Originally Posted in Newbies...

[BrilliantLight]

But seeing that is should have been the place, reposting here::sad:
Hello out there! I'm new...and here to divulge and vent. Hopefully the rage will pass soon...

Where to start? Sorry if this is very long...

I'm 35, a single-mom of a very active 10-year-old. And I used to sing. I had trained classically for close to 20 years...life was great...up till recently.

I've lived my life mostly healthy. I've generally been fit. Was thin up till pregancy and then gained fifty pounds or so. Never did drugs. Never smoked. Rarely took pills for headaches or any sort of pain. I always fought illness. Rarely took antibiotics. Tried to let my body do all the work it needed to. Did a summer juice cleanse two years ago - lost the excess weight. And then the problems started that winter.

It was gradual...I thought I was imagining it, in hindsight, but there was definitely gradual stenosis of my lower trachea. Or maybe it was a case of denial. I refused to believe something major could be wrong. It was barely noticeable up till December 2013, when I was hit with a horrible bout of bronchitis. My trachea never recovered and the inflammation never went away all the way. Again I thought I was imagining it all...because it didn't really affect much. I was able to adjust - but in the summer following, it regressed further (the inflammation progresses, rather).

Over the course of the following two to three months (September, October, November), it got so bad that I could barely breathe, and dropping my daughter off at the end of our driveway made me gasp for air. This is when I realized something was horribly wrong...It was furstrating, and an emotional roller-coaster ride...to no longer be in control over your own body...I would intermittently research and what I saw scared the crap out me. I still refused to be believe THIS was happening to me.

One morning, I ended up going to EMERG, and was tested for Asthma, but didn't experience any sort of relief in response to Ventolin. It wasn't Asthmatic. The doctor at the EMERG proceeded to refer me to an ENT for the following day, and handed me a 50g pill of prednisone to get me started (to take the edge off?). The next day, ENT looked down, and found webbing with visible stenosis just beyond my chords...I was officially prescribed prednisone, 10mg for 18 days to tide me over. He requested to have me tested for P-ANCA antibodies. I was then referred to the hospital at downtown Toronto, to a Head & Neck surgeon. It was about a month later, I had a CT done, and the follow-up appointment with the Surgeon and I saw the CT Scan results with my own eyes that the airway was restricted by half. I could see what I was feeling...well, no wonder. The potential diagnosis went from Idiopathic Sublottic Stenosis to potential Wegener's within a span of a few minutes. P-ANCA results came back negative...but he wanted to redo for both C and P. By the end of this appointment, they determined that I needed urgent help to breathe so I was scheduled the weekend following (Sunday night admittance) to have the dilatation done. This was a week short of Christmas. It was somewhat a traumatic experience...but felt normal right after waking up. Oh, I was high as a kite, but great. I had never taken drugs (to see colourful things) so you could imagine how low my threshold was for T3s.

I was in hospital for 3 days, awaiting the procedure...and was out the day after it was done. Before leaving the hospital, had blood take to test for everything but mostly P-ANCA as well as C-ANCA. Was prescribed prednisone, starting at 50mg, tapering (till the end of December. I was able to consume some sparkling wine for New Year's, two days after finishing!).

On my mid-January follow-up, was told that the second test for ANCA was negative, and the biopsy showed no evidence of WG/GPA, but then was told that it didn't rule it out. My happy mood was barely affected, until they stuck the camera down again, and said the inflammation was back at 10%...it was coming back!! I wasn't feeling it yet...but depression hit. I started researching again...it's sort of become an obsession. It has totally hijacked my psyche. And I'm always on the verge of tears. I have now been referred to another specialist: A Rheumatalogist at another hospital. My appointment is the 11th of March...not quite sure what to expect...I'd appreciate stories of this experience. :smile:

By the end of January, full-blown allergy symptoms were back and my throat became tighter. I'm now back to breathlessness from a simple stroll from my car to my office (especially from cool air to warm - that is the WORST), and had an emergency appointment to determine how bad it is. They prescribed prednisone to tide me over till my rheumy appointment.

