After Disease Claims Lungs, A New Life Begins With Care Center's Expert Help

March 2012

Eight years after Jennifer Julian began to feel a bit short of breath, she sat in an exam room at Stanford Hospital & Clinics and listened to her doctor tell her that her right lung was failing and her left soon to follow. At just 48 years old, she would need a transplant to live to see her next birthday. “My head was spinning,” Julian said.

For years, Julian had known that her lungs were stiffening, altered by a reaction to something in the environment from a condition called chronic hypersensitivity pneumonitis. Medications and close monitoring had kept her going, even allowed her to scuba dive, ski, golf and visit one of her favorite places, a family-owned cabin in Montana at 7,000 feet.

Bit by bit, however, as the lining of her lungs scarred and prevented oxygen from passing through, each breath Julian drew grew shallower and she developed a cough that wouldn’t go away. The summer before she found herself in that exam room, she’d gone to that cabin in Montana and the altitude was too much. “I had a really, really tough time. It was horrible and I thought, ‘I’m never going to see this place again.’”

Jenn Julian
As Julian’s disease progressed, an oxygen tank became her constant companion. With each passing month, she could do less and needed more oxygen. Since her transplant, Julian has few restrictions on what she can do. She flies, and golfs, bikes, scuba dives and hikes.

"All of these diseases are interconnected. With the center, we can share resources. We have a critical mass of doctors, nurses, dieticians and social workers who can closely follow our patients."
--David Weill, MD, director, Stanford Center for Advanced Lung Disease

Then came that conversation with her doctor and the beginning of a journey toward a new life with new lungs. Julian’s vibrance and enthusiasm makes her a glowing example of reclaimed health supported by expert medical care at the Stanford Center for Advanced Lung Disease.

Once Damaged, No Repair

The recently established center is one of a small handful of treatment locations in the US to offer special experience and knowledge in diseases and disorders that can impede that most basic of body functions. With each breath in and each breath out, oxygen and carbon dioxide pass through the delicate filtering system of the lungs. “The lung is the only organ in our body that’s exposed to the environment 24 hours a day. The rest of the organs in the body, except the skin, are protected in some way. It’s a physiologically very complex organ,” said Glenn Rosen, MD, director of the Center’s Interstitial Lung Disease program, who became Julian’s doctor.

The lining of the lung, the interstitial space, is particularly remarkable, Rosen said. “It needs to be very thin so that air can pass quickly from the air sacs into the blood. Disease’s like Ms. Julian’s inflame and scar the space so you can’t get enough oxygen in or carbon dioxide out. Once that scar tissue appears, it can’t be dissolved.”

Unfortunately, science has not yet learned how to heal the lung from those most common lung diseases like emphysema, cystic fibrosis and hypersensitivity pneumonitis.  There are more than 100 chronic lung disorders in the interstitial lung disease group. “We are looking at finding ways to repair the lung with stem cells,” Rosen said. “We’re at the early stages of that and we’re learning more about the specific cells in the lung that are at risk.”

Jenn Julian and her flight instructor
Kelly O’Dea, Julian’s flying instructor, is another admirer of what Julian’s doctor, Glenn Rosen, calls Julian’s unique attitude. “She looks at setbacks as challenges, tries new things and lives life to the fullest. She’s a great example of someone who is passionate about wanting to enjoy her life."

Bringing all Stanford’s lung specialists together in one working group was a means to enhance collaboration for care that is best when multidisciplinary, said David Weill, MD, director of Stanford’s lung transplant program and now also director of the new center. “All of these diseases are interconnected. With the center, we can share resources. We have a critical mass of doctors, nurses, dieticians and social workers who can closely follow our patients.”

For many lung diseases, the only cure is new lungs. “You want to delay that as long as you can,” Rosen said. “You don’t want to do it too early because it’s a big operation and lungs can be rejected. But we don’t want to wait too long, until the patient is too weak. There’s a balance. We’re constantly assessing. We have a caring, empathetic team that works to create a comprehensive, individualized treatment plan. We also work closely with referring physicians—that’s critical because we’re not the primary care doctors for a lot of our patients.”

"The last thing I said to the team was, 'I have the utmost confidence in you guys,' and I'll see you on the flip side."
--Jennifer Julian, patient, Stanford Hospital & Clinics

The Lung: From Illness to Transplant

• Interstitial lung diseases can be unpredictable. Their symptoms can vary from mild to severe.

• Symptoms can include shortness of breath, fatigue and weakness, dry cough, labored breathing, chest discomfort, fatigue and weakness.

• What causes such illnesses remains under study. Environment, however, can play a major role. Family history, radiation and some medications can also contribute.

• Diagnosis may include pulmonary function tests, bronchoscopy, blood tests, X-rays or CT scans.

• Treatment choices can be influenced by age, overall health, extent of the disease and tolerance of medication.

• According to the Organ Procurement and Transplantation Network, about 1,800 lung transplants took place in the US in 2010. Stanford Hospital performs between 50 and 60 lung transplants annually, which places it in the top 10 for lung transplant volume.

