Functional capacity and quality of life in children and adolescents with sickle cell anemia

To evaluate the functional and exercise capacity, lung function, quality of life of children and adolescents with sickle cell anemia (SCA HbSS) and to test the reproducibility of functional capacity tests in this population.

Cross-sectional study with volunteers with SCA Hb-SS (SCAG), aged 6-18 years matched in age and gender to the control group (CG) with healthy individuals. Spirometry, 5-repetition sit-to-stand test (5STS-test), modified shuttle test (MST), and Pediatric Quality of Life Questionnaire (PedsQL) were performed. The reproducibility of 5STS-test and MST was evaluated: RESULTS: Forty eight volunteers of SCAG and 48 of CG were evaluated. Lung function of SCAG (FVC: 92 ± 15% pred.; FEV₁ /FVC: 84 ± 8% pred.) was worse than the CG (104 ± 15% pred.; FEV₁ /FVC: 90 ± 6% pred.) p < 0.05. SCAG had worse functional capacity registered by distance walked: 576 m (515-672 m) and 5STS-test: 8 s (7.4-8.9 s) compared with the CG who showed distance walked: 1010 m (887-1219 m) and 5STS-test: 7 s (7.0-8.1 s), p < 0.001. SCAG had worse quality of life compared to CG, p < 0.05. The reproducibility of MST (ICC 0.99 (0.98-0.99 IC-95%)) and 5STS-test (ICC 0.80 (0.69-0.88) was considered good, p < 0.001.

Children and adolescents with sickle cell anemia presented worse capacity to walk or run, and to perform sit-to-stand test when compared with their control peers. Additionally, they have poorer quality of life. The MST and 5STS-test showed good reproducibility to be applied in pediatric individuals with SCA.