Lung-Pancreas transplant is 1st of its kind

Surgery may extend life of cystic fibrosis patients with insulin-Dependent diabetes

By Shari Rudavsky

Looking good: Jose "Joey" Garcia, 25, is recovering at Methodist Hospital after the 12-hour surgery. - Kevin Martin / The Star

WHAT IS CYSTIC FIBROSIS? Cystic fibrosis is a common genetic disease that affects the entire body, causing progressive disability and early death. Shortness of breath is the most common symptom and results from frequent lung infections such as pneumonia that are treated, though not always cured, by antibiotics and other medicines. Other symptoms, including sinus infections, failure to thrive, diarrhea and infertility, result from the effects of cystic fibrosis on other parts of the body.< /p> Cystic fibrosis is one of the most common fatal inherited diseases. Individuals with cystic fibrosis can be diagnosed prior to birth by genetic testing or in early childhood by a sweat test. Lung transplantation is often necessary as the disease worsens. Cystic fibrosis is caused by a mutation in a gene called the cystic fibrosis transmembrane conductance regulator. This gene helps produce sweat, digestion juices and mucus. Although most people have two working copies of the gene, only one is needed to prevent cystic fibrosis. The disease develops when neither gene works normally. Therefore, cystic fibrosis is considered an autosomal recessive disease. The name refers to the characteristic scarring (fibrosis) and cyst formation within the pancreas, first recognized in the 1930s. Source: http://cysticfibrosis.com/ ABOUT ORGAN TRANSPLANTS In 2005, 518 multiple organ transplants were performed in the United States, 23 of those in Indiana. A total of 28,108 transplants were performed, 621 in Indiana. There are more than 92,600 candidates in the U.S. on a transplant waiting list and more than 700 in Indiana. A name is added to the list every 12 seconds. In 2005, there were 14,488 donors in the U.S. and 246 in Indiana. About 19 people die every day waiting for a transplant. Source: The Organ Procurement and Transplantation Network, http://www.organdonor.gov/

After 25 years of battling cystic fibrosis, Jose "Joey" Garcia needed new lungs to help him breathe.

His pancreas also had stopped functioning normally, another side effect of the disease that had ravaged his lungs through chronic infections.

The Fort Wayne man this week received new lungs and a healthy pancreas from the same donor in the first operation of its kind in the nation, and perhaps the world, by surgeons with The Clarian Transplant Center in Indianapolis.

The lung-pancreas transplant operation represents a potential milestone for cystic fibrosis patients, about 40 percent of whom develop a unique type of insulin-dependent diabetes by adulthood that destroys the pancreas.

In an interview Friday at Methodist Hospital, Gloria Garcia, Joey's mother, confessed she had originally opposed the operation but agreed her son made the right choice.

"I'm glad he made the decision," she said, as Joey recuperated in a hospital bed nearby. "Now if God's willing, he's going to have a better quality of life."

Doctors view the surgery as a potential life-extender in a fight against a disease that has no cure and afflicts about 30,000 people in the United States. People with cystic fibrosis have an average life span of about 35 years.

The surgeons will be watching Garcia to see how he fares.

"He looks great now, but we don't know how he'll look in six months," said Dr. Thomas Wozniak, surgical director of cardiothoracic surgery at The Clarian Transplant Center, which includes Riley, Methodist and Indiana University hospitals.

Transplant surgeons had done a variety of multiple-organ surgeries in the past but had never before ventured a lung-pancreas combination.

The fact that Clarian's program, the third largest in the country, had surgeons experienced in both areas helped lead to the double operation, said Dr. Jonathan Fridell, director of pancreatic transplantation at Indiana University Hospital.

Garcia's lungs caused the greatest concern, but a pancreas transplant could free patients like Garcia of diabetes forever, doctors agreed.

"They are being transplanted primarily for lung disease, and the pancreas is coming along for (the) ride," Wozniak said.

An injured pancreas can interfere with the absorption of the necessary anti-rejection drugs that patients take after a transplant. In addition, those medications can exacerbate the diabetes, Wozniak said.

Any cystic fibrosis patient with diabetes could be a candidate for the operation, Fridell said.

"I think it's what we're planning to offer for our standard from now on," he said.

Other cystic fibrosis experts welcomed the news of the double transplant.

"It's a lifesaver," said Lynn Figg, a nurse practitioner at a cystic fibrosis clinic in South Bend, who has two patients on the waiting list for the double transplant. "This completely gives them a new lease on life."

But others cautioned that the widespread availability of this operation would rest on already-scarce organ resources. Many cystic fibrosis patients die before donor lungs become available, said Dr. James Yankaskas, a professor of medicine at the University of North Carolina-Chapel Hill.

Still, he added, he's interested in how Garcia and others will fare over time.

"This is a great opportunity to see what happens," he said.

Diagnosed at the age of 6 months, Garcia has battled cystic fibrosis all his life. In his healthier years, he worked as a Fort Wayne bus driver, just like his father, a job he relished.

About three years ago, he developed diabetes and had to give up the wheel.

Garcia was awaiting a lung transplant when the surgeons raised the prospect of receiving a pancreas at the same time. His mother fretted, but he did not waver.

From the standpoint of both surgeons, Tuesday's operation was relatively routine, if lengthy, taking about 12 hours.

First Wozniak replaced Garcia's damaged lungs with the anonymous donor's organs, a process that took about seven hours. Then, as Wozniak napped, Fridell transplanted the pancreas.

Garcia may stand a better chance of rejecting neither organ because both came from the same donor, Wozniak said.

Right after surgery, Gloria Garcia said, she saw an improvement. By Thursday, her son could sit up briefly.

He told his parents he's never felt as good as he does now.

Call Star reporter Shari Rudavsky at (317) 444-6354.

Copyright 2006 IndyStar.com.

This article posted August 30, 2006.