Hello and very nervous

[cathy2330]

Thanks Sanyge and Jan for all your information. I don't want to live this way either and I am making appts and seeing docs and have been since Jan. and being admitted to the hospital. Everything takes SO LONG though (and I think I am just feeling sorry for myself and sometimes it seems easier to give up) but I would not do that. I would not go ahead with a procedure without advice etc but I don't think that we have the same doctors who know as much about Wegs as you do in US. I googled here looking for a specialist in Vancouver Area and nothing...My doc will try the doc in Mayo clinic but I can't go there for surgery so I need to find someone here. I am hoping to talk with the enT I am seeing tomorrow and see what his ideas are.

Jan are you on medication now after your surgery or is everything good other than the saddle nose? I looked at the sight you sent me and very good stuff. I looked into more of the type of surgery you had and it really seems like something that would work on my subglottic area also. But then I have the bronchis to deal with and don't know what to do about that..

I really am scared for the med, cytoxan IV drip, and have heard so much about nauseau, fatigue etc and long term effects . Doc really seems to think that I need medication though because she is concerned of never inflamation in bronchis, trachea etc. About 8 yrs ago I only had the narrowing in right bronchi and somewhere in the last eight years and went to my left bronchi and the windpipe. They do not want anymore narrowing and only think med will work.

What I read though med does not seem to help prevent subglotiic stenosis? I am not sure if that is correct or not, but then how do I stop any more in brochi's.. It really is confusing to me and hopefully I will get more info from the ENT. I am aslo seeing an opthomologist tomorrow also as I have problems with my eyes and it needs to get checked.

Hope everyone has a great night...I don't know what time it is in your area but it is about 7:30PM here and I am going to have my bath and read my book and get to bed early as I am off to more medical appts. tomorrow.

Good night everyone!

[jola57]

Cathy why is you doc giving you IV cyclo, if possible ask for the pills, although you must take them every day they are not as volitile and are less likey to cause nausea.

[Sangye]

IV ctx is not as effective as oral, either.

It's easy to get overwhelmed, Cathy. Especially when you're already sick and in your case, low on air! Stay determined, though. No one else will fight for your life more than you.

I don't see any way around the big guns-- cytoxan or rituxan. Your lungs are almost closed for business. From a medical perspective, that's the biggest issue. They could always do a tracheotomy to establish an airway higher up, but if your bronchii are closed, it's over.

Tell your doc to get in touch with Mayo AND Dr Lebovics. You don't have to go there to get help-- they can guide your local docs. You can trust whatever they tell you.

[JanW]

I am only on mtx, 15 mg/week...I go for a follow up in a couple of weeks so we'll see if there's been any change in the CRP and SED rate (my doc doesn't monitor ANCA or P3 as a marker of disease -- it is for some people but not all). Since my more general inflammation markers were high enough as it was, that will at least give him something to work off of -- since I'm not on prednisone the only thing that would lower them would be the mtx.

I don't think that my docs believe at all that medication will prevent scar tissue from recurring in my windpipe -- but it's likely that my nose won't keep getting worse and worse -- the tip is still holding pretty firm at this point. Lebovics requires that you be drug free for six months before he'll do the nose surgery (with a plastics guy, of course) but that's down from a year and frankly, he says, he's just starting to do them on people still on meds, because lots of people don't get off and it doesn't mean that it will collaspe again (and for some people -- not my case -- it's incredibly disfiguring to live with; they just get stared at all the time). They wouldn't give me a 'stronger' drug -- let's say rtx vs. mtx just because I had SS...it's more like I'm being medically treated because I have positive ANCA, very high PR3s and high levels of inflammation in my body. But the reason that it isn't being treated with steroids is that it's not urgent to get those numbers down -- my health hasn't deteriorated at all in the five months since those levels were taken. In fact, I would say that I 'feel' better in the month I've been on mtx.

