Lung Transplant Breathes New Life Into Patients With Cystic Fibrosis

Jennifer Jones, a young woman diagnosed with cystic fibrosis (CF) eleven years ago, has recently gained internet fame due to a video, gone viral, posted by her fiancé.

The video that has shot Jennifer to stardom is of her first breath with her new lungs. “At first I was scared and then it was like, ‘oh, my goodness, I can breathe! I can still breathe and I’m still taking in this air,’ and it was overwhelming. It was incredible,” Jennifer explained in an interview with a local news source.

More donors now than ever

According to data from the United Network for Organ Sharing (UNOS), in 2017, organs were recovered from 10,281 deceased donors—more than a three percent (3%) increase from 2016 and a 27% increase over the last ten years.

Those donated organs contributed to the 2,449 lung transplants performed in 2017—a record number for organ transplants in the United States.

Approximately 30,000 Americans live with cystic fibrosis, a genetic and inherited disease that causes patients to suffer from chronic lung infections and progressively interferes with their ability to breathe without mechanical assistance, as well as impacting other areas of the body. Cystic fibrosis is a chronic disease related to a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which causes patients to overproduce bulky and sticky mucus that will accumulate in organs, such as the lungs and pancreas. When present in the lungs, the mucus can clog airways, reduce the ability of oxygen exchange in the body and promote the growth of unhealthy bacteria. In the pancreas, the mucus will disable the release of digestive enzymes, thus impeding the ability for the digestive tract to properly utilize food.

For end-stage cystic fibrosis, a lung transplant may be a patient's final hope

Cystic fibrosis can affect many different areas of the body, but the lungs draw the primary focus of patients and their physicians, due to CF’s innate ability to take away one’s capacity to properly breathe. In most situations, patients are treated with CFTR modulator therapies, which are designed to help correct the function of the defective genetic protein, as well as with inhaled medications, like mucolytics and antibiotics, which are used to help clear clogged airways and fight infections. In addition to medicinal therapies, cystic fibrosis sufferers often must use therapeutic airway clearance techniques, like vesting, to help loosen the thick, sticky mucus and enable the clearance of the airways and lungs.

Cystic fibrosis, however, is a progressive disease, meaning that symptoms and complications will progress over time and prescription therapies may lose their efficacy. A lung transplant is the number one option for end-stage cystic fibrosis and can be the only option to improve a patient’s quality of life.

The controversy with lung transplants

A 2007 study published in the New England Journal of Medicine prompted major controversy over whether lung transplants offered enough survival benefits for those diagnosed with cystic fibrosis. Ultimately, doctors and researchers made the decision that lung transplants do offer enough benefit for the right patient where other medicinal therapies have failed.

Read on to learn more about the reality of lung transplants for patients with cystic fibrosis.