Zion, 10, is a fun, outgoing kid. He and his 3-year-old sister, Zuri, both have sickle cell disease and have been Children's Hospital of Philadelphia (CHOP) patients since birth.
Sickle cell disease affects so many aspects of a child’s life. Zion loves playing football and swimming, but he has to be careful not to over-exert himself or experience sudden temperature changes, as it can bring on a severe sickle cell pain episode in his hips, legs or arms. Last year, Zion lost hearing in his right ear as a result of the disease.
A little girl with a big personality, Zuri loves going to church on Sunday with her family, playing with her dolls, and spending time with her cousin Jada. Zuri has had a lot of health problems in her short life, including breathing issues after birth, an enlarged spleen at age 2, and pain crises. She and her brother have both been hospitalized for acute chest syndrome, a life-threatening complication of sickle cell disease.
Children with sickle cell disease can experience excruciating pain and repeated visits to the Emergency Department. They have a high risk of stroke and an average life expectancy of only 50 years. Currently the only cure for this chronic, inherited disease is a bone marrow transplantation, which is so limited that relatively few procedures are completed. At CHOP, our researchers are on the cusp of a history-making breakthrough: a curative gene therapy that can bring healthy bone marrow into the blood cells. Donor support will bring us one step closer to making this innovative research a reality.
Caring for these vulnerable children
CHOP’s large and comprehensive sickle cell disease program offers all the specialty services children with sickle cell disease need. These services include newborn screenings, transfusions, 24-hour access to a hematologist, pulmonary and cerebrovascular care, psychosocial services, and an acute care unit.
When Zion experiences painful episodes, he often suffers in silence until he can’t endure it anymore, says his mom, Shirldaya, because he doesn’t want to miss school and hanging out with his friends. “He’s very determined,” says Shirldaya. “He doesn’t let anything stand in his way.”
But sickle cell disease is a formidable foe. “Even a little fever can go from small to serious,” says Shirldaya. “It can be life or death. So I’m always fast to react. Whenever the kids say something’s wrong, I have to think it could be the worst-case scenario, because you never know.”
Shirldaya is excited for the possibility of a treatment breakthrough that could help her kids live long, pain-free lives.