Breathing Test Results

[Blessed1]

- My wife is scheduled to be seen @ an ALS Clinic, for a second opinion 3 day workup, but the appointment is over a month away. The doctors are undecided if she has ALS or PLS

- Her not very involved local neurologist scheduled a pulmonology test for her (2 months ago), and she finally had the test this past week. and I went and got the results. We are still waiting for him to call us with his opinion about the results.

My wife is currently on a CPAP when sleeping, and she is experiencing shortness of breath, mostly when lying down. She has had insomnia for several months that medication does nothing for.

Just wondering if it is safe for her to continue use of her CPAP while waiting on the ALS clinic visit. I know none of yall are doctors, just looking for some practical advice from others who have maybe gone though this. Just a concerned husband.

They did the test sitting up and lying down. But results show only PE Max and PI Max sitting vs lying down.

Anyway here are her results:
Note: No airflow obstruction

Spirometry
PVC- 90% of predicted
FEV1 - 80% of predicted
FEV1/FVC - 88% of predicted
FEF25-75% - 57% of predicted
PEF - 78% of predicted
PI Max - 86% of predicted sitting up | 56% of predicted lying down
PE Max - 44% of predicted sitting up | 35% of predicted lying down

Lung Volumes
VC - 90% predicted
IC - 103% predicted
ERV - 38% predicted

Thank you for your time.

 

[GregK]

Laurie will be the best to chime in, but

CPAP isn't appropriate for ALS, we use Bipap, which has 2 levels of pressure ( hence BiPap), to make exhalation easier.

 

[lgelb]

Hi, Louisiana,
The results suggest that your wife is weaker in the muscles that we use for breathing out, than breathing in. So where she is having difficulty is getting CO2 out. Why does she have a CPAP at the moment and is she overweight?

If you can tell me what model CPAP she has, I can tell you if it has an EPR or "flex" option that can provide some level of "pressure support," where the pressure she breathes against when breathing out is lower than the air she gets breathing in. Some machines provide up to 3cm difference, which she very much needs based on these tests.

My recommendation without knowing the answers to the questions I asked, would be either to max out that difference (I can tell you how) or if the machine doesn't have that option, to minimize the PAP pressure overall, to 4.

Do you know her current settings and how to change them?

As to whether it's better to use the machine or not, that depends on your answers.

Best,
Laurie

 

[Blessed1]

Hello, Laurie

Thanks for your response.

About 4 years ago after she had a surgery and while monitoring in the hospital they discovered her oxygen would drop off when she went to sleep. They did a sleep study and diagnosed her as having obstructive sleep apnea. She has been on a CPAP every since. Yes she is overweight. When this all started she had a neuro that thought she had MS and he put her on two separate week long rounds of IV Steroids to hopefully help her, but all it did was make her put on weight.

She has a Resmed S9 Series Autoset
Pressure Setting: Fixed 9 (cmH2O)
Pressure Relief EPR: Full Time 3cmH2O

Not sure how to change the Pressure Relief.

From reading the machine:
Past 30 Days:
AHI: 4.79
Obstructive Index: 4.19
Hypopnea Index: .57
Clear Airway Index: .01
(These numbers have steadily gotten worse over the past 3 months)

Thank you so so much for your help

 

[lgelb]

The pressure relief is maxed out. Full-time/3 is the highest level that machine can give her. Her numbers are OK for someone with obstructive apnea, but of course we know that is not her only problem, and we won't get numbers from the CPAP about CO2 retention or work of breathing, which I suspect has increased.

I would certainly buy an oximeter and track her pulse and respiratory rate.

So for now I would change the mode on your machine from fixed to Autoset, set the min pressure to 7 (it has to be that high because of the pressure relief being so limited) and the max to 9. It's actually more important to support exhalation than inhalation in sleep at the moment, given her shortness of breath, so I'd start there (let me know if you need help), erring on the side of lower EPAP [exhalation pressure], and then see what the numbers are, and how she feels/breathes. It's a process to get the settings right, even with the right machine, which yours is not.

But as we all agree, she needs BiPAP with volume control modes (perhaps during the day at times, as well as in sleep), and shouldn't need to wait for an ALS clinic appointment to get it, based on an MND dx and her symptoms. My husband and many others got the change-from-CPAP-to-BiPAP rx from a primary care doc. She can, as well. If necessary, hand the PFTs and the dx to her PCP and stand there until you get a script. The settings don't matter. They can (and must) be adjusted to her condition. Definitely stay with ResMed S9 machines -- switching to Respironics would feel like opening a window on the freeway -- try for the S9 VPAP ST.

Ultimately, she will need an Astral or a Trilogy to go out. But for now, not knowing your financial situation, I'll just throw out that new/used BiPAPs are available online once you have a rx -- you don't have to go the DME route if you don't want to. For many people, the copays exceed what you'd pay in cash.

 

[Blessed1]

Thank you so so so much for your help. I have already made your recommended changes on her CPAP machine.
If her neurologist doesn't take care of it this week, we will get her PCP to take care if next week.
As far as our financial situation, right now her out of pocket and deductible have been met through private insurance. So If it can be prescribed she can probably get it out of pocket for no cost to us.

Thanks again for your time!