Finding the right sickle cell disease care team
Chandra Wallace does not remember a time when she was not “sick.”
Her childhood was burdened with repeated hospital visits while cold, tired and in pain. At age 10, she had her gallbladder removed because of gallstones. When Wallace was 16, she developed painful ulcers on her leg. She got COVID twice in 2021 and was hospitalized for weeks.
Wallace, now 33, has sickle cell disease, which is the most common genetic disorder in the United States, and is widely prevalent in the African American community. It remains incurable.
“For a sickle cell patient, there is never a state of normalcy,” she said. “You have to learn how to cope with your body. You have to learn your triggers and what you can or cannot do.”
This is why finding her care team at UCLA Health’s sickle cell center, which opened last year, has been a blessing. Her painful crisis after the pandemic would have continued had she not found a team that knew exactly how to manage it, Wallace said.
The center, which currently has two hematologists, half-a-dozen primary care doctors, one nurse practitioner and a social worker, has been an invaluable resource for patients like Wallace who have otherwise struggled to find comprehensive care for this chronic condition.
Disease without a cure
Sickle cell disease is a genetic disorder associated with the production of an abnormal hemoglobin molecule, which performs the vital function of carrying oxygen to the tissues. In a person with sickle cell disorder, the red blood cells become deformed into a crescent or a sickle shape. Their consistency becomes like Jell-O rather than water, causing the red blood cells to become deformed and stuck.
“As a result, every organ in the body can become affected,” said Gary Schiller, MD, a hematologist at the center who treats Wallace. “This condition blocks the flow of blood to the brain, heart, lungs, skin and bone.”
According to the U.S. Centers for Disease Control and Prevention (CDC), sickle cell disease affects about 100,000 people in the United States.
It occurs in about one out of every 365 African American births, and around one in 13 African American babies is born with the sickle cell genetic trait. The disease is also common among people from parts of the world where malaria is or was common, the CDC states.
Dr. Schiller said while there is no cure for this disease, there are therapies that prevent the red blood cells from sticking. One new therapy makes the hemoglobin hold on to oxygen, which gives it a liquid, water-like consistency so undamaged red blood cells can freely flow to the vital organs.
The center uses drugs approved by the U.S. Food and Drug Administration (FDA), red blood cell exchange transfusions, investigative drugs and gene therapy to treat patients like Wallace, Dr. Schiller said.
Challenges are daunting
While pediatric care for those with sickle cell disease is excellent in the United States, care for adults is “fragmented,” Dr. Schiller said, because of the many challenges patients face.
The disease itself is devastating because it induces chronic illnesses. Patients experience problems with their eye health, teeth, skin, fertility, bones, heart and lungs. So, serving this population becomes even more challenging for the medical community.
“You need a dedicated group of medical colleagues,” Dr. Schiller said. “There are physical issues, logistical issues in terms of staff, pain management issues that require an integrated, multi-disciplinary team, and also social issues related to chronic disability and absenteeism from work.”
The goal of UCLA Health’s sickle cell disease center, he said, is to help patients avoid emergency room and urgent care visits for management of sickle cell complications, fluid hydration, oxygen and pain management.
The center also aims to provide access to dental and ophthalmological services, cardiac consultation, and social work support over time, he said. For the last year, the center’s team has worked to create a free-standing hematology area with a nurse practitioner and a social worker.
“We identify patients who could receive primary care in the community but can take advantage of things that we have here for the treatment of cancer patients, so they can avail of the same kind of seven-days-a-week outpatient care,” Dr. Schiller said, adding that the clinic hopes to serve 100 patients by the end of this year and 200 by 2024.
Barriers to care
Emotional and psychological care for patients is also lacking, Dr. Schiller said.
“There are so many social and mental health issues because of the chronic illness,” he said. “People with sickle cell disease find it difficult to maintain employment because of high absenteeism. The cost of treatment is high.”
Before this program at UCLA, there were “limited options for adult patients,” said Erica Martinez, nurse practitioner at the center, which currently sees 32 patients both at the center and at satellite clinics. The pain crisis for patients can last anywhere from four days to two weeks. From stress to changes in the weather, anything could trigger it, Martinez said.
While the sickle cell champions focus on the primary care aspect such as eye exams and immunizations, those on the hematology side at the center manage sickle cell disease with medications, transfusions and infusions, which helps reduce the frequency and severity of pain crises, she said.
Martinez said the center is also utilizing the infusion center to manage crises in an outpatient setting, and treating pain crisis via IV pain management and fluids, adding that the infusion center is being used as a resource to help manage crisis in an outpatient setting rather than in the emergency room. Because transportation is also a major challenge and barrier to care for many patients, UCLA also has a contract with Lyft to help patients get to the clinics.
Dealing with bias
Wallace said she counts on the UCLA team to get her monthly infusions and hemoglobin checks. She also keeps them regularly informed about her health.
In addition, she started psychological therapy about four years ago.
“This is a very traumatic illness,” she said. “I have a lot of PTSD and fears of being sick. Psychological well-being is a very important part. I did not grow up in a family that prioritized it. So, I had to learn how to practice self-care and process trauma with my therapist.”
She also believes it is important to have a diverse care team because of the prejudice she has faced as a Black woman.
“I just wish more doctors would release the prejudice that Black people don’t feel as much pain,” Wallace said. “I wish I didn’t have to prove that I’m not addicted to drugs and I’m not going to the ER to get drugs. I’m not choosing to go there. I have a chronic blood disorder.”
She recalls one trip to the emergency room when a Black doctor was the only one who recognized that she was a sickle cell disease patient.
“He explained to the other doctors that I was in excruciating pain because he was the only one who knew,” she said. “I do think diversity in the medical profession would greatly help.”
She also wishes the public were more knowledgeable about her condition -- that those with sickle cell disease are individuals with a disability who are still functioning.
“I feel like people automatically judge you when you say you are experiencing chronic pain,” Wallace said. “They don’t understand it. As a society, people need to understand that suffering.”