Lung Function Question

[MVFinVA]

My husband was diagnosed in May 2017, but we can look back and see symptoms that we didn't recognize in August 2016. He had his first ALS Clinic visit in June 2017. At that time his Forced Vital Capacity (FVC) was 3.9 and they said his lungs were great. It dropped to 3.6 in October and 3.3 in January. He was just tested today and it had dropped to 2.4. I had anticipated it was going to drop. The FVC was the only test they could get accurate results on today because he has FTD also and has trouble following through on directions - he couldn't keep his lips sealed.

His symptoms are all upper body - legs are fine. He is about to receive a feeding tube on Tuesday due to difficulty eating and weight loss.

From an entirely un-medical view, just a wife's of 30 years gut feeling - the progression seems to have really sped up in the last three months.

Any insight into the lung function numbers would be greatly appreciated.

Mary

 

[KimT]

Hi Mary,

If they couldn't get a good seal, it's possible that his reading was inaccurate because air can escape.

Is he having trouble breathing or does he seem to get out of breath when he walks? Is he using BiPAP?

It's hard to interpret raw values in FVC because they are based on height, weight, and gender. Also, different clinics use different normal values. Did they give you a percent next to the raw value? Did they mention that not getting a good seal could affect the reading?

 

[MVFinVA]

Thanks Kim. They seemed to get a seal fine on the FVC as they did that test first. It seemed to get more difficult as he progressed. They just ordered a BiPAP last week and are waiting for it to be delivered. He does seem to be getting out of breath when he walks. In the fall he could walk about a mile around our neighborhood and not get winded. This spring he seems worn out at 1/4 mile. I've encouraged him that maybe just sitting outside would be good.

They didn't give us any percents, although they will be on the report. We were at the VA Medical Center. I can get a copy of the report when it's completed. I just wrote down the raw value as they were doing the test. Previously when the test was done it was just with the handheld spirometer. This time we were actually in the Pulmonary Lab and it was hooked up to a computer.

Mary

 

[KimT]

Full pulmonary function test can be exhausting. I coughed for about an hour after my last one.

The report should give a better overall view of where he stands but just remember numbers don't always coincide with how a PALS feels and what he needs. Maybe shorter walks that are more frequent would help.

For what it's worth, I had three full pulmonary function tests at three different ALS Centers and got three different readings. They were done six months apart and my best one was the one in the middle.

I'm starting to use my cough assist machine to expand my lungs. I have done breath stacking in the past but the cough assist, operated in reverse, seems to work better. Did they order a cough assist machine for him?

I would not discourage him from walking because retaining the muscles in his legs for as long as possible will help. I would make sure he isn't exhausted when he does walk.

I'm glad he is getting a BiPAP. That should give him more energy. Try different masks. Let him ease into it. You can start with as little as 5 minutes sitting in the LR watching TV. Also, settings will need to be adjusted as time goes on and, from my experience, even in the beginning.

I think sitting outside is always good for PALS.

 

[MVFinVA]

They didn't order a cough assist as far as I know. But with the VA things seem to just show up

 

[lgelb]

VA, the VA may keep throwing exactly what your husband needs your way, but it's always best to know what, when and why, to be the squeaky wheel when needed, and keep your own records.

As others have said, the %s and each of the measures together, not just FVC, will give a clearer picture. I am also happy to help you tweak the BiPAP settings if/as needed.

Best,
Laurie

 

[MVFinVA]

Laurie,
Thank you! I am going to try to get a copy of today's test results and the previous ones also. The next hurdle is the feeding tube, then the BiPAP. Feeling a little overwhelmed that they are happening almost on top of each other! I knew they would both come but not at the same time

I am so thankful for this forum, although I have to be careful reading or I can have too much info at once. I need to process as I go or I can get anxious. So many things we will have to deal with that I never wanted to need to know about. But through the encouragement of PALS and CALS here, and CALS at the monthly support group I attend, I know we can do things we never thought we could! Just need to put one step in front of the other and keep moving forward.

Mary

 

[whereswaldo]

Mary,
I think you are doing fine, and you sound like you can handle it. I received both of mine about the same time and it worked out ok.

I think the feeding tube is what you should put most of your efforts into. To me, after the fact, taking care of the tube can be overwhelming when you are at a point having to, instead of, learning when you have time. The more you can learn before the procedure, the better off you will be. A nurse was trying to explain to my wife and I in recovery, with a full house going on around us. Needless to say, we had to make a couple of phone calls that first night. It ended up no big deal, but it didn't feel like that at the time.

The bipap will take some time to get it adjusted right and will require some visits from a RT to get it right. Comfort will be the important part. There are many masks to find something that will work.

Good Luck

 

[lgelb]

Waldo is right on -- get the tube stabilized first. You will find many helpful hints here on options such as gravity feeding, blending real food, etc.

And as Kim says, you will want to go slow and easy with the BiPAP, esp. with FTD. Literally, I would start with five minutes during the day. The settings are frequently set too high in the beginning, but it's easy to drop down and work your way back up to the right level, so just be prepared to reassure him that it can be adjusted to his comfort and make sure that happens.

 

[MVFinVA]

Thanks Laurie and Waldo! It will be just 13 days between when the doc said we should move forward with the feeding tube and the procedure. It was feeling too fast. But the last couple of days he has really struggled when eating - lots of coughing. Now I an feeling relieved it will be in in four more days! We know someone who had a loved one with a feeding tube for over 30 years. Thankful to have people near who can give calm advice if needed!

 

[wishmobbing]

Good the feeding tube is right around the corner and that you have experienced people a call away.