Improving Sickle Cell Care in the U.S. and Abroad

In a recent interview with the National Initiative for Children’s Healthcare Quality (NICHQ), Kwaku Ohene-Frempong, MD, Doctor Emeritus of CHOP’s Comprehensive Sickle Cell Center, discussed his work with sickle cell disease. Dr. Ohene-Frempong, who has worked for more than thirty years to improve sickle cell disease treatments in the U.S. and abroad, noted that in Africa sickle cell disease is a “a major health problem.”

According to the CDC, sickle cell disease (SCD), a group of inherited blood disorders, affects between 90,000 and 100,000 Americans, most of them African American or Black. SCD is characterized by episodes of pain, chronic anemia, and other complications. While SCD may only affect a small percentage of Americans, millions more worldwide live with the effects of SCD every day.

Originally from Ghana, Dr. Ohene-Frempong has a very personal relationship with SCD: both he and his son carry the disease. He first discovered that he was a carrier of SCD when he was tested after being selected to represent Ghana in the 1968 Olympics in Mexico City. And when Dr. Ohene-Frempong’s son was born, it was discovered that he too carried SCD.

Later, while studying for his medical school thesis in Kumasi, Ghana, Dr. Ohene-Frempong found that most children born with SCD in Ghana likely died undiagnosed. “My mother, knowing then that my son had SCD, advised me to become a doctor for children with SCD,” he said.

Since then, screening for SCD has become a routine part of newborn testing. “Previously, the first three years of life used to have the highest mortality in SCD. Now in the United States, we don’t see many deaths in the first three years, which is a real triumph for newborn screening and the care that follows.”

Though great strides in the fight against SCD have been made in the U.S., the disorder remains “a completely different public health problem in Africa,” Dr. Ohene-Frempong said. Citing the World Health Organization, he pointed out that SCD “contributes somewhere between 9 to 16 percent of deaths for children under the age of five,” in Africa.

“In the United States, there was already newborn screening going on when SCD was added,” Dr. Ohene-Frempong said. “But in Africa, national newborn screening programs do not exist for any diseases so it requires introducing a complete new public health service, which takes a lot more capacity-building both in technology and in human resources.”

To read the full interview with Dr. Ohene-Frempong, see the NICHQ’s website. To learn more about CHOP’s Sickle Cell Clinical Center and research, see the Center’s site.