Introduction
Sickle cell disease (SCD) disproportionately affects rural population of India. It remains endemic in many states. Morbidity and mortality associated with SCD is well known. This inherited disorder is caused by formation of HbS. These polymers have the capacity of intravascular polymerization blocking the small vessels , leading to all resultant clinical complications. Complications of the disease like veno-occlusive crisis, chest pain, anemia requiring transfusions add to the decreasing quality of life over time. If there is higher level of HbF, the chances of intravascular polymerization reduce, leading to lesser clinical events and better quality of life. Maintaining higher levels of HbF reduces severity of the disease. Pharmacological intervention like usage of hydroxyurea (HU) induces formation of HbF with resultant increase in its levels. Hydroxyurea has been proven to be most cost-effective intervention. Safety and efficacy of hydroxyurea has been well documented to reduce morbidity and mortality. Long term safety data also have been reassuring. Usage of HU is an internationally recommended standard of care and adopted strategy for the management of sickle cell disease.
Wide variety of programs have been initiated by the governments for hemoglobinopathies, majorly focusing on screening, early detection, screening of pregnant woman. SCD patients are encouraged to visit sickle cell centers where it is a common place practice to initiate hydroxyurea. The system of drug delivery is well designed in few districts. There is no regular follow up of these patients and once initiated hydroxyurea is maintained by patient himself and intervention and corrections are rare. There is no led down care pathway available in the periphery. To assess the real world scenario of SCD patients, particularly there levels of HbF, a random clinical examination with blood sampling was initiated and compared to baseline available data. Objective of this intervention was to understand real world levels of HbF and clinical scenario, requiring presumably lesser clinical interventions.