On His Own Terms

When Rob Lyon was diagnosed with Alpha-1, a rare and serious hereditary disease, he needed treatment that worked with his lungs and his lifestyle.

Rob Lyon,alpha-1 antitrypsin deficiency

After a diagnosis of a rare hereditary disease, alpha-1 antitrypsin deficiency, Rob Lyon has learned how to make treatments a regular part of his weekly routine.

When it comes to the people he loves, Rob Lyon makes time.

He once rode his motorcycle 35 miles balancing a birthday cake between his knees to surprise his wife, Amelia. They lived in Lucerne Valley and the nearest market with a bakery was in Barstow. He shared a slice of cake, kissed her, then jumped back on his bike. That quick bite of cake was all he had time for before he had to get back to work.

"And it was worth it," says Rob, now 36, remembering their brief but happy celebration.

When it comes to himself, Rob is a little more likely to put things off. It took a good scare before he grew serious about confronting his health issues—and Rob doesn't scare easily.

In the midst of a 12-week construction project on the 710 Freeway, Rob was coiling up a hose, one of the easier tasks in his job. He cranked the handle to wind up the hose, and on the third turn, he couldn't take a breath.

He retreated into a nearby truck and panicked.

"I thought I'd drawn my last breath," says Rob, 36. "And that's what finally got me to the doctor."

Rob had been battling what he thought was a series of upper respiratory infections stretching throughout most of 2011. He'd get on antibiotics, take refuge in the steam of a hot shower and do his best to clear his lungs. He also smoked heavily.

The scans showed signs of chronic obstructive pulmonary disease, or COPD, a condition that causes inflammation and blocks airflow from the lungs. This new diagnosis prompted Rob to share with his doctor some additional family health history. His newborn niece had recently been diagnosed with alpha-1 antitrypsin deficiency, a hereditary condition that can affect the liver or the lungs. His father had died from severe liver disease, despite never drinking alcohol.

A blood test revealed Rob did have Alpha-1, and it was the underlying cause of his COPD. Early symptoms include wheezing, shortness of breath and fatigue. Over time, the disease can worsen and damage lung function if left untreated.

"My doctors made it seem like I would be dead by the time I was 45," Rob says.

That prompted him to seek out an Alpha-1 expert, and he made an appointment with Jeremy Falk, MD, a pulmonary specialist at Cedars-Sinai.

"Dr. Falk was able to put my mind at ease," Rob says. "He walked me through what Alpha-1 is, how it works and how we can treat it."

Treatment on his own terms

The first step for Rob was quitting his 3-pack-a-day smoking habit. He admits it wasn't easy, and he had a few relapses, but now he hasn't touched a cigarette in years.

Rob's condition is treatable with weekly infusions. However, taking off work early once a week for treatment was going to be a problem.

"We're a single-income family, and I could lose my job," he says. "These treatments can save my life but finding a different job or taking a lot of time off weren't options."

Rob looked into having a nurse come to his home to administer the infusions, but that was too expensive. So, Amelia stepped in: She worked with nursing staff at Cedars-Sinai to learn how to administer the infusions herself, at home.

"Ideally, when patients are diagnosed with Alpha-1 and meet certain criteria, we set them up for weekly infusions at a center or with a nurse at home," Falk says. "Given his work schedule, that was never going to work. In the real world, economic and medical pressures are in conflict with one another and there needs to be a creative solution."

He's now been doing the home treatments for 7 years. His wife is a pro, and now their children help with the 30-minute treatments, too. Haylee, his 11-year-old daughter, and Alex, his 11-year-old niece he recently adopted, help get the supplies out. His son Landon, 6, and daughter Loralie, 3, help break down the boxes and carry them to the recycling bin.

They all get a turn to help dad.

"Everyone's satisfied they're helping me stay healthy, and I get to be at home," he says. "I'm really grateful my wife, in all her amazingness, was able to do this and that Cedars-Sinai helped us work that out."

Rob's lung function was at 48% when his treatment began, Falk says. His lung function has steadily improved, and he's breathing much easier. He continues working on construction projects, including his own—a home east of Las Vegas with plenty of land for their two horses, as well as dogs, cats and other pets.

The sweet moments still come between hectic work schedules and many responsibilities, but there are lots of them. He makes time for them and so does his family.