Brad is a 25-year-old living with cystic fibrosis. He has spent much of his life dealing with the harsh symptoms of the disease and the reality that his life would probably be short. However, he received a double lung transplant and can now do things he wasn’t able to do before, like going on trips and even rock climbing! He writes a blog about living with the illness and is president of the Northern California chapter of the Lung Transplant Foundation.

Question

What is cystic fibrosis?

Explain why Brad would assume his life would be short.

How is Brad now able to do high intensity activities like rock climbing, even though he still lives with cystic fibrosis?

Answer 1

What is Cystic Fibrosis?

Cystic fibrosis (CF) is a genetic disorder that primarily affects the lungs and digestive system. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, leading to the production of thick, sticky mucus that can clog the airways and trap bacteria. This results in chronic respiratory infections, lung damage, and difficulty in breathing. The thick mucus also affects the pancreas, making it difficult to digest food and absorb nutrients. CF is a progressive disease, meaning that symptoms typically worsen over time, and it can lead to serious respiratory and digestive complications.

Why Would Brad Assume His Life Would Be Short?

Historically, the prognosis for individuals with cystic fibrosis has been poor, with many patients experiencing significant health challenges and decreased life expectancy. In the past, most individuals diagnosed with CF did not live past their teenage years, and even in more recent decades, many patients faced severe limitations on their physical activities, leading to complications and a shortened lifespan. The uncertainty of disease progression and the potential for life-threatening infections would lead someone like Brad to assume that his life could be relatively short. Moreover, individuals with CF also often experience complications such as lung failure, which further contributes to the understanding that their lives may be limited.

How is Brad Now Able to Do High Intensity Activities Like Rock Climbing?

After receiving a double lung transplant, Brad's ability to engage in high-intensity activities like rock climbing has significantly improved. The transplant replaces his diseased lungs with healthy ones, alleviating the severe respiratory limitations that come with cystic fibrosis. Post-transplant, patients typically experience enhanced lung function, which allows for increased oxygen intake and improved physical endurance. This enables them to participate in a wider variety of activities, including those that require significant exertion, such as rock climbing.

Additionally, the psychological and emotional benefits of receiving a transplant can lead individuals like Brad to feel empowered and motivated to explore new challenges, traveling and engaging in activities they may have previously thought impossible. While he still lives with the underlying condition of cystic fibrosis, the successful transplant provides him the opportunity to live a more active and fulfilling life.