Cindy's story: The will to get a second chance at life

The bulky oxygen machines now sit unused in a corner of Cindy's sunny living room.

Once tethered by tubes to these constant companions, Cindy's Class IV pulmonary hypertension (PH) was so severe she required the devices for every supermarket trip, lunch outing and even a journey up the short staircase in her home near Boston's north shore.

Today, after a groundbreaking infusion vasodilator therapy trial and a bilateral lung transplant from the Lung Center at Brigham Health, life couldn't be more different.

"I'm on a miracle journey," says Cindy, a trim, smiling grandmother, quilter, and animal lover who is rarely far from her beloved husband of 39 years, John, a retired pilot.

Cindy's journey began in 2007, when at age 54 she began to experience repeated shortness of breath upon exertion. Walking uphill became almost impossible. As a longtime ICU nurse at a local hospital who prided herself on an active lifestyle, she was immediately concerned by her symptoms. 

"I was someone who was used to taking power walks at Disney," Cindy recalls. "But I couldn't catch my breath, I was turning white and I had to go to the ER."  After several local hospitalizations, it became clear that pulmonary hypertension -- a progressive and often-fatal type of high blood pressure that affects the arteries in her lungs and heart -- was the diagnosis.

"I did not expect to be in this situation. I still had so much life ahead of me. It felt devastating," she says. "I wasn't expecting to spend my 50s sick like this."

Cindy sought out the expertise of Aaron Waxman, MD, who now serves as director of Brigham Health's Pulmonary Vascular Disease Program, for diagnosis and treatment.

Cindy knew a pulmonary hypertension plan would require frequent clinic visits and round-the-clock medication delivered via catheter.  "I knew I would work with my health care team very closely. They become like your second family," she says. 

Choosing Dr. Waxman to lead her treatment would turn out to be one of the most important decisions of her life, she points out.

"With a disease like this you have to be with someone you trust completely to bring you through," she says. "Having a good relationship reduced my stress and anxiety about the disease, because (he) was there."

Waxman agrees that a relationship between doctor, care team, and patient is particularly crucial while managing a complex illness such as severe PH. "The most important thing is good communication and making constant adjustments based on how patients are feeling. The more informed and engaged a patient is, the more we can do for them," he says, reflecting on her case. 

Cindy's relationship with The Lung Center at Brigham Health -- which features pulmonologists and thoracic surgeons collaborating in the same space so patients can easily see multiple specialists in a single visit -- opened up state-of-the art PH treatment options to her that were not widely available elsewhere, including her participation in two clinical trials.

In 2011, Cindy was selected as the first Brigham Health patient -- and the third nationwide -- to participate in an innovative pump infusion therapy trial that delivered medication directly into her pulmonary artery.

"We invited our patients to be part of that trial, we wanted their input to design the best system for them," Waxman says. According to the doctor, the results have been gratifying. "You can see an immediate impact on patient quality and safety."

Cindy's successful treatment with the pump gave her an improved quality of life for several years, allowing her to do some of the travel, gardening, and walking with her two munsterlander hounds, Oakley and Felda, she and John had enjoyed before she became symptomatic.

But by 2017, she had slowed considerably and was suffering bouts of hospitalization with pneumonia and the RSV virus. Careful adherence to her Brigham Health care plan and the state-of-the-art pump therapy had kept her stable for many years, but even the world-class medical care she was receiving did not have the means to repair her diseased lungs. 

Her team explained that a double lung transplant, a complex procedure that would rely upon her body's ability to accept donor lungs that were free of disease, was perhaps her best next option.

"I was failing. I was wasting. I was not doing well, I knew I had to consider a transplant. I didn't have an option if I was going to live," she says. She was resolved to move forward, despite her fear. "I had so much confidence in the medical team behind me."

On September 7, 2018 she and her husband were called to Brigham Health's academic medical center, Brigham and Women's Hospital. Donor lungs had become available for Cindy. Within a few hours after the surgery, she was up and walking slowly assisted around the ICU.

"I received the supreme gift. A second chance at life," she says. "I feel so blessed." 

To John's delight and relief, his wife now had the lung capacity to whistle at the variety of friendly birds who frequent the backyard bird feeder, and to move around their home with ease.

"You could hear the strength return in her voice and I felt such relief as she got back to a new normal. You take so many things for granted before something like this," he says.

Cindy's long partnership with the lung experts at Brigham Health helped pave the way for her successful bilateral lung transplant, Hari R. Mallidi MD, Co-Director, Lung Transplant, ECMO and Lung Assist Devices at the Lung Center, and executive director for ECMO, Heart & Vascular Center says.

The Center's goal is to explore cutting-edge therapies to help patients with advanced lung disease live better and longer, says Dr. Mallidi. Its specialists facilitate communication and collaboration with world health leaders specializing in the innovative therapies that can be integrated into the care of lung patients with complex illness. 

Cindy's wholehearted commitment to her recovery, including the hard work of rehabilitation, recovery and ongoing lifestyle, medication and follow-up has been instrumental to her care, Dr. Mallidi says.

"In a sense, we are tethered to each other now," he says of the relationship Brigham Health strives to build with patients.  "Our program is committed to taking care of these patients for the rest of their lives, and offering the best evidence-based treatment recommendations that we can make for them."

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