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A triumph of the human spirit: Youth don't let sickle cell stop them from living

sicklecell

People play a game involving trivia about sickle cell disease at an event hosted by the ECU Pediatric Comprehensive Sickle Cell Program to mark World Sickle Cell Day on June 19.

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Sharieka Botex

Saturday, June 22, 2019

There was a time in Carla Pastor’s life when she thought sickle cell disease was a death sentence. Now that her daughter is 12, she’s happy to report she was wrong.

Pastor was among many family, friends and caregivers of people affected by the disease who gathered at the “Shine the Light on Sickle Cell Disease” event at East Carolina University.

The event held on World Sickle Cell Day, observed annually on June 19, raises awareness about the condition and celebrates the strength and ability of those affected by it to overcome its challenges daily.

“I thought it was like a death sentence, honestly,” said Pastor, who grew up in the Caribbean, where diagnosis and treatment was substandard. “So I didn’t know a lot of people with sickle cell ... Those who had sickle cell didn’t live very long, and so to me, that was my concern.”

After the birth of Carlie Nunez, she prayed, researched and learned about the disease with help of caregivers. She decided she was not going to limit Carlie. Her daughter would live life to the fullest, Pastor said.

“How she understands her condition will depend on how I treat her, so I never restrict Carlie in anything. Carlie started dancing at 5, even though … for five years of her life she had to go for transfusions every three weeks … Every three weeks she’d go for a blood transfusion, and she did that for five years, and Carlie never missed a dance rehearsal, recital. She played sports, she did everything else. We’d do the full day at school, and then we would get the transfusion, and we’d go back home, and she would be back at school the next day.”

That’s the spirit those gathered at ECU’s medical campus student center praised on Wednesday with awards and uplifting words at the event hosted by the Brody School of Medicine Department of Pediatric Hematology/Oncology, the ECU Sickle Cell Committee, the North Carolina Sickle Cell Syndrome Program, Piedmont Health Services and Sickle Cell Agency and other partners.

“We want to take time today to acknowledge our patients and their accomplishments,” Calvin Bradley, a chaplain at the James and Connie Maynard Children’s Hospital, said during the event. “Each and every day you go out into your lives, and you live your lives, and you play sports, you excel in academics. You do a lot of great things, and that sometimes doesn’t get put on the front page of sickle cell, and so we really want to take time today to really highlight each of you, and to really celebrate you and your accomplishments, and all that you’re out there doing and all the great things that you’re achieving.”

Blood disorder

Sickle cell disease is an inherited blood disorder found primarily in people of African descent. According to the experts at ECU, it causes red blood cells — which are usually disc-shaped — to become hard and crescent-shaped, which slows and blocks the flow oxygen throughout the body. People living with the disease can experience frequent infections, blood clots and even organ damage. Most suffer from episodes of intense pain that require hospitalization.

About 100,000 people in the United States suffer from the disease. According to the Centers for Disease Control, 1 in 365 African American children are diagnosed with it, and 1 in 13 carry the trait.

The ECU Comprehensive Sickle Cell program serves more than 900 people with sickle cell from approximately 30 counties in eastern North Carolina, making it one of the largest sickle cell disease programs in the United States.

Nunez, who attends St. Peter Catholic School in Greenville, excels despite the condition. On Wednesday, organizers recognized her for maintaining As and Bs throughout the school year, placing first in a Daughters of the American Revolution essay contest, baking cookies for troops overseas, ballet recitals and playing on a first-place basketball team.

“Having sickle cell can be tough sometimes,” she said. “Even for me, because you feel like you can do anything, but there’s like certain guidelines. ... You have to hydrate … a decent amount of times. If you’re tired you have to slow down, and stuff like that.”

‘You can’t go too hard’

For Nunez, Wednesday’s event also was an opportunity to celebrate others with the disease, like Heaven Glaspie, a 17-year-old who recently graduated from Southern Wayne High School.

