Lower hair cortisol among patients with sickle cell disease may indicate decreased adrenal reserves

Sickle cell disease (SCD) is a chronic illness that presents with a wide range of phenotypic variation. Stress may be a contributing factor to differences that are found in this population.

Our objective is to determine the relationship between hair cortisol content (HCC), a biomarker of stress, and other clinical measures in individuals with SCD.

We collected hair samples and other clinical measures from 73 subjects with SCD (mean age: 39 ± 12 years, 63% female).

HCC was lower among individuals who had greater than 30% hemoglobin S, compared with those who had less than 30% hemoglobin S (W=272.5, P=0.01). Lower HCC was also associated with report of not being on a chronic transfusion program (β=48.34, SE=14.09, P=0.001) and higher ferritin levels (β=-0.006, SE=0.002, P=0.02). Furthermore, HCC was significantly correlated with serum cortisol (r_s=0.26, P=0.03) and corticosterone (r_s=0.29, P=0.01). We also observed a consistent pattern of low steroid values among our population.

Our findings suggest that individuals with higher hemoglobin S and ferritin, both markers of severe SCD, may have decreased cortisol levels. This is consistent with the relationship we observed between higher HCC among individuals who are on a chronic blood transfusion program, which typically increases quality of life. Our results suggest that hair cortisol may be an indicator in patients with SCD who could be at risk for developing adrenal insufficiency. We recommend that clinicians treating patients with SCD follow the Endocrine Society guidelines for testing for adrenal insufficiency and treat accordingly.