Sickle cell anemia is an inherited blood (red blood cells) disorders caused by abnormal hemoglobin (the oxygen-carrying protein within the red blood cells) responsible for the sickled red blood cells. These sickled red blood cells are not able to carry oxygen around the body of the sufferer of the disease.
What did you know about this, one of the most common genetic disorders?
How did this disease that threatens the health of people, especially the black, take root in the history of mankind?
What trait could lead to this life-threatening illness?
What crisis does a person suffering from the disease face?
Are there possible treatments for the disease?
The aforementioned questions will be addressed in this article.
Brief History of Sickle Cell Anemia
The abnormal characteristics of red blood cells was first discovered by Ernest E. Irons, president of the American Medical Association (1949-1950) who led different prominent medical organizations; he was a medical student under Chicago cardiologist and professor of medicine, James B. Herrick, who first discovered the sickle-shaped red blood cells of a medical student in 1910.
When Ernest Irons was examining the student (Walter C. Noel), a dental student from Grenada, he saw ‘peculiar elongated and sickle-shaped cell’ of the student. He (Noel) was admitted to the hospital of Presbyterian in December 1904. Shortly after the report by Professor Herrick, another case appeared in the Virginia Medical Semi-Monthly which has the same title, ‘peculiar elongated and sickle-shaped red blood.’
In a later research and description of the disease, Vern Mason, chairman of the Howard Hughes Medical Institute’s medical advisory committee named the disease, sickle cell anemia.
Dr. Mason also discovered that all patients with the disease were black. This gave the misapprehension that the disease took its origin from the black—Africans.
Sickle Cell Trait
If you inherited a sickle cell gene from one of your parents, you definitely have a condition known as sickle cell trait.
Sickle cell trait is when a person inherits a sickle cell gene from one parent. Sickle cell trait is not the same as sickle cell disease—a condition in which the person inherits two sickle cell genes from both parents.
What is the difference between sickle cell trait and sickle cell disease?
A person battling sickle cell disease has just one type of hemoglobin, known as hemoglobin S (the abnormal hemoglobin). Hemoglobin S turns the person’s normal red blood cells into abnormal curved or sickle shapes.
In contrast, a person with sickle cell trait has both normal hemoglobin A and abnormal hemoglobin S, and as such, he doesn’t suffer from sickle cell disease, still, this person is a carrier and can pass the gene to his children.
Individuals with sickle cell trait enjoy normal lives and are not carriers of the disease.
Even though sickle cell trait can be gotten by anyone, it is most common among those who originate from Africa, the black Caribbean, Middle East, India and the Eastern Mediterranean.
Another thought-provoking question is this, “what causes sickle cell trait?” This could be explained using what you inherit from your [parent]. If a person inherits hemoglobin from one of his parents, he is likely to have one gene of the sickle cell trait.
In a nutshell, if both parents have two sickle cell traits; there is the possibility that their children will inherit the sickle cell trait; in this case, there is a 1 in 4 chance of inheriting a sickle cell disease. But if one parent has the sickle cell disease and the other has the sickle cell trait, there is a 1 in 2 chance of inheriting sickle cell trait and a 1 in 2 chance of inheriting sickle cell disease.
The trait can be detected with a simple blood test. A pregnant woman can take a test to know if baby will have a sickle cell trait or sickle cell disease. Most states in the United States test for the status of the newborn at birth.
In college activities, some colleges test students to check if they have the sickle cell disease or not before performing in any sort of physically demanding activities.
What Is Sickle Cell Crisis?
Sickle cell disease changes the shape of the normal red blood cells. Rather than having a flexible and disc-like shape, the shape of a sickle cell is curved. Because of the curved shape, the blood cannot flow through the veins of the sufferer. So, they clog (block) the small vessels. This is what doctors refer to as sickle cell crisis.
People who have sickle cell crisis have a problem with breathing- they do not find it very easy to breathe. These individuals also suffer from extreme chest and stomach pains. Pains in their back, knees and legs are also associated with sickle cell crisis. The vision of the sufferer suddenly changes. A sufferer also has an extreme loss of consciousness. Painful swelling of hands and feet is also experienced.
Thus, a sufferer is advised to see a doctor if he begins to observe any of those symptoms.
It is not always easy to circumvent the crisis, but learning to prevent it would suffice, to some extents.
This could be done by taking all prescribed drugs. Do not involve yourself in activities that will put you under stress to take in oxygen.
Also, drinking enough water would help to prevent it.
Treatment for Sickle Cell Anemia
Bone marrow transplant, which is also known as stem hematopoietic cell transplant (HSCT) is known to permanently cure children under 16 who are battling this disease. Finding a donor for the disease is extremely challenging if at all the genes will be similar.
Treatments may also include medication to reduce pains and prevent complications as well as a blood transfusion.
Children with sickle cell anemia can also take the antibiotic penicillin when they are 2 months old, and continue until they clock 5.
Adults who are suffering from pneumonia are to take penicillin throughout their life.