Morbidity Pattern in Sickle Cell Disease in Central Gujarat, India — Single Centre Perspective

INTRODUCTION:

Sickle cell disease (SCD) poses a considerable health burden in India. About 20 per cent of children with SCD die by the age of two and 30 per cent children with SCD among the tribal community die before they reach adulthood is reported in one ICMR survey. As they are vulnerable to mortality, require regular clinical follow up. This retrospective analysis study was, undertaken to evaluate the morbidity pattern in SCD patients observed in patients followed up at our centre

METHODS:

SCD patients registered and followed up at HOCC between Jan 2011 to June 2017 were included in this study. At first visit to HOCC baseline CBC, PLT, RBC indices, LFT, Creatinine, S.Ferritin,Urine R/M,HPLC pattern,USG abdomen were noted. All 342 patients registered at HOCC were advised to take Hydroxyurea (dose adjusted according to CBC), Folic acid, B12, Multivitamin and Calcium. Vaccination given to selected patients. Out of these 342 patients, 57(16%) patients had 79 hospitalization events, 11 patients (3.2%) had recurrent hospitalizations. All patients were hospitalized in Sterling Hospital Vadodara.CBC PLT,RBC indices,LFT, RFT done on admission in all patients, Blood and urine culture, PS for MP, Dengue NS1,IgG,IgM, in all febrile patients, Viral markers ,CXR,USG Abdomen, MRI pelvis with HIP done in selected patients. All hospitalized patients received adequate hydration by IV fluids.Paracetamol, Contramol, Buprenorphine transdermal patch for pain relief. PCV transfusion to keep HB 8 to 10 gm%.Antibiotics if any evidence of infection, pre-emptive or based on culture report.Oxygen by nasal prongs or NRBM,NIV or Invasive Ventilator support to maintain SPO2 more than 95%.Exchange transfusion if no pain relief in vasoocclusive crisis in 48 hrs or has Acute Chest Syndrome, Splenic Sequestration or Hepatic cell crisis

RESULTS:

Seventy nine hospitalization events of 57 SCD patients during study period were evaluated to analyse morbidity pattern. Out of 57 patients 47 (82%)patients were not taking regular treatment,10 patients (17%)inspite of regular treatment and follow up developed morbid events .Age range 7 yrs to 57 years, M:F ratio 2:1(Male 38,Female 19),5(8%) patients were pregnant ,Vaso-occlusive crisis was most common morbid event 46(58% ) followed by LRTI,ARDS,Acute chest syndrome 9(11%),Hepatic cell crisis 8(10%),Septicemia 6(7.5%),Splenic sequestration 3(3.7%), Aplastic crisis 2(2.5%),Dengue 2(2.5%),Vivax Malaria 2(2.5%),AVN 2(2.5%).Septic arthritis 1(1.2%),Majority of hospitalization observed in month of July and Aug followed by in September ,October, least admissions in the month of April and December,HB F level was more than 20% in 16 patients(28%) and between 10 to 20 % in remaining patients(72%).Duration of hospitalization less than 10 days in majority 69(87%),14 days in 10(12%),Ventilator support was required for 7(8.8%) ,O2 by NRBM in 2(2.5%) ,O2 by nasal prongs 1(1.2%),Blood and Urine Culture for gram negative bacterial growth detected in 6(7.5%),none of the patient had gram positive growth, Exchange transfusion was required for 19 patients(24%) Outcome was favourable in 75(94%),whereas 4 patients(5%) who were not on regular follow up and they reached hospital almost 72 hrs after initial symptoms of crisis succumbed ,Major cause of mortality was Hypoxia, Septicemia with multiorgan failure on day1 of admission in hospital with rapid deterioration within 24 hrs.

CONCLUSION:

SCD patients on Hydroxyurea and supportive therapy have significantly lower incidence of morbid events requiring hospitalization.Vaso-occlusive crisis is a major reason of hospitalization. Gram negative septicaemia is the leading cause of infective morbidity. Hepatic cell crisis observed predominantly in ethanol addicted patients, Mortality is highest with delayed treatment of ARDS, Acute chest syndrome. Four strong predictors of good outcome are adequate hydration, early control on infection, maintaining adequate oxygenation and early initiation of treatment before organ failure sets in.