One Company's Angola Sickle Cell Program

摘要

Sickle cell disease is an inherited blood disorder characterized by misshapen red blood cells, susceptibility to infection, hemolytic anemia, sporadic blockage of blood vessels, and chronic organ damage. The sickle cell gene arose as a genetic selection against malaria. Currently all newborn infants in the United States are tested for sickle cell disease at birth as part of a national screening program, which allows prompt diagnosis, followed by early identification, and preventive care and treatment. About 3000 infants are diagnosed with sickle cell disease each year in the United States. In Africa, the problem of sickle cell disease is much greater, due to higher sickle gene prevalence. Newborn screening for sickle cell disease does not exist in most parts of Africa, and affected infants often die from infection or anemia. It is estimated that over 300,000 affected births occur annually, but 50-90% die before age 5 years. Sickle cell thus contributes substantially to the under-5 mortality in most sub-Saharan African countries. In Angola, for example, an estimated 12,000 babies are born with sickle cell diseases each year but most will die without a correct diagnosis. Angola has just one sixteenth the population of the United States, but at least four times the number of babies born each year with sickle cell disease. In March 2011, the Republic of Angola entered into a public-private partnership with Chevron and Baylor College of Medicine/Texas Children’s Hospital to develop a comprehensive sickle cell program. This public-private partnership was established to improve the health of Angolan children with sickle cell disease, with the hope of reducing mortality. The primary goal of the program is to identify infants with sickle cell anemia and provide simple treatments that will prevent life-threatening complications of the disease. The project also trains Angolan health professionals to identify and treat the disease. This newborn screening and treatment program is the first of its kind in Africa. The Angola Sickle Cell Initiative has developed to include three primary components: 1) Newborn Screening: Newborn screening was initiated in Luanda at a single maternity hospital in July 2011 with the goal of demonstrating the feasibility and effectiveness of screening for sickle cell disease in an environment with limited healthcare resources such as Angola. Early successes of this program resulted in expansion of the newborn screening program such that five birthing centers in Luanda and two birthing centers in Cabinda now screen babies. The 3rd Quarter of 2013 has been the most prolific quarter for screening since the start of the program with 9,584 babies screened. Whereas the program had some initial difficulties with adequate participation of the maternity hospital nurses in screening the babies, continued education and encouragement has resulted in a steady increase in the number of babies screened. These improved screening results are testament to the local respect and success the screening program has had since its initiation in July 2011.