Latest Update: I've been prescribed 60g of prednisone, tapering over a 24 day period. I'm on the second day of the regime and feeling very loopy. The meds run out the day I leave for an out of country vacation to somewhere warm and sandy (I'm in Canada...no further comment on this)...and that will be after my 11th of March appointment. Hopefully some light will be shed of a resolution. I want to get on with my life...

Can anyone here relate to any of this, in this order?

If you've come this far, thanks for reading my novel. ^

[chrisTIn@]

Welcome to the forum. That's quite a story!


Two weeks in this circumstances is a long period of time to wait for the appointment with the reumatologist.
I can understand that you want to know what 's going on, and why you have trouble breathing.
Must be very hard when you are a trained classical singer.
You said your ANCA was negative, I hope other forum members will have some advice for you.

[drz]

They will most likely evaluate you more trying to pin down the diagnosis. If they conclude it to be Wegs (GPA) most likely they will start some drugs to suppress the immune system to try stop the Weg activity. Usually CTX and RTX are used for more serious case. Milder cases might get MTX or AZA to try knock the Wegs activity. If the stenosis persists more surgical intervention is likely needed and some people have this done several times on an on going basis to try reduce the blockage. The other concern would be if the Wegs attacks some other organ and then how to get aggressive treatment before it does more damage. A doctor with experience in treating Wegs is very important if it turns out to be GPA.

[annekat]

Welcome to the forum! I'm sorry you had a reason to find us. Everyone's story is a little different. And it was OK for you to post in the newbies section or here... your choice. Prednisone makes some people emotionally hair-trigger, which could partly explain your being on the verge of tears. Any depression you are feeling is understandable. The stenosis is the scariest part of your story for me, as I don't have it, but could get it; some of us have it and many don't, and there are those who have it without having WG. There is a Facebook group for Idiopathic Tracheal Stenosis, if you don't already know that. In any case, of course the most important thing here is getting you a diagnosis, whether it is WG, another type of vasculitis, or something else. Best of luck in getting this accomplished so you can begin proper treatment. Please keep us updated.

[mrtmeo]

Hi Brilliantlight,
Really sorry that you have this and you don't have a diagnosis, yet.
I don't know if this will help you, but it discusses many reasons for stenosis.
If you can handle the complicated literature, here it is
http://www.pjcm.net/pdf_v18_n1_a6.pdf

[BrilliantLight]

Thank you mrtmeo. This really did help. :) I've skimmed it but will definitely read it more thoroughly soon. Another thank you: I was also referred to an awesome YouTube video link by Swb21188 that you had posted in July of last year: http://www.wegeners-granulomatosis.com/forum/tracheal-stenosis/4218-video-treatment-methods-subglottic-stenosis.html - this was amazingly eye-opening. It sort of helped with dealing...I watched it twice just to get all of the details. It gives one hope for more than a hopeless future. :)

[BrilliantLight]

Initial diagnosis was heading toward Idiopathic Stenosis, but then for some reason they changed their minds. My fear is that they want it to Wegener's for the sake of solving the issue. It's harder to deal with if it's idiopathic because then they don't know what caused it. I don't want a diagnosis pinned to my person without enough evidence...although I don't know if there is such thing as "enough". I've come to accept that if I have Wegener's then I have it. I just don't want to get misdiagnosed. I have no symptoms except allergy-like...besides the stenosis, that is. No nosebleeds. No pus in mucous. No blood. I'm so stumped. I think they are too. Each time I've seen them they say, "Yes, we still believe it's Wegener's." Even though I don't actually ask them, "Are you sure I have that?" It's like they're more trying to convince themselves.

Ah well. In any case, your message is really appreciated. :)

[BrilliantLight]

Thanks for the message chrisTIn@. I appreciate the support :)

Truth be told, a part of me has kissed singing goodbye...or at least I'm beginning to. Not sure how I'll be able to recover well enough to actually function in singing properly.

[BrilliantLight]

Thanks for the feedback drz. Actually, I don't know what to make of this. Everything I've ever read about Wegener's speaks of symptoms I don't and have never had. I do have allergy like symptoms...and that's it with clear mucous. Now that I'm on the prednisone, I'm not feeling that...hopefully they'll pin the correct diagnosis...It's been a bit more than 6 months with these symptoms (minus the time after the procedure)...I really, really hate living in the grey area.

