An ambulance rushed her to the hospital. It was her blood pressure, again. The patient had considered herself pretty healthy, at least until recently. Sure, she had high blood pressure and diabetes, but she never let them slow her down. And then she had a heart attack — almost a month ago — and ever since, nothing had been right.
The patient's husband felt overwhelmed and angered by his inability to help his wife of 36 years. She had yet another doctor's appointment that afternoon — this time with her internist — but she didn't feel well enough to go. She was just too weak. She was scared, and as much as her husband tried to hide it, so was he.
Suddenly, he knew what he had to do. He picked up the phone and called the Fairfield, Conn., office of Dr. Deborah Mayer, his wife's internist. "My wife has an appointment this afternoon, but she's not well enough to come in," he told the receptionist. He wanted to come instead, to talk with the doctor about his wife. They simply couldn't go on this way.
The patient first saw Mayer six years earlier, the husband reminded the internist later that afternoon. He referred to the notes that his wife had kept since then. At that first visit, her blood pressure was a little high, and so was her weight. So the doctor advised her to lose a few pounds and start exercising. She was supposed to come back in a month or two, but life got busy, and she didn't make her way back to Mayer's office for another four years. At that point her blood pressure was really high, and Mayer started her on a blood-pressure medicine. When that wasn't enough, Mayer added a second. Her blood pressure remained high. The doctor fiddled with the medicines, increasing the doses, adding a third. Her blood pressure still remained high. Mayer instructed the patient to buy a blood-pressure cuff to use at home to make certain she didn't have "white-coat hypertension": blood pressure that goes up just because you're at the doctor's office. The home readings were all over the place: sometimes very high, sometimes very low. Her blood pressure was never normal — not in the office, not at home.
Then she had a heart attack. She was in the hospital for four days. They finally sent her home with a bunch of new pills, but she'd been a mess ever since. "Something has to be done," the husband told the doctor. "We can't go on this way."
Mayer reviewed the patient's chart. Her last appointment was a couple of weeks ago, just after she left the hospital following her heart attack. At that time, her blood pressure was a little high when she was sitting, but when she stood up it plummeted.
This kind of position-related drop in blood pressure — a condition known as orthostatic hypotension — is pretty common and is usually linked to dehydration or overmedication. After that last visit, Mayer lowered the medication dosages and advised drinking plenty of fluids.
But the patient sounded as if she was feeling no better and maybe even a little worse.
As the husband went through his wife's story, Mayer paged through her chart. She was shocked to note that there wasn't a single normal blood pressure recorded. Not one. Each visit had triggered yet another effort to control the patient's blood pressure. None of it worked.
Getting high blood pressure under control is a process — it can take weeks, even months. A patient starts on a medicine and then returns in a couple of weeks to see if the dose needs to be increased or another medicine added. It's a crude process of trial and error, but usually it works.
It hadn't worked here.
"I think maybe we are focusing on the wrong thing," Mayer said, pronouncing each word slowly and carefully as she thought through the problem. At each visit the patient had an abnormal blood pressure — usually too high, but not always. And at each visit Mayer had adjusted the medications. She hadn't really stepped back to look at the big picture. Until now. It was clear that this wasn't a case of blood pressure that wasn't being controlled; it was a case of blood pressure that couldn't be controlled, and that was a very different kind of problem.
Usually when blood pressure stays high despite treatment, it's because the patient either isn't taking her medications or isn't taking them correctly. Studies show than nearly half of all patients with high blood pressure don't take their medications as prescribed by their doctors. But Mayer didn't believe that this patient wasn't taking her medicines. She was too sick and too worried not to take them.
Certain blood-pressure medications — most commonly diuretics, which can make the patient dehydrated — can cause orthostatic hypotension. This patient had been on a diuretic, but Mayer stopped it at that last visit, and that hadn't helped.
No, it wasn't going to be one of the usual suspects. What else could cause these episodes of wildly fluctuating blood pressure — hypertension alternating with orthostatic hypotension? Then it struck her. Could this combination of symptoms be caused by too much adrenaline? There are rare tumors — pheochromocytomas — that secrete high levels of adrenaline, and such tumors can cause this combination of high blood pressure and orthostatic hypotension. It's a really rare disease. In a sample of 100,000 patients with high blood pressure, maybe only 5 of them will end up having this kind of tumor as the cause. But if the patient did have a "pheo" (as it's called), that could explain why her symptoms became so much worse after her heart attack. A class of medicines known as beta blockers is regularly used in patients with heart disease, and when taken by a patient with an excess of adrenaline, these drugs can cause erratic fluctuations in heart rate and blood pressure. This patient was on a whopping dose of a beta blocker. Mayer was certain she had a pheo.
She quickly explained her thinking to the husband and ordered an abdominal ultrasound for his wife. A pheo is usually a large tumor, and it would be easy to spot. And indeed, the ultrasoundrevealed a mass the size of a plum on top of her left kidney. A blood test revealed that she had more than 100 times the normal amount of adrenaline in her system. She was referred to a surgeon at Yale for removal of the tumor. That was six months ago. After the operation, the patient's blood pressure was controlled with only one medication. And she feels great.
In medical school, I was often told that if you listen, the patient will tell you what she has. It turns out that sometimes the patient's husband will, too.
Lisa Sanders is the author of "Every Patient Tells a Story: Medical Mysteries and the Art of Diagnosis."
http://www.nytimes.com/2010/12/05/magazine/05FOB-Diagnosis-t.html?ref=magazine
