Sickle Cell Disease & African Americans

A sickle cell microscopic image sitting beside a normal blood cellCan you imagine having a child who you have to see spend their entire life suffering from physical complications and indescribable pain? I mean really suffering; not enjoying the life of joy and good health experienced by the children of your friends?

This is the reality for up to a million parents. This is a very painful, limiting, and in more than one case, an often deadly disease. It is particularly unkind to African Americans. In fact, estimates are that 1 in 10 African Americans are carriers of sickle cell.

Sickle cell disease is an inherited condition. Two genes for sickle blood cell must be inherited from one’s parents in order to have the disease. A person who receives a gene for sickle cell disease from one parent and a normal gene from the other has a condition called “sickle cell trait.” A one-in-four chance exists that a child will inherit two normal genes from the parents. One-in-four chances also exist that a child will inherit two sickle cell genes, and have sickle cell disease. A one-in-two chance exists that the child will inherit a normal gene from one parent and a sickle gene from the other. This would produce sickle trait.

However, the life expectancy for sickle cell patients continues to increase as treatments improve. In the past, sickle cell patients did not live to adulthood. Now, the life expectancy is about 45 years. In fact, it is not uncommon for a sickle cell patient to live well into their mid-60’s. These advances do not make sickle cell disease any less deadly or less a health issue of great concern, especially to the African American community.

Sickle cell trait produces no symptoms or problems for most people. Sickle cell disease can neither be contracted nor passed on to another person. The severity of sickle cell disease varies tremendously. Some people with sickle cell disease lead lives that are nearly normal. Most are less fortunate, and can suffer from a variety of complications. These complications can result in the following conditions:

Hand-foot syndrome.When the small blood vessels in the hands or feet are blocked, pain and swelling can result, along with fever. This may be the first symptom of sickle cell anemia in infants.

Fatigue, paleness, and shortness of breath. All symptoms of anemia, or a shortage of red blood cells.

Pain that occurs unpredictably in any body organ or joint, wherever the sickled blood cells block oxygen flow to the tissues. The frequency and amount of pain varies. Some patients have painful episodes (also called crises) less than once a year, and some have as many as 15 or even more episodes in a year. Sometimes the pain lasts only a few hours; somtimes it lasts several weeks. For especially severe, ongoing pain, the patient may have to be hospitalized and treated with painkillers and intravenous fluids. Pain is the principal symptom of sickle cell anemia in both children and adults.

The frequency and amount of pain varies. Some patients have painful episodes (also called crises) less than once a year, and some have as many as 15 or even more episodes in a year. Sometimes the pain lasts only a few hours; somtimes it lasts several weeks. For especially severe, ongoing pain, the patient may have to be hospitalized and treated with painkillers and intravenous fluids. Pain is the principal symptom of sickle cell anemia in both children and adults.

Eye problems. When the retina, the “film” at the back of the eye that receives and processes visual images, does not get enough nourishment from circulating red blood cells, it can deteriorate. Damage to the retina can be serious enough to cause blindness.

When the retina, the “film” at the back of the eye that receives and processes visual images, does not get enough nourishment from circulating red blood cells, it can deteriorate. Damage to the retina can be serious enough to cause blindness.

Yellowing of the skin and eyes. These are signs of jaundice, resulting from the rapid breakdown of red blood cells.

These are signs of jaundice, resulting from the rapid breakdown of red blood cells.

Delayed growth and puberty in children and often a slight build in adults. The slow rate of growth is caused by a shortage of red blood cells.

The slow rate of growth is caused by a shortage of red blood cells.

Infections. In general, both children and adults with sickle cell anemia are more vulnerable to infections and have a harder time fighting them off once they start. This is the result of damage to the spleen from the sickled red cells that prevent the spleen from destroying bacteria in the blood. Infants and young children, especially, are susceptible to bacterial infections that can kill them in as little as nine hours from onset of fever.

In general, both children and adults with sickle cell anemia are more vulnerable to infections and have a harder time fighting them off once they start. This is the result of damage to the spleen from the sickled red cells that prevent the spleen from destroying bacteria in the blood. Infants and young children, especially, are susceptible to bacterial infections that can kill them in as little as nine hours from onset of fever.

Stroke. The defective hemoglobin damages the walls of the red blood cells, causing them to stick to blood vessel walls. This can result in the development of narrowed or blocked, small blood vessels in the brain, causing a serious, life-threatening stroke. This type of stroke occurs primarily in children.

The defective hemoglobin damages the walls of the red blood cells, causing them to stick to blood vessel walls. This can result in the development of narrowed or blocked, small blood vessels in the brain, causing a serious, life-threatening stroke. This type of stroke occurs primarily in children.

Acute chest syndrome. A life-threatening complication of sickle cell anemia, similar to pneumonia that is caused by infection or trapped sickled cells in the lung. This is characterized by chest pain, fever, and an abnormal chest x ray.

A life-threatening complication of sickle cell anemia, similar to pneumonia that is caused by infection or trapped sickled cells in the lung. This is characterized by chest pain, fever, and an abnormal chest x ray.

Now, stop and ask yourself, “When have I ever paid attention to, or supported efforts to combat or help find more effective treatments and/or cures for sickle cell disease?” Or how about asking, “How can I justify standing by and doing nothing to fight such a destructive condition that affects so many people who look like me?”

The time has long come that we all must recognize, become educated, and take action. Scientists have learned a great deal about sickle cell anemia over the years–what causes it, how it affects the patient and how to treat some of the complications. They also have begun to have success in developing treatments that will prevent the symptoms of sickle cell anemia and procedures that should ultimately provide a cure.

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