At Children’s Hospital Oakland, a lifelong program helps people with sickle cell anemia

There is a stretch of California Interstate 80 that Lynette Davis knows better than the back of her hand.  Each month she loads her children—18 year-old De’von and 12 year-old Khaliah—into the car and drives the hour and a half from their home in Davis, California, to Children’s Hospital in Oakland. Davis makes the monthly trip down that long section of highway so that her kids can lay back in hospital beds as intravenous lines send blood laden with perfectly disc-shaped, oxygen-rich red blood cells coursing into their young bodies. Without it, aches and pains begin to riddle their joints and fatigue shows on their faces when they try to keep up with other kids on the playground or around the neighborhood. That’s because De’von and Khaliah are two of the approximately 100,000 Americans that suffer from sickle cell anemia.

Sickle cell is an inherited blood disorder in which the oxygen-carrying molecule of red blood cells—hemoglobin—is defective and can’t hold on to oxygen the way it should. As a result, the red blood cells form long, rod-like structures that look like sickles, instead of their characteristic donut shape. They can stack up and cause blockages in blood vessels, causing pain and leading to other serious medical problems.

The only cure for sickle cell is a bone marrow transplant, an option that is not available to everyone—you have to have a match, and finding one can take years, if you are lucky enough to find one at all. Public perception of the disease is plagued by the misconception that it was cured back in the 1970s. What’s more accurate is that while it can be cured with a transplant, that doesn’t mean it’s cured for everyone. For most people it’s a chronic, lifelong illness.

One of the ways that people with sickle cell cope with the disease is to have blood transfusions every month to bring fresh red blood cells to their oxygen-starved bodies. Davis says she can see the change it makes for De’von and Khaliah. “It gives them more energy,” she says “They’re more vibrant. Like, being able to jump and play—they’re more able when they get the blood.”

In addition to the monthly visits for transfusions, the Davises make the East Bay trek at least once a week for check-ups, bloodwork and to get medications. That’s a lot of time on the road for Davis, who is a full-time nursing student in addition to being a full-time mother and caretaker. But the trips are worth it, she insists, to get the quality of care that her family has found at the Center for Sickle Cell Disease at Children’s, which offers the largest and most comprehensive sickle cell treatment program in California.

“There were times we would go to the emergency room in Davis,” she says. “De’von would have a temperature of 103 degrees and lots of pain and the triage nurse, not knowing about sickle cell, would make us wait for hours.” One of these times, after giving him fluids and pain meds the hospital sent him home, Davis says. A few hours later, her son was back and in the ICU on a ventilator. “It’s really important that the first people they see in an emergency situation really know all the necessary information about sickle cell,” Davis says. “Here at Children’s they get all the necessary tests and medications immediately.”

Children’s Hospital Oakland’s program is also unique within the hospital, in that sickle cell patients don’t transfer out when they turn 18 the way other patients at the pediatric institution must. Instead, patients continue to come to Oakland to be treated for sickle cell from birth through old age. The hospital’s oldest patient is 70 years old, and has been a part of the program since it was first established in 1978. Back then, the average lifespan of someone living with sickle cell was only 30 years, but with better medications and treatment options, patients are living longer.

“It’s a childhood illness that adults are living with now,” says Janice Earl, a registered nurse with the Center for Sickle Cell Disease. “Adult internists are not medically trained to take care of these patients. There’s not a lot of knowledge or training in the medical schools these days about sickle cell. But here, all of our physicians and nurses are highly trained specifically in the disease.”

According to Earl, this level of expertise and quality care is what brings many people to the Bay Area. “Patients choose to live here, or close to here, because they want to get their lifelong care here,” she says. “We still see patients that were here when they were barely a couple of days old—now married, with children of their own with the disease. There are generations of patients that are still here.”

The program, which currently treats 370 children with the disease and about the same number of adults, also places an emphasis on providing emotional support for patients and their families. Social workers and support groups help kids deal with the psychological stress of sickle cell disease. “It can be very depressing at times for children to have to come in and get poked with an IV or come into the hospital when they have other things they want to be participating in,” Earl says.

Davis agrees that the disease has taken an emotional toll on her children. “De’von has to control all these different aspects of his life because he can’t control getting sick,” she says.  “He just wants to prove himself in everything—in school, even going to the grocery store and choosing what he’s going to have for dinner. Khaliah is not as outgoing as she could be, and that’s just feeling like she doesn’t measure up because she’s different.”

But Davis says the support her family has found at Children’s Hospital Oakland, both in their staff and through other patients they have met through the program, has been invaluable in helping her children live full and happy lives. De’von graduated high school this past spring and is planning to go on to college. He is currently transitioning into taking ownership of his own healthcare—taking himself to his own appointments and being in charge of his medication regimen. Khaliah, who recently had to take some time at home to recover from surgery, will return to school in the fall.

“I can never understand what they go through,” Davis says. “I can only go through it with them. But there’s this feeling you get as a parent when you’re children are in great hands. At Children’s, I know they’re getting everything.”

Image: De’von Davis and his sister Khaliah during a trip to Children’s Hospital. Photo courtesy Children’s Hospital Oakland.