Sickle Cell: The Last Health Care Frontier for Black Lives
This story was produced as a project for the 2018 Impact Fund, a program of the Center for Health Journalism at USC Annenberg.
For La Chondra Rhymes, growing up with sickle cell meant hiding her disease — and silently suffering through the pain that came with it. Rhymes said her aunt, who became her legal guardian after her grandmother passed away and she was briefly placed in foster care, did not want to take her to the hospital when she was in bouts of pain, even though her aunt was a nurse. Embarrassed of her disease, Rhymes sought solace in school, where she excelled.
At age 16, Rhymes ran away from home. But her disease continued to progress. When she was 20, she developed arthritis. She also had a stroke and a minor heart attack in her early 30s. Now, at age 43, she deals with pain nearly every day. "I feel like I have the body of an 80-year-old," she said. Although once ashamed of her disease, Rhymes is speaking out about her affliction. "I used to be so embarrassed," she said. "Now I don't care if people know that I have it. I think it's more important to educate people about sickle cell."
An estimated 100,000 people in the U.S. live with sickle cell disease. The genetic disease — which primarily affects African Americans — changes the shape of a person's red blood cells, which are responsible for transferring oxygen throughout the body. In a patient with sickle cell disease, the red blood cells are sickle-shaped, which can block blood flow. This often leads to neurological impairment, renal failure, and bone disease.
Sickle cell can also cause severe pain. About 50 percent of adult sickle cell patients have daily, chronic pain that "affects their ability to work, young people's ability to go to school," said Marsha Treadwell, a clinical scientist in the Department of Hematology/Oncology at UCSF Benioff Children's Hospital Oakland and director of the Sickle Cell Care Coordination Initiative at the hospital. "And there are life-threatening complications."
While sickle cell received widespread attention in the '60s and '70s during the Civil Rights and Black Power movements, the disease has mostly fallen off the radar of the general public. Although the treatment of pediatric patients improved immensely in the ensuing decades, the number of facilities with quality care for sickle cell patients remains extremely limited. And when adult patients don't have access to a specialty doctor or clinic, they often end up in the emergency room, where medical professionals may not have experience in treating sickle cell symptoms.
Because the disease primarily affects African Americans, the dearth of treatment options is considered a legacy of slavery and racism. "The origins of sickle cell disease are abuse, neglect, and racism," said Elliott Vichinsky, director of Hematology/Oncology at UCSF Benioff Children's Hospital Oakland.
Another major obstacle to care is that most sickle cell patients in California are on Medi-Cal. Treadwell noted that the Medi-Cal reimbursement rate "is really very poor," and that 70 percent of providers in the U.S. don't even accept Medicaid.
Sickle cell patients in the Bay Area have been more fortunate: UCSF Benioff Children's Hospital Oakland is home to one of the oldest and most respected sickle cell centers in the country. Few places have a comprehensive sickle cell treatment center like the one in Oakland. Annually, the Northern California Comprehensive Sickle Cell Center at UCSF Benioff Children's Hospital Oakland treats roughly 800 sickle cell patients annually, nearly half of whom are adults who have been going there since they were kids. About 70 percent of the hospital's patients are on Medi-Cal.
After running away from her aunt's house, Rhymes began receiving care at Children's Hospital Oakland. (She had also been treated there before she lived with her aunt.) She also attended the summer camps put on by hospital staff. "We'd pick our outfits out a year ahead — we were that excited about it," she said. At camp, among other children with sickle cell, Rhymes didn't have to hide her disease or feel ashamed.
For Rhymes, and countless other East Bay sickle cell patients, Children's offered much more than medical care. "I've been going to Children's Hospital since I was one, so they really know me," Rhymes said. "Being in foster care and not really having a core family, they kind of became my family."
So, about six months ago, when word spread at the hospital that UCSF was going to shutter the researching portion of Children's Hospital, Children's Hospital Oakland Research Institute (CHORI), doctors and patients were alarmed. The closure would have hurt critical research on sickle cell, which, in turn, would impact care, doctors said.
