That was incredibly inspiring, and incredibly depressing.
As a Drupal fanatic who built my career on it, and a person also dealing with a terminal illness (though I have a couple years left, if all goes well), it's upsetting to see that someone who gave me so much, now has to go through all that. I feel genuine pain and am quite legitimately upset now. I wish Aaron and his family all the best (considering).
I've occasionally asked other people what they thought the worst problem in the world was. Nobody has ever answered with my answer: that all of our loved ones are going to die. The utter inevitability of death within a few decades of birth is so thoroughly taken for granted that people are vastly more concerned about the NSA or carbon dioxide than about literally saving the lives of everyone they care about.
We need a working backup and recovery plan for people as soon as possible. How soon it will happen will probably depend a great deal on how soon a critical mass of people can be made to see it as an actual engineering problem urgently requiring a solution.
(I'm unsure whether Aaron got a response from Hal.)
Hal's response to his diagnosis has been defiant. He got the tracheotomy and is determined to live a long, productive, and worthwhile life.
I have great reverence for Aaron's concern for the burden he would be on his family, and I certainly don't claim to understand what it would be like to be in his shoes. But I personally am a big supporter of Hal's philosophy. I encourage Aaron and other ALS sufferers to consider this path. Never in history has an intellectually constructive and satisfying life with ALS been as technologically feasible as it is now.
[Edited to reflect the first link.]
Edit 2: It's also worth noting that ALS is what has crippled Stephen Hawking. He got a tracheotomy in 1985, at which point his "A Brief History of Time" was only partially completed. He finished it, and has made many important professional contributions since.
Having lost a grandfather and an aunt to ALS, I can completely relate to what this family is going through. It is heartbreaking, and emotionally draining to watch someone go through it.
It also scares the crap out me knowing this is a genetic disease and two of my family members have already succumbed to it. This is known as Familial ALS. Genetically speaking, it comes down to a 50/50 chance I could get it. I can get screened to know for sure if I'll get it, but it requires you do counseling before you even take the tests, considering how it could change your life overnight.
- Familial ALS- occurring more than once in a family and accounts for 5 to 10% of all cases.
- In 1991 a team of ALS Association-funded researchers linked familial ALS to chromosome 21. In 1993 the research team identified the precise defect, a change in the DNA for the protein called copper-zinc superoxide dismutase 1 (SOD1). Researchers since have found more than 100 mutations in different places in the coded DNA instructions for making SOD1.
It's frustrating because it seems like every time they get close to finding THE mutated gene, they identify several hundred others. It's like a game where the goal posts in a game are constantly changing.
I'm a SOD1 researcher and my heart goes out to you and the many patients suffering with this terrible disease and their families. Though rest assured, we're working hard to uncover why this happens and what we can do about it.
I just wanted to clarify some points. I'm not sure what you mean about "the mutated gene". SOD1 mutations definitely cause ALS. It's just that there isn't a single SOD1 mutation that's responsible. Over 100 different mutations of the same gene can lead to the disease. Other genes are also implicated in ALS, such as TDP-43 and FUS. Sure there are other genetic factors responsible for ALS, many of which are yet unknown. But these are known factors.
I would be very hesitant to sequence my SOD1 gene to be honest. Let's say it came up with a new mutation. Not all mutations will lead to disease. So while this may not cause you to get ALS in the long run, it may lead to you worrying incessantly as you get older. Furthermore, most of these 100+ mutations have only been found in a single family or even a single patient. It's quite possible there are other genetic polymorphisms in these families that make these patients susceptible to their SOD1 mutations. Also there are examples of SOD1 mutations that aren't 100% penetrant. That is if you have the mutation, you aren't 100% guaranteed to get the disease. For instance, in scandinavian populations the aspartate 90 to valine mutation is recessive.
Basically there are a lot of complicating factors. Even if your SOD1 gene comes back as the normal sequence, you couldn't know you were safe. That is unless you knew that your grandfather and aunt actually had SOD1 mutations. Of course these are only my opinions as a scientist. I am not a physician.
So... do the counseling! Knowing that the answer is out there for the taking is already changing your life, probably for the worst. "What is true is already so. Owning up to it doesn't make it worse..."
I couldn't imagine preparing yourself with counselling just to prepare to find out if you have ALS or any other life changing disease. That thought just blows me away.
