Five things to know about sickle cell disease

1. It is the most common inherited blood disorder.

Sickle cell disease (SCD), most often diagnosed through a newborn screening test, is a genetic condition affecting millions of people that is passed down when both parents carry and pass on the sickle cell gene. Instead of making round, flexible red blood cells, people with sickle cell produce red blood cells that are fragile, sticky, and shaped like a sickle (or a half-moon). These cells can block blood flow, leading to severe complications like stroke, kidney damage and infections.

2. It affects the whole body, not just the blood.

Because sickled red blood cells block blood vessels and break down too quickly, SCD can cause complications anywhere the blood touches, from your head down to your toes. These include complications like stroke, lung disease, kidney disease, vision loss and delayed growth. It affects the whole body.

3. Treatments exist, but gaps remain.

Hydroxyurea and chronic transfusions can significantly reduce complications, but those are the only treatment options available that alter the disease, and they are not always easily available to patients. Curative options like stem cell transplant or transformative options like gene therapy are available for some, but access, cost and eligibility remain major barriers. We need more treatment options, and we need better access to these options.

4. Health disparities and awareness matter.

SCD mainly affects people of African descent, as well as those from Hispanic, Middle Eastern, Mediterranean and South Asian backgrounds. Despite being the most common inherited blood disorder in the U.S., it is often underfunded and under-resourced.

5. Comprehensive specialty care is necessary for best outcomes.

Early diagnosis (through newborn screening), preventive care and consistent access to specialized care are crucial to improving outcomes. However, less than 70% of patients receive SCD care, and even fewer receive care from an SCD specialist due to limited experts and disparities in access. For the greatest impact, patients with sickle cell disease should be treated by a team with expertise in the disease, connected to a comprehensive SCD center like Riley and IU Health.

Learn more about Riley Hospital’s Sickle Cell Program here.

Photos by Mike Dickbernd, IU Health visual journalist, mdickbernd@iuhealth.org