Something extraordinary happened yesterday at the North American Cystic Fibrosis Conference – the Cystic Fibrosis Foundation announced that the median life expectancy in cystic fibrosis leaped from 41 to 47 years. It’s nothing short of remarkable, but what does that mean for you? What does that mean for me?
Not a whole lot.
I’m still coughing up the same green shit (actually a little red) today that I was coughing up yesterday. Nothing remarkable has changed in my life, and I’d be willing to bet the same about yours.
I urge caution when we talk about life expectancy.
I have expressed my frustrations with this particular estimated stat over the years since it’s really just a way to show progress with fundraising, research and development.
Concrete data in Cystic Fibrosis Foundation Patient Registry showed that in 2016 half of reported deaths occurred before the age of 30.
That is a long way off from 47.
Admittedly the Patient Registry is just a bunch of numbers, and as it states, it does not take into account “personal characteristics,” but it is pretty good at showing trends.
Now, I don’t have access to all the concrete data being reported at NACFC (I never go because I don’t want to assume the risk inherent in cross-infection [thank God for the Internet]), so I’m pontificating off the information made public.
The available data shows us that cystic fibrosis is a very different condition for those born in the 70’s, 80’s and 90’s than it is for those patients born in recent years, which is really kind of a strange thing.
Haven’t us older patients been the ones on the forefront of fundraising and awareness for several decades? How much have we benefited?
How many older patients are in end-stage respiratory illness or post transplant?
We’ve made small gains here or there – Pulmozyme, Cayston, new Vest/airway clearance options aimed at improving patient compliance, nutritional standards, a handful of IV antibiotics, CFTR modulators that are helping a portion of the patient population (some more than others) and other decent treatment/care options.
Put together this is significant, but the simple fact remains that not every option is available to all patients.
We are still dying before the age of 30.
We are still dying before we have the chance to start a family and collect retirement.
We are still dying younger than we should be.
If you think I’m frustrated, you’re damn well right that I am.
Maybe it’s because I coughed up a little blood last night, maybe it’s not.
The progress brought about by CFF is nothing short of a MIRACLE. In fact as CF research and development relates to the entirety of human history, the accomplishments in cystic fibrosis science will go down as one of mankind’s most remarkable achievements.
But when I wake up each morning feeling like an elephant is sitting on my chest, it’s hard to feel like I have a miracle sitting in front of me.
Progress moves at a different pace for a patient than it does for someone who is healthy.
I won’t be sold on any progress until I am holding a concrete treatment in my hands that actually makes a significant difference in my health. There are plenty of things that I would like to do, like cruise across the US in my truck or move towards independence, which at this point seem impossible to me. When that changes I’ll be blogging about something different here.
Ultimately when we are talking about progress in the medical field, it is important to hold off the celebrations until you are experiencing it. You must continue to advocate for your health. That is the most important thing you can do.
Dramatic changes in our health may be coming very soon, but until then… I’ll continue to wave this flag.
