June 19th marked World Sickle Cell Disease Day, promoting education of a blood disorder that affects millions of people worldwide, yet receives little attention in the media.  Sickle cell disease is a disease of the red blood cells, which deliver oxygen throughout the body.  Normally red blood cells are doughnut shaped, which allows them easy passage through the small blood vessels.  Sickle cell disease damages the cells, rendering them spiked and sickle shaped, causing blockage in the small blood vessels.  Cells affected by sickle cell disease also deteriorate faster than normal cells, causing anemia, a separate illness caused by a decrease in red blood cells.

Sickle cell disease most predominantly affects people of African descent, though it has been found in people from Spanish speaking regions, as well as India, Saudi Arabia and the Mediterranean.  1 in 12 African-Americans is a carrier for the disease, while 1 in every 400 is afflicted by it.  A hereditary disorder, sickle cell disease is not contagious, and can only be transmitted from parent to child.  Because it causes red blood cells to become misshapen and block the flow of blood through smaller vessels, some complications of sickle cell disease include organ damage, erectile dysfunction, blindness, stroke and acute chest syndrome. It is a life-threatening illness that can cause chest pain, fever and difficulty breathing.  Some patients with sickle cell disease may also have hypertension, jaundice, skin ulcers and gallstones.

Complications of sickle cell disease are usually treated with pain medication, antibiotics, folic acid to ward off the effects of anemia and blood transfusions to replace lost red blood cells.  More severe cases can be cured with a bone marrow transplant, though success of this treatment depends on the severity of the illness and if a suitable donor match can be located.  The need for registered bone marrow donors is particularly great in the African-American community, which is often left out of education on the registration and donor process.

Get the word out: encourage anyone you know who might be a carrier of sickle cell disease to get tested.  If they’re not a carrier, encourage them to register as a blood, or even better, bone marrow donor.  Registering is quick and easy and can be done through local donor drives or by requesting information online for free.  Blood donation for transfusions takes about an hour, with a minimal amount of pain, while bone marrow donation is considered a minor surgical procedure, with some discomfort involved.  The vast majority of blood and bone marrow donors recover from the procedure quickly with no long-term ill effects.  Risks are minimal for something that could save another person’s life.

Gena Radcliffe

Medex Supply Blogger

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