Perceptions of CAH self-management and stress dosing in relation to health-related quality of life as measured by CAHQL.

BACKGROUND

Classic congenital adrenal hyperplasia (CAH) requires lifelong glucocorticoid (GC) therapy and the knowledge of how and when to administer GC stress doses to prevent life-threatening adrenal crises. The aim of our study was to investigate patient perception of CAH self-management in relation to clinical and socioeconomic characteristics and health-related quality of life (HRQoL).

METHODS

Sixty-nine patients with classic CAH, aged 16 to 75 years, participating in the study to create the disease-specific patient-reported outcome tool, CAHQL, answered 7 questions about disease self-management. Patient perceptions regarding CAH self-management were analyzed in relation to clinical and socioeconomic characteristics, biochemical disease control and CAHQL domain scores (General Health, Adrenal Insufficiency, Glucocorticoid Excess, Physical Functioning, Mental Health and Cognition, Social Functioning, and Sexual Functioning).

RESULTS

Eighty percent reported that they mostly or strongly agree that they feel comfortable managing CAH whereas over half worry about adrenal crises. All CAHQL domain scores were strongly correlated with at least one self-management question (p < 0.05); better HRQoL was associated with greater confidence in self-managing CAH. Increased worry about stress dosing and adrenal crises was associated with greater number of stress doses and hospitalizations in the past 6 months (p < 0.05). Greater number of household members, being married and male sex were associated with increased confidence in receiving help from others regarding adrenal crisis (p < 0.05). After adjusting for relationship status, sex remained significant. No associations were found between patient perceptions regarding CAH self-management and phenotype, age, biomarkers of disease control, insurance type, income bracket, education or work status.

CONCLUSION

Confidence regarding self-management of CAH is associated with better HRQoL as measured by CAHQL and was not related to socioeconomic characteristics or level of disease control. Integrating systematic evaluation of self-management skills and the HRQoL burden of having CAH into standard of care is recommended.