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Urgently referred to ALS clinic. Anyone here have respiratory and bulbar onset?
<span style=”font-weight: 400;”>Greetings all, the day before yesterday I received an urgent referral from a neurologist to a nationally renowned ALS clinic; based on the neurologist’s exam findings, the ALS clinic called me almost immediately and scheduled back-to-back all day appointments with the director M.D. of this clinic. In other words, it’s about 99% certain I have ALS. I’m here to learn as much as I can and mentally prepare for what’s ahead. </span>
<span style=”font-weight: 400;”>My situation seems to be “backwards” from the typical course of ALS from what I’ve read, and I’m hoping to connect with others that might have similar experiences (or anyone really!).</span>
<span style=”font-weight: 400;”>My problems started in January 2021 with chronic respiratory failure on exertion, diagnosed by my pulmonologist. Months of exhaustive pulmonary and cardiac testing revealed nothing. Then, just a few weeks ago (June 2021) I suddenly developed alarming symptoms: as the day wore on, towards afternoon and strongly in the evenings – my speech has become slurred, my tongue feels “thick,” I can’t raise my voice to a normal level; additionally by late afternoon I have significant trouble keeping my head upright, and constant drooling (have to keep a bib towel all night). I got a cervical collar from Amazon but it feels like it’s choking me, unfortunately. </span>
<span style=”font-weight: 400;”>My pulmonologist (last week) then suspected respiratory muscle weakness and PFT (pulmonary function test) confirmed it. Saw a neurologist day before yesterday and she did a full neuro exam, she was frank with me and said the results are “extremely concerning” and that I have bulbar weakness and upper and lower motor neuron disease signs.</span>
<span style=”font-weight: 400;”>She made an urgent referral to an ALS clinic and they called me within hours. I’m scheduled for a 2 day back to back all day workup at the clinic in a bit less than 2 weeks, with the director (M.D.) of the clinic.</span>
<span style=”font-weight: 400;”>According to the neurologist, this all looks like ALS. If it walks like a duck, quacks like a duck, looks like a duck….</span>
<span style=”font-weight: 400;”>Is there anyone here who has or is familiar with respiratory and/or bulbar onset ALS? The vast majority of what I read is about limb onset, and I’m feeling pretty lost, scared and confused. Any feedback, advice, guidance, input, etc. would be very much appreciated. </span>
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22 Replies
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I also have bulbar and respiratory onset ALS. My symptoms became noticeable in Dec 2018. My Pulmonologist and Neurologist gave a preliminary diagnosis in June 2019. I had the diagnosis confirmed by an ALS Clinic in December 2019. My primary symptoms were speech and swallowing with some shortness of breath. In December 2020 I had a peg tube inserted. I was very hesitant to do this but it has been blessing. Now my meds and food are through the tube and I have lost all speech. I communicate through a white boards and a Text to Speech App. It is user friendly and easy to customize. I developed head drop in the past month. I have a neck brace that is a wire moldable brace. I don’t wear it all time just when I get tired in the afternoon or evening. This has a journey like no other but I try to celebrate the little things in everyday life. My advice is listen to your body and rest when you need to and enjoy the things you’re able to do.
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Thank you Susan for sharing your experience! I appreciate the information and the advice very much.
If it’s not too personal, would you mind sharing what the time frame was that you lost your speech? My “bulbar” symptoms came hard and heavy just weeks ago. I’m totally fine in the mornings but start losing steam around 1pm and it goes down from there. By the time the sun goes down it’s too tiring to talk – though I do still have the ability to do so, it comes out very slurred.
I do know everyone’s situation/path is very unique, I just am completely in the dark about how fast the speech loss can happen, I’d like to be prepared.
Thank you again for sharing!
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Anne,
My speech started like yours and stayed that way for a about 6 months. Once it started to be difficult in the mornings it progressed to non verbal in about 2 months. It was very fast.
Any other questions please don’t hesitate to ask.
Susan -
Thank you Susan, I was actually going to ask just that – if there was a general timeframe from bulbar onset to full speech loss. I know that every ALS patient is unique, but I do wonder. I’m going to start making videos not just of myself, but with me and my husband and kids. We have like a thousand “still” pics but no videos! And if this does lead to speech loss (which from I understand, it does) I’d like to have as many videos of myself talking, laughing, etc. possible.
I do have one question: my speech tends to be “ok” during the day, it starts getting slurred and strained starting in the afternoon, that’s when the drooling starts as well. Was your experience like this as well?
