For scores of Harvard Medical School physicians and scientists, the study and treatment of deadly infectious diseases around the world is a vocation. Their work embodies the HMS mission to alleviate human suffering caused by disease.
Venée Tubman, an HMS instructor in pediatrics and an attending physician in pediatric hematology/oncology at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, has been working to improve the health of children with sickle cell anemia in Liberia for the past seven years.
As has been widely reported, an Ebola outbreak in Guinea that has spread to Liberia, Sierra Leone and Nigeria, has claimed the lives of approximately 900 people in West Africa since February, prompting global health experts to gather at the World Health Organization headquarters in Geneva last week to determine whether a global health emergency should be declared.
Tubman recently appealed to her American colleagues for help supporting Liberian health care providers.
HMS writer Susan Karcz had an opportunity to sit down with Tubman earlier this summer to learn more about her work implementing sickle cell disease screening in Liberia.
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Venée Tubman was barely a year old when her parents fled Liberia for the suburbs of Washington, DC, to escape the political turmoil of the 1980 coup d’etat in their native land. In her family’s case, staying would have been especially dangerous: Tubman’s grandfather had been president of the country from 1944 until his death in 1971.
After 27 years, Tubman’s work has taken her back to Liberia, where she is changing the lives of children by improving their access to pediatric care and by introducing newborn screening for sickle cell disease, a genetic blood disease that results in an abnormal hemoglobin, known as hemoglobin S.
As a Harvard Medical School instructor in pediatrics and attending physician in pediatric hematology/oncology at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, Tubman is working in Liberia and Boston to serve children’s health and the physicians who safeguard it.
There and back again
Born in the U.S. just before her parents went back to Liberia, Tubman grew up in the Washington, DC, metro area. There, she lived among a strong Liberian community, one that she cherishes as an influence on her decision to become a physician. Family and teachers encouraged her budding interest in science when she was in middle and high school, and from this fertile ground, Tubman bloomed.
Tubman’s early interest in science, combined with her desire for a human connection in her work, made medicine seem like the ideal vocation.
“In college, I studied chemistry and had an opportunity to work with a physician here at Dana-Farber. She had me work on a project that involved developing clinical practice guidelines for sickle cell disease,” said Tubman. “I became completely fascinated with the disease process, with the science of the disease and with the patients themselves.”
During medical school at the University of Pennsylvania, Tubman traveled to Botswana for a clinical rotation at the Princess Marina Hospital, and later to a Navajo Nation family practice center in Gallup, New Mexico. Both experiences led her to think about returning to Liberia. In 2008, during her residency, she did.
“When I first returned to Liberia, there were no fully trained pediatricians working in the entire country,” she said. “A country with more than 2 million children had no fully trained pediatricians to provide their care.”
Although she notes that there are excellent, well-trained, dedicated physicians in Liberia, Tubman saw an immediate need.
“In order to move the care of children to the next level, you really do need somebody who has pediatric training, both to provide the care, and also to train the next generation of pediatricians,” she said.
Planting the seed
Between completing her residency in pediatrics at Boston Children’s Hospital/Boston Medical Center in 2009 and beginning a fellowship in pediatric hematology/oncology in 2010 at Dana-Farber/Boston Children’s, Tubman took a year to hone her primary care skills at Boston Children’s and in Liberia.
It was during that year, she said, that she started to notice how rarely she saw children with sickle cell disease at the John F. Kennedy Medical Center in Monrovia, the capital of Liberia. This struck Tubman as odd because she would have expected to see more children with the disease. In many West and Central African countries, 1 to 2 percent of babies are born with sickle cell disease, and close to 90 percent of those children will die before the age of five. In the U.S., 95 percent of children with sickle cell disease survive well into their 50s.
Tubman learned that newborns in Liberia were not being screened for sickle cell disease, as they are routinely in the U.S. and other countries. This discovery planted the germ of an idea in Tubman’s mind. A former medical school mentor, Kwaku Ohene-Frempong, had launched a successful newborn screening program in Ghana 20 years earlier. Perhaps, Tubman thought, she could do the same in Liberia.
Routine newborn screening is the key to successful management of sickle cell disease. Without it, children with sickle cell disease cannot benefit from early diagnosis and treatment, and frequently die within the first few years of life.
Hemoglobin S can become sickle-, or crescent-, shaped, making it difficult for the blood cells to flow through blood vessels easily. This can block blood flow and cause pain and organ damage, as well as raise the risk of infection. Sickled cells also do not survive as long as cells with normal hemoglobin, which causes anemia. There is no widely available cure for sickle cell disease, but with early interventions and follow-up care, it can be managed.
According to the World Health Organization, sickle cell disease can be managed by simple procedures, including high fluid intake, a healthy diet, folic acid supplementation, pain medication, penicillin prophylaxis and pneumococcal vaccination against infections.
