Sickle-cell disease affects one in every 500 African-American children born in the United States, and one in 12 African-Americans carries the sickle cell trait. While there are highly effective treatments for the sometimes deadly condition, patients don't always have access to or comply with the medical regimen, which includes regularly taking their medications and keeping clinic appointments.
Monica Mitchell, PhD, is working to change that. As a pediatric clinical psychologist at Cincinnati Children's Hospital Medical Center, she is conducting innovative studies that involve families in children's treatment plans, as well as through community events—such as Sickle Cell Research and Education Day—that promote family involvement and raise community awareness about the disease. Promoting adherence to medication and physician visits is vital because sporadic medication use can lead to a range of toxic reactions, and irregular clinic visits mean doctors can't properly monitor these children's health or medication tolerance.
Mitchell's aim is to maximize treatment effectiveness for the disease, which causes the body to produce abnormal, crescent-shaped red blood cells that can impede blood flow to limbs and organs. If untreated, sickle cell disease can cause pain, infections, organ damage and stroke.
"While the disease is a prominent and important part of who these kids are, it's not all of who they are," Mitchell says. "My work is to understand sickle cell disease in the context of the entire family system so we can help these children lead normal lives. One way to do that is by helping them adhere to their medications so they can be in school more often, for example."
Mitchell is a pioneer in the way she has involved the community and families in research and treatment, says Michael R. DeBaun, MD, director of the Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease at Vanderbilt University.
"I don't know of any other individual who has engaged the [sickle cell] community this successfully, not just in helping patients and their families understand the importance of research, but in getting them actively involved," he says. "She has created a new paradigm, which says that individuals with this disease should be as informed as possible … and that they should see their interaction with the medical community, particularly the research community, as a partnership."
That's especially important, he says, given African- Americans' historical maltreatment by—and mistrust of—medical researchers.
Mitchell began thinking about sickle cell medication adherence as a resident at Cincinnati Children's in 1997. During her rotation with young sickle-cell patients, she noticed that many of their caregivers faced a number of real-world obstacles—getting school support services, managing transportation for clinic visits and trying to keep their jobs while their children were hospitalized, for example. For families balancing multiple demands with disease management, treatment often fell by the wayside.
In a 2007 article in Clinical Pediatrics, based on focus groups at four Ohio hospitals, Mitchell and her and colleagues reported that family-based approaches are vital to care because compliance relies on good parent-child communication and effective strategies both agree on, as well as on good coordination and transparent communication between the care team and the entire family. The focus groups also revealed that these children often had multiple caretakers—not just parents, but grandparents, aunts and others. As a result, "You almost have to give a child a care pack wherever they're going to be," Mitchell says.
Making adherence easier
These findings led Mitchell and colleague Lori Crosby, PsyD, to launch a study funded by the National Institute of Mental Health that has parents, other primary caregivers and children working together to develop care plans that help children take their medications and come to clinic appointments regularly.
In the study, which is ongoing and expected to involve 45 children and their parents, participants log onto a website that helps them identify the biggest barriers to the child's compliance and suggests strategies for overcoming them. A common obstacle is forgetfulness: A child forgets to take her pills or a parent forgets to refill his child's medication. Suggested strategies might be maintaining a calendar, setting an alarm clock or using a pharmacy refill service.
Families identify one barrier at a time, because people are more likely to stick to a simple plan than a complex one, says Mitchell. "Choosing one barrier forces them to marshal their thinking and resources around addressing it," she says. Some of the children also receive electronic pill bottles that signal when they've been opened, which allows the team to precisely track when the youngsters take their medicines. A health professional checks in with the families after one week and again after one, three, six and 12 months to see how they are adhering to their plan and to help them address any pitfalls.
To date, the team has followed about 30 children for six months, and the results are promising. "They're increasing their visits relative to their own baseline data, and they're adhering to their medication more often," Mitchell says. In addition, a paper under review at Anemia shows that the Web-based tool integrated seamlessly with other clinical tools, such as patients' electronic medical records, staff reports and clinical lab data. Patients were highly satisfied with the tool, as well. These findings suggest it will be possible to translate the intervention more widely, Mitchell says.
