The lung specialist fixed Kimberlee Ford with an unwavering gaze as he peppered her with questions that left no doubt about his skepticism: Was she looking for an excuse to miss work or school? Was she depressed? Did she use illegal drugs?
Ford, a nursing student working at Walter Reed Army Medical Center, was dumbfounded, answering each question with an emphatic no. The pulmonologist, who had told her previously that she had asthma, finished the visit with a blunt assessment: “I think this is all in your mind and you need to see a psychiatrist,” Ford, now 32, recalls him telling her. In response, she said, she began to cry.
By then, she half-wondered if he might be right. In the previous month, her bizarre constellation of symptoms, the most prominent of which was shortness of breath, had grown much worse, and she had a gnawing sense that something serious was wrong. But neither the pulmonologist nor her internist, whom she had seen nearly a dozen times in the previous eight months, could find anything amiss, except asthma for which she had been prescribed drugs that didn’t help.
Yet asthma was the one illness Ford was pretty certain she did not have. Her mother did, and had told Ford, “My asthma at its worst is nothing like what you have.”
Less than a month after Ford’s July 2009 appointment with the lung specialist, a series of dramatic events revealed the reason for her breathlessness, a diagnosis that forever changed her life.
The first symptom occurred around Thanksgiving 2008, when Ford began feeling unusually tired. She was working full time and also attending nursing school at Trinity University in the District, but her fatigue seemed unusual. Sometimes her legs felt so heavy it was as if bricks were attached to them.
During her workouts at Curves, where she went four or five times a week, Ford would sometimes have to stop midway. “Some of the people noticed and asked, ‘Are you getting enough sleep?’ ”
In January 2009, she consulted her internist, who gave Ford a quick check. She assurred her that “everything looked good and maybe I was doing too much at a fast pace.”
But within a month, new symptoms emerged. Ford began experiencing dizzy spells and shortness of breath; like the fatigue, both were intermittent. She went back to her internist who performed a complete physical and an electrocardiogram and found nothing awry.
By March, the fatigue, shortness of breath and dizziness were worse. Her co-workers were, for the most part, unsympathetic. “They thought I was being lazy” and suggested that Ford exercise. That seemed out of the question: She had given up her gym membership because she no longer had the stamina to work out.
At times she felt unusually weak. Once while helping a patient put on his pants, she found herself suddenly bathed in sweat. Even though the man weighed only about 100 pounds, “it felt like I was trying to move a truck.”
She scheduled another visit with her internist. “At this point I was getting very scared,” she said, because it seemed clear something was wrong; her belly seemed swollen, and sometimes so did her legs. After five visits, the internist referred her to a lung specialist.
At the first visit to the pulmonologist in April, she told him about the latest strange episode: She had slept 36 hours, straight through a work day. She said she also told him that her chest felt heavy. After performing several tests, the lung specialist told her she had exercise-induced asthma and prescribed two inhalers. They made her feel worse.
In June, the baffled internist summoned two of her partners and together they examined Ford, who had gained about 25 pounds in the previous six months. By then she had given up her apartment and moved in with her parents, unable to climb the three flights to her apartment. “When I saw those steps, I saw mountains,” she said. She dragged herself through work, unable to walk down the hall without stopping.
The trio of doctors found nothing and told Ford her excess weight and exercise-induced asthma were probably the cause of her symptoms. In July she went back to the pulmonologist, who told her to see a psychiatrist.
Less than three weeks later, Ford fainted while at work, then awoke with severe chest pains. She managed to drive herself home where, she later learned, she collapsed on her parents’ sofa. The next thing she remembers is waking up in the emergency room of Fort Washington Medical Center, where she had been taken by ambulance.
Doctors gave her morphine for chest pain; she fainted again and was intubated because she was having trouble breathing. She was transferred to the much larger Washington Hospital Center; records show she arrived in a state described as “basically unresponsive.”
