Andrew J. He wasn’t surprised when Rosen was diagnosed with high blood pressure at the age of 39. Both his parents had taken medication for years, which effectively controlled the condition that affects about half of all American adults and often runs in families.
Rosen said he repeatedly asked his doctors if an undiagnosed condition could be to blame. Each time, they got the same response: They had “essential hypertension”—high blood pressure with no underlying cause.
Sometimes, doctors told them, this condition, also known as primary hypertension, can be difficult to control. Poorly controlled high blood pressure increases the risk of heart disease, stroke, irreversible kidney damage and premature death.
More than a decade would pass before Rosen learned that he was asking the right question but was given the wrong answer. His persistent high blood pressure had an underlying, treatable cause.
“He was on a bucket load of medicine,” which was the first clue, noted William F. Young Jr., a Mayo Clinic specialist Rosen consulted in 2019. Young said Rosen’s case is “extremely typical” of patients whose delay in diagnosis can lead to prevention. injury.
For a number of reasons, said Young, a professor of medicine at Mayo, “clinicians often don’t think about what might be” resistant hypertension — high blood pressure that isn’t controlled by three or more drugs. This is a mistake he is trying to correct.
“To be honest,” Young said, “it’s one of the simplest things to diagnose.”
Now 60, Rosen, senior vice president of development for a company that makes rehabilitation hospitals, suspects she developed high blood pressure in her late 20s, though there’s no way of knowing. He hated having his blood pressure measured, so readings were limited to periodic medical visits.
Those measurements routinely exceeded 140/90 mmHg, the cutoff that then indicated high blood pressure. (It has since been reduced to 130/80.) Rosen said that he avoided that diagnosis by assuring doctors of “white-coat syndrome,” also known as “white-coat hypertension.” Pointed out – readings that are high in a medical setting but otherwise normal. Because of his youth, doctors generally agreed.
In fact, Rosen didn’t know if this was true; He strictly avoided taking his blood pressure at home. “It was worrying,” he said. “It was always high.” He hated to feel the cuff being squeezed out of his hand and preferred to assume he didn’t have high blood pressure.
Not all doctors were persuaded. His allergist in Atlanta, where Rosen was then living, expressed doubts about the white-coat explanation. “He said, ‘I don’t know. You’re too young to have high blood pressure,'” Rosen recalled.
In 2001, after her internist diagnosed high blood pressure, Rosen began taking a beta blocker, a drug that blocks adrenaline. When his blood pressure didn’t drop, his internist added two other classes of drugs: a calcium channel blocker and an ACE inhibitor. When that trio proved ineffective, the doctor increased the dosage.
In his mid-40s, Rosen was told he had high levels of cholesterol and type 2 diabetes, a chronic condition in which processing of sugar is impaired. Cholesterol-lowering and diabetes medications controlled those problems.
In 2011, after Rosen moved to the San Diego area, his new primary care doctor changed his blood pressure medications. When this resulted in only a minimal reduction, the doctor added two more drugs.
But the advanced readings remained. “It would be 148/90 when the nurse measured it on the machine several times,” Rosen said. At the end of a visit, Rosen’s doctor will do a quick manual reading and announce that it has dropped to 118/69.
Rosen, whose sister is a physician, was relieved. “I assumed he was a really good doctor,” he said. “And I liked his answer.”
But over the next several years, he became more and more uncomfortable. Even though she took her medication faithfully, the high readings were so high that she couldn’t believe her blood pressure was under control.
In 2017, after cardiac bypass surgery for blocked coronary arteries from both his parents, Rosen consulted a cardiologist.
The heart specialist ordered a stress echocardiogram, a test that measures how well the heart is working. This showed that Rosen’s heart seemed normal, but cardiologists were concerned about his blood pressure, which was sometimes as high as 179/85, and increased the amount of a drug that Rosen was not taking at the maximum dose. . He suggested that Rosen’s high blood pressure may be the result of a kidney problem, but a kidney scan found nothing.
At this point, Rosen tells his family doctor that he wants to start looking for a “zebra,” which doctors use for rare diagnoses. The doctor ordered tests for pheochromocytoma, a rare, usually benign tumor that arises in one or both of the adrenal glands, which sit on top of the kidneys.
After tests didn’t reveal “Feo,” Rosen was referred to an endocrinologist, a physician who specializes in treating hormone-related diseases.
