What Is Sickle Cell Anemia?

Sickle cell anemia (Hbss) is a condition of the red blood cells. It is part of a group of disorders called sickle cell disease. Sickle cell anemia is the most common form of sickle cell disease. Many times, it is also the most serious. Parents pass down the condition to their children through their genes.^1,2

Red blood cells are normally round and flexible. With sickle cell anemia, they are stiff and shaped like crescent moons or sickles. These cells get caught inside tiny blood vessels. They can slow or block the body’s oxygen and blood flow. The illness causes crippling pain and other symptoms.^1,2

Sickle cell anemia symptoms

Symptoms of sickle cell anemia differ for each person and may come and go. They include:^1,3

• Pain crises – Misshapen red blood cells block blood flow to vessels and cause bouts of severe pain. Pain happens in the chest, belly, arms, legs, joints, and other parts of the body.
• Anemia – Red blood cells typically live for 120 days. Sickle cell anemia cuts that time down to 10 to 20 days. A shortage of red blood cells (anemia) leads to a lack of oxygen in the body and extreme tiredness.
• Swelling – Sickle cells can block blood flow to the hands and feet, causing swelling.
• Infection – People with this condition are more exposed to infection when sickle cells damage the spleen. The spleen helps protect you from organisms that cause disease.
• Slowed growth – Healthy red blood cells are essential for proper growth. A lack of them may delay growth and puberty in children.
• Vision issues –Sickle cells stick inside the vessels leading to your eyes. They can damage the part of the eye that sends images to your brain (retina).

Other health problems

With sickle cell anemia, you are at a greater risk of other health problems, such as:¹

• Stroke – A stroke happens when sickle cells block blood flow to part of the brain.
• Acute chest syndrome – Sickle cells can also plug vessels to the lungs, causing this health issue.
• High blood pressure in the lungs – This issue, called pulmonary hypertension, leads to trouble breathing and tiredness.
• Organ damage – A low supply of oxygen in the blood can damage the kidneys, liver, and spleen.
• Vision loss – A lack of blood supply to vessels in the eyes can cause blindness.
• Leg ulcers – Sickle cell anemia can lead to open leg sores.
• Gallstones – A spike in a substance called bilirubin can cause gallstones. Your body makes bilirubin as your red blood cells die.

Sickle cell anemia treatments

Sickle cell anemia is a lifelong condition for most people. However, there are treatments to ease pain and other medical issues that come along with it.^1,2

Medicines

Most medicines for sickle cell anemia help with pain and anemia and stop infection:^1,2

• Hydroxyurea (Droxia^®, Hydrea^®, Siklos^®)
• L-glutamine oral powder (Endari™)
• Crizanlizumab (Adakveo^®)
• Prescription narcotics for pain relief
• Penicillin
• Vaccinations (pneumonia, meningitis, flu, and others)

Blood transfusions

A blood transfusion aims to boost healthy blood cells, which lowers sickle cell anemia symptoms. Doctors remove red blood cells from donor blood. Then they inject them into the vein of someone with sickle cell anemia.^1,2

Stem cell transplant

Right now, a stem cell transplant is the only cure for sickle cell anemia. It is normally reserved for kids with serious symptoms. Doctors replace unhealthy bone marrow with bone marrow from a healthy matched donor like a sibling.^1,2

Lifestyle changes

There are some things you can do at home to help avoid pain crises or other sickle cell-related issues:¹

• Drink lots of water (about 8 glasses a day)
• Do not smoke
• Avoid extreme heat or cold
• Get regular exercise
• Take over-the-counter (OTC) pain medicine
• Take folic acid

Talk to your doctor before starting an exercise program or taking any supplements or OTC medicines.