What Is Sickle Cell Anemia
Sickle Cell Anemia
Sickle cell anemia is a very common (affects 70,000 to 100,000 Americans) blood disorder that has several different characteristics.
These cells to form in a sickle or crescent shape
They stick together and form long, rod like structures
Sickle cells cannot travel normally through blood vessels, like normal RBCs
They also block the blood vessels, which restricts normal blood flow throughout the body
Sickle cells only survive ten to twenty days
Human blood is composed of red blood cells (40-45%), plasma (water, sugar, fat, salt, protein), white blood cells (disease fighting), and platelets. Blood has many responsibilities including:
Prevents excessive blood loss by forming blood clots
Transports waste products to the kidneys (urea) and liver to be filtered and cleaned
Body temperature regulation
Since sickle cells do not survive longer than twenty days, the body cannot possibly keep up with the production needs, which is what causes the anemia.
Signs And Symptoms
When the sickle cells block the blood vessels individuals will begin to experience moderate to severe symptoms of the disorder.
Acrocyanosis (extremities are cold to touch, with a bluish color)
Jaundice (yellow pigmentation) of the skin and sclera (whites of the eye ball)
Joint Crisis involves painful joints and poor range of motion
Acute chest syndrome is characterized by angina (chest pain), hemoptysis (coughing up blood), and dyspnea
Abdominal crisis is characterized by abdominal pain, nausea, vomiting, and diarrhea
Sickle cell anemia is most often diagnosed by one type of blood test, hemoglobin S. This test identifies the presence of hemoglobin S and the number of RBCs and hemoglobin level in the blood.
Sickle cell anemia can sometimes be treated with a allogeneic transplant (bone marrow). Prior to the transplant the individual is administered chemotherapy and sometimes radiation. The donor is most often a family member. This type of treatment can last over several weeks to months.
Other forms of treatment include pain management and hydroxyurea. Hydroxyurea is an oral medication that boosts the body’s production of fetal hemoglobin (hemoglobin F). This drug has been scientifically tested and is effective in reducing the sickle cell crisis incidents and the need for red blood cell transfusions. Individuals taking hydroxyurea must undergo routine blood testing
Major Complications Associated with Sickle Cell Anemia
Sickle cell anemia can potentially cause organ (kidney, liver) damage and make individuals at a higher risk for infection, eye damage (retinopathy), myocardial infarction (heart attack) and CVA (stroke).
It is not uncommon for men to suffer with priapism (prolonged erection).
Factors Of Acute Sickle Cell Crisis
There are several factors that may be linked to the onset of an acute sickle cell crisis.
Excessive Drug and Alcohol use
Sickle cell anemia is a genetic disorder, where the individual will inherit a sickle cell from each parent. Sickle cell trait (not the disease) is when the individual will inherit one sickle cell gene from one parent and a normal gene from the other. Hemoglobin SC is when the individual inherits a sickle cell gene from one parent a different abnormal gene from the other.
An individual with the sickle cell trait will not exhibit symptoms of the disorder but acts as a carrier and can pass it on to their children.
Genetic testing for sickle cell anemia has become a normal part of the newborn screening process in the United States. A DNA screening involves the collection of blood, skin, bone, or other body tissues, which and be used in the testing for genetic diseases. Chorionic villus sampling (first trimester) and amniotic fluid sampling (amniocentesis, second & third trimester) may also be required to get a better diagnosis.