Through the haze of exhaustion and elation that often characterizes the first disorienting weeks of motherhood, Karen Good felt something else: a gnawing fear for her own health.
Good, then 41, had given birth to a healthy baby boy shortly after Christmas 2007. Her son was delivered four weeks early because she had developed preeclampsia, a potentially life-threatening condition characterized by high blood pressure and protein in the urine. In most cases the remedy is delivery; once the baby is born the mother’s blood pressure usually returns to normal.
But Good’s did not. Her blood pressure remained dangerously elevated, sometimes reaching 180/120 mmHg, far above the 120/80 considered the upper end of normal. She had severe headaches and sometimes would see stars. Doctors had started her on one drug, but it hadn’t done much. Before her pregnancy no one had told her she had high blood pressure.
“I was really scared,” recalled Good, a physical therapist in Baltimore. She had worked with young stroke victims and didn’t want to join their ranks.
Four months after her baby’s birth, Good would look with new understanding at the seemingly disparate problems that had plagued her for much of the previous decade and at clues that had been overlooked for longer than that.Her situation resembled the fable of the blind men and the elephant. Each of the men touches only one part of the animal — tusk, hide or trunk — and assumes it is the totality, missing the larger truth.
“I didn’t have a primary-care doctor, so no one could see the whole elephant,” Good said.
“She saw a lot of doctors, and sometimes the big picture does get missed,” said Kantha Stoll, the Alexandria internist whose hunch led to the correct diagnosis.
Good’s case was complicated by another key variable. Adopted shortly after her birth at Georgetown University Hospital in 1966, she knew nothing about her medical history for more than three decades.
Looking back, Good realized that the first sign of a problem was not, as she’d long believed, a fluke. In 1984, when she was 18, Good decided to have her blood pressure checked at a drugstore. The reading was high: 140/90, which would indicate hypertension. A student athlete and vegetarian, Good assumed the reading was simply wrong.
“I never told anyone, but there was no one to tell,” she said. Beginning in college and until she was well into her 30s, she had no primary-care doctor.
“I was married to a doctor then, and if I had a problem I would go right to a specialist related to the problem. And I often got health care from people I knew socially,” sometimes relying on advice casually dispensed at cocktail parties. Often, she said, one doctor didn’t know what another had prescribed or advised.
In 1997, when she was 31 and living in St. Louis, Good began having frequent headaches and dizzy spells. A neurologist friend began treating her for migraines. Good, who had only recently met her biological parents when they sought her out, was worried about something far more serious: a brain tumor. Her biological mother had undergone surgery for a very large meningioma, which can cause headaches and dizziness, and Good knew that these usually benign tumors can run in families.
Her fears were allayed when an MRI scan found nothing. A year later Good sought treatment for another problem: She was aware of a loud pounding in her ears, which sounded like her heartbeat; sometimes she could hear a whooshing sound. An ear, nose and throat specialist, another acquaintance, dismissed it. “Everyone can hear their heartbeat,” he said.
Good thought maybe her problems were related to her overall condition. “When I was out of shape they seemed to be worse, so I thought it was weight-related,” said Good, who often wrestled with an unwanted 10 pounds.
By 2003, Good had divorced and moved to Baltimore, where she was contending with new concerns: drenching night sweats as well as urinary urgency and frequency. An obstetrician-gynecologist prescribed a bladder drug, which didn’t work, and treated her for recurrent urinary tract infections. Another doctor did a urological work-up and concluded her problem was “weak bladder muscles.” Between the noise in her ears and her inability to be away from a bathroom for more than 30 minutes, Good recalls, “I felt like an old lady.” At 38 she learned to live with the problems, which waxed and waned.
“Since it was the first visit I didn’t put her on medication because I wanted to see more readings,” Stoll recalled, noting that blood pressure can sometimes reflect stress but not actual hypertension. “I figured it was traffic,” Stoll said. “She had just driven down from Reisterstown.” During subsequent visits, her blood pressure bounced around, but tended to be close to normal.
When Good, who had remarried, became pregnant in 2007, most of her medical care was dispensed by her OB-GYN. When medication prescribed by the OB-GYN failed to lower her blood pressure after her son was born, she went to see Stoll. During a January 2008 visit, the internist noticed something unusual: Good’s blood pressure reading dropped dramatically when she lay on her side. And her level of uric acid was high; after-pregnancy levels are typically low. Stoll thought both findings indicated impaired blood flow to Good’s kidneys. After she detected bruits in Good’s neck — blowing noises heard through a stethoscope that can indicate an obstruction — Stoll ordered a renal ultrasound and an echocardiogram to check her heart.
Stoll suspected that Good’s high blood pressure might be caused by a rare and often unrecognized condition called fibromuscular dysplasia, or FMD. The internist had seen one other case, years earlier, in a woman in her 50s.
First identified in 1938, FMD is a vascular disease that results in reduced blood flow to arteries in the neck, kidney and abdomen, causing high blood pressure, stroke, heart attacks and aneurysms, which can be fatal. The condition often goes undiagnosed, although autopsy studies estimate that between 1 and 4 percent of the population may have it. FMD ranges in severity; some patients have no symptoms, while others report the headaches, dizziness and ear noise that Good experienced.
FMD is most often diagnosed in women beween 25 and 50. The cause is unknown, although genetics are believed to play a role; about 10 percent of cases are familial, according to the Cleveland Clinic. There is no cure, but the disorder can be treated through medication and in more severe cases by angioplasty to reopen blocked arteries in the kidneys and elsewhere.
Specialized tests to confirm the diagnosis were performed at Johns Hopkins and the University of Maryland and took several weeks to complete. One specialist noted Good’s “extraordinarily strong” family history of hypertension. Three grandparents had suffered strokes, one dying before age 40. Her biological father, who had suffered several heart attacks before he was 60, had been given a diagnosis of high blood pressure at 17; one of her sisters was found to have hypertension in her 20s. None have been tested for FMD, according to Good.
Good, who had learned about her family history only a few years earlier after meeting her birth parents, was floored. A triathelete who never smoked, she had assumed she had little in common with her relatives, some of whom were overweight, sedentary smokers.
In February, as tests were underway, Good’s sense of urgency increased. She spent a night in a Baltimore emergency room for treatment of “the worst headache I ever had.” A CT scan revealed a lesion indicating an earlier small stroke. “I realized I could have a brain aneurysm at any time,” she said.
Good began making appointments with vascular specialists in Washington and Baltimore. After conferring with her ex-husband, a radiologist, and members of an FMD support group, she traveled to New York to consult specialists at Mount Sinai School of Medicine, who are experts in FMD treatment. “I didn’t want to see just anyone, because I knew this was rare,” she said.
In April 2008 Good underwent renal angioplasty, which opened the narrowed arteries to her kidneys. A month later, for the first time in decades, her blood pressure was under control, dropping to 100/50.
She takes medicines to maintain her blood pressure and reduce her cholesterol, since she remains at elevated risk for a stroke or heart attack. Every six months Good undergoes ultrasounds of her kidneys and neck and other tests to monitor her condition. Since the angioplasty, her night sweats have vanished, along with the noise in her ears, the urinary problems, as well as her headaches and dizziness.
Good said her experience has taught her to stop shopping for specialists and to eschew treatment from friends. “Now I don’t mess around,” she said. “I collect all the tests myself and send the results to all my doctors. And if I have a new symptom or problem, I go see my internist and we figure it out.”
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