Sickle cell disease (SCD) occurs as a result of inheritance of sickle hemoglobin (HbS) that causes red blood cells to become hard, sticky and sickle-shaped, making them fragile and easily destroyed. SCD is common in Africa, imposing a substantial clinical and public health burden. In six Equatorial African countries alone, including Sierra Leone, more than an estimated 180,0000 SCD babies are born annually. Children who inherit the HbS gene from one parent have the harmless sickle cell trait, but those inheriting sickle cell genes from both parents will develop SCD. Sickle cell disease is a chronic illness resulting from the sickled cells blocking flow in small blood vessels, thus depriving body organs of blood and oxygen. The consequences are chronic ill-health arising from organ damage, general debility, poor growth, chronic anemia and jaundice, punctuated by frequent acute illness that includes episodes of intense pain, anemia, chest symptoms strokes and, consequently, frequent school absence. Acute episodes are commonly precipitated by infections such as malaria, pneumonia and diarrhoea, resulting in many infant deaths. Those who survive childhood have a bleak outlook, as often, their poor health combined with minimal educational attainment subject them to reduced life fulfilment and life expectancy. Pregnant women are at increased risk of foetal loss and death. SCD cannot be cured, but as has been demonstrated elsewhere, much can be done with simple cost-effective interventions to reduce complications, improve quality of life and prolong survival. In recognition of the challenge posed by SCD, the WHO’s Regional Committee for Africa, at its one-hundred-and-seventeenth Executive Board session in January 2006, adopted Resolution EB117.R3 that urged the development, implementation and reinforcement of comprehensive national integrated programmes for the prevention and management of SCD. Sierra Leone has an estimated sickle trait prevalence of about 25%, which, compounded with limited socio-economic resources, places it especially at risk. Over the years we have been promoting educational and public information programmes directed towards fostering better knowledge of SCD among patients, families and the public in order to reduce the incidence of SCD, as well as clinical programmes directed at relieving suffering. In spite of this, there has been no significant impact in management and outcomes. We have, therefore, decided to embark on a structured approach to determining disease incidence and understand factors that determine outcomes and causes of morbidity and mortality. We have, therefore, designed this project in order to define interventions that would address causes of early mortality among Sierra Leonean infants with SCD. This information will be of value in government healthcare planning for Sierra Leone and possibly throughout Africa, as recommended by WHO. SCD cannot be cured It has been shown that if babies with SCD are identified at birth, interventions can reduce morbidity and mortality SCD is a cause of high infant mortality in Sierra Leone SCD causes severe sickness and under-achievement in childhood survivors In Sierra Leone, it is unknown what are the precise causes of morbidity and mortality in SCD infants If we can determine how infants die, we should be able to prevent their deaths and sickness Details on project end goals 1. Determine the proportion of babies born with sickle cell disease in Sierra Leone. 2. Determine the causes of infant mortality among sickle cell disease babies. 3. Define the evolution of haematological and biochemical changes among infants with SCD, HbS trait and normal genotype 4. Identify clinical and public health interventions that could reduce morbidity and mortality among SCD babies. 5. Extrapolate lessons learned to include the community at large. Brief financial breakdown Personnel £136333 Capital Equipment £76667 Administrative costs £46160 Services £17800 Laboratory consumables £140083 Molecular sub-contract £18667 Total £435,710 over 5 years