"CHORI has had a focus over the years in developing and integrating research projects that are synergistic with sickle cell treatment," said Vichinsky, who is the director of the Northern California Comprehensive Sickle Cell Center at UCSF Benioff Children's Hospital Oakland, where he has worked for nearly 40 years. Interviewed in February, Vichinsky said he was "very insecure about the whole situation."
After some public outcry and consideration, however, UCSF recently told the Express that CHORI would not only remain open, but is "preparing to enhance and grow clinical and translational pediatric research programs at the Oakland campus," according to Michael Anderson, president of UCSF Benioff Children's Hospitals.
While that's undoubtedly good news for local sickle cell patients, adults with sickle cell continue to die prematurely across the country — including in California. "We have more deaths of individuals under 30 than anywhere else in the country," said Mary Brown, president and CEO of the Sickle Disease Foundation of California, the oldest sickle cell foundation in the U.S.
"Children get the best care possible, no doubt. It's when that child becomes an adult, that's when they fall into a black hole."
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.
"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."
readwell of Children's Hospital Oakland said there's a direct correlation between these two facts. "The expectation decades ago was people wouldn't live beyond early adulthood, if not their teenage years," she said. "Ninety percent of children now are living into adulthood, and the adult medical world wasn't ready to receive all those patients."
Even within the Black community, sickle cell was misunderstood or just unheard of. The history of the sickle cell center at Children's Hospital isn't well-known, but it was born in part from the Civil Rights and Black Power movements, with a direct tie to the Black Panther Party. Oakland was the origin and center for activism around sickle cell.
Tolbert Small acted as a link between the Black Panther Party and sickle cell awareness. On a Sunday evening in a quiet home in the Oakland hills, Small took out a box of old newspapers, folders, black-and-white photographs, writings he's done, and speeches he's presented since the '60s.
Small explained how, in the late '60s, he became the primary physician for members of the Black Panther Party, and then became the medical director of the party's Sickle Cell Anemia Foundation in 1970. Now 76 years old, he's still a practicing doctor, at the Native American Health Center in Oakland.
Bobby Seale and Huey P. Newton founded the Black Panther Party while they were students at Merritt College in Oakland. (Coincidentally, the former site of Merritt College is now home to CHORI, the research facility of Children's Hospital.) The Black Panthers saw health-care as an integral part of Black liberation and strived to improve health-care for Black communities. Sickle cell screening and awareness became a focal point.
"Bobby [Seale] came out with the term 'Black genocide,'" Small said about how the founders of the Black Panther Party referred to sickle cell disease. Small worked with the Panthers from 1970 to 1974.
Small took out editions of The Black Panther, the official newspaper of the party, with cover stories about sickle cell. The Black Panthers opened community health clinics, including one in Berkeley that Small helped start, the George Jackson Free Clinic, which was one of the first to offer sickle cell screening.
"He was one of the rare doctors actively taking care of adult sickle cell patients," Vichinsky said about Small. "He remains a historical figure for sickle cell disease over the decades."
As the Black Panthers grew to 44 chapters and 14 free clinics across the country, they continued to advocate for sickle cell awareness. Each clinic screened for sickle cell, and some of the chapters without clinics did so as well. "They were the first wave, nationally, of sickle cell awareness. Given their resources, they did more than anybody," Vichinksy added.
"They were very popular at the time. It was kind of the heyday of sickle cell [awareness] in my mind," said Keith Quirolo, the former director of Pediatric Sickle Cell Program and Apheresis Program at UCSF Benioff Children's Hospital Oakland.
The grassroots approach within the community paid off: Small believes that the Panthers' high profile in mainstream media — their main image being Black folks carrying guns, not the social service programs such as free breakfast and free health-care — helped put pressure on Congress and President Richard Nixon to sign off on legislation to fund sickle cell centers nationwide.
In 1972, Nixon signed legislation to fund sickle cell research, in hopes of finding a cure, and to improve care for patients. An NIH grant funded 10 comprehensive sickle cell centers across the country, which lasted for several decades. One of these centers was at Children's Hospital Oakland. The Comprehensive Sickle Cell Disease Center opened at Children's Hospital Oakland in 1973.
But by 2005, the NIH discontinued funding for the 10 sickle cell comprehensive centers. For decades, while individual hospitals continued to care for children and some had specialized care for adults, the disease essentially was forgotten.
