Sickle cell anemia is a serious condition in which the red blood cells can become sickle-shaped (that is, shaped like a “C”).

Normal red blood cells are smooth and round like a doughnut without a hole. They move easily through blood vessels to carry oxygen to all parts of the body. Sickle-shaped cells don’t move easily through blood. They’re stiff and sticky and tend to form clumps and get stuck in blood vessels.

The clumps of sickle cells block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessels can cause pain, serious infections, and organ damage.

Symptoms

The signs and symptoms of sickle cell anemia are different in each person. The most common signs and symptoms are linked to anemia and pain. Other signs and symptoms are linked to some of the complications of the condition.

The general signs and symptoms of anemia are fatigue (tiredness), pale skin and nail beds, jaundice (yellowing of the skin and eyes), and shortness of breath.

Sudden episodes of pain throughout the body are a common symptom of sickle cell anemia and are often referred to as “sickle cell crises.” A sickle cell crisis occurs when the red blood cells sickle (become “C” shaped) and stick together in clumps. The clumps block the flow of blood through the small blood vessels (capillaries) in the limbs and organs.

Almost all people with sickle cell anemia have painful crises at some point in their lives. Some have a crisis less than once a year. Others may have 15 or more crises in a year.

Complications of sickle cell anemia come from the effects of sickle cell crises on different parts of the body.

• Hand-Foot Syndrome
• Splenic (Sequestration) crisis
• Infections
• Acute Chest Syndrome
• Delayed growth and puberty in children
• Stroke
• Eye problems
• Priapism
• Gallstones
• Ulcers on the legs
• Pulmonary Arterial Hypertension (high blood pressure)
• Multiple organ failure

Treatment

The goals of treating sickle cell anemia are to relieve pain; prevent infections, eye damage, and strokes; and control complications if they occur. The treatments include medicine, blood transfusions, and specific treatment for complications.

Mild painful crises can be managed with treatments such as over-the-counter medicine and heating pads. However, severe pain may need to be treated in a hospital. Painful crises are the leading cause of emergency room visits and hospitalizations of people with sickle cell anemia. The pain-killing medicines most often used are acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), and narcotics such as meperidine, morphine, oxycodone, and others.

A medicine called hydroxyurea may be given to adults and older adolescents with severe sickle cell anemia to reduce their number of painful crises. This medicine is used only to prevent these crises, not to treat them when they occur. Given daily, hydroxyurea can reduce the frequency of painful crises and of acute chest syndrome. People taking the medicine also may need fewer blood transfusions.

To prevent infections, treatments include:

• Daily doses of penicillin. Treatment may begin as early as 2 months of age and continue until the child is at least 5 years old.
• Vaccinations for pneumonia, meningitis, influenza, and hepatitis.
• A yearly flu shot.

Stroke prevention and treatment is now possible for children and adults who have sickle cell anemia. Starting at age 2, children with sickle cell anemia often receive regular ultrasound scans of the head (this is called transcranial Doppler ultrasound). These scans are used to monitor blood flow in the brain. The scans allow doctors to find out which children are at high risk for a stroke and treat them with regular blood transfusions.

Blood transfusions are used to treat worsening anemia and sickle cell complications. A sudden worsening of anemia due to an infection or enlargement of the spleen is a common reason for a blood transfusion.

Some other treatments for common complications include:

• Hospitalization, oxygen, blood transfusions, antibiotics and pain medicine for acute chest syndrome.
• Pain medicines, medicated creams, and sometimes skin grafts for leg ulcers.
• Gallbladder surgery to remove gallstones.
• Fluids or surgery for priapism.