[drz]

Thanks for the feedback drz. Actually, I don't know what to make of this. Everything I've ever read about Wegener's speaks of symptoms I don't and have never had. I do have allergy like symptoms...and that's it with clear mucous. Now that I'm on the prednisone, I'm not feeling that...hopefully they'll pin the correct diagnosis...It's been a bit more than 6 months with these symptoms (minus the time after the procedure)...I really, really hate living in the grey area.

Some of the early symptoms like nasal crusting and joint pain can last months (mine lasted 2-3 years) before Wegs progresses to some thing more serious enough to eventually get diagnosed. I had ear infections, sinus infections. and was treated for allergies for many years (decades) before I developed Wegs or knew I had Wegs. These symptoms often precede Wegs and might some how contribute to its development years or decades later but how is still very unknown.

[im so blessed]

I have a tracheal stenosis. It lays very close to my vocal chords and i lose my voice when it flares. The fact that urs was better after surgery and returned, sounds much like mine. My wegeners is usually at one spot at a time. It started as sinus stuff n went to joint pain. My last bout was the stenosis. I too, loved to sing. Was always a dream altho i never had training beyond grade school. If u have any questions dont hesitate!

[drz]

Can't get to post 11. Is this thread having problems? Turned out there was no 11 even though it was indicated at the top and this is now it.

[mrtmeo]

Initial diagnosis was heading toward Idiopathic Stenosis, but then for some reason they changed their minds. My fear is that they want it to Wegener's for the sake of solving the issue. It's harder to deal with if it's idiopathic because then they don't know what caused it. I don't want a diagnosis pinned to my person without enough evidence...although I don't know if there is such thing as "enough". I've come to accept that if I have Wegener's then I have it. I just don't want to get misdiagnosed. I have no symptoms except allergy-like...besides the stenosis, that is. No nosebleeds. No pus in mucous. No blood. I'm so stumped. I think they are too. Each time I've seen them they say, "Yes, we still believe it's Wegener's." Even though I don't actually ask them, "Are you sure I have that?" It's like they're more trying to convince themselves.

Ah well. In any case, your message is really appreciated. :)

I sure pray it isn't weg's, but that what ever it is will be treatable.
At least with weg's, they have proven treatments.
With weg's and MPA, the kidneys and lungs are usually involved and they do a biopsy to determine it.
Lung biopsies are not always conclusive, but kidney biopsies usually are a better choice.
With weg's, there can be granulomas, but not with MPA.

I am assuming if you had active inflammation in the trachia, they would have used a topical steroid in the trachia?
Did they test your immunoglobulin levels?
If you haven't seen this video, it explains treatments for subglottic stenosis.
http://www.wegeners-granulomatosis.com/forum/tracheal-stenosis/4218-video-treatment-methods-subglottic-stenosis.html

[annekat]

Some of the early symptoms like nasal crusting and joint pain can last months (mine lasted 2-3 years) before Wegs progresses to some thing more serious enough to eventually get diagnosed. I had ear infections, sinus infections. and was treated for allergies for many years (decades) before I developed Wegs or knew I had Wegs. These symptoms often precede Wegs and might some how contribute to its development years or decades later but how is still very unknown. This is what happened to me, though not for as long as drz... 2.5 years of ear and sinus and allergy-like issues. I was even tested and found to be allergic to a lot of things and took allergy shots for awhile. Things came to a head when Wegs went into my lungs and it was serious enough to get me diagnosed and treated. Not that I still might not be allergic to all those things, but Wegs was hiding in the background, and once I was treated, the meds suppressed the allergic reactions to some extent, too. I still feel like I have some seasonal allergies, though, and if I ever go off Wegs meds, may have to deal with that a little more.

[BrilliantLight]

Hello,

First of all, thank you all to those that replied. I appreciate the support and the amazing resource function of this forum.