Heaven said he has experienced hospital stays and time out of school due to the condition. He also has excelled academically and was part of his high school football and track teams.

“To be honest, whenever I’m playing sports, everything just goes away,” Heaven said. “I love playing football; I play travel basketball. Whenever I’m running and stuff, it just takes everything away. … But you can’t go too hard. You got to know your limits because if you push it hard, you’ll be hurting later that night, or you’ll have a pain crisis coming sooner or later, and it’s just wearing down your body when your body can’t actually take it.”

Hospital stays are a fact of life, he said. “Whenever you get admitted to the hospital, it’s like a lot on you, especially when it’s during school, it’s like you don’t know how long you’ll miss school,” he said.

Although Heaven said some teachers were mindful about his condition, some teachers were less likely to work with him on make-up assignments, forcing him to learn discipline.

“Don’t procrastinate because you never know what can happen to you,” Heaven said. “You can just wake up one morning and you’re hurting, and wow you can’t go to school, and you might have a big project you gotta turn in, but now you can’t do it ‘cause you got a sickle crisis. So don’t procrastinate. Go ahead and get your work done and then you have time to play and have fun later.”

Heaven, who will be attending ECU in the fall, is focused, ambitious and determined to achieve his goals, and will not let his condition stop him. He suggests that people treat those living with the condition with love and care. He knows the benefits of such treatment.

“A big support system, is my mom, my parents and my grandparents,” he said. “They are really, like there for me, anything I need … I really appreciate all my family members for being there for me and supporting me through my whole journey, ‘cause sometimes, deep down inside, it really hurts and it takes a long time.”

Ability to overcome

Charmaine Bond, a social worker with ECU Pediatric Comprehensive Sickle Cell Clinic, praised her patients’ “amazing” ability to overcome “the unimaginable.” She works to raise awareness about the disease and services available here.

“We try to link them to financial resources, because their parents may have a loss of income due to hospitalization,” Bond said. “The financial support is very limited for this population. We also service pediatric oncology. There are plenty of resources available when that parent with a child with cancer misses work, but with our sickle cell population the resources are … far and few between.”

Dr. Beng Fuh, director of the Sickle Cell Center and associate professor of pediatrics, said highlighting the achievements of those living with sickle cell shows the public that they are very active participants in the community. “We hope it’s inspiration for the community to see the humanity in them and see the value in them and support them in their growth,” he said.

Fuh said the the condition causes pain like lying on a bed of needles and nails and being on fire.

“That is pain some have to deal with every day of their lives,” Fuh said. “Some people have this type of pain every day; they still need to go to work, take care of their children, sing, dance, do what everybody else is doing.”

Patients with sickle cell also can experience complications such as being more prone to infections, Fuh said.

“When they get a fever or otherwise sick, we need to treat them more aggressively,” Fuh said. “One in ten will have a stroke by the age of 25 if left untreated.”

Fuh works with patients to deal with symptoms like jaundice, which can cause the yellowing of eyes, he said. He helps patients build their self-esteem, “so they aren’t bothered by what people are thinking,” he said.

Newborn screening in the U.S. catches the disease early, Fuh said. The results of the screening are sent to the child’s pediatrician, who works to educate families.

Ongoing treatment

Treatment continues throughout their life, Fuh said. Patients are started on Penicillin immediately, and they are monitored for the risk of stroke.

“The third thing we do, is make sure they get their vaccines to protect them from bad infections,” he said.

A drug named Hydroxyurea, which was previously used to treat cancer, is used for patients with and can reduce their complications, Fuh said.

Along with Penicillin and Hydroxyurea, Fuh said Endari is another drug that is used to help with the reduction of complications. Preventative medications cost less than admitting patients for complications, he sad.

Pastor said she wishes that more minorities would donate bone marrow. As of now, bone marrow transplantation is the only cure, but Fuh expressed optimism about another potential way to cure the disease.

“We hope that in the near future we will be able to cure a lot more people with gene therapy,” Fuh said.

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