TMC PULSE

t m c » p u l s e | a p r i l 2 0 1 6 24 E ach year, approximately 300,000 babies are born worldwide with hemoglobin disorders like sickle cell disease. Often their survival depends on the circumstances of their birth. For babies born in the United States, advances in diagnosis and treatment have led to a drastic reduction in mor- tality rate. For those born in countries like Angola, however, which has one of the highest rates of sickle cell disease in the world, the prognosis is mark- edly worse. The desire to combat this disparity led to a unique collaboration between biomedical engineers and physician-researchers to develop a low-cost, low-tech screening test with the potential to save millions of lives in medically underserved areas. In 2011, a partnership between Texas Children's Hospital, Baylor College of Medicine, Chevron and the Angola Ministry of Health led to the development of the Angola Sickle Cell Initiative. Since the program's launch, over 136,000 newborns have been screened in Angola. Though this marks significant expansion of sickle cell disease screening in the country, Sergey Shevkoplyas, Ph.D., a University of Houston associate professor of bio- medical engineering and adjunct pro- fessor of pediatrics at Baylor, Gladstone Airewele, M.D., associate professor of pediatrics at Baylor and director of global programs at the Texas Children's expensive. It's about $5 a sample, which is a lot of money in Angola. Secondly, it takes a lot of resources. You need elec- tricity, you need gels and also there's a long turnaround time." IEF tests typically take about a month from sample collection to obtaining a result. By that time, parents have left the hospital with their new- borns and notifying them of the test results can be difficult. George said the follow-up rate for babies who are diagnosed is typically between 30 and 50 percent, a significant problem when patient survival depends on early initia- tion of prophylactic treatment. "One of the major complications early on is infection," George said. "Babies with sickle cell disease are very prone to infection by a couple of bac- teria. Historically, 50 to 90 percent of babies would die from these infections before reaching the age of five." Since the advent of prophylactic penicillin treatment, that percentage has been drastically reduced here in the U.S., where very few babies die from sickle cell-associated infections in early childhood. In sub-Saharan Africa, it's a different story. George and Shevkoplyas aim to change that with a simple piece of paper—a screening test that costs less than 15 cents. "We position our test as a very inex- pensive way to screen every baby born in Angola for identifying those at risk of having sickle cell disease," Shevkoplyas said. "When you start talking about countrywide population screening, every cent counts, especially in a coun- try with limited health care resources." The paper-based test screens for sickle hemoglobin, or hemoglobin S, the abnormal hemoglobin those A partnership between the University of Houston's Sergey Shevkoplyas, Ph.D., and Alex George, M.D., Ph.D., of Texas Children's and Baylor, resulted in the simple paper screening test. Screening Made Simple A paper-based screening test for sickle cell disease has the potential to save millions of lives B y S h e a C o n n e l l y We position our test as a very inexpensive way to screen every baby born in Angola for identifying those at risk of having sickle cell disease. When you start talking about country-wide population screening, every cent counts. — SERGEY SHEVKOPLYAS, PH.D. Associate Professor of Biomedical Engineering at the University of Houston Hematology Center, and Alex George, M.D., Ph.D., co-director of the Sickle Cell Program at Texas Children's Hematology Center and an assistant professor of pediatrics at Baylor, still saw room for improvement. "The protocol currently used is something called isoelectric focusing, IEF, which is basically a gel where you run protein samples to look for the sickle hemoglobin," George said. "It's a very effective, robust technology that's been used for a long time in the U.S. The problem is, first of all it's relatively

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