WRNMMC, Bethesda, Md.  –   When you meet U.S. Army Lt. Col. (Dr.) Benjamin R. Smith, a medical professor with the Uniformed Services University, you sense his enthusiasm for sharing knowledge and improving the landscape for patients. Smith, who is also the chairman of the Pediatric Hematology Department at Walter Reed National Military Medical Center (WRNMMC), oversees a team dedicated to diagnosing and curing blood disorders - such as sickle cell disease (SCD) - in children, teens, and young adults.

September is National Sickle Cell Disease Awareness Month

Each September, as part of Sickle Cell Disease Awareness Month, Smith and his team remind the community that SCD affects approximately 100,000 Americans and more than 20 million people worldwide, mostly African American or Latino.

Researchers say SCD originated in Africa, where it's estimated to prematurely kill 50% to 90% of the millions of people born with it. A genetic mutation that alters the oxygen-carrying protein hemoglobin inside red blood cells is the culprit. Normal red blood cells are flexible enough to squeeze through small blood vessels. But the mutation causes hemoglobin to aggregate into rock-hard rods that give cells a sickle shape, potentially causing damage to a carrier’s heart, spleen, and kidneys.

“I run the [SCD] clinical registry for the Defense Health Agency (DHA),” explained Smith. “I know we have around 30 pediatric patients in the National Capital Region, and about 300 in the entire DHA,” Smith reflected – elated that improved blood therapy treatments have increased the life expectancy for SCD patients by more than 30-years compared to the 1970s.

Historical Context

Since 1949, scientists have made steady progress in understanding the complex molecular pathology of SCD, according to NIH. However, until about two decades ago, no known cure for this genetic disorder existed. Medical historians credit Dr. Mary T. Basset, who currently serves as a professor at Harvard University’s School of Public Health, for championing a new era of federal research grants aimed at better understanding SCD and finding a path to cure the disease, which previously caused mortality before the age of 20.

Sociologists say one of the significant achievements of the civil rights movement in the 1970s was the establishment of the Sickle Cell Disease Association of America, and the creation of the Sickle Cell Anemia Act of 1972. Since then, there has been greater public awareness of SCD and incrementally more funding towards finding a cure, culminating in 1982 when St. Jude Children’s Research Hospital conducted the first successful stem cell bone marrow transplant as a therapeutic treatment for SCD.

Bone Marrow Transplant Yields Promising Results for SCD Patients

After conducting a vast amount of research on the latest SCD therapies, Smith recently referred one of his patients to Children's National Hospital for a bone marrow transplantation operation. “I’m thrilled that the procedure was successful, and my patient - for all intents and purposes - is cured, resulting in his being at least 90 percent as healthy as a person in his 20s with no SCD traits,” shared Smith, elated with this remarkable milestone.
Impact of SCD On a Patient’s Life

For those afflicted with SCD, the condition profoundly affects their personal and professional lives, in addition to altering relationships with family and friends.

James Smith, who is an unrelated longtime patient of Dr. Benjamin Smith, has undergone a series of therapeutic treatments at Walter Reed to manage his SCD. Patient Smith found out he had SCD when he was three-years old. Since then, he has overcome many obstacles, aided by the pain care management and physical therapy he has received along the way at Walter Reed.

“I’ve had my spleen and gallbladder taken out,” grimaced Smith while recalling the pain associated with healing from those procedures.
Like Smith, thousands of SCD suffers deal with the collateral effects of the blood disorder. “I’ve missed out on family events, sporting activities, birthdays and college graduations,” emphasized Smith – who has also lost jobs because employers misunderstood the debilitating effects of SCD.

Patient Smith credits Dr. Smith and his staff for saving his life, providing him with hope along his challenging journey.