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The lack of research funding, attention, and treatment of sickle cell are considered a matter of racial inequity.
"In the whole state and the nation, it's really about health equity," said Wendy Bloom, a nurse at Children's Hospital Oakland who has treated sickle cell patients for more than three decades. "It is a Black Lives Matter issue because it's primarily a genetic disease that affects Black people who often also have fewer financial resources." Bloom said these patients often don't get access to the complex care they need, and research on sickle cell gets much less funding than other rare genetic diseases that affect the white population, such as hemophilia and cystic fibrosis.
Sickle cell has its origins in countries that have malaria. The sickle cell gene mutation was a genetic adaptation that has helped people naturally fight malaria. Children who have the trait are more likely to survive malaria infection.
Because the blood cells in someone with sickle cell disease are misshapen, anywhere that blood passes through can be damaged, creating clots or blockages in veins and organs. It can cause strokes and organ failure. Due to hospitalizations, sickle cell patients have a harder time finishing school or maintaining jobs. The amount of pain is hard to describe, but all the patients interviewed for this story said that unless they are working with doctors who are aware of the disease, health-care practitioners underestimate how much pain medication they need. One patient said even wind on a chilly day can cause pain.
But because of stereotypes, many patients face barriers and discrimination when trying to access treatment.
"People face racism when they are seeking medical care," said Marsha Treadwell of Children's Hospital Oakland. As an example, she said when a young patient walks into the emergency room and asks for pain medication, "they're labeled as drug seekers."
Studies show that health-care providers, who are mostly white, treat Black patients experiencing pain differently than white patients: They are given less pain medication and, according to one study, white medical students believe they have less sensitive nerve endings.
There is also an economic factor. "There are a number of deaths that are definitely preventable," Treadwell said. "Patients don't go on a whim to the emergency room. They've been suffering at home for two to three days already." Often, those patients are forced to wait for pain medication for hours, and they may not be evaluated for life-threatening compilations because they're assumed to be a drug seeker. "So it's really a very negative cycle that patients are caught in," Treadwell continued. As a result, people can die from organ failure, stroke, or heart attack.
All the more reason that the sickle cell treatment at Children's Hospital Oakland has been invaluable for patients, especially adults. Rochelle Richard has been receiving treatment at Children's Hospital Oakland since she was a child. Her parents moved from Dallas, Tex., to Oakland in the '80s, in part, because the climate in Oakland was milder. At the time, Oakland's African American population was around 50 percent; today, it's dipped to 25 percent of the population.
Richard says she's grateful to have gotten care at Children's Hospital. She's now 50, above the average life span of adults with sickle cell. The average life expectancy for people with sickle cell is between 42 and 47. She's seen many of her peers with the disease die.
To maintain her health, Richard undergoes monthly blood transfusions. Days prior to getting a transfusion, she becomes extremely weak, tired, and in pain to the point where she can't bear to be touched. Once she gets the transfusions, it takes her about two to three days to feel back to normal. She balances Western medical care with a Chinese doctor she visits in Oakland Chinatown. She said she is in pain almost every day.
- Photo by Drew Costley
- Rochelle Richard balances care she receives at Children’s Hospital Oakland with Chinese medicine.
"Sometimes, you just don't have enough blood," Richard said. "Your cells don't last as long as the average person's cells. You could feel fine and then all of a sudden — boom — five minutes later, I'm sick or have to call an ambulance."
Nationally, 2.5 million Americans carry the genetic trait for sickle cell. In California, one in 12 African Americans have the trait. Parents pass on the trait to their children. If both parents carry the trait, there is a one in four chance their baby will have the disease. California, like some other states, started screening newborns for the disease beginning in 1990, which reduced the infant mortality rate. Infants with the disease were treated with penicillin, which dramatically decreased the rate of death from infection — the most common cause of death for infants.
In the U.S., the survival rate of children under age 18 has continued to improve since the '70s. "Children survive to adulthood more than they did 30 years ago," said John Strouse, a doctor and director of the adult sickle cell program at Duke University and a member of the American Society of Hematology.
But as treatment of children improved, adult care did not. "What many people are concerned about is the average age for sickle cell disease has appeared to be the same — around 40," said Strouse. "While survival has improved for kids, and more are getting to become adults, they're still dying as adults."