Understanding Sickle Cell Disease and Living Well

Our family history and DNA play a large role in our health and well-being. Genetic changes and traits carried by parents often appear in their children —even when the parents don’t have any symptoms of a genetic condition. Some conditions have a higher genetic link than others. Heart disease, high cholesterol and Type 2 diabetes are a few.

Some diseases affect the blood. One group of blood disorders is sickle cell disease (SCD). It harms red blood cells by changing the shape. Healthy red blood cells are a flexible disc shape. Their shape lets them to move easily through the body. With SDC, red blood cells become stiff, sickle-shape cells that block blood flow, cause pain and damage organs.

The National Heart, Lung, and Blood Institute reveals people with SCD also have abnormal hemoglobin (hemoglobin S) — a protein that helps our bodies use oxygen.

When someone has SCD, it means they’ve inherited two abnormal hemoglobin genes (one from each parent). Despite the widespread misconception that SCD is only found in the Black community, SCD can affect anyone of any race or ethnicity.    

Sickle Cell Symptoms

Fortunately, modern medicine can now identify SCD before it starts to affect a child’s daily life. When a child is born with SCD, parents will know long before symptoms start to show (at around five to six months of age). In the U.S., all newborns get screening for SCD. Some children experience problems early on while others don’t have problems until later in life.

Early symptoms include:

• Painful swelling in the hands and feet
• Fatigue or fussiness from low blood iron
• Yellowing skin or whites of the eyes

As every person is different, symptoms may be different for each person and change over time. Most of the visible signs of SCD are linked to complications.

Sickle Cell Complications

Pain can happen in people with SCD when oxygen isn’t delivered throughout the body. They may feel crisis pain — pain that lasts several hours or even several days. This happens when sickled cells block small blood vessels that carry blood to the bones. The pain is described as sharp, intense, stabbing or throbbing. People who have a pain crisis can in the hospital for several days. The pain can affect any part of the body and more than one part at a time. Pain often occurs in the:

• Lower back
• Legs
• Arms
• Abdomen
• Chest

Along with crisis pain, people with SCD can suffer from other complications:

• Chronic pain: The cause of chronic pain is not well understood and is often different from crisis pain. Patients often say chronic pain is hard to describe.
• Severe anemia: People with SCD often have mild to moderate anemia, however severe, life-threatening anemia can also develop.
• Infections: Sickle cell can damage the spleen, the organ that protects the body against certain types of germs. People with a damaged spleen are at risk for serious, life-threatening bacterial infections. These infections can morph into blood, lung, brain, spinal cord or bone infections.
• Acute chest syndrome: SCD can deprive the lungs of oxygen. When this happens, it can damage lung tissue and cause chest pain and difficulty breathing.
• Jaundice or icterus: The skin and whites of the eyes take on a yellowish color when a large number of red blood cells rupture.

Living with Sickle Cell Disease

Education and understanding are always important first steps when living with any kind of long-term health problem. If you or your child has SCD, learn as much as possible. Build a relationship with your doctor so you feel comfortable asking questions. Developing a plan to manage your disease and complications can also help.

Babies and children with SCD should be seen by a doctor with experience caring for patients with the disease. Seeing a hematologist (blood disease specialist) may be a care option. People living with SCD should see their care provider regularly — every three to 12 months for preventive measures.

Pursue a Healthy Lifestyle

As with many other conditions, maintaining a healthy lifestyle can help limit complications. Strengthen your body with:

• A nourishing diet
• Plenty of sleep
• Regular physical activity

Limit activities that can harm your health. Stop smoking. Drink alcohol in moderation or not at all. Steer clear of situations that can trigger a pain crisis. Take time to rest. Drink plenty of water to stay hydrated and avoid extreme heat or cold.

For more information about SCD and resources, visit the Centers for Disease Control and Prevention  website or the American Society of Hematology.

Sources Sickle Cell Disease,  Centers for Disease Control and Prevention, 2023; Sickle Cell Disease,  National Heart, Lung and Blood Institute, 2022; Sickle Cell Disease,  American Society of Hematology

Originally published 2/3/2017; Revised 2021, 2023