A recently announced sickle cell disease program in Luanda, Angola, is making progress in its objective to establish the Central African nation's first comprehensive initiatives to fight the disease.

The program, established as a public-private partnership between Baylor College of Medicine, Texas Children's Hospital, The Republic of Angola and the Chevron Corporation, which has committed $4 million in funding over four years, has begun the pilot phase to assess the frequency of disease in Luanda, the capitol of Angola and its largest city.

No sickle cell screening program had been established in Angola to date, making it hard to determine exact rates of sickle cell disease in the city and surrounding province.

Assessing needs

Outside of the United States, resources for screening and treatment are scarce. Many children die from the disease without even having a diagnosis, said Dr. Russell E. Ware, who serves as director of the program.

Angola also has one of the worst mortality rates in the world for children under age 5. Ware said a substantial portion of this could be attributed to unrecognized sickle cell disease and its complications.

"To get the program started, we had to first determine the size of the problem," said Ware, also a professor of pediatrics at Baylor College of Medicine and director of the Texas Children's Center for Global Health and director of the Texas Children's Hematology Center. "We predicted there would be a high prevalence of sickle cell disease in Angola, but we found it to be even more common over there than we expected."

Pilot project

To begin the pilot phase, Ware and his team screened babies born in a local maternity center by taking a small blood sample from their heel, only a few hours after birth.

The first baby's sample was collected on July 19, 2011, by a team of BCM healthcare providers who have since trained local providers to test the samples for the presence of sickle cell disease.

"In less than two months, we have already identified a 22 percent rate of sickle cell trait in the babies born during that time, twice the predicted rate, and about 2 percent with sickle cell disease," said Ware.

To put that in perspective, Ware said if you screened about 2,000 babies born in Texas, only one would have sickle cell disease. In Luanda, you would find about 40 with the disease, which is remarkably high.

Data collection

The future hope is that by identifying babies with the disease, the infant mortality rate in the city can be reduced. "Long term, we want to establish a screening program that will enable us to enact and treat these children across the country of Angola."

When the team collected the data, they also collected contact information to ensure they could follow up with the sickle cell disease patients and their families.

Babies affected with sickle cell disease will begin antibiotic treatment and receive special immunizations to prevent life-threatening infections.

Another important element of the program is the teaching aspect, said Ware. "We are teaching health care professionals from the city how to collect the blood, do the screening blood tests, and provide education about sickle cell disease. We hope to expand much more in education as the program progresses."

The program builds on the model of the Baylor College of Medicine International Pediatric AIDS Initiative at Texas Children's Hospital, which treats more than 100,000 children with HIV/AIDS across a network of clinical centers in Romania and Africa.

This pilot phase is a small, yet very important step forward in establishing a comprehensive national program for sickle cell disease in Angola, Ware said. "We have a lot of work to do," said Ware. "Together with our partners we are committed to overcoming this major global health problem in Angola and ultimately across sub-Saharan Africa."