One in 2,400 are born with sickle cell disease (SCD) in the U.S. As one of the most common inherited blood disorders, it is widely misunderstood.
Last month, the Minnesota Sickle Cell Coalition — comprised of patients, doctors, healthcare providers, and advocates — co-sponsored a Sickle Cell Disease Stakeholder Forum at Sanctuary Covenant Church in North Minneapolis. Approximately 60 healthcare professionals and others attended the Dec. 10 event to not only help improve care for those afflicted by the disease but also to increase awareness of the disease, screening and healthcare disparities.
SCD is a group of inherited red blood cell disorders including Sickle Cell Anemia (Hgb SS Disease), Hemoglobin SC Disease, and Hemoglobin Sickle Beta Thalassemia. People with sickle cell disease produce abnormal hemoglobin (Hgb S), which can interrupt blood flow and prevent oxygen from getting to important parts of the body. This can cause many potential health complications, including extreme pain, risk of stroke, and pulmonary hypertension.
“Pain is what I have been born into,” Isaiah Lane, who has SCD, told the MSR.
It has historically been seen as a childhood disease because those stricken typically did not live to adulthood. Yet, according to a 2013 Harvard study, the average life expectancy for persons in the U.S. with SCD is 38 years for men and 42 years for women.
While state and national health experts report a longer life expectancy, it still remains an incurable, often unbearable disease.
“I’m 30. I live with SC disease,” said Brianna (her last name withheld by request). “I have near constant pain. When I don’t have pain, it is a memorable day.”
Those afflicted are not only burdened with shortened lives of pain, they face uncertainty in communities that are ill prepared to provide unbiased care and support.
The national Sickle Cell Disease Coalition recently released its 2018 State of Sickle Cell Disease Report Card focused on four major areas: access to health care, training and professional education, research and clinical trials, and global issues.
According to its report card, there have been initiatives around the country that have “moved the needle in the right direction,” but there still exist “too many barriers to quality care,” “implicit bias,” and not enough healthcare providers “with comprehensive knowledge and expertise to care for individuals with SCD.”
SCD is also mainly seen as a “Black disease,” said Dr. Steve Nelson of Children’s Minnesota. Nelson, a hematologist and a member of a 14-person “sickle cell team” of doctors and nurses at Children’s Minnesota in Minneapolis, where he also is chief of staff. He explained, “We talk about one in 600 African Americans [having SCD] instead of saying one in 2,400 Americans. It is actually the most common inherited disorder in the United States.
While the percentage of African Americans afflicted by the disease is higher than their White counterparts, the percentage of Black care providers is not.
Nelson’s own team was all-White, until Rae Blaylark, the founder and executive director of Sickle Cell Foundation of Minnesota (SCFM), recently came on board as a patient advocate.
“If you are a person of color in Minnesota, you often don’t have a choice in finding a provider that looks like you,” Nelson told the MSR. “We know that there is more trust when it is racially and culturally [a good fit] between patient and provider. Just with the addition of [Blaylark] to the team…people will show up to the clinic and take their medication and follow up. A lot of that is about trust building. That’s a national issue.”
A person with the Sickle Cell Trait (SCT) inherits a single Hgb S gene and usually does not have related health problems but is at highest risk for having children with SCD. If both parents have SCT, they will have a one-in-four chance with each pregnancy of having a child with SCD.
If one parent has SCD and another has the trait, the risk increases to 50 percent. If the child inherits two abnormal hemoglobin genes, including at least one sickle hemoglobin gene (Hgb S), they will have SCD.
The United States mandated in 1986 that newborn babies be screened for SCD, but it wasn’t until 2006 that all 50 states, Puerto Rico and the Virgin Islands had newborn screening programs in place. Minnesota instituted its program in 1989.
The Minnesota Department of Health reported that 1.5 percent of 67,232 newborns screened in 2017 were identified as having Hgb issues — 992 of 1,026 infants found with abnormal Hgb had the SCD trait, and 34 had SCD.
SCD is also a major killer of infants and children in developing nations, especially in India and sub-Saharan Africa. The Centers for Disease Control’s Mary Hulihan said via video that there are “gaps in knowledge” about SCD, including how many people may have it due to the lack of a national database.
Blaylark, who founded her nonprofit organization in 2015, has a child with SCD. She told the MSR that she first learned she had the SCD trait when she was in high school. “The 11th grade was the first time I even heard the words ‘sickle cell.’ I never heard the words again until I had a child and learned the results of [his] newborn screening when my son was two weeks old and I was told that he had sickle cell disease. I was 24.”
Blaylark’s son, now 22 years old, “is doing well,” she said. “Having a support network and an advocate at your beck and call has made a huge difference. But it doesn’t mean he hasn’t been faced with medical bias.”
Living with the disease
Both Lane and Brianna pointed out that as adults, SCD can be more difficult to navigate than as children. Lane stressed how his fear of being properly treated has increased as an adult:
“I don’t know where to go now. Emergency room? Do I want to go through that?” he said. “It is very shocking…to go into the adult world starting at age 18 and start to experience more blatant discrimination. While I was at Children’s, I did experience discrimination from different nurses. They didn’t check in as much or [would] say I didn’t look sick.”
“I got more discrimination going into the ER,” Brianna added. “I wonder how [having SCD] increases the racial bias not only in the medical field, but in our day-to-day lives in how it impacts us as sickle cell patients.”
“Whether you have sickle cell or know someone who is affected, someone in your family is affected by at minimum the sickle cell trait,” Blaylark said. “And if you don’t know who that is, someone in your community is affected by a disease that does not exclusively affect us, but primarily affects us [as Black people]. We have to be ready to take action at any given moment, and we do that by having ongoing conversations.”
She said that such forums are needed throughout the year. “I think it is important to keep the community informed of what we are doing,” Blaylark concluded. “I think we can do a better job of expressing, explaining, and educating the community.”