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.