Eva was born the year I entered medical school; our life paths would cross two decades later. She went through school, played volleyball regularly, married, and lived a normal and carefree life. Then in her early twenties she gradually began getting breathless and increasingly tired, and had dizzy spells, fainting episodes, palpitations, and chest pain on exertion. She consulted a cardiologist who found that her echocardiogram showed an alarmingly high pulmonary artery pressure without any cardiac lesions to account for it. He referred her to me to rule out other lung conditions: all the usual tests were normal but she was hypoxemic at rest, her blood oxygen dipping further on minimal exertion. She had never used dangerous drugs, and had no other risk factors for vascular disease: this looked like primary pulmonary hypertension, a diagnosis equivalent to terminal cancer in those days, literally a death sentence. Heart-lung transplantation, with its own difficulties and uncertainties, was the only option available, a dishearteningly long shot.
In the course of my training in Britain I had met one of the leading experts in pulmonary vascular disease, and knew that he was conducting clinical trials with experimental drugs in such desperately ill patients. There was nothing else we could do for Eva, so after some hurried correspondence she flew – rather precariously and with supplemental oxygen – to the research center. There a Hickman line was inserted, and she was started on a continuous iloprost infusion, a drug that dilates pulmonary vessels. This promptly improved her breathing and sense of wellbeing and doubled her walking distance. She returned home virtually a new person.
In the years that followed she went through all sorts of problems. Drug tolerance necessitated a gradual increase in the required dose of iloprost, and this meant repeated bureaucratic battles with the state insurance fund, which every so often had to approve the steep cost of this treatment. She developed sweating, tachycardia, tremor, and weight loss from thyrotoxicosis triggered by her medication. The antithyroid drugs gave her neutropenia, and had to be stopped: radioiodine was used to control her thyroid. The Hickman line became infected and had to be replaced. These were just the major complications: there were numerous minor ones.
The turning point came in 2003, when novel therapeutic agents for pulmonary hypertension first appeared. She flew to Britain again and was started on a new regimen. Gradually the iloprost was withdrawn, the central line was removed, and she was controlled with oral bosentan and sildenafil, newer agents to relax the pulmonary vessels. Further changes were required as drugs either gradually failed or gave her side effects. She went under the care of a tertiary center away from my city and I lost contact with her for quite a while. We met again in 2011 when she was admitted to our hospital for some unrelated minor surgery. I was glad to see that she was well controlled and quite happy. She had an uneventful operation, and contact was again lost.
Then years later I happened to attend a case presentation at a local postgraduate meeting: a colleague described a patient with the above familiar history, who eventually passed away while on a transplantation list. “Was it Eva?” I asked the presenter afterwards, knowing the answer. That was seventeen years after the original diagnosis.
Eva happened to live at a time when effective treatments for a lethal disease were developed and introduced into clinical practice with dramatic results. When we first met she had just months to live. However, she fought against all odds, medical complications, and the red tape that conspired to rob her of life-saving treatments. She never gave up, and she won the fight: the death sentence was commuted to a long lease of life. Her story could be described as a chapter in the book of modern medical miracles. This brief note is written as a tribute to her persistence and bravery.
ANTHONY PAPAGIANNIS is a practicing pulmonologist in Thessaloniki, Greece. He graduated from the Aristotle University of Thessaloniki Medical School. He trained in Internal Medicine in Greece and subsequently in the United Kingdom, and specialized in Pulmonary Medicine. He also holds a postgraduate Diploma in Palliative Medicine from the University of Cardiff, Wales, United Kingdom. He is a postgraduate instructor in palliative medicine in the University of Thessaly, Larissa, Greece. He edits the journal of the Thessaloniki Medical Association, and blogs regularly.