Respiratory Onset ALS

[pinky]

As a CAL, I recently joined the forum. I am wondering if there are any folks out there with respiratory onset ALS? If so, would they like to establish a discussion? It seems to me that there are quite a few different considerations with this form of the disease, as the breath goes first, while arms and legs are still mostly functional. Our diagnosing neurologist says the incidence is about 3% of ALS diagnoses. Anyone else out there?
In my husband's case, he had respiratory symptoms for years, followed by the development of swallowing problems. His disease was acute at the time of diagnosis. So he progressed through loss of volume in his voice, breathing with neck and upper chest muscles, diaphragm cramps, weight loss, CO2 retention and so forth, becoming acutely ill.

 

[Janie H]

I was diagnosed with COPD in 2006, then went through a botched hysterectomy that took a year and 4 surgeries to recover. I decided to then start working out, I was up to five miles a day on the treadmill, went back to the lung doctor and he reversed his diagnosis to mild asthma and took me off the Advair that I had been taking for years.
About three months later I started getting choked a lot, I didn't notice my tongue faculations until a neurolist asked me how long it had been that way. breathing problems started soon after that. I have a trilogy machine and the last clinic visit they gave me a T that hooks to my nebulizer and trilogy and a asthma med called budesonide, it has helped a lot.

Janie H

 

[Clearwater AL]

Pinky, I see no one has responded to your Thread. Being your husband's diagnosis is only 3% of those diagnosed with ALS he has sadly cornered the "rarest of rare." Being that ALS is still touted as a rare disease. PLS supposedly being even rarer. Gee, 3% is like the period at the end of the sentence, "My husband has ALS." How difficult to deal with.

In my short time on the Forum I don't recall anyone with respiratory ALS onset. Maybe ALS members with respiratory difficulties will chime in with whatever support/conversation/discussion they can contribute.

Hopefully some CALS will give you the helpful but sad, "Welcome to the Forum.'

PS. Janie H, posted after you did... thanks.

 

[gooseberry]

Pinky, they have officially said my husband was bulbar onset but he presented with shortness of breath and pneumonia. His left diaphragm was paralyzed and the left phrenic nerve was gone.His neck muscles were so weak his head dropped forward. He has a lot of atrophy of his back,neck, and chest muscles He is using his rib muscles and remaining chest muscles to breathe. His shoulders lift significantly with each breath.


Its been a hard road. His voice was high pitched and sounded weak. It now sounds hoarse. The slp said the change is due to spasticity in the vocal cords causing the hoarseness. There was a fair amount of debate about als because umn signs werent apparent at diagnosis and because his presentation was so atypical. Now there is no doubt. Limbs are atrophying at a fast rate and he is losing function but the breathing remains the main issue.

 

[dalvin]

Can't say I was respiratory onset but by the time they figured out what was going on and sent me to the clinic so the rt could test, I was already down to 60 percent. That was a little over a year ago. Held steady there until I got an upper respiratory infection in February which caused it to drop to 21. After I recovered from the infection it came back up to 25 and has stayed steady there. If I remember correctly the other numbers such as breath intake volume and stuff were very low as well. I remember them saying a couple of them were less than 2 liters but have no idea what the numbers mean. Think a lot of my breathing is being done with my stomach muscles now

 

[ECpara]

Pinky, a few years ago my husband and I went to an ALS convention at Duke. We sat next to a man who had Respiratory Onset ALS. That was the first and only person I ever knew to have this. Rare indeed. I'm so sorry.

 

[affected]

It seems to me that there are quite a few different considerations with this form of the disease, as the breath goes first, while arms and legs are still mostly functional.

Hi pinky and welcome.

My Chris was bulbar onset, so not quite what you are after.

I'm wondering what the different considerations are, we may still be able to discuss them with you?

He was diagnosed July last year, can you tell us a little about what limb and speech/swallowing capacity he has now?

 

[Barbie]

I have a local friend whose husband has respiratory onset. he was on a vent before he was even diagnosed with ALS. he could still play the guitar a year after that--now he is too weak but the rest of his progression has been slow. he is about 3 years in post diagnosis.