‘You don’t have’
Rosen first saw an endocrinologist in November 2018. She suspected she might have a disorder most commonly associated with obstructive hypertension — primary aldosteronism (PA), which was described by University of Michigan endocrinologist Jerome W. Also known as Conn or Conn syndrome after Conn, who discovered it. 1954.
PA is caused by an excess of aldosterone, a hormone produced by the adrenal glands. Excess aldosterone can cause the kidneys to retain sodium and lose potassium, which raises blood pressure.
The disease can be diagnosed with a blood test that measures the levels of aldosterone and renin, an enzyme made in the kidneys that help control blood pressure, then calculates the ratio between the two. Further testing is needed to confirm the diagnosis and determine whether one or both adrenal glands are affected. In the latter case, PA is treated with medication. But in 30 percent of cases, PA is caused by a benign tumor on a gland. Surgically removing that gland can normalize blood pressure.
While awaiting the results of her blood tests, Rosen heeded the clinical guidelines for the diagnosis and treatment of PA issued in 2016 by the Endocrine Society, the international medical organization of endocrinologists.
They found that sleep apnea and low potassium levels, which they both had, are associated with the disease. And he was delighted when his blood tests pointed to it.
“To me, it actually seemed like a good thing, because it’s treatable,” Rosen said.
But the junior doctor working with his endocrinologist ruled out the possibility. “You don’t have to. Your Aldo[sterone] too little,” Rosen is being told. Rosen said he protested that his understanding of the 50-page Endocrine Society guidelines, along with calculating the blood test ratio, suggested otherwise.
The partner disagreed. Rosen said he ended the call and immediately began looking for a new specialist.
Soon after, Rosen saw a second endocrinologist, who agreed that PA was likely. She ordered additional testing, along with a CT scan, which provided confirmation. The final step was a technically difficult procedure known as adrenal venous sampling, which involves inserting a catheter into the adrenal veins to determine whether one or both are affected. That discovery will guide treatment.
After consulting with Young, another endocrinologist advised Rosen to undergo a vein sampling procedure at Mayo. In April 2019, Rosen and her sister flew to Minnesota to meet with Young, the clinic’s former president of endocrinology and a past president of the Endocrine Society.
An interventional radiologist specializing in the vein sampling procedure found that only one adrenal gland was involved, which meant that Rosen was a candidate for surgery. (“You only need half of an adrenal gland to be completely normal,” Young said.)
In June 2019, Rosen underwent laparoscopic surgery at UCLA Medical Center. The following year, he lost 35 pounds and his health improved dramatically. She now only needs a low dose of a blood pressure medication to achieve a reading of 124/80.
He said, ‘I feel much better now than before. “And I’m a lot more active.”
But the delayed diagnosis inflicted an irreversible toll. Years of uncontrolled high blood pressure led to stage 3B kidney disease, for which Rosen takes medications and visits a kidney specialist regularly. If his disease worsens, Rosen is told that he may need a kidney transplant.
Young said he sees patients like Rosen very often, a primary reason he has become a campaigner for PA screening.
Although doctors have long been taught that the disease is rare, Young said recent studies show it is not. Researchers estimate that 5 to 10 percent of people with high blood pressure and 20 percent of people with resistant hypertension have PA. Most do not know this because they have never been tested.
A 2020 Stanford study found that only 2.1 percent of patients with resistant hypertension were screened for PA; At the University of Minnesota, the figure was 4.2 percent. And a 2003 study from Australia found an unexpectedly large number of PA cases in a group of patients with hypertension.
“It’s underdiagnosed, not just in the US,” said Young, who advocates for everyone with high blood pressure to be screened at least once. “From my perspective, it comes down to physician awareness. Endocrinologists and nephrologists think about this. Primary care doctors, not so much.”
People with PA are more likely to develop heart and kidney disease and have a poorer quality of life, Young noted in a 2018 article. Timely treatment can minimize those consequences.
Rosen said he too sought to educate his doctors. “I’ve tried to tell every doctor I go to if you have three or more patients” [blood pressure] meds and they’re not well controlled, you need to test them” for PAs, he said, echoing the Endocrine Society’s guidelines.
After his surgery, Rosen said he “sent nice letters to all my old doctors saying, ‘You missed it.’ The only doctor she heard was the first endocrinologist, who asked for an apology, saying the fellow had misinterpreted Rosen’s tests.
Rosen said he hopes his experience will spare others. “Every day I wish I had done more research,” he said. “If I had done it 20 years ago, I wouldn’t have had kidney failure.”