I'm just writing to provide an update on my very first Rheumy appointment that took place this week on Wednesday. The visit was completely stress-free and I was made to feel very comfortable. I completed a written survey of how I'm feeling, and then answered a slew of questions about symptoms.

So it turns out, that they can’t conclusively diagnose me for Wegener’s because my symptoms are so limited. And from before, all blood tests for white cell, red cell, etc. came back perfectly in the middle of the ranges. ANCA is still negative.

Now I’ve been referred for some more blood/urine tests, as well as a wider CT for lungs and sinuses. They said that likely the results will be coming back normal, under the circumstances, but they want to rule out other areas of potential involvement at this point… I was offered a choice of being medicated or not. My stenosis has improved from the prednisone, and they said that likely I'll be needed another dilatation but this time get steroid injections into the inflammation...and see what comes from it...

As for actually getting tested now, they recommended I wait till I’m off the prednisone for the most accurate results. Today was my last day on the Prednisone (10mg), and I’ll be leaving for my island vacation a week from today…I’ve been provided a newer prescription by the Rheumy for another session of Prednisone just in case to take with me…but hopefully when I come back I’ll be able to schedule all of the tests for the next (unscheduled) Rheumy appointment in 6-8 weeks. I'm sort of dreading what will happen in a few weeks when the full effects of the prednisone will have worn off.

And so this nightmare of an adventure continues...

[annekat]

Thanks for the update, Brilliant Light. I'm glad things don't look too bad for you right now, although dealing with stenosis would be a scary thing I haven't experienced. Enjoy your vacation and let us know how things go in the near future.

[MikeG-2012]

I ditto Anne's thoughts, and sincerely wish you a WONDERFUL TRIP.

[BrilliantLight]

Thanks for the update, Brilliant Light. I'm glad things don't look too bad for you right now, although dealing with stenosis would be a scary thing I haven't experienced. Enjoy your vacation and let us know how things go in the near future.

You're absolutely right. Things aren't that bad. :) Thank you for the words of encouragement.

[BrilliantLight]

I ditto Anne's thoughts, and sincerely wish you a WONDERFUL TRIP.

Thank you, MikeG-2012. You guys and this site are very much appreciated.

[BrilliantLight]

I have a tracheal stenosis. It lays very close to my vocal chords and i lose my voice when it flares. The fact that urs was better after surgery and returned, sounds much like mine. My wegeners is usually at one spot at a time. It started as sinus stuff n went to joint pain. My last bout was the stenosis. I too, loved to sing. Was always a dream altho i never had training beyond grade school. If u have any questions dont hesitate!

Wow, same situation here, with the voice being affected (the inflammation so close to the voice box.) That's actually the reason they can't do re-sectioning - because it's too close to the cords. Now that I think about it, I wouldn't do it anyway, because 1) if they don't know what caused it, how the hell can they guarantee it won't come back AFTER and then what? 2) I worked almost two decades on this voice. To lose it would be devastating 3) the complications that arise from that procedure are so life-altering - don't think I can ever be mentally prepared well enough for that...

I've been singing since I was 17. That's half my life...of singing. What I noticed was that my high notes are gone now. I can't seem to breathe fast enough or push hard enough to get higher notes. Used to high E, F, and G. Sing classical stuff like Mozart... now I'm singing pop, and gasping. It was always a dream to sing and I can't live without it. So I'm still hoping there will be times after dilatation I can do this like I used to a few years ago. Thanks for the message - I appreciate it.

[annekat]

Wow, same situation here, with the voice being affected (the inflammation so close to the voice box.) That's actually the reason they can't do re-sectioning - because it's too close to the cords. Now that I think about it, I wouldn't do it anyway, because 1) if they don't know what caused it, how the hell can they guarantee it won't come back AFTER and then what? 2) I worked almost two decades on this voice. To lose it would be devastating 3) the complications that arise from that procedure are so life-altering - don't think I can ever be mentally prepared well enough for that...