I remember a couple of years ago coming out a clinic where I was told I have a kidney disease and if not treated within the next half decade I could die of kidney failure in the next 15 to 20 years and remember feeling pist off and indignant about it like: "Dammit! Why me? I'm a good person. I didn't do anything majorly wrong in life!" and now that I'm 4 1/2 months into my treatment I am being humbled by amazing people who have been dealt a much, much, much worse hand in life who have a better outlook on life.
I sure hope you don't get it. And whether or not you believe in God, I say to you, God be with you, and may he watch over you and your family.
That video is heartbreaking. "You may have heard of her, her name is Wonder Woman", tears.
He lived in a Buddhist monastery at one point; life is suffering. He'll soon leave his loved ones, and he knows it's very much sooner than later.
What can you do in such a situation? Clinging to life, little time remaining, what to do? I thought something ridiculous, like go on retreat until the end, but right where he is, with his wife and kids is probably just perfect.
They're all being transformed by the experience. Yours is not to reason why rings true. Who's to say that an "early" death is not in fact a gift for the living?
Apologies to anyone offended by my views, this struck an unintended chord for some of you, was just expressing myself in the moment based on my own experience of the death of a loved one.
That's really where you get to experience the emotions that make you happy to be alive. Joy, love, hope, desire, anger, frustration, etc. I'll say that a NEAR death experience tends to be more of a gift, it happened to my brother and changed him drastically. Not all for the better, but much of it was good.
I think the idea of calling death a gift is bullshit. Yes, you can learn and grow from the death of a loved one, but that isn't a gift, it's the absence of their presence (the real gift) that makes you realize what you had.
To me, life without the lows and highs, is boring. That is because lows make people realize how special the high points are. But I know other people who would be content without either.
In some sense everything is a "gift" because all the lessons we learn lead us to some ultimate spiritual goal. That said, I don't think "gift" is the best word and prefer "lesson" instead.
Would you apply the word "gift" to any horrible genocide/natural disaster that has happened? Perhaps you would, but for most people, "Lesson" will resonate better.
The research lab that Jamie founded is ALS TDI [1], which has a database of ALS clinical trials [2]. Jamie later co-founded PatientsLikeMe, a web site for sharing medical data which has over 6,000 users who are patients with ALS [3], the first of whom was his brother Stephen [4].
Maybe he could get into a clinical trial with NeuralStem Inc's therapy. They seem to be having some good results with treating people with ALS with spinal stem cell injections: http://www.neuralstem.com/cell-therapy-for-als
The body is still such a mysterious device. It's so frustrating that we still can't figure out diseases like ALS. The greatest invention would be a body debugger.
Still pretty barbaric compared to what we'll see in the future, but useful while we drag ourselves forward.
Anecdote: I was bit by a tick about 2 weeks ago. To get a lyme disease/meningitis test done, I had to pay my copay ($30) at my primary doctor (for him to approve the tests) and then had to make another appointment to have my blood drawn and tested by Lab Corp (~$200). I then had to wait for my doctor to call my back because Lab Corp would not provide my results directly to me (!!!).
It's probably just a matter of figuring out how to funnel enough money and people on the problem. Smartphone advances, for example, will be incredible over the next decade. Much better graphics, CPU, batteries, etc. That's because there's a huge market for them. Another example is 4k TV's, which are out of reach. Within 5 years, they'll be cheap, and a lot better.
Figure out a way to have consumers directly spend more money on medical research/technology and great things will happen.
The question of people being frozen in the hope of being revived is mentioned in a very peculiar way in the "Transmetropolitan" comics (by Waren Elis) . I really hope for Mr Winborn that the future's going to be a better place :))
Perhaps I am a pessimist, but I don't see the future turning out well for those who have chosen to cryogenically preserve their bodies.
That's not to say that the people practising it don't believe in what they're preaching - I'm sure many people involved in the cryonics industry deeply believe in the technology. But consider this: what value is there in reviving the dead and frozen rather than preserving the living?
As it stands today, cryonics is an act of faith, not science. And you're more than welcome to believe in it, just like any other faith, but I personally don't see why future society would put the needs of the frozen above those of the living. And unlike some other faiths, it's a very expensive option to partake in.
My brother was diagnosed with this awful disease about a year ago. He's 45 and has two children a few years older than Aaron's, so this definitely hits home. He already has a very hard time speaking, which means the disease started off by attacking his respiratory system. Aaron seems to be doing OK in that regard, but who knows how quickly that will go.
