Sickle Cell Research Using Biochip Technology Advances at University Hospitals, CWRU

A new study that recently began enrolling patients at University Hospitals Case Medical Center is using biochip technology that one day may help physicians better manage the treatment of patients with sickle cell disease. Sickle cell disease is one of the most common genetic diseases in the United States; sickle cell anemia affects an estimated 100,000 people, mainly African Americans.

The disease can cause frequent bouts of joint or organ pain, vulnerability to infection, stroke, slow growth or internal organ damage. When red blood cells sickle, or become malformed (shaped like a sickle or a crescent), it makes blood flow and oxygen flow to nearby tissue more difficult, causing pain and organ injury. The biochip technology determines the "stickiness" of red blood cells – a quality that physicians think can indicate something about disease activity. If doctors can spot changes in the blood cells early, the disease can be treated earlier with medication changes or hydration before patients experience sickle cell crises. Right now, the only way physicians can gauge a treatment's effectiveness is by looking to see if pain experienced by a patient goes away. "Sickle cell has so many different faces that I don't think we've been very good at [understanding] what might be a targeted therapy for painful crisis," said Dr. Jane Little, director of the Adult Sickle Cell Anemia Center at UH who is part of the research team. "I don't think we have a grip of how cellular 'stickiness' may change in this disease. "Every little bit helps, the more we understand how the disease works, the better," she said. One of the big benefits of the biochip technology – developed by Umut Gurkan, assistant professor of mechanical and aerospace engineering at Case Western Reserve University – is that it provides important information with just a few drops of blood from patients, and without drastically changing the blood's composition. Earlier this month, at the annual meeting of the American Society of Hematology, the UH and CWRU researchers presented results of an earlier study launched in late 2013. In that study, drops of blood from adult patients were placed on the biochip, which examined the properties of red blood cells and provided information about a patient's sickle cell disease. "The goal was to try to think about a test that was widely applicable that would use a few drops of blood, and you didn't need [a lot of] machinery to use," Little said. Based on those promising findings, researchers have begun getting consent from child and adult sickle cell patients to use their blood samples for the new study. The study, which will enroll up to 100 children and adults, is being funded by a $486,000 grant that CWRU and UH received in 2013 from the Doris Duke Foundation. A $7,500 Belcher-Weir Family Pediatric Innovation Award from UH Case, to apply the technology for children, also will be used. It's been long known that the red cell surface in sickle cell patients is "really screwed up," Little said. The hemoglobin in the red cell is mutated, which leads to blood vessel damage. While previous research has been done in a small number of patients, the biochip technology makes it possible to analyze blood samples from hundreds of patients, Little said. The current study now under way will seek to link those findings to other clinical characteristics, Little said. She said preliminary data is expected in the next six months. "There are not many tools to help us investigate what's happening to the red cells in sickle cell patients," Little said. "The complete blood count shows you what your hemoglobin is, your white blood count, your platelet counts – but it doesn't tell you anything about function." The new technology, Little said, "gives us another dimension. It's like a little window in how the sickle cells act."

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