Empower Yourself or a Loved One: Questions to Ask Your Doctor about Managing Sickle Cell Disease

Sickle Cell Disease (SCD) describes a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells. Sickle cell disease is a painful and potentially fatal disease that affects approximately 100,000 Americans, mostly African-Americans, every year. We’ve struggled for years to find a cure and treatments, and the latest news is encouraging. Bone marrow stem cell transplantation represents the strongest potential cure for sickle cell disease, but scientists are still working to refine this therapy so that it can ultimately be widely available. For now, it is still important for patients and their families to obtain as much medical knowledge as possible about this potentially debilitating genetic condition so they can live the healthiest, pain-free lives possible. Speak with your health care provider to come up with a plan for preventing or reducing the complications of sickle cell disease, such as severe pain, infections, and stroke.

The National Heart, Lung, and Blood Institute (NHLBI), part of the National Institutes of Health, offers the following questions as examples of topics that patients might discuss in detail with their doctors:

• How do I develop a health maintenance plan for my sickle cell disease? This plan should involve having the routine immunizations and screenings recommended by the Advisory Committee on Immunization Practices for all individuals, as well as the additional immunizations recommended for persons with sickle cell disease. It also should include preventive measures such as drinking plenty of water, avoiding exposure to extreme weather conditions, and getting adequate rest. Immunization recommendations as well as other treatment guidelines are located at http://1.usa.gov/1IhXvid. It is important to always take the medications as prescribed by your physician and to speak with your doctor first, before changing any medication.
• What can I do to prevent infections? Talk to your doctor about the use of vaccines and penicillin to prevent infections, such as pneumonia and meningitis. How do I know if treatments like hydroxyurea [hi-droxee-you-REE-ah] are right for me? Hydroxyurea is an oral medication that can help reduce the frequency of pain episodes, or “crises,” and acute chest syndrome (a condition in which lung tissue is damaged) in many individuals. Your doctor can provide additional information on hydroxyurea and together you can decide if this is a medication that could help you.
• Should blood transfusions be a part of my care? Blood transfusions can be an effective therapy for individuals with sickle cell disease, particularly those at risk for stroke. Patients receiving frequent transfusions should be monitored closely for side effects like iron buildup. Your doctor can help you decide when this option is right for you.

If you do not have sickle cell disease, but would like to support the community, here are some examples of steps you can take:

• Become a blood donor. About 5 percent of the eligible U.S. population gives blood. Only about 1 percent of the African-American community donates. Blood donations may save lives.
• Consider enrolling in clinical trials. In order for us to advance our knowledge about sickle cell disease and other diseases that disproportionately affect the African-American community, we need participation from all members of the community in clinical trials. To learn more about clinical trials (including sickle cell disease trials), visit www.clinicaltrials.gov.

Thanks to advances in science, people with sickle cell disease are living longer, more productive lives than in the past. NHLBI is committed to sickle cell disease research and director Dr. Gary H. Gibbons believes that with the scientific possibilities before us, we have the potential to realize a generation of children with sickle cell disease who can live without fear of suffering a stroke – a stroke-free generation. I’m excited to be a part of a team that’s working to turn that vision into reality. To learn more about sickle cell disease, visit NHLBI’s website: http://www.nhlbi.nih.gov/health/health-topics/topics/sca.