Saturday, September 6, 2014

The Facts on Sickle Cell Anemia

September is Sickle Cell Awareness Month and for a disease that affects so many minorities in this country it was important to shed some light on it.

What is it??

Sickle Cell Disease or (SCD) is a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle”. The sickle cells die early, which causes a constant shortage of red blood cells.

Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems such infection, acute chest syndrome and stroke. Other effects of the disease include bad reaction in high altitudes due to less oxygen and dehydration. There are a list of others and treatments here. One of the few pluses to the disease is that carriers of the trait are less susceptible to malaria. 

What some may not know is that there are actually 6 different forms of the disease and 1 that is a trait carrier. The most common strain is HbSS, People who have this form of SCD inherit two sickle cell genes (“S”), one from each parent. This is commonly called sickle cell anemia and is usually the most severe form of the disease. Others strains include: HbS beta thalassemia, HbSC, HbSD, HbSE, and HbSO.

People also have what is referred to as the Sickle Cell Trait (SCT). The strain is called HbAS. People who have SCT inherit one sickle cell gene (“S”) from one parent and one normal gene (“A”) from the other parent. This is called sickle cell trait (SCT). People with SCT usually do not have any of the signs of the disease and live a normal life, but they can pass the trait on to their children. Additionally, there are a few, uncommon health problems that may potentially be related to sickle cell trait.

Who does it effect??

It is estimated that:
  • SCD affects 90,000 to 100,000 Americans.
  • SCD occurs among about 1 out of every 500 Black or African-American births.
  • SCD occurs among about 1 out of every 36,000 Latino-American births.
  • SCT occurs among about 1 in 12 Blacks or African Americans.
  • Some famous people with the disease are: Singer Tionne "T-Boz" Watkins from  TLC, Rapper Prodigy from Mobb Deep, Former NFL athlete Tiki Barber, the late Jazz legend Miles Davis, and Ryan Clark of the Pittsburgh Steelers who cannot play in Denver because of the elevation.
What is the cure??

There is no widely available cure for SCD. Several emerging new treatments are currently being evaluated. In young patients with severe SCD, a stem cell transplant involving the transplant of bone marrow of a patient with SCD with healthy bone marrow from a donor, ideally from a sibling without SCD, could be an option. However, although bone marrow transplant can cure SCD in small number of patients, it can have life-threatening side-effects in other patients.


Written by E. Rey

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