September is “National Sickle Cell Awareness Month”, in which, focused attention is given to the need for research and treatment of sickle cell disease, the inherited condition that currently affects 70,000-100,000 Americans, primarily African Americans and persons of Mediterranean descent.
Sickle Cell Anemia is a disorder of the body’s red blood cells. The condition occurs at birth , being passed from one or both parents. In other words, therefore, a person is either born with it, or not. If they are, then they will always have it. A major symptom of sickle cell anemia is severe painful attacks. However, there is much better treatment for sickle cell anemia in recent years than in the past.
Normal red blood cells are round, shaped like doughnuts, and move easily through small blood vessels or tubes in the body that carry oxygen to the tissues. By comparison, after sickle cells carry oxygen to the tissues, they become hard and shaped like sickles that are used for cutting tall grass and wheat. When these hard and pointed cells pass through small blood vessels, they slow down the flow and break. As a result, they often cause pain, damage to various body tissues, and a low blood count (or anemia).
Why Red Blood Cells Sickle
There is an iron-containing protein in the red blood cells called hemoglobin, which carries oxygen to the body’s tissues. In most people, the main type of hemoglobin, which is inherited, is called hemoglobin A. However, people who develop sickle cells inherit a different type of hemoglobin, called hemoglobin S, because of one little change in the protein. Hemoglobin S forms long rods in the red blood cells after they deliver oxygen. As a result of the long rods, instead of the red cells staying round, they form the sickle shape. People with sickle cell anemia have hemoglobin type SS.
How Sickle Cell Anemia is Inherited
Hemoglobin is inherited from a persons’ parents. If the person inherits one sickle cell gene, he or she will have sickle cell trait. On the other hand, if the person inherits two sickle cell genes, he or she will have sickle cell anemia. He or she might also be said to have sickle cell disease, which term is used for several related inherited red blood cell disorders.
Racial and Ethnic Groups that Get Sickle Cell Anemia (SCD)
Sickle cell anemia occurs in many racial and ethnic groups including African-Americans, others of African descent, Arabs, Greeks, Italians, Latin Americans, persons from India, and Caucasians. Therefore, all newborns should be tested for sickle cell anemia, which involves a simple blood test.
Some people with sickle cell anemia can live productive lives and enjoy most of the activities that people without it do. Following are some things that people with sickle cell anemia can do to stay as healthy as possible:
- Get regular checkups. Regular medical checkups to decrease the chance of serious problems.
- Prevent infections. Common illnesses, like influenza quickly can become dangerous for a person with SCD. The best defense is to take simple steps to help prevent infections.
- Learn healthy habits. People with SCD should drink 8 to 10 glasses of water every day and eat healthy food. They also should try not to get too hot, too cold, or too tired.
- Look for clinical studies. New clinical research studies are happening all the time to find better treatments and, hopefully, a cure for SCD. People who take part in these clinical studies might receive access to new medicines and treatments.
- Get support. People with SCD, and their parents, should find a patient support group or other organization in the community that can provide information, assistance, and support.
There’s no cure for most people with sickle cell anemia. But treatment can relieve pain and help prevent problems associated with the disease. Treatment might can also include hydroxyurea and folic acid supplements. On occasion, blood transfusions are given, and in some instances, patients are given a bone marrow transplant.
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