Sickle Cell Disease: The Most Common Inherited Disease In The World

June 19, 2013

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One drop of blood can save a life. There is a baby with sickle cell disease born every hour in Angola. Without the availability of newborn screening and simple interventions, a majority of these babies will die before they reach 5 years of age. In the United States, all babies are tested at birth for sickle cell disease and as a result, over 95% of these children will survive to be adults. The rate of sickle cell disease is 40 times as high in Angola and other sub-Saharan African countries but basic, lifesaving interventions remain largely unavailable. Sickle cell disease is an inherited blood disorder and the most common inherited disease worldwide, affecting over 400,000 babies annually with the greatest burden of disease within sub-Saharan Africa. Early diagnosis by newborn screening and simple interventions can significantly reduce this high mortality rate, but these capacities are limited mostly to babies born in developed countries, which represent less than 5% of the global burden of disease. The majority of children in Angola and across Africa with sickle cell disease will die within the first 5 years of life of preventable causes, often before a diagnosis is even made. Easy and inexpensive interventions such as early diagnosis, parental education and early treatment can result in dramatic improvements in the quality and length of life for these children. In 2008, a resolution from the United Nations (UN) declared June 19 as World Sickle Cell Day, with a focus on improved public awareness and an international call to action to improve the diagnosis and care for those affected by the disease. A key component of this UN resolution and a subsequent statement by the World Health Organization is the improvement of sickle cell care through international partnerships. Responding to that call, Texas Children’s has begun to fight sickle cell disease in the Republic of Angola. In July 2011, Texas Children’s Hospital and Baylor College of Medicine partnered with Chevron Corporation and the Ministry of Health in the Republic of Angola to form the Angola Sickle Cell Initiative. This initiative is focused on improving the diagnosis, care and survival for Angolan children affected by this inherited blood disorder. The key focus in the first 2 years was to develop a newborn screening program for sickle cell disease in Angola, as diagnosis is simple and requires only a drop of blood. Over 40,000 babies have been screened for sickle cell disease in Angola and over 600 babies have been diagnosed. Treatment is focused on preventing the most severe and life-threatening complications and includes special vaccinations, prophylactic antibiotics and anti-malaria mosquito nets. The work that has been done to date is only the beginning. I foresee this mission growing to become a cornerstone of Angolan child health and a model for many other sub-Saharan African countries faced with an equally high burden of sickle cell disease. I thank you for your interest and urge you to spread the word about sickle cell disease and how important it is to fight to ensure the health of children around the globe.

Post by:

Amber M. Yates, MD

Dr. Amber Yates is Assistant Director of the Clinical Division where she oversees outpatient care. She is also Co-Director of the Sickle Cell Program and a member of the Hematology Center.

Her primary clinical interest is in the care of children with sickle cell disease and other...

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