I've been singing since I was 17. That's half my life...of singing. What I noticed was that my high notes are gone now. I can't seem to breathe fast enough or push hard enough to get higher notes. Used to high E, F, and G. Sing classical stuff like Mozart... now I'm singing pop, and gasping. It was always a dream to sing and I can't live without it. So I'm still hoping there will be times after dilatation I can do this like I used to a few years ago. Thanks for the message - I appreciate it. I'd forgotten about your singing. What a shame that this should strike you right in that area. Though I'm not a singer to anywhere near that extent, and I haven't had any TS that I know of, I can report that earlier in my WG, my voice was much worse and I could not sing a note, and now I can. I'm hopeful they can alleviate those problems for you so that you can sing the way you want to again.

[renidrag]

I also lost my singing voice but lately has been slowly returning. Breathing remains an issue.
Dale

[BrilliantLight]

Hello all,

Short update to follow. Vacation seems to have helped immensely - went into the ocean every day and it seems to have helped with allergy like symptoms. They went away completely.

Back from vacation - it's been a dreadful time back to colder weather from plus 30 degrees. The frigid air on the plane didn't help things. The entire family came down with something that feels like bronchitis. Symptoms have eased up a lot since last Tuesday...state of Stenosis seems to have stagnated - it's been the same since I left. Not sure whether I'm imagining it but the prednisone didn't do much. I didn't have to go back on them while on vacation, thank goodness.

Still coughing from the bronchitis...bit of a head cold going on now. Breathing is difficult, but mostly just loud. Waiting for a CT appointment for sinuses and lungs. Already have my follow-up booked with the rheumy for the results (for tests I haven't taken yet) on the 28th. That's all I've got for now. Oh yeah, it snowed again here today. *Sigh*

Hope everyone is doing well...hugs to all.

[BrilliantLight]

Hello all,

Hope everyone is doing well as can be.

Well, I'm just writing to provide an update since last post...should anyone be interested. :P

I had my follow-up with the rheumy. Was able to get extensive blood work and new CT scan for lungs and sinuses without the effects of Prednisone. Was told that prednisone in my system might skew results as it suppresses inflammation symptoms. So now, prednisone has been out of my system since mid-March and breathing hasn't worsened (it's actually gotten better since). Here's what I found out:

1. Blood results came back normal across the board. For all ranges, I'm smack dab in the middle. With the exception of ESR which is still within normal parameters. Kidneys are working as they should.

2. CT Scan revealed nothing in lungs or sinuses. No sign of GPA. All organ function is normal (kidneys are good, liver is good, lungs are clear, sinuses have mucous but are clear...no evidence of GPA anywhere)

3. My ESR was 25 in February. Most recent blood work revealed ESR to be at 9. There was some sort of infection/inflammation (which I believe was eliminated by the antibiotics i had between then and now).

4. My breathing has improved, but allergies are horrendous (I'm going to blame spring for now, and see what happens once stuff stops blooming. It's so depressing, considering I used to LOVE this season. Loved lilacs and buds...)

5. When I got back from Cuba, I got sick. Like bronchitis sick. The airplane was like a freezer and the vents were blowing something in my face all the way home. Was afraid the stenosis would worsen so I got antibiotics. The stenosis actually improved; I mean, the narrowing is not that narrow now. And day to day, it seems to be improving, if possible. (?? Is it?)

The next step is getting another dilatation but this time with cortisone. I may or may not have scar tissue down there...so we'll see. This will be the 2nd dilatation. I have yet to schedule an appointment with my ENT.

With this being said, I have not been diagnosed with GPA. Due to lack of symptoms and evidence, it could not be confirmed. The rheumy says that aside from the stenosis, I'm healthy as ever. I don't know what to make of this. On one hand, I'm elated. But on the other, I know it doesn't mean I DON'T have it.

I guess I'll wait for a few more days to see if the narrowing becomes less of a narrowing...I may need to have scar tissue cut...but I'm still hoping that the stenosis is just going away VERY SLOWLY. This is what if feels like. Or I'm just trying to convince my sense of the impossible. Not sure whether it's possible.

I dearly hope everyone is well. And I think I am blessed to have the health I do have...I wake up every morning, feeling lucky...and fortunate to have this blog as a support. I am grateful for this site.

