June 19 is World Sickle Cell Day and to shed light on this disease and the children who are affected by it, we spent some time talking with staff at SUNY Downstate Medical Center in Brooklyn, New York. They provide medical services and child life support to a community impacted by the disease.
What is Sickle Cell Disease?
Sickle cell disease is an inherited blood disorder. The blood cells become sickle shaped or crescent moon shaped and have difficulty passing through blood vessels. When vessels become blocked, oxygen and blood can’t reach bones or vital organs; this can cause severe pain and organ damage.
Children suffering from sickle cell disease are often admitted into the hospital for treatment of excruciating pain, pain that has been described as being hit with a hammer repeatedly in one area of the body. These episodes are called “pain crises” and are very common in some people with sickle cell disease.
“When they’re experiencing a pain crisis, we try relaxation techniques. We encourage the child to think of a more calming or peaceful place, or sometimes it’s just holding their hand,” says Lisa Smith, Director, Child Life and Parenting Education at SUNY.
The Forgotten Disease
According to the CDC, 100,000 Americans are diagnosed with sickle cell disease. Despite this alarming statistic, it is sometimes referred to as “the forgotten disease” because it is seemingly underfunded and lacks media attention.
Many patients at SUNY have sickle cell disease. All states in the US now require newborns to be tested for the disease. Babies as young as two months old can begin taking preventative measures to reduce symptoms and risk of infant death.
Dr. Scott Miller, Director, Division of Pediatric Hematology and Oncology at SUNY Downstate is an outspoken advocate for people and families affected by sickle cell disease. He supports wide use of a certain medication that has shown to significantly improve the quality of life for children. But, it lacks much needed research.
“We’re very aggressive in recommending hydroxyurea to our families, starting as early as age nine months,” Dr. Miller says. “It can reduce pain episodes by half and seems to have long-term benefits as well. I estimate 80 percent of people who see us for sickle cell are now taking it.”
Starlight Fun Center Entertains Kids During Transfusions
On average, Dr. Miller and his team see a hundred children a month who are battling sickle cell, to monitor treatment and address other issues. He recalls years ago treating a 3-year-old who had already experienced 17 pain crises. That little boy went on to receive a full ride scholarship to Howard University.
“That’s a success story and according to his mom, his early health track record was frightening,” says Dr. Miller.
Sickle cell can be especially frightening since affected children can have strokes. Fortunately, strokes can be prevented in children at highest risk of blood transfusions every 3 or 4 weeks. Lisa and her team at SUNY try their hardest to educate and distract kids during the timely procedure. One of the many techniques is the Starlight Fun Center.
“There are some points during the transfusions that kids can go visit the child life playroom and we have two Starlight Fun Centers there. They really look forward to that,” Lisa says.
Living with Sickle Cell Disease
A pain crisis can cause a child to stay in the hospital for days and even weeks. That means they miss out on school and hanging out with their friends. The SUNY team develop a trusting and comfortable relationship with the kids.
“Our patients come almost exclusively from low income homes. Many families aren’t able to stay during the child’s hospital visit, so it’s important for our staff to provide a familiar presence,” Lisa says.
According to Dr. Miller, the only potentially available cure for sickle cell is a stem cell transplant. Finding a donor is extremely difficult, it’s an expensive procedure and has serious risks associated with it. For most people living with sickle cell, it’s more about preventative care and managing the symptoms, like eating healthy, staying hydrated, avoiding extreme temperatures and maintaining a positive outlook.
You can help hospital staff like Lisa and Dr. Miller create moments of joy and comfort for children suffering from sickle cell disease when you donate to Starlight. Our programs such as the Starlight Fun Center, Starlight Brave Gowns and Starlight Visits offer entertainment and a fun distraction to kids so they feel less stress and stay happier.