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Yes, as the day wore on my speech would get very weak and it was hard to articulate words. I actually got exhausted trying to talk. At that time I was not drooling. For me the drooling started when my speech was totally gone. The drooling for me is less in the morning and gets more intense as the days goes on. I use a cough assist and suction machine to help manage it.
The recording of videos is a good idea. My 7 year old granddaughter asked when my voice was coming back! -
Thank you for sharing. I develop the “head drop,” drooling and my voice does exactly as you described starting in the late afternoon, gets much worse by evening – I just get too tired to talk, it takes massive effort. Last night when I was by myself with my dogs I tried singing to them and my voice when I went “ahhhh” made a weird….warbling (?) kind of sound? I was fascinated so I kept doing it and every time, it would make that sound – I wish I could describe it! Warbling is all I can think of.
This morning, it’s fine. (not that I’m a good singer ha ha, it’s just it’s never made that bizarre sound before.
I’m going to fix my hair and put on makeup today and do some videos with the family. Thank you again for sharing your experience!
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My husband had bulbar onset.
He had slowed speech at first. In the early years of the diagnosis process, they originally thought he had Lyme tick disease. He did but that only delayed the ultimate diagnosis of ALS.
He had difficulty swallowing.
After dropping in excess of 60 lbs, he got the feeding tube placed in his stomach. Afterwards, he said his one regret was not getting the feeding tube sooner bc it made his life so much easier. Before the tube, it took 45 minutes to take all the medicine. After the tube, he could take the medicine immediately. The tube was inserted above his stomach area and was hidden beneath his shirt. We bought a “blend tech” blender with a sound proof chamber and put food through the blender. We would make chicken or grass fed beef and put the blended amount in a red solo cup. Then we used the feeding tube syringe and put the food in the tube…very fast and easy. We put blueberries in a separate batch and he would have several syringefuls of that also. We would add fresh turmeric root and fresh ginger to one of the food batches also bc for the anti inflammatory qualities. For the third batch, we put fresh spinach, kale, one green skinned apple and other vegetables. We would also use Diabetisource formula to provide enough calories to keep the weight on.
He beat all the odds and lived 8 years. We kept a positive atmosphere and put a hospital bed in the living room so he was always in the center of the hubbub and the kids would talk to him every time they breezed through the room.
I am very sorry this happened to you. My husband never had the head drop.
It seems to affect each person so differently as far as what happens when etc. Keep as much weight on as you can. I am pulling for you. My husband took 10 days a month of IV Radicava infusions to slow down the progression. He also took Rilizole pills daily to slow down progression. We tried to get into the clinical trials for Nurown but weren’t accepted. I hope and pray for medical advancements to help live with ALS. Godspeed to you.-
Liz, thanks so much for sharing your experience and that of yourself and your family. The positive nature of how he/you all handled the situation is inspiring! Really appreciate the information and your willingness to share!
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Hi,
My first symptom was failing grip strength which I thought was due to age. However, I did have a couple of respiratory incidents that ultimately led to my diagnosis last September. I also developed some speech issues in April of 2020 that my doctor said was due to “post nasal drip”. My speech has been about the same for 15 months. I have an amplification system that helps. I do not have serious swallowing or drooling issues. Most importantly, last November my FVC was 50. I was having major breathing issues and I could not sleep for more than an hour at a time. My NP got me a bipap ventilator which I use when I sleep and for a couple hours each afternoon. My FVC gradually improved to 90. This has made a world of difference in how I feel and my other symptoms. If you don’t have access to a bipap, you should. Also, it’s important to recognize that we are all unique and we need clearly communicate our needs to our providers.
Tom
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Thanks for sharing, Tom! That’s awful about the “post nasal drip” diagnosis, ugh. Glad you finally got answers, even though it’s not the good kind. That’s great to hear about your FVC going up! I’m not on bipap but I am on continuous supplemental oxygen because once I get up and start moving my oxygen saturation will drop into the 80s. I use it at night too. This was ordered by my pulmonologist, it’s possible they will change this after I am tested at the ALS clinic.
Again, thanks for opening up about your experience, much appreciated.
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Time to see a physical therapist who specializes in ALS.
While the nerves die, the muscles can still be exercised, and that minimizes or slows the atrophy. Stay active and do not let depression get to you, ALS does not give us time for pity parties. Find the joy in your heart, and let that light up the room everywhere you go! You will not regret being happy.
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Hi John, thanks for the advice! I totally agree with you. I do stretching exercises every morning, through a program called “Classical Stretch” which feel amazing (that’s my time frame where I still feel pretty good – it’s only afternoons and evenings that full on symptoms start showing up). I’ve had to wear my portable oxygen the last few days while doing them, but I think it’s good for my physical and mental well being!
