Pfizer Inc., the National Newspaper Publishers Association, and scholars from Howard University recently announced results from a new national poll designed to deepen understanding and gauge perceptions around sickle cell disease among African Americans. The poll, which included responses from adults in the US who self-identified as African American, revealed that while the majority of respondents were familiar with SCD and understood the disease in general, only one-third (36%) were aware that it disproportionately affects people of African descent, demonstrating a critical need for education and awareness.
Dr. Dr. Kevin Williams, Chief Medical Officer at the Pfizer Rare Disease Unit isn’t surprised by these numbers.
In the United States, sickle cell disease is considered rare because it affects only about 100,000 people nationwide. As such, not many people in the U.S. are aware of what SCD is, or if they are aware, they have limited experience with the disease.
“As with any disease, low awareness can spur myths and misperceptions and make it more difficult for those affected to obtain the care and support they need,” says Dr. Williams.
Sickle cell disease is a lifelong and debilitating disorder that affects red blood cells. It is the most common inherited blood disorder in the US, and most people living with sickle cell disease are of African descent. In fact, SCD occurs in one out of every 365 African American births.
“Throughout my career, I’ve been asked a number of questions about SCD—many of which are common myths,” wrote Williams, who went on to debunk many of the common myths.
Sickle cell disease is a “Black” disease.
Myth! It’s true that in the U.S. more than 90 percent of people living with SCD are of African descent. In fact, SCD occurs in one out of every 365 African American births. However, it’s not exclusive to this patient population. SCD is also found in people of Indian, Middle Eastern, Hispanic, and Mediterranean ethnicities.
A person with the sickle cell trait will automatically develop sickle cell disease.
Myth! Sickle cell trait is different from SCD. Just because a person carries the sickle cell trait does not mean they will have the disease. What’s the difference? When a person has only one copy of the sickle cell gene, he or she will have sickle cell trait. Someone with SCD will have two copies of the sickle cell gene.
A person with sickle cell trait can, however, pass the disease on to his or her child, if the other parent also has trait. That’s why testing for sickle cell trait or disease is incredibly important. Sickle cell disease (and sickle cell trait) can be diagnosed through a simple blood test.5 In developed countries, like the U.S., babies are now routinely screened at birth to determine if they carry the trait or have the disease.
A baby born with SCD will die before reaching adulthood.
Myth! Until the 1990s, SCD was considered a life-threatening condition as many children born with the disease did not live to adulthood. In developed countries, like the US, this is no longer true, with the majority of children living to adulthood, thanks to advances in SCD care.
However, the life expectancy of someone with SCD in the US is only between 40 and 60 years, compared to average US life expectancy of 78.74 years. There is still much more work to do to improve the outcomes of people with SCD in the US and worldwide, particularly in underdeveloped countries. Although the disease was identified more than 100 years ago, there are still very few medicines available to help patients or address SCD symptoms. While there has been recent progress in this area, more still needs to be done. At Pfizer Rare Disease, we are working tirelessly to bring safe, effective treatment options to those in need.
Participants in Clinical Trials Needed
One way to help address this is to encourage participation in clinical trials. To date, there have been several challenges in securing adequate participation of African Americans in clinical trials, which has been one of the key barriers to the development of new SCD medications.