When she regained consciousness three days later in the intensive care unit, doctors told her “we didn’t think you were going to make it,” she recalled. They also told her what several tests had revealed: She was suffering from a severe case of primary pulmonary hypertension, not asthma.
“I’d never heard of it and I said, ‘Well, give me a high blood pressure pill,’ ” she remembers saying. Doctors gently explained that unlike garden-variety hypertension, pulmonary hypertension is a rare and incurable disorder, characterized by excessively high blood pressure in the arteries of the lungs, which makes the right side of the heart work harder than normal and impedes the flow of oxygen in the body.
When it occurs for no discernable reason, as in Ford’s case, it is called idiopathic. Between 500 and 1,000 such cases are diagnosed annually in the United States, most among women between the ages of 20 and 40, according to the American Heart Association.
Pulmonologist Octavius Polk, part of the team that treated Ford at Washington Hospital Center, said that doctors made the diagnosis based on her abnormal echocardiogram and a catheterization of the right side of her heart, which revealed significantly elevated pressure in the pulmonary artery.
“It’s a really virulent disease” but not an obvious one in young adults because it is so rare, Polk said. Ford’s is the fourth case of idiopathic pulmonary hypertension he has seen in 20 years. The hallmark symptom, shortness of breath, can signify many less serious disorders, including asthma, he noted.
“By the time she presented to us, it was pretty advanced and more obvious,” he said. Once she began treatment with various medications including a diuretic, Ford said, she lost 30 pounds, which was not excess fat, but accumulated fluid.
Pulmonary hypertension is more commonly caused by underlying diseases, such as sickle cell anemia or congestive heart failure, or by certain drugs. The best known is the diet drug combination known as fen-phen, which was pulled from the market in 1997 after it was linked to pulmonary hypertension and rare heart valve problems.
Because pulmonary hypertension is progressive, a lung transplant may be required for survival. The life expectancy for untreated PH is about 3 years and patients who, like Ford, have the most severe form of the disease, often need a lung transplant. New drugs and treatment approaches are extending the lives of some patients.
Polk said he could not explain why Ford’s first lung specialist insisted she had asthma without conducting a test that would have confirmed it — or suggested otherwise — or why he told her she had a psychiatric problem. An earlier diagnosis, he said, would not have changed the ultimate outcome but might have allowed her to take oral medications to control her disease. Ford might have avoided surgery to implant a catheter that delivers a potent and expensive new drug continuously through an IV pump. The devices require scrupulous and continuing attention because of their risks, which include lethal infection.
For Ford, the diagnosis was devastating, although she has worked hard to fashion a new life. The worst blow, she said, was learning she could never have children. Working and going to school were out of the question. And after years on her own and 18 months away from becoming a registered nurse, she became completely dependent on her parents, who had recently retired from jobs as grocery store managers.
Although she receives disability income and has had good insurance coverage through the federal government, Ford struggles financially. One of the drugs she takes costs $2,000 per month; Ford is set to receive Medicaid in August because her insurance coverage will end in a few months. She has also undergone a transplant work-up at Inova Fairfax Hospital, but hopes to avoid the operation.
Ford says she has some bad days, but is grateful to her parents and siblings who care for her and for the support of her church. Her former co-workers at Walter Reed, she said, call and e-mail her and sometimes send flowers. She goes to pulmonary rehabilitation sessions three times per week and heads a patient support group called Capital Breathers, which meets four times a year.
When her internist learned what had happened, she was “extremely upset,” Ford recalled, and told Ford during a long conversation that she had never heard of pulmonary hypertension. Ford said the pulmonologist who told her the problem was psychiatric has never contacted her, although she told his nurse about her hospitalization and diagnosis.
“In the beginning I always wanted to know why this had happened to me,” said Ford, adding that she no longer ponders that question. “I see people who have PH who are back at work, back at school, and I hope that will be me. They tell me I’m one of the lucky ones. I’m still here — and alive.”