Weakness, Fatigue, Wasting

[AlabamaGal]

Just wondering if there are others that have been stricken by extreme fatigue and weakness, but seen little wasting of muscle tissue. My husband was diagnosed June 2010. Two of his brothers have ALS and have wasted away, but kept leg strength for a long time. My hubby has been so tired and weak from the beginning. I see some wasting in his left arm and hand now, but it may be from lack of use as this is the arm/hand where his problems began. Now, his right humeral head (shoulder) is broken from a severe fall this week. He is on cpap, but not bipap. Jaw muscles tire easily and he has to "throw his voice" when talking. The more fatigued he gets, the more slurred he gets. It seems this awful disease affects everyone differently. Just wondering if there is someone out there that has seen the disease progress in this fashion.

 

[Georgia Peach]

Does your husband puff when he sleeps? Mine has to throw out his words the more tired he gets during the day and especially if he is up in his power chair. We are getting an amplifier to help him speak from the VA. He says he hopes it sounds like Darth Vader. I think it will just sound louder!

My husband has atrophy in all of his muscles now. He was an avid bicyclist and all of those strong muscles that he developed have disappeared.

Yes to major fatigue and weakness. When he gets up for visitors or outings usually to the doctor, he only has a small window of opportunity before he needs to go back to bed.

 

[glupavomomiche]

My progression is pretty similar to your husbands. I have lots of fatigue and weakness, chewing and some swallowing probs, and my speech gets pretty yucky when I'm tired. There is not a lot of muscle wasting, or at least not as much as would be expected at this point. My right leg is definitely smaller than the left, and the doctor can tell I have intrinsic hand muscle wasting too. He said this is due to being UMN dominant and that most of my weakness comes from there. Could be your husband is the same and perhaps his brothers are LMN dominant.

 

[AlabamaGal]

My progression is pretty similar to your husbands. I have lots of fatigue and weakness, chewing and some swallowing probs, and my speech gets pretty yucky when I'm tired. There is not a lot of muscle wasting, or at least not as much as would be expected at this point. My right leg is definitely smaller than the left, and the doctor can tell I have intrinsic hand muscle wasting too. He said this is due to being UMN dominant and that most of my weakness comes from there. Could be your husband is the same and perhaps his brothers are LMN dominant.

Yes, I remember the neuro at the ALS clinic said she felt strongly that my hubby has more upper motor neuron involvement, asopposed to his brother's lower motor neuron issues. I am trying to decipher the differences and while I know there is no way to know progression issues, just trying to prepare myself and my kids. My husband has issues with balance (which is why he has been immobilized which a fractured humeral head), excess saliva, forced speech, sometimes slurring, head bobbing, spasticity, and, of course the fatigue, and weakness. He also has the unofficial symptoms of extreme eye watering, eye discomfort with lighting, incontinence to some degree. So frustrating. We are trying to get wills, living wills, and such in place. I think the peg tube issue is causing us the most grief. We already use hospice, and they are positively wonderful.

 

[CAHPAH]

AlabamaGal
Not that I want to second guess your doctor's but everything I have been told suggests that CPAP are not recommended for PALS. The constant air pressure means they have to exhale against the same air pressure they inhale with. This can fatique to diaphram. Where as the BIPAP dicreases the exhaltion pressure so that the diaphram does not have to work quite so hard. It might be a question to ask your doctor.

As for the over all fatique, my wife sleeps 11-12 hours a night and even if we don't go out and just sit around the house all day she is still exhausted by bed time.

 

[momap53]

Overwhelming fatigue has been a major issue for me even in the very early days. Getting dressed makes me tired, showering makes me tired, going out makes me tired, everything makes me tired. My FVC is around 92% so haven't been placed on BiPap yet.
(If you'll scroll to the bottom of the page you'll see links to Past discussions about fatigue)

CAPAH is right about BiPap being prescribed for PALS over CPap. The mechanics of PALS breathing is different from those who are prescribed CPAP. You can do a search of BiPap to see some past discussions on this.

 

[hjlindley]

Every case if different. I started with weakness in my right hand 4 years ago. Then I started walking stranggely, then my ankles got wobbly, now my left hand is weakening and the fatigue is limiting. My first dirty emg was 9/2011 but my neurologist has it in his notes from 2009. But can still, talk, eat, breathe. Just don't ask me to go upstairs too many times a day, and sewing on a button or mendingis out of the question. I am trying to focus on what I can still do and not what I can no longer do. My PT hase seen wasting inj my lower are muscles and atrophy in my upper back. But I have a limited amount of energy in any given day, and sometimes I don't know where the wall is until I hit it. Good luck, and we are here for you.

 

[KissJ]

Yes, everyone is different, but I know you want to know. I always want to know how others are doing/dealing with all of this. My husband has spells of fatique. Daily, he takes a nap in the middle of the day to gain some strength. If he does any tasks requiring movement, arm strength it really makes him tired. He has muscle wasting in his hands, legs, and feet.

 

[AlabamaGal]

Thank you all for your replies. My hubby was placed on cpap long before his ALS diagnosis. BiPap has been suggested, but we have to have a sleep study to get one and we cant afford that. And, now with his very severe break of the humeral head, I have no idea when we can make the several-hour trip to clinic again. And, then today we found out that while x-raying his shoulder at the hospital they also found a 7mm mass/nodule density in his right lung. What now? My goodness, we are overwhelmed. My little den is now a mini-hospital room. So very stressed.

 

[LizT]

I know you have explained that he got the cpap before, and cannot afford the sleep study, but if its possible, find a way to make that happen. A cpap will make it WORSE for PALS when it comes to their breathing. Which in turn makes them incredibly tired, and weak, fatigued and lethargic. From my understanding, cpaps will weaken the diaphram faster.
Im not trying to scare you here, but just making sure that you know the importance of having a bipap over a cpap.

With the correct machine, he may gain back some of his energy and strength.
Best wishes.

 

[AlabamaGal]

Thank you. I will work on this.

Blessings!

 

[LizT]

Are you in contact with the ALSA of Atlanta? They may be able to help get you guys the bipap.
is your hubby a vet? if so have you been in contact with those folks? they may be able to help as well.

 

[LizT]

so im not sure why i said Atlanta. I meant in Alabama. Sorry bout that.

 

[AlabamaGal]

We were diagnosed at the MDA/ALS Clinic at Vanderbilt in Nashville. We went to clinic several times, but it is a 7 hour drive (we are in southeast Alabama), and the trip became too much for him. We plan to donate nervous system tissue to the Emory ALS Clinic in Atlanta, so I am considering taking him there...but even that is a few hours away and may be too much, especially with his immobility at this time. The Vanderbilt people said we had to have a sleep study to get the bipap. Everyone down here says not to fool with a sleep study locally, as they are focusing on sleep apnea and know little, if anything, about ALS.

 

[LizT]

I think they may be right, that its important to have the sleep study done at an ALS clinic. Dang... this is a subject i dont know a lot about because I didnt meet my PALS until he was already pretty much 100% paralyzed and on a vent. I am hoping that others with more knowledge will chime in here and help you out. You can also read other threads dealing with bipap and sleep studies. Use the search option.

May I ask- are your hubby's brothers both still living with ALS? How far are they into it and do they live close? Im guessing there is a strong family gene if 3 sibling all have it. Not trying to be nosey. Just interested.