Another important part of treatment involves educating families about when to seek medical care.
Starting up a screening program was not easy, Tubman said, “But with Kwaku’s support, and support from my mentors in Boston, Matthew Heeney and Ellis Neufeld, who established a sickle cell program at the Blood Disorders Center at Dana-Farber/Boston Children’s, I was able to put together a team that could really move this idea forward. The other critical piece was having local mentors on the ground in Liberia.”
From the start, Tubman realized that to build a successful program, she would need help. The American Academy of Pediatrics provided funding for travel to Liberia; HEARTT (Health, Education, and Relief through Teaching) assisted with planning and logistics for physicians traveling to Liberia; the Thrasher Research Fund provided initial funding for the pilot program; and the Association of Public Health Laboratories and PerkinElmer, a medical device corporation, donated newborn screening cards and a screening platform that allows test processing to be done in Liberia instead of sending samples out of the country.
Tending the soil
By the time Tubman’s program started in 2012, she had been traveling to Liberia regularly for four years. She credits her success there only partly to her family connections. Instead, she made it her business to demonstrate a solid and sustained commitment to the patients and to the medical and scientific community in Liberia.
For Tubman, the project is not only about helping children and families, but also about helping to develop the medical profession.
“When you have a medical community that rarely would see sickle cell disease because it wasn’t being diagnosed, there’s reasonably a loss of knowledge about the disease and the management of it. The practical knowledge is not passed along,” she said.
Tubman realized that to create long-lasting improvements, it wouldn’t be enough to simply institute screening and diagnostic procedures. There would also have to be stringent follow-ups and continued treatment for the children, as well as counseling and education for parents and parents-to-be. And, of course, additional training for the medical professionals themselves.
“One of the important pieces of establishing a newborn screening program was that we also had to ensure that once we provided this lifelong diagnosis, that these children had a means to get appropriate medical care. In Liberia, there’s no regular primary care system. So children only go to the hospital when they’re ill,” Tubman said. This would not work for children with sickle cell disease, who need regular growth and nutrition monitoring and supervision of their prophylactic antibiotic use.
Even before Tubman and her colleagues established the newborn screening program, she worked with a group of Liberian physicians and several U.S. physicians to establish a chronic illness clinic so that children could be followed over time.
“The clinic was a place where we could effectively provide primary care for kids with complex medical issues, including sickle cell disease, but also for conditions like asthma, type 1 diabetes and cerebral palsy—all things that you should be seen regularly for,” she said.
Taking root and taking off
There are now more than 500 patients affiliated with the clinic, and the program, known as the Chronic Care Clinic, has grown quickly. An additional newborn clinic at the JFK Medical Center provides close follow-up care for children who require an advanced level of care shortly after birth. There is also a program for teenage mothers that provides them with additional education and support, and the program is now able to provide pretest and post-test counseling with newborn screening.
Tubman is part of a group of U.S.-based pediatricians, the Academic Collaborative to Support Medical Education in Liberia, working with the Graduate Medical Council of Liberia to establish a pediatrics residency program, with the goal of soon having homegrown pediatricians in Liberia. Tubman takes seriously her role in professional education, and makes it standard practice to teach by working side by side with a Liberian colleague whenever she is in Liberia.
“This is certainly not work that is going to be done alone, and it’s not work that can be sustainably done alone,” she said. “We now have a cohort of almost 90 patients with sickle cell disease that we follow, and getting to this point has been a fantastic learning opportunity for several of the Liberian pediatric residents.”
Tubman is encouraged by the very early data from the program. Thus far, no children have died, and the cohort’s first child will turn two in August. When Tubman visited Liberia earlier this year, the number of infants screened reached 4,000, representing another milestone for the screening program. One of the outcomes the study looked at was under-five mortality, a basic indicator of a population’s health status in a country or region.
“There have been estimates that sickle cell is associated with 9 to 16 percent of under-five mortality throughout West Africa,” Tubman said. “And we now know from our study that, at least in Liberia, the incidence of sickle cell disease is about 1.2 percent. So if you consider the proportion of children who are born with sickle cell disease and the proportion of children who are likely to die from complications of the disease, you can see that it represents an important contributor to under-five mortality in Liberia.”
Tubman’s work in Liberia is particularly important from a global public health perspective: The burden of sickle cell disease is expected to increase over the next several decades, as infant and childhood mortality decreases in resource-poor countries and as more people from these countries emigrate to industrialized countries, thus making universal screening tools and intervention models critical.
“The early data we have collected have strengthened our partnership with the Liberian Ministry of Health and Social Welfare and will help us think concretely about how we can broaden the reach of this program and how we can broaden basic education about sickle cell disease,” she said. “But certainly as our program grows, and as our population grows, we will be able to learn so much more about sickle cell disease in general and sickle cell disease in Africa in particular.”
