Clinical Profile of Patients of Sickle Cell Crisis in a Rural Tertiary Care Hospital

BACKGROUND Sickle cell disease (SCD) is a part of the spectrum of sickle cell syndromes which occurs due to a genetic mutation causing substitution of glutamic acid to valine at the 6th position of the beta-globin chain. This can cause patients to present with a wide spectrum of crises including acute bone pain, pulmonary crises, vaso-occlusive crises, aplastic crises, and splenic crises. Central and western part of India harbours the majority of the sickle cell disease burden. Thus, this study was conducted to assess the clinical profile of patients with SCD who were admitted at a tertiary care hospital with sickle cell crisis. METHODS This was a cross-sectional study conducted in the Dept. of Medicine, and Central Clinical Laboratory, Acharya Vinoba Bhave Rural Hospital (AVBRH), Sawangi (Meghe), Wardha, for a period of 10 months from 1st January 2018 to 31st October 2018. Fifty sickle cell patients were selected as study population, the sample size was taken based on the convenience of the study, and a proforma detailing relevant clinical history was filled first, followed by collection of blood sample for assessment of laboratory parameters including haemoglobin levels, platelet counts, and S. LDH levels. Data was compared and contrasted between the Recovery group and the Mortality group. RESULTS Most common crises amongst patients encountered was vaso-occlusive crisis 23 out of 50 (60.5%) in recovery group versus 9 out of 50 (75%) in mortality group, and the most common trigger for it was dehydration. While no statistically significant differences were found between the Recovery group and Mortality group on comparison of age groups, gender, or caste of patients, serum haemoglobin levels were statistically lower (p < 0.001), and platelet and S. LDH (4302.41 +/- 2560.34 U/L) versus (504.98 +/- 223.82 U/L), were statistically higher (p<0.001) in the Mortality group compared to the Recovery group. Duration of stay in the hospital was also significantly higher (p<0.001) in the Mortality group compared to the Recovery group. CONCLUSIONS Thus, haemoglobin levels, platelet counts, and S. LDH appear to act as good prognostic markers to assess and follow-up in cases of sickle cell crises. Early detection and correction of these variables can ensure better patient outcomes in sickle cell disease.