Growing with Turner’s syndrome should occur in staged steps

American Association of Clinical Endocrinologists 17th Annual Meeting & Clinical Congress

The physical and emotional developmental process of patients with Turner’s syndrome requires a combination of education, collaboration, communication and should occur in concurrence with the patient’s developmental stages, according to Karen R. Rubin, MD.

“An essential component of quality health care transition, especially for a complex condition such as Turner’s syndrome, requires a staged process aimed at better assuring that these patients will continue to receive, as adults, the continuous monitoring required to address their needs and maximize their longevity and quality of life,” said Rubin. professor of pediatrics and chief of endocrinology at Connecticut Children’s Medical Center.

Changing health needs

During early adolescence, the focus of care should shift from maximizing final adult height to inducing feminization with gradually increased doses of estrogen, according to Rubin. Recent evidence indicates that most girls with Turner's syndrome can reap the psychological benefit from a more age-appropriate induction of puberty by age 12 without compromising their final adult height.

“We need to ensure the patient knows her medications and hormones, what their indications are and when to take them,” she said. “We also need to make sure they know what Turner’s syndrome is, including their current health condition, and the long-term risks for morbidities.”

In addition to the previously known Turner’s syndrome susceptibility to autoimmune disease, congenital heart disease and hypertension, Turner's syndrome patients more recently have been observed to have a risk increase for several common adult conditions, including features of metabolic syndrome, type 2 diabetes, ischemic heart disease and osteoporosis. Because these conditions are, to some extent, preventable with early detection and intervention, transition provides the ideal setting to raise awareness of these future health risks and promote healthy lifestyle behaviors to lower these risks.

The typical neurocognitive profile, personality type and difficult social adjustment in some patients with Turner's syndrome may pose psychosocial risks that, if not addressed, can impair the individual's psychological well-being. The impact of Turner's syndrome on puberty should be discussed in simple terms with the patient by age 10 to 12, with more sophisticated explanations offered later on.

During late stage transition, the pediatric endocrinologist should have a close collaboration with the adult provider(s) of the patient in effort to convey important medical information effectively. The pediatric endocrinologist should also engage the patient in developing a comprehensive adult care roadmap or “transition passport.”

“During transition, pediatric providers should help their Turner's syndrome patients gain greater awareness of their health story and needs and counsel them about the evolving impact of their condition into adulthood," she said. “Preparing them to accept their adult responsibility of self-care and encouraging them to practice independent health care behaviors is essential.” - by Tara Grassia

For more information:

Rubin KR. Turner’s syndrome: transitions from pediatric to adulthood. Presented at: the American Association of Clinical Endocrinologists 17^th Annual Meeting & Clinical Congress; May 14-18, 2008; Orlando, Fla.