What can medicine do for people with genes for ALS?

This is not easy for people of limited means, hence a federal law enshrining people’s civil rights over their own existence.

Mostly technically true. Once you're locked in, or paralyzed, it becomes quite more difficult.

But right now, most suicide methods that are available live in the less pleasant octants of the availability/suffering/uncertainty space. We used to have some fairly pleasant -- by comparison -- deaths available to us but they're getting harder to access, leaving the nasty route behind. Coal gas? Gone the way of Plath. Barbiturates? Out-moded now, know a vet? It isn't like you can nip down to the corner for some laudanum like back in the day.

Shockingly, this is not a legitimate method of euthanasia. At the point that you’ve defrauded an autopsy to that degree, you could just have a doctor shoot them in the head and write heart attack on the death certificate.

This is usually painful and unpleasant, and also difficult for people with serious impairments (like ALS, for example). Perhaps euthanasia should be legal and perhaps not, but in any case the quick and painless death available via injection is very different from the death you would experience from hanging yourself (for example). There are dignified deaths and undignified deaths, and I think we should be clear about this distinction.

There is a word for killing someone against their will. It is murder. You may be surprised to find that murders still happen despite it being illegal pretty much everywhere.

We allow euthanaisa for pets because it is more humane than letting them suffer. For some reason that doesn't apply to people?

Anyway, I am guessing you have not witnessed someone you care about slowly die in agony. The theoretical abuse angle doesn't hold up to that reality.

That coin-flip-at-worst still probably gives one a better than evens chance of falling on the side of the average life expectancy where folks don't live to enjoy a long healthy retirement, for example.

You might, though, so you can't burn it all either.

There's also some added complexity that comes along for the ride when choosing to have children or not, saddled with a family history of nasty, fairly early onset baggage.

Another commenter said that embryo selection can help in that regard, which is an interesting angle that I hadn't considered.

What's the common factor - are they all autoimmune diseases?

Would the "inverse vaccine" approach discussed here recently help?

Honestly my takeaway from the article, and from a lot of the comments here, is that we should be (but aren't) updating our definition of "disease" for some diseases as our understanding grows.

If you have partially blocked arteries, you're not actively suffering from heart disease, but it's normal to give that person drugs and advise lifestyle changes as soon as that's detected. You don't wait for them to have a heart attack.

Of course, the cause and effect is much better understood with heart attacks and isn't at all clear with ALS. But the fact that ALS doctors were bewildered that someone might want to take prophylactic measures says to me that they have some blinders on.

Imagine getting some tests done, learning your coronary arteries are 60% blocked and your LDL levels are at the 98th percentile, and having a cardiologist tell you "Don't think about it for now, come back once your heart stops beating".

"Right to try" might be worthless in the last case. (Unless there's reason to believe that it's reversible with some as-of-yet uninvented treatment.)

I specifically addressed timing of onset being important in my original comment, because that matters to the person living with the problem.

We don't simply don't know that. There is an incredibly wide spectrum of phenotypes, even for SOD1 mutations (~2 of cases) the life span could be as short as 6 months and as large as 25 years.

There are ~120 gene mutations that are incriminated in what scientists call ALS. Each of them has dozens alleles on average.

In addition there are associations between ALS and dozens of occupations, and exposure to thousands of substances. The scientists tell of multi-step diseases.

You are only right in the sense that we will all die at the end.

In fact, we know that ALS is a complex disease because we know that common genetic variants influence ALS risk, but that doesn’t mean that every monogenic ALS variant is going to have its penetrance and onset modified to the same degree. So by saying that ALS is a complex disease I don't mean to take away from the fact that for some families, their causal variant may have a consistent effect with only minimal variation from the genetic background.

I’m fully supportive of preventive therapy. I think that is the actual future of medicine. And so I am a big fan of the idea of using genetics to identify a population at risk, doing individual biochemistry (or whatever endophenotype people use - perhaps imaging or other measurements) to see if the pathophysiologic process is occurring, and treating to prevent disease.