Hello and very nervous

[cathy2330]

Hi for some reason I am very nervous to be on here. I have been reading all the stories and following for sometime but finally got the courage to become a member and now post on here. I am from Maple Ridge, BC Canada. My story goes back aways but I will try to summarize. About 10 years ago I got tired of people always telling me I had asthma and was finding breathing getting more and more difficult. Was referred to a respirologist in New West who order many tests, pulmanary function, bronchoscopy, blood etc. She discovered a narrowing in my right lower brochi and scarring on my lungs. She referred me to Thoracic Dr. Ken Evans who did bronchoscopy and noted the same things. I don't remember how narrow it was but fairly narrow. He did not want to stent (as he felt stents had more negative than positive). They just decided to monitor me over the years. I went yearly to the resporologist and yearly CT scans. Nothing new ever showed on the scans and I just tolerated being short of breath. In the summer of 2009 I noticed that breathing was getting harder and harder. I could not vacuum, shower even talk without being short of breath and dizzy. Went to resporologist who did another CT scan and pulmanary function test(my pulm. tests are never normal) and nothing new showed on ct scan. She decided to do another brochoscopy on Jan. 11 and was greatly surprised to see another large narrowing in my left bronchi , my right bronchi and shrunk to the size of a pen and a large narrowing at the top of my trachea extremely close to my vocal cords. That narrowing was down to 4mm. I spent the night in emergency and then transferred to Vancouver General Hospital. I spent 5 weeks in the hospital having every test imanginable. Infectious disease, tests, Weg tests, lupus everything. Everything negative. Dr. Evans did a balloon dialation on the 3rd day in the hospital.

After all the tests he brought a rheumatologist in who decided on a iv high dose 3 day treatment of prednisone. It did not do anything. Dr. Evans then did another bronch to do some measuring as he decided the best thing would be a Y stent. On Feb. 1 Surgery to put in stent. After 2 hrs he could not get stent in. He was using smallest stent and it was too big. He was also very nervous of how close it was to the vocal cords. he Did another dialation while in as it had shrunk back some again. Spent 5 days in ICU as my throat which was already narrowed swelled up very badly and I could not breath. Something interesting that was discovered was the heliox works for narrowed airway. It is a combination on helium and oxygen and it is less dense that oxygen and goes through the narrowings easier. I had a huge helium tank by my bedside. Rheumatloogist decided to treat as Wegs as they had no other ideas and needed to stop the narrowing and this was the best guess. Started me on 50 ml of Imuran. Imuran for the first couple of weeks made me nasaues and tired but did tolerate. Then he upped to 100ml and have never been so ill. Severe vomiting for hours, shakes, chills awful. Told to stop immediatly.

Released from the hospital, changed rheumatoid docs (did not like the last one brushed off my concerns of vomiting etc.) and started again. New rheumatoid did whole new battery of tests and again Weg test negative. Also while in hospital saw a ENT who did a look down nasal passages and discovered extreme crusting, narrowing down the entire nasal passages. Narrowing is old scar tissue. I could never breath through my nose. Met again with rheumy who decided to say I do have Wegs. as she has seen some instances when confined to subglottic area and nasal where initially neg. result. She has seen a study where only 57% of patients with presumed WG with subglottic stenosis intially have a positive anca level. She is sending me for a more thorough exam with new ENT at St. Pauls hospital on Wed. and has decided on a course of IV cytoxan once monthly for 6 months starting a week today.

