During the weekend, I learned a friend was hospitalized, in ICU and intubated. It was because of complications of Cystic Fibrosis. I didn’t know she had that. In fact, I realized I knew next to nothing about the disease itself.
Here’s my public service announcement:
What Is Cystic Fibrosis?
Cystic fibrosis is a progressive, genetic disease that affects the lungs, pancreas, and other organs.
There are close to 40,000 children and adults living with cystic fibrosis in the United States (and an estimated 105,000 people have been diagnosed with CF across 94 countries), and CF can affect people of every racial and ethnic group.
There are many misconceptions about CF. Learn the facts on our page, Dispelling Misconceptions About Cystic Fibrosis.
In people with CF, mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to become dysfunctional. When the protein is not working correctly, it’s unable to help move chloride — a component of salt — to the cell surface. Without the chloride to attract water to the cell surface, the mucus in various organs becomes thick and sticky.
In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. For this reason, avoiding germs is a top concern for people with CF.
Today, because of improved medical treatments and care, more than half of people with CF are age 18 or older. Many people with CF can expect to live healthy, fulfilling lives into their 30s, 40s, and beyond.
Our friend is in her early 30s. I was shocked to learn from a doctor friend that people with Cystic Fibrosis often don’t make it to 40 years old.
Another thing I learned was almost as upsetting. There’s a new drug that is effective in treating Cystic Fibrosis, but costs $300,000 a year — without insurance. I’m not sure how much it is with insurance, but it has to be way too much.
Patient cost for Trikafta: more than $300,000 annually
Trikafta (elexacaftor/tezacaftor/ivacaftor) is a triple-combination therapy containing three CFTR modulators. These molecules can boost the functionality of the mutated CFTR protein in people with CF caused by specific mutations.
CFTR modulators are a relatively new class of medications for cystic fibrosis — the first such therapy was approved in the U.S. in 2012. While shown to be effective, these treatments come with a high price tag: The cost of Trikafta is more than $300,000 annually.
On the bright side, our friend is out of ICU, is going home and will live to see another day.
Do you know anyone with Cystic Fibrosis?
Were you aware that this disease is so severe?
I was aware that most with CF do not live long lives and that in the past there were rather barbaric treatments given to children to “break up” the sticky mucous secretions within the lungs. I’m glad there was a good outcome for your friend. I am not surprised however regarding the cost surrounding this medication. Pharmaceutical companies are not about helping people, but about making their owners billionaires.
I was shocked to learn all of this about CF, because I didn’t know anything except for the name. I’m so relieved our friend is doing better. Her doctor is looking into getting the RX for her for free. With something so ridiculously expensive, you’d think there would be a lot of grants or discounts offered. I understand that it’s a small market for a drug company, it’s not like they can sell it to millions. But this is beyond any understanding.
I’m glad to hear your friend is improving. Two of my sister’s friends over the years have passed away due to complications with CF and other chronic health challenges, multiple disabilities. 💔
I’m sorry about Lisa’s friends. I knew nothing about CF and the horrible prognosis. I’m relieved our friend is doing better and hopefully lives a long, full life.
❤️❤️❤️
💕💕
Wow, I just learned several new things as well. Thanks, Elizabeth. I’m glad your friend is improving!
I am so relieved our friend is doing better. Her lungs were filling with blood and it didn’t look good. I was so worried. I guess CF is under the radar because 100,000 worldwide is not a huge number of people who get this disease.
I knew nothing about this disease! Thank you for sharing with us Elizabeth! Good news that your friend is improving. It’s frustrating to know there is medicine that can treat this but it’s too costly. Hugs, C
Thank you. We are so relieved she’s getting better. I wonder how we never heard about CF before and that people don’t live into their 40s? How sad is that. I can’t believe how expensive the drug is that can double the length of their lives.
Yowza. I struggle with posts like this: I like the educational part, I learned something new about CF, but how do I like your friend’s situation? Don’t you yearn sometimes for ST:NG days where a tricorder can just “fix” these horrible diseases?
Absolutely. You’d think with all our science and medicine they could do more by now.
It’s such a horrible disease. I knew that it deeply impacts life expectancy. Good to hear they’re making progress on medications. And yes, I bet they cost an arm an a leg to get. Kind of sad. Glad to hear your friend is doing better!!!!
Thank you! I wasn’t aware she had CF and we’ve known her most of her life. We are so relieved she’s improving and out of the hospital. It was a serious episode.
Wow, amazing that she never talked about it. Glad she’s improving!!!
She’s my kids age and they knew. Somehow I didn’t. We are so relieved she’s doing better. It was a close call.
Do you know anyone with Cystic Fibrosis? A woman I knew as a girl in high school was diagnosed with Cystic Fibrosis. She didn’t live to 40 y.o.
Were you aware that this disease is so severe? Yes.
I had no idea. I’m sorry to hear about the girl in high school. I sure hope with new medications and care, our friend lives a longer life.
Oh my gosh! That must’ve been so scary for your friend! I’m glad she’s better now. ❤️
I am too! So scary for her family and friends.
Thanks. I learned something, too.
Thank you for reading.
One of my daughter’s best friends from high school has a sister with CF. She has the more severe type with a much lower life expectancy than 40. She just had a double lung transplant recently that will hopefully extend her life and make her quality of life much better
I’m so sorry to hear that. How scary. Our friend had a less severe case, but still suffers with pneumonia and this past weekend had a very tough time.
I had several students with cystic fibrosis and they didn’t make it out of their teens. There are improved treatments for it but unfortunately, not enough give them a full life span. That medication cost is outrageous!
I knew nothing about it except it was a chronic disease. I’m sorry about your students. The reports are that the expense of the medication aren’t justified for the impact. I’m not sure doubling your lifespan wouldn’t be worth it, but who could afford it?
I did know about this disease and can echo what Deb said. I’m glad there is treatment and not surprised by the outrageous cost of it. Glad your friend made it out of the hospital.
I am relieved she made it out of the hospital. They had to stop the bleeding in her lungs. We were especially worried that she was intubated. I can’t believe the cost of the medication.
I don’t know anyone with CF but have read about it. The cost of the treatment is so high how could anyone afford it?
I am sure they have grants or insurance but It would most likely be too high still.
Yes it is extremely high. I hope she can afford it
No, she couldn’t. After this last episode, her doctor is trying to get the fee waived for her.
Sending her lots of positive energy and prayers
Thank you! 💕
My friend from church’s daughter was diagnosed shortly after birth . She’s doing okay but they are aggressively working to get her in drug trials .
I’m so glad your friend is out of ICU, Elizabeth, but what a horrible disease. I’ve heard of it but knew nothing about it. She’s so young too. There are so many horrible diseases or conditions that we don’t know anything about until it touches our families or friends. And the cost of the medication is outrageous! It’s like we can’t afford to be sick! What’s wrong with this picture? Thanks for bringing awareness.
I do not know anyone with cf. I also didn’t realize how serious it is. I find there are so many things I don’t know enough about
I had no idea it was so serious either.