Abstract

OBJECTIVES: To determine the factors associated with exercise capacity in adults with sickle cell disease (SCD) and its relationship to hospitalizations and mortality. METHODS: A total of 223 participants in the Cooperative Study of Sickle Cell Disease (CSSCD) (64% female, 70% hemoglobin SS/Sβ⁰ thalassemia, mean age 43.3 ± 7.5 years) underwent maximal exercise testing using a treadmill protocol with a mean duration of 11.6 ± 5.2 minutes. RESULTS: Female sex (β = -3.34, 95% CI [-1.80, -4.88], P < 0.001), older age (β = -0.14, 95% CI [-0.24, -0.04], P = 0.005), higher body mass index (β = -0.23, 95% CI [-0.37, -0.10]; P = 0.001), and lower hemoglobin (β = 0.56, 95% CI [0.08, 1.04], P = 0.02) were independently associated with lower fitness, while there was a trend with abnormal pulmonary function testing (β = -1.42, 95% CI [-2.92, 0.07]; P = 0.06). Lower percent-predicted forced expiratory volume in 1 second (FEV₁ ) was independently associated with lower fitness (β = 0.08, 95% CI [0.03, 0.13], P = 0.001). Genotype and hospitalization rates for pain and acute chest syndrome (ACS) prior to testing were not associated with exercise capacity. Baseline exercise capacity predicted neither future pain or ACS nor survival in our cohort. Adults with SCD tolerated maximal exercise testing. CONCLUSIONS: Prospective studies are needed to further evaluate the impact of regular exercise and improved fitness on clinical outcomes and mortality in SCD.