Bulbar onset, developing quickly

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Elkhorn3

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Hello,
I'm new. My husband has bulbar onset, just diagnosed, with all the classic symptoms. In just 3 months his speaking has gone from normal to almost impossible to understand, even for me. Of course some days better than others. I have studied this whole forum in the past few weeks, and am so appreciative of this warm and supportive group.

So far he can walk strongly and do things but it's changing fast. I've read that bulbar onset can progress quite quickly. I'm wondering who out there has experience with this? I know no 2 cases are alike, but we live in the mountains in Montana, and I'm feeling like I have just a few short summer months to prepare for the unknown in front of us. Any specific bulbar experience or knowledge would be helpful. Thanks.
 

affected

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Hi Elkhorn - so very sorry we are welcoming you here. In general, bulbar onset PALS are often rapid progression, but not always. So unpredictable, but when it is rapid like this from the start, it is more likely to continue to be rapid. (more likely, not guaranteed) The really important thing about this statement is that it is still so early for you, but getting things planned, legals in place and equipment orders started is really wise. It feels like a whirlwind, but if you are suddenly in a crisis it is worse.

My husband was rapid progression bulbar onset. It did take us quite a while to get diagnosed, partly because he was in denial for more than 6 months after his speech changes started. Once I insisted we get him looked at, it took another 6 months as no once suspected ALS so we were bouncing around in the wrong medical specialties.

By the time he was diagnosed, his speech was quite bad, he was having trouble swallowing and his hands were weak. He had already sold his cafe 2 months previously as he was unable to work. He was gone 11 months after diagnosis. I will note he also had FTD (fronto temporal dementia) and did not use bipap at all. His life may have been prolonged by some weeks or months with bipap early, but he was still rapid so it would not have made a huge difference. Many of these great strategies can make a big difference, especially in quality of life, but when FTD and rapid progression are involved they don't always.

My husband had a feeding tube but he wouldn't agree to it being placed until he was emaciated and dehydrated (about 5 months after diagnosis), and he never adjusted to being fed well and continued to lose weight and refuse feeds. I believe in advocating for what the PALS wants, even if the CALS doesn't think it is the best course. It was his body and his life, and even with a level of dementia, I believe he had the right to do it 'his way'.

This post might help you a lot.

And this one on planning in advance. I often had equipment stored away ready for the next thing as he could go from just being able to do something one week to suddenly unable completely.

Ask away, read and search here, then ask more.
 

Elkhorn3

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Thanks, affected. I was hoping to hear from someone who had this experience, and I really appreciate the practical advice. I don't know if FTD is involved, but definitely some kind of brain damage. Personality change was actually the first symptom, looking back on the last 7 months. My husband has used a bipap for years, so that's probably helping. We will start speech, occupational, and physical therapies soon. No ALS specialist in our area, but we're working with a very dedicated primary care physician so that's good.

The links you sent were helpful, thanks. And I appreciate your affirming that this is his journey more than anyone's. I am in complete agreement there. Wherever he goes with this, I will go with him. But I recognize that a lot of this will be mine to figure out, so the companionship is really appreciated! Your experience is a gift to others.
 

lgelb

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Hi, Elkhorn,
Sorry to welcome you here. His progression does sound very fast, but if it's limited to speech so far, maybe not super-fast.

I gather travel will be difficult in a few months due to snow. Ordinarily, I would advise you to try to get anything tangible that he needs to be fitted for personally. That would include a power wheelchair, most particularly. But if he's still walking, that wouldn't be approved now. Still, you can make sure he has a means to communicate that works well for the two of you, and anyone else you are with, from a dry erase board to a smart phone/laptop, and some backup plans as his mobility wanes. If you have any issues in winter with power or Internet, I would address those now.

Since travel works both ways, I'd also gather in whatever materials and expertise are needed to make your home and vehicle accessible for a patient lift, power wheelchair and the like.

By the numbers, the older he is, the sooner he has respiratory involvement and if he does not take riluzole, those are the major prognostic factors in bulbar ALS.

I would advise you to use the time to make sure you have a care team (equipment supplier, OT/PT, RT, neurologist, primary care doc) that is willing to work via video as much as possible, and are in contact with anyone that might help you through, such as your local ALSA and MDA chapters and your state's assistive technology agencies. If his team is a long way out in snow and there is someone closer who does primary care, for example, I would get or stay very friendly with that person.

The rest of the summer, I'd use to help him do everything and anything that he wants [what may well be] his last summer to be. Take pictures. Shoot video. Eat ice cream. Hike. Watch sunsets. Whatever makes him happy.

Best,
Laurie
 

affected

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I don't know how much age plays, it is a general statement. My Chris had never been sick a day in his life, didn't even know his blood type as he had never had blood drawn. He died at 55 years old. It's a moot point really, but being young, and being healthy doesn't always seem to have any bearing on speed of progression.

I'm very happy to help support any way I can. Looking back I know that I began to notice very subtle personality changes at least a year before the bulbar symptoms started. Hindsight puts things in perspective differently doesn't it. It's a matter of if the changes had happened, but then had cleared again I may never have really remembered them. But as things progressed and as the diagnosis of ALS came in, and as his behaviour changed more and more, I realised there had been something insidious going on very subtly for some time.

