Patients and Physicians Seek More Awareness for Sickle Cell Disease

May 21, 2018

Lena Hill and Azeez Butali are both professors at the University of Iowa. They have something else in common: they are both parents to children with sickle cell disease.

Sickle cell disease is an inherited blood disorder. It can cause chronic pain, multi-organ failure, and stroke, and affects an estimated 100,000 people in the United States, most of them African American. 

For Hill and her husband, and Butali and his wife, the sickle cell diagnosis of their respective children came as a surprise. Hill knew she was a carrier, but her husband did not. And Butali, whose mother lost seven siblings to sickle cell disease, knew he was a carrier. His wife was screened, but was incorrectly identified as a non-carrier.

While pursuing his PhD in Scotland, he and his wife made sure they had the medical resources they needed to manage their daughter's disease. People with sickle cell disease face frequent hospitalizations as a result of their symptoms.

"The first thing I did there was access the hematology team to let them know my daughter had sickle cell," Butali says.

Hill was similarly proactive for her daughter, Caitlyn. Before accepting jobs at the university, she and her husband met with the hematology and pediatrics departments at the University of Iowa Hospitals and Clinics.

When Caitlyn's strokes would not subside with blood transfusions at UIHC, the Hill family temporarily moved to St. Louis so Caitlyn could receive a cord blood transplant.

"Caitlyn wrote a wonderful story as she was there because she wanted to think about what was going on at the hospital at night," Hill says. "She always reminds me that the other two characters in the story - there were three characters - were other kids on the transplant unit. [...] Neither of those children are alive today. She often will remind me that she's the only one in her story that's still with us."

Dr. Anjali Sharathkumar, clinical director of pediatric hematology and associate professor of pediatrics at the University of Iowa, says that, though there is no cure, advances in treatment have prolonged life expectancy for people with sickle cell disease.

"Now, we are seeing patients with sickle cell disease in their mid 50s, and this is a great achievement," Dr. Sharathkumar says. 

Still, 30-years-old is generally considered the life expectancy for those with sickle cell disease, and the disease's symptoms can be overwhelming.

The pain crises that Butali's daughter, Amirah, experienced and dwindling treatment options led her to opt for a bone marrow transplant. The 14-year-old admits she was afraid to die, as well as worried about the procedure and its side-effects. 

"I didn't want to say it out loud because I'm known to be a fighter," Amirah says.

Her younger brother was her donor match.

"My baby brother - he's six now," Amirah says, "and he still tells me, 'Remember when I saved your life?'"

Though both Caitlyn and Amirah have been cured of their symptoms through transplants, sickle cell disease remains misunderstood. Hill believes that the reason sickle cell disease does not receive as much attention is because of the population the disease affects.

"Unfortunately, many African Americans, people of color, who suffer from sickle cell disease are not those who have the megaphone," Hill says. "They don't know people who have power and resources to shine a light on this disease."