Patient stories: Surviving and thriving with sickle cell disease

Sickle cell disease, an inherited blood disorder where normally round red blood cells are instead crescent-shaped (or ‘sickle-shaped’), is most often diagnosed in infancy or early childhood. Symptoms like jaundice (a yellowing of the skin), painful swelling of hands and feet and fussiness or extreme tiredness may appear as early as five or six months old.

Sickle cell disease’s most well-known symptom is its ongoing severe pain events, often called a ‘pain crises’ or ‘vaso-occlusive crisis.’ This pain can be felt really anywhere, including lower legs or hands and feet. It begins in infancy and reoccurs as the crescent-shaped red blood cells build up and become stuck in blood vessels, blocking the flow of blood and oxygen. Children like Kayana Holland spend much of their childhood in hospital settings, being treated for pain, fever, fatigue and more.

“Kayana Holland, she’s a now young adult who we have followed here since she moved to the Columbia area when she was ten years old,” said pediatric hematologist oncologist Carla Roberts, MD. “We’ve really grown up together and seen her blossom into this beautiful young adult. She’s in college for nursing and working while taking a medication to reduce sickling, Hydroxyurea, and she comes into our clinic once a month to get IV medication to help reduce sickling as well.”

One of the biggest challenges that children with sickle cell disease face is growing out of the more readily available options for pediatric care. There are few clinics worldwide that help the transition from childhood to adult care. Those that do offer those like Kayana Holland a safe, familiar space to receive ongoing treatment with a medical team they’ve come to rely on.