Charm City won’t wait until September to put a spotlight on sickle cell disease.
On Aug. 26 hundreds are expected to join the Sickle Cell Disease Association of America (SCDAA) in Baltimore to bring attention to the disease, educate the public, and raise money for research.
“This is an awareness event,” said Sonja L. Banks, president and chief operating officer of the SCDAA. “We want people to understand that sickle cell still exist and we have to raise national awareness.”
Banks said that “over 80 percent of all the dollars raised goes back into the community based organizations that really serve patients. We’re raising money so they can provide those services and bolster research.”
Participants can register for the 4th Annual Walk with the Stars 5K beforehand on the SCDAA website, or register on-site from 8-9:30 a.m. at Canton Waterfront Park before the 10 a.m. kickoff.
The walk is one of many sickle cell awareness events taking place from June to Dec. 31, 2017 as the SCDAA’s “One Community- One Cause” campaign sweeps across the country. The disease affects approximately 100,000 Americans- almost all of whom are Black.
Banks said African-American churches, schools, and community organizations need to “make sickle cell disease part of our agenda. We have diabetes, heart disease, AIDs, and cancer as part of our agenda. We need to step it up and add sickle cell disease.”
According to the Centers for Disease Control, the term “sickle cell disease” (SCD) covers a group of “inherited red blood cell disorders.” SCD occurs when red blood cells take on a “sickle” or “C-shaped form” instead of a normal circle shape.
Red blood cells deliver oxygen throughout the body via tiny blood vessels, but this job gets complicated when the sickle cells become “hard and sticky,” die prematurely, and clog blood vessel entrances. “This can cause pain and other serious problems such as infection, acute chest syndrome and stroke.”
One out of every 13 African Americans born has the sickle cell trait (SCT) but no SCD symptoms. However, when two parents have the sickle cell trait there is a 25 percent chance that their child will be born with SCD, and a 50 percent chance that someone will pass along the trait. One out of every 365 Black births lead to an SCD diagnosis.
Dr. Sophie Miriam Lanzkron, director of the Sickle Cell Center for Adults at The Johns Hopkins Hospital in Baltimore told the AFRO, “The most commonly used therapy is hydroxyurea. It doesn’t bring crisis frequencies to zero, but it cuts it in half for people with the most common form of sickle cell disease.”
Until last month, hydroxyurea was the only drug approved to treat the disease. Lanzkron said the latest therapy, Endari, is shown to decrease painful episodes by 25 percent. It is not available to the public yet, but could possibly be used along with hydroxyurea in the future.
Lanzkron also said that 98 percent of her patients are African American. Many of them receive chronic transfusion therapy, a monthly blood transfusion that replenishes blood cells and decreases the occurrence of painful crisis. This type of therapy highlights the importance of “having blood from the community of the person who needs it.”
Other treatments include bone marrow transplants and gene therapy, but both are typically out of reach for patients for a number of reasons.
“We used to do bone marrow transplants only with donors who were an exact match but we do half- matches now. A parent or a child can be a donor,” said Lanzkron. Still, between the inability to complete preparative regimens, rejection of transplants, the three-month recovery period, and money, bone marrow transplants are rarely an option- especially for adults. “In the last decade we’ve probably had about 50 transplants at Johns Hopkins. That number doesn’t include children.”
Because pain is the most common symptom of SCD, the disease has presented a unique problem to lawmakers trying to regulate opioid abuse. Lanzkron said pain from SCD can present as early as four to six months, and eventually becomes an everyday occurrence for as many as 60 percent of adults.
“These episodes of excruciating pain have been described as worse than child labor. All we can do is give opioids. The new restrictions on the amount and use of opioids thankfully said sickle cell is an exclusion to these new rules.”
Lanzkron said, “In this day in age everyone should know their trait status,” something that Michael L. Matthews, Executive Director of the Children’s Sickle Cell Foundation, urges as well.
“Find out if you are a carrier or not- before you decide to have a family,” said Matthews, whose own son was diagnosed with SCD. “You don’t want the first time you hear the term “sickle cell” to be when the doctor is telling you that your beautiful newborn baby has the disease.”
Banks said that information about sickle cell trait status is held by the public health department and some states are not required to tell you if you have the trait- only if you have the disease.
The SCDAA will hold their 45th Annual Convention from Oct. 25- 28 in Atlanta. They will also be raising awareness through social media during Sickle Cell Awareness Month in September with several Twitter campaigns focused on advocacy, awareness, access to treatment, and finding a universal cure.