World Sickle Cell Day

Sickle cell disease (SCD) is the most common inherited blood disorder among Americans, affecting approximately 100,000 people in the country. Although mortality rates have vastly improved since a vaccine was introduced in 2000, the costs of managing the disease remain high. We've got some facts about sickle cell disease below!

What is Sickle Cell Disease?

SCD represents a group of disorders that affect hemoglobin, the molecule in red blood cells that carries oxygen through the body. Typically these cells are round and flexible, allowing them to move freely through the body; with sickle-cell disease, they stiffen and become shaped like a crescent or sickle. This causes the breakdown of red blood cells, leading to anemia, a reduction in oxygen flow through the body that leads to shortness of breath, fatigue, and delayed growth and development in children.

Sickle cell disease can also cause the following conditions:

• Sickle cell crisis: when the red blood cells get stuck in small blood vessels, causing intense pain and sometimes organ damage.
• Jaundice: yellowing of the eyes and skin.
• Pulmonary hypertension: high blood pressure in the blood vessels that supply the lungs. This occurs in 1 out of 3 adults with SCD, and can lead to heart failure.

Other complications can include stroke, acute chest syndrome, priapism, blindness, and bone damage.

Frequency and Inheritance

Sickle cell disease is much more common in people whose ancestors come from Africa, Mediterranean nations, the Arabian Peninsula, India, the Caribbean, or Spanish-speaking South American countries.. The sickle cell trait is inherited from one parent, but sickle cell disease is inherited from both:

• SCD occurs in about 1 out of 365 Black or African-American births.
• About 1 in 13 Black or African-American babies is born with the sickle cell trait (SCT).
• SCD occurs in about 1 out of every 16,300 Hispanic-American births.
• SCD is more common among people from areas of the world with high malaria rates; people with the SCT may be less likely to have more severe forms of malaria.

The Costs of Sickle Cell Disease

Since a vaccine against invasive pneumococcal disease was introduced in 2000, sickle cell-related death among Black or African-American children younger than 4 years of age fell by 42%. This is promising, but the medical costs associated with the disease are high, and access to health care for it is not always guaranteed:

• 31 percent of infants diagnosed with SCD do not receive long-term followup care.
• 75 percent of adults with SCD who suffer from frequent pain crises do not receive hydroxyurea, the only SCD drug approved by the U.S. FDA.
• SCD patients who reach 45 years old have elevated lifetime medical costs of about $1 million.
• Adult SCD patients have $30,000 in annual medical costs; care for children and infants costs $10,000 yearly.

To learn more about sickle cell disease and the stories of those who have it, the CDC has resources available for World Sickle Cell Day!

For ideas on how to celebrate World Sickle Cell Day, check out this PDF guide from Shine a Light on Sickle Cell!