Sickle Cell Anemia

What is Sickle Cell Anemia?

Sickle cell anemia (sickle cell disease) is a genetic disease that causes red blood cells to become sticky, stiff and crescent moon or sickle shaped. One in 12 African Americans carry the sickle cell gene and have what is called sickle cell trait. Those with sickle cell anemia carry two sickle cell genes, and the disease affects 1 in 375 African American babies.

Because the blood cells are not round and healthy, they do not easily travel through the bloodstream. They also die-off at around 20 days. That’s 100 days sooner than normal healthy cells. As a result, those with the disease become chronically anemic. The sickle-shaped cells build up and prevent oxygen-rich blood from reaching certain areas of the body. It can cause painful and debilitating complications.

What are the Symptoms of Sickle Cell Anemia?

Infants in the USA are checked for sickle cell anemia during routine screenings. However, symptoms do not usually appear before 4 months of age. Symptoms of sickle cell anemia may include:

  • Swollen and painful hands and feet (dactylitis)
  • Fussiness or irritability
  • Painful flair-ups, especially in the legs, arms, chest, lumbar region and abdomen
  • Jaundice
  • Anemia (low blood-iron levels)
  • Fatigue
  • Recurring infections
  • Growth delays in babies and children
  • Vision difficulties

Symptoms that require immediate medical care include:

  • Jaundice
  • Pallid nails and/or skin
  • High temperature
  • Swollen and painful hands and feet (dactylitis)
  • Signs of a stroke
  • Confusion
  • Facial or limb weakness or paralysis
  • Numbness
  • Head pain
  • Problems speaking
  • Trouble walking
  • Sudden vision difficulties

Sickle Cell Anemia Causes

An inherited abnormal hemoglobin (the oxygen-carrying protein in the red blood cells) causes Sickle Cell Anemia. Sick red blood cells are the result of the abnormal hemoglobin. These red blood cells are very fragile and susceptible to burst. The moment these cells reduce in number due to Hemolysis, anemia begins.

A gene unlinked to a sex chromosome – an Autosomal recessive condition, is inherited, leading to a condition known as Sickle Cell Anemia. An inherited sickle cell gene from both parents results in a child with two sickle cell genes. In some cases, only one cell is inherited thus the child becomes a sickle cell child carrier.

Due to the weakening of the red blood cells in patients with sickle cell anemia, the cells take up an abnormal shape and reduced flexibility. The following conditions are associated with little oxygen levels, dehydration of the blood and increased acidity causing the weakening of the red blood cells. The conditions mentioned above occur because of injury to the body tissue or anesthesia.

How is Sickle Cell Anemia Treated?

There are no curative treatments for sickle cell anemia, but the symptoms can be treated and complications can be avoided. Treatment may include:

  • Antibiotics
  • Pain medication
  • Applying heat for pain
  • Hydroxyurea (may reduce number of painful flair-ups)
  • Blood transfusions
  • Oxygen
  • Stem cell therapy
  • Treatment of complications

Sickle Cell Anemia Prevention

The wise men say ‘prevention is better than cure.’ The first step to prevention is asking your doctor to carry out a blood test. Ignorance has no defense. You may be carrying an abnormal hemoglobin gene without your knowledge. For couples who are expecting a child or planning to have children and know they are at risk, need to meet a genetics counselor. The counselor will respond to all their concerns and explain available options. One other thing is to take a lot of folic acid supplements, watch your diet, avoid extreme temperatures and drink a lot of water to stay healthy.

Last Reviewed:
October 10, 2016
Last Updated:
November 08, 2017
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