What is a trach?

[katarzena]

edit: actually tracheostomy? and why do some wg patients need it?

[Jack]

Some Wegener's patients suffer from Tracheal Stenosis, this means a narrowing of the wind pipe and causes difficulty in breathing. A tracheostomy is an operation which makes an opening in the wind pipe through the neck in an area below the narrowing. This allows you to at least breath! An operation can then be performed to dilate the wind pipe, but this is often not 100% successful and may have to be repeated. There is a whole section on the subject elsewhere on the Forum pages.

[katarzena]

Oh I see. Thanks for answering. Is it common for wg patients to have tracheostomy? And how do you see if you'll be needing one?

[Jack]

It is one of the few things that I have not had so you'll have to rely on someone else's expertise. ;)
I'm sure someone will come along with some first hand experience.

[JanW]

It's relatively uncommon -- less than 20 percent of WG patients have tracheal or subglottic involvement. Of those that do, probably most will end up with continued scarring necessitating surgery (like I had). Jack is right -- it's not so much that the surgery is unsuccessful but rather that they have not discovered anything that stop the scar tissue from returning -- it's only a matter of how quickly it will return. Mine has not in three months, but I had surgery in addition to balloon dilation (some people just have a balloon to spread the tissue apart -- my doc actually cuts into the tissue but very few are trained in how to do that. You only end up with a trach if no one caught it in time or you are from someplace where they simply don't know how to/are unwilling to do this surgery (for a whole host of reasons, mostly because they lack expertise because this is a rare condition). I had a pretty small opening and I still didn't have a trach -- my doc was confident that he could schedule surgery before my windpipe collasped.

You'll know if you have this condition because it will be increasingly difficult to breathe.

[katarzena]

Oh I see. Thanks for answering JanW I hope you're ok now.
I guess if it's tracheal stenosis it is more common for patients with uppe respiratory involvment? I hope not!
I was wondering if it happend to you before you were diagnosed or during the treatment?

[Jack]

Not sure it is as simple as that. I've had a huge amount of sinus and lung trouble, but no stenosis yet.

[JanW]

No, Katazena, bottom line is that it isn't really that common at all. However, if you have it, it is only caused by a few known conditions, WG being the top one.

I've never had lung trouble, and my sinus involvement has been extremely minimal, although it was sinus problems that eventually led to my diagnosis.

I had it for five years and the docs thought it was asthma. I never got any better on the steroids, although they did manage to wreck my bones ;-(.

[katarzena]

That is really comforting to hear that it isn't common. The hole in my septum is giving me enough worries on top of everything, so I got really frustrated when I read about the stenosis!
Did you have your nose fixed? I remember you saying that you'd like to do that as soon as possible.
I have only one more question about the stenosis, I see it is important to tell about even a minor difficulty in breathing but is the stenosis when you can't breath at all, like even through your mouth? Or if my nose is closed but I can breath through my mouth: should I mention that? (I have sinus involvment so I had my nose closed for quite a while before diagnosed so I thought that's normal during the treatment, until remission and not necessary to mention).

[Jack]

With stenosis you can't breath through nose or mouth because the restriction is in the region of your throat. You can expect breathing difficulties when breathing through your nose if you have sinus involvement, but should be able to relieve this to a large extent with regular use of a saline rinse.

[katarzena]

I use one spray prescribed by otorinolaringolgyst, it's called aqua marine. It's pure water with salt. I guess it's similar to what you suggested Jack. It does help, but I had problems with nose before I started treatment. I still use it 3 times a day tho. I forgot what it was like to breath with full lungs! I seriously thought I was breathing normally. After the first dose of prednisone I lost the crustings and bleeding and was like: Wow, cant be! :D

[Jack]

I was meaning something a bit more effective than a spray as in these threads - - - , but it is only really helpful if you have crusting and bleeding.

Edit:
For some reason I can't get the links to work so just do an Advanced Search for "irrigation" searching thread titles only.


