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Sickle cell anemia

Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease

Sickle cell anemia is a disease passed down through families. The red blood cells that are normally shaped like a disc take on a sickle or crescent shape. Red blood cells carry oxygen throughout the body.

Causes

Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen.

  • Hemoglobin S changes the red blood cells. The red blood cells become fragile and shaped like crescents or sickles.
  • The abnormal cells deliver less oxygen to the body's tissues.
  • They can also easily get stuck in small blood vessels and break into pieces. This can interrupt healthy blood flow and cut down even more on the amount of oxygen flowing to body tissues.

Sickle cell anemia is inherited from both parents. If you get the sickle cell gene from only 1 parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell anemia.

Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East.

Symptoms

Symptoms usually do not occur until after the age of 4 months.

Almost all people with sickle cell anemia have painful episodes called crises. These can last from hours to days. Crises can cause pain in the lower back, leg, joints, and chest.

Some people have 1 episode every few years. Others have many episodes each year. The crises can be severe enough to require a hospital stay.

When the anemia becomes more severe, symptoms may include:

Younger children with sickle cell anemia have attacks of abdominal pain.

The following symptoms may occur because small blood vessels become blocked by the abnormal cells:

  • Painful and prolonged erection (priapism)
  • Poor eyesight or blindness
  • Problems with thinking or confusion caused by small strokes
  • Ulcers on the lower legs (in adolescents and adults)

Over time, the spleen stops working. As a result, people with sickle cell anemia may have symptoms of infections such as:

  • Bone infection (osteomyelitis)
  • Gallbladder infection (cholecystitis)
  • Lung infection (pneumonia)
  • Urinary tract infection

Other signs and symptoms include:

  • Delayed growth and puberty
  • Painful joints caused by arthritis
  • Heart or liver failure due to too much iron (from blood transfusions)

Exams and Tests

Tests commonly done to diagnose and monitor people with sickle cell anemia include:

Treatment

The goal of treatment is to manage and control symptoms, and to limit the number of crises. People with sickle cell disease need ongoing treatment, even when not having a crisis.

People with this condition should take folic acid supplements. Folic acid helps make new red blood cells.

Treatment for a sickle cell crisis includes:

  • Blood transfusions (may also be given regularly to prevent stroke)
  • Pain medicines
  • Plenty of fluids

Other treatments for sickle cell anemia may include:

  • Hydroxyurea (Hydrea), which helps reduce the number of pain episodes (including chest pain and breathing problems) in some people
  • Antibiotics, which help prevent bacterial infections that are common in children with sickle cell disease
  • Medicines that reduce the amount of iron in the body.

Treatments that may be needed to manage complications of sickle cell anemia include:

Bone marrow or stem cell transplants can cure sickle cell anemia, but this treatment is not an option for most people. Sickle cell anemia people often cannot find well-matched stem cell donors.

People with sickle cell disease should have the following vaccinations to lower the risk of infection:

  • Haemophilus influenzae vaccine (Hib)
  • Pneumococcal conjugate vaccine (PCV)
  • Pneumococcal polysaccharide vaccine (PPV)

Support Groups

Joining a support group where members share common issues can relieve the stress of a chronic disease.

Outlook (Prognosis)

In the past, people with sickle cell disease often died between ages 20 and 40. Thanks to a modern care, people now can live to the age of 50 and beyond.

Causes of death include organ failure and infection.

When to Contact a Medical Professional

Call your health care provider if you have:

  • Any symptoms of infection (fever, body aches, headache, fatigue)
  • Pain crises
  • Painful and long-term erection (in men)

References

Heeney MM, Ware RE. Sickle cell disease. In: Orkin SH, Fisher DE, Ginsburg D, Look AT, Lux SE, Nathan DG, eds. Nathan and Oski's Hematology and Oncology of Infancy and Childhood. 6th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 20.

National Heart Lung and Blood Institute. Evidence-based management of sickle cell disease: expert panel report 2014. www.nhlbi.nih.gov/sites/www.nhlbi.nih.gov/files/sickle-cell-disease-report.pdf. Accessed February 19, 2016.

Steinberg MH. Sickle cell disease and other hemoglobinopathies. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 163.

