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-and-sickled-red-blood-cell

Normal Sickled
Red Blood Cell Red Blood Cell

What are the four main subtypes of sickle cell disease?

People inherit 2 Hemoglobin “S” genes from their parents. This is generally the most severe form of SCD.

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 is the treatment for sickle cell disease?

Medications can help manage sickle cell symptoms. New treatments are available that can prevent future sickle cell crises. Please see the links below for more information.

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

Most people with SCD will see a hematologist for regular checkups

Bloodwork/lab work will be completed, as directed by a healthcare provider

Children will need transcranial doppler screening as directed from ages 2-16 to check the blood flow in and around their brain

Regular vision exams by an ophthalmologist

Oral medications (pills or liquids) to help the blood flow freely

Some people need intravenous (IV) fluids or blood transfusions

Some people need to see other specialty providers for complications

Some people are candidates for stem cell transplants or new genetic therapies that can eliminate sickle cell crises and improve the chronic pain they may experience from SCD

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.