She did not want to use methomextrate as I have lung (bronchi) involvement and said this was not the best medication for this. She did mention rituxan but it is not Pharmacare approved in Canada but she did say my condition was quite dire and she could get it for me. I am scared of Rituxan as I know people can have fatal reactions to this but I also know cytoxan is just as bad. I also have had 3 more balloon dialations as the narrowing in the subglottic only stays open for a couple of weeks. They think that this is such older scar tissue and was never caught early enough. Doc does not know if cytoxan or rituxan will work on old scarring but are hoping for no more new inflammation. I asked my doc (thoracic) about doing a resection on the subglottic but he said that would be ENT and he thinks it is too close to the vocal cords. I am going to ask ENT on Wed. when I go. I don't know what else to do as my life is so limited with the narrowing. I can hardly hold a conversation without gasping and I cannot keep getting dilated every couple of weeks. Maybe the meds will work. I don't want a trachea, and nobody has mentioned that so far. Maybe they don't really do that in Canada. Anyway, that is my story for now. Sorry it is so long.

[Sangye]

Wow, Cathy-- it's a miracle that you're still here. I was nervous when I first joined the group too. It seemed like it made it more real that I had Wegs if I talked to others who did.

I know that Jan will pop on here and have lots to say about your subglottal stenosis. She knows a great deal about it.

Meanwhile, I can comment on ritux a bit. My Wegs doc at JHU treats hundreds of Weggies and increasing numbers of them are on rtx. He's never had anyone with a fatal reaction. He only has one person who developed an allergy to it (after many treatments) that can't be prevented with the pre-meds they give during the infusion. He can't have it again. The risk of a fatal allergic reaction is real, but I think it's probably quite small. Knowing the major risks of cytoxan, I would vote for ritux instead. If you had trouble with imuran (far less toxic than ctx) I think you might have trouble with ctx. That's a question for your doc, though.

[elephant]

Hi Cathy and welcome, I have never taken Ritux but I personally would take that over cytoxan. That would be my next step anyways, my Rhuemy does not want me on cytoxan because of my kidney transplant ( lovely wg attacked it). As you know you will receive alot of knowledge from everyone here. We all love to give our two cents worth, because we all have something to give on this forum.

[jola57]

Hi Cathy, we must meet for tea. You story sounds so dire, its amazing that you were in the hospital for so long and they did not diagnose Wegeners. I have had 2 bouts of chemo, once 6 months and once 1 year and am doing fine. Do not be scared. If you need to talk, I will do all the talking, call me.

[Sangye]

Cathy, I'm really curious about the bronchial narrowing. I've never heard of that with Wegs. Usually lung involvement is either alveolar hemorrhage or granulomas/nodules. I would love to know what a Wegs specialist says about it and what can be done.

[cathy2330]

I do not have a weg specialist, i don't think they exist here. I am seeing a rheumatologist who seems fairly knowledgeable and she is sending me to ENT that has seen about a dozen cases of Wegs. He is hopefully going to be able to confirm the diagnosis. My anca is negative (I think I accidently said positive in my story). The biopsy's were inconclusive and because everything else has been ruled out this is what they think. When the first ENT looeds at the nasal and down the nasal passage he said it looks exactly like wegeners. I am hoping that this ENT who is more knowledgable on the disease will also confirm. I am told and have found articles that when there is subglottic stenosis it is very common for a negative anca reading. I have had cough, crusting of the nasal, (did not get a hole in) shortness of breath for many many years. I never went to the docs for it until the first time about 10 years ago. I never go to docs because nobody could figure me out and always tried inhalers and said I had asthma and nothing worked. The narrowing is down low in my right bronchi and is about the size of a pen. They can barely get the bronchoscope down. The left side is up towards the top of the bronchi and is slightly larger. The thoracic surgeon has been doing dialations to stretch. He stated it is too risky to try to stent (and the outcome of stents is somewhat contriversial). The main thing they said they can do is just try to keep it open. If it narrows and shuts completely then they will not be able to open again. If one side does close you can survive off the other lung. The lung will just die(apparently). So I have the problem of keeping the subglottic stenosis as dialated as I can and then also worrying about the bronchials. Needless to say I am always breathless!!

Has anyone else been negative on the anca test but still been told they have wegeners? I am told that it does happen? I honestly as wierd as it sounds just want to know officially that this is what I have. So many tests and so many years and still not exactly knowing is so frustrating.

