Bulbar Onset Decline

[Graybeard]

Looking to the future, I need to know the chances I will lose use of my arms and legs. I know every case is different, but I would like to know the odds.

So far, only my right hand is affected. I still type with it, but cannot do buttons, and soreness is creeping up my thumb. My ability to enunciate words is failing, and my diaphragm no longer responds to the phrenic nerve.

 

[Nikki J]

I am not sure that there are any statistics on this. On this board we have seen both ends of the spectrum including one woman who reportedly walked over a mile the day before she died. I would think the variables are breathing ( rate of decline modified by chosen level of respiratory support) vs rate of progression in the limbs and as you know all of these vary from person to person plus our progression rates can fluctuate over time. The other variable is nutrition but I believe you have a feeding tube?

 

[bigmark1954]

I think everybody wants to find a common denominator, in regard to progression, to put a time line on progression. That was my biggest question for the first several months on this forum.
I was diagnosed about 10 months ago, and have many of Greybeard's symptoms. He has more advanced respiratory issues though.
This disease can be similar in most PALS, yet the dynamic of progression can be so variable.

 

[davbo49]

i think it's like one of max rules. not to be moving around so much. muscles are not building up any more and what you do have your using them up. my hands started on me they was doing funny things at the first. i could open things but i would not be able to open my fingers back out they would not move. then 2 years latter my arms went. what i had in my hands was gone. i could still walk i was running my rc rig up and down trails on the weekends. we would go for 2 to 10 miles at a time long as the rigs would hold up. after i lost my arms it seem like every time we went out. my lags was not doing as good i would have to set more. well there is that rule. my als team is working with another one that's been fighting this now for over 20 years. it don't hit us the same.

 

[Graybeard]

Yes, Nikki, eating and drinking are highly overrated; I'm on the tit (tube). I lost 66 pounds before, and have gained 11 back. I did have a little yam, cranberries and pumpkin pie yesterday, but they didn't give any pleasure.

I will try everything short of a vent to extend my life. I even have substantial financial incentive to stay around - for my family. What concerns me now is possibly needing a lift to get from bed to bathroom.

 

[gooseberry]

Graybeard, my husbands breathing is quite compromised as he is technically classified as bulbar onset but presented as a respiratory onset case. Confusing but just the way our clinic does things. His phrenic nerve is pretty shot since he presented in May with a paralyzed left diaphragm and issue swith the right lung. Fvc is pretty low. He is losing limb function now. He has substantial atrophy throughout his body. His left shoulder is mostly unusable. The muscles have atrophied and are stretching the tendons an dligaments that hold the joint together. They say this is what gives him all the pain so he takes tramadol for it and hot warm moist compresses. His right hand has lost most of his grip and he cant write more than 2words. His gait is.very unsteady. Outside of the breathing issues, These are all changes since clinic in August.

I think some people progress quickly and some slowly. My husband was well muscled in his legs 3 months ago. Now he has atrophy in his feet,calves, along the shinbone, thighs, buttocks...this disease has a mind of its own and we are all along for the crazy ride!

Hang on tight Graybeard and try to enjoy each day. There are things to enjoy and be thankful for each day. Sometimes we have to look hard for them but we can find them!

 

[affected]

Graybeard I'm sorry I can't quite remember where you mobility is at.

If you are worried about this, you should be proactive and start looking at equipment for transferring like a hoist. They are so easy to use and honestly so much safer for both PALS and CALS.

 

[4tloml]

Been wondering the same, Graybeard. Like yours, my husband's right hand is affected, speech much more so, and phrenic nerve not good (right diaphragm partially paralyzed/atrophied). Just wish there was a guide better than a guess. For the first time ever, the docs tell him not to lose weight--there's that silver lining. Hoping his extra weight will slow down progression--doc says that it can, so now we just try to keep him swallowing effectively. I think Neudexta has really helped all his bulbar symptoms. As for the limbs, well I guess if we're learning anything, it's trying to take life a day at a time. Some days it's just easier than others.

 

[affected]

It is so hard when we are like stumbling about nearly blind.

I just took the outlook of having everything ready in advance as much as I could, didn't care how long things sat unused, chances were they would be needed one day and I didn't want to be caught in crisis.

Kind of like, expect the best but plan for the worst ...

 

[Gembead]

Thanks Tillie, I think that's the best way to approach it, having things on hand when they are needed will make life just that little bit smoother for both the PAL and CAL.
Love Gem

 

[affected]

Gem one of the things with Chris was that he would demand equipment, then look at me like I was a lunatic when it would arrive and say - I'm not using THAT! (gotta love FTD)

So it would be put away, and either he would ask about it weeks later, or he would hit a crisis and I would magically produce just what we needed and he would simply have to start using it ...

It does make sense though to order equipment in advance. Some equipment can take time to be organised and arrive, so if they suddenly cannot weight bear and you don't even have a basic manual wheelchair, and maybe it's a weekend, then you are in crisis.

Thankfully we never hit any true crisis like that, although having the hoist would have been great when he would fall. Getting someone off the floor when they have no arm strength is NOT easy. By the time we had the hoist he was no longer walking except with walker frame and me right behind constantly kind of guiding and straightening and sometimes supporting him to stay upright.

 

[Brooks78]

Hello All,

I am new to this forum and have read all of your comments...This is not new information to anyone but my mom has had ALS for about 3 years - the first 2.5 years we only saw minimal disabilities to her hands then legs. Her condition since then (last 3 months) has exponentially gotten worse. According to the Doctors, her particular case had a mild on-set, but a very aggressive progression of ALS, and they have now diagnosed her with palliative care, which is about 6 months to live. Since that time we (my sister and I) have procured multiple assets from our health care system (hospital bed, breathing machine, portable toilet etc.) to improve her quality of life. Since then, I have found that the attention to the smallest detail that we may over look, like where the Christmas Tree is placed this year is so much more important to her than all the stress that we/her are succumbed to because of this disease. I understand that all people affected by this disease will experience a different beginning, middle, and end to their story....but I would just like to add this: If you or one of your loved ones are suffering from this disease, please take the time to tell them you love them, visit them as often as possible, and don't sweat the small stuff...their time is limited, and your love and kindness is sometimes all you can give. Thank you for your time - God bless.