21 comments

  • sleepyguy 11 hours ago ago
  • dogmatism 6 hours ago ago

    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

    • quantumwoke 5 hours ago ago

      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

      • epcoa 4 hours ago ago

        > 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.

        • quantumwoke 3 hours ago ago

          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.

      • philwelch 2 hours ago ago

        “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.

        • lokrian 43 minutes ago ago

          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.

        • Larrikin 2 hours ago ago

          It would be unethical to not email this comment to everyone in your family and friends that could possibly consider you as a person on their emergency contact list.

    • dnautics 6 hours ago ago

      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.

      • jamiek88 11 minutes ago ago

        NSAIDs absolutely aren’t for long term use.

      • cake-rusk 5 hours ago ago

        NSAIDs can kill your kidneys.

        • epcoa 4 hours ago ago

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

  • pfdietz 10 hours ago ago

    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...

    • maxlybbert 5 hours ago ago

      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.

    • dogmatism 6 hours ago ago

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

  • LoganDark 8 hours ago ago

    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...

    • refurb 30 minutes ago ago

      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.

    • pfdietz 6 hours ago ago

      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.

  • snvzz 2 hours ago ago

    >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.

    • zozbot234 2 hours ago ago

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

  • cma 8 hours ago ago