Living with Cystic Fibrosis

Almost four years ago, I met a woman. We became best friends. We fell in love. We were married. She is a special for so many reasons. She also faces special challenges. She was born with a genetic disorder called Cystic Fibrosis. At first it frightened me. I read all that I could. It scared me to think about it. But she is healthy. And she is strong. And time marches on. We have dealt with hospital visits, home IVs, daily therapies, medications and appointments. But we have also climbed mountains, explored other countries, had adventures and loved each other more everyday. We are learning to live with Cystic Fibrosis.

The respiratory system is greatly affected by Cystic Fibrosis. The thicker mucus coats lungs surfaces and leaves them prone to infection. Each time a person with CF goes to the clinic, he or she has their lung capacity tested.

The pancreatic ducts are clogged by the thick mucus. This blocks digestive enzymes from exiting the pancreas, complicating the digestive process.

One of the strange conditions associated with CF, as well as other respiratory ailments, is that of club fingers. There aren't any harmful effects associated with club fingers, and I find them beautiful.

The short answer is yes--Cystic Fibrosis is a fatal disease, without a cure. The longer answer gives me hope. In 1962, the life expectancy was 10 years. When my wife was born, it had reached 18 years. Today, life-expectancy is nearly 37 years. We hope and pray that this trend will continue.

Infection by bacteria is a major problem in the respiratory system of people with CF.

There are many different treatments and therapies for Cystic Fibrosis. While no cure exists, these therapies and treatments have both lengthened and improved the quality of life for people with CF.

Airway Clearance Therapy is important for Cystic Fibrosis. In this picture my wife is wearing a vest that fills with air and then vibrates. This helps shake loose mucus and clear her airways. The mouthpiece she is using is part of the nebulizer, which allows her to inhale an antibiotic. People with CF must also take enzymes evey time they eat so that they can digest their food. There are various other treatments and therapies that can be prescribed by a doctor to help treat CF.

Does Cystic Fibrosis limit what you can do?

Cystic Fibrosis requires careful and consistent care. Does this limit your activities? I can only speak from our experience. My wife takes her enzymes after eating tacos from a street vendor in Mexico City. She does her airway clearance therapy in the morning before exploring Tikal. She takes her antibiotic at night after a climbing trip. She takes a bit of extra rest when we hike. Does it stop her from doing what she loves? No, not at all.

Of course, as the disease progresses, activity will become more difficult. But we will always make the most of what we have, and we are loving our life so far. We are living with Cystic Fibrosis.