Abstract
When Jan Travioli was 31 years old, she had a good job with Bank of America, close friends, and had recently put a down payment on a condo. She had been noticing she felt short of breath when exercising, but assumed she was out of shape. Then one day while taking a walk, she felt like her lungs were going to explode. “It just hit me overnight,” she recalled. She consulted with her regular doctor, who “just kind of pacified me” and suggested “it was all in my head.” Travioli insisted the problem was real, so he referred her to a cardiologist, who “diagnosed me pretty quick” with primary pulmonary hypertension (PPH), which is high blood pressure in the lungs that eventually builds up so much it causes heart failure. A rare disease, it was not surprising that Travioli and her family knew nothing about it and were “in shock” when they realized its severity. She was lucky in a couple of ways, though, to have a doctor that recognized the rare condition and to get diagnosed at a time when a new medication called Flolan had recently become available. “It kept me alive,” she reported. “If I was without the drug for five minutes, I could tell.” Flolan worked well for her for about a year, but then the disease resumed its deadly path. Soon doing her job became difficult since she was always short of breath; her supervisors let her work from home for a while, but eventually that became impossible and she had to go on disability. She became unable to make the payments on her condo, and her mother moved from another state to help buy the condo and care for Travioli.1
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Notes
Case history included in Martin Bodian, Fibrocystic Disease of the Pancreas (New York: Grune and Stratton, 1953), 167.
Various researchers around the world were observing the symptoms of the disease, so it is difficult to know who to credit for its “discovery.” Charles May calls the phenomenon “a revealing example of the gradual accumulation of observations from a variety of independent sources which may be required to attain a clear conception of a disease.” Charles D. May, Cystic Fibrosis of the Pancreas in Infants and Children (Springfield, IL: Charles C. Thomas, 1954), 6–9;
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Burton L. Shapiro and Ralph C. Heussner, A Parent’s Guide to Cystic Fibrosis (Minneapolis: University of Minnesota Press, 1991), 17.
Keith Wailoo and Stephen Pemberton observed that although at certain moments in UnitedStates history (when it was useful to get research funding) its pan-ethnic nature was emphasized, CF was frequently described as “the most common fatal inherited disease … among Caucasians.” The disease has a high prevalence in Europe and is less common in Asian Americans, African Americans, and Native Americans than in European Americans. There is greater incidence in Denmark than in Finland and more in parts of Central America than in others. Despite this complexity, some have portrayed it as a particularly “European” disease or treated it as a proxy for European identity. Keith Wailoo and Stephen Pemberton, The Troubled Dream of Genetic Medicine; Ethnicity and Innovation in Tay-Sachs, Cystic Fibrosis, and Sickle Cell Disease (Baltimore: The Johns Hopkins Press, 2006), 62–67, 110;
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© 2012 Mary Jo Festle
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Festle, M.J. (2012). End-stage Lung Disease. In: Second Wind. PALGRAVE Studies in Oral History. Palgrave Macmillan, New York. https://doi.org/10.1057/9781137011503_2
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