Need question answered, please help!

[nmpearce]

Hello All,
I am new to this forum. I came here hoping for some anwers to my questions. My husband was diagnosed in Feb of 2007 with Bulbar onset ALS. At that time he was still eating. today, he can no longer swallow anything at all. he is fed via a peg tube. He has such emormous amounts of mucus that is constantly choking him. The excessiveness started a couple of months ago. He has a suction machine to help with it. My question is What does it mean as far as where he is on the progressive scale? I know everyone is different. But when a person gets to this point what can I expect to happen now. He can walk and use his hands, however, his arms are greatly diminished. His speech is terribly garbled and sometimes even I have trouble interpreting it. I know he can use a speech device for that if he wants to. I am concerned about what this excessive mucus means to where he is in the process. Please help if you can. Thanks.

 

[indigosd]

My husband is on Robinul and that helps with his scretions. He takes 1 mg at Bedtime. If you search there are many informative threads on the forum about this issue. There just seems to be absolutely no definate time line of progression for the PALS-everyone experiences the symptoms to different degrees and at different speeds but all always end up locked in at some point. Sorry that I couldn't help you much. Sorry that you had to find us but a big WELCOME! You will find much support and practical advice here!

 

[nmpearce]

What do you mean by 'Locked In"?

My husband is stubborn and refused the med when the doctor offered. I will try to get him to agree to it. Thanks for your help.

Tell me a little about your husband and what steps you have gone through so far.

 

[MtPockets]

Locked in refers to when we get to the point we can no longer move on our own or talk. Our minds are still active as ever, but we are locked in a body that will not move. I hope this helps.

 

[nmpearce]

Yes, I understand that.
I feel fortunate that my husband, Mike, is not 'locked in' at this point. He is still able to walk. His legs at this point seem the least affected. However the faciculations are evident all over his body. It's strange to watch it happen. the ones in his cheeks are active all the time. Probably a good thing meaning those muscles are not dead yet.

We've had a pretty good year this past year. Mike agreed to a peg tube last december and put on some weight. He was so frail at the time because he couldn't eat much at all. Since then he has lost all ability to swallow and his speech has further deteriorated. His current issue is the excessive mucus. It depletes his energy trying to keep his throat clear. He has a suction machine but doesn't always use it. I think he is resisting being tied to it.

I get aggitated watching him go through this constantly. I cannot help. He does not want to be treated like an invalid and he does not want me to hover. I guess I should feel lucky that he can still do stuff for himself. But the choking scares me to death. I have to go to work and I am afraid he will choke to death while I'm gone. Most people don't understand. Because he has good color and can walk, they think he is just great. They don't live with it and he is good at hiding stuff when he wants to.

 

[BarryG]

Hi and welcome to the forum. I am in much the same state as your husband. Bulbar onset symptoms started in July of 2007, diagnosed in Jan 2008, talker in June of 2008, peg in Feb 2009. Now almost all (except for morning smoothie) of my nutrition is by peg tube and no one can understand anything I say now so I am totally reliant on my electronic speech devices. But I am still walking (slow and floppy) with a cane, my hands and arms still working but they get tired easily so as long as I keep my mouth shut and don't drool most people don't know that there is much wrong with me.

I have been plagued with excess saliva and phlem for many months and with a combination of my suction device and a Waterpik water jet device I am able to clear out my mouth and upper throat however it is an ongoing problem. The waterpik has an attachment that allows me to spray a small amount of water around my mouth and to the back of my throat and if I am bent over the sink it all comes right out (water and goo) and I don't have to worry about choking or aspirating. It does take a little practice and care and I do use it at least a couple of times a day but once my mouth and throat are cleared they are good for a few hours.

I have tried Amytryptyline but it caused me blood pressure problems (although that may have been connected to my extremely low weight and weakness pre peg) and I now have Glycopyrrolate which seems to work but I hate taking medicine so I haven't been taking it.

As far as what is next, I will let you know when it happens. :wink:

 

[Danijela]

Welcome to the forum.

Most ALS symtoms such as secretions can be managed, and it is worth trying a range of things. It is good to hear your husband is still mobile, as many aren't after couple of years from onset of symptoms. And that he had a peg inserted in time.

You don't mention his breathing or FVC (forced vital capacity). It is when the muscles that help us breath become affected that further complications occur, so if his FVC is good and stable he may be able to go on for quite a while. If he is experiencing difficulties breathing then it is time to consider NIV (non invasive ventialtion) or trache/vent (invasive ventilation). Different people make different choices when it comes to it, espacially the latter.

Very few people die from chalking or aspiration, but persistant aspiration can lead to pulmonary infection, which in combination with poor function of breathing muscles can lead to death.

As for being 'locked in', this too does not neccessary occur in all cases. There are different types of motor neuron disease, and some people live for a long time with fairly good degree of functionality. With modern technology 'locked in' state can be deferred for a long time, as one for example only needs to be able to move one's eyes to communicate using particular software.

All the best, Danijela

p.s. Barry's response appeared as I am typing this. Hope what I wrote is still valid.

 

[nmpearce]

Thank you very much for the info.
I am glad to hear that he is unlikely to choke to death. That has been my greatest fear lately. A horrible gasping for air and choking to death senerio. You guys have relieved that worry somewhat.

thanks for the water pick idea, I will try that and see if that helps him out. Currently he just pours water in his mouth and lets it drain out, nothing will go down any more, and he still can spit some. His poor tongue is useless.

