VALPARAISO | Diane Fike and her family were in Chicago two years ago for their annual Black Friday shopping trip when she started losing her breath.

"Literally, I was stopping and uggghhh," she said, sounding like she's gasping for air. "It was like, I need to go the doctor."

She went to a physician, who told her it was a sinus infection and prescribed her antibiotics.

But her breathing problems continued. In December, she was diagnosed with bronchitis. In January, pneumonia. In February, recurrent pneumonia.

Then, miraculously, she started feeling better again ... until one day in April.

"I got up to go to work in the morning and woke up on the kitchen floor," she recalled.

Fike was rushed to the emergency room. Doctors found large blood clots in her lungs. Her pulmonary pressure was about four or five times what was considered normal. She was treated and, five days later, sent home.

But after the shortness of breath returned in August, Fike was referred to a respiratory specialist, who finally figured out what was wrong: She had chronic thromboembolic pulmonary hypertension, or CTEPH, an underdiagnosed disorder where artery blockage leads to heightened pressure in the lungs.

More than a year later, however, she is cured: All it required was surgeons stopping her heart, putting her body into a hypothermic state and draining the blood from her arteries.

No matter what it took for her to get there, Fike can once again go swimming, walk up a flight of stairs and cut the grass, which, believe it or not, is one of her favorite activities.

"I can't even explain the difference. It truly is life-changing," she said on a recent day in the kitchen of her Valparaiso country home, surrounded by her husband, son and sister, who along with her pastor and a few friends, accompanied her to the Cleveland Clinic for the surgery.

Fike, 53, has a stoic demeanor but seems like she might still be caught off guard by the whole experience.

"You take it for granted, and then you can't even walk into the back of a store when you're grocery shopping," she said. "It feels really good to walk and be able to do what I want to do."

Dr. Gustavo Heresi, a pulmonologist at the Cleveland Clinic, said the disease is so underdiagnosed, because, while many people suffer from blood clots in the lungs, few end up developing CTEPH.

"It is a rare complication of a very common problem," he said. "This condition happens perhaps 1 percent of the time, for an estimated 2,500 to 5,000 cases a year."

Adding to the complexity is the fact that, in about a quarter of cases, blood clots don't even precede CTEPH, Heresi added.

Risk factors for pulmonary blockage includes lack of exercise, obesity and smoking, but it's unknown why the clots lead to CTEPH in rare instances.

"General practitioners and internal medicine doctors need to be aware of this condition, because they are the front-line workers taking care of these patients after pulmonary clots," he said.

The only way to treat CTEPH is through a procedure called pulmonary thromboendarterectomy. During surgery, doctors stop the heart, drain the blood from the cardiac and pulmonary arteries, and cool the person's body to about 60 degrees, which prevents brain damage. Dr. Nicholas Smedira, the Cleveland Clinic surgeon who performed the operation, compares it to "being immersed in Lake Erie at the end of winter." Doctors then peel the scar tissue off the arteries.

Only a few facilities in the nation have the capabilities to offer the procedure, which has about a 5 percent mortality rate and cures CTEPH in roughly nine out of 10 cases.

"If the patient doesn't have any other chronic condition or hasn't smoked for 50 years, they should return to normal physiology within six months or so," Smedira said.

Pike, who works for the Porter County clerk of the circuit court, first found out what the surgery would entail while researching her condition online. "I pretty much got sick to my stomach when I read it," she said.

Smedira told her she could wait to have the procedure until later, but her health could deteriorate by then, lessening the chances of a positive surgical outcome. Realizing her quality of life wasn't good as it was, she went ahead with the operation.

It turns out she had nothing to be worried about: She snapped back quicker than expected after the procedure.

"The last time we talked to Dr. Smedira before surgery, he said, 'When you see her in intensive care, she's going to look like she's dead,' " recalled Fike's husband, Steve. "But when we walked in, she had color and everything. The nurses said she should look terrible but doesn't."

"When she came out of it and started asking, 'Where's my lip balm?' that's when we knew she was OK," said her sister, Kathy Sheely.

The surgery also cured Fike of high blood pressure, which she had suffered from for nearly two decades. Her enlarged heart and heart murmur also went away. 

After a bit of back and forth, her insurance company agreed to pay for her treatment at the Cleveland Clinic. The cost, $132,000, surprised her family. "What I found amazing was the price," said her son, Nick. "My dad once had something done. Seven days in the ICU alone cost over $200,000."

Fike enjoyed the patient-centered experience at the Cleveland Clinic (valet parking, free chair massages, particularly cordial staffers). "The only bad thing I have to say is their food is as bad as any other hospital," she said. "It leaves a lot to be desired."

She returned home after a little more than a week in Cleveland. "I felt great until I got pneumonia a month later and ended up in the hospital here for five days. That was far worse than the surgery," she said, adding, "The surgery was a piece of cake."


Giles is the health reporter for The Times, covering the business of health care as well as consumer and public health. He previously wrote about health for the Lawrence (Kansas) Journal-World. He is a graduate of Northern Illinois University.