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Sickle cell anemia

Definition

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.

Alternative Names

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

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.

Sickle cell anemia is inherited from both parents. If you get the sickle cell gene from only one 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 one 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:

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

Other signs and symptoms include:

Exams and Tests

Tests commonly done diagnose and monitor patients 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:

Other treatments for sickle cell anemia may include:

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 patients often cannot find well-matched stem cell donors.

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

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 better 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:

References

DeBaun MR, Frei-Jones M, Vichinsky E. Hemoglobinopathies. In: Kliegman RM, Stanton BF, St. Geme JW III, et al., eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 456.

Saunthararajah Y, Vichinsky EP. Sickle cell disease: clinical features and management. In: Hoffman R, Benz EJ Jr, Silberstein LE, et al., eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, Pa: Elsevier Saunders; 2012:chap 40.

Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-2031.


Review Date: 2/24/2014
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.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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