Extreme makeover includes new lungs and fixed heart

“Whenever I saw a flight of stairs or a hill, I panicked,” says Martha Carruthers, better known to her family and friends as Korkee. “When you have Alpha-1, you are always out-of-breath.”

Korkee, who lives in the southeast Iowa town of Mediapolis, was born with a rare genetic lung disorder called Alpha-1 antitrypsin deficiency (Alpha-1). Alpha-1 antitrypsin is a protein made largely in the liver that helps protect the lungs against damage from smoking and environmental respiratory irritants. In most patients with Alpha-1, the protein cannot get out of the liver cells and into the bloodstream. An estimated 80,000 to 100,000 Americans are living with severe Alpha-1, but less than 10% have been diagnosed.

"AAT deficiency is a rare disorder that can lead to severe emphysema necessitating lung transplantation," says Julia Klesney-Tait, MD, PhD, assistant professor of internal medicine and medical director of lung transplantation at UI Hospitals & Clinics.

The most common symptoms of Alpha-1 include shortness of breath, wheezing, decreased tolerance for exercise, recurring respiratory infections, and rapid deterioration of lung function with or without a history of significant smoking, chronic liver problems, and elevated liver enzymes.

Korkee remained relatively healthy until she reached her 40s. Her lung function declined to the point that she needed continuous supplemental oxygen treatments.

“I knew that I needed to do something when I was helping my son move into his dorm at The University of Iowa,” Korkee says. “I literally did not make it up the steps because I could not catch my breath.”

That difficult experience led Korkee to make an appointment to see her local physician. After her oxygen levels dropped dramatically during a treadmill test, she was referred to John Fieselmann, MD, a pulmonologist at UI Hospitals & Clinics.

Fieselmann explains: "We treated Korkee with inhalers and regular intravenous infusions of prolastin that can replace the deficient protein. That therapy may prevent some of the respiratory complications or at least slow the progression of the disease, but I told her the first time we met that she might eventually need a lung transplant."

Later, when Korkee was evaluated for a lung transplant, testing revealed that she had a serious blockage in the arteries of her heart and needed a cardiac artery bypass graft (CABG).

“A team of physicians and surgeons at the UI Heart and Vascular Center reviewed her case and concluded that bypass surgery at this stage was too risky because of the severity of lung disease,” Klesney-Tait says. “We agreed that the best option was to perform the needed surgeries simultaneously. We decided to wait until she had her lung transplant to fix the heart problem.”

The plan worked. On August 8, 2010, a pair of surgical teams took Korkee to the operating room. First, a clinical associate professor in cardiothoracic surgery, completed the cardiac artery bypass graft. Next, Kalpaj Parekh, MD, an assistant professor in cardiothoracic surgery and surgical director of the lung transplant program, transplanted the donor lungs. One week later, Korkee walked out of the hospital without her oxygen tank, striding eagerly toward a new life.

She is the only patient in Iowa to ever receive a lung transplant and a CABG at the same time.

“If I had known how much better I was going to feel, I would have not been so scared about the surgery,” Korkee says.

Klesney-Tait adds: "Treating a patient with a CABG and a lung transplant during the same surgical session is rare. We can manage complex cases like this one because of the expertise of our surgeons and the collaborative programs we have at the UI Heart and Vascular Center and the Organ Transplant Center. Her prognosis is excellent.”

Today Korkee says her post-transplant life is filled with new experiences. She recently bought tennis shoes to wear on her first trip to view the fall foliage in northeast Iowa.

“I’m feeling so much better. The people at University of Iowa Hospitals & Clinics literally saved my life, more than once, plus they have improved my quality of life. I had so many years when I could not do something as simple as pick up my grandchildren. I now have a lot to look forward to."