Sickle Cell Anemia

By:    Published: September 10, 2012

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According to the Centers for Disease Control and Prevention (CDC), sickle cell anemia affects about 90,000 to 100,000 Americans and millions of people throughout the world. While increased awareness has greatly reduced the number of sickle cell-related deaths in recent decades, many people with this condition have less access to comprehensive care than those with other genetic disorders such as cystic fibrosis and hemophilia. Learn more about sickle cell anemia and how it can be treated.

Definition

Sickle cell anemia is an inherited disease. It is a type of anemia, which is a condition where there aren’t a sufficient number of healthy red blood cells in the body to transport enough oxygen. Sickle cell anemia causes these cells to be crescent-shaped, rigid and sticky rather than flexible, round and easily transported like healthy red blood cells. This results in blood vessels getting blocked with irregularly shaped cells, thereby slowing blood flow and preventing oxygen from getting to other parts of the body.

Symptoms

Since sickle cell anemia is inherited; a person has the condition at the time of birth. However, symptoms usually only show up after an infant is about 4 months old. Those symptoms may include:

Anemia: This is a chronic shortage of red blood cells. Unlike healthy red blood cells which live for about 120 days, sickle cells die after about 10 to 20 days. This leads to the body not getting enough oxygen and the following anemia-related symptoms:

  • Fatigue
  • Dizziness
  • Headaches
  • Shortness of breath
  • Rapid heart rate
  • Cold hands and feet
  • Skin looking paler than normal
  • Jaundice

Painful episodes: Sickle cell anemia may lead to sudden episodes of pain called sickle cell crises. The pain, which can last from hours to a week or more, is a result of the blood vessels being blocked by sickle cells, cutting off blood flow to the abdomen, chest and joints. Some episodes of pain are so severe that hospitalization in necessary.

Chronic pain: Some people with this condition also have chronic pain in their bones. This pain may last for weeks or months.

Frequent infections: Sickle cells can cause damage to the spleen, an important infection-fighting organ. These infections can be life-threatening, especially in infants and children. Antibiotics are sometimes given to patients in these age groups to help prevent infections, which are common among those with sickle cell anemia.

Delayed growth: Without sufficient oxygen, the body may not be able to grow on a normal schedule. Growth may be slowed in infants and children with sickle cell anemia, and it can also cause delayed puberty in teens.

Vision problems: Sickle cell anemia can also affect vision by causing tiny blood vessels in and around the eyes to become plugged with sickle cells, damaging the retina.

Hand-foot syndrome: This is a condition in which the hands and feet become swollen. In this case, the swelling is caused by sickle cells blocking the flow of blood out from the hands and feet.

Causes And Risk Factors

Sickle cell anemia is caused by a gene mutation. This mutation directs the body to make an abnormal type of hemoglobin, which is the protein inside red blood cells that carries the oxygen to different parts of the body. When red blood cells have this type of hemoglobin, their shape is changed from round to crescent shaped and they become rigid and sticky. They also deliver less oxygen to tissues in the body.

In order to have sickle cell anemia, a child must inherit the gene mutation from both parents. Having only one parent with the defective gene can result in sickle cell trait, which generally has no symptoms. If both parents are carriers, there is a 25 percent chance with each pregnancy that their child will have sickle cell anemia. Therefore, the risk for having the disease is determined by whether both parents are carriers. However, sickle cell anemia is more common in families from:

  • Africa
  • The Mediterranean region
  • South America
  • Central America
  • The Caribbean
  • The Middle East

Prevention

There is no way to prevent sickle cell anemia since it is an inherited disease. However, parents who are carriers of the sickle cell trait may be able to help prevent their children from having this condition by seeing a genetic counselor before trying to conceive. This will provide them with more information about their future children’s risk of inheriting the disease along with possible preventive measures and reproductive options.

Treatment

In most cases, sickle cell anemia cannot be cured. In some cases, bone marrow transplant may cure the disease, but it is a potentially fatal procedure and finding a donor is difficult.

Fortunately, there are some options for relieving symptoms and preventing sickle cell-related complications. These treatment options include:

  • Immunizations: Getting vaccinated can help prevent infections, which are potentially more dangerous in children with sickle cell anemia.
  • Blood transfusions: This procedure can be completed to increase the number of healthy red blood cells in the body, which relieves anemia.
  • Medication: Antibiotics can help ward off or treat infection, while pain-relief medications can be used during sickle cell crises. Another drug called hydroxyurea can reduce the frequency of painful crises and may reduce the need for blood transfusions.
  • Supplemental oxygen: This can be used for those with difficulty breathing due to their anemia.
  • Evaluating stroke risk: Some children may be at risk for stroke due to their sickle cell anemia. They can be assessed for their risk and given regular blood transfusions if their stroke risk is high.
  • Stem cell transplant: A stem cell transplant involves taking healthy stem cells form a donor and injecting them into the bloodstream of a patient with sickle cell anemia. This is a serious procedure used only for those with significant symptoms or complications from sickle cell anemia.

In addition, making the following lifestyle adjustments can relieve sickle cell-related symptoms:

  • Take folic acid supplements daily
  • Eat a healthy diet
  • Drink plenty of water
  • Reduce stress
  • Exercise regularly
  • Avoid extreme heat or cold
  • Read medication labels carefully since some can constrict blood vessels
  • Be careful when traveling to high-altitude areas
  • Fly only on airplanes with pressurized cabins

Sickle cell anemia is typically a lifelong challenge for those who have the disease, so it is important for these individuals to see a doctor regularly and to discuss their symptoms in detail. In addition, there are many counseling centers which can provide support for those living with sickle cell anemia.

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