Sickle Cell Multidisciplinary Clinic
Lifespan Cancer Institute

Complications and Treatment of Sickle Cell Disease

Symptoms and complications of sickle cell disease vary depending on which type a patient inherited, as well as other health factors. The most common symptoms are pain and anemia. Preventing organ damage is a primary treatment goal, along with pain management and prevention of infections. Treatment is varied depending on the complications experienced:

  • pain medication to prevent or treat pain
  • Hydrea to prevent complications
  • folic acid to help to make new red blood cells
  • antibiotics to treat infections
  • vaccinations to prevent diseases that would be especially risky for those with sickle cell disease
  • blood transfusions to treat complications and help prevent stroke
  • bone marrow transplant for some patients with severe symptoms

Patients can help minimize the risks of sickle cell crises (episodes of severe pain) by drinking at least eight glasses of water each day. Being well-hydrated helps to prevent the sickle cells from sticking together to form the clumps that block blood vessels. Patients also should avoid extreme heat or cold, travel to high altitudes, and air travel. Getting plenty of sleep and following a healthy diet also help maintain health.

Serious Complications

Because sickle cell disease can cause damage throughout the body, including to major organs, serious complications include:

Anemia

The most common symptom of sickle cell disease, anemia, is caused by an insufficiency of healthy red blood cells to deliver oxygen in the body. An anemic patient typically feels tired, and may experience shortness of breath or dizziness. Treatments include taking the medication hydroxyurea and taking the vitamin folic acid for prevention. Blood transfusions treat acute anemia.

Hydroxyurea is a medication that causes the body to make a type of hemoglobin normally found only in fetuses and very young children, which has been found to lessen side effects and complications of sickle cell disease.

Vaso-occlusive Crisis

In a vaso-occlusive crisis, sudden, pronounced pain is caused when the blood flow is limited or blocked by blood cells that cluster in the small vessels. The site of the pain depends on where the blockage is located. A vaso-occlusive crisis can be life-threatening and should be treated immediately.

To avoid a pain crisis, patients are advised to drink a minimum of eight glasses of water each day to prevent dehydration. If a crisis occurs, a patient will receive intravenous fluids and pain medications (anti-inflammatories and opioids).

Acute Chest Syndrome

Acute chest syndrome is caused when blood flow is blocked or impeded in the lungs, resulting in pneumonia-like symptoms (difficulty breathing, fever, pain, cough). This can be life-threatening and should be treated immediately.

Cerebrovascular Crisis and Stroke

A blockage in the blood vessels that supply the brain can result in brain damage. Patients who have had a cerebrovascular crisis or stroke, or who have been determined through testing to be at risk, may undergo periodic blood transfusions to minimize the risk of this complication.

Bacterial Infection and Sepsis

Patients should be treated immediately if they experience any signs of infection. Patients should also stay current with vaccinations, including annual flu vaccine and less frequent vaccination against pneumococcus and meningococcus.

Enlarged Spleen

If sickle cells become trapped in the spleen, the organ becomes enlarged and painful. Blood pooling in the spleen causes damage, and hemoglobin levels drop in the body. This can be life-threatening and should be treated immediately.

Jaundice

A yellow cast to the skin and eyes indicates jaundice. Sickle cells die more quickly than normal red blood cells and can overwhelm the liver’s ability to filter them from the blood, leading to a buildup of bilirubin (a byproduct of the breakdown of red blood cells) in the blood. The excess bilirubin results in jaundice.