Children and adolescents with sickle cell disease (SCD) have a lower capacity to walk, run, and perform sit-to-stand testing, as well as a significantly reduced quality of life in comparison with healthy peers, according to a recent article in Pediatric Pulmonology.

For the purpose of this cross-sectional study, the authors recruited 48 volunteers with SCD from the Pediatric Hematology Outpatient Clinic of Darcy Vargas Children’s Hospital in São Paulo, Brazil, between October 2018 and July 2019. The participants, aged 6 to 18 years, were matched in age and sex with healthy controls.

To evaluate the quality of life, functional exercise capacity, and pulmonary function of patients with SCD, the researchers conducted the pediatric quality of life questionnaire, a 5-repetition sit-to-stand test (5STS-test), and a modified shuttle test (MST), as well as spirometry.

As there is scarce evidence to confirm the clinical feasibility of functionality testing in patients with SCD, the authors also evaluated the reproducibility of the 5STS-test and MST.

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According to the results, the spirometry values, including forced vital capacity (FVC), forced expiratory volume in the first second (FEV1), FEV1/FVC, and forced expiratory flow between 25% and 75% of FVC (FEF25-75%), were reduced in patients with SCD compared to those of the healthy individuals. Respiratory muscle strength as assessed by measuring the maximum pressure during respiration was also reduced.

Patients with SCD walked an average of 576 meters and scored 8 seconds on the 5STS-test, while healthy controls walked an average of 1010 meters and scored 7 seconds on the 5STS-test, indicating reduced functional capacity in those with SCD.

The pediatric quality of life questionnaire showed lower quality of life in the group of patients with SCD. Moreover, the evaluation of 5STS-test and MST reproducibility was satisfactory.

“Patients with [sickle cell anemia] tend to have the syndrome of high cardiac output, described as the disease that most increases cardiac output at rest, contributing to lower performance in activities such as walking fast or running and playing,” da Silva and colleagues wrote.

“In this study, both groups reached higher values of heart rate (more than 80% of the maximum predicted heart rate), at the peak of the MST,” they explained. “However, the patients [with SCD] reached these values with a much shorter distance walked than the [control group], which highlights the aspect of a sedentary lifestyle, lower exercise capacity, chronicity of the disease, and syndrome of high cardiac output.”

As SCD is associated with vaso-occlusive events caused by altered circulating erythrocytes, permanent damage is often seen in the lungs and other organs. Impaired lung and cardiovascular functions favor a sedentary lifestyle, reduced functionality, and lower quality of life in these patients.

Reference

da Silva JL, Soares BA, Silva JC, et al. Functional capacity and quality of life in children and adolescents with sickle cell anemia. Pediatr Pulmonol. Published January 2, 2023. doi:10.1002/ppul.26300