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What really is sickle cell anemia?
By Dr Zulfikar Bux
Assistant Professor of Emergency Medicine
Life with sickle cell anemia is literally very painful and debilitating. It is a lifelong condition and is more common in Guyana than we may think. Being undiagnosed with sickle cell anemia leads to unexplained repeated bouts of
illness than can leave those affected by it in a conundrum. Knowing about this disorder is an important step in ensuring it is diagnosed because appropriate treatment will improve quality of life with sickle cell anemia.
What is sickle cell anemia?
Sickle cell anemia, also called sickle cell disease, is a condition that affects the red blood cells. The red blood cells carry oxygen to organs in the body. Normal red blood cells are round.
When people have sickle cell anemia, some of their red blood cells can have an abnormal shape. They look like a crescent (or an old-time tool called a “sickle”). The abnormal red blood cells get stuck in the blood vessels easily, and so they don’t bring enough oxygen to the body’s organs. This can cause pain or organ damage.
Sickle cell anemia is a life-long condition that people are born with. It is caused by an abnormal gene. To get the disease, people need to get the abnormal gene from both their mother and father. If people get the abnormal gene from only one parent, it’s called “sickle cell trait.” People with sickle cell trait do not usually have any of the symptoms of sickle cell anemia. Patients with sickle cell anemia tend to have a life based around regular hospitalizations for treatment of the many different complications that can arise with this illness. Their lives are shortened and sadly they rarely live past the 5th decade of life.
What are the symptoms of sickle cell anemia?
Symptoms usually start after a baby is a few months old. Most commonly, people with sickle cell anemia have episodes of pain. Some doctors use the term “sickle cell crisis.” The pain can be in the bones, chest, or other parts of the body. It can be mild or severe, and last hours to days. People can have other symptoms with their pain, including: Fever, Swelling (for example, in the hands or feet) and Trouble breathing normally.
Most episodes of pain just happen because of the abnormal red blood cells, and can’t be predicted. But sometimes, the pain can be related to other things, such as: Infection – People with sickle cell anemia are more likely than others to get certain infections; Dehydration – Dehydration is when the body loses too much water; Weather or air conditions; Travel to high-altitude places – These are places where there is less oxygen in the air, such as mountains. Flying in an airplane might also cause symptoms for people with sickle cell anemia.
Sickle cell anemia can also cause many different health problems, including: Anemia – Anemia is a condition in which the body has too few red blood cells. It makes people tired or weak; Getting a lot of infections; Lung problems – Symptoms can include trouble breathing or chest pain; Strokes – A stroke is when a part of the brain dies because it goes without blood for too long; Open sores on the skin (usually on the legs); An erection that lasts too long and is very painful (in men).
Who’s at risk?
Sickle cell anemia is an inherited illness and is more common in certain ethnic groups, including:
· People of African descent, including African-Americans (among whom 1 in 12 carries a sickle cell gene)
· Hispanic-Americans from Central and South America
· People of Middle Eastern, Asian, Indian, and Mediterranean descent
Is there a test for sickle cell anemia?
A blood test can show if someone has sickle cell anemia. Some parents choose to test their unborn baby for sickle cell anemia. The most common test done is called Hb Electrophoresis and will tell if you have the abnormal red blood cells associated with sickle cell anemia.
Because sickle cell anemia is one of the most common blood disorders, every newborn in the US is tested for it. Babies with unexplained recurrent illnesses should be tested for sickle cell anemia in our setting. Life with sickle cell anemia can be a bit easier if it is diagnosed.
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