Original Article International Journal of Basic & Applied Physiology

Background: The inherited disorders of haemoglobin are the commonest single gene disorder of the world population. Sickle cell anaemia being the most widespread and numerically the most important haemoglobinopathy in the world today. The major features of sickle cell disease (SCD) in most patients are lifelong anaemia and the consequences of recurrent vaso-occlusion. Many complications of SCD involve anaemia, vaso-occlusion etc. Impairment of pulmonary function is a common complication of SCD. The patients suffering from this disease frequently present with complaints referable to the pulmonary system although other systems are also involved. Hence present study was carried out to evaluate the pulmonary function tests in sickle cell disease patients ( HbSS ),sickle cell trait patients(Hb AS) with normal person ( HbAA) non-sicklers. Aim and Objectives: 1) To study the pulmonary alterations in cases of homozygous SS & heterozygous AS Sickle Cell Disorder subjects.2) To compare the parameters with normal healthy controls & assess the importance of PFT in "Steady State"(free from complications or crisis)as an objective evidence to predict the risk of "Sickle Cell Chronic Lung Disease'' in future. Method: A cross sectional study was done in 50 cases of SCD (22HbSS& 28HbAS) and age and sex matched normal 50 HbAA controls. From the various measured pulmonary function test( PFT )parameters Forced Vital Capacity( FVC),Forced Expiratory Volume in 1 sec(FEV1),FEV1/FVC were selected for the study. The data collected was subjected to statistical analysis involving computation of Mean, Standard deviation, Independent T test. Result: Mean value of FVC, FEV1 and FEV1/FVC were found to be significantly lower than normal controls, but the difference in FVC between HbAS and HbSS turned out to be statistically non-significant whereas the difference in FEV1, FEV1/FVC between HbAS and HbSS was statistically significant. Conclusion: There were significant reductions in pulmonary function test parameters in sickle cell anaemia patients as compared to normal controls indicative of mixed pattern (both restrictive and obstructive) lung impairment in sickle cell anaemia.