Shayla, 12, has been living with the pain and complications of sickle cell disease all her life. From countless trips to the Emergency Department to several major surgeries and frequent clinic visits for preventive treatments, the experts at Cincinnati Children’s have been here for Shayla and her family every step of the way.
Lying back on her bed and texting her friends, Shayla looks like any other 12-year-old relaxing after a day of school.
Except her bed isn’t in her room at home – it’s in a clinic room at the Comprehensive Sickle Cell Center of Cincinnati Children’s. A thin tube delivers blood through a port in her chest while she undergoes a transfusion to treat her sickle cell disease, a genetic blood disorder that can cause many lifelong problems, including chronic pain, fatigue and organ damage.
Sickle cell disease struck Shayla at a very early age. At just 3 months old, Shayla had an enlarged spleen, a common complication of the disease, and the pain that accompanies it. Her parents brought her to Cincinnati Children’s – and the dedicated team at the Comprehensive Sickle Cell Center within the Cancer and Blood Diseases Institute – for expert care.
Since that first crisis, Shayla has come often to Cincinnati Children’s for specialized care. The 7th grader has had her tonsils, spleen and gallbladder removed, and she’s had serious lung infections and several bouts of pneumonia. She has needed frequent inpatient care to manage acute chest syndrome, a pneumonia-like inflammation of the lungs common in sickle cell patients. And while ibuprofen sometimes hedges off pain at home, severe pain can warrant a trip to the Emergency Department for morphine.
“When you look at Shayla, you’d have no idea that something so serious was going on,” says Megan Harris, Shayla’s godmother and close family friend. “When she has a crisis, her energy is affected greatly, and she doesn’t feel good. Just saying hello and shaking someone’s hand takes too much effort.”
Early Diagnosis for Shayla
Routine blood screening at birth revealed that Shayla had sickle cell disease. The news came as a shock to Angela, Shayla’s mother. While Angela knew she carried the trait for the disease, it was thought at the time that Shayla’s father did not. When both parents are carriers, there is an increased chance that their children will have the disease.
Around 700 children and adults in the Greater Cincinnati area have sickle cell disease, and one out of 10 African Americans carry the trait, meaning that they do not have the disease but can pass it to their offspring. An estimated 20,000 – 30,000 people in Cincinnati are carriers.
The effects of sickle cell can vary greatly among individuals. Some may have very mild symptoms. But those who have a more severe form of the disease, like Shayla, feel its impact daily.
Sickle Cell Disease Affects the Whole Family
While sickle cell can be very hard on the children who have it, the disease can take a heavy toll on the entire family, too. Because pain can strike suddenly without warning, it can be difficult, if not impossible, to make plans for any given day. Angela often has to miss work to care for Shayla.
“I can’t even count how many crises Shayla has had, how many times she’s been in the hospital,” Angela says. “She’s been hospitalized over almost every holiday, including Christmas, Halloween and Easter. All except four of her birthdays have been celebrated in the hospital.”
As difficult as the situation is for Shayla and her loved ones, Angela says she couldn’t ask for a more kind and high-quality team than the caregivers at Cincinnati Children’s, who have been there for Shayla whenever she’s needed them.
“I love Cincinnati Children’s, and Shayla loves all of her doctors and nurses. She gets attached to them, and they have watched her grow. I’ve been very pleased with the care Shayla has received here,” Angela says.
Prevention and Early Treatment Are Key
While Shayla is encouraged to live her life as normally as possible, she must be careful to avoid the things that can cause her blood to sickle and lead to a crisis. Cold weather and getting chilled from being in water are major culprits.
“The one thing we draw the line with is swimming – it’s just too risky,” Angela says.
Shayla can help to manage her symptoms by drinking plenty of water, which increases the number of red blood cells in her system. Her regular transfusions help minimize sickling in the blood. And because she also has asthma, she comes to Cincinnati Children’s regularly for asthma treatments in addition to home therapy. It’s an important thing for Shayla to get under control, because the wheezing during an asthma attack can decrease the oxygen in her blood and trigger a pain episode.
“The ways we’ve cared for our patients have changed enormously over the years,” says Karen Kalinyak, MD, clinical director of the Comprehensive Sickle Cell Center. “Fortunately, we are now able to prevent complications rather than just treat them when they happen. And an important part of that is early intervention and education.”
Dr. Kalinyak compares the impact that sickle cell disease has on the body to hypertension. “If you have high blood pressure, you’re having hits to your kidneys, heart and lungs that you may not be completely aware of until much later. But if we start treating you early, you have a better outcome.
“The same thing is true of sickle cell disease. The sickling affects virtually every single organ with tiny little hits all the time. If we can prevent those hits and stop the sickling, you can do much better down the road,” Dr. Kalinyak explains.
When a newborn tests positive for sickle cell disease, the center invites the family to the clinic within the baby’s first four months of life to educate them about the disease and the importance of preventing complications. And, to ward off infections, babies are usually put on a regimen of penicillin.
The Promise of Research
There is currently no cure for sickle cell disease. But the researchers and physicians at Cincinnati Children’s continuously look for better ways to treat and prevent symptoms of the disease and improve the quality of life for children like Shayla.
Shayla has been involved in two research studies already, and every year the family looks forward to attending Sickle Cell Disease Family Research Day, a fun and informational event held by Cincinnati Children’s each August.
“We didn’t know a lot at the beginning, but we’re learning more,” Angela says. “And as Shayla is getting older, she’s asking more questions and starting to understand more.”
Dr. Kalinyak says she is pleased to see the excitement and interest that families have about the research being conducted at the medical center.
“The only way we’re going to make major strides is through research,” Dr. Kalinyak says. “I encourage families to seriously consider enrolling in the many studies going on at Cincinnati Children’s. Research is how we understand the disease better and really make progress.”
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