Sickle Cell Anemia

Sickle cell anemia is a genetic disorder. It alters the shape of the red blood cells (RBCs). This decreases their ability to carry oxygen. It can also cause acute episodes of pain. These are called a sickle cell crisis. The body will also destroy the sickle cells. The loss of RBCs results in anemia . The change in the ability and number of RBCs can decrease the amount of oxygen for the body.

  • Causes

    Sickle cell disease is a genetic disorder. If you receive one defective gene from each of your parents, then you will have sickle cell disease. If you only have one defective gene, you are said to have sickle cell trait, but not sickle cell disease. Although you won’t usually have any symptoms, you can pass this gene on to your children.

  • Definition


    Sickle cell anemia is a genetic disorder. It alters the shape of the red blood cells (RBCs). This decreases their ability to carry oxygen. It can also cause acute episodes of pain. These are called a sickle cell crisis. The body will also destroy the sickle cells. The loss of RBCs results in
    anemia
    .

    The change in the ability and number of RBCs can decrease the amount of oxygen for the body.

    Red Blood Cells: Normal and Sickle
    IMAGE
    Copyright © Nucleus Medical Media, Inc.

  • Diagnosis

    Hemoglobin electrophoresis is a simple blood test. It can be done by
    most laboratories

    or a local sickle cell foundation. Most states require testing of newborns for sickle cell.

    Amniocentesis
    is a form of prenatal testing. It can also detect sickle cell disease.

  • Prevention

    Sickle cell anemia cannot be prevented. There are some general guidelines that may keep the condition under control. Be sure to discuss these with your doctor:


    • Take daily
      folic acid
      supplements. This will help to build new RBCs.
    • Drink plenty of water to prevent dehydration.
    • Avoid temperature extremes.
    • Avoid overexertion and stress.
    • Get plenty of rest.
    • Get regular check-ups with knowledgeable healthcare providers.
    • Seek genetic counseling.

  • Risk Factors

    Factors that increase your chance for sickle cell anemia include:

    • Race: Blacks of sub-Saharan origin
    • Ethnicity: Greeks, Italians, and people from some parts of India, Central and South America, and the Arabian Peninsula

  • Symptoms

    This condition produces a group of symptoms known as a sickle cell crisis. These are episodes of pain that occur with varying frequency and severity. It is usually followed by periods of remission. The risk for a sickle cell crisis increases with any activity that boosts the body's requirement for oxygen. This may include illness, physical or emotional

    stress, or high altitudes.

    These painful crises can last hours or days. They affect the bones of the back, the long bones, and the chest. The crises can be severe enough to require hospital admission for pain control and IV fluids.

    Symptoms of sickle cell crisis include:

    • Pain and swelling in the hands and feet
    • Fever
    • Jaundice
    • Pale skin color
    • Chest pain, or episodic pain in joints, abdomen, or back
    • Shortness of breath
    • Fatigue
    • Abdominal swelling
    • Unusual or prolonged headache
    • Any sudden weakness or loss of sensation
    • Prolonged erection
    • Sudden vision changes

    • Sudden, severe anemia can cause:

      • Weakness
      • Shortness of breath
      • Loss of consciousness

    Complications of sickle cell anemia include:

    • Destruction of the spleen

    • Severe bacterial infections:

      • Pneumonia
      • Meningitis
      • Kidney and bone infections
    • Damage to the joints, especially hip and shoulder
    • Gallstones
    • Damage to eyes, resulting in impaired vision
    • Stroke
      or other neurological impairment
    • Seizures
    • Liver disease

    • High rate of
      hepatitis C

    • Damage to penis, due to prolonged erection, which may result in
      impotence

    • Leg
      ulcers
    • Heart murmurs
      or enlarged heart
    • Delayed growth
    • Delayed sexual development
    • Problems with thinking, memory, and performance
    • Aplastic crisis or transient red cell aplasia

  • Treatment

    Basic treatment of sickle cell crisis includes:

    • Bed rest
    • Pain relief medicines
    • Oral and IV fluids
    • Oxygen—to reduce pain and prevent complications

    In addition, treatment may include: