Once a death sentence, cardiac amyloidosis is finally treatable | Hacker News Reader

Once a death sentence, cardiac amyloidosis is finally treatable

nytimes.com

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http://archive.today/uTakY

Robert Jordan, author of the "Wheel of Time" fantasy series, died of this disease in 2007.

https://en.wikipedia.org/wiki/Robert_Jordan#Illness_and_deat...

it's not clear if he had ATTR or AL amyloid

That was my first thought, too. I guess the condition's name is pretty memorable.

It's been almost twenty years, so it shouldn't surprise me that new research means that people who died in the past maybe could have survived longer if we had known better. And, of course, Jordan was going to die some day. But I certainly wish he'd had more time.

Another example of this is the famous mathematician David Hilbert. He came down with pernicious anemia in 1925 and suffered permanent damage, so much so that in the words of Eugene Wigner, "was hardly a scientist after 1925, and certainly not a Hilbert".

Today, PA is easily treated with vitamin B12 injections.

It's incredibly good to see Medicare covers these drugs, considering how expensive they are. Meanwhile, my diabetic friend can't get their insulin covered by Medicaid...

It's expensive because it's categorized as an "orphan drug". I have to wonder if the underestimation of the prevalence of CA was part of that.

Presumably your friend wants a particular brand of insulin, not the insulins that Medicaid covers because they are the most cost effective?

Medicaid negotiates with manufacturers to get the best price. When they are successful in securing a low price, they preferentially cover them.

Usually if the doctor can show why those brands aren’t good enough, Medicaid will cover alternatives that aren’t covered, but it can be a lot of work for the doctor.

> Presumably your friend wants a particular brand of insulin, not the insulins that Medicaid covers because they are the most cost effective?

I don't think they're picky about brand, I seem to recall that they didn't know about the cheap insulin at Fred Meyer when their Medicaid stopped covering what they were getting before. I think that insulin does work for them.

a) average age is 77

b) drugs cost a shitload (hundereds of thousands/yr) to extend lifespan by...months

c) only for ATTR (not AL) amyloidosis

d) the drive to diagnose and treat only really started after tafamidis (1st drug with any effectiveness) was marketed...hmmm

e) the dude in the article used as an example was probably helped more by treating his afib than by the fancy drugs

for sure there are some genetically transmitted younger patient for whom this is important. But there are a lot of frail older people who are getting diagnosed with wild-type ATTR amyloid for...questionable benefit at massive cost. IMO, the jury is still out

if you can't afford Tafamidis, you could probably get away with taking

- Flufenamic acid

- Valtoren (Diclofenac)

- Diflunisal

off-label.

https://www.benthamdirect.com/content/journals/cdtcnsnd/10.2...

IANAD but I believe that Valtoren has the least side effects, but in general since they're all NSAIDs they have been tested for long-term analgesic use, so they're relatively safe and quite inexpensive.

NSAIDs can kill your kidneys.

And your stomach lining. Even better if you’re already on a DOAC for Afib.

NSAIDs absolutely aren’t for long term use.

What's with the kneejerk takedown?

a) Yes, it's more common in older people. A lot of old people end up in hospital

b) 30% fewer deaths and hospital admissions is a good thing in my book

c) The more common form according to my wife

> The more common form according to my wife

Not sure what relevance the source has here, but it’s not correct. Primary (AL) is the most common in the developed world and secondary (AA) elsewhere. There are some foci of ATTR but it is by far not the most common.

I should have mentioned my wife is a physician, apologies. The true prevalence of ATTR is not known since it's only been investigated for recently as described in the article. If you look up recent data you'll see a big difference.

“Fewer deaths” is a meaningless concept. Every human being inevitably dies. If you prevent someone from dying today, you have only delayed the inevitable. In some cases this is extremely valuable; if you save a newborn baby, that baby could live eighty more years. If you save a 77 year old, they will not live eighty more years. And if you repeatedly save elderly people from natural conditions that could easily kill them, their quality of life gets worse and worse over time as their bodies wear out and decay and the side effects of these interventions build up. Which is why the vast majority of doctors have DNR’s.

While this is logical, the more diseases of old age we cure the longer and better the quality of life the elderly get, and treating amyloidosis is one small step towards that.