(http://www.wegeners-granulomatosis.com/forum/114-sinus-irrigation-bottle-do-you-know-about.html)

[JanW]

No, I haven't gotten my nose fixed yet - Lebovics likes for his patients to be medication free totally (even pred) for minimum six months. I think that the rheumy and he are going to talk about that though, as the rheumy thinks that's an unreasonable goal (not just for me, but for patients in general, because let's say I made it 5.5 months, but then had to restart medication, I'd have to start the clock all over again).

I don't have a hole in my septum, but of course that could have the same effect of collasping the bridge of your nose. The doc may be able to say how likely that is to happen though, I don't know.

[Jack]

Lebovics likes for his patients to be medication free totally (even pred) for minimum six months.
That is a ridiculous requirement. He obviously does not have much confidence in his own abilities.

[JanW]

Why would you say that, Jack? Fixing a saddle nose really is an aesthetic issue except in the most extreme cases. My nose is completely functional. He would simply prefer not to 'waste' aesthetic surgery on a nose that going to collaspe in six months, or take cartilage out of your ribs that will be reabsorbed or not properly attach to your face. I've read the horror stories on the internet of people who had this surgery done because they wanted to look normal so badly that they did it when their disease was not fully controlled. I'll take a pass on that!

And he's done a couple of hundred of them med-free so clearly in his practice he sees people in medication free remission on a regular basis.

I don't necessarily think that I will ever be off medication (and I don't think about it much) but frankly if someone doesn't want to give me cosmetic surgery (albeit one that is occuring because of a deformity), I really can't quibble with the guy. It's not like he was putting off my stenosis surgery until my WG was under control.

[Jack]

Well for one thing, saddle nose is a pretty serious issue for many people so I'm not sure that it quite fits into the traditional "cosmetic surgery" bracket and secondly, it rules out almost everyone who has Wegener's. I'm yet to hear of anyone who has reached an entirely drug free condition. There are plenty of sound medical reasons why operations should not be performed do to the increased possibility of complications (no one want to operate on me for example! ;) ), but most of the drugs we take do not put us into that category or fall into the area where adequate precautions can be taken.

[JanW]

Of course it is serious -- I don't like people staring at my face either -- and it impacts the quality of your life. It's a deformity. However, it's not so simple as saying let the person be on meds and we'll fix the nose anyway -- lots of things can continue to happen to that nose, and you've already disturbed it, and the second surgery is going to be more traumatic and on and on. He doesn't have this rule because he can't accomplish a surgery that will make your nose look almost normal. He doesn't do it because he would prefer to not have your nose collaspe again in six months and no amount of skill that he has will prevent that.

And he does see many, many medication free people. Since he's one of the top WG ENTs in the country, I'm just going to have to believe him on this one, which is not to say that my doc will not try to persuade him into doing my surgery sooner. In my favor is the fact that I'm not on pred, so they know my case has remained pretty stable thus far only on an immunosuppressant.

[katarzena]

That sucks! If I ever get the saddle nose I believe that I'd run to get it fixed immediately. But now when I've read this...
What horror stories did you read?

[JanW]

Really it's like any other nose job -- sometimes you get a less than perfect result. I'm sure you've seen those shows (at least with have them here in the US) where someone is getting a botched plastics jobs fixed. Unfortunately, for people WG, the new cartilage can get reabsorbed or you can have scarring and other problems that you wouldn't have without the vascular inflammation that we do have. So while in stenosis docs operate because they have to to save our lives knowing that the damned problem will likely just grow back, in less urgent cases it is understandable to that they are reluctant to do so. I think it would be horrible to have cartilage that was taken from my rib in a painful procedure reabsorb into my nose.

[pberggren1]

Jack, I just wanted to say that I was totally drug free from August 2006 to November 2007 and felt great as well. I still had some minor bleeding and crusting in the sinuses, but no inflamation in that area. I only had to irrigate 1 or 2 times a day, and barely anything came out, at least compared to what it is like now where I have to irrigate 3 to 5 times day and a bunch of stuff comes out as well as me coughing up all that crap once or twice a day. I also have some inflamation in the sinuses right now. I also had way more energy and no aches or pains anywhere.

So maybe I fluked out with that remission. It was my own decision to quit the Imuran in August of 2006 and quit the docs as well. I am surprised I lasted that long compared to what I have been going through the last 32 months.