Now, armed with a four-year National Institutes of Health Academic Career Development Award grant, Crosby is exploring ways to help young people with the disease navigate the next step of their journey: transitioning from pediatric to adult care. "When adolescents go to adult medical care, many of them fall through the cracks because of the difficulty adjusting to the adult-care setting," explains Crosby. "They may not know the physicians as well, and they're getting older, so parent support may not be as high."
Because of the way the disease tends to progress, there is an increased risk for disease complications as well. Crosby will examine these factors and develop support tools, including a patient self-management intervention and a Web portal that helps patients prepare for the transition by improving their ability to manage their disease and keep track of appointments, medications and lab values.
A special day
In addition to conducting traditional research, Mitchell is raising family and public awareness about sickle cell disease. "If a kid has a fever"—often the first sign of dangerous infections —"we want everyone in the family to know when to bring that child to the hospital, what to do, what the signs are," says Mitchell. "We want everyone to be educated on what sickle cell disease is, and to become advocates who can educate other people in the community."
That's why in 2001 she started Sickle Cell Research and Education Day. The event brings together sickle cell patients, families, volunteers, hospital staff, celebrities and corporate sponsors for educational events and fun activities. Children swap strategies for managing their pain and take part in yoga classes and art therapy sessions, while parents join in educational games and demonstrations.
The day also features something that most similar health events don't: educating participants on the importance of research. Attendees can take part in short-term projects, such as answering questionnaires related to the disease or designing flyers to educate the community about the condition. They can also receive information about or sign up for longer research projects.
Lisa Berry Hughes, whose daughter Briana, 19, has sickle cell disease, attended the first research day and has gone back every year since.
"Growing up, we didn't have a lot of education on what to do if you had the sickle cell trait or sickle cell disease," she says. "After going to some of these research days and other classes at Children's Hospital, that changed. They actually want you to know some things—they take time to explain stuff to you, help you learn how to cope, help you have as normal a life as possible. The education component is key, because when you're educated you can make better choices."
Data, too, show the activity is hitting the mark. At the 2011 event, 79 percent of participants said they enjoyed learning about research; 73 percent said that they or their children participated in research that day; and 81 percent that they felt better about research since attending it. Others are impressed, too. The event is being replicated at five other hospitals nationwide, including Texas Children's Hospital in Houston and Vanderbilt's Medical Center. The Heart Institute at Cincinnati Children's Hospital is also latching onto the idea.
By blending the worlds of research and community interventions, Mitchell is adding something novel and important to the field, says Scott W. Powers, PhD, director of Cincinnati Children's Office for Clinical and Translational Research.
"Some people are good at getting up and advocating and getting people going, but they may not turn that into results like a scientist can," he says. "That's what makes Monica so special in her field. She's does all of those things and she integrates them."
For her part, Mitchell sees the event as one more way to reach youth who need help in a language and venue they can relate to.
"I want children and adolescents to receive new approaches to treatment and to be inspired to manage their health without even knowing it," she says. "As psychologists, we can empower children, parents and communities by engaging them as vital partners in improving their health."
Tori DeAngelis is a writer in Syracuse, N.Y.
Sickle cell facts
- Sickle cell disease affects millions of people throughout the world and is particularly common among those whose ancestors came from sub-Saharan Africa, Spanish-speaking regions in the Western Hemisphere, Saudi Arabia, India and Mediterranean countries, such as Turkey, Greece and Italy.
- Sickle cell disease affects about 90,000 to 100,000 Americans. It occurs in roughly one in every 500 African-American births and one in every 36,000 Hispanic-American births.
- Sickle cell disease occurs more often among people from parts of the world where malaria is or was common. Sickle cells may provide resistance against the malarial parasite, but more research is needed to prove the association.
- Sickle cell-related deaths among African-American children younger than 4 years fell by 42 percent between 1999–2002, coinciding with the introduction in 2000 of a vaccine that protects against invasive pneumococcal disease.
Source: Centers for Disease Control and Prevention
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