The other problem without exactly knowing is whether the medication will actually work? But I don't have a choice as this seems to be the best guess?? and as the doc says we cannot afford anymore stenosises. Don't think it will work on the existing but hopefully will prevent any new and prevent the existing from getting smaller.... thanks everyone and thanks jolanta, it is nice you are so close. I am currently off work until I get this straightened out and then back to work (hopefully). I work in Pitt Meadows and live in West Maple Ridge.

[elephant]

Cathy, I suppose it would be out of the question for you to go to Jan's doctor to get a second opinion since you live in Canada. Sounds like you really need the best doctor in the world right now. I am wishing and praying that the stenosis will stop! Wishing you the best!

[Sangye]

Cathy--I sure understand about wanting a firm diagnosis. Then you'd know what you're dealing with and which treatments to use. Having a (-) ANCA is not that uncommon with Wegs regardless of the presentation.

Have you contacted the VF to ask for their help connecting you with a Wegs doc? You can have one in the US consult with your local docs. The fact that your trachea is closing off so quickly each time is alarming-- I don't think that rate is very common. You need expert help asap. A doc who's only seen a dozen Weggies definitely lacks the necessary skills. If we just took 12 people in this group, our cases are so wildly different there's not much to extrapolate from one to another. You really need input from a doc who has hundreds of Weggies.

[Col 23]

Hi Cathy2330
Welcome to the group. Sounds like youve been having a really rough time. Good luck with your wednesday appointment.
Lots of helpful people on this site and stories that you can relate to even though we all have individual wegs symptoms and outcomes.
All the best
Col 23

[JanW]

Hi, Cathy, and welcome to these boards.

I had a balloon dilation of my SS in March and, so far, so good. The relief I have felt and contiue to feel is immense. I will go back to get it checked again in a month, but my doc did indicate that I would likely be able to feel the slightest change -- as he said, "you'll be knocking down my door to get it fixed again, not the other way around."

Now, to your case. It sounds to me (correct me if I am wrong) that they are attempting to merely dilate the scar tissue rather than cutting into that scar tissue to create a larger (but very ugly and irregular) opening. My doc first cut at the points of maximum tension all around the scar tissues (think of something springing back like a cut rubber band) swabbed it with steroids (to lessen swelling) antiobiotics (to fight infection) and myomicin (chemotherapy -- to retard regrowth, but frankly no one knows whether this works). He has mentioned the four month mark as a point where I might 'be back for a touch' up, but also, right after me he was operating on someone who hadn't needed a second one for 2 years. After two or three you're pretty much done for life.

My guy is one of three in the world who does the procedure like this---he learned it at NIH. He has done about 400, and when you consider how many people have it done multiple times, it shows you just how few people make their way to him (or even have SS). It is an unusual manifestation of WG (about 15% of patients) and not uncommon in people whose nose eventually saddle (which mine has). My doc's name is Robert Leibovics and he is on the VF website. His phone number is 212 262 4444 and I've referred people to him (non-WG related) and he's gotten them in within a couple days. When I called for my first appointment I was seen in less than 24 hours and was told that I would need surgery within six months after that visit (I had it in 2).

Mine was a 2.5 to 3 and included the first two trachial rings. Beyond that, he works with a thoracic surgeon and they do their own area. Has sarcoidosis been explored in your case? It also causes SS as well as the kind of narrowing you are describing in the bronchi.

Sorry but the rtx or even ctx may not do much for you SS. For reasons that are unclear SS doesn't track disease activity very well. So you could have a very high ANCA, lots of inflammation in your body, be in poor general health and never develop SS, or your SS might not get worse. On the other hand, I'm only on 15 mg of mtx (so far), no pred, in generally very good health, have little sinus involvement -- yet had total tissue death on the bridge of my nose, leading to collaspe (saddling doesn't track disease progression either). This is one of the reasons that docs don't know whether even swabbing it with chemo helps -- it just tends to come back. Although I don't really understand why yours is coming back so quickly if it is actually being cut into.

