Imagine reading in your morning newspaper that less than 5 percent of children born with sickle cell disease in the United States live past the age of five. Wouldn’t you want to help more children live longer?
Although not a reality in North America, where screening and treatment of sickle cell disease is readily available, this is the disturbing statistic for African children who suffer from the disease, an inherited blood disorder.
“We estimate that 400,000 babies are born each year in Africa with sickle cell disease,” said Kwaku Ohene-Frempong, M.D., director of the Comprehensive Sickle Cell Center at the Children’s Hospital of Philadelphia and an international authority on sickle cell disease research, in an e-mail interview. “No one knows what happens to most of these children. However, it is expected that most of these babies die before three to five years of age.”
Dr. Frempong, a native of the West African country of Ghana, has witnessed the damaging effects of sickle cell disease personally and professionally. He first learned about the disease at a lecture while an undergraduate student at Yale University. After hearing a description of its symptoms, he realized that sickle cell disease caused the death of his cousin back in Ghana. As his interest in the disease grew, he discovered that it also claimed the lives of other family members in his native country. However, it was the birth of his first child, Kwame, while Dr. Frempong was attending Yale Medical School that caused him to turn his interest in sickle cell disease into a lifelong mission.
Kwame was born with sickle cell disease. Although neither Dr. Frempong nor his wife Janet has the disease, each carries the sickle cell trait. When both parents carry the sickle cell trait, there is a 25% chance that their baby will suffer from sickle cell disease and a 50% probability that the baby will not have the disease, but will be a carrier like the parents. Kwame is 30 years old and in good health.
The sickle cell trait arose in parts of Africa, Arabia and India as a defense against malaria. Those who carry the sickle cell trait, but not the disease, tend to survive malaria outbreaks better than non-carriers, which widened the presence of the trait in these tropical regions. Slavery and other migrations led to the spread of the genetic condition to other parts of the world, including North and South America, the Caribbean, the Mediterranean and Western Europe. The sickle cell trait is believed to be present in up to 40% of the population in some parts of West Africa.
A person with sickle cell disease produces abnormal hemoglobin that causes red blood cells to form an abnormal crescent or “sickle” shape instead of the normal round shape. The sickled cells break down rapidly, leading to a shortage of healthy red blood cells and anemia. The cells block small blood vessels, obstructing the flow of blood to major organs, causing severe pain and organ damage. Other complications include strokes and leg ulcers in adults and life-threatening bacterial infections and delayed growth in children. These complications reduce the life expectancies of sickle cell sufferers. Sickling occurs in sporadic, painful episodes, often referred to as sickle cell crises, which are separated by periods of relatively normal health. According to Dr. Frempong, women sufferers describe the pain associated with a sickle cell crisis as greater than that of childbirth.
As opposed to millions of people in other parts of the world, about 72,000 Americans suffer from sickle cell disease. This may explain why sickle cell disease, unlike other acute diseases such as HIV/AIDS, is not closely followed by the media in the United States. In addition, only one in 4,000 American babies, about 1,000 infants annually, is born with the disease. In Ghana, which has less than one-tenth the population of the United States, it is estimated that one in every 50 babies, or 16,000 total, is born with sickle cell disease each year. In the late 1980s, an American baby born with sickle cell disease had a 95% chance of living to age 20, with a median life expectancy of 42 for men and 48 for women. Today’s sickle cell sufferers may live even longer because of advances in medical treatment, although there is still no cure. However, in Africa, where there is a grave lack of early diagnosis, treatment, and education about the disease, they die much earlier.
In an effort to provide African sickle cell sufferers with much-needed services, Dr. Frempong was instrumental in establishing the first newborn-screening program for sickle cell in Africa as part of a Sickle Cell Clinic in Kumasi, Ghana. His reason for getting involved in the project was simple.
“…Studies in the United States had proven the value of newborn screening for sickle cell disease,” said Dr. Frempong. “We wanted to determine whether a newborn-screening program can be implemented in a developing country like Ghana.”
The Sickle Cell Clinic in Kumasi, which opened in December 1992 with 10 patients, currently treats 5,000 people each year. Dr. Frempong’s most memorable patient at the Ghana clinic was actually an adult man. “Both of his legs were supposed to be amputated four years ago because of severe ulcers that had not healed in 25 years. He walks around now with no ulcers after undergoing six weeks of treatment we introduced in Ghana,” said Dr. Frempong.
The Sickle Cell Clinic in Kumasi has reached capacity in the number of patients it can effectively treat. As a result, the clinic currently is seeking $1.5 million for a new building to house an expanded testing and outpatient treatment clinic, a conference center and a library. Dr. Frempong expects that the new facility, to be named the Otumfuo Osei Tutu II Center for Sickle Cell Disease (after the current king of the Asante people of Ghana), will treat 8,000 to 10,000 patients regularly through clinics held five to six days each week and an acute care outpatient unit. The center also hopes to encourage the development of new treatment programs in Africa by hosting gatherings of sickle cell experts throughout the continent and the world.
According to Dr. Frempong, less than $25,000 has been raised for construction of the new center. The Sickle Cell Foundation of Ghana is being established to handle fund-raising efforts for the center and for creating other sickle cell centers in Ghana. The foundation will be able to receive funds from the United States through designated donations made to other non-profit organizations, such as the Children’s Hospital Foundation at the Children’s Hospital of Philadelphia. Palisades Hudson Charitable Portfolio, Inc. (PHCP) has selected the Children’s Hospital Foundation as one of the prospective charities to include in its Science & Health Fund, to direct contributions to the construction of the new facility.You can read more about PHCP at www.palisadeshudson.com and about the Arts & Culture Fund in the 2003-2004 Charitable Giving Program, also available at our Web site.
Once the center is complete, Dr. Frempong does not expect it to cost much to operate each year. The Ghanaian government, which has approved the transfer of land for the new facility, is expected to be a major contributor in the provision of medical services. According to Dr. Frempong, staffing, supplies and other costs should not exceed $250,000 annually. The center will include a hematology lab that will provide testing to the Ghanaian population at large for a fee, which will be used to cover the majority of costs. The newborn screening, however, will be free.
“Our project has been extremely well received by the people in Ghana, especially the parents of young children,” said Dr. Frempong. With your help, the project will be able to expand and help more Ghanaian children suffering from sickle cell disease.
Organization: The Sickle Cell Foundation of Ghana/Children’s Hospital Foundation