Hello...new to the CALS board

[Aswanson]

Hi...while I've visited the other boards and even posted, this is my first time posting to the CALS board. This past week my husband got his official diagnosis of progressive bulbar palsy.

About us- We live in NH. I'm a stay at home mom, 3 kids - 2 are from my prior marriage (21 & 17) and we have a 7 yr old daughter together who was born with congenital glaucoma and is visually impaired.

About 18 months ago hubby started producing excess saliva to the point it was just running out of his mouth, a few months after that it was causing him to choke at night...then there were other symptoms that popped up, but months apart so we never put any of them together until Dec when he started having this feeling in his throat, started choking on liquids/foods and then in Jan when his voice began to change (a mumbled word here and there at first, then a slurred word, then full blown- all within a couple weeks) When I took him into the hospital at the end of Jan they had found he had had a mini stroke a few weeks prior, so much time was spent trying to figure out the cause of that. Since Jan his voice has deteriorated- he can't pronounce many syllables, things are mumbly/strained/slurred and sometimes so bad I can't even understand what he said. He doesn't talk much these days as after about 15-20 mins his muscles are fatigued and his voice is hoarse. His face, tongue and throat muscles have gone drastically down hill as well. His tongue is atrophied and has fasciculations and he can't move it very much at all to manipulate his food. If he talks too much he has more trouble swallowing and has a higher choking risk.

As of his last appointment everything looked good below the neck, which is good. Coming up he is getting baselines for everything to monitor him as we move forward and nutrition consult as he keeps losing weight....this seems to be our biggest battle. (Jan-198lb ; current-176lb most within the past couple months)

He had been on FMLA pretty much since the end of Jan, but went back to work this past Thursday to see if he can work a little bit longer. I worry about his fatigue and him being able to get his nutrition in while at work since he's on the road all day long. If anyone has any ideas of soft, mushy, high protein/high fat, transportable foods- I would be grateful!

I thought when we went through our daughters diagnosis THAT was going to be our crisis.....who knew there was more to come in our future.

~Amy

 

[GregK]

22 pound loss. Need to stop that.

You should seriously consider having a G tube. As his hands still work he can manage feelings while on the road.

 

[soonerwife]

So sorry to hear about your husbands dx. It's alot to to digest.

My husband has Bulbar Onset ALS. I'm not sure what the difference is in the two? Maybe someone can enlighten me?

You have come to the right place for support. Everyone is great on here. You can vent, get and give info. We are here for you!

 

[affected]

Sorry to be welcoming you.

Greg is right - think about a PEG now so that he can get lots of nutrition in easily, safely and using far less energy. Losing weight will very often hasten progression and is something that can be addressed.

I guess he will have to figure out how much working affects his fatigue and weight loss, sometimes as a CALS we have to kind of step back and let our PALS do what they want and figure things out as it is their body and disease. It's never easy to watch.

My husband was bulbar onset ALS, so I can identify with all you describe there.

@sooner - they are subsets of the MND diseases. Often PBP becomes full blown ALS, it's a classification depending on the upper and lower MN involvement. With bulbar onset there is still widespread upper and lower MN involvement, but with PBP it is only really the bulbar region of the brain being affected mostly. Usually this does change as things progress, but sometimes not. In a way the classification is useful and in another way it's still in the same class of disease unfortunately. Someone else may be able to explain it better.

 

[lgelb]

Welcome, Amy. Sorry that you find yourself here. I agree that a feeding tube is well worth considering, and soon. You will want to find an interventional radiologist with expertise in placing tubes in neuromuscular dz. I can't imagine why your clinic thinks it's OK to watch and wait.

Sooner, bulbar onset ALS is ALS that begins with the bulbar muscles instead of the limbs (think head/neck). PBP is a cousin of ALS that affects only the bulbar region and not the limbs. But about 25% of PBP dx (in one study, anyway) become ALS as limb involvement emerges.

 

[Aswanson]

GregK,

I am in total agreement. He has a nutrition consult coming up and that will be on the discussion list for sure. I hadn't anticipated the difficulty of eating once he went back to work until I made him pack something to bring. He used to go all day without eating until he got home- not even an option now. I think the tube would be great for him while he's on the road all day and then eat by mouth prior/ after work. Thank you for your imput.

Amy

 

[Aswanson]

Hi sooner... Thank you for the welcome. It is a wonderful group....one I never thought I'd be a part of. As some of the more experienced PALS/ CALS have said, it's a cousin and may or may not turn into bulbar ALS. Prognosis unfortunately is not any better.

Amy

 

[Aswanson]

Affected...

Thank you for the welcome. That's exactly where I am at with the work situation. I wasn't too keen about it....I worry for him and think it will be a lot, but in the end it's up to him and he loves his job. If it helps him mentally and even just gets a couple months more out of work it'll be worth it for him. He'll have gone and tried. I'm watching carefully though to make sure he's paying attention to his body. As you said....never easy to watch.

Amy

 

[Aswanson]

Igelb,

First, I have to thank you.....you've been so helpful with my other posts in the past. It's not really a community you WANT to join, but there are the most helpful and kind people here
Last I posted he had just had the EMG....he's seen the stroke specialist and neuro muscular specialist again, but not ALS specialist. First appt for that one is end of July as well as nutrition consult, so hopefully we can get it going at one of those appt's.

Amy

 

[affected]

Amy, honestly I would get on the phone and get a consult now to get the ball rolling for a peg if you husband is agreeing to having one.

If you appointment is nearly 2 months away he could lose a lot of weight in this time and it could put him back a long way. Of course he has to want to peg, but if he does, sooner than later is always recommended.

 

[pittsburghgal]

Hi, Amy. Sorry to welcome you here but we will be here for you throughout this journey.

I echo what others have said in that you really need to pursue getting the PEG now rather than waiting until your appointment at the end of July. If you contact the neuromuscular specialist that your husband has seen and inform him about the amount of weight loss that has occurred, I'm sure you will be able to get a referral to an interventional radiologist to get the PEG as soon as possible.

Sharon

 

[DreamsEnd]

Welcome Amy. Sorry you are joining us!

I'm with Tillie on the phone call(s). You'll find you will have to become tilter of windmills (assertive) to get him the care he needs.

Sending you courage and strength.

Sherry

 

[Aswanson]

Tillie,

I brought it up to him when he got home today and it didn't go so well. I don't think he's pro tube. I'm going to let him go this week, have him do his weekly weigh in (I have him weigh in weekly & we keep a log) and I'm sure he'll have lost. Maybe that and doing his own research will persuade him it's a better option if he's going to be working (and long term ).

Today was his one office day a week and what voice he has left was just about shot when he got home, so looking into writing tablets vs. text to speak for him.

Amy

 

[Aswanson]

Sharon,

Thank you for the welcome and the info. As soon as hubby is on board I will hop on the phone! Somewhere over the past month he must have changed his mind.....maybe now that's it's becoming all too real? Breaks my heart.

Amy

 

[Aswanson]

Sherry,

Thank your welcome and thoughts. I have definitely found I have needed to be his voice, much to some Dr's displeasure.


Amy