What's frustrating is that he can't qualify for experimental treatments unless his lung capacity is at a certain level (e.g. Min. Vital Capacity here: http://www.alsconsortium.org/trial.php?id=1). This is one reason why we need to get more money into ALS research and treatment. Please consider a donation to the ALS Association at http://www.alsa.org/. Thank you.
Early this year my father was diagnosed ALS. During one year his hand muscles are completely gone. Though the legs are still OK but the disease does progress rapidly and it's just matter of time.
This is terrible disease. I know how hard it is for his family.
The problem with web filters is ████████████████████████████████████████. I didn't see any porn there, (and I'm fairly confident that I would have been able to recognize it.)
"Find the nearest intelligent life form" ...that is not carnivorous and is not interested in our resources and is not interested in exterminating us for whatever reason.
I think it would be nice if we could do something like polymath (from Terry Tao) for medicine. Maybe have a combination of medical experts + statisticians + bright people collaborate on a large scale to tackle some of the health problems out there.
My grandfather died of ALS a few years ago in his late 70s. It's sad and ironic, but he was a runner until the last three or four months of his life, when the disease rapidly crippled him and took his life. He was the sort of guy you would expect to live well into his 90s: He ate a perfect diet and exercised like he was 35. At the time, my grandmother was battling bone cancer, and we were quite concerned that my grandfather would be depressed living for multiple decades without his wife. Unfortunately and surprisingly, he died almost two years before my grandmother.
This article was very sad, moving and, in a sense, motivational. Thinking about Aaron's situation makes me put all of my problems in perspective and realize how trivial most of them are.
I hope we can figure out the mechanics here of ALS and reverse or arrest them. I was reminded of the article below I read in Scientific American where questions are asked about Stephen Hawkings success in staying alive.
Dear Aaron, you've probably heard about the Neuralstem and their NSI-566 product which is still in the trial phase. But in case you haven't here is the info:
http://www.neuralstem.com/cell-therapy-for-als
[+] [-] Andrenid|12 years ago|reply
As a Drupal fanatic who built my career on it, and a person also dealing with a terminal illness (though I have a couple years left, if all goes well), it's upsetting to see that someone who gave me so much, now has to go through all that. I feel genuine pain and am quite legitimately upset now. I wish Aaron and his family all the best (considering).
[+] [-] SiVal|12 years ago|reply
I've occasionally asked other people what they thought the worst problem in the world was. Nobody has ever answered with my answer: that all of our loved ones are going to die. The utter inevitability of death within a few decades of birth is so thoroughly taken for granted that people are vastly more concerned about the NSA or carbon dioxide than about literally saving the lives of everyone they care about.
We need a working backup and recovery plan for people as soon as possible. How soon it will happen will probably depend a great deal on how soon a critical mass of people can be made to see it as an actual engineering problem urgently requiring a solution.
[+] [-] jessriedel|12 years ago|reply
https://bitcointalk.org/index.php?topic=155054.100
http://www.finney.org/~hal/
http://www.noozhawk.com/local_news/article/101710_hal_finney...
(I'm unsure whether Aaron got a response from Hal.)
Hal's response to his diagnosis has been defiant. He got the tracheotomy and is determined to live a long, productive, and worthwhile life.
I have great reverence for Aaron's concern for the burden he would be on his family, and I certainly don't claim to understand what it would be like to be in his shoes. But I personally am a big supporter of Hal's philosophy. I encourage Aaron and other ALS sufferers to consider this path. Never in history has an intellectually constructive and satisfying life with ALS been as technologically feasible as it is now.
[Edited to reflect the first link.]
Edit 2: It's also worth noting that ALS is what has crippled Stephen Hawking. He got a tracheotomy in 1985, at which point his "A Brief History of Time" was only partially completed. He finished it, and has made many important professional contributions since.
http://www.hawking.org.uk/living-with-als.html
[+] [-] at-fates-hands|12 years ago|reply
It also scares the crap out me knowing this is a genetic disease and two of my family members have already succumbed to it. This is known as Familial ALS. Genetically speaking, it comes down to a 50/50 chance I could get it. I can get screened to know for sure if I'll get it, but it requires you do counseling before you even take the tests, considering how it could change your life overnight.