I'd had mine for at least five years and there wasn't even any pinkness left on the tissue and the biopsy showed no WG. I don't think that the length of time that you have had it should be an issue because many people are misdiagnosed for years with asthma before having this surgery.

Stent in general are a bad idea for WG patients -- granulomas can grow around them and you could eventually need a trach. There are also issues with how anesthesia is performed because your windpipe is compromised as they are bringing you out of being under they actually put in a breathing tube a couple of minutes before hand to make sure there are no problems.

My SS was right at my true vocal chords and that was not an issue, but I do know others on this board who have had surgeons refuse to do it because it was close to the vocal chords. I'm sure this has to do with the experience level of the surgeon.

Feel free to ask me any other questions -- there is at least one other person on these boards who has had this surgery with my surgeon.

[cathy2330]

Thanks Jan for answering back. Its funny just a few mins. ago my rheumatologist called to talk to me about a afew things. She apparently knows someone at the Mayo Clinic that is a specialist in Wegs. and has a call into them to discuss my case a little. When I have had the dialations they have not cut into it as far as I know. He is just dialating it. Even on the phone the doc noticed that my breathing was heavy and I only had it dialated last Wed. I don't really notice a huge difference at all after the dialations. I don't know how much he is able to stretch the last one before this he said he got to about 6 or 7mm. But because of the bronchial narrowings also i guess it never is really easy to get everything opened a decent amount. Also my nasal passages are really bad. ALways seem to have like a hard layering it feels all through the nasal area. Sometimes after a hot shower it feels a little better but gets progressively worse throughout the day. As far as my throat I can tell when its bad also by i cough alot more, feel the need to continously clear my throat and it feels like a furrball is stuck in my throat. I am not sure about the sarcoidosis, I will ask my doc next time. I know they tested for so many things. Doc does not know if the meds will work but right now not sure what else to do as the main goal is to stop any new inflammation and try and control the existing. I am going to ask her about the cutting into the scar tissue. I had peviously mentioned this to the thoracic surgeon, that was my first question before I started learning more was why can you not just scrape away all the scar tissue. He said that it would just grow back thicker and worse than before. Mine is very hard and calcified, apparetnly it was hard to even get a sample for a biopsy. They think it has been there for a long time. I don't know if there is any docs that do that procedure here in Canada.

Anyway thanks for your comments and expertise. Hope you have a good day today.

Cathy

[JanW]

Your doc is right -- the last thing that they would want to do would be to attempt to remove the scar tissue because that will create more scarring. They don't do that with WG patients at all. If you are just being dilated then I wouldn't really expect that to work for any length of time. I am surprised that there is a benefit to do things given the risk of anesthesia on someone whose windpipe with compromised during during surgery. It would seem that this risk/reward doesn't really make sense. Also, he's only getting you open to the point where you'd have to start thinking about the next dilation anyway (in other words, still not an optimal breathing size) vs. fully open (I'm at an 8 or 9 and would get repaired maybe at a 7 or 6).

Mine was completely calcified - it had been there for five years.

I have to believe that the folks at Mayo know Leibovics - he worked with a bunch of rheumys now at the Cleveland Clinic (also a major WG center) when he was at the NIH. By the way, the surgery and dilation would be the same if you turned out to have sarc.

