June 19th is World Sickle Cell Awareness Day and commemorates the date in 2008 when the United Nations began recognizing this group of genetic blood disorders as a public health concern.
Sickle cell disease affects millions around the globe and nearly 100,000 people in the United States. It’s relatively common in Latin America, the Caribbean, Saudi Arabia, India, Mediterranean countries, and especially prevalent in those with ancestry tracing to sub-Saharan Africa. In the US roughly one in 500 black or African-American babies is born with the disease.
Geno Atkins and Sickle Cell Trait
In his two seasons in the NFL playing with the Cincinnati Bengals, defensive tackle Gene Renard “Geno” Atkins Jr. has showed few weaknesses, notching up 48 tackles and eight sacks.
But throughout his time on the field — not just professionally, but when he was a standout player in college and high school— Geno has played with what is called the “sickle cell trait.”
While it isn’t full-blown sickle cell anemia, the sickle cell trait can pose health risks for those who don’t know they have it. For instance people with the trait may be more vulnerable to heat stroke and muscle breakdown as a result of strenuous exercise. Geno hopes to bring attention to the issue and recently wrote about his own experience in a piece on the CDC website. Because testing isn’t currently in place for high school athletes, many play without knowing they are at risk.
“I was blessed to not suffer any side effects or have any health related issues due to the sickle cell trait,” Geno wrote in his blog two years ago. “I would urge parents, friends, and extended families of all Athletes participating in any Sports or working out at a high level to have their Athlete tested.”
After he learned of his risk in college, both Geno and his coaches paid particular attention to whether he was healthy during practice and games.
Prevalence of sickle cell disease in African populations is high due to the fact that many people with African ancestry carry a genetic mutation in the HBB gene that causes the condition. You need two copies of this mutation (one from each parent) to have the disease, but if you have just one, you have what’s known as “sickle cell trait”. This trait protects you from malaria which is why the mutation has become relatively common in sub-Saharan Africa, where malaria is endemic. Unfortunately, it also increases the risk that your child may be born with sickle cell disease, if your partner also has one copy of the mutation.
Roughly one in 12 African Americans carries a copy of the HBB mutation and the World Health Organization estimates that sickle cell disease contributes to 5% of deaths in children under 5 years of age in some African countries. World Sickle Cell Awareness Day aims to highlight and combat this public health burden.
Although improved access to care is needed to counteract some of the public health burden, appropriate genetic screening could lower the rates of disease and/or equip families with knowledge about their chances of passing the disease to future generations.
To raise awareness about sickle cell disease, the Centers for Disease Control (CDC) is piloting a data collection system called the Registry and Surveillance System for Hemoglobinopathies (or RuSH) to find out exactly how many US individuals suffer from sickle cell disease and related red blood cell disorders like beta thalassemia. They are also addressing unmet needs in research, surveillance and education.
So we ask you today — do you suffer from sickle cell disease or know anyone living with it? What about sickle cell trait — have you been screened for a mutation that you could pass on to your child? We welcome your comments on this important health topic and encourage you to also raise awareness about this disease.