September is Sickle Cell Awareness Month. Let me give you a brief history about sickle cell disease.
Sickle cell disease is an inherited blood disorder that affects the red blood cells, which are made up of hemoglobin. It is a recessive trait, which means both parents would need to have the sickle or (S) trait or another abnormal hemoglobin that combines with the S hemoglobin in order to have the disease. Sickle cell disease is primarily seen in people of African, Asian and Mediterranean descent. It is a genetic mutation that occurred as protection against malaria. Dr. James B. Herrick, a Chicago physician, first identified the disease in the United States in 1910 in a West Indian dental student. Since then, the focus has been to identify individuals with sickle cell disease early and to prevent serious complications of the disease.
In the United States and Canada, newborn screenings identify sickle cell disease. In Mississippi, about 3,500 individuals live with sickle cell disease. About 900 of those are children. There are about 50 babies diagnosed by newborn screening in Mississippi every year. Individuals with sickle cell disease have problems with acute and chronic pain, as well as serious infections, pneumonia, and are at risk for stroke. Raising awareness not only informs the community about sickle cell disease and the importance of improving routine care, but it also encourages the community to lobby for better care and funds to help with supporting research, treatment and resources for timely treatment of acute issues.
Presently, there is no 100 percent cure for sickle cell disease. There have been incidences where a bone marrow transplant has served as a cure, yet there have also have been incidences where sickle cell disease has returned in patients who underwent transplants.
Sickle cell disease research must continue in order to find better means of treating pain and reducing the incidence of pain by increased use of certain medicines like Hydroxyurea. HU, as it is commonly known, reduces incidences of pain and treats complications of the disease, such as pulmonary hypertension and renal disease, to prevent chronic organ failure. Glutamine has also been shown to help manage incidences of pain. Both of these medicines can be taken by mouth. Blood transfusions happen when certain problems occur unexpectedly in people with sickle cell disease and to prevent other issues including bad infections, a quick drop in the blood count, stroke and problems after surgery. However, many transfusions over time can lead to excess buildup of iron in the body, which can lead to other problems. Special medicines such as Exjade and Desferal help to control iron overload. Ultimately, the future of sickle cell disease research is to find a cure through continued clinical research.
The Mississippi Sickle Cell Foundation is working tirelessly to touch all corners of the state, informing communities about sickle cell disease. Our efforts have not gone unnoticed. Several parent and patient support groups are popping up in various metropolitan areas across Mississippi. The University of Mississippi Medical Center re-opened a day clinic at the main hospital to alleviate wait time in the emergency room while providing specialized care. Our annual activities, such as our Gospel Benefit Program in conjunction with Westhaven Funeral Home Choir, Camp Sickle Stars, Annual Gala, Sickle Cell Walk, Tailgate Party, Education Seminars and Christmas Carnival, help with spreading awareness of sickle cell disease and enhancing the quality of life for individuals and families living with this chronic disease.
You, too, can help “Break the Silence, Give Voice to the Cause, and Work to Find A Cure.” Visit us on Facebook at Mississippi Sickle Cell Foundation and follow us on Twitter @mssicklecell. Get plugged in! Volunteer with our Foundation. Together, we will find a cure.
Dr. Mary Gail Smith is a retired pediatric hematologist/oncologist at UMMC’s Blair E. Batson Children’s Hospital. She is also an advisory board member for the Mississippi Sickle Cell Foundation.