In the United States, sickle cell disease is considered rare because it affects only approximately 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. Throughout my career, I’ve been asked a number of questions about SCD, many of which are common myths.
In this article, I will share some of the more common myths, in an effort to separate the facts from fiction.
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.
Sickle cell disease is contagious
Myth! You cannot “catch” SCD. It is a genetically inherited condition (passed down from parent to child). For a child to inherit SCD, both parents must carry the sickle cell trait (or have the disease), and each must pass the sickle cell gene to the child.
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 the person will have the disease. What’s the difference? When a person has only one copy of the sickle cell gene that person 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. In developed countries, such as the U.S., babies are now routinely screened at birth to determine if they carry the trait or have the disease.
People with sickle cell disease abuse pain medication
Myth! Studies have shown that there is no increased substance abuse in patients with SCD. The most common symptom of SCD is excruciating, debilitating pain that often does not respond to over-the-counter medications and needs opioids to provide relief. Over time, the body becomes used to the opioids—ultimately leading to high doses being required to manage SCD pain. This need for high doses of opioids (which may not even provide complete relief from the pain) has unfortunately led to the perception that these patients are “drug-seeking.”
A baby born with SCD will die before reaching adulthood
Myth! Until the 1990s, SCD was considered a life-threatening condition because many children born with the disease did not live to adulthood. In developed countries, such as the U.S., this outcome is no longer true. Most of the children live to adulthood, thanks to advances in SCD care.
However, the life expectancy of someone with SCD in the U.S. is only between 40 and 60 years, compared with the average U.S. life expectancy of 78.74 years. There is still much more work to do to improve the outcomes of people with SCD in the U.S. 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. Although 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.
One way to help address this need 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.
Addressing barriers in clinical trial participation has been a big focus of Pfizer Rare Disease, and working with organizations such as the National Newspaper Publishers Association has been critical in helping spur engagement and action. As part of our collaboration with NNPA, we have sponsored a national poll among the African-American community to better understand their perceptions about SCD and participation in clinical trials, and will look to these findings to guide future efforts in this area.
Hopefully, this article has helped to clear up some of the more common misperceptions about SCD. By increasing awareness and providing education about this disease, we aim to improve the care of those affected. Stay tuned for my next article, which will discuss clinical trials in more depth and their role in helping to develop potential new SCD treatments.
Dr. Kevin Williams, the chief medical officer of Pfizer’s Rare Disease Unit, debunks common myths about sickle cell disease in the Black community.