It depends; for instance there are a lot of cancer treatments that effectively replace a quicker and potentially comfortable death with a prolonged period of suffering. And my argument isn’t that these treatments are universally worthless, but that it’s perfectly fair to observe that their benefits are marginal at best.

It's all incremental statistical improvements, and that's a good thing. We used to live to 35 on average, now we live to 75 on average. That's amazing. It was done not by solving any one illness, but by solving them all in aggregate.

When the life expectancy was 35, nobody was dying of old age at 35. There were still septuagenarians. To provide an intentionally simplified example, a population where half of the people die of old age at 70 and the other half die in childbirth at age 0 has a life expectancy of 35. Even adding ten years to the life of every adult in that population only improves life expectancy by five years. Reducing infant mortality was a much better investment, though (fortunately!) we’ve been so successful at it that we may be at a point of diminishing returns.

All correct points that do not impact the core of the argument. It is all of it, in aggregate, that improves life expectancy. If you read literature, people who were older than 40 or 50 were 'wise old men', and 80 was a rare Methuselah, who was described as 100s of years old. Thanks to statins, and various cancer treatments, and stints, and blood thinners, and many other remedies, living to 80 is not rare, and most people who live beyond it can best be described as people who listen to their doctor and take their meds on time.

Even in today’s tech industry, people who are older than 40 or 50 are considered wise old men when they can get hired at all. But yes, we have extended adult lifespans, just not by 40 years which is the naive assumption many people make from the observation that life expectancy used to be 35. Also, I would expect that most of the extension in adult lifespan has less to do with old people having better medication than with broad declines in smoking and alcohol consumption. How long into your eighties and beyond you will live is largely determined by lifestyle decisions made in your teens, twenties, and thirties (though it is never too late to quit smoking!)

The drug: https://en.wikipedia.org/wiki/Tafamidis

>They are incredibly expensive, costing $250,000 to $500,000 per year.

No way they cost that much to make.

Big pharma is out of control.

The first dose costs billions to make, every dose after that might only cost a few cents. It all averages out.

From a study looking at cost-effectiveness of tafamidis:

> Orphan drugs enjoy substantial pricing power because there are few or no therapeutic competitors. As a result, discounts off the list price, if any, tend to be small. In a recent study of 50 patients receiving tafamidis, the mean (SD) cost of a 30-day supply was $23,485 ($2); the resulting annual cost of $281,820 is greater than the $225,000 list price we assumed. In fact, U.S. prices for specialty pharmaceuticals typically experience substantial year-on-year price increases during the period of market exclusivity.

I mean, we shouldn't be surprised what happens to prices when the law goes out of its way to create a monopoly.

https://pmc.ncbi.nlm.nih.gov/articles/PMC8170666/

You should've seen how unaffordable cars were 100 years ago.

The Ford Model T sold for $260 in 1925, which is $4,056 in 2021 dollars.

Prices were higher in 1922, and the Model T was basic and mass produced leading to a falling price.

Across the board, in 1922: https://www.1920-30.com/automobiles/1922-car-prices.html

> The Ford Model T sold for $260 in 1925, which is $4,056 in 2021 dollars.

I'm not sure I buy this conversion. It was targeting the middle classes (and on credit), and middle classes in 2025 could buy that cash. Working classes in 2025 could probably buy that cash, and definitely on credit.

The conversion isn't a hill I'd die on, it was sourced from a reddit economics thread as a bold assertion of fact:

https://old.reddit.com/r/AskEconomics/comments/q20fr4/the_fo...

The 1922 price list looks solid though, it's from a dedicated 1920s 'fan' site, but, again, I haven't personally verified or cross checked the numbers - it just seemed like an interesting bit of info to chase :)

To put a different perspective, the average American income in 1925 was $5,425. So buying a Model T (Touring) at that time would cost you 4.7% of your yearly salary.

In 2023-2024, the average car price was $48,274, and the median income was $80,610. It now costs a whopping 60% of your yearly salary.

You are missing the point, poster should have said 200 years ago. The point is that these treatments cost so much because they must be invented and tested. About 50% of that drug cost is the invention, 50% is the testing (in clinical trials) and .0000000001% is the cost to manufacture.

Why don't you make some and sell it for a mere 200k?

Because patents, despite publicly funded research.

Every damn time.

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