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Sickle Cell Disease

What is Sickle Cell Disease?

A genetic disease, most prevalent in the African-American community, sickle cell disease (also known as “sickle cell anemia”) is a disease in which red blood cells are an abnormal crescent shape. Red blood cells are normally shaped like a disc. They clump together, blocking blood vessels and creating intense pain. The disease’s other “crises” can be divided into three groups: 

  • Hemolytic crisis occurs when damaged red blood cells break down rapidly
  • Splenic sequestration crisis is when the spleen enlarges and traps the blood cells
  • Aplastic crisis results when an infection causes the bone marrow to stop producing red blood cells

Symptoms

Common symptoms include:

  • Attacks of abdominal pain
  • Bone pain
  • Breathlessness
  • Delayed growth and puberty
  • Fatigue
  • Fever
  • Jaundice
  • Paleness
  • Rapid heart rate
  • Susceptibility to infections
  • Ulcers on the lower legs (in adolescents and adults)
  • Strokes

Diagnosis

Tests commonly performed to diagnose and monitor patients with sickle cell anemia include:

  • Complete blood count (CBC)
  • Hemoglobin electrophoresis
  • Sickle cell test
  • Sonograms to look for abnormal blood flow in the head
  • X-rays and MRIs

Treatments

Common treatments for sickle cell disease include:

  • Pain medicines to treat painful crisis
  • Fluids
  • Oxygen
  • Antibiotics to prevent and treat crises
  • Immunizations
  • Hydroxyurea (a drug that increases fetal hemoglobin and prevents painful crisis)
  • Transfusions
  • Bone Marrow Transplantation

Sickle Cell Disease is treated by our Division of Pediatric Hematology


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