[cathy2330]

The doc who is doing the dialations has stated that he does not like to keep putting me under general anasthecic as it is tricky for me especially when waking up, they have had to bag me??, and put a tube in once, the anesthgiolist are all aware of the compromiused air ways. He does not want to keep doing but until we can figure something esle out that works he does not want it to close off entirely and like I said nobody has mentioned a trachea to me (not really wanting them to either). The only time was when I go in for the surgery if they have to do an Emergency trach but otherwise not. They just don't know what to do unfortuneltly and this seems to be the only option right now. They are hopeing the meds will work (but who knows I don't think as you have said it will help the old stuff) Mine is the same as yours very hard and very calcified. I will ask the doc who she knows at the Mayo clinic. I am seeing the ENT and also an opthomologist tomorrow. I am hoping this ENT will have some suggestions to help deal with the subglottic area. Because of the subglottic area and the bronchi area involve two different types of docs. Thoracic surgeon has been doing all the dialations now but really as the area up top is throat area is the ENT speciality. I really want it cut out (resectioned) and put back together. I think in my head this is the only option. I have been doing alot of investigating and this seems to be the best long term soloution. Obviously the dialations are not working, they don' t hold much hope for the meds to help the old stuff and you can't scrape it out so I don't know what else is left.

I am almost to the point of just forgetting everything and taking my chances with whatever happens. I have lived with the shortness of breath for most of my life and maybe it will not close off entirely and who knows what will happen?

[Sangye]

Cathy, I've been at that place-- thinking I'll just take my chances and see. Unfortunately, that doesn't work unless you want to die soon. Thinking maybe it won't close off entirely is living in denial. I don't mean to be harsh, just direct. Your airways are closing at an alarming rate. What are the chances of that process just stopping right now? Nil.

Even if it's not Wegs, it's progressive and you're almost out of airway. If it is Wegs, it's 100% fatal if not treated. If it were me, I'd be on the phone with my doc today asking for him/her to call Mayo and/or Dr Lebovics this week.

My Wegs doc said lung issues are the most dangerous because if you lose your lungs you lose your life. Right now they can't even intubate you. It doesn't get much more serious than that.

[cathy2330]

Oh and i also wanted to add Thank you to everyone for being so nice. As i mentioned I am not really a talker and was very scared to come on here and discusss. I did find this sight a while ago and did do alot of reading and found it very theariputic and then got the courage to post. I really appreciate everyones comments and suggestions and from everything I have read so many of you have it so much worse than me. I am one of those people also that do not like to bother people , I must say my 5 week hospital stay i was the perfect patient, I don't think I pressed my nurses call button once. That actually can be a problem as some of you may know is we do too much ourselves when we shouldn't be. I was making up my own bed in the hospital one morning because I didn't want to bother the nurse and they actually scolded me for doing that. My heart rate goes really high on any type of activity as it has to work extra hard to compensate for the lack of oxygen. I think I get up into the 140ish when making bed. Anyway I wish everyone all the best with their treatments and just wanted to thank you all in one fell swoop.

Cathy

[JanW]

Cathy - I can only give it to you straight, like Dr. L. gave it to me, "your condition right now is incompatible with life."

That opening only goes in one direction and that is to get smaller, and smaller and smaller. And there is no guarantee that the progression will be linear. As it was explained to me, one day you might wake up in the middle of the night and not be able to breathe hardly at all. Or, "if you get into an accident, and have to get a breathing tube, they will have to trach you." He was very real to me about the possibility of collaspe -- probably not imminent, but if it happened, I would have been in an emergency situation.

Dr. L. does your kind of surgeries with a thoracic guy, which is what I said in my previous post. I suspect many docs here in the US (maybe ENTs more than thoracic guys) would have trached you by now to keep you safe. There have been people here trached at four. Lebovics doesn't do it because he can just schedule you for surgery, but most ENTs across the country can't do the surgery.

I absolutely suggest you give him a call as the patient, or have your doc give him a call. Seriously, if you say Wegener's on the phone, they jump through hoops. He has said many times that Weggies are his favorite patients because in his side of it he sees the real deformities (the noses and the messed up throats) and he has real empathy for us. Plus he can fix it right away.

But, I do want to assure you that, if your main breathinng problem is stenosis, particularly SS, it can be taken care of and your quality of life will improve 100 percent within two hours of your surgery. Seriously. It is my surgeon's favorite surgery, hands down because the results are so dramatic with such little downside to the patient. That said, it is a risky surgery; the highest complication is death.

