Moving young people with sickle cell disease from paediatric to adult services

Abstract

This article reviews data about transition from paediatric to adult services in patients with sickle cell disease, the most common inherited disease in the UK, and outlines how this has been addressed in a large UK sickle cell centre.

Improving Sickle Cell Transitions of Care Through Health Information Technology

“…Specific fears discussed in the literature and in the focus groups included loss of familiar and trusted providers, concern that adult care providers may not meet their needs, and fear about being treated as adults without adequate preparation, skills, or support. [13][14][15][16][17] The literature noted these fears are common and well founded for individuals with SCD for a range of reasons, including the dramatic increase in life expectancy for SCD patients in the last several decades 18 and the ongoing dearth of adult providers with specialized SCD knowledge and experience. 13 Furthermore, patient focus group participants of all ages expressed anxiety about some of the differences between pediatric and adult healthcare settings.…”

The Evidence Base for Transition Programmes for Adolescents with Sickle Cell Disease

“…This lack of an evidence base to guide SCD transition is evident in the fact that NHS recommendations for transitioning adolescents is based on Category C/Level IV evidence, described as "evidence from expert committee reports and/or clinical experience of respected authorities; indicates absence of directly applicable studies of good quality" [4]. It must be acknowledged that there are excellent models of transition schemes within the NHS [14], but current practices have not been evaluated for clinical efficacy. It appears that current research into transitioning in SCD is focused on qualitative studies that explore patients' and doctors' perspectives and the psychosocial challenges associated with the disease and its management during adolescence [6,15].…”

“…SCD and transition education also exerts an effect on patient self-efficacy, another cornerstone of patient readiness. Self-efficacy is described as the degree to which patients believe they have control over their disease [14]; this encompasses patient's perceived competence and resourcefulness in managing their own disease [9]. Demographic variables such as age, educational grade and gender are found to exert little influence on self-efficacy [13]; it is evident that education is responsible for generating high levels of self-efficacy in a significant proportion of adolescents, especially in the daily management if SCD such as adherence of medication regimens and appointments.…”

“…Nevertheless this highlights the need for psychosocial support in many SCD transition programmes in line with other chronic diseases such as renal failure and cystic fibrosis [26]. IA review of a London-based transition programme reports that a support group for SCD patients at university was valued by the participants [14]. Conversely, another study reports little success in starting peer-support groups, with only 17% of the adolescents with SCD showing interest in group meetings [22].…”

“…Developing EBM in SCD transitioning to drive NICE guidelines is also important to help secure funding for transitioning. Howard et al [14] found no comprehensive transition services for UK adolescents with SCD; thus building an evidence-base for SCD transitioning may identify areas of unmet need and lead to the inclusion of the wide range of services required in SCD transition into NICE guidelines.…”