Looking to learn

[DawneO]

My dearest friend was diagnosed with ALS on Jan 11. She has bulbar presentation. Speech is slurred, swallowing troubles at times, voice has changed. First sleep study and nocturnal oximetry are done, showing obstructive sleep apnea. Some fasiculations. Occasional lyrangospasm are really scary. We are trying to manage symptoms......

Lyrangospasms are the scariest. Pulmonary doc said they are all self limiting- worst case scenario she will pass out and then she will be able to breathe again. As you can imagine, I am looking for a better option that does not involve her dropping to the floor!

fasiculations....she feels a humming over her sternum- usually at night only. Could that be a fasiculation? she says it feels like a cat purring in her chest?

problems couching, especially at night...wakes up to cough, has trouble telling her muscles how to cough- feels like it might be upper airway- mucous in her upper airway. Will chest percussion help with that? Stacked breathing to try and cough? Will the CPAP help?

new development in the past couple days is an almost constant need to clear her throat. Anyone have that?

And also- any norms for the longevity associated with Bulbar presentation ALS? How much time do we have? we have a lot of things to do and people to see....a lot of living to pack into the time. Any stats on the percentage of people who start bulbar and progress to limb involvement? how will we know if she is developing limb involvement? just wait and see?

thanks for all of your help.

 

[sadiemae]

For the laryngospasms, have her breathe through her nose. Also she should be on BiPap and not CPap.

 

[DawneO]

I know she should be on BiPAP instead of CPAP but the pulmonary doc won't order bipap and she cant get into the ALS clinic until March. it has been a headache. we are going to start with the CPAP so that she has something and switch to bipap when the ALS clinical sees.

 

[Ms. Pie]

During the Laryngospasm the best thing to do is stay as calm as she can and like Sadiemae says, she needs to take small breaths through her nose. They are very scary but her larynx will relax within a minute or two.

 

[momap53]

Look into the cough assist machine. It's a tremendous help as the respiratory muscles weaken. A percussion "Vest" is also helpful.
At one time bulbar presentation was considered to be quicker, but with the use of PEGs and maintaining weight, that's not necessarily true. Progression varies so much from PALS to PALS it's hard to predict how much time is left. Most recommend doing the things you want while you can

 

[hangingon1]

Just read past post and you will have more knowledge than her physician. Honestly, since ALS is so rare, the medical profession could give a hang about it. She probable does not have a lot of time with the bulbar onset. I have noticed that when that is the first symptom, the patient usually does not linger and suffer too long.