What is the Difference between Sickle Cell Anemia & Sickle Cell Disease?
So What is Sickle Cell?
These are some of the main questions that I am asked on a regular basis. Sickle Cell Anemia is a direct result of Sickle Cell Disease, which is an inherited disease of the red blood cells. Many think it’s just a black person’s disease , but truly it effects people of color from Spanish speaking countries, Turkey, Arabian, Italian, Sardinian, Greek, Maltese, and Indian ancestry.
Also, there are several types of this disease:
“SS”-The most deadly & common inherited form is when one sickle cell gene is taken from both parents.
“SC”- This is the form that I have, as well as my baby son, because his father did not know he was a carrier. My parents didn’t know one had the sickle cell gene & one abnormal hemoglobin gene “C”.
S-Beta thalassemia- one sickle cell gene & one beta thalassemia.
The red blood cells have a protein inside called hemoglobin, which are abnormal in people with this hereditary blood disorder. One out of 500 African-Americans have this abnormal hemoglobin which causes the red blood cells to become a stiff sickle(like the farm tool) shaped. These stiff sickled shaped cells clump in the tiny blood vessels and can cause organ damage, and causes painful episodes called crises because the blood supply is cut off.
These abnormal cells die quickly resulting in anemia, which is why sicklers have to have transfusions.
But, every individual is effected differently. When some have to be hospitalized frequently as I did, and a few have healthier normal lives, in which I have now as an adult. But, solely because of this new drug treatment of Hydroxyurea, chemo in a pill, that has decreased the number of painful episodes that a person may have while taking this drug.
My hospitalizations have gone from every 3 months @2-3 weeks at a time. While being given narcotics thru (IV push) or (IV piggyback)intravenously or (IM)in the muscle, and IV fluids(sodium chloride) or (potassium chloride) along with oxygen. However, being in and out of conscientiousness was definitely not considered a day at a 5 star resort, but I was receiving treatment some countries didn’t offer to Sickle Cell patients. However, some sicklers level of pain, whether it's a mild crisis or severe, are treated as individuals. I am told some can be treated at home with less powerful drugs, although I never met anyone suffering with this disease tolerate the pain with over the counter drugs.
Meanwhile, being on this new drug I have been hospitalized less. I am down to 1 crises a year now & counting. Besides, this new drug causes the body to make and form fetal hemoglobin. This is preventative towards red blood cells from sickling. But, the medicine does not stop a crises if it has started. It weakens your immune system which, furthermore lowers the bodies abilities to fight off infection. Infections are the enabler, which causes sickling.
Written by: Zenobia Jackson
Also, blood stem cell transplant that come from the bone marrow & a few from umbilical cord blood have been found as the cure for Sickle Cell patients, a small study showed for about 85%. Also, artificial bone marrow transplants are being done, for half siblings. Finally, something to look forward to.
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