Quality of life and disease perceptions in caregivers of children with Congenital Adrenal Hyperplasia

Abstract

BackgroundCongenital adrenal hyperplasia (CAH) is a chronic disorder requiring lifelong therapy. False perceptions, poor knowledge, and social isolation adversely affect the psychosocial health and quality of life of patients and caregivers. The study was undertaken to ascertain the quality of life of caregivers of children with CAH. MethodA hospital-based cross-sectional study enrolled caregivers of children diagnosed with CAH where newborn screening was unavailable. Quality of life (QoL) was measured using the WHO-QoL-BREF questionnaire to compare physical, psychological health, social relationships, and environment. ResultsEighteen children with CAH (9 males), 14 salt-wasting, and 4 simple virilizing CAH with median (IQR) age of 4.3 (2.6, 9.8) years and a follow-up period of 3.8 (1.3, 8.4) years were enrolled. Out of 18 caregivers, 6 were fathers and 12 mothers with a mean (SD) age of 38 (8) years. The mean (SD) of the total score was 69.2 (10.6); physical, psychological, environmental, and social domain were 69.8 (15.1), 57 (12.6), 64 (14.5), and 65 (9.9), respectively. Physical, psychological, and environmental domain scores were significantly higher in upper-middle than lower-middle socioeconomic strata (P < 0.05) and similar between salt-wasting and simple virilizing phenotypes. There was no significant correlation between QoL scores and duration of disease (r = 0.257, P = 0.44). Parents of 7 (38%) patients discussed disease with extended families or neighbors, but none of the school teachers were informed. Death (56%), social acceptance (27%), and infertility (18%) were the major concerns acknowledged. ConclusionThe study reflects the suboptimal quality of life in caregivers of children with CAH. There was no correlation of the duration of disease with the QoL scores. Psychological and social relationship scores were poorest, indicating the need to creating more awareness to remove social taboos associated with this disease.