Here's some facts about Familial ALS:
http://www.alsa.org/als-care/familial-als/familial-als.html
- Familial ALS- occurring more than once in a family and accounts for 5 to 10% of all cases.
- In 1991 a team of ALS Association-funded researchers linked familial ALS to chromosome 21. In 1993 the research team identified the precise defect, a change in the DNA for the protein called copper-zinc superoxide dismutase 1 (SOD1). Researchers since have found more than 100 mutations in different places in the coded DNA instructions for making SOD1.
It's frustrating because it seems like every time they get close to finding THE mutated gene, they identify several hundred others. It's like a game where the goal posts in a game are constantly changing.
[+] [-] ckayatek|12 years ago|reply
I just wanted to clarify some points. I'm not sure what you mean about "the mutated gene". SOD1 mutations definitely cause ALS. It's just that there isn't a single SOD1 mutation that's responsible. Over 100 different mutations of the same gene can lead to the disease. Other genes are also implicated in ALS, such as TDP-43 and FUS. Sure there are other genetic factors responsible for ALS, many of which are yet unknown. But these are known factors.
I would be very hesitant to sequence my SOD1 gene to be honest. Let's say it came up with a new mutation. Not all mutations will lead to disease. So while this may not cause you to get ALS in the long run, it may lead to you worrying incessantly as you get older. Furthermore, most of these 100+ mutations have only been found in a single family or even a single patient. It's quite possible there are other genetic polymorphisms in these families that make these patients susceptible to their SOD1 mutations. Also there are examples of SOD1 mutations that aren't 100% penetrant. That is if you have the mutation, you aren't 100% guaranteed to get the disease. For instance, in scandinavian populations the aspartate 90 to valine mutation is recessive.
Basically there are a lot of complicating factors. Even if your SOD1 gene comes back as the normal sequence, you couldn't know you were safe. That is unless you knew that your grandfather and aunt actually had SOD1 mutations. Of course these are only my opinions as a scientist. I am not a physician.
[+] [-] crpatino|12 years ago|reply
[+] [-] adventured|12 years ago|reply
[+] [-] racl101|12 years ago|reply
I remember a couple of years ago coming out a clinic where I was told I have a kidney disease and if not treated within the next half decade I could die of kidney failure in the next 15 to 20 years and remember feeling pist off and indignant about it like: "Dammit! Why me? I'm a good person. I didn't do anything majorly wrong in life!" and now that I'm 4 1/2 months into my treatment I am being humbled by amazing people who have been dealt a much, much, much worse hand in life who have a better outlook on life.
I sure hope you don't get it. And whether or not you believe in God, I say to you, God be with you, and may he watch over you and your family.
[+] [-] virtualwhys|12 years ago|reply
He lived in a Buddhist monastery at one point; life is suffering. He'll soon leave his loved ones, and he knows it's very much sooner than later.
What can you do in such a situation? Clinging to life, little time remaining, what to do? I thought something ridiculous, like go on retreat until the end, but right where he is, with his wife and kids is probably just perfect.
They're all being transformed by the experience. Yours is not to reason why rings true. Who's to say that an "early" death is not in fact a gift for the living?
[+] [-] bcgraham|12 years ago|reply
Everyone who's experienced it?
[+] [-] nova|12 years ago|reply
You've got to be kidding. (A very dark kind of humor, I guess)
[+] [-] virtualwhys|12 years ago|reply
[+] [-] ars|12 years ago|reply
That's horrible. Life is not suffering, life is joy and happiness. Does Buddhism really believe such a terrible thing?
[+] [-] bargl|12 years ago|reply
The time we get with our loved ones is the gift.
That's really where you get to experience the emotions that make you happy to be alive. Joy, love, hope, desire, anger, frustration, etc. I'll say that a NEAR death experience tends to be more of a gift, it happened to my brother and changed him drastically. Not all for the better, but much of it was good.
I think the idea of calling death a gift is bullshit. Yes, you can learn and grow from the death of a loved one, but that isn't a gift, it's the absence of their presence (the real gift) that makes you realize what you had.
To me, life without the lows and highs, is boring. That is because lows make people realize how special the high points are. But I know other people who would be content without either.
[+] [-] VMG|12 years ago|reply
[+] [-] darkarmani|12 years ago|reply
Gifts are generally things given willingly. I think you might want to rephrase.
