Lymphedema Awareness and Experience in the Turner Syndrome Community

Lymphedema commonly affects females with Turner syndrome (TS), although it may be underrecognized and untreated. The objective of this study was to better understand self-reported lymphedema prevalence, knowledge, and experience in TS.

A 24-question anonymous survey was distributed to females with TS or their caregivers at a TS community event, a TS adult comprehensive care center, and an associated pediatrics clinic. Survey questions assessed lymphedema awareness, diagnosis and history, treatments and compliance, and sequelae. Data were analyzed using descriptive statistics.

Forty-one surveys were returned, with 51.2% (n = 21) completed by the patient (mean age 25.8 years), 26.8% by the caregiver (n = 11), 2.5% by both the patient and the caregiver (n = 1), and 19.5% (n = 8) with unmarked respondent type. Greater than 75% (n = 31) reported having heard of lymphedema, 26.8% (n = 11) reported discussing lymphedema with a healthcare provider, and 22% (n = 9) reported receiving a diagnosis of lymphedema. The median age at the time of lymphedema diagnosis was 2 years. The most commonly affected body sites were legs/feet, followed by arms/hands and neck/face; the majority of patients experienced lymphedema at multiple body sites. Lymphedema resolution was reported by half, with a median duration of 4.25 years. Only one-third of those with lymphedema reported receiving treatment for the condition, including use of specialized massage/manual lymphatic drainage and compression garments.

Identification of knowledge gaps and experience with lymphedema in TS can help with the development of targeted educational interventions. Greater awareness and understanding of this treatable condition in TS may contribute to improved health outcomes.