There isn’t a day that goes by when a family, somewhere, experiences the horrors of cystic fibrosis in a close, personal way. Overshadowed in the constant talk of advances within our drug development pipeline and the widespread improved prognosis for a lot of us, is the simple fact that cystic fibrosis continues to kill people who are far too young to die.
I am terribly saddened to learn of Claire Wineland’s death. She was a tremendously public figure in the world of CF, and while I may not have agreed with everything she had to say about living in the face of chronic illness, she did a lot of amazing things for the CF community and inspired countless people. Our conversation on the Making it Matter podcast was one of our most watched podcasts ever. She will be missed.
She’s not the only person with CF to have passed within the last several days, though.
We also remember and celebrate the life of Storm Johnson, a young man of 22, who passed away over the weekend after complications from a recent double lung transplant. Like Claire and anyone else who has succumbed to CF, Storm had a personal impact on countless people. Storm will be missed.
Their lives were no less important than any of us who continue to live. No family should have to go through what their families are going through now.
Every life is important and worth saving and celebrating, yet we continue to fail the sickest among us.
There is no more proof needed for that statement than a humble stat coming off the Cystic Fibrosis Foundation Patient Registry – the median age of death for people with cystic fibrosis in a given year remains BELOW 30 years old, and has for quite sometime.
Simply put, half of the people cystic fibrosis kills are younger than 30 years old.
Claire and Storm are the statistic.
While so many people in the cystic fibrosis community are finally getting better (for the first time in the history of our illness!), so many others continue to decline, yet it seems as if there is so little we can do for them before sending them into the unknown of transplant.
We cannot disregard the people who can’t wait for future FDA decisions, medical breakthroughs and innovation. If anything, we must PRIORITIZE the people who have been a part of our community for decades, but have no viable treatment options in end stage illness. We must be working to develop treatment and therapy options for people who have run out of options.
We must see and realize the importance of the median age of death statistic instead of hiding behind it. It is our responsibility to prevent patients from dying in their 20’s. This cannot go on forever.
As our medications and the prognosis improve for the youngest generations of people with CF, so must the medications and prognosis improve for those of us who have suffered decades of lung scaring and oxygen deprivation.
We have to develop a viable way to treat and reverse the effects of the illness in the final stages of the disease.
Don’t ever forget that the people who identify most with the median age of death statistic are the people who have been on this planet fighting, fundraising and surviving far longer than our youngest generations.
I know it’s hard to imagine for some people, but there was an incredibly long, brutal time before Kalydeco, Orkambi and Sydmeko, and sadly the sickest among us are still living in that era.
Solving this problem is how we honor the people like Storm, Claire and the tens of thousands of others whom cystic fibrosis has taken from us.
Please join me in celebrating and honoring all of those people who have been taken from us too early.
https://www.instagram.com/p/BWs_fxfj0YP/?hl=en&taken-by=claire.wineland
https://www.facebook.com/StormSJohnsonStrong/photos/a.2015987978653088/2015988125319740/?type=3&theater
