Monday, September 8, 2014

GET INFORMED! Sickle Cell Disease: What is it?

GET INFORMED! 
Sickle Cell Disease: What is it?
by Maya Bryant

Maybe you have heard of sickle cell disease, or even possibly know someone with the disease, but do you really know what it is? Sickle cell disease was introduced to me in high school as an inherited disease that affects mostly African-Americans, and then the teacher proceeded to the next topic.  In fear that most Americans also only had a brief synopsis of this major disease, I have created the ultimate guide to teach everyone all of the ins-and-outs of sickle cell disease.  Please follow throughout this series entitled “GET INFORMED!” to learn the p’s and q’s of disorders and diseases such as sickle cell disease and more.

How do blood cells work anyway?


A red blood cell is a mature blood cell that contains hemoglobin which is a protein that carries oxygen throughout the body. Normal red blood cells have hemoglobin A.  These blood cells are biconcave (curving in or hollowed inward) discs that are round and flat: suitable for changing shape and bending in arteries, veins, and tiny capillaries in the body without breaking.  These cells shoot through the body at a rapid speed allowing them to transport oxygen all over the body.  

What does this have to do with Sickle Cell Disease?


People born with sickle cell disease have genetically inherited the trait that makes red blood cells create Hemoglobin S rather than Hemoglobin A.  This causes the red blood cells to be converted into abnormally curved (sickle) shapes.  This abnormal shape decreases their ability to carry oxygen making the person anemic as well as causes the cells to become stiff and sticky.  These cells easily block blood flow in the blood vessels of the limbs and organs which causes pain and organ damage.  Patients with sickle cell also have a higher risk of infection than those who do not have the disorder.  


Normal Red Blood Cells and Sickle Cells

Who is impacted by Sickle Cell Disease?


Sickle Cell Disease affects 72,000 people around the world and is most prevalent in areas where malaria is endemic (ex: Sub Saharan Africa and India).  All races are affected by the disease, but in the United States it mostly affects African Americans.  1 in 12 African American have the sickle cell trait and 1 in 500 African Americans have the disease.  The disease affects roughly 1 in 36,000 Latin Americans. 



Image from http://anthro.palomar.edu/synthetic/synth_4.htm 

Stay tuned for more about Sickle Cell Disease, its effects on its victims, and how Preserve Our legacy is playing a role in helping to fight it.