Abstract
Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS.
Highlights
Sickle cell anemia (SCA) is a hereditary form of hemolytic anemia characterized by production of abnormal hemoglobin (HbS)
Exercise capacity decreases in patients with sickle cell anemia (SCA), as a result of reduced oxygen carrying capacity related to high HbS levels, functional and structural cardiac adaptations resulting from chronic anemia, pulmonary dysfunction caused by repeated episodes of acute chest syndrome, and peripheral vascular impairment due to microvascular occlusion [4]
The present study demonstrates the clinical improvement in functional capacity (6MWT: 76 m), respiratory muscle strength (MIP: 11 cmH2O, maximal expiratory pressure (MEP): 35 cmH2O), health related quality of life, dyspnea (MMRC: 2), fatigue (FSS: 11), and pain (VAS: 5.5 cm) perception after inspiratory muscle training (IMT)
Summary
Sickle cell anemia (SCA) is a hereditary form of hemolytic anemia characterized by production of abnormal hemoglobin (HbS). The sickling of red cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations including palpitations, dyspnea, fatigue, and pain; these symptoms worsen during physical effort [1]. Multiple complications such as vasoocclusive painful crisis, acute chest syndrome, pulmonary hypertension, and stroke occur in SCA [2]. Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS
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