"Nixon wanted the Black vote," Quirolo said about why he believes Nixon approved funding for sickle cell. "The government looked at [the Black Panther Party] as terrorists. They started these sickle cell centers with a huge grant to take that away from the Panthers."
The Black Panther Party became public enemy number one in the eyes of CIA Director J. Edgar Hoover. COINTELPRO, the Counterintelligence Program of the FBI, infiltrated the Black Panther Party, wreaking havoc, distrust, violence, and more. By the late '70s, most chapters had become inactive.
But the legacy of the party continues today. Much of the research about sickle cell has taken place at CHORI. And the hospital's comprehensive center continues to treat patients using the results of the most current research, serving a vulnerable population that can't get specialized care elsewhere. Besides Children's Hospital in Oakland, there is only one other comprehensive treatment center for sickle cell in the state — at Children's Hospital in Los Angeles. There are other clinics and programs, such as in Fresno and at UC Davis, but they are not considered comprehensive centers.
Treyvonn Chadwick, 20, is a patient at Children's Hospital Oakland who undergoes monthly apheresis treatments — similar to a blood transfusion in which a patient receives new blood, but it also takes out the patient's blood at the same time. Children's Hospital Oakland is one of the few places in California that uses apheresis treatment and has trained staff to administer it — a program that Quirolo, along with a nurse, Alicia Garcia, started in 1995. While blood transfusions are more standard, and many sickle cell patients do undergo monthly transfusions, the apheresis treatment helps prevent strokes, treats an acute form of pneumonia, and can help alleviate severe pain — with the least amount of side effects, according to Bloom, an apheresis nurse.
Chadwick said his experience at Children's Hospital for the past 15 years led to his decision to work in the medical field. He's currently studying to be a medical assistant. "This experience of being here at the hospital, seeing the people that work here, actually guided me to do what I want to do," he said while lying in a hospital bed waiting for a treatment one day.
Currently, the only cure for sickle cell is a bone marrow transplant, which is not only risky and costly, but requires a bone marrow match and a willing donor. UCSF Benioff Children's Hospital Oakland does perform bone marrow transplants, and has cured 25 patients with sickle cell disease to date. But, it's mostly reserved for patients who have no other alternatives. Patients must undergo chemotherapy to destroy the old bone marrow, which severely weakens the body. And, in some cases, the treatment can be fatal if the patient's body doesn't accept the new marrow.
September is National Sickle Cell Awareness Month, a month in which the late Ron Dellums, who served as Oakland's mayor from 2007 to 2011, helped herald. In 1983, as a member of the U.S. House of Representatives representing the East Bay, Dellums co-sponsored a bill introduced by Julian Dixon, a member of the Congressional Black Caucus, designating this month as National Sickle Cell Awareness Month.
The future of sickle cell treatment — and cures — may be in gene therapy. Tentatively, there are promising clinical trials taking place at UCSF Benioff Children's Hospital Oakland. In addition, there is a new treatment that has not yet been tested but is being developed by Children's Hospital Oakland in collaboration with UC Berkeley. It is based on gene editing that would correct the mutation, taking one's own healthy stem cells from their own bone marrow. The trial is scheduled to begin in 2019 with a small group of patients. "What we'd be doing is actually correcting the mutation directly," said Mark Walters, interim director of research at UCSF Benioff Children's Hospital Oakland, who oversees operations at CHORI.
For decades, there was only one FDA-approved drug for sickle cell. But a year ago, hydroxyurea and L-glutamine were approved to help manage symptoms of sickle cell. Hydroxyurea is a chemotherapeutic drug that reduces pain crises and the need for blood transfusions. L-glutamine is based on naturally occurring glutamine, the building blocks of protein in the body, and has almost no side effects. A recent study published by Vichinsky and CHORI shows that L-glutamine decreased the number of pain episodes and hospitalizations in sickle cell patients.
But even with newly approved drugs — and a hope for a less risky cure in the future — access remains an issue. Seventy percent of people who could be on hydroxyurea aren't because doctors don't want to prescribe it, Quirolo said. And because treatments are costly and most patients are on Medi-Cal, insurance companies don't want to reimburse many of the drugs and procedures. With only two comprehensive sickle cell centers in the state, many patients continue to rely on emergency rooms for care.
