Sickle cell disease (SCD) is a group of painful and life-threatening genetic disorders that affect hemoglobin, the major protein that carries oxygen in red blood cells. It occurs in nearly 100,000 people in the United States and millions worldwide. The most common type is hemoglobin SS (HbSS), which affects about 65% of those with sickle cell disease.
Less familiar and understudied is hemoglobin SC, or HbSC, the second most common type, which affects about 25% of those with sickle cell disease. Clinicians have long regarded HbSC as a "mild" type of the disease, but a new study published in the British Journal of Haematology challenges that notion. The research is led by Julie Kanter, M.
D., a professor at the University of Alabama at Birmingham and director of the school's Adult Sickle Cell Program. Conducted as part of the Sickle Cell Disease Implementation Consortium (SCDIC), the study included 2,282 individuals ages 15–45 years, with sickle cell disease.
About 500 (22%) had HbSC. For the study, the researchers compared clinical outcomes in people with HbSC to those with HbSS over a median period of about five years. In a recent conversation, Kanter discussed the findings of the new study and what they may mean for people living with HbSC.
Why did you undertake this research on HbSC? I wanted to try to clear up some of the misconceptions about the disease. For example, in childhood, many people with HbSC have milder manifestations of their sickle cell disease and fewer com.
