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Atsugi

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I copied some statements (below) about life expectancy. Here’s what I think I know from the doctors and from helping my wife as she progressed:


In ALS, death usually comes from impaired breathing, especially aspiration pneumonia due to swallowing problems. If the patient lives long enough, paralysis of the expiratory muscles will allow CO2 to build up, so the patient sleeps longer and eventually the organs fail. Morphine prevents the sensation of air hunger and the anxiety associated with that.

For planning purposes, I wonder how useful these stats are. Many factors apply to each individual case. For instance:



- A trache and a PEG will help an ALS patient live longer, but will not slow the progress of paralysis.

- Bulbar onset patients tend to have shorter life expectancy.

- Young ALS patients tend to live longer.

- Familial ALS “usually” kills within one to two years after symptoms appear.

- If Riluzole is prescribed early and taken continuously, it delays the onset of ventilator-dependence or tracheostomy and may increase survival by approximately two to three months.

Watching my wife’s rapid progress (less than one year total), I opted NOT to ask the question “How long will I live until death?” Instead, I decided to ask the question: “How long before I lose the ability to communicate because I’m locked in?”



In my wife’s case, she chose to go quickly, without any tubes. She was locked in exactly one day, but was pretty well morphined up during that day.


When my time comes, though, I might ask the question “when can I choose to die on my own terms”?

FROM ALSA.ORG: “Although the life expectancy of a person with ALS averages about two to five years from the time of diagnosis, this disease is variable, and many people can live with the disease for five years and more. More than half of all people with ALS live more than three years after diagnosis.


“Half of all those affected live at least three years or more after diagnosis. About 20 percent live five years or more, and up to ten percent will survive more than ten years.”

FROM MDA.ORG: “Each person's disease course is unique. There are a number of examples of people who are leading productive and active lives more than two decades after an ALS diagnosis.


“Standard longevity statistics citing an average survival time of three to five years after diagnosis may be somewhat out of date because changes in supportive care and technology — especially for breathing and nutrition — may help prolong life.”

FROM THE CDC: “{Most people live from 3 to 5 years after symptoms develop. How long a person lives with ALS seems to be related to age; people who are younger when the illness starts live slightly longer.


“People with familial ALS usually do not fare as well as a person with ALS who are not related, and typically live only one to two years after symptoms appear.”

FROM ALSFROMBOTHSIDES: “It is important to note that these figures are based on patients who do not go on BiPAP or a full ventilator. Patients who opt for tube feedings and a ventilator when swallowing and respiratory muscles fail can generally be maintained for many more years. Life expectancy among these patients has not been reported, but the patient's age, other health problems and the quality of nursing care available will affect life expectancy.”
 

Nikki J

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Hi Mike

A couple of things I would like to comment on.

Bulbar is more common in older patients. I believe I read somewhere ( Emory series maybe) when adjusted for age it is not true their survival is shorter.

Familial ALS is not one entity. It depends on your genetic variant what the average survival is. Some forms of SOD1 are brutally swift and many so called FALS statistics are based on SOD1. An example of slow FALS is the very rare type that Anthony of Ellen fame has. Your wife went very quickly for C9 one series said the average is about 2.6 years for C9

Riluzole should more correctly be described as extending life by 10 percent. How long that might be depends on the inherent rate of progression. Slow progression longer benefit. Early prescription is important you are right. I read somewhere the transport mechanisms into the brain become impaired with progression so it works less well. Someone on this board once posted they were told very early prescription might have a benefit as much as 25 per cent.
 

Atsugi

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Thanks, Nikki. I know you get your knowledge from very good sources. More the better.

I'd like to incorporate your knowledge into my post, but when I try to edit a very long post, the system presents an empty box.

Feel free to correct or alter my post as you see fit. --Mike
 

Green Queen

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I have just been told by my neurologist that there's an average life span of 19 years for PLS, the range 4-35 years. From onset. I checked these against the Pringle thing, which seems to be what people read and quote from.
It's not fair. For all of us.

Doesn't matter how you look at it, MND is just not fair.

God bless, Janelle x
 

lgelb

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I posted a better abstract not too long ago, Mike, as compared with the warmed-over generalities, updated once in a blue moon, on the major sites. I don't think they serve our community as well as more recent literature, to be honest, and their very similarity can be discouraging, esp. to newbies. For example, Nikki and her sister are testaments to the fallacy of the 1-2 year theory in familial variants, of which there are obviously several that cannot be generalized.
 

Atsugi

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Thanks for posting that abstract here. That one and others referenced on the same page offer a lot of insight. But they are perhaps too hard for the average reader. I wish someone had the time to summarize the best literature for the average reader.

As I wrote: "I wonder how useful these stats are?" I provided the quotes as the best thing available to the average reader.

Many people ask the question regularly and it would be good if there were some sort of answer we could give that would be more useful than the quotes I provided.
 