[elephant]

Hey Jan, so glad you are feeling better! Hopefully the only drug for you will be methotrexate and it seems to be working for you. Give the foot some more time. You might be surprised.

[Col 23]

Hi Jan
Do you get much nausea on the Mtx Ive found that the longer Ive been on it the more nauseous I get. However its not every day so thats good. How long have you just been taking Mtx. Im going to refuse any raising of the preds as soon as I get back into my Rheumy or if my GP will sort it out.
Gone crazy be back soon Col 23

[JanW]

I've never even had a hint of nausea so so far, so good. I've been at 15 mg for 3 wks, before that 7.5 for 3 weeks.

Keep in mind that I don't take pred, I don't know how that would affect it. And I'm the kind who gets nausea pretty easily, actually.

[cathy2330]

Hello everybody I would really love your opinions!

Went to the ENT yesterday and after examination of nasal which involves crusting and scarring down both sides of nasal passages he decided to have a neck and sinus ct scan. Had never had one yet. Got it down right away and went back to him and he stated that in the sinus cavities above the cheek bone the is severe inflammation/infection??and he has scheduled tomorrow for a cleaning??and examination of that area and also to do a Deep Tissue Biopsy to see if we can get a confirmation of Wegs. Also went to opthamolgist and eyes look good none of the typical signs of Wegs.

Then my rheumy called me when I got home and asked how things went. Itold her what was being done. She told me she talked to a wegs specialist at the Mayo clinic named Dr. Langston, I believe and proceed to try to explain to me what she said about my case. After what seemed like going around in circles I gather she believes that we need a definite sign that this is Weg. Possibly the deep tissue biopsy will confirm or not confirm. Does anyone know the approximation of this bein accurate on this area?? She kept saying that this was dire and we need to do something but wants to wait until she gets the results. Two weeks ago she was all ready to start the cytoxan.. I can appreciate that but my problem is either yes this will come back and confirm Weg or no it will not.. To me there should be a plan in place for either scenerio as I have been tested up the ying yang , been in the hospital for over 5 weeks, been under general anesthic over 7 times in the last 4 months and feel I am no closer to knowing what is happening or what to do about it than I was. In my mind we should have a plan if it comes back negative or if it comes back positive. I find that she is very unsure of what to do. She has told me that maybe we should do prednisone along with the IV Cytoxan. I told her I was really nervous of the pred but if she really thinks it is necessary that of course I will do. She said that we could start the dosage really low and then increase and try a very low level of cytoxan. Then she kinda recinds on that and states lets just see. I explained that if it does not confirm Wegs, obviously something is going on as normal people do not have the sinus issues, narrowing of windpipe and major airways without something causing it and something needs to be done to treat it.

Oh and the ENT truly thinks it is the classical sign of Wegs. just from what he has heard and seen in my sinuses. Also went on Monday and had blood work done. Everything ESR, CBC and urinalysis all within normal range. I am now getting copies sent to me (learned through this website thank you for the great info). Would that mean that I don't have Wegs? Or is it in remission?? Can it go into remission without ever being treated with pred. or any med.? Could all of this happened a while ago even the subglottical area and now it is not active (hence the normal blood). I have had the increasing probs. with shortness of breath for over a year before I finally went to the docs. If it is not Wegs does anyone have any idea of what I should do.? I don't feel like my doctor knows and I don't honestly have faith that she would know what to do as we have no idea then what would be causing this. I then thought that maybe just see if ENT can do something to help the areas in the sinus and subglottic that have been damaged to help keep it open for a longer period and the thoracic surgeon do something for the airway?? I don't know what as he stated stents to dangerous I guess keep dialating. And then just hope that no new inflammation occurs and if noticing any problems get bronched right away. Or I could just assume it is some sort of autoimmune disease and try the meds and see if they help, but they are so toxic that not sure if I should do. I am really at a loss and I know with my luck it will come back negative, I was telling my husband that this is absolutely insane, but I want a diagnosis of Wegs. so that at least I know it can be treated and how it can be treated. If it is negative I am so LOST... I don't know if another rheumy would help as this is the 2nd and I have had every specialist in the hospital, Infectious disease etc andd nobody seems to know. Fingers crossed that it is positive Ha Ha HA, i think I am truly CRAZY!!!!

