Study of Pulmonary Function Tests in Patients with Sickle Cell Disease

Abstract

Background : Sickle-cell disease (SCD) is a life-long haematological disorder characterized by red blood cells that assume an abnormal, rigid, and sickle shape. Pulmonary involvement is a major cause of morbidity and mortality in patients with SCD. The present study was undertaken to determine pulmonary functions in SCD patients and to compare the pulmonary functions between sickle cell anemia (SCA) and sickle cell trait.

Method: Total 155 patients of SCD were enrolled in the study. A detailed history, clinical and relevant investigations were done for all the patients. Pulmonary function test was performed and findings were compared between SCA and sickle cell trait.

Results: The patients of SCA (HBSS) were 80(51.61%) while that of sickle cell trait (HBAS) was 75(48.38%). There were lower values of FEV1, FVC and PEFR among SS population as compared to AS population with increase in FEV1/FVC ratio. The restrictive pattern of spirometry was more common among sickle cell patients (53.54%). Among SS population in both male (72%) and female (28%) restrictive pattern was common while among AS population most common spirometry pattern was normal spirometry while second most common pattern was restrictive pattern which occurs in 13 patients (39.40%) in males and 20 patients(60.60%) in females.

Conclusion: Chronic pulmonary complications in SCA are common. Abnormal pulmonary function was present in 63.87% of total population of SCD. SCA patients have reduced FEV1 and FVC values, despite this reduction they had significantly increased FEV1/FVC ratio which is keeping a restrictive pattern of pulmonary dysfunction.