Sickle Cell Disease

Sickle cell disease (SCD) is a blood disorder involving oxygen-carrying hemoglobin in red blood cells.

Red blood cells are usually round and able to bounce off each other as blood travels through the body.

With SCD, red blood cells become sickle- shaped and get stuck together. This blocks blood flow to parts of the body.

What causes sickle cell disease?

Sickle cell disease is inherited. A baby must receive the sickle cell gene from both parents to be diagnosed with SCD.

If a baby only gets the gene from one parent, the baby is not likely to have any sickle cell symptoms. However, the baby will be a carrier of the sickle cell trait.

Carriers can pass the disease on to future children if their partner also carries the trait or has SCD.

SCD is most common among people of African, Mediterranean, and Middle Eastern descent. In the United States, the most common forms of SCD are found by universal newborn screening (NBS) programs.

Normal Sickled
Red Blood Cell Red Blood Cell

What are the four main subtypes of sickle cell disease?

Hemoglobin SS

Hemoglobin SC

Hemoglobin S Beta Thalassemia +

Hemoglobin S Beta Thalassemia o (or null)

How does sickle cell disease affect a person?

Health problems from SCD can include pain (sickle cell crisis), anemia, swelling in the hands and feet, bacterial infections, and stroke. Long-term pain can develop as people age. A sickle cell crisis can occur with viral illnesses, temperature changes, stress, dehydration, and high altitude.

What testing is available for sickle cell disease?

Blood tests and lab work are done regularly to help doctors watch for problems and guide treatment.
Children usually have a special ultrasound test of the brain (called a transcranial Doppler, or TCD) as directed from ages 2 to 16 to check blood flow in and around the brain.
Eye exams are performed to watch for vision or eye problems caused by sickle cell disease.

What treatments are available for people living with sickle cell disease?

Most people with sickle cell disease (SCD) will see a blood doctor (hematologist) for regular checkups and care planning.
Treatment may include medications taken by mouth (pills or liquids) to help prevent pain episodes and other problems.
Some people need IV fluids, pain medication, or blood transfusions, especially during pain crises or serious illness.
Many people with sickle cell disease also see other doctors to help care for problems affecting other parts of the body.
Preventive care is important and includes vaccines, steps to prevent infections, and regular health checkups.
Some people may be able to receive treatments that can cure SCD, such as stem cell transplants or new gene therapies, which are done at special treatment centers for people who qualify.

What are ways to lower the risk of a sickle cell crisis?

Stay well hydrated.
Wash hands often to prevent the spread of germs.
Get enough rest.
Avoid extreme temperatures.
Eat a healthy diet.
Go to all follow-up appointments and tests that are ordered by a healthcare provider.

How Fidelis Care Can Help

Fidelis Care offers Case Management support to members with sickle cell disease. A Case Manager is a registered nurse or medical social worker who works closely with the member, family, healthcare provider, and members of the care team to help individuals reach their health goals.

Please call 1-800-247-1441, ext. 16875 to learn more about how Fidelis Care’s Case Managers can help you meet your health goals.