Kathleen Anderson was only 26 and a manager for a major drug chain when she first developed a blood clot in her leg, and then 11 unrelated blood clots in her lungs. She was a smoker and overweight, so doctors told her to quit and lose weight.
Anderson lost 100 pounds, but over nearly a decade her health problems got worse. She was eventually diagnosed with lupus and an unnamed clotting disorder.
"Over the years, I had symptoms – I was tired all the times and irritable and had trouble breathing," said Anderson, now 42 and living in Lake in the Hills, Ill. "My joints hurt all the time. At one point, I was diagnosed with fibromyalgia."
Anderson had suffered numerous lung clots, which had, in turn, had triggered congestive heart failure. Her condition was so disabling, she was confined to a wheelchair and couldn't work.
In 2011, she was told she had chronic thromboembolic pulmonary hypertension or CTEPH, a largely unrecognized disorder. Its prevalence is hard to measure because many people die before they are even diagnosed.
"I was told I wouldn't make it," she said. "They got me an advanced [breathing] machine and I was on eight liters of oxygen a day. My sister became my caregiver. I couldn't even fold the laundry. I couldn't do dishes or cook a meal."
When she arrived at the Cleveland Clinic in October 2012, her doctors didn't believe she would live beyond a month.
There, surgeons performed an intense operation, cooling down her body as they stopped her heart, so no brain damage occurs, then delicately stripping the blockages in her pulmonary arteries that were robbing her body of oxygen.
"Once the clot was removed and we effectively reduced the pulmonary pressure, her prognosis was excellent," said her pulmonologist at the Cleveland Clinic, Dr. Gustavo Heresi-Davila.. "It was absolutely successful."
"Prior to coming here, her doctors told me Kathleen was literally dying and we agreed," said Heresi-Davila. "She looked horrible and couldn't breathe. Her oxygen levels were low and she was getting medicine through an IV with a catheter sticking out of her upper chest."
Now her blood pressure in the lungs is normal, below 20, according to Heresi-Davila. Before, it had risen to 49. "She's cured."
The surgery was pioneered at University of California, San Diego, but now Cleveland Clinic is one of only a handful of hospitals with an expertise in the field. Heresi-Davila said he has performed 11 such operations in the last two years and has already done six this year.
"This is a deadly disease that can be cured by this operation," he said. "I think a larger number of people could be helped."
Chronic thromboembolic pulmonary hypertension or CTEPH occurs when clots travel from veins in the body and clog the arteries in the lungs. Once lodged in the lung arteries, they can cause more clots to form and add more resistance to the blood flow through the lungs. This increased resistance from the clots increases the pressure inside the lungs.
When the body cannot reabsorb a clot, it can trigger CTEPH. But up to half of all patients with the disease do not have a history of pulmonary embolisms, according to the Cleveland Clinic. About 5,000 Americans are diagnosed each year, but many cases are overlooked. Risk factors include more than one episode of clotting, chronic inflammatory disease and a history of cancer. Women are also at higher risk than men.