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Mike and all thank you for the post and comments. Life and quality of life are two uniquely different things that for me cause my biggest struggle. I am no where near as far along as many here and yet I cannot see wanting to prolong things even where I am at. This becomes the real problem with the life expectancy figures. They are simply statistical facts that do not take into account (nor could they) the extreme difficulty of daily life with this disease. I would love to know how long I have, at the same time the question becomes mute when most days are intolerable.
 

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Pete brings up what I consider a very key point that makes stats like this so difficult to interpret or apply to an individual case and that is "quality of life" and the decisions made for that reason. Our fALS is TARDBP and I have watched so many of our family go through it. But each case is has been different... different onset, different ages, different "quality of life" decisions. I have family members right now who were diagnosed in 2010, 2011, and 2012 who are still living. My Mom on the other hand was respiratory and bulbar onset, diagnosed in late 2000 and died in May of 2001. The decisions she made were based solely on quality of life so while she did have a PEG I seem to remember her decision was based more on helping her take meds. She didn't use her bipap but she did use oxygen for a time. She refused a vent from the beginning.

Just looking at the stats from my family, and knowing a bit more of the personal stories, I don't pay much attention to the published stats. My wife went through breast cancer a few years back and after her surgery she made her treatment decisions based on her quality of life, not life expectancy stats. In our discussions about ALS we have both agreed that if I am diagnosed with ALS we will not ask the "how long" questions but base all of our decisions on our quality of life. And Pete is right, it's mute when life becomes intolerable...
 

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I agree with Pete and Bill. The question should not be how long but how well? And circumstances vary so much that was is intolerable to one of us may be quite acceptable to another even as we all wish to be free of any of this

Asking how long and combing the statistics is common and probably a form of the bargaining phase of the grief process but it is ultimately pointless somewhat like another common question. Why did I get this? We get periodic threads on that too- was it where I worked, lived, was it what I ate or how I exercised. There are not good answers to that yet either unless you are FALS
 

BlueandGold

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Yes and no Nikki. For some of us having some sort of life expectancy can help with preparations and specifically with choosing to exercise an Accelerated Death Benefit Rider on a life insurance policy as most won't pay unless you are given 12 months to live.

Vince
 

Atsugi

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Good points, all.
Great new information, Vince.
 

Nikki J

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I can see that Vince but things are so variable that these numbers mean very little. Your best information would come from a good neuro who has seen you or at least has records over a number of months. If my family members had taken that 2.6 years statistic it would have been way off base. So far everyone has lasted years beyond it and my sister is at 4 years. I am hoping to do the same
 

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Yes and no Nikki. For some of us having some sort of life expectancy can help with preparations and specifically with choosing to exercise an Accelerated Death Benefit Rider on a life insurance policy as most won't pay unless you are given 12 months to live.

Vince

Vince, we found that with ALS diagnosis our doc would sign a letter saying 12 month or less, but the interest charged on Accelerated Death Benefit was 10% a year. Hoping for a longer ride, we looked elsewhere for ready cash.
 

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I completely agree that it is the "how long will I have the quality of life that makes me want to wake up in the morning" question that is paramount. We didn't have any life insurance on Larry to worry about, though, since he was uninsurable from birth.

ALS docs are generally willing to supply more personalized prognostic information from their broader perspective, particularly if asked more than once. So those of you with a financial need for better info should ask early and often to be informed.

I thought my original summary was decent, so for those who don't want to go back through that thread:

In the Emory series, median [better measure than "the average" in this context; half did better, half worse] overall survival was 30 months from symptom onset, 16 months from diagnosis, and 14 months from the initial clinic visit. Combining this and other studies, the FRS and age (older fares worse) when you are diagnosed, your baseline FVC and early weight loss and whether or not it was a quick diagnostic journey (quick generally means more severe onset) are the most correlated w/ survival.

Bulbar onset has also been an unfavorable factor in some earlier studies, but I think that may change somewhat (or has) as BiPAP becomes easier to access early -- hopefully!

Anyway, FWIW, I continue to believe that the quantity of quality life, if you will, is heavily influenced in the "susceptible" by nutrition (what you eat, not just how much), selected supplements that mitigate suboptimal dietary intakes/hypermetabolism, respiratory support as many hours and to the extent needed (no more; too-high settings make things worse), sufficient rest/deep sleep (bed, BiPAP, joint support all critical), range of motion exercise/massage to reduce pain and improve microcirculation, even things like fresh air and sun.

And doing something while awake that you want to, as opposed to waiting out the day/waiting for the end.

But I also think there is a fast-progressor group, that is less susceptible. Most people know fairly early what group they're in. As others have pointed out, you can plot your FRS on PLM and compare, but it's a wacky measure.

I'm sure I started off with a point here...maybe it's this: you can start off thinking you have no control and any improvement in your quality of life is a bonus, or you can begin with the assumption that you have some. The latter group, in pretty much any non-comatose condition, will do better, and ALS is no exception.
 
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