Treatment adherence yields QoL gains in congenital adrenal hyperplasia patients

Among teens and adults with congenital adrenal hyperplasia (CAH), adherence to treatment regimens, rather than type of glucocorticoid, is the factor that affects quality of life (QoL), according to a study.

A total of 75 patients (58.66 percent female) aged 15–72 years (mean 35 years) with CAH due to 21-hydroxylase deficiency were recruited from Karolinska University Hospital, Stockholm, Sweden. Seventy-one of them completed both the Addison Quality of Life (AddiQoL) and RAND 36 questionnaires.

Researchers assessed adherence to therapy regimens using the Adherence Starts with Knowledge questionnaire (ASK-12). They performed regression analysis to determine associations among QoL, type of glucocorticoid therapy prescribed, and ASK-12 results. Results were then compared to reference RAND 36 data obtained from a representative sample from the general Swedish population.

All 15 women with salt-wasting CAH had undergone genital surgery in the neonatal period. On the other hand, some women with simple virilizing CAH had not received surgery. Among adult patients, the 31 (41 percent) who lived with a partner had much higher AddiQoL and RAND 36 scores compared with the 20 (27 percent) who lived alone.

Better QoL was also seen among patients with good adherence to therapy regimens and those who were younger. Of note, younger patients on hydrocortisone and showed good treatment adherence had higher RAND 36 scores than older patients on prednisolone regardless of adherence.

Furthermore, patients with classic CAH (both the salt-wasting and simple virilizing forms) reported higher QoL than those with nonclassic CAH. Patients with CAH, particularly nonclassic, were more likely to report an impaired QoL than the general population, especially regarding limitations related to body pain, vitality, and mental health.