of medical fields, and adherence promotion. Nephrologists play a key role in transition due the fact that most cystinotic patients suffer severe chronic kidney disease, and need kidney transplantation before adulthood.

Conclusion: we present a document providing recommendations and suggesting a chronogram to help the process of transition of adolescents and young adults with cystinosis in our area.

Keywords: Adherence; Adherencia; Adolescent; Adolescente; Cistinosis; Cystinosis; Kidney transplant; Transición; Transition; Trasplante renal.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Cystinosis / therapy*
  • Humans
  • Kidney Transplantation
  • Pediatrics
  • Renal Insufficiency, Chronic / therapy
  • Self Care
  • Transition to Adult Care*
  • Young Adult