UI researcher talks of personal experience with sickle cell disease

Azeez Butali, DDS, PhD, a researcher at University of Iowa Carver College of Medicine, knows firsthand what it’s like for parents who have to watch their children struggle with sickle cell disease. His daughter was born with the painful disease, and he and his wife spent years looking for something that would help alleviate her pain.

He also knows there is hope.

Sickle cell disease refers to a group of inherited, sometimes very painful, blood disorders – inherited because the gene causing these disorders is passed from parents to their children. Parents who carry the sickle cell trait may have no symptoms of the disease, but can pass the gene on to their children. 

The disease affects a person’s red blood cells, which carry oxygen throughout the body. Those with sickle cell disease have abnormal red blood cells – regular red blood cells are disc-shaped and flexible, making it easy for them to pass through blood vessels, but sickled red blood cells are stiff and shaped like a crescent, making it more difficult to pass through the vessels and, therefore, more difficult to carry oxygen.

That lack of oxygen can cause episodes of sudden, severe pain, called pain crises. They often occur without warning and require hospitalization for treatment.

Butali's daughter, Amirah, struggled with sickle cell disease before getting a life-changing bone marrow transplant from her 5-year-old brother in 2016. The transplant changed the makeup of Amirah’s red blood cells and she is now a carrier of the gene but is free of the disease.

“When Amirah was 1 she had malaria, and the doctors then determined her red blood cells were sickled,” Butali says. “That was a real turning point in our life.”

Amirah would be hospitalized in Nigeria several times over the next two years, all from complications from sickle cell disease. She was seen by Adekunle Charles, MD, at St Paul Hospital in Lagos in Nigeria who developed a treatment plan. The plan was slightly modified in 2006, when Butali was studying for his doctorate in Scotland, when Amirah was 3-year-old.

The family moved to Iowa in 2010 and used the same plan for Amirah that was working in Scotland, but in 2015 – about the same time Amirah was going through puberty – there were signs the plan was no longer working, and Amirah had more frequent instances of sickle cell crisis. That’s when the family started talking about a bone marrow transplant.

“Science is improving every day,” Butali says. He says he hopes to talk to sickle cell-affected families about treatment options and answer questions they may have.