Sisters Breathe in the Good Life after Both Receiving Double-Lung Transplants

Twins born with cystic fibrosis now focus on staying physically fit

Isabel Stenzel Byrnes remembers her childhood nightly ritual of settling in beside her bed, clasping her hands and praying for a cure for cystic fibrosis. She and her identical twin sister, Anabel, were born with the condition, which is caused by a defective gene that regulates salt concentration in cells, resulting in a thick, sticky mucous that coats the airways and digestive tract.

Complications of cystic fibrosis include chronic lung infections that lead to loss of function, sinusitis, inefficient digestion or diabetes – and a lifetime spent combating these symptoms.
 
In 1989, Isa recalled, friends rushed over to their house with a newspaper clipping announcing the discovery of the cystic fibrosis gene, excited that there would soon be a cure. Unfortunately, knowing more about the condition has not yet translated into a cure.
 
But Ana and Isa, 32, have benefited from the next best thing: they both underwent double-lung transplants at Stanford and now enjoy the benefits of healthy lungs.
 
They still need to be diligent about avoiding infections – they both use hand sanitizer religiously – and taking anti-rejection medications, but they are liberated from the constant coughing, lung-clearing treatments and exhaustion of the past.
 
The two now focus on staying physically fit and even participated last week in the U.S. Transplant Games in Minneapolis, a four-day athletic competition for recipients of organ transplants. Ana won two bronze medals in speed-walking and swimming events, while Isa took part in speed-walking.
 
The twins originally came to Stanford as tourists in 1986 and were amazed when they heard that lung transplantation was taking place here. Bruce Reitz, MD, chair of cardiothoracic surgery, performed the procedure here for the first time five years earlier.
 

Go for the Gold Standard of CF Care Webcast LPCH, the Stanford University Medical Center and the Cystic Fibrosis Foundation are hosting a live CF patient education Webcast on September 21, 2004 at 7:00 pm.  The Webcast will cover the fundamentals of CF care.  Learn more and preregister for the Webcast.

They came to the university as undergraduates, graduating in 1994, and both ended up working at Lucile Packard Children’s Hospital where Ana is a genetic counselor and Isa, until recently, was a social worker. The sisters eventually had lung transplants at Stanford Hospital performed by Reitz.
 
Ana was first, at age 28 in June 2000. She had experienced a progressive decline in lung function, but for several years held on to the idea that she was strong enough to overcome the disability through relentless therapy and healthy living.
 
“I was really ambivalent about whether or not I wanted a transplant,” said Ana. “Part of growing up with cystic fibrosis is developing a comfort level with a lifestyle that is very familiar. It became who I was, and my passion for living was to fight cystic fibrosis. I felt that by getting a transplant, I was giving up or I was losing the battle.”
 
Eventually, her only choice for survival was a transplant after she suffered a bad bout of the flu in 1999 and never really recovered. Her lung capacity dropped to 20 percent of normal. She waited more than a year until a donor became available.
 
“It’s the hardest thing I ever did in my life, but what was the other option?” she mused. “The option is not being here. I wouldn’t have experienced living a very full life, which I never had before. I was always busy doing treatments or not feeling well. It’s been great, like a party for four years.”
 
Isa had her transplant in February of this year. Her progression to near-total loss of lung function was much more rapid than Ana’s.
 
In January, Isa listed herself for a transplant and checked into the hospital, insisting that there was going to be a miracle despite her rapidly declining lung function.
 
Although the average wait can be a year or two, suitable lungs became available for Isa after only two weeks – and not a moment too soon. Even with rigorous compliance with treatments and medications, Isa had no functional lung tissue left, according to the pathology report that came back after her surgery.
 
After the transplant, Reitz, who is also the Norman E. Shumway Professor in Cardiovascular Surgery, informed Isa that he had never seen anyone survive with blood levels of carbon dioxide as high as hers as she entered surgery. When she regained consciousness two days later, the first person she saw was Noreen Henig, MD, assistant professor of medicine, who runs the adult cystic fibrosis program. Isa told Henig, “It’s a miracle,” to which Henig replied, “It sure was.”
 
“I didn’t have the issues that Ana had about whether or not I’m giving in to cystic fibrosis; I was at the end,” said Isa. “So everything after Feb. 6 is a great blessing and an extra day of life.”