Methotrexate not working -

[Brooke]

This past weekend, I was coughing more and felt more wheezy/out of breath. I go to mayo in a couple of weeks and I also put a call in to my pulmonologist today.
I am wondering if there is a different drug they might try if the methotrexate isn't working.
Is there something similar to metho or would I have to go on the powerful drug?

[JanW]

Since I know that you have SS, Brooke, you are going to want to have them check your windpipe again (maybe they do this anyway?). All the symptoms you note are also true of SS and severity of disease doesn't correlate with it -- that is your stenosis can worsen quickly while you disease is fairly quiet. Do you have a local ENT on the case?

[Brooke]

Thanks Jan~ I know they will check my windpipe when I am at mayo because I am getting a bronchoscopy, plus I will be seeing ENT there. I do have an ENT that I see here but he is not managing my case. I could probably get in to see him but I think I will wait until I go to mayo if I can.
The doctor at mayo also believes I have asthma. I have done numerous breathing tests and they still believe I have asthma along with wegeners. Maybe I do then?

[Jack]

Are you taking bets? ;)

[Brooke]

Whats that Jack? Are you going to bet that I will have to be on something different? Oh man, I hope not.

[JanW]

I'm with Jack -- are you take bets?

On what basis does he believe that you have asthma? And is this adult onset. Simply not that common...but a very common misdiagnosis in us Weggies. Look, all they have to do is look at the flow volume loop on your breathing test --- it's not even anything they have to figure out on their own. The machine tells them where the obstruction in breathing is coming from.

Could you have a bunch of other stuff going on with your lungs -- sure? But in you SS is still going to be the likliest cause of breathlessness, I think.

[Brooke]

Jan~
I have had that test done, but I am not sure the results of that or even how to read it?
Does prednisone help with SS? I am currently on 20 mg. I have been on methotrexate since November, as I said in a previous post, my lungs looked good via CT Scan in January but my sinuses are still funky.

[JanW]

If you go on the internet and look up flow volume loop and wegener's in google, you will actually be able to see some graphical examples. The loop flattens when an SS patient breathes in -- that shows that there is extra-thoracic (outside of the lungs, in other words) involvement. The point is that your docs should be reading this though -- not that you should have to -- and that they should be making sure that loop corresponds with obstruction or tightening in the lungs and not higher up in the airway.

Medically, who knows what drugs work with SS? SS is very rare -- most WGs will never encounter this problem. Occuring as it does in less than 20 percent of WGs, all the studies are tiny. Some seem to indicate improvement if treated with ctx and steroids -- but I don't even know if that's the latest thinking (looks like the study was done before RAVE established the efficacy of rtx). The windpipe action does runs independent of the course of your WG -- we know that. WG SS patients are more likely to get saddle nose involvement but less likely to have renal involvement, if you want to take the good with the bad. There are a few power point presenations on the internet that are quite good on this subject.

What does work, if it gets bad enough, is surgery. When they go in, they will swab the area with chemo drugs, which, as my doc says, may work or may not. It's just the state of the art at this point. I had no medical option at the point that mine was discovered and I was certainly made aware that in the future it would be handled surgically as well.

[Brooke]

Wow, thanks Jan. I had no idea that wgs with ss are more likely to get saddle nose - which I had! I think I would rather have that than the kidney involvement.
I will talk to my doctor at mayo and ask her about my results of the breathing test. She obviously knows I have SS so I will see what she says now about asthma.
She did mention that we can fix SS with surgery but she didn't want me to have that at this point with the active wgs going on. I think once I get some inflammation down, it is a possibility if needed.

[elephant]

Brooke, you have many options when it comes to medicines for the Wegeners. What kind of sinus problems are you having now?

