Monday, December 17, 2018

A Maryland fitness fanatic runs into an alarming ailment that was caught in the nick of time

B.G. went to great lengths to stay out of the doctor's office.

His belief in the power of exercise - particularly running - to keep him fit and healthy had long been an article of faith. If he wasn't feeling well Goldsmith would lace up his shoes and "run it off." The Maryland patent lawyer routinely racked up about 30 miles per week - more when he was training for a marathon or triathlon - interspersed with swimming, cycling and weight training.

For more than 30 years, his strategy worked.

"B. is incredibly fit and he's always been this major athlete to avoid seeing doctors," said his wife, a former teacher. His aversion persisted even after his sister became a family physician.

But several years ago, G., now 56, experienced a series of alarming episodes he couldn't outrun.
They followed a pattern: first a rush of what felt like acid would shoot up G's legs. Then came 10 minutes or so of pounding heart palpitations that ended with a wave of nausea.

At first, the episodes were sporadic. But eventually they increased in frequency and duration, leaving G crumpled on a neighbor's lawn at the start of a run.

The couple were shocked to discover that G's problem was potentially fatal and had been germinating for years, partly camouflaged by his stellar level of fitness.

"Being in good physical health was a blessing and a curse," G. said recently. "The longer I waited, the more I put myself at risk."

Off the charts

G.  first noticed the problem in 2014 at the end of a cross-country flight. Landing in California, he stood up and felt the rush of acid, palpitations and nausea, which subsided fairly quickly.

Maybe, G. remembers thinking, he had been sitting too long. When the episodes recurred every month or so, he found them "concerning," but for months did not mention them to his wife or his internist whom he saw every year or so. In 2015, he developed occasional morning headaches.
G. consulted the cardiologist he had seen as a precaution for a low heart rate, which can result from vigorous exercise, but did not mention the episodes. The doctor said that G. appeared to be healthy.
"He told me, 'I'm fine now, I cleared it,' " his wife recalled, "and kept pushing."

By 2016, the morning headaches were occurring more often. At times G. felt tired, but assumed he was getting older or hadn't slept well.

"We both just kind of ignored it," his wife said. "Nothing intensified and most days he was fine."
But during a 5K race in July 2017, G. felt so weak he had to stop midway and walk to the finish line. The palpitations and nausea were more frequent.

G. called his sister, who suggested he see an endocrinologist. Some of their relatives had thyroid problems; perhaps he did, too.

The endocrinologist he saw in September ordered blood tests, which revealed a slightly sluggish thyroid. During the visit, G. mentioned in passing the running problem, which hadn't recurred, and his morning headaches. The endocrinologist suggested he might want to try thyroid medication and recommended that he return in three months.

By late fall, G. was having more trouble completing a run; his headaches had become a near-daily event.

An ophthalmologist friend suggested that he might be experiencing blood pressure spikes, even though his readings in doctors' offices were normal. That made sense: both his parents had developed mild hypertension in their 60s.

At his friend's suggestion, G. bought a blood pressure cuff. He immediately suspected the device was malfunctioning: the readings at times were off the charts - so high they couldn't possibly be accurate, he thought. One registered 204/118, a reading consistent with a hypertensive crisis, which can require emergency treatment. At other times they were normal, about 125/85.

G. tried using a friend's cuff; the same thing happened.

Despite his wife's increasing insistence that he call his doctor, G. waited a month. He said he wanted to see if the readings stabilized.

"I kept pushing, but he fought all of it," she recalled. "He downplayed everything. That was his style," a characterization with which G. agrees.

In December, G. acceded to his wife's wishes and saw one of his internist's partners. His blood pressure reading in the office was a worrisome 170/87. G. told the doctor, who prescribed medicine to reduce his blood pressure, that he was switching jobs and under a lot of stress. Goldsmith took the medicine faithfully, but the spikes continued.

Things fall apart

In January 2018, G.  returned to the endocrinologist, accompanied by his wife. He mentioned the blood pressure spikes and headaches. The specialist ordered blood tests to check G's hormone levels and convinced him to start taking a low dose of medicine for his underactive thyroid.

As they were leaving the office his wife suggested they stop at the lab downstairs for the tests the endocrinologist had ordered. G. demurred: he hated needles. His internist had recently increased the dose of his blood pressure drug and he said he wanted to see if the new regimen worked before getting the blood tests.

