FUNCTIONAL CAPACITY and quality of life IN CHILDREN AND ADOLESCENTS WITH
SICkLE CELL ANEMIA
Abstract
Objective: To evaluate the functional capacity, pulmonary
function and quality of life of children and adolescents with sickle
cell anemia (SCA) and to test the reproducibility of functional capacity
tests in this population. Method: Cross-sectional study with
volunteers with SCA genotype Hb-SS (SCAG), aged 6 to 18 years matched
in age and gender to the control group (CG). Spirometry, 5-repetition
sit-to-stand test (5STS-test), modified shuttle walk test (MSWT), and
Pediatric Quality of Life Questionnaire (PedsQL) were performed. The
reproducibility of 5STS-test and MSWT was evaluated: Results:
48 volunteers of SCAG and 48 of CG were evaluated. Pulmonary function of
SCAG (FVC: 92 ± 15% pred.; FEV 1 /FVC: 84 ± 8% pred.) was worse than
the CG (104 ± 15% pred.; FEV 1 /FVC: 90 ± 6% pred.) p <
0.05. SCAG had worse functional capacity registered by distance walked:
576m (515-672m) and 5STS-test: 8 seconds (7.4-8.9seconds) compared to
the CG who showed distance walked: 1010m (887- 1219m) and 5STS-test: 7
seconds (7.0-8.1seconds), p < 0.001. SCAG had worse quality
compared to CG, p < 0.05. The reproducibility was good of MSWT
(ICC 0.99 (0.98-0.99 IC-95%)) and 5STS-test (ICC 0.80 (0.69 – 0.88), p
< 0.001 . Conclusion: Children and adolescents with
sickle cell anemia showed worse capacity to walk or run, and to perform
sit-to-stand test. Additionally, they have poor quality of life when
compared with their control peers. The MSWT and 5STS-test showed
reproducible to be applied in pediatric individual with SCA.