Symptoms and Signs of Sickle Cell Disease

Medical Author: John P. Cunha, DO, FACOEP
Medically Reviewed on 8/1/2022

Doctor's Notes on Sickle Cell Disease

Sickle cell disease is the most common hereditary blood disorder, in which, red blood cells become crescent-shaped (the sickle cell). In healthy individuals, hemoglobin (a molecule in the red blood cell) takes on oxygen and releases carbon dioxide. In sickle cell disease, the red blood cells carry an abnormal hemoglobin known as hemoglobin S which reverses normal hemoglobin activity by releasing oxygen and taking on carbon dioxide (deoxygenation). Sickle cells are very fragile and people with the disease are more likely to get infections. Sickle cell disease occurs almost exclusively among black Americans and black Africans.

Symptoms of sickle cell disease include infections (such as flu viruses, pneumonia, and salmonella). In an acute sickle cell attack (sickle cell crises) severe pain is common, and conditions or symptoms that may contribute to the painful sickle cell crisis include dehydration, infection, fever, decrease in oxygen to body tissue (hypoxia), bleeding, cold exposure, drug and alcohol use, pregnancy, and stress.

Other serious symptoms of sickle cell disease include

What Is the Treatment for Sickle Cell Disease?

Treatments for sickle cell disease include medicines or transfusions to manage complications, including chronic pain. Medication treatment is often overseen by the patient’s hematologist and a pain management specialist.

Treatment for sickle cell disease is life-long and may include:

  • Adequate hydration
  • Drink 8 to 10 glasses of water daily to prevent and treat pain crises
  • Intravenous (IV) fluids may be needed in some cases
  • Medications
    • Voxelotor (Oxbryta)
    • L-glutamine (Endari)
    • Crizanlizumab (Adakveo) 
    • Hydroxyurea (Droxia, Hydrea)
      • Helps the body produce more normal red blood cells 
    • Medicines to prevent and treat infections
      • Vaccines, including a yearly flu shot and Covid vaccines and boosters, to help prevent infections
      • Early use of antibiotics to help prevent infections 
    • Pain medicines 
  • Folic acid (folate) supplementation to helps make red blood cells
  • Blood transfusions to treat anemia and prevent stroke
  • Bone marrow transplant (also called a stem cell transplant) 
    • This procedure can cure sickle cell disease; however, it’s not routinely performed because it has numerous side effects and patients must meet certain criteria
  • Gene therapy (under investigation as a potential treatment)

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REFERENCE:

Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.