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Hi Anne,
It is good to be positive like you are!
I think the oldest person I know of was a veteran who has to be at least 80 years old. He was diagnosed with ALS in 1959, 62 years ago. Also, Stephen Hawkin was on a vent for well over 30 years. The steps we are taking today are small in comparison with those who have been here before us. While ALS is a struggle, no doubt it is one that I can handle but only if I face it one day at a time.
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Thank you John. I’m definitely trying to take it one day at a time. A bit anxious waiting for my testing (two days of back to back, 7am-5pm at an ALS clinic), but trying to make a mix of information gathering without, well, “freaking out,” so to speak. Thanks again for your advice. 🙂
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Question for Anne: What Classical Stretch program do you use? My husband’s onset was upper limbs and he’s been having some lower limb issues for a while. I’m curious to find out if what you are doing would be a beneficial add-on to my husband’s self-care routine. Thank you for sharing your story.
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Hi Anne! I first noted a change in my singing voice, which was all about breath control. Not long after that I was suspected of “day drinking”, as I would start slurring by afternoon. I saw an internist, an ENT, then a Neurologist. She sent me for two MRIs suspecting a stroke or a tumor, then for an EMG. That was the evidence for ALS, in the summer of 2017. It was confirmed in early December that year. Bulbar Onset with respiratory weakness. I am still talking, slowly, and walking! My right hand has joined the party, and I have early ambulatory difficulties. I was a dancer, singer and actress, as well as a fine artist. Right handed, of course! ALS is a challenge but I have not given up! Take care of yourself and live everyday! Lisa
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Lisa, thank you SO much for sharing your experience! It sounds very similar to mine for the most part, and your attitude is so admirable and inspirational! Can’t thank you enough for your reply here. It’s very helpful and a solace to me.
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Hi Anne, please see a Lyme Literate doctor. I am aware of instances when the diagnosis was wrong. Happened actually to a physician. It can’t hurt. I’m sorry that ALS is 100% fatal. My 29 year old was diagnosed with Bulbar-onset last July. We are going to explore that route. As slim a chance as it is, anything is better than nothing. Message me if you’d like.
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I’m so sorry to hear about your child. 🙁
As far as Lyme, I don’t live anywhere near an area nor have I traveled anywhere near an area where that could be a possibility, but I appreciate the input.
Best of luck in your journey, so sorry you are here.
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Hi Anne,
My mom was diagnosed with bulbar onset about 4 months after her voice started slurring. Hers was similar to yours, where there was definitely a noticeable difference in her voice from morning to evening, and I could tell her voice got weaker throughout the day. It was about 6 months after her diagnosis (so about 10 months after the start of slurring) that her speaking was too hard for us to understand, and then she lost it entirely.
I love the idea of taking videos – I wish that I had more of my mom. I really treasure the ones that I was able to get. Also, if you’re up to it, it might be worthwhile to start voice banking now so that you could use your actual voice via a text-to-speak tool down the line.
Feel free to reach out with any questions! I learned a lot taking care of my mom and I’d be happy to give you the nitty gritty on her/our experience, if you think that’d be helpful.
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Hello Jen, thank you so much for sharing your experience, though I’m very sorry you and your mom are having to go through it. 🙁
Did/does she also have problems with drooling and with keeping her head up? These problems are happening to me, they start up in the late afternoon and worsen throughout the evening. I vaguely recall the drooling thing from at least back in May, I was embarrassed because it kept happening without my noticing til it was dripping down my chin – but it didn’t even occur to me that it could be an actual medical problem!
It’s frustrating to not be able to remember when the slurring started, I know it was definitely obvious to others starting about a month ago. I also can’t enunciate words well at night, it’s like my tongue is “thick” – hard to describe. That and my voice becomes very monotone (according to my husband) versus how “animated” it used to be, if that makes sense? My previously loud voice is definitely gone (I can find some humor in that, I remember always knowing I was too loud and feeling self-conscious but didn’t know how to change it, so well, problem solved! Sorry, I have to keep my sense of humor intact!).
I’ve been taking videos lately in the mornings, one with my husband and most just me yammering. I plan to make other ones with the kids, etc. (not morose, sad stuff, just “a day in the life” type stuff).
Great advice on the voice banking, I will start researching. This is all frightening and I want to be prepared.
Thank you so much again for sharing your experience and the general timeline – I know everyone is different, but it DOES help to get some ideas of what others have gone through! I will probably take you up on your offer to message you, thank you so much for that, it’s very kind.