I don't mean that to frighten you (and Lebovics hasn't lost a patient to the procedure) but it shows you the seriousness of the surgery.

[cathy2330]

Hi Jan I do appreciate everything and I know that I need to be more agressive, sometimes i am just too tired to call and call. I don't like to be a bother ( I KNOW THAT THIS IS WRONG) it is my health but my personality is like that. If I do get angry enough I will get agressive and I have but sometimes it tough. I feel sometimes that I am over reacting to things (which I know logically i am not) but again one can't always help there feelings.

I wanted to clarify the "surgery" you are talking about. Is it where they did the cuts and put the steriod and chemo into it. How does that keep it from springing back? Have you thought about having the resectioning or is this procedure working or you? I also have to keep the bronchial areas open. They are very small also, my doc says think about breathing through a straw, that is the size. No doc here has mentioned the cutting and putting the steriod topically on. I don't know if any do but I am going to the ENT tomorrow and I am going to ask him about whether he could do this? or has heard of it? When did you have your procedure done again? Has it stayed open for you and your breathingis good now? That would be amazing. Do you know the name of the procedure so I can google to get more info. Or should I just google cutting scar tissue in the subglottical region or something do you think?

Thanks..Cathy

[JanW]

Cathy - just picture this -- the doc makes the incisions all around the windpipe (so it looks like a crown, if you know what I mean), so he widens the opening. Those flaps of skin have to grow back together by forming new scar tissue -- so that takes a while. That's why you have to have it done again -- but not in a month or something. More like six months, or maybe two years. For some people, they can enter remission and never need surgery again (but this is very rare). The chemo is suppose to slow down the growth of this tissue when applied directly to the site, but no one knows that this is always the case. Some docs use a LOT more chemo than he does, with the thought that if a little is good, more is better but he says that hasn't been established at all.

I don't know that any rheumy here would suggest ctx or necessarily even rtx if your om;y symptom was SS because it's a really poor tracker of disease (again, they aren't sure why). It doesn't say anything whether you will get sicker with your WG, and you may still get a lot of scarring and have very little systemic disease.

Take a look at this: Wiley InterScience :: Session Cookies (http://www3.interscience.wiley.com/journal/121605862/abstract?CRETRY=1&SRETRY=0)

[Sangye]

Cathy, did you see my post right before yours? We must have been posting at the same time. I think you can't possibly decide what's best for you (eg tracheal resection) without the input from experts. Your case is much more complicated because of the lung involvement. Can't ignore that.

[JanW]

I just realized I didn't answer all of your questions, so I made another post. I had my surgery on March 4, and my follow up visit with the doc two weeks after. He said my windpipe was at an 8 or 9 (normal). I posted on here immediately after the surgery all about it, so you can read for yourself. I see him again in May, but I don't feel any change. He said it was a very successful surgery.

To say that my life changed from night to day would be an understatement. I went from not being able to do something as simple as walking and talking -- or singing along with the radio -- to being able to do that with no problem. I was a radio personality in a former life and the not being able to speak effectively without sucking in breath was bothering me both physically and psychologically.

I don't know much about resection, sorry.

I just have to add that Lebovics stressed that "we'll do this procedure on someone who is half dead (his words, not mine), someone who is pregnant, someone who has got WG running rampant through their body -- that's how important it is." As he said to me, every medical student learns their ABCs -- and the A stands for airway. In a medical emergency the first thing that you always do is establish an airway.

I understand this must be completely overwhelming for you -- it was for me and not only was I sitting in the chair of the guy who is world renowned and would do the surgery, but I had had previous scan that essentially said that my case should be explored for WG (not to mention my nose problem), so on a subconscious level I was used to the idea that I had this disease even though I had yet to have my ancas come back. But still, this surgery was absolutely my worst nightmare when i pictured a life with WG. And this surgery is so easy (and my bout with this disease so far has been blessedly easy as well) that I would only tell people that it really isn't worth worrying about. By the time I went into the operating room I wasn't scared anymore -- I truly knew I couldn't keep living like that.

