First off, what is Cystic Fibrosis? Well, Google is always a place to start:
“Cystic Fibrosis is a hereditary disorder affecting the exocrine glands. It causes the production of abnormally thick mucus, leading to the blockage of the pancreatic ducts, intestines, and bronchi and often resulting in respiratory infection.”
Another way to put that, CF is a genetic disease that causes continuous/progressive lung infections and limits the ability to breathe. The buildup of the aforementioned sticky mucus does not act as a natural lubricant, but it actually severely clogs the airway and ultimately traps bacteria in the lungs which leads to the infections, lung damage, and possible respiratory failure. CF currently affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide).
So what does all that mean? Well, to those affected without access to proper treatment and medicine, it can mean life or death. Pneumothorax, chronic infections, and respiratory failure are just a few of the life-threatening complications that can arise within a patient. Which makes it crucial to become educated and recognize the early symptoms, especially if your family has a history with CF.
Some of the earlier symptoms and signs shown in those with CF are:
- Chronic or persistent coughing, produces a thick mucus
- Shortness of breath
- The excessive “saltiness” of skin or sweat
- Exercise intolerance
- Poor weight gain/growth
What can those with CF do to better their quality of life? Thankfully, there have been phenomenal advancements in medication for various gene mutations, clinical trials, and exercise/nutrition methods for those with CF within the past decade. Although this may be true, this does not mean the elimination of CF within the patient. It is difficult to pinpoint a turn for the worse for the health in those fighting CF, which ultimately leads to the question of possible lung transplant.
CF patients undergo double lung transplants every year (people with CF always need two lungs). This does not eliminate the disease from the patient, but it can provide an extension of life. Lung transplantation advancements across all lung diseases have been improving the quality of life and lung acceptance rates are at an all-time high; great news for those with CF who turn to double lung transplantation.
But all of those who need a new set of lungs do not receive them. It could take months to years to receive a possible donor match, if ever. And if they do, survival is not guaranteed; the risks of organ rejection, lung infections, CLAD, and continued airway problems can all arise. That is where the Lung Transplant Project comes in. We are dedicated to funding the research that seeks to improve the acceptance rates of lungs and the length of survival post-transplant. For more information on our mission or to donate, check out our Research We Fund page.
Let’s not rest until everyone in need of a new lung gets one.
Columbia University Department of Surgery
Boomer Esiason Foundation
Cystic Fibrosis Foundation
Lung Transplant Project