The cystic-fibrosis breakthrough that changed everything

Cystic fibrosis once all but guaranteed an early death. When the disease was first identified, in the 1930s, most babies born with CF died in infancy. The next decades were a grind of incremental medical progress: A child born with CF in the ’50s could expect to live until age 5. In the ’70s, age 10. In the early 2000s, age 35. With Trikafta came a quantum leap. Today, those who begin taking the drug in early adolescence, a recent study projected, can expect to survive to age 82.5—an essentially normal life span.

Of course, there are issues:

Side effects: “It can cause cataracts as well as liver injury. More perplexing, Trikafta may affect the brain. For Jenny, starting Trikafta coincided with a wave of intense insomnia, brain fog, and anxiety.”
Trikafta repairs the results of a specific mutation: “For another group of CF patients, Trikafta simply does not work. About 10 percent lack the F508del mutation that the triple combination was specifically designed to fix.”
Since so many people have been more or less cured, support groups for CF patient have been disappearing, even for the 10% who are not helped by Trikafta: “Recently, Make-A-Wish announced that children with CF would no longer automatically be eligible for the program, because “life-changing advances” had radically improved the outlook for them.”
And… CF patients expected to die young. Often they have no savings, no professional skills, no kids, because they did not expect to live long enough to deal with those. But now they will live.

7,700 words but all interesting: https://www.theatlantic.com/magazine/archive/2024/04/cystic-fibrosis-trikafta-breakthrough-treatment/677471/. Send me a note if you can’t read it.

From the internet (2018, part 1)

I save interesting sayings that I find on the internet. Here are some from 2016-2018 but which still make me laugh… or learn:

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> Old Chinese proverb: You can’t polish a turd.

Rlippa: But you can sure step in it… over and over and over again.

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” …You Don’t Tug On Superman’s Cape…

You Don’t Spit Into The Wind
You Don’t Pull The Mask Off The Old Lone Ranger
And You Don’t Mess Around With “A Grand Jury Subpoena.”

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Richard Haass, president of the Council on Foreign Relations, tells me: “The result will violate a cardinal national security rule: Avoid having more than one nuclear crisis at a time.”

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biffbobfred Ars Scholae Palatinae:

<<…>>

My grandmother was kinda like this. At night she got up and laid on the bathroom floor, probably because the tiles were cool and soothing (no evidence of a fall). My sis found her in the morning. No known cause of death. I can guarantee she was not part of Project Mayhem or whatever. Human bodies are incredibly complex. Sometimes I wonder why we don’t randomly explode. The fact that some people live for 30,000 days or more is still magic to me. Not a miracle but magic.

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I don’t know why men go to bars to meet women.

Go to Target.

The female to male ratio is 10 to 1 & they’re already looking for things they don’t need.

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Remember, folks, you can lead a gift horse to water, but you can’t look him in the mouth. (Or something like that.) Sometimes you just have to bite the hand that you’re dealt.

We could stand here and talk until the cows turn blue. But we have to get all our ducks on the same page, or the fan is gonna hit the roof.

Wake up and smell the music. Don’t count your chickens without breaking a few eggs!

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You never realize how many shortcuts a computer has until a cat sits on the keyboard.

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Libertarians believe consenting adults have the right to do whatever they choose, except band together. — Emo Philips

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https://www.washingtonpost.com/science/2018/11/14/raccoons-drunk-crab-apples-cause-false-rabies-scare-west-virginia/:

High animals are more common as more jurisdictions legalize marijuana and people plop the drug into tasty edibles that also appeal to their pets, who can’t read warning labels and don’t typically have the impulse control to stop at one, Brulliard reported.

Societal collapses

Historians are now using scientific methods of analysis to summarize how and why societies collapse:

By looking back at past polycrises (and there were many) we can try and figure out which societies coped best…. Pouring through the historical record, we have started noticing some very important themes rhyming through history. Even major ecological disasters and unpredictable climates are nothing new.

Inequality and elite infighting

One of the most common patterns that has jumped out is how extreme inequality shows up in nearly every case of major crisis. When big gaps exist between the haves and have-nots, not just in material wealth but also access to positions of power, this breeds frustration, dissent and turmoil.

About 4,100 words: https://theconversation.com/historys-crisis-detectives-how-were-using-maths-and-data-to-reveal-why-societies-collapse-and-clues-about-the-future-218969.

Something to take a little heart from:

Fascists and fascist governments, despite their positioning are generally bad at war…

War is something fascists value intensely because the beating heart of fascist ideology is a desire to prove heroic masculinity in the crucible of violent conflict (arising out of deep insecurity, generally). Or as Eco puts it, “For Ur-Fascism there is no struggle for life, but, rather, life is lived for struggle…life is permanent warfare” and as a result, “everyone is educated to become a hero.”² Being good at war is fundamentally central to fascism in nearly all of its forms – indeed, I’d argue nothing is so central. Consequently, there is real value in showing that fascism is, in fact, bad at war, which it is.

Concluding:

The more standard pattern is that fascist or near-fascist regimes regularly start wars of choice which they then lose catastrophically. That is about as bad at war as one can be.

