This is such a concern for Black athletes in the NCAA, that anyone who has the sickle trait must go through an extra level of evaluation before they can play Division I sports. “This is something that people just don’t know about.”
– Dr. Corey Hébert
Sickle Cell disease is causing major morbidity and mortality in the African-American community. This rare and inherited blood disorder causes debilitating pain, disability, and decreases life expectancy by 25 to 30 years.
Although only 100,000 Americans are afflicted with Sickle Cell Anemia (SCA), it is estimated as many as four million Americans carry the trait including between eight and ten percent of African-Americans.
Even though it primarily impacts Blacks—Hispanics, South Asians, Caucasians (from southern Europe), and people from Middle Eastern countries can also carry the trait and suffer from the illness.
The increasing impact of SCA in the Black community in recent years has become a growing area of concern for Dr. Corey Hébert who hosts a new four-part video series titled “What You Need to Know About Sickle Cell Anemia.”
Only five percent of active physicians in America are African-American, thus it was a privilege for Black Voice News to speak one-on-one recently with Dr. Hébert about the rising number of Black babies being born with SCA; new information about the vulnerabilities of those who carry the sickle cell trait; a breakthrough in medication; and the need to raise awareness in the Black community regarding all of this.
Hébert is a physician, journalist, educator, Chief Medical Officer at Dillard University and professor at Louisiana State University and Tulane University Medical Centers in Louisiana. He is also well recognized, highly regarded and respected as a medical reporter and health expert who frequently appears as a consultant on national television.
Hébert explained he started getting really worked up regarding Sickle Cell Anemia about five years ago.
“I started seeing a lot more young people having babies that had sickle cell disease. And, when I would explain to them about the trait, they would look at me like I had three heads. Like, ‘what are you talking about? I thought this was cured. I never knew I had sickle cell trait.’”
As an African American physician, Hébert shared how these types of comments weighed on him. He was really concerned because he knew the test for sickle cell is done at birth on every person across the country.
“So, whether you have sickle cell trait or not, it is known at birth,” he stressed.
For those with the trait who do not know they have it, he admitted, upset him because not being aware one is a carrier of the trait can inadvertently result in a child being born with SCA if either, or both parents are unaware of their trait status.
To prevent this from happening people must be made aware they have the trait.
“It’s not even that we would have to do anything or get people tested, they’re already tested. They just don’t know,” he declared incredulous. “That’s one of the things we just kind of can’t have in this country.”
Hébert further noted how America has the ‘ultimate systems of medical communications,’ and as such, it is difficult to accept babies are still being born with a disease that is going to inflict them and cause them pain for the rest of their lives.
With nearly four million people carrying the trait he offered, it is hard to believe so many are unaware they carry it.
“They just don’t know,” he opined, noting how young people, even older people, having babies born with SCA are often overwhelmed. “If they knew and decided to have a baby anyway, then that was the risk,” he stated. “That’s one thing, but we know there’s a huge percentage where that’s not the case.”
Hébert said he started addressing this concern when he came to realize “a lot of the patients that actually have the disease [SCA], are just not being taken care of.”
Adults who suffer with Sickle Cell Anemia–it is a very painful condition—are often labeled as drug seekers and nobody wants to treat them because of the opioid crisis and other considerations according to Hébert.
Although it is considered a rare disease because only about 100,000 people are diagnosed “it’s not rare to the people that have it,” he stressed.
“And [the number of people impacted by the illness] can go up exponentially,” he continued, “if people don’t understand and know what their status is,” he offered regarding the high number of people who may not realize they carry the trait. “It’s just unacceptable,” he declared.
In response, Hébert is now focused on addressing the issue on two fronts. Those fronts include prevention first, and then making sure patients diagnosed with SCA have access to the medical information, medical procedures, and appropriate medication when prevention has failed.
Emphasizing how most people who have this disease are African American, he pointed to the lack of fiscal support impeding efforts in this regard.
Comparing SCA to another illness he explained, “It’s a kind of a similar type of inheritance pattern as you have with cystic fibrosis,” he offered, adding however there is a big difference when it comes to funding between the two inherited diseases. Regarding cystic fibrosis, “They get 40 times more funding dollars,” than SCA, highlighting the differences in equity. Cystic fibrosis is a genetic disorder impacting the lungs that primarily affects White people.
Decades ago, there was a big push for awareness regarding sickle cell anemia in the Black community and the numbers started to decline but, according to Hébert, “Once we got universal testing, I think people started thinking, ‘Well, since we have the universal test at birth, things would be fine because people will know [their status] and people will have the information that they need.’”
Although it was hoped at the time this would prevent children from being born with SCA, “it just didn’t happen,” he confirmed. “The numbers didn’t go crazy high, but they didn’t go away like they thought.”
Regarding treatment for SCA patients Hébert reports they are primarily treated by hematologists/ oncologists who primarily take care of cancer patients where there is a lot of money involved.
“And, then there’s a lot of ways to make that [cancer] patient better,” Hébert shared. “But when the sickle cell patient comes in, they [the hematologist/oncologist] has got nothing for you. The doctor can’t be like the savior and the hero.”
