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.