• Of the 30 heart-lung transplants in the US, eight were done at Stanford. The average waiting time for a lung transplant at Stanford is between 45-90 days; the national average is nine months.

• Potential donors must be 18. Registration is available online at https://www.donatelifecalifornia.org/register/, or when renewing or obtaining a California Drivers License or California ID card. There are no disqualifying age or health condition requirements to register.

For more information about the Center for Advanced Lung Disease, call 650.736.5400 or visit stanfordhospital.org/lungtransplant. Join us at stanfordhospital.org/socialmedia.

Covering All the Angles

Watching each patient carefully, with different points of view, is essential. “Everybody plays a role—it is very much a team approach,” said Susan Jacobs, who along with another nurse coordinator, Virginia Adi , coordinates evaluations for about 30 new patients each month and follows over 300. “People are searching for information,” Jacobs said, “to confirm a diagnosis or to find out their diagnosis. They always want to know how serious it is, how quickly it’s going to progress and what their treatment options are.”

More than anything else, Jacobs said, the Stanford team wants their patients “to come away from the clinic, whether they’ve received positive or negative news, feeling like a multidisciplinary group of experts has given their case a thorough review and addressed all possible treatment options.”

With Julian, as she does with other patients, Jacobs is very honest. “I always tell them I can’t really know what it’s like in their position, but I’ve done this a long time and I do understand their needs. I want to educate them and give them a sense of control and to let them know we will be there to support them.”

"A lot of people don't get second chances. I did."
--Jennifer Julian, patient, Stanford Hospital & Clinics

“When I was first told that I needed to get a double lung transplant, I didn’t know that that existed,” Julian said. “As I went through the process of trying to understand more, I didn’t know if I was going to live or not. You face the reality of death. You really do ask yourself, ‘Is Stanford going to get me in time?’”

Moving Towards a New Life

Support was what Julian needed to convince Stanford that she would be a good candidate for a transplant. Apart from her lung condition, she was healthy but overweight. She needed to lose at least 35 pounds, a difficult challenge considering the limits on her ability to exercise. Jacobs directed her to a pulmonary rehabilitation therapy program for people with chronic lung problems. In that program, Julian learned to control shortness of breath during exercise and followed an exercise routine. With the help of a nutritionist, she followed a special diet.  She lost 47 pounds and was put on the list.

The disease progressed and an oxygen tank became her constant companion. With each passing month, she could do less and needed more oxygen. “I couldn’t do the simplest things—laundry, preparing meals. I would walk to the bathroom and it would take me 20 minutes to recoup.”

Her partner, Gail Strzepa watched her slowly fade away. Friends pitched in. “We had an incredible circle of friends who cooked meals, watched the dogs, called her, sent her cards, relieved me. Everybody was willing to help,” she said. Finally, the call came. Strzepa, who’d packed a bag, forgot it.

Jenn Julian
Julian has made her first solo flight, but has a ways to go before she’ll earn her pilot’s license from federal officials – there are few transplant patients who’ve been granted approval, but Weill and Rosen are on her side.

“It was a long night,” she said. As they wheeled Julian into the operating room, she had a smile on her face. “The last thing I said to the team was, ‘I have the utmost confidence in you guys,’ and I’ll see you on the flip side.’”

When she woke up after the surgery, she still had a breathing tube in her throat and couldn’t feel any change. Not until two days later, as she walked with the help of a therapist, sipping air with shallow breaths as she had for years, that there came a deeper breath. “It was one of the deepest breaths I’d taken in two years and it was incredible. I was crying and the therapist said,’ What happened?’ She’s looking at all my tubes, and I said, ‘Yes! I just took my first breath!”

Always in Mind

Another three months would pass before she really began to regain her strength. But, with permission from her doctors, she was skiing by January, just six months after her transplant. She had to wait a year to go scuba diving again, but that happened, too. She went back to golfing and brought home a gold medal in that sport from the Transplant Games competition. She got back on her bike. “I did everything I did before and something I’d always wanted to do—I’m learning to fly.” She has a ways to go before she’ll earn her pilot’s license from federal officials—there are few transplant patients who’ve been granted approval, but Weill and Rosen are on her side.

“Jen has a unique attitude,” said Rosen. “She looks at setbacks as challenges, tries new things and lives life to the fullest. She’s a great example of someone who is passionate about wanting to enjoy her life.  She’s a real fighter.”

She also spends a good portion of her time coaching other lung disease and transplant patients at support group meetings and individually. “I’m honored to be able to do that,” she said. And she begins each day with a few moments focused on that gift that gave her a second chance at a future. She’s written a letter of thanks that’s been sent to her donor’s family and hopes one day to give them the gold medal she won at the Transplant Games.

“Before the transplant we’d just kind of go through life, but after the transplant, it’s appreciating every single day waking up going, ‘Oh my god, it’s a beautiful day. Thank god I’m alive. Thank you, donor family,” Strzepa said.  “The donor family made that very difficult choice to give life to others. They are the real heroes.”

“A lot of people don’t get second chances,” said Julian. “I did.”

 

For more Stanford Hospital Health Notes Click Here.

 

Stanford Medicine Resources:

Footer Links: