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Sickle Cell Anemia

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
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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.

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:
  • 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

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.

Treatment

Basic treatment of sickle cell crisis includes:

In addition, treatment may include:

Penicillin

Newborns with sickle cell disease may be given penicillin . It is given twice a day, beginning at age two months. It is continued until at least five years of age.

Pneumococcal Vaccine

It is recommended that children with sickle cell disease who are 2-5 years of age receive pneumococcal vaccine (PCV-7) . Other routine immunizations should also be given, such as an annual flu shot.

Hydroxyurea (Hydrea)

Hydroxyurea is the first drug to significantly prevent complications of sickle cell disease. It increases the production of fetal hemoglobin. This decreases the number of deformed RBCs. As a result, it reduces the frequency of sickle cell crisis. Hydrea is not appropriate for everyone with sickle cell disease.

Blood Transfusions

Blood transfusions can treat and prevent some of the complications. Transfusion therapy can help prevent recurring strokes in children.

Bone Marrow Transplant

A bone marrow transplant from a matched donor may be effective. There are medical risks involved. Recipients must also take drugs that suppress the immune system for the rest of their lives.

If you or your child is diagnosed with sickle cell anemia, follow your doctor's instructions .

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.

Revision Information

  • American Sickle Cell Anemia Association

    http://www.ascaa.org

  • Sickle Cell Information Center

    http://scinfo.org

  • About Kids Health

    http://www.aboutkidshealth.ca

  • Sickle Cell Foundation of Alberta

    http://www.sicklecellfoundationofalberta.org

  • Furie KL, Kasner SE, et al. Guidelines for the Prevention of Stroke in Patients With Stroke or Transient Ischemic Attack: A Guideline for Healthcare Professionals From the American Heart Association/American Stroke Association. Stroke . 2010 October 21.

  • Mehanna AS. Sickle Cell Anemia and Antisickling Agents then and now. Curr Med Chem . 2001;8:79-88.

  • Sickle cell disease. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated June 6, 2013. Accessed July 1, 2013.

  • Sickle cell disease. Nemours' KidsHealth.org website. Available at: http://kidshealth.org/parent/medical/heart/sickle%5Fcell%5Fanemia.html . Updated September 2012. Accessed July 1, 2013.

  • Sickle cell disease (SCD). Centers for Disease Control and Prevention website. Available at: http://www.cdc.gov/ncbddd/sicklecell/index.html . Updated September 27, 2012. Accessed July 1, 2013.

  • Sickle Cell Disease. American Academy of Family Physicians' Family Doctor.org website. Available at: http://familydoctor.org/familydoctor/en/diseases-conditions/sickle-cell-disease.html . Updated January 2011. Accessed July 1, 2013.

  • What is sickle cell anemia? National Heart, Lung, and Blood Institute website. Available at: http://www.nhlbi.nih.gov/health/health-topics/topics/sca/ . Updated September 28, 2012. Accessed July 1, 2013.

  • 1/4/2011 DynaMed's Systematic Literature Surveillance https://dynamed.ebscohost.com/about/about-us : Vichinsky EP, Neumayr LD, Gold JI, et al. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2010;303(18):1823-1831.