Why?

[KANSASTOM]

If you have only lower motor neuron involvement, then you have Spinal Muscular Atrophy, a pure motor neuropathy or some sort of other lmn disease. From what I have been reading these are disabling but not fatal. If you have pure upper motor neuron involvement then you have HSP or PLS and both of these are disabling but not fatal. Now if you have both then it is ALS and it is fatal. My question is why? Why is the sum of two non-fatal diseases fatal? I understand that the decline of respitatory function is what makes ALS fatal, but why wouldn't you have the same result with a pure lmn?

 

[PDaddy]

Wright?

That's a damn good question, if your assumptions are correct. Maybe Wright (does he have a first name, or is it Wright?) will shed some light...

 

[wright]

Hello Tom

Spinal muscular atrophy (SMA) is different than progressive muscular atrophy (PMA).

SMA is a condition found in infants, children and young adults. The time in the life that it is contracted dictates what type it is. They are categorized from Type I all the way to Type IV, with Type I being found in infants, Type II in children, Type III in teenagers and people in their twenties and Type IV is an adult form found in people older than 30. It is very, very, very slow in its progression and is typically not fatal.

PMA on the other hand, is fatal. This is a variant of ALS where there is solely lower motor neuron involvement. It's progression isn't typically as fast as ALS but the way it progresses is the same. It will march up and down limbs, have bulbar involvement and will make it's way to the respiratory muscles eventually.

There are certainly other conditions that cause lower motor neuron problems (peripheral neuropathy for example) that are not typically fatal, because they are not progressive (for the most part). They more or less hit you then stop. Flare-ups can certainly occur (and these flare-ups can be acute or chronic) but the progression isn't like ALS or PMA, which is relentless; once ALS or PMA start, they simply do not stop.

 

[KANSASTOM]

So it looks like the difference between an Adult form of Spinal Muscular Atrophy and PMA is the rate of progression.
In the early stages of the adult form of Spinal Muscular Atrophy or a pure motor neuropathy does the emg results look the same as ALS or PMA?

 

[joelc]

I sincerely believe ALS does not have to be fatal!

With the technology we have today we can live, breath and communicate just fine for a long long time! We just have to be willing to accept these helpful items!

I for one have accepted these and I plan on being around for at least another 20 years.

GOD BLESS YOU ALL!

 

[Al]

I'm sure Quadbliss would agree with you Joel.

AL.

 

[KANSASTOM]

I was very surprised tah there are alot of people that have had Als for a very long time. When I ask my neurologists how long did I have, he told me that he didn't have a crystal ball but the mean time was three years, I think he is way out of touch with reality.

 

[wright]

Joel, I agree fully. What I wrote about progression and what one sees written on progression, is when there are no medical interventions taken.

Quadbliss is a perfect example of someone who has lived a long time with ALS because he has chosen to use technology. I applaud him, you and anyone else who chooses to do so.

Having said that, I respect anyone's decision as to how they handle this disease. It is certainly a personal choice.

 

[Al]

Not really. The stats still say 80% of diagnosed people will be dead in 2 to 5 years. Three as a mean would not be far off sorry to say. Personally I'm coming up on 5 years. So I'm well past the mean. Still fighting.

AL.

 

[joelc]

Al, with all due respect those numbers would change significantly if more PALS chose to live by accepting either a PEG or tracheostomy and vent or both. Unfortunately it is still a very small number who choose these life saving items.

 

[Al]

For sure Joel but we have to try to educate the PALS and CALS here and elsewhere. There have been countless people here say that their Doctor said that it is not possible to keep a PALS at home without specialised care and help and they discourage them from it. Totally false. It can be done. It's not easy but it can be done. We now have a few trach patients and quite a few PEG's. A lot more than 4 years ago. People telling the good results here help. Thanks for sharing yours.

AL.

 

[CindyM]

So I'm well past the mean. Still fighting.

Amen, Al. And I am fighting for you and Lee in spirit! Cindy