She chooses to fight against deadly lung disease

Eleven years ago, Karen Erickson was told she had two years to live.

Erickson, 45, with lungs that work at less than 20 percent, was diagnosed with a disease so little known that the physician's assistant who delivered the news didn't get it quite right.

“She said: ‘You have alpha-1 antitrypsin deficiency. Your body is eating your own lungs,'” Erickson said. “She said, ‘There's no cure, but if we get you on treatment, we think we could get you a couple more years.' ... I thought: ‘What the hell?'”

Today, the Thousand Oaks, Calif., woman wears a black backpack for the small tank that delivers oxygen through clear tubes that hiss when she inhales.

She uses two tanks when she pedals the wheels of her stationary bike at a speed that wouldn't keep her upright outdoors.

Everything in her life — the exercise, the 50-foot lifeline that leashes her to an oxygen concentrator when she sleeps, the speeches to people with similar disorders and the double-lung transplant she hopes will come within a year — return to one goal. She wants to help people understand the disease that forced her to take control of her life.

“It's her attitude,” said Dr. Sandy Sandhaus, a friend and medical director of the Alpha 1 Foundation in Miami. “That sounds kind of hokey, but it has to do with whether you believe what someone tells you or you choose to fight. She's definitely a fighter. ... Instead of going and hiding in her room, she got on her bike and started riding.”

Alpha-1 deficiency is a hereditary disease in which defective genes rob the liver of some of its ability to produce the alpha-1 antitrypsin protein. In some cases, the protein can't be released from the liver and builds up there, killing cells and causing cirrhosis-type scarring.

For Erickson, who was adopted and doesn't know her birth parents, the deficiency means she lacks a shield designed to protect her lungs from infection. The resulting deterioration has brought on emphysema and made her one of 24 million Americans believed to have chronic obstructive pulmonary disease, better known as COPD.

Because alpha-1 is marked by common symptoms such as wheezing and shortness of breath, it's often diagnosed as asthma or attributed solely to smoking.

Foundation leaders say 10,000 Americans have been diagnosed with the disease, but they think 90,000 more suffer from it. They believe it's more than twice as common as cystic fibrosis.

“The most important thing is people not getting tested,” said Sandhaus, explaining efforts to screen babies and raise awareness in health care professionals. “Even physicians say, ‘I haven't thought about it since medical school, when I got a 10-minute lecture on it.'”

Erickson was in a spin class when she was 34 and a fitness maniac with energy to burn. She struggled so hard to breathe that she temporarily lost her sight.

Doctors said she had asthma. Additional tests showed extensive damage.

“They said to me, ‘You have the lungs of an 83-year-old,'” she said.

When it finally came, the alpha-1 diagnosis didn't help much. She took weekly injection treatments of the protein her body struggles to make but did little else.

“I was definitely in denial,” she said. “I was like: ‘What happens? Do I just die in 730 days?'”

She moved to Ireland for a year and met doctors who had researched alpha-1. They explained how the disease works and dismissed the idea that she would die in two years.

“They fed me information that this isn't a death sentence,” she said.

She started researching transplants, diet and medical care and found associations and foundations for alpha-1 and other lung disorders. She met people who preached the importance of fitness, worked to raise money for a cure and tried to find ways to help others understand the disease.

She has become one of them. She takes an oxygen concentrator and her tubes on planes to give speeches in Las Vegas and Phoenix about overcoming limitations. She mentors newly diagnosed people. She reaches out to COPD patients, urging them to get tested for alpha-1 and figure out whether their children are at risk.

“She changes lives around her with her positive energy,” said friend Penny OBrien, a wellness and fitness instructor in Park City, Utah. “People are so far removed from understanding their bodies and how to make themselves better. She's just literally a needle in a haystack. She can barely breathe. She still gets herself up and gets herself moving.”