the young woman i am thinking of was twenty-two, healthy and full of life and, well, a normal young adult. the reason for her admission was progressive shortness of breath. by the time of her admission, she was in dire straights with her breathing, walking only a few feet before finding herself gasping for air. i don't remember the details of her outpatient work up but her diagnosis was this - severe idiopathic pulmonary hypertension.
the lungs have one job to do - gas exchange. bring oxygen in, move carbon dioxide out. to do this well they must have very low blood pressures. normal systolic blood pressure in your systemic arteries is 120 mmHg. in your lungs, normal pressure is only 10 to 25 mmHg. her pulmonary artery pressures were in the 80's and that is way too high to allow proper gas exchange. the right side of her heart, which normally has the cushier job of pumping blood into the low pressure system of the lungs, was now struggling to push against these tremendous pressures.
what i remember so vividly when i picture her now are her eyes - big and dark, beautiful really. she would sit cross-legged on her hospital bed leaning forward with her elbows resting on the bedside table, barely able to speak a full sentence despite the oxygen she was wearing, her eyes sparkling while the rest of her struggled.
at the time of her admission, there were only three things we could do, give her oxygen, reduce her afterload to take pressure off the right side of her heart (but if you lowered her systemic pressure below her pulmonary pressure she might suddenly die so that was tricky) and give her the only drug we had specifically for pulmonary hypertension - intravenous prostacyclin. the prostaclyclin drip was her lifeline. at one point she started crashing - oxygen saturations plummeting, panic in her eyes, and it was a frantic several moments before we realized her central catheter had become disconnected from the prostacyclin drip, she was that tenuous.
one day her father came in armed with stacks of papers, studies in their infancies about new and novel treatments for his daughter's disease. one of them was a very preliminary report about viagra. he begged us on morning on rounds to give his daughter viagra. at the time there was very little data to support the use of viagra in pulmonary hypertension, but turns out it actually helps and now it is a standard part of managing this disease. i remember my attending repeatedly saying no to his pleas. that poor father got so frustrated with us that he had his daughter transferred to a nearby hospital where she died a few weeks later.
why didn't we give her viagra? would she have responded? maybe she would have held on until a better treatment arrived. i remember my attending trying to explain that with so little data available regarding its use, she could just as easily deteriorate (for her to deteriorate beyond where she already was would mean to die) as improve. at the time it was a complete unknown, a complete gamble. how many times has a drug or therapy looked promising in early trials only to fall apart under more vigorous study? but what i remember most was that i was too young to understand a father's anguish over losing his daughter. and i still feel that way sometimes, too young, too inexperienced, too ill equipped, not for the medical aspects of medicine, but for everything else it demands of you.