Teaming with our twin in India, to take on hemophilia
May 20, 2013
The Boston Hemophilia Center (BHC) at Boston Children's Hospital is entering into a new international twining partnership with the Hematology program at Sawai ManSingh (SMS) Medical College and Hospital, University of Rajasthan, located in Jaipur, India. Kapil Saxena, MD, MS, associate director of BHC, and Laura Gray, LICSW, BHC projects director, are the driving forces behind this partnership - the latest in a string of twinning partnerships that includes a 2011 partnership with the National Pediatric Hospital in Phnom Penh, Vietnam, focused on establishing a sister Pediatric Intensive Care Unit (PICU).
Twinning partnerships match developed treatment centers with burgeoning centers throughout the developing world in order to exchange knowledge and build local expertise. The hospital's partnership with the Jaipur center, the result of a yearlong application process and an assessment visit, is supported by a competitive grant from the World Federation of Hemophilia (WFH) awarded for four years, starting in January 2013.
Home to a quarter of the world's hemophilia cases, India is a prime target for a hemophilia twinning partnership. SMS, which serves as the only hemophiliac clinic in its home state of Rajasthan, is plagued by shortages in human resources, knowledge, and technology. "They don't have an organized comprehensive hemophilia treatment center (HTC) and lack the laboratory diagnostic capabilities," says Saxena.
Over the course of the four-year partnership, Saxena and Gray hope to turn this around. At the top of their agenda is enhancing the college's diagnostic capabilities. Given the population size and rate of incidence, they believe there should be as many as 7,000 cases of hemophilia in the state of Rajasthan; yet only 400 cases are known currently.
In part because the disorder is so rare, myths surround its symptoms and treatment. For instance, many believe hemophilia can be outgrown or that severe cases can become mild over time when, in fact, it is a lifetime condition. And while it is true that hemophiliacs will bleed longer than normal people, they are in no more danger of dying from a paper cut than people who do not suffer from the disorder.
In reality, hemophilia can be mild, moderate or severe. Patients with mild or moderate hemophilia have excessive bleeding with trauma. Patients with severe hemophilia can have spontaneous bleeds without trauma, including joint bleeds, which is the hallmark of the disease and leads to chronic joint damage.
In the United States and other developed countries, hemophilia is treated through Prophylaxis, a regular replacement therapy for missing blood-clotting proteins known as Factors. This involves intravenous injection of Factor VIII or IX, depending on the deficiency, into the blood stream. Because the lack of an organized HTC infrastructure and trained personnel prevents SMS from implementing the same treatment regimens, the partnership will focus on the education and training of these personnel.
The first year of the twinning partnership will be devoted to training SMS' hematologist and lab pathologist at Boston Children's, with the goal of improving the lab quality in order to enhance the college's diagnostic capabilities. "Without accurate and rapid diagnostic lab capabilities, the care of hemophilia patients is difficult, the diagnosis of inhibitors is delayed, and any surgery is almost impossible," Saxena says.
Down the road, the two will provide guidance on establishing hemophilia infusion centers, organize educational camps for local healthcare providers, and work with government officials to raise awareness of hemophilia in and around Jaipur. For Gray and Saxena, the priorities are establishing a dedicated HTC in Jaipur and a fully functioning coagulation lab. According to Gray, "If we could do just that, we would have accomplished an enormous amount."