Striving to thrive: A family-centered approach to treating youth with sickle cell disease
Knai Bridges, who was a senior at Nottingham High School when the above photo was taken, wrote a college application essay that described the years of pain and hospital visits that her sickle cell disease had caused but noted, “although there was so much pain, there was joy”: Her condition eventually improved, her mother, Nailah Beyah, was by her side all the way, and she met her best friend in the hospital. Knai’s essay included the affirmation: “I have sickle cell. Sickle cell doesn’t have me.”
STORY BY JIM HOWE
PHOTOS BY ROBERT MESCAVAGE
It’s the world’s most common inherited blood disorder, yet it is widely misunderstood or even unknown to much of the public.
Children with sickle cell disease must learn from an early age to avoid infections, temperature extremes or changing temperatures, and physical or emotional stress.
Their parents can feel they are fighting a lonely battle as they talk to teachers, gym instructors, school nurses and even relatives and neighbors about their children’s needs and limitations.
The children’s family members must learn how to help out and to deal with the challenges the disease brings to their households.
In response to these challenges, the pediatric sickle cell program at Upstate aims to treat the illness as something that affects all aspects of a family’s life.
Kyren Herring, 8, of Syracuse, who got his face painted with a Buffalo Bills logo, describes his illness: “Sometimes it makes me tired. Sometimes I hurt. Sometimes I have to go to the hospital.”
Pediatric psychologist Jill Majeski, PsyD, explains how the sickle cell team addresses patients’ needs, such as:
medical: doctor visits and hospitalizations, medications, pain episodes (called crises) and infections.
social: stigmas related to mistaken ideas about the disease; its impact on school and relationships.
psychological: how the disease affects mood, worries and self-esteem.
Upstate’s program treats about a hundred patients from infancy through age 21 across a wide swath of Upstate New York. Adults are treated through a separate program.
To reach out to families of sickle cell patients, the pediatric sickle cell program held an event in late 2019 called “Thriving Together: Starring Kids With Sickle Cell Family Event,” which was underwritten by charitable groups, such as The Kara Fund, a local charitable group that helps children with life-threatening diseases and their families.
The sickle cell team hopes to repeat the event.
“Thriving Together” allowed people touched by the disease to meet and to hear a talk on the disease, advice on being a child’s advocate and how to cope with the disease’s complications.
Insights from ‘Thriving Together’
Playing a game that uses elbow macaroni to teach about sickle cell showed Ryleigh Farrell, 9, of Syracuse that “when parts of my body hurt, like my arms or legs, it’s your blood clumping together like a crescent moon.”
Iliyana Funderburg, 10, of Syracuse, who played the game with her, thought it was ”really cool” to see how her blood cells are affected. She noted that neither adults nor children at school usually understand her sickle cell disease: “My mom has to tell my teachers every year of what to do if I get cold or sick at school,” she said.
Emanuel “Manny” Liles, 15, of Liverpool, who has sickle cell disease, describes it as “complicated to talk about and hard for people to understand.” His mother, Sheryl Liles, appreciates the emotional support of meeting other people who are dealing with what can be an “overwhelming” condition. Anything that helps spread accurate information about sickle cell disease is helpful, she added.
Antoinette Singleton of Syracuse, who attended the event with her grandson, Kyren Herring, age 9, who has sickle cell disease, agreed that meeting others facing the condition was “wonderful.” She said it added another aspect of support to the medical, social and educational help provided by the members of Upstate’s pediatric sickle cell team, which she said “has been nothing but positive.”
“A lot of success with kids relates to how parents are instructing and teaching them,” said Kathryn E. “Katy” Scott, MD, the director of the pediatric sickle cell program.
Among the program’s goals are preparing the young people to understand and manage their disease, so they will be able to deal with it in adulthood.
Since pain is often an issue, a second goal is to minimize the use of medications when possible. “I teach parents about how to help children with chronic pain, coping when we can’t make it go away,” said Majeski. “I work with kids and parents to focus on those two goals.”
While there is no universal, risk-free cure for sickle cell disease at the moment, there are promising developments, such as:
-- voxelotor, a recently approved drug for older teens and adults;
-- stem cell transplants, which carry risks but can result in a cure;
-- and treatments still being researched, including gene therapy and new drugs.
Scott is hopeful that a cure will be available to everyone with sickle cell disease in the next several decades, within the lifetime of the young patients she sees now.
Sickle cell disease is an inherited
Sickle cell disease can turn normally flexible and round red blood cells into hard, sickle-shaped (or C-shaped) cells.
This disrupts normal blood flow and can lead to painful episodes known as sickle cell crises as well as anemia, infections, fevers, yellowed eyes, organ damage and early death.
People with sickle cell trait (who are sometimes called sickle cell carriers) do not have sickle cell disease but can pass the chance of getting it to their children, as the chart at left shows.
Sickle cell disease affects about 100,000 Americans and occurs more often in people whose ancestors came from Africa, the Mediterranean region, the Middle East and other parts of Asia.
Various treatments can alleviate the disease, but there is no universal cure at the moment.
American Society of Hematology
Sickle Cell Disease Association of America Inc.