Adult Care of Pediatric Conditions: Lessons from Turner’s Syndrome

Abstract

Transition from pediatric to adult care receives a lot of column inches in medical journals, but there is little in the way of real evidence regarding the process. The paper by Devernay et al. (1) is one of a very few that begins to inform us of factors that determine successful transition of a common endocrine condition—Turner’s syndrome (TS). This paper reveals what we all may have suspected to be true; only a small minority of girls with TS (3.5%) transition to an adult service that provides health surveillance according to international guidelines. The authors have taken advantage of a cohort of girls with TS who were ascertained by virtue of their receiving GH between 1986 and 1997. Those that were over 18 were targeted for a postal questionnaire. The response rate of 69% from the original 891 is impressively high for this type of exercise. The nonrespondents were 2.7 cm shorter than the respondents, possibly because they were more severely affected or less compliant with GH therapy. Whichever was the case, the outcomes must be seen as better than might be expected for the whole cohort. The average age of the study group was 22 yr, which was about 6 yr after they stopped treatment with GH. The main outcome of only 3.5% of subjects receiving optimal adult care may not be surprising because the reference guidelines for follow-up of TS describe a gold standard that few centers would achieve in reality (2). Consider, however, two essential tests for TS care. Only 36% of subjects had a simple thyroid function testing (probably the single most cost-effective test), and only 21% of subjects without known heart disease had had an echocardiogram (the single most effective life-saving maneuver). Endocrinologists can take heart from the fact that they performed better than other care groups in the standard of care provided, and this is important evidence for TS health care to come firmly under the endocrine umbrella. Suboptimal adult care for women with TS is not unique to France. Smaller studies from Belgium and the United States have highlighted similar problems (3, 4). It is humbling that even in the French system, with full lifetime reimbursement for TS, the most vulnerable groups are lost—those of lower socioeconomic grouping and educational level. This observation makes the point that a cardinal issue in transition is case tracking. Often, when considering the process of transition, emphasis is placed on combined clinics, shared protocols, and “meeting the new team.” It may be that the administrative task of following up each case is the most essential component of transition with the pediatric team making contact with patients within 1 yr of handover to make sure that adequate future care has been established. Throughout health systems, the structure of care all too often prioritizes convenience for the physician rather than effectiveness for the patient. In the light of current discussions taking place in the United States regarding healthcare, one voice that is seldom heard is that of patients with chronic conditions. In the patient support groups for TS, congenital adrenal hyperplasia, and disorders of sexual development, there is a constant complaint that a private insurance system seldom meets the needs of individuals with chronic conditions where multidisciplinary teams are considered optimal. The rather dismal outcome in the paper by Devernay et al. (1) has relevance across many lifelong conditions in endocrinology including hypopituitarism, Klinefelter’s syndrome, congenital adrenal hyperplasia, and disorders of sexual development. Among these, TS stands out as