[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.

[Sangye]

Just saw Jan's reply. I agree with her that your rheumy should call Dr Lebovics. I agree with everything else with a "but" for the ctx. True, it won't affect SS, but you have major lung problems and it is likely to need a big gun to stop that.

As far as spontaneous remission, it doesn't really happen with Wegs. Symptoms often ebb and flow, though and that can give the appearance of spontaneous remission. Lung involvement tends to progress slowly, but without treatment Wegs is 100% fatal. The pulmy who diagnosed me made a point of repeating that last phrase many times a day to get me to take it seriously.

[elephant]

Kathy, I agree with Sangye and Jan. I was thinking the same thing...Need to call Jan's surgeon. It is time to get a Wegeners specialist.
Dr Carol Langford is a WG specialist at the Cleveland Clinic in Ohio. I see her and she is fabulous!
I think you already know this too, need to get aggressive and don't worry about what people think. This is your life and need to fight.
I love pleasing people too, but I realized I have to fight for my life and get great health care.
I want you to have the best care possible.

[JanW]

I had forgotten about your lung problems - yes you would need a big gun to take care of that.

The biggest issue remains what can be done about your windpipe. That the urgent/emergent situation at this point. You don't want to end up with a trach if you can avoid it. It can be hard to get off of them.

[cathy2330]

Hi again all you lovely people, thank you so much for the quick responses! Sanyge what is the CSR blood test, I have a creatinine of 72??? is that what you mean. Again that is within normal range, my ESR was 6 which I have no idea what that means but was told anyting under 21 is good and I am assuming lower is better?? The only thing that seemed a little high as to what I was told was normal was my RCB (red blood count??) which was 4.99 and apprt. 3.5 - 5.00 is normal so on the high end of normal??? I am going to take all your suggestions and have the nasal surgery tomorrow (one more general anesthtic) recoup from that and then see this ENT on thurs. and he will have the biopsy results then. I am also going to ask him his opinion on treatment as he seems to know a bit more about Wegs (if it is that) than my rheumatologist. I will also maybe see if I can call one of your docs next week. Unfortuneatly here it does not look like we have any real "specialists" in this area which makes it rather difficult. I am hoping to find someone maybe this ent that will help me with the subglottic stenosis (something more permanent) and also the bronchi's.

You would be proud though as I did get a little angry on the phone with the rheumy, but then I got upset and started crying (oh the emotions) and then I couldn't really talk. I think I am getting close to me limit (which for me is a good thing because I then get angry) and will be more persistant. I know that you need to be and really my husband is extremely laid back and not outspoken so he doesnt really help but I do have a girlfriend that I am going to bring with me next time to the rheumy, and boy oh boy watch out if you upset one of her friends. She has no problem speaking up for you and that is what I need. Thanks everybody for all the support and help, like I said I think I am more confident in your suggestions at this time. I do think in my heart that I really do need some medication of some sort to help this not to keep happening , the inflammation, but I just don't know what it is????

So thats all for now everyone thank you again so much for your thoughtful info, I am heading out for dinner tonight, no eating or drinking after midnight, its my daughters 22nd birthday tomorrow!! Thats my baby too!!! Hope everyone has a terrific evening.

Cathy

[Sangye]

Cathy, the CRP (C-Reactive Protein) test measures inflammation like the ESR/Sed Rate. The CRP is better at detecting acute inflammation and also drops quicker than the ESR when the inflammation is decreasing. Docs have to measure both to keep an eye on Wegs.

Though I've never been shy to express my opinion, before Wegs (and lots of therapy!) I was very confrontational about it. I only knew how to defend myself and get angry. I've learned how to be direct and clear without being angry. Takes care of most things and I have good communication with my docs.