Take in a deep breath, then blow it all out as quickly as you can. Sounds easy enough, right? This is a task required of the cystic fibrosis patients on the 13th floor of Barnes Jewish Hospital in St. Louis, when performing a routine pulmonary function test.

Cystic fibrosis, or CF, is a genetic disease that causes thick, sticky mucus to line the walls of organs, specifically the lungs, causing repeated infections and over time, scarring.

In advanced cases, the scarring in the lungs can become so severe that the lungs ultimately fail. For these advanced cases, having a patient with 1/8 of their lung function take in a deep breath and then blow it all out as quickly as possible is like asking a man with no arms to do a push-up.

The test starts with a weak, shallow breath in followed by a wheezy exhale that ends with a violent coughing fit and flushed face. After several minutes of labored breathing and coughing, the patient repeats the process all over again.

Having CF doesn’t just limit the ability to perform a pulmonary function test; it limits the ability to do everything. If you need to do laundry, you’d better allow an extra 30 minutes for time between loading washers to catch your breath. If you need to take a shower, you will need to have oxygen tubing long enough to take with you into the water because trying to breath in a steamy bathroom is like trying to run a 5k while breathing through a straw. In addition, you can pretty much write off things like stairs since you will be toting around an oxygen tank on wheels behind you every time you go out.  

Unfortunately, CF doesn’t limit its grasp to your lungs. Once one of your vital organs begins to fail, a few others seem to fall in line. The lungs and heart are teammates.

When one begins to struggle, the other picks up the slack.

A heart compensates for a lack of oxygen in the blood by pumping faster and harder. As you can imagine, over time, parts of the heart become enlarged and overworked.

This little side effect is called pulmonary hypertension. This can cause chest pain, swelling in extremities and bluish colored lips.

In addition, when the body is starved for oxygen it starts doing other funny things. Your hair becomes thinner, finger and toenails curl inward and you take on a pale, gaunt appearance. Furthermore your infected and inflamed CF lungs fill with mucus during the night and cause surprise coughing attacks at random intervals. This makes a solid night’s sleep a sad joke.   

The bottom line is that someone diagnosed with CF has a body that inadvertently suffocates itself over the course of two to three decades. There is treatment but no real cure for this dickish disease. As a last resort, a double lung transplant can be an option for CF patients who meet certain criteria; assuming you are even keen on the idea of having someone saw open your thoracic cavity, pull out your lungs and stitch in someone else’s. The thought alone is enough to make you lose control of your bowels, even if the alternative is death.

Before a patient can be deemed a suitable candidate for transplant, they must undergo a week of rigorous tests that ultimately determine if you are sick enough to need a transplant and healthy enough to survive one. For some, this window of opportunity is gone quicker than a decent parking spot on campus. Patients who do fit the criteria are placed on lung transplant waiting list; a long list of equally urgent patients in desperate need of a double lung transplant.

With already more than 96,000 people on the waitlist for an organ donation and the unpredictable nature of cystic fibrosis, playing the waiting game is more like a game of Russian roulette. An average of 17 people die each day while on the waiting list for an organ donation, so being listed is hardly a get out of death free card.  It’s more like six months to a year of shitting yourself with anticipation each time you have an unexpected phone call from your doctor.

If and when that call comes telling you they’ve found a donor pair of lungs that matches your size and blood type, you and your oxygen tank can speed off to the hospital for the lung swap. Only after a couple weeks of complication-free recovery can you begin to celebrate your narrow escape from death and begin to get comfortable with the fact your lungs are in a biohazard waste bucket and someone else’s lungs are the only reason you are alive and able to attempt to wrap your mind around what just happened.

Until a cure is found, this stage is the best the 13th floor CF patients can hope to achieve. The truth is, many of them won’t, without a considerable amount of organ donations. The issue isn’t that people aren’t willing, but rather that people don’t understand the problem enough to really care one way or another about signing the back of their driver’s license.

However, for those banking on a transplant, a single person’s decision to donate their organs can mean a promising future for several recipients who would otherwise die.