[+] [-] unknown|12 years ago|reply
[deleted]
[+] [-] dtauzell|12 years ago|reply
Would you apply the word "gift" to any horrible genocide/natural disaster that has happened? Perhaps you would, but for most people, "Lesson" will resonate better.
[+] [-] mtraven|12 years ago|reply
[+] [-] andrewem|12 years ago|reply
(Full disclosure: I work for PatientsLikeMe.)
[1] http://www.als.net/ [2] http://www.als.net/ALS-Research/ALS-Clinical-Trials/ [3] https://www.patientslikeme.com/conditions/9-als-amyotrophic-... [4] https://www.patientslikeme.com/patients/view/40
[+] [-] narrator|12 years ago|reply
[+] [-] msie|12 years ago|reply
[+] [-] toomuchtodo|12 years ago|reply
Imaging + Genome Sequencing + Blood Tests
Still pretty barbaric compared to what we'll see in the future, but useful while we drag ourselves forward.
Anecdote: I was bit by a tick about 2 weeks ago. To get a lyme disease/meningitis test done, I had to pay my copay ($30) at my primary doctor (for him to approve the tests) and then had to make another appointment to have my blood drawn and tested by Lab Corp (~$200). I then had to wait for my doctor to call my back because Lab Corp would not provide my results directly to me (!!!).
Like I said. Barbaric.
[+] [-] melling|12 years ago|reply
http://en.wikipedia.org/wiki/Gross_world_product
It's probably just a matter of figuring out how to funnel enough money and people on the problem. Smartphone advances, for example, will be incredible over the next decade. Much better graphics, CPU, batteries, etc. That's because there's a huge market for them. Another example is 4k TV's, which are out of reach. Within 5 years, they'll be cheap, and a lot better.
Figure out a way to have consumers directly spend more money on medical research/technology and great things will happen.
[+] [-] ttrreeww|12 years ago|reply
[+] [-] bsaul|12 years ago|reply
[+] [-] objclxt|12 years ago|reply
That's not to say that the people practising it don't believe in what they're preaching - I'm sure many people involved in the cryonics industry deeply believe in the technology. But consider this: what value is there in reviving the dead and frozen rather than preserving the living?
As it stands today, cryonics is an act of faith, not science. And you're more than welcome to believe in it, just like any other faith, but I personally don't see why future society would put the needs of the frozen above those of the living. And unlike some other faiths, it's a very expensive option to partake in.
[+] [-] andyjohnson0|12 years ago|reply
[+] [-] indrax|12 years ago|reply
But it can't go on, this is not a stable or reliable way to fund cryonics.
Sooner or later the novelty will wear off and the money will run out, or someone just won't be sympathetic enough, and the goal will not be met.
Life insurance is the way to handle this for most people. Charity needs to be for the exceptional cases.
[+] [-] webXL|12 years ago|reply
What's frustrating is that he can't qualify for experimental treatments unless his lung capacity is at a certain level (e.g. Min. Vital Capacity here: http://www.alsconsortium.org/trial.php?id=1). This is one reason why we need to get more money into ALS research and treatment. Please consider a donation to the ALS Association at http://www.alsa.org/. Thank you.
[+] [-] grigy|12 years ago|reply
This is terrible disease. I know how hard it is for his family.
[+] [-] RKearney|12 years ago|reply
[+] [-] fnordfnordfnord|12 years ago|reply
[+] [-] marcosscriven|12 years ago|reply
I can't comment on his blog directly, so my answer to him is:
Find the nearest intelligent lifeform to our solar system, and let them know exactly where we are, and that we'd like to have a chat please.
[+] [-] Stranger2013|12 years ago|reply
[+] [-] psb|12 years ago|reply
[+] [-] alexvr|12 years ago|reply
[+] [-] hawkharris|12 years ago|reply
[+] [-] unknown|12 years ago|reply
[deleted]
[+] [-] ChuckMcM|12 years ago|reply
http://www.scientificamerican.com/article.cfm?id=stephen-haw...
[+] [-] rikelmens|12 years ago|reply
[+] [-] Eliezer|12 years ago|reply
[+] [-] kareemm|12 years ago|reply
https://webcache.googleusercontent.com/search?q=cache:5_8ePS...
[+] [-] beala|12 years ago|reply
[+] [-] Aardwolf|12 years ago|reply