As a result, he continued, “They kind of started to disenfranchise these patients. Even when there’s new innovative medicines that come out many doctors do not even want to know about such innovations because they don’t really want to serve SCA patients—there’s no money in it for them as opposed to oncology.”
Not being shy about sharing his frustration, the doctor acknowledged, “[W]ere SCA a disease that afflicted mostly Caucasians, it would probably be more a part of the daily lexicon.”
Hébert also concurred there should be more intervention in terms of educating parents when a child is born with the SCA trait. “Pediatricians need to stress that more.”
Doctors like Hébert continue to learn more and more about those who carry the SCA trait, “We thought, I mean even myself up until maybe five or six years ago, we always thought the trait didn’t cause problems, but we now know about 14 young African American males [with the trait] have dropped dead in division one sports.”
Those with SCA frequently experience a condition called hypoxia, or low oxygen levels, in tissues due to the reduced oxygen-carrying capacity of hemoglobin S. Medical experts now understand this also happens in individuals who carry the trait. (Hemoglobin S is what causes red blood cells to become stiff and instead of having a normal round, disk shape, they become sickle- or crescent-shaped).
Hébert explained, when someone with the sickle cell trait—not SCA—develops hypoxia, and stress is added to the situation, it can precipitate an actual sickle cell crisis in a patient, even though they only carry the trait.
This is such a concern for athletes that anyone now playing in the NCAA who has the trait must go through an extra level of evaluation before they can play Division I sports, Hébert informed.
“This is something that people just don’t know about,” he added.
People know that the trait exists and if you have two people with the trait who have a baby, chances are the child may have SCA or carry the trait. Yet, according to Hébert, those who have the trait do not understand why, when they go skiing for example, their legs hurt. “They don’t understand that,” he reiterated.
“That’s why within the last year and a half, it has been my mission to make sure that everybody knows. People with the trait need to know that they can also have the disease. That is a big deal.”
Continuing he stressed, “Sickle cell disease will exacerbate any pre-existing illness, pretty much, and the reason why is because it’s hypoxia.”
The need to educate the approximate four million people who carry the sickle cell trait about these important findings is why Hébert was so excited when an opportunity presented itself for him to be involved in the video project “What you should know about Sickle Cell Anemia.”
“I said, I have to make a video series that is going to break this down so people can understand the disease following their child’s diagnosis.”
Regarding treatment of the illness, Hébert is also a spokesperson for Medunik USA who sponsored the video series and also markets and distributes the medication, Siklos®, the only FDA-approved prescription medicine that can reduce the frequency of painful SCA crises.
There are limited treatment options for SCA patients, Hébert explained noting since most doctors have never seen a patient with the illness, when there’s new innovative medicines that come out, most are not aware of it.
There’s been only three medicines introduced to treat SCA in 40 years, “All we do is treat the pain,” he said but this medicine, Siklos®, decreases the risk of painful crises.”
Although the medication has been around for years it’s been difficult to dose.
With Siklos®, Medunik USA has made it much easier to administer. “And, you can give it to children two years of age and older. That means children don’t start to feel the psychological impact of this horrible disease at such an early age. They don’t have to be in the hospital all the time and in pain all the time.”
Speaking about the current COVID-19 crisis and any added risk for SCA patients he advised, “Sickle cell trait or sickle cell disease will exacerbate any pre-existing illness, pretty much, because it’s hypoxia.”
To date he continued regarding COVID-19 and individuals with SCA, “Their numbers are pretty much on par with the horrible numbers seen for everybody else in the Black community.” However, they are in the process of assembling because this is a respiratory disease and experts know that SCA causes a lot of acute respiratory problems and pneumonia.
“So, when we start thinking about African-Americans being afflicted more and dying more, we don’t really have the data to say how many of those people [with SCA] are dying.” Experts are still looking for a link.
“[They claim because COVID-19 is] affecting African Americans disproportionately this is going to shine a light on all these health ‘inequalities.’” Although they call it health ‘disparities,’ he continued, “I have a problem with that too. First, it is not going to shine a light because there’s a light shining on this all the time. There’s a billion dollars spent on African-Americans and health–quote, unquote—’disparities.’
In closing, Hébert was adamant in his belief in the importance of having “the right message and the right messengers” to deliver this information to the African American community. When it comes to the impact of COVID-19 on Blacks, he stressed, the message is wrong because the messenger(s) delivering it “doesn’t know what the hell they’re talking about.”
“As Black people,” Hébert emphasized, “We have a responsibility. I have a responsibility to get this information out.”
There are four million Americans, many of them Black, who, though tested at birth, do not know whether they carry the sickle trait.
“Know your status,” Hébert declared.
It is important for your own health and well-being, as well as the health and wellbeing of any children you may have in the future if you carry sickle cell trait.
Follow this link to view the four-part video series “What You Need to Know About Sickle Cell Anemia,” hosted by Dr. Corey Hébert.
Header Photo: Dr. Corey Hébert