More details at https://acoup.blog/2024/02/23/fireside-friday-february-23-2024-on-the-military-failures-of-fascism/ (approx 2,200 words).

And some more good climate news from the Wall Street Journal via FutureCrunch:

In 2020, Chinese leader Xi Jinping pledged that the country’s emissions would begin falling before 2030 and hit net zero before 2060, part of its plan prepared under the Paris accord. He also said China would have 1,200 gigawatts of total solar- and wind-power capacity by the end of this decade. The country is six years ahead of schedule: China reached 1,050 gigawatts of wind and solar capacity at the end of 2023, and the China Electricity Council forecast last month that capacity would top 1,300 gigawatts by the end of this year.

40% of US electricity now emissions-free

((2023)) Wind and solar are likely to be in a dead heat with coal, and all carbon-emissions-free sources combined will account for roughly 40 percent of US electricity production.

Overall electricity production year-to-date is down by just over one percent from 2022, though demand was higher this October compared to last year. This is in keeping with a general trend of flat-to-declining electricity use as greater efficiency is offsetting factors like population growth and expanding electrification.

That’s important because it means that any newly added capacity will displace the use of existing facilities. And, at the moment, that displacement is happening to coal.

Excellent progress! Of course, electricity generation is not the only source of global warming gases, but it’s an important part. Details at https://arstechnica.com/science/2023/12/40-of-us-electricity-is-now-emissions-free/.

And from FutureCrunch (my bolding):

For the first time in a decade, the EPA has tightened regulations on air pollution, lowering the allowable limit for annual PM2.5 levels from 12 micrograms per cubic meter to 9. The reduction is predicted to reap $46 billion in net health benefits by 2032, including prevention of up to 800,000 asthma attacks and 4,500 premature deaths. NPR

Alzheimer’s progress

For years, Alzheimer’s conferences were like the obituary pages in the local newspaper: It’s where clinicians and researchers in the field went to find out the names of the latest promising drugs to die. Between 1998 and 2017 alone, 146 clinical trials of new Alzheimer’s drugs failed…

The big potential benefit (of new drugs), say Alzheimer’s experts, lies in using these drugs, or others soon to come, in conjunction with a second recent development in the field: diagnostic blood tests that can identify the presence of Alzheimer’s-associated proteins…. Scientists believe that in a few years clinicians may be able to use them to make quick, early diagnoses cheaply, even before patients show any outward symptoms. That suggests a new strategy against the disease: GPs could screen otherwise healthy people for early-stage Alzheimer’s and treat them with drugs that slow the progress of the disease before major damage has occurred. The hope is that eventually, Alzheimer’s will no longer be a terminal disease but a chronic one that can be managed with drugs and perhaps be staved off indefinitely.

Full article also covers the history of the disease, why it was so hard to develop effective drugs, and how PET scans (to detect amyloid and tau in living people) were a game-changer. And remember, this is just the start — the future will only bring better treatments. 4,400 words: https://www.newsweek.com/2023/10/20/can-we-prevent-alzheimers-scientists-say-new-tests-treatments-game-changer-1832957.html.

Bonus: an item from FutureCrunch:

Ten people died from unprovoked shark attacks globally in 2023, a slight uptick over the five-year average. This makes sharks less dangerous than lawn mowers, ladders, champagne corks, jet skis, and lightning strikes.

Guess which one of those things got an entire article in ABC News?

Lithium “shortage” bubble implodes

Lithium is a necessary component of modern batteries. It’s needed in greater and greater quantities for electric vehicles and for energy storage for wind and solar power. The huge increases in EVs and storage have led to questions about whether we can find enough:

Currently, Australia, Chile, and China dominate lithium production. Australia alone accounts for nearly half the global production. The three combined account for about 90% of global production.

But production is growing: “Price collapsed 77% in a year”: https://wolfstreet.com/2023/11/23/lithium-shortage-bubble-implodes-once-again-as-demand-and-production-both-surged/…

Bonus: an item from futurecrunch.com’s latest email. I’m pretty sure you haven’t heard about this:

One of the most underrated ecological phenomena of our time is the regeneration of abandoned farmlands, thanks to the more efficient land use of modern agriculture. Since the 1990s, the EU has reforested an area the size of Portugal, the United States uses 40% less cropland than in 1960, and globally, an area of farmland half the size of Australia is abandoned every year.

Welcome to the Internet

By Bo Burnham. Pretty funny and pretty on-target: https://www.youtube.com/watch?v=k1BneeJTDcU. 4 min 40 seconds. Lyrics: https://genius.com/Bo-burnham-welcome-to-the-internet-lyrics.

Welcome to the internet! Have a look around.
Anything that brain of yours can think of can be found.
We’ve got mountains of content, some better, some worse.
If none of it’s of interest to you, you’d be the first.

And as a bonus, one item from dozens in the latest Future Crunch weekly newsletter:

Last year China more than doubled its solar capacity and increased wind capacity by 66%. This year is going to be all about storage. The country currently has 210 GW of pumped storage and 100 GW of batteries either in operation, under construction, or contracted. That’s going to take a huge chunk out of coal and gas.