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Hi Anne. I too experienced uncontrolled drooling and total loss of speech just a few months after I was diagnosed with bulbar ALS in March 2020. Like you, I was embarrassed whenever I drooled and often didn’t realize it until it dripped down my chin. Now I wear a bib, even at night. But the good news is my doctor prescribed HYOSCYAMINE SULFATE 0.125 MG SL TAB, which I take dissolved in a small cup of coffee three times a day (don’t even notice it). It really works well. I get minimal secretions after taking it. I began to lose my speaking ability early on. It started with mispronouncing certain words, eventually my voice pitch changed dramatically and finally all I can make are moans and groans. Taking videos now while you can is a great idea. Remember, things that you can do today will not be possible in the days, weeks and months ahead. That’s the nature of the beast. If you are able to word bank, you need to get on it right away before it is too late. I too have difficulty keeping my head upright. Been like that for months now. It’s extremely painful when my head moves in either direction. I take a low dose of morphine twice a day and it pretty much eliminates the pain (Hospice provides the morphine). Hope this information helps. I wish you the very best Anne.
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Hi Anne, I’ve been following this discussion and all the good comments from our members. I’ll add mine as well; mine are tips & suggestions for how I deal with the various symptoms of ALS.
– “Losing my voice” …I’ve been living with this for several years now and am happy to share with you what I do to not only keep speaking with my own voice but have others understand me as well.
About 6 years ago (5 years after the onset of my ALS), I began to have the “thick tongue” feeling and noticed trouble speaking – – slow words, breathy sound, and unintelligible as the evening wore on. Here’s what I did, and now 6 years later, I am still speaking.
– I took the online course by voice coach Andrea Caban. http://www.andreacaban.com/als And wrote about it here:“Dysarthria? Use These Innovative Techniques” I learned to redirect my breath from “upper chest speaking” to using my diaphragm, like an opera singer. This gave me volume, extended the number of words with one breath, and I was less tired speaking. I also learned to move consonants like “gah” and “kah” more forward in my mouth, and not in my throat.
– I use the Talk-to-text app on my phone to speak to our Alexa. This relieves my frustration with voice-activated devices not understanding me.
– When in a group, or riding in our van, I am heard above the noise by using a personal microphone. I wrote about it here: “Listen! Can You Hear Me Now?” and here: “Hooray! You Can Hear Me Now” Here is the microphone.
– Everyday day I practice breathing exercises. I describe them here: “How Blowing Bubbles Helps Me Breathe” And I do a tongue warm-up plus mouth stretches like: pronouncing an elongated “EEE”, “KEUWW”, “EEE”, “ARRR”.
– Sitting posture during the day is also important. I try to avoid slouching and slumping, which restrict correct breathing and weakens your breathing muscles. I wrote about sitting posture here: “A Healthy Sit”
I share more tips on my ALS and Wellness Blog
Hope these tips help you! I’m happy to offer more. -
Hi Anne, I’ve been following this discussion and all the good comments from our members. I’ll add mine as well; mine are tips & suggestions for how I deal with the various symptoms of ALS.
– “Losing my voice” …I’ve been living with this for several years now and am happy to share with you what I do to not only keep speaking with my own voice but have others understand me as well.
About 6 years ago (5 years after the onset of my ALS), I began to have the “thick tongue” feeling and noticed trouble speaking – – slow words, breathy sound, and unintelligible as the evening wore on. Here’s what I did, and now 6 years later, I am still speaking.
– I took the online course by voice coach Andrea Caban. http://www.andreacaban.com/als And wrote about it here:“Dysarthria? Use These Innovative Techniques” I learned to redirect my breath from “upper chest speaking” to using my diaphragm, like an opera singer. This gave me volume, extended the number of words with one breath, and I was less tired speaking. I also learned to move consonants like “gah” and “kah” more forward in my mouth, and not in my throat.
– I use the Talk-to-text app on my phone to speak to our Alexa. This relieves my frustration with voice-activated devices not understanding me.
– When in a group, or riding in our van, I am heard above the noise by using a personal microphone. I wrote about it here: “Listen! Can You Hear Me Now?” and here: “Hooray! You Can Hear Me Now” Here is the microphone.
– Everyday day I practice breathing exercises. I describe them here: “How Blowing Bubbles Helps Me Breathe” And I do a tongue warm-up plus mouth stretches like: pronouncing an elongated “EEE”, “KEUWW”, “EEE”, “ARRR”.
– Sitting posture during the day is also important. I try to avoid slouching and slumping, which restrict correct breathing and weakens your breathing muscles. I wrote about sitting posture here: “A Healthy Sit”
I share more tips on my ALS and Wellness Blog
Hope these tips help you! I’m happy to offer more.
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