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Overview

What Is Sickle Cell?

Sickle cell disease is an inherited blood disorder that affects hemoglobin, the protein red blood cells use to carry oxygen. Abnormal hemoglobin can make red blood cells become stiff, sticky, and crescent-shaped instead of round and flexible. These cells may block small blood vessels, break down early, and cause anemia and pain. Sickle cell disease can affect people of many racial and ethnic backgrounds.

Emergency Warning Signs

Emergency Warning Signs of Sickle Cell Disease

Seek emergency care

Know when a crisis is urgent

Severe or unusual symptoms during a possible sickle cell crisis need immediate medical assessment. Seek emergency care for symptoms that may signal infection, breathing problems, stroke, or severe anemia, and do not wait for symptoms to improve on their own.

  • Trouble breathing or chest pain
  • Sudden weakness, numbness, trouble speaking, or confusion
  • A high fever
  • Severe or rapidly worsening pain
  • Fainting or feeling as though you may pass out
  • Extreme tiredness or unusually pale skin
  • Inability to wake up or stay awake

Symptoms

Sickle Cell Disease Symptoms

  • Episodes of severe pain (vaso-occlusive crisis)
  • Tiredness (anemia)
  • Pale skin
  • Yellowing of the eyes or skin (jaundice)
  • Swelling of the hands or feet
  • Frequent infections
  • Delayed growth
  • Vision changes
  • Bedwetting in children

Causes

Causes of Sickle Cell Disease

Sickle cell disease develops when gene variants affecting hemoglobin are inherited from parents. The altered hemoglobin can cause red blood cells to become rigid and crescent-shaped under conditions such as low oxygen or dehydration. These sickle cells carry oxygen less effectively, break down sooner than normal, and can obstruct blood flow.

FeatureSickle cell traitSickle cell disease
Inherited gene patternUsually one sickle hemoglobin gene and one typical hemoglobin geneDisease-causing combinations of hemoglobin gene variants, often including two sickle hemoglobin genes
Usual symptomsUsually no symptoms of sickle cell diseaseMay cause anemia, pain episodes, infections, and other complications
Passing the gene to childrenCan pass the sickle hemoglobin gene to childrenCan pass a disease-associated hemoglobin gene to children

Risk Factors

Risk Factors for Sickle Cell Disease

  • Having parents who carry a sickle hemoglobin gene
  • Having a family history of sickle cell disease or sickle cell trait
  • Having ancestry from regions where sickle hemoglobin genes are more common, including parts of Africa, the Mediterranean, the Middle East, South Asia, and the Caribbean
  • Being part of any racial or ethnic group, because people of any background can have sickle cell disease or sickle cell trait

Complications

Complications of Sickle Cell Disease

  • Recurrent pain crises
  • Acute chest syndrome
  • Stroke
  • Serious infections
  • Severe anemia
  • Kidney problems
  • Eye damage
  • Bone or joint damage
  • Gallstones
  • Delayed growth
  • Pregnancy complications

Regular follow-up and preventive treatment can lower the risk of complications from sickle cell disease. Prognosis varies with the disease type, access to specialist care, and how well an individual treatment plan controls symptoms and protects organs.

Treatment & Management

Sickle Cell Treatment and Management

Medicines and routine prevention

Long-term sickle cell treatment may include a personalized pain-management plan, hydroxyurea or another disease-modifying therapy when appropriate, recommended vaccines, infection prevention, and folic acid or other clinician-directed support. During a sickle cell crisis, clinicians may use fluids, pain medicines, and oxygen when needed, while checking for infection or acute chest syndrome. Treatment should follow an individualized medical plan, because medicines and supportive care are not the same for everyone.

Transfusions and stem cell transplantation

Outlook and Prognosis

Outlook and Prognosis for Sickle Cell Disease

Sickle cell disease is a lifelong inherited condition rather than a temporary illness. Preventive care and disease-modifying treatments can reduce pain crises, infections, and organ damage, although they do not work equally for every person. Ongoing specialist care is important for managing risks and adjusting treatment over time.

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Symptoms

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