Sickle Cell Disease

Learn more about the condition

Sickle cell disease (SCD) is an inherited blood disorder. With SCD, the hemoglobin in red blood cells is abnormal. This damages the red blood cells. SCD prevents the hemoglobin in red blood cells from carrying oxygen to all parts of the body.

Continued high morbidity and mortality

No “universal” cure

Few disease-modifying therapies

Poor access to high quality care

Need to improve existing treatment regimens

Pain

Strokes and “silent”

infarcts

Increased risk for infection

Pneumonia-like illness

ACS

Splenic sequestration

Worsening anemia

Eye problems

GI problems

Hip and shoulder disease

Pulmonary hypertension

The sickle mutation is common in malaria-prone parts of the world

The mutant sickle gene produces hemoglobin molecules that are “sickled” and rigid

The parasite that causes malaria, Plasmodium, cannot digest the sickled hemoglobin

and causes the sickled red blood cells to rupture prematurely, before the parasite can

reproduce

In areas where malaria is endemic, people with sickle cell trait, or one copy of the

sickle mutation, have a survival advantage over people without it

The majority of affected individuals are from low-income countries

90% of children with SCD in resource-poor countries do not survive to adulthood

By 2050, the number of people with SCD is expected to increase by 30%

— Dr Marj Dejoie-Brewer