[JanW]

I of course defer to the experts at Mayo (and I remember you telling me that your peak flow is about 400 so it doesn't seem as though you are in any immediate danger) but you don't have to wait until your WGs isn't active before having this surgery. For some of us, WGs will always be active and we will always on medication. I stress this because I know that others have been told (I'm looking at you ticklytoes) that they can't have surgery with active WG because it will just come back. Well, it will or it won't but that's not determined by the progression of the systemic disease. When I expressed surprise to my surgeon that he would operate on me newly diagnosed and as yet medically untreated he replied, "We (meaning surgeons) operate on people who are half dead all of the time, and you're not even that." He then followed it up with the old joke about "why do they nail cancer patients' coffins shut?" "so the oncologists can't get one last round in!" Sorry if this offends anyone, but my only point is is that when this surgery is urgent enough you do it as long as you believe that the patient will get out alive, regardless of what other stuff is going on. And as you have read, there are people on these boards who have surgeries similiar to my own done several times.

[elephant]

Well stated Jan, and it makes sense especially when your dealing with the windpipe to keep you alive. In my case it was the maxillary sinus ( I had four surgeries) and everytime the surgeon went in to clean it out and open it up it would close back in 4 weeks! My disease was active and was not on medicine for the WG because no one knew I had it.

[Jack]

I was on the transplant waiting list while still having regular flares and had a couple of them after transplant, but no harm was done.

[JanW]

The surgeon described it as the doctors' ABCs -- A - establish an airway, B stop the bleeding, C control the circulation (blood pressure, etc.). There is nothing more important that your airway. No life without breath.

[Brooke]

I am anxious to see how my windpipe looks compared to last September, 2009. In September, doctor at mayo said I have scarring and narrowing but said that it wasn't so bad that I needed surgery right away.

[JanW]

The thing is that they never know how quickly something like this is going to progress...but it's definitely time to have it looked at again. Particularly if you seem to be feeling new symptoms. I noticed that I was getting increasingly short of breath in the last six months despite the fact that I was moving around very little due to problems with my ankle. The nurses were surprised that I could even breathe easily while lying down, but I could (although nothing compared to what I can do now).

[Brooke]

Thanks Jan, I will be sure to get it checked out:) This isn't a new symptom to me, it just seems it was better for a while and now back to where I was. It seems like every week is different for me, some days are good and some are not. The minute I think I am getting better, for example I might have great nights sleep - well then the next night I'm coughing again!

[JanW]

Definitely the same here. It's also more difficult to clear your throat when you have a stenosis -- so there's that problem as well.

[Brooke]

Jan~
Do you ever cough "stuff" up or just have a cough? I do a lot, it is clear sometimes whitish in color. I am thinking it is my sinus drainage that gets into my chest.

[Sangye]

Wow-- this is a GREAT thread! Jan, you've got so much useful info about SS. I LOVE the onco joke. Never heard that one, but it's so true! My hematologist/oncologist keeps telling my Wegs doc to do more rtx infusions. I've told him the Weggie protocol is 4 infusions every 4-9 months. He says "Nah, we give NHL patients 16 infusions at a time!" Wegs ain't NHL.

Brooke, last September is a long way back in Weggie Time. If your Wegs hasn't been responding well to mtx, there very well could have been progression of the SS. If you were just going to a local rheumy and ENT I'd be worried. But I know you're in good hands at Mayo. Like Elephant said, you have many treatment options. Don't worry about which one. Just take it one step at a time. :)

[JanW]

Sangye -- SS is pretty much my "thing" on this board, and because I have had my surgery done with such a reknowned specialist, I really consider it a "duty" to share what I know. I know so many Weggies end up with traches -- even with their airways more open than mine was at it worst -- and it doesn't have to be that way. I also know that SS doesn't follow disease progression -- so your mtx could be working, and yet your SS could be progressing. And it's a stubborn little bugger to fix, too. I just don't want anyone's SS to get as bad as mine got -- it was awful and I didn't even know it! And the recovery, at least from the way that my particular surgery was done, was so easy it was like a night in the spa for someone who is normally taking care of youngish kids!

[Sangye]

You go, girl. You're helping a lot of people, and more to come (unfortunately).

[elephant]

I always enjoy reading every one's comments. They are all so valuable and informative. We are all learning from each other and understanding the disease, this is better than a medical book and any other book. I feel by this summer....I will be a WG expert!

[Brooke]

I agree - so much great information from everyone, thanks so much.