Once a month Ragina Bell travels to UCSF Benioff Children’s Hospital in Oakland for a blood transfusion that literally keeps her alive by replacing her faulty red blood cells with healthy ones. The trip requires an overnight stay and she must find someone to watch her two sons while she’s away.
“When I first get a transfusion I feel tingly like I had a lot of caffeine, and I have a lot of energy. I feel great. But as the month wears on I get more tired, and I sleep more,” said Bell, who was born with sickle cell anemia, a genetic disease that disproportionately strikes Blacks and Hispanics. “Even though I’m 32, Oakland children’s hospital is still taking care of me since they have one of the few adult sickle cell clinics.”
Until recently, the Valley’s estimated 575 adults with sickle cell disease had to seek care in the Bay Area or Los Angeles region. In July, the Sickle Cell Anemia Clinic opened in the Community Cancer Institute headed by Dr. Mohammed Sani Bukari, a hematology oncologist and clinical faculty member at UCSF Fresno. Now Bell sees Dr. Bukari at the clinic on the Clovis Community Medical Center campus and she hopes her next transfusion will be close to her Fresno home.
Funding to help set up the Clovis clinic came through a California Department of Public Health grant to help set up a network of collaborative sickle cell clinics to serve patients across the state. The Fresno area has higher sickle cell rates than California as a whole – 32 per 100,000 people compared to California’s 26.6 cases per 100,000.
Sickle cell anemia affects Blacks and Hispanics more than other races
Sickle cell anemia hits certain races more than others. One out of every 13 Black babies is born with the sickle cell trait and 1 out 365 will have sickle cell disease having inherited the gene from both parents. One out of every 16,300 Hispanics is born with sickle cell disease. In California, 37% of newborns with the sickle cell trait are Hispanic and the rest are nearly all Black. And in California, those born with sickle cell disease have a lower life expectancy than elsewhere.
“In the United States, life expectancy for sickle cell is 61 years. In the UK (United Kingdom) it’s 70 years. In the state of California, it’s now down to 43 years,” Dr. Diane Nugent, president and medical director for the Center for Inherited Blood Disorders, told the California legislature last year when it approved funding for sickle cell clinics so patients don’t need to travel so far. Without ready access to care, adults with sickle cell anemia in California often go without.
“Overall pediatric experts have done a great job of identifying and taking care of patients as soon as they are born because of newborn screening,” Dr. Bukari explained. “But unfortunately there’s not a lot of adult doctors specializing in hematology oncology and particularly those interested in sickle cell disease so there’s a problem in transitioning from pediatric to adult care. This means people usually aren’t getting the follow up care they need and most tend to get their care in the emergency room and that leads to lower life expectancy.”
Dr. Bukari is collaborating with Valley Children’s Hospital to better transition pediatric patients to the clinic once they reach adulthood. He is also working with Cayenne Wellness Center, a local sickle cell advocacy and support organization, to inform adults without regular care about the new Fresno area clinic now available for specialized care.
Sickle cell disease starves cells and causes pain
People with sickle cell anemia inherit a gene from both parents that causes red blood cells to lose their flexibility so they’re unable to squeeze through small blood vessels. The cells curve in a sharp sickle shape and clump together rather than delivering oxygen to tissues through the body’s tiny blood vessels, explained Dr. Bukari. “This clumping is what actually causes the pain crisis that is associated with the disease.”
Bell had frequent debilitating pain episodes as a child and in her early 20s that pain occasionally sent her to the hospital. “The pain is indescribable. It’s not sharp but just constant throbbing,” she explained. “Sometimes I have trouble getting out of bed. Sometimes, though, if I take a hot shower and drink hot liquids to warm up my body, it’ll go away.”
The unpredictability of her pain episodes makes it difficult to have a job, Bell said. “I never know how my days are going to go. I try to push through, but sometimes I just can’t. I look fine, but I don’t feel fine.”
When she does feel fine, Bell helps organize blood drives. She knows firsthand how vital the blood supply is. She depends on 6 pints a month matched to her blood type to stay healthy.
New clinic provides access to pain management, specialized treatments
Bell said she’s having more and more good days, having learned to stay hydrated and rested besides getting regular transfusions. Now she’s working with Dr. Bukari to closely monitor the effects of lack of oxygen on her tissues and to try to prevent damage to vital organs.
“Most people with sickle cell disease die of too much iron,” Dr. Bukari explained. “Their bodies have a tendency to absorb a lot of iron, especially because we give them medications that increase the iron to help with anemia. And iron is hard on the liver and the heart. Most people with sickle cell anemia die of heart failure, liver failure or kidney failure.”
Community’s Sickle Cell Anemia Clinic patients will also have access to pain specialists and nutritionists who support other patients at the Cancer Institute. “Our goal is to reduce patients’ use of the ER and improve their care with new medications and techniques just approved in the last few years,” said Dr. Bukari. New treatments include stem cell transplants and gene therapy.
Dr. Bukari, who also carries the sickle cell trait, hopes the clinic will serve to train UCSF Fresno residents in internal medicine and primary care/family medicine about sickle cell disease. “We’d also like to do outreach and education in the community, especially for family practitioners,” he said. He has hopes that rural areas with only a handful of sickle cell patients may also have a doctor who knows how to care for them.