By Genoa Barrow | OBSERVER Senior Staff Writer

Mary Brown. Courtesy photo

Back in the 1970s, much focus was placed on sickle cell anemia and its devastating impact on the African American community. Parents were unknowingly passing on a mysterious disease to their children and precious little babies were dying. Something had to be done.

While there have been many advancements in science and technology the past five decades, help for those living with sickle cell has been slow to develop. 

Sickle cell disease (SCD) is an inherited disease in which a person does not have enough healthy red blood cells to carry oxygen throughout the body. SCD affects the oxygen-carrying protein inside the red blood cells called hemoglobin. It occurs when an individual inherits two sickle hemoglobin genes from both parents or a combination of one sickle hemoglobin gene and another abnormal hemoglobin gene from the parents. SCD is most common in people whose families come from Africa; South or Central America; the Caribbean; Mediterranean countries such as Turkey, Greece and Italy; India; and Saudi Arabia.

SCD causes many disabling symptoms such as anemia, which causes a person to feel tired, weak or short of breath, severe pain, or even stroke. According to estimates by the Centers for Disease Control and Prevention, SCD affects approximately 100,000 Americans, occurring in about 1 of every 365 African American births. About 1 in 13 Black babies is born with sickle cell trait (SCT).

Those who carry the trait have to be extra diligent about relationships and who they have children with – if they know they are carriers. Many still don’t.

“The bottom line is that the follow-up when a baby has the trait is not good,” said Dr. Marsha Treadwell, a leader at UCSF Benioff Children’s Hospital Oakland.

“What they’ll do is just send the family a letter because it’s not felt to be a life-threatening condition, like the disease is, that it’s just going to affect the person’s reproduction later on. Then most people by the time they get to the reproductive years, don’t remember they ever got the letter, or they maybe never were informed that they got that letter.”

SS genes, fetal hemoglobin, reticulocytes, beta-globin gene clusters and beta thalassemia. It can be a lot to wrap your brain around, but those living with sickle cell become knowledgeable because lives depend on it.

“It’s more complicated than people used to think,” Dr. Treadwell said.

Today, there are prenatal screenings and all 50 states screen newborns for SCD, along with tests for eyesight and hearing. There are also more Black doctors to offer culturally competent care and champion patients’ causes. There are more scientists of color determined to find solutions because they know Black health matters. Yet those living and working with sickle cell say there’s so much more work to be done.

“I really think there’s a lot more conversation about sickle cell disease today because back in the day, they didn’t expect adults to live with sickle cell disease,” said Sickle Cell Disease Foundation President and CEO Mary Brown.

Patients, known widely as “sickle cell warriors,” often confront systemic racism alongside constant pain that comes with the disease.

“Black and brown people, they still get and have always been pushed down in the health care system,” Brown said. “This is a problem with equity and quality of life. They deserve to have the same quality of life as anyone who doesn’t have sickle cell disease.”

Advocates continue to chip away at the issue.

“You keep talking about it. You keep putting it out there. You try to keep opening the minds and hearts of people,” Brown said.

A Matter Of Time

Designated by Congress, National Sickle Cell Awareness Month is observed in September. Led by the Centers for Disease Control and Prevention, World Sickle Cell Awareness Day is marked June 19. These efforts help increase public knowledge and awareness of sickle cell disease, but much like African Americans and Black History Month, living with sickle cell isn’t something that can be relegated to just a few times out of the year. For those who live with sickle cell, it’s an everyday reality.

With “Painfully Aware,” The OBSERVER lifts the voices of those living with sickle cell and amplifies those working to educate people on the disease and the importance of providing quality and consistent care. Through this series, readers will be introduced to sickle cell warriors and stakeholders who want to see sickle cell disease get equal focus as other chronic ailments that disproportionately impact the Black community, such as diabetes, stroke, heart disease and cancer.

A lot has been happening in California in recent years. Gov. Gavin Newsom’s 2019 Budget Act, securing $15 million to improve access to comprehensive SCD care by creating Networking California for Sickle Cell Care (NCSCC). With a plan and funding in hand, NCSCC, led by the Center for Inherited Blood Disorders in partnership with the Sickle Cell Disease Foundation, got to work supporting 12 clinics.

In February, the network announced it has successfully reduced preventable hospitalizations of adults with SCD by 20% and total length of hospital stays by 50% since its inception three years ago. According to its data, before NCSCC was established, California spent more than $162 million annually on sickle cell-related emergency room visits and hospitalizations. Leaders say once NCSCC has reached full capacity, it will save the state $80 million to $100 million per year.

“The level of difficulty for patients to access sickle cell disease care three years ago was unacceptable. It was quite literally costing lives,” said Dr. Diane Nugent, founder and president of the Center for Inherited Blood Disorders.

“Today, patients have access to 12 specialized clinics in their local communities. As a direct result, we’re reducing the number of preventable ER visits and hospitalizations, saving state resources and most importantly saving lives,” Dr. Nugent continued.

Brown estimates 6,000 California adults live with SCD.

“There was no real transition from pediatric to adult care, they weren’t prepared for adult care, so now we’re in a space where these kids are living longer,” she said. “They’re doing well, but they get to this place to transition from pediatric care to adult medicine and they fall into a hole, a black hole. Our goal was to build up adult sickle cell clinics in the state of California, to provide them with appropriate access to care, specialty care services and an understanding medical team that knows how to treat sickle cell disease.”

Brown, who has been at the work for decades, said, “I fell in love with the tenacity and the courage of the children who just want to be like anyone else.”

This article is part of a series that highlights sickle cell’s impact on the African American community and how medical professionals are working to remove barriers to care and ensure that patients get the treatment they deserve.