Some really good extra practical stuff in the reply above to add on to the links I gave. We got you :)
 

lgelb

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Age plays in the aggregate according to most studies available. The older with bulbar onset, the shorter life expectancy. But, as Tillie notes, that's in no way a rule, just a tendency across the population studied.
 

Fusia

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Elkhorn,
I live in Bozeman, I was diagnosed last August (upper limb onset). I visit the ALS clinic in Billings about once each quarter, and I am generally pleased with their capabilities and resources. If traveling to Billings is an option for you and your PALS, their ALS-specific expertise may be of some value to you.
 

Kristina1

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Hi Elkhorn, I have bulbar onset, my first symptoms were speech changes and PBA (inappropriate laughter/crying). However my speech decline then slowed, and it has taken until now, 3 years since diagnosis, to become indecipherable to those outside my husband and children. Currently I am unable to stand, walk, turn in bed, or do much with my hands. But I can still move all my limbs to a limited degree, can still talk (very poorly), and eat a little by mouth. I'm 34.

I have a friend with bulbar onset, 50s, who very quickly lost all his speech and voice altogether. But a year in, his only other issue is swallowing and hand weakness.

So it really can vary.
 

Elkhorn3

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Wow, this is all so helpful to hear, thanks to all of you who've responded. Fusia, we have the Billings Clinic on our radar, but haven't visited yet. What kind of help do they provide, from your distance, if you don't mind me asking?
Thanks. This forum is weirdly keeping me company. In a good way!
 

Fusia

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Elkhorn,
The Billings ALS Clinic offers all of the resources that any certified ALS clinic does–neurology, Respiratory, physiatry, OT, PT, nutrition, speech therapy, social worker… I have only had two visits to clinic so far (the third one is next week), and I've mainly used them to monitor my respiratory numbers, and provide guidance on adaptive equipment. While I have not done this yet, I know they have assistive technology systems to try out –perhaps that's something that you could tap into right away.

While I have always gone into the clinic in person for my clinic day, I was able to do a telemedicine visit with the physiatrist recently in order to get my power wheelchair evaluation completed. Also, the OT did a
televisit to give me some pointers and advice on what things I might consider updating in my home, especially during the upcoming bathroom remodel. The nurse navigator is very responsive, and getting appointments seems to happen quickly.

Since your PALS is dealing with bulbar issues, you may also want to talk to Jackee Gaddis with the Evergreen chapter of ALSA. She is a resource for adaptive communications / technology. She may able to provide some guidance and/or have some loaner equipment available.
 

Elkhorn3

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Fusia, thank you! We met our speech therapist yesterday, and I asked him about Billings Clinic. Maybe the need for virtual visits can help make that 250 miles disappear... :)I'll definitely contact Jackee Gaddis.
 

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Elkhorn - I was diagnosed in 2011 at the age of 73 and have bulbar onset. The way I understand my diagnosis is that my official diagnosis is ALS and my working diagnosis is PLS. I had some mild symptoms for years--slight foot drop, occasional slurred speech, back pain, and spasticity--which I attributed to old age and back problems. At about age 70, I realized something more was wrong.

At 80, I my attempts at speech are pretty pitiful. One person can understand me about 50% of the time. I communicate almost entirely with a computer. I have no dietary restrictions, although I must eat very slowly, and, so far, no significant breathing problems and do not use a bi-pap.

I'm telling you this because, as is so often repeated, every case is different. In my case, bulbar onset does not seem to mean I am not a slow progressor.

I hope you can visit the Billings clinic and find the help you need.
 

NorthernCalif

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We live in Northern California—5 hours north of San Francisco. My husband was diagnosed in May 2020 with Bulbar ALS. He started slurring his speech in October 2019. He is a veteran so we are working through the Veterans Administration for his health care. In January 2020 we figured he might have Bulbar ALS but due to the coronavirus it took us until May for his diagnosis. His speech is gone, his hands are weak but like your husband he is pretty strong in walking. He is still able to eat on his own, but we have made some adjustments to his diet. The VA immediately gave him a bi-pap machine and a lung assist machine.... which we are thankful for! He also just started on Radicava infusions that we are hoping will help slow the progression of the disease. We are trying to figure out our new normal... between ALS and the Coronavirus...We both have a great sense of humor, a loving family, and great friends to help us navigate this disease.... but , as you know, no one can know the everyday struggles we are going through just to appear like life is normal. We don’t know what the future holds but we will go down this bumpy road together!
 

Elkhorn3

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It is a wild ride, for sure. Two weeks ago my husband was hiking every other day, 4 miles in the mountains, on a trail that took him 1800 feet up, over fences, through gates, and now he can barely get up the stairs. So many losses, until nothing is left but love itself.

Sending best wishes to you all on this beautiful summer night.
 

lgelb

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I'm so sorry, Elkhorn. You will definitely want to get started on the power wheelchair, hospital bed, and patient lift given the progression you've described. Let us know how we can help.
 
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