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Breathing Restored
08/09/09

Her Life Threatened by Pulmonary Hypertension, a Young Woman Finally Finds the Right Treatment

"We used to hear you huffing and puffing," Carrie Leon's coworkers tell her with a laugh.

The happy change came about when Leon, a 34-year-old financial analyst from New Rochelle, was treated successfully at Westchester Medical Center this year for the illness that sneaked up on her: pulmonary hypertension.

Before that, however, she endured the unfortunate medical equivalent of a wild goose chase.

A difficult problem to diagnose

In pulmonary hypertension, blood pressure rises sharply in the arteries that go from the heart to the lungs. It's not directly related to the blood pressure doctors measure with a cuff on the arm. But it can make it hard for the lungs to oxygenate the blood so the body can function properly. The results can be shortness of breath, dizziness, fainting, impaired mobility and eventual heart failure.

Few physicians know how to treat pulmonary hypertension properly. Until Leon found the right doctor – Warren Rosenblum, M.D., Westchester's Associate Medical Director of the Heart Failure/Cardiac Transplant Programs – the treatments she was given missed the mark.

For a couple of years Leon had fainting spells. "At first I'd get dizzy when I exerted myself – say, when I walked up a hill," she recalls. "But it got to the point where I'd get that way just walking from my bedroom to my bathroom." Her breathing also became labored.

Leon's doctor sent her to a cardiologist, who found nothing wrong with her heart. A pulmonologist, however, diagnosed an infection in her lungs and treated that with heavy doses of antibiotics, which caused liver problems. When her hand swelled from the liver medication she was given, she was diagnosed with rheumatoid arthritis and treated for that. But her breathing problems continued to worsen. Last fall, she passed out twice.

Over the winter, another doctor took a lung biopsy and diagnosed lung disease. She was put on prednisone, a strong steroid, which may have contributed to the stomach pain that sent her to a local hospital this April. There, physicians thought she needed gallbladder surgery.

Before they could operate, however, she showed signs of heart failure. Her lips were blue and she had massive swelling in her abdomen and legs. Finally a doctor ordered an echocardiogram, which revealed that the pressure in her pulmonary artery was 96 millimeters of mercury (mmHg), five times the normal reading of 20 mmHg.

"That's when they called me," says Dr. Rosenblum.

He has been treating pulmonary hypertension for 15 years, he says, so he knew the problem immediately. "She had all the signs and symptoms, and she'd been seen by lots of specialists, including some heart specialists," he says. "But they didn't have the experience to identify it."

Pulmonary hypertension is often a byproduct of other heart and lung diseases. Or it may be inherited or caused by cirrhosis of the liver or connective-tissue disorders such as lupus or rheumatoid arthritis. In still other cases – such as Leon's – the cause is unknown.

Unfortunately, there is no cure. But medications developed in recent years have improved treatment a lot.

Better odds through better treatment

"Fifteen years ago, our only choice was one very potent intravenous medication, and it was used only for patients awaiting a lung transplant," says Dr. Rosenblum. "There was nothing for more moderate cases. Median survival was quite poor then – less than three years." Now, though, the five-year survival rate approaches 90 percent in some forms of pulmonary hypertension, he says.

"Once we got Carrie on the right meds, she did a lot better," he says. The pressure in her pulmonary artery was reduced, and she was given a diuretic that helped her shed 65 pounds of water weight in a month.

"I feel great, like I did five years ago," she says. She is on a restricted, low-salt and low-fluid diet, still takes medication, including an inhaler, and sees Dr. Rosenblum every month for follow-up care. But she's back at work and feeling better – and grateful.

Says Leon: "I found the right doctor, thank God!"

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