His breathing is pretty good, however, he has a breathing machine he is suppose to use at night to help the oxygen levels in his blood. He doesn't like to use it. He thinks his beathing is just fine and maybe it is good enough. I don't know. I know eventually he won't have that ability. He has already signed a DNR and says absolutely no trach-ventilator. Will he change his mind? I don't know, probably not. He is very strong willed.

I've been hoping to have him with me at least another couple of years or so. But do not want to be selfish and have him living and suffering because I want him with me. I want the time he has left to be as good as possible and so far, with the peg he has done great. If not for it, he would be gone now. I had a hard time convincing him to do that. As I said he's stubborn and does not want to live by artificial means. He's ok with the NIV for now but doesn't use it everynight like the Dr. said. The Dr told him he'd feel better if he used it.

The struggle to cope with his disease has zapped his energy. He sleeps intermittently off and on during the night and day. He had to retire from his job last May. It became too difficult. I just wish I could stay home with him every day but I have to work and he says he's fine by himself.

I guess I need to just relax and take it one day at a time. I try to do that. It's just when a change occurs that the fears rise up and take over. I know also that nothing is in my control, I can only do the best I can when things occur.

Thanks again for the comments and advice.

Nancy

 

[BethU]

Hi, nmpearce ... welcome (but as we always say, I'm sorry you have to be here).

As far as what the secretions mean as far as progress, I don't think they predict much except that the bulbar symptoms are progressing. Limbs and the bulbar areas are usually on different timetables. Once weakness starts in any area, it keeps progressing. His speech will probably continue to deteriorate. Frankly, I find it easier to be without speech completely than it was to try to communicate with very impaired speech. The mucus is part of this process. It's good that he got the feeding tube. With the tube, a communication device and a suction machine, he can maintain his weight and remain fairly comfortable and functioning in his daily life.

His limbs and his breathing will be on their own timetable. After 4 years of bulbar symptoms, I am still walking, but very weak, and have some limited use of my hands. It is impossible to predict how the progression will go. People do plateau.

Hope for the best and encourage him to use all the tools available.

 

[nmpearce]

Thanks. I will do my best to convince him to use the tools available. I'm sorry that you are going through the same things he is. I am very grateful that he can walk and use his hands. They have begun to be harder for his to use but he still can. His legs are his the strongest of his limbs and he has not begun to fall down. He is quite tired all the time but has determination. He still does whatever he can and sometimes things he shouldn't try.
Thanks for your input. It is very helpful.

 

[Blubear]

Hi nmpierce, first of all WELCOME TO OUR FAMILY FORUM! I am so sorry you to have to go through this, and I am sorry for your husband too. My father is not that far along yet, so I dont have the answers to what you need yet on this thread. Once he does get that bad, this is the place I will come for all the advice. We are also here for any emotional support you need. We really really care! If you need to vent at any time we are here for you! Give your husband a big bear hug from me, and I am sending you one too!
Kari

 

[MtPockets]

Welcome to the forum. I guess I was fortunate to get the limb onset type of ALS, if you can call that fortunate. DX May 11, 2006, Lost use of my legs that year, now the spasms have almost completely stopped in my legs, whether due to the medications or muscle loss I cannot tell. Now losing arm, hand, and fingers. Have two fingers to type with.
Shared this just to say each and every case of ALS is different, and we learn to adapt to what we have been given.
I pray for the best for you and your family as you live with this.

 

[dadsdaughter]

Hi,
My dad had bulbar onset too. As for the speech, he will need to start relying on his dynowrite. If you don't have one, contact your nearest ALS society and they will probably lend you one--they did for Dad. As for the mucus, when my father suffered from that I read a lot of posts on the PALS site that were helpful. Most talked about different remedies and mmany mentioned the cough assist machine. They didn't use it for coughing but for loosening the mucus that gets stuck way back. There are a lot of encouraging and helpful posts on this matter. Use the "search" tool at the top of the site and put in key words one at a time like "mucus" or choking etc. I also read a lot of the posts to my dad and it seemed to help him calm down a bit because he could see that what he was experiencing wasn't unique. It seems when he choked like that on the "crud" he would panic and fear that he would choke to death. After I read a bunch of posts from fellow sufferers, he felt better. He also really like the coke suggestion. It seems coke or pepsi really helps thin out the crud. I am so glad your husband can still walk etc. The bulbar onset is one of the most cruel onsets of ALS, particularly if your husband it a talker like my father was...it was the greatest blow to him to lose his speech, but he learned to work with the dynowrite quite well. If you husband can't type I recommend he start working on his skills with a typing program. Good Luck!

 

[MtPockets]

Home of Luigi's Real Italian Ice
This is a product my speech swallow therapist told me to use to help clear out my throat after eating or when there is mucous. It is sort of like frozen ice cream, and kind of scrubs the throat as it goes down. Works good for me. There are some store brands that are cheaper.

 

[Marjorie R. Wilcox]

Rick is different. He has had morning mucus for at least 6 years...long before we knew he needed a machine for sleeping. He doesn't have any other bulbar symptoms at all. He got loss of balance and depleting muscles in his calves.... and now he is just beginning to have curled fingers in the morning.... and one and a half fingers almost useless. Nothing has changed with the mucus, but he blows out a half a box of tissues each morning, and spits out clear phlegm. He swallows fine, can yell and be heard far away, and doesn't drool or choke or anything.
He was diagnosed 2 1/2 years ago now... and we are thankful for slow progression. See, everyone is different, and morning mucus doesn't mean quicker progression.