But the increased dose of blood pressure medicine, later augmented by a second drug, made him feel worse. And G. had developed a new symptom: profuse sweating.

Keeping close track of his blood pressure, he had noticed a pattern. Shortly after waking up, when he had a headache, his blood pressure was usually sky-high. An hour later, it returned to normal. At that point, he took his blood pressure medicine, went for a run and took another reading. It was an alarmingly low 83/48, and G. typically felt weak and spent, fearful that he might pass out. But in a few hours, the reading returned to normal.

Doctors told him that calibrating the proper dose of blood pressure medication can be tricky and suggested he change the timing.

In May, things fell apart.

During a run with friends, G. stopped after less than a mile. He then vomited and sank onto a neighbor's lawn, too weak to stand.

His wife arrived minutes later. By then he seemed to have recovered somewhat.

She remembers feeling furious and terrified. "I said, 'This is not working! You're damaging your heart. You need to find a blood pressure specialist,' " she remembered.
G. went online to see what he could learn about the causes of blood pressure spikes. References to a rare, typically benign tumor of the adrenal gland called a pheochromocytoma kept popping up. The symptoms sounded familiar: sudden hypertension or blood pressure spikes, profuse sweating, rapid heart rate, weakness and headaches. These result when the tumor releases uncontrolled bursts of hormones including adrenaline, particularly in response to physical exertion or stress. Adrenal hormones help control many functions including heart rate, metabolism, blood pressure and response to stress.

G.  emailed his internist and asked if it was possible that he had such a tumor, known in medical jargon as a "pheo."

The internist replied that it was and told him to call the endocrinologist.

A week later, he was back in her office. The doctor told him that one of the blood tests she had ordered six months earlier - which he had not gotten - could have helped answer this question. It measured the level of catecholamines - hormones secreted by the adrenal glands, the paired organs atop the kidneys. Elevated levels of these hormones can signal an endocrine tumor.

G's levels turned out to be far above normal.

The endocrinologist sent him for an abdominal CT scan, which revealed an enormous tumor, roughly the dimensions of a small acorn squash, engulfing his right adrenal gland. It was probably a pheo, about 10 percent of which are malignant.

The tumors, which are diagnosed in about 2 of every million adults annually in the United States, are rarely the cause of high blood pressure, accounting for 0.2 percent of hypertension cases.

Pheos often develop for unknown reasons, but in at least 25 percent of cases, genetic factors play a role. Doctors consider the tumors a "time bomb"; they can cause a stroke, kidney failure, heart attack or sudden, unexplained death. Treatment typically involves surgery to remove the tumor, along with the affected adrenal gland.

G's endocrinologist immediately called Washington endocrine surgeon.

An anxious wait

Surgeon  met with the couple two days later.

The size of G's slow-growing tumor - 10 centimeters - made laparoscopic removal a challenge that would require two surgeons, she told the Gs.

"It's clear he's had it for many years," the Dr. said.

Although frightened by the prospect of surgery and the risk of sudden death posed by such a tumor, G. said he was reassured by Dr.'s experience, her straightforward manner and by the fact that she, too, was a runner.

The Dr., who has performed more than 250 such surgeries, ticked off possible complications, which include postoperative heart problems and blood loss requiring a transfusion.

He first needed to take drugs to stabilize his blood pressure, which could shoot up significantly during the operation.

For his wife, the two week pre-op period crawled by, dominated by the specter of her husband's sudden death. Every morning, she said, she awoke wondering whether he was still alive.

"I didn't want to leave his side," she recalled.

Dr. said that the June 29 procedure "went perfectly." He was scheduled to spend two days in the intensive care unit and a total of five days in the hospital, but G. was well enough to be discharged the day after his surgery.

Six weeks later, with Dr.'s approval, G resumed running, although initially at a slower pace and a reduced distance. His blood pressure remains slightly elevated, which is common after surgery. He will require lifelong monitoring to make sure the tumor doesn't recur, which is rarely the case.

In the months before his diagnosis, G said, fear was an obstacle: he was increasingly afraid of what doctors might find.

In retrospect, he wishes he'd acted with greater dispatch. "I could have saved a few years," he said.

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