There has been one bright spot, however: A new adult sickle cell treatment clinic opened in South Central Los Angeles last year. (About half of the state's sickle cell patients live in the greater Los Angeles area.)
Meanwhile, doctors, nurses, and researchers — many who have committed their entire careers to caring for patients with sickle cell — are now advocating for broader changes on the legislative and national level. Quirolo, who grew up in Oakland and retired from Children's Hospital a few years ago, helped author legislation that would fund more comprehensive sickle cell centers in the state. While this legislation has been tabled for now, its supporters hope that legislators and governmental agencies will pay more attention to the disease. They say that better, more comprehensive care for sickle cell patients will save millions of dollars in emergency room visits. California spent $338 million in 2014 alone for emergency room visits for sickle cell patients, according to the CDC.
In a small but significant victory, sickle cell patients have been exempt from new opioid legislation that restricts the amount of opioids that doctors can prescribe patients. Quirolo and others worked with legislators to make the exemption. Even though the law allows more pain medication to be given to sickle cell patients, it's still a different story in the ER when hospital staff are unaware of sickle cell symptoms. "You're treated like you're a drug abuser," Rhymes said.
Some patients themselves are trying to bring about awareness. Safia Abdul-Sabur, who immigrated from Jamaica as a child, experienced chronic high fevers, pain, and pneumonia-like symptoms.
She had hopes of becoming a doctor to help other sickle cell patients, but during her first year of college at UC Davis, where she was studying molecular cell biology, she got sick and missed a lot of her classes. As a result, she had to change her major. Today, she's managing the disease and is a preschool teacher in Berkeley.
Still, sickle cell research and care is close to her heart. "I feel like sickle cell is just as much an emotional crisis as it is a physical one," Abdul-Sabur said. "Sometimes you feel very isolated. Who do you really turn to that you can talk to that will understand? That was one of my drives to go to medical school, because I understand the patient's plight. I'm a patient myself." Although she didn't become a doctor, Abdul-Sabur said she tries to incorporate her passion for advocating and working with those affected by sickle cell into her teaching.
Abdul-Sabur credits all of her doctors, and especially the ones at Children's Hospital, for supporting her. She said they would give her their personal cell phone numbers even when she was no longer a patient, so that if she ended up in an emergency room, they could communicate with the on-call hematologist to ensure she received the care she needed. "I would be en route to the emergency room while on the phone with the doctors," she said. "By the time I got there, the nurses at the check-in station would be aware of my emergency."
All of the patients interviewed for this story said that the specialized care they received at Children's Hospital helped them significantly over the years, and that they could not have imagined their lives without the doctors and nurses who were their advocates. Many of these health-care providers and patients volunteer at the hospital's sickle cell summer camps and support groups. Wendy Bloom, for example, started a knitting circle for patients in hopes of providing them with other tools to cope with pain, and to build friendships outside of the hospital setting. There are groups for children and teens, and parents of children with sickle cell. This is what a truly comprehensive center looks like — not just primary care, but also care that encompasses the whole person and their families.
Richard, who has outlived most of her friends who had the disease, chose not to have children. "My ideology is like, if my body can barely make enough blood for me, how can it make blood for another person?" she said. "With having a purpose being here and living with sickle cell for so long, it's like, I speak up, and not in a sassy way, but it's like, I've been given gifts to do things that are gonna help me stay as much together as possible, you know what I mean, when it comes to sickle cell."
As for Vichinsky, he is more hopeful now that CHORI is not closing. He continues to do research about sickle cell and is trying to recruit top researchers to come to Oakland in hopes of improving the lives of patients. "I'm really excited that they're not closing it," Vichinsky said. "I'm looking forward to UCSF helping in expanding CHORI scientist recruitment."
And Rhymes? She also chose not to have children for fear of passing on sickle cell. "And, I don't want to be a burden on my kids," she added. But she's been a mother figure since she was in her late-teens, helping to take care of her foster siblings. And she's helping to raise her younger sister's two kids — "I see them as my kids, too."
[This story was originally published by East Bay Express.]