Any ideas I would love. Thank you so much everyone this has been so great...I can say at this point I am more confident in your guyes help and suggestions as you all together as a group, each with your own experiences are much more knowledgeable it seems than the docs. I know that ultimately they are the docs but suggestions and ideas I think are very important as this seems to be areal puzzler and I trust all of you on here. Doesn't mean I am going to do exactly as you say (I'm not totally CRAZY ha ha ha only slightly).

Thanks so much everyone. Hope you all are doing well.........and have a really great day today!!

Cathy xoxoxo

[JanW]

First off..it is possible to have WG and normal bloodwork. My rheumy has patients like this (I'm not one of them).

Second, you'll have to treat your airway problem regardless -- but your doc is right in that she may want to do things differently is medically there is absolutely no sign of WG -- did you say that your c-ANCA and P3 was normal also, I forget? My surgeon would have treated me the same whether I had WG or not, so it didn't matter, including the chemo treatment. That would work for sarc as well which was the second most likely diagnosis (especially because I'm black).

Third, it's notoriously difficult to get biopsiable tissue from the sinsus -- Lebovics said so. There's nothing in mine to get but he cut some material out of my subglottis and it yielded a big fat nothing. A biopsy that indicates WG is definitely the 'gold standard' but a rheumy who has seen a lot of cases is definitely willing to make the call without it based on clinical symptoms and blood work -- and mine did a week after I saw him for the first time.

Certainly it's not uncommon to go for years without getting the SS fixed, and I definitely would say that my WG was quiet most of the time (5+ years). Was it technically in remission? We'll never know since I didn't know that I had it. However, once I started flaring and I started losing the bridge of my nose, I definitely found it was getting more difficult to breathe. And that happened over a period of months. Your SS will get worse. Believe it. You don't need to have even started treatment for your WG to have the surgery. I hadn't. The surgery came first.

I think it's also risky and unwise for you to be having all of these dilations which really do nothing other than putting you under general anesthesia. I do think your rheumy or your ENT needs to call Lebovics. If you can notice that they are in over their heads, they are in over their heads.

I'm not sure why they want to start with ctx...are they just pulling out big guns because of stenosis? Because it won't stop that. Stenosis says NOTHING about how sick you really are. It's simply a rare yet classic presentation of the disease.

Regardless of your diagnosis, you are going to have to get a real airway established sooner rather than later. I think either you or a doc should make a call to my surgeon.

[Sangye]

Hi Carol, well I'm glad your rheumy talked to Dr Langford. She's a wonderful Wegs specialist at Mayo.

Sinus biopsies are very often inconclusive or (-). They just can't take a large enough tissue. But that's not true 100% of the time, and if it's your only option then I think you have to go with it. (The only thing they could biopsy on me was ONE 3 mm wide skin lesion and it came back positive, so it can happen) I think if your case was clearly Wegs, they would have no hesitation in starting aggressive treatment. Many patients are given a diagnosis based on empirical evidence alone--ie, without biopsy.

I'm assuming they measured a CRP along with the ESR? Even if they are both normal, you can have active Wegs. For some reason, mine don't go up until I'm in the ICU. I've heard that from other Weggies, too. Not common, but can happen. Please tell your doc that and ignore if she rolls his eyes. I've had hemorrhaging lungs with normal inflammatory markers.

I'm very concerned about you having to guide treatment (asking if you should do pred, doses for ctx, etc...). I think you're right that your doc is in way over her head. To be honest, any rheumy would be with Wegs. It takes a Wegs specialist to treat it.

So my advice is just get the biopsy and go from there. You can't decide on anything without it.