September is National Sickle Cell Disease Awareness Month

(Columbia Tribune) September is National Sickle Cell Disease Awareness Month, and health care professionals who work with sickle-cell patients are hoping others will learn more about their risk of one day passing it along to their children.

Sickle cell disease, commonly known as sickle cell anemia, is a disorder in which the red blood cells, which typically are pliable and round, instead are rigid and crescent-shaped.

“When you have that rigidity,” the blood cell “doesn’t flow through the blood vessel like a normal blood cell. They become sticky and want to stick together,” said Elizabeth Gunier, a registered nurse with the Children’s Blood Disorders and Cancer Unit at Children’s Hospital.

When the blood cells don’t flow as they should, they can cause inflammation to the blood vessels’ lining, Gunier said.

The most common manifestation of the disorder is pain, Gunier said, but others can be an increased susceptibility to bacterial infections and a higher risk of stroke, as well as damage to the kidneys, liver or spleen.

However, Gunier said regular treatment and a healthy lifestyle can largely ameliorate symptoms for those with the disease.

Part of prompt and proper treatment relies on parents’ knowing their risk for passing the disease on to their children. Even parents who don’t have the disease can still have the trait. If both parents have the trait, there’s a 25 percent chance their child will have sickle cell disease. The odds jump to 50 percent if one parent has the trait and the other has the disease. For that reason, those most at risk — in the United States, that’s people of African and Hispanic descent — are advised to undergo a genetic test before having children.

Since 1989, all babies born in Missouri are tested for sickle cell disorder. Gunier said those trying to find out whether they have the trait can check with the hospital where they were born if they were born after 1989, or they can ask their primary care doctors to be tested.

Gunier said that infants who test positive for sickle cell disorder are typically referred to a pediatric hematology center to start treatment, which typically includes antibiotics and pain management.