One gigawatt is enough energy to power about 750,000 homes. And doubling its solar capacity in one year? Wow!

Good news from 2023

Yes, the US and the world have serious problems. Yes, we are looking at an uncertain future. And yes, lots of things have gotten better and better:

Cancer? “European cancer mortality for 2023 was estimated to be 6.5% lower for men and 3.7% lower for women than in 2018, the United States reported cancer death rates have fallen by a third in the last three decades, Australia reported significant reductions in skin cancer in under 40s, there were major breakthroughs in treatments for colon, skin, bladder and cervical cancer…”

AIDS? “Two decades ago, the disease seemed unstoppable, killing two million people a year, but today, it’s a very different story. In July, the United Nations revealed that in 2022, deaths fell to 630,000, there were an estimated 1.3 million new infections, the lowest since the early 1990s, and only 130,000 new infections in children, the lowest since the 1980s.”

Clean energy? “Humanity will install an astonishing 413 GW of solar this year, 58% more than in 2022, which itself marked an almost 42% increase from 2021. That means the world’s solar capacity has doubled in the last 18 months, and that solar is now the fastest-growing energy technology in history. …If solar maintains this kind of growth, it will become the world’s dominant source of energy before the end of this decade.”

Electric vehicles? “Global electric vehicle sales increased by 36% this year, bringing the world’s total to 41 million electric vehicles. The shift is remarkable: just two years ago, one in 25 cars sold globally was an electric vehicle. This year it will be one in five, and by 2025, one in two. The IEA now says that electric vehicle sales, like solar installations, are tracking ahead of its net zero scenarios. In the United States, where the media spent much of the year insisting there’s been a slowdown, sales were up 50%…”

Suicide? “Over the past three decades, global suicide rates have fallen by more than a third, thanks primarily to rising living standards in the two most populous countries in the world.”

Women’s rights? “Uzbekistan passed a law giving women greater legal protection against gender-based violence, the Netherlands and Switzerland amended their laws to introduce a consent-based definition of rape, Sierra Leone passed landmark legislation advancing women’s rights, Oman passed a law prohibiting the termination of employment due to pregnancy, childbirth or breastfeeding. In China, a new law protecting women against discrimination and sexual harassment came into effect…”

And dozens more! https://futurecrunch.com/goodnews2023/. Sign up for their free weekly good-news email.

Dave Barry Year in Review 2023

And so it is with a heavy heart and an upset stomach that we look back at 2023…

FEBRUARY: In sports, LeBron James sets a new NBA record for points scored, breaking the record previously set by U.S. Rep. George Santos. Major League Baseball spring training gets under way with new rules intended to shorten the game, including breaking ties via “Rock, Paper, Scissors” and the elimination of third base.

MARCH: …Silicon Valley Bank, whose depositors include many super-smart high-tech hedge-fundy individuals, collapses like a cheap lawn chair at a sumo wrestler picnic when the person in charge of managing the bank’s finances accidentally deletes the Quicken file….

The Academy Awards for Best Actor and Best Actress both go to U.S. Rep. George Santos.

JUNE: In other disturbing environmental news, yachtsmen in the Strait of Gibraltar report that orcas have been deliberately attacking, and sometimes sinking, sailboats. What is even more troubling, marine biologists say, is that the orcas are posting videos on TikTok.

OCTOBER: Conflict erupts between two bitter foes, ancient enemies whose intractable hatred for each other has defied all efforts to resolve the historic differences between them: House Republicans and other House Republicans.

NOVEMBER: In entertainment news, the Rolling Stones announce plans for a new tour, to be sponsored — really — by AARP (Official Motto: “AARP! It’s the Last Sound You Make Before You Die”). The venerable rockers will travel to 16 North American cities and perform a three-hour show, including two 45-minute bathroom breaks.

https://www.miamiherald.com/living/liv-columns-blogs/dave-barry/article283068803.html

The Select Committee on the Modernization of Congress

The ((previous)) select committee created to reform Congress, which focused on budgeting, passed exactly zero recommendations by the time it ended in 2018. So, how did this modernization committee become one of the most high-functioning bipartisan workplaces on Capitol Hill, creating what a Roll Call reporter called a “parallel congressional universe”? How did it manage to adopt, in just four years, 202 bipartisan recommendations, about two-thirds of which have already been executed or made significant progress in that direction? What in God’s name is going on over there?

And what, if anything, can the rest of us learn about how to get things done in our own divided institutions and families?

A lot of it was rearranging the usual way of doing things so both sides could see and interact with each other in normal ways:

They stopped sitting up on high, on a dais, like every other committee and started sitting in a round table format, at the same level of the people who came to testify. Turns out that fixing politics starts by rearranging the furniture. “You can foster more productive conversation when you can look each other in the eye,” Kilmer says when I ask him to explain the obvious.

Also, having bipartisan dinners together. Having Republicans sit next to Democrats instead of separate groups. And talking about the difficult things… like being afraid of being murdered during the 1/6 treason.

https://www.washingtonpost.com/opinions/2023/02/09/house-modernization-committee-bipartisan-collaboration-lessons/. About 2,500 words.