About this study
Sickle cell disease affects the hemoglobin in red blood cells. Hemoglobin is a protein that carries oxygen through the body. In people with sickle cell disease, red blood cells become hard, sticky, and shaped like a sickle or banana instead of round and soft. These sickle-shaped cells can block blood flow and lead to pain, organ damage, or stroke. These cells also make it easier for the blood to clot, which can be dangerous.
People with sickle cell disease usually have fewer complications from their sickle cell disease if they have high levels of a special kind of hemoglobin called hemoglobin F (HbF). But some people still have health issues, even when their HbF levels are high. We want to find out why that happens.
To learn more, we are asking some patients who already part of the SCCRIP clinical trial to give about 1 teaspoon of extra blood during a clinic visit. We will use this blood sample to find out how HbF is spread inside red blood cells. We will also check for special substances in the blood (markers) that affect how blood clots, since people with sickle cell disease often have problems with blood clots.
Part of the study will focus on young red blood cells and cells that are becoming red blood cells. Scientists will study the genes (DNA) of these cells to study changes in people who still get sick even though they have high HbF levels. We also want to see if the spread of HbF in their cells affects how well HbF prevents the sickling that leads to blood clots.
Another part of the study will look at the participant’s blood clotting proteins. Sickle cell disease patients can have increased blood clotting proteins, which can make the blood clot too easily. We want to see if people with more HbF who get sick often have higher blood clotting proteins. This could help explain why some people with sickle cell disease stay healthier than others.
This study is important because it could help doctors understand why some treatments work better for some people than others. If researchers can learn how HbF reduces the complications of sickle cell disease, or how to reduce clotting risks, they might be able to improve care for many people with sickle cell disease.
Eligibility overview
- Sickle cell disease
- Older than 5 years