BrilliantLight

[annekat]

Thanks for the update, and I'm so glad to hear you are doing so well and have no signs of WG in any of your tests. Do keep us posted on how the dilation goes. I hope that everything positive you are imagining, the narrowing becoming less pronounced, and all that, is absolutely true!
So sorry about the allergy stuff, though. Maybe you can get that dealt with as a separate issue, like with shots, perhaps, once you are more sure what is going on. I got allergy shots for a year or more before my WG dx, and I thought they were helping. I discontinued them once i knew I had WG... not sure I had to, but the doc thought I should, and it just made things less complicated.

[vdub]

Cuba! You Canadians get to do some cool stuff.... Maybe I'll be able to go to Cuba soon now that talks are underway. I see where a ferry line just opened for business between Florida and Cuba. I would love to go there and see the sunny beaches.

I'm glad they are now thinking you don't have gpa. That is a real blessing. Good for you!

[BrilliantLight]

Nothing new to report...I have a follow-up scheduled with my ENT after rheumy couldn't confirm Wegener's. I want to schedule my 2nd dilation as this time my ESR is down. I'm optimistic that this might mean no recurrence of the stenosis. The first and only time it came back, I had an ESR of 25.

I tried to get a copy of the biopsy results but my ENT's office says they don't have it.

Is it possible that all they checked was for evidence of vasculitis and cancer? I find that so difficult to believe. Why wouldn't a doctor do a test for a wide range of possibilities? It certainly hints at arrogance that's detrimental to patients. Based on a single symptom, they've ruled out everything else...?

On top of which, I find it ludicrous that they test for vasculitis in an area that's never yielded positive test results in any known case...but they keep checking anyway from case to case. OH, and get this: I'm also told that if someone tests positive for P-ANCA and/or C-ANCA, that STILL doesn't mean/prove they have Wegener's. WHAT??? That's what a Fellow at the rheumy's office told me.

Something interesting: I've forwarded my files overseas for a second opinion. All they require is the biopsy details...it is frustrating that this is so difficult to obtain. Over there, based on all the notes in my files, they have tentatively assessed that it doesn't look like Wegener's. VERY strange. I feel cheated. And I officially don't trust any doctors here.

Cue self-pity: It would be so nice to be able to run or jog (play!) with my 10 year old daughter. Or walk up a f-ing single flight of stairs without seeing stars due to shortness of breath. I feel like I'm 80 if I even try to quicken my pace. So very angry at my body. Still haven't stopped asking, "why?"

[kaysee]

Glad to hear they don't think you have WG, but I sure hope they can figure out what is going on. It sounds like there is definately a problem going on. I wish you the best. Keep after the doctors to figure it out. Have they checked your heart? When I was so very short of breath it turned out I had something called tachycardia. My tachycardia was probably caused by the WG. Once I was given meds to make the heart beat regular I no longer had shortness of breath.

[BrilliantLight]

Thanks Karen,

It turns out that I've been diagnosed with ITS (Idiopathic Tracheal Stenosis), not Wegener's.

Going in for my second dilatation within the next two weeks - inflammation seems to have gone down. But ENT has indicated that I have a "shelf-like" band scarring below the chords, and limited options leave me with nothing but the re-sectioning surgery....if ever I want to breathe well again. Singing the way I used to sing is probably off the table, but at least I'll be able to function after.

Thank you everyone for your support.

[annekat]

I'm sorry to hear about this diagnosis and the severity of your breathing issues, requiring surgery to correct. Breathing issues are one of the worst and scariest things, and I know because of having asthma off and on throughout my life and having it basically ignored when I was younger. However, this thing you have is much worse. There is a Facebook group for Idiopathic Tracheal Stenosis, if you are on Facebook... I joined at one point for some silly reason, I guess because I wanted to see what TS is all about, having heard that it could happen with WG (and possibly not knowing at the time what 'idiopathic' meant). I'm not sure if I'm still a member or if they are very active, but you might check it out. The WG and vasculitis groups on Facebook are pretty good. Anyway, good luck, and I hope you are able to get that surgery and it isn't too bad to go through. Let us know.