Cystic fibrosis breakthrough has given patients a chance to live longer
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Re: Cystic fibrosis breakthrough has given patients a chance to live longer
#2I lost a cousin and a family friend to CF. Both in their 20's. One after a lung transplant added some time.
It is such a horrible disease and the breakthroughs are amazing. So many families will be much more fortunate than mine and I am happy for them!
Re: Cystic fibrosis breakthrough has given patients a chance to live longer
#3I had a coworker with CF that started this treatment. Over the course of their first year of treatment, as it became plain to them just how effective it was and what the implications for their long-term survival might be, their behavior changed drastically. Within 18 months of starting treatment they were showing worrying indications of both budding mental health issues and heavy substance abuse. Their marriage, which had been stable for years, was in shambles as well. I can only speculate that their exuberance at being given a stay of execution lead them to "oversteer" into some questionable lifestyle choices.
Re: Cystic fibrosis breakthrough has given patients a chance to live longer
#4I have CF, and I take Trikafta.
Before Trikafta, I usually had a 5+ day stay in the hospital every year, and sometimes I have would very stubborn respiratory infections that just wouldn't go away. I was mentally preparing myself for inevitable decline and eventual death.
Trikafta changed my situation dramatically. I've had no hospitalizations, and most the classic CF symptoms are either gone or extremely diminished. It can't undo a life of damage to things like my pancreas, vas deferens, etc, and I still take medicine to digest my food, but overall, it's as close to "normal" as I could hope for at this point.
The only real downside: weight gain. With CF the pancreas is blocked, so you lack in digestive enzymes, and it's a struggle to maintain a healthy enough weight to battle respiratory infections. After Trikafta, I gained some 30 pounds, and have a big belly on my small frame, and went up some 6 inches in the waist. To add insult to injury, it happened during the spring and summer of 2020, when buying clothes was a challenge due to the pandemic.
Speaking of the pandemic, the timing of Trikafta was amazing: it kept the normally full "CF floors" of hospitals empty, opening up those beds for those with COVID and keeping CF patients less exposed.
One side effect I should mention: many report extreme anxiety. However, I was starting an anxiety medication for the first time (something I should have done 20 years ago, but alas ...) and so those effects were muted or hidden to me.
(copied from the last time Trikafta was mentioned here: https://news.ycombinator.com/item?id=37540731)
Re: Cystic fibrosis breakthrough has given patients a chance to live longer
#5Interesting note about Trikafta and other advancements in cystic fibrosis: the Make a Wish Foundation announced that children with CF now no longer automatically qualify for their program due to the advancements in care.
Re: Cystic fibrosis breakthrough has given patients a chance to live longer
#6I have CF, and I take Trikafta. Before Trikafta, I usually had a 5+ day stay in the hospital every year, and sometimes I have would very stubborn respiratory infections that just wouldn't go away. I was mentally preparing myself for inevitable decline and eventual death. Trikafta changed my situation dramatically. I've had no hospitalizations, and most the classic CF symptoms are either gone or extremely diminished.…
Sorry for the potentially insensitive question, but I really am wondering about this:
>One side effect I should mention: many report extreme anxiety. However, I was starting an anxiety medication for the first time (something I should have done 20 years ago, but alas ...) and so those effects were muted or hidden to me.
I would have imagined that most people with CF already had extreme anxiety - wondering when the infection that is going to end one's life will arrive. Is it really possible that Trikafta is causing noticeably worse anxiety?
Re: Cystic fibrosis breakthrough has given patients a chance to live longer
#7Oh wow. I remember a kid in elementary school with CF. I didn't understand at the time what he and his family was going through. I remember his personality and intelligence, his skill on the soccer team. He was unusually driven for an 8-9 year old.
As I got older and into my 20s, I thought about him a lot -- it was understood that most CF patients don't make it out of their early 20s and I knew even if he were particularly lucky, as time went on and I aged, he was probably gone.
My father also recently died from lung cancer, and had a few months where he was effectively drowning in his own lung fluids, requiring doctors to drain his lungs with long needles through his back. That experience also brought me back to that schoolmate, considering what he had had to endure.
Recently, I was reorganizing my personal library and came across my elementary school yearbook and flipping through, saw his picture. It's been decades since he's likely passed on. It gave me pause to contemplate certain priorities in my life and try to cultivate greater compassion.
It's a particularly cruel disease, and this news is wonderful.
Re: Cystic fibrosis breakthrough has given patients a chance to live longer
#8I had a coworker with CF that started this treatment. Over the course of their first year of treatment, as it became plain to them just how effective it was and what the implications for their long-term survival might be, their behavior changed drastically. Within 18 months of starting treatment they were showing worrying indications of both budding mental health issues and heavy substance abuse. Their marriage, whic…
This section seems to speak to your observation (edited for brevity):
Doctors told me they could think of only one other comparable breakthrough in recent memory: the arrival of powerful HIV drugs in the 1990s. Like Trikafta, those drugs were not a cure, but they transformed AIDS from a terminal illness into a manageable chronic one. Young men got up from their deathbed, newly strong and hale. ... This was a remarkable turn of events. But it elicited a complicated mix of emotions, not all of them joyful. Some patients who were no longer dying grew depressed, anxious, and even suicidal at the thought of living. This phenomenon became known as “Lazarus syndrome.”
Death is an end, after all. Life comes with problems...the writer Andrew Sullivan, who is HIV-positive, described life after the advent of the HIV drugs in his essay “When Plagues End”:
When you have spent several years girding yourself for the possibility of death, it is not so easy to gird yourself instead for the possibility of life. What you expect to greet with the euphoria of victory comes instead like the slow withdrawal of an excuse. And you resist it.
The intensity with which you had learned to approach each day turns into a banality, a banality that refuses to understand or even appreciate the experience you have just gone through.
Re: Cystic fibrosis breakthrough has given patients a chance to live longer
#9I remember studying about how CF was an agonizing death sentence just 10 years ago in highschool. It's both interesting to learn that even at that time the life expectancy info was outdated, and even cooler to hear this news.
Re: Cystic fibrosis breakthrough has given patients a chance to live longer
#10It appeared too late to make a difference for my dear friend and the kindest human being I knew. The manifestation of life's injustice drives me mad ngl.