Bailey lives active life with sickle cell disease

Bailey is a quintessential 7-year-old girl. She’s vivacious and energetic, an active member of her Cub Scouts troop. She likes to paint her nails and try on frilly dresses. Around her house, Bailey likes to style her hair like her mother’s and click-clacks through the halls in her mother’s heels, which are too big for her, but that’s not the point. She wants to be just like her mother.

For a few moments every day, Bailey does something that sets her apart from most 7-year-olds. She takes a pill to treat sickle cell disease, a debilitating condition she was diagnosed with a week after she was born. When she takes it, Bailey hears her mother’s words.

You are resilient. You are strong. Stay mindful and positive.

 When Bailey’s parents Monica and Tony first received their daughter’s sickle cell diagnosis, they were wracked with worry because everything they knew about the disease, a disease that affects approximately 100,000 Americans every year,  was not good.

They knew they carried the trait for the disease. They also knew how sickle cell disease affects red blood cells, causing them to warp and become rigid. And how this distortion hinders those cells’ ability to move through the body and supply major organs with oxygen, leading to a host of potential lifelong health complications, including organ damage, stroke, frequent infections and pain crises. Even now, with advancements in modern medicine and screenings, the life expectancy of a person with sickle cell disease is typically 20 to 30 years shorter than those without the disease.

“We did a lot of crying, a lot of praying. Just a lot of seeking advice from other people, but it was hard news to receive,” Tony said, recalling the early days of his daughter’s diagnosis.

But then their pediatrician referred Bailey to St. Jude Children’s Research Hospital®, where Monica and Tony say they not only found solutions for treatment, but hope as well. 

They learned that St. Jude has been researching and improving standards of care for people with sickle cell disease for more than 60 years. They learned how St. Jude has one of the largest pediatric sickle cell programs in the country, serving more than 900 children.  After talking with doctors, nurses and other patient families, they learned St. Jude has helped lead major advances in sickle cell disease treatment, while exploring new curative therapies for the disease.

All that training and knowledge has helped Bailey avoid the serious infections and pain crises commonly associated with sickle cell disease thanks to the daily medicine she takes, Tony said.

Doctors at St. Jude were pioneers in the use of the medicine Bailey takes, hydroxyurea, for children with sickle cell disease. Initially developed in the 1960s as a possible anti-cancer agent, hydroxyurea has since been shown to reduce episodes of severe pain and improve patient quality of life by increasing production of fetal hemoglobin, a form of the oxygen-carrying protein that is unaffected by the mutations that cause sickle cell disease and reducing the sickling of red blood cells. 

The drug had been underutilized as treatment for sickle cell anemia in children until a groundbreaking study published by St. Jude physician, the late Winfred Wang, MD, in 2013. Earlier studies had demonstrated that adults and adolescents with the disease benefited from hydroxyurea. But research led by Wang showed the medicine, which is inexpensive and easy to administer, was safe and effective for young children, sharply reducing episodes of acute pain and pneumonia-like illness, decreasing the need for blood transfusions and cutting hospitalizations for infants and toddlers with sickle cell anemia. 

“St. Jude took care of (Bailey), not just with medicine,” said Bailey’s mother, Monica. “They’ve also been able to instill in her what her disease is so that she knows, and can let others know, if something is going wrong. She’s able to recognize when she’s not feeling well and identify triggers.”

For instance, Bailey knows she has to hydrate well and be mindful of sharp temperature transitions; she can’t get too hot or too cold too fast or it could trigger a pain crisis. Continuity of care is important for patients with sickle cell disease. Therefore, in addition to returning to St. Jude regularly for checkups until she turns 18, Bailey will also go through a transition program that will help prepare her for adult care, pair her with a physician at a local clinic, and teach her to identify the start of an acute pain crisis.  

“Most people think St. Jude is a cancer hospital, and that’s what we thought, too, before bringing Bailey here,” Monica said. “But they treat all kinds of diseases, including blood disorders. Until you really see it and are immersed in the St. Jude world, you don’t know the extent of all they do.”