Sometimes, life with Cystic Fibrosis makes no sense. You can go years without needing to go into the hospital or requiring IV antibiotics, and then suddenly need to do so five times a year. One day, you can have a relatively manageable lung function, and a week later lose half of it with a single infection flare-up.
I’ve seen each of those scenarios play out often enough to not trust CF one bit.
You see, we can do everything right – perform hours of airway clearance a day (my doctor wants me to do six hours when I have an active infection), inhale medications morning, noon and night, take dozens of medications and supplements, eat high calorie meals to replace lost calories spent simply breathing, see our (many) doctors regularly, gand o into the hospital for maintenance and acute flare-ups – and most us still aren’t going to live a “normal” or “typical” life span.
What about all the research and new treatments like Kalydeco?
While exciting, there are a few caveats about the newest medications created for CF patients.
- The much-celebrated drug Kalydeco doesn’t cure CF. In CF, defective proteins affect how salt and water move through cells. Kalydeco basically opens the gates to let them move freely through the cells.
- Kalydeco only works for about 4% of CF patients. There are more than 1,800 different known CF gene mutations, each with a different defect. This is part of the reason no two people with CF are exactly the same. Different combinations of the CF gene (remember, you need one from each parent to have CF) can cause varying combinations of symptoms. .
- When new treatments become available, the best possible scenario is that they will help maintain current levels of health. In other words, your lungs probably won’t get any healthier, but you’ll stop losing lung function. That’s why we have to try to keep up our health in every way possible.
Hasn’t the life expectancy has increased?
That number – “median life expectancy” or “median predicted age of survival” – so often thrown around doesn’t mean “average life expectancy.” It doesn’t represent how long most people with CF live (or at what age we’ll die). Median Life Expectancy is a number that tells us that of those CF patients alive today as tracked in the CF Patient Registry, half will die before that number, and half will die after that number. Statistics are tricky, and it’s sometimes hard to make clear conclusions about how they relate to us.
Even experts disagree on use of “median life expectancy” as a measuring stick for CF survival.
“Median age at death is not a good measure of CF survival. Though median predicted survival improved in each country over the study period, between-country comparison at a given time point may be misleading for rare disorders like CF.” – Jackson AD1, Daly L, Kelleher C, Marshall BC, Quinton HB, Foley L, Fitzpatrick P.
The fact remains, however, that about half of all CF patients listed in the CF Patient Registry are adults (over 18). That means that more of us are getting a higher education, working, volunteering, marrying or having children, and that’s great news.
The flip side of the coin is that as we age, our organs being showing signs of wear that wasn’t often seen even 20 years ago. Years of infection and inflammation, poor digestion, years/decades of heavy antibiotic use, and strain on organs secondary to typical CF involvement start to add up. Some examples:
- 43% of adults over 35 have CF Related Diabetes.
- 10% of CF patients have Liver disease.
- 10% of patents aged 16-53 have Osteoporosis, and 36% have Osteopenia, the precursor to Osteoporosis.
- 98% of men with CF are infertile
- Kidney disease due to heavy antibiotic use at any age, and chronic kidney disease as a result of Diabetes, as high as 19.2% in patients over 55.
- Years of strain on the heart from compensating for low Lung function can cause Congestive Heart Failure.
It’s no cliché to say that people with CF are fighting all the time. It’s like a car with 100,000 miles on that still shines – on the outside, we might look like everything’s running well, but under the hood, our engines are worn down, and the thousands of other pieces need to be examined, repaired, or tuned up.
It all comes down to quality versus quantity. It’s hard not to want both.