Functional capacity in sickle cell disease: A pilot study with 1-minute sit-to-stand test

Abstract

Background

Sickle cell disease, the most prevalent monogenic recessive genetic disorder in the world, is characterized by two main pathogenic mechanisms: vaso-occlusion and hemolysis. These characteristics lead to reduced tolerance to physical exertion and, consequently, a reduced functional capacity which can be assessed using the one-minute sit-to-stand test. Complications from sickle cell disease result in poor quality of life, increased absenteeism from school and work, and impaired social interaction.

Method

Between January 2023 and April 2024, a pilot cross-sectional study was conducted with sickle cell disease patients aged from 18 to 60 years. The one-minute sit-to-stand test, Borg's perceived exertion scale, and the SF-36 quality of life questionnaire were utilized. Patients were monitored during the test. The sample was dichotomized based on test performance and SF-36 scores. Furthermore, clinical and demographic variables were analyzed.

Main results

Fifty-eight individuals participated in the final analysis. The mean age was 29.84 ± 11.20 years; 55.1 % were men, and 79.3 % identified themselves as Black or mixed race. The most prevalent genotype was hemoglobin SS (67.2 %), and 77.5 % were taking Hydroxyurea. The group with a better performance in the one-minute sit-to-stand test showed better quality of life as assessed using the SF-36 questionnaire.

Conclusion

Functional capacity is a significant factor in the autonomy and quality of life of patients with sickle cell disease. The one-minute sit-to-stand test is a low-cost and easily applicable test, which can contribute to the assessment of functional capacity in the routine follow-up of these patients.