    • Red blood cells, sickle cell

      Red blood cells, sickle cell - illustration

      Sickle cell anemia is an inherited blood disease in which the red blood cells produce abnormal pigment (hemoglobin). The abnormal hemoglobin causes deformity of the red blood cells into crescent or sickle-shapes, as seen in this photomicrograph.

      Red blood cells, sickle cell

      illustration

    • Red blood cells, normal

      Red blood cells, normal - illustration

      This photomicrograph shows normal red blood cells (RBCs) as seen in the microscope after staining.

      Red blood cells, normal

      illustration

    • Red blood cells, multiple sickle cells

      Red blood cells, multiple sickle cells - illustration

      Sickle cell anemia is an inherited disorder in which abnormal hemoglobin (the red pigment inside red blood cells) is produced. The abnormal hemoglobin causes red blood cells to assume a sickle shape, like the ones seen in this photomicrograph.

      Red blood cells, multiple sickle cells

      illustration

    • Red blood cells, sickle cells

      Red blood cells, sickle cells - illustration

      These crescent or sickle-shaped red blood cells (RBCs) are present with Sickle cell anemia, and stand out clearly against the normal round RBCs. These abnormally shaped cells may become entangled and block blood flow in the small blood vessels (capillaries).

      Red blood cells, sickle cells

      illustration

    • Red blood cells, sickle and pappenheimer

      Red blood cells, sickle and pappenheimer - illustration

      This photomicrograph of red blood cells (RBCs) shows both sickle-shaped and Pappenheimer bodies.

      Red blood cells, sickle and pappenheimer

      illustration

    • Formed elements of blood

      Formed elements of blood - illustration

      Blood transports oxygen and nutrients to body tissues and returns waste and carbon dioxide. Blood distributes nearly everything that is carried from one area in the body to another place within the body. For example, blood transports hormones from endocrine organs to their target organs and tissues. Blood helps maintain body temperature and normal pH levels in body tissues. The protective functions of blood include clot formation and the prevention of infection.

      Formed elements of blood

      illustration

    • Blood cells

      Blood cells - illustration

      Blood is comprised of red blood cells, platelets, and various white blood cells.

      Blood cells

      illustration

      • Red blood cells, sickle cell

        Red blood cells, sickle cell - illustration

        Sickle cell anemia is an inherited blood disease in which the red blood cells produce abnormal pigment (hemoglobin). The abnormal hemoglobin causes deformity of the red blood cells into crescent or sickle-shapes, as seen in this photomicrograph.

        Red blood cells, sickle cell

        illustration

      • Red blood cells, normal

        Red blood cells, normal - illustration

        This photomicrograph shows normal red blood cells (RBCs) as seen in the microscope after staining.

        Red blood cells, normal

        illustration

      • Red blood cells, multiple sickle cells

        Red blood cells, multiple sickle cells - illustration

        Sickle cell anemia is an inherited disorder in which abnormal hemoglobin (the red pigment inside red blood cells) is produced. The abnormal hemoglobin causes red blood cells to assume a sickle shape, like the ones seen in this photomicrograph.

        Red blood cells, multiple sickle cells

        illustration

      • Red blood cells, sickle cells

        Red blood cells, sickle cells - illustration

        These crescent or sickle-shaped red blood cells (RBCs) are present with Sickle cell anemia, and stand out clearly against the normal round RBCs. These abnormally shaped cells may become entangled and block blood flow in the small blood vessels (capillaries).

        Red blood cells, sickle cells

        illustration

      • Red blood cells, sickle and pappenheimer

        Red blood cells, sickle and pappenheimer - illustration

        This photomicrograph of red blood cells (RBCs) shows both sickle-shaped and Pappenheimer bodies.

        Red blood cells, sickle and pappenheimer

        illustration

      • Formed elements of blood

        Formed elements of blood - illustration

        Blood transports oxygen and nutrients to body tissues and returns waste and carbon dioxide. Blood distributes nearly everything that is carried from one area in the body to another place within the body. For example, blood transports hormones from endocrine organs to their target organs and tissues. Blood helps maintain body temperature and normal pH levels in body tissues. The protective functions of blood include clot formation and the prevention of infection.

        Formed elements of blood

        illustration

      • Blood cells

        Blood cells - illustration

        Blood is comprised of red blood cells, platelets, and various white blood cells.

        Blood cells

        illustration

      A Closer Look

       

      Tests for Sickle cell anemia

       

      Review Date: 2/1/2016

      Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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