[Jack]

Sorry, but if you had to irrigate 1 or 2 times a day you were not doing great on the absolute scale, you were just well by comparison to previous lows. In fact, if I was in that condition (and I have been many times!) I would consider that my Wegener's was still active and there is no way I would have stopped medication at that point.

[pberggren1]

Now you are scaring me Jack.

With hindsight I was stupid to quit the meds.

Maybe what I am trying to say is that for the last few months I have this recurring sinus infection. It must be chronic because my right nostril closes up, I improve only slightly while on an antibiotic and then get worse when I get off of it again, and have sinus headaches in the frontal, ethmoid, sphenoid, and maxillary sinus.

I have never had or felt like this before and it all started with lung infection in June of last year. I started the Cellcept again in January last year. We tried the Cellcept at a lower dose of 2g per day back in May and June of 2008 but by the end of June 2008 I was flaring again and went back on ctx again.

It is just frustrating sometimes. Hopefully I can get some clearer answers from Mayo.

[Jack]

I think we all tend to speak from our own experience and as we know, Wegener's does not follow a book of rules and everyone's case is different. My comments are strongly influenced by my own experience with serious sinus trouble. I had lots of treatment for infections that nearly killed me because the true cause was Wegener's and ever since that time the condition of my nose and sinus has always been a good indicator of active disease. However, we are also prone to infections and this can cause the same symptoms, but when antibiotics don't seem to work my alarm bells start to ring.

[pberggren1]

I hear what you mean about the alarm bells going off. But for me this is a totally different type of sinus feeling and trouble I get when I have flared before - 3 times. So hopefully Mayo will be able to help with this.

I was looking at some videos online of the Caldwell-Luc sinus operation. Very grusome. I sure feel for you Jack having to go through that.

I also looked at a video online in google videos called: "New Treatments For Chronic Sinusitis" It seemed fairly interesting. It seems to suggest that if the opening from the Maxillary to the nose is not large enough or is inflamed then they will not be ablt to go in that way to take a look or do a wash out or take a sample of infection.

I think if any ENT suggests something like a Caldwell-Luc procedure for me I will just say no. I will rather look into these new approaches that are less invasive.

[Sangye]

Jack, when you said you hadn't heard of anyone being drug-free were you just referring to those with saddle nose? We've had members in this group who are/ were drug-free. Mike Caven went 27 years without drugs or any symptoms of Wegs, and if I remember correctly when he flared last year the ctx/pred quickly put him into another drug-free remission. I don't think Doug is on any Wegs drug, either. I've known people from the other support group I used to belong to who aren't on drugs and feel great. I mean, there is such a thing as drug-free remission. I bet we don't see it in this group as often as it exists because they're not in need of support. But hey, this could be a good poll!

Jan, with all due respect to Dr Lebovics, there are plenty of Weggies whose disease is stable for decades on low doses of pred and/or other drugs. Surely he must mean he wouldn't operate on Weggies whose disease is not stable. Also, Jack is right about saddle nose not only being cosmetic. Some people with a saddle nose can't breathe through their nose at all.

[Jack]

Yea. That wasn't one of my better posts was it? I must have been having an off day. ;)

[Sangye]

LOL-- You should see all of mine I delete.... :D

[katarzena]

That's exactly what I've been thinking, Sangye. Here on forums aren't many people that are completely drug free but there are many in real life. They just don't need support anymore, they have moved on with their lives. I think that's completely understandable.
And speaking of saddle nose I agree it's not good idea to operate when wg is very unstable but if someone is in remission with low doses of medicines I think it's safe for them to get the surgery, isn't it?

[Jack]

it's not good idea to operate when wg is very unstable but if someone is in remission with low doses of medicines I think it's safe for them to get the surgery, isn't it?
Pretty much the point I was trying to make, but I did not do it as well as you. ;)

[katarzena]

Jack I guess we're in the same time zone. You're the only one that answers right away when I post something. :D I feel like everyone is a sleep while at my place it's a day, so I usually post something and then read answers next morning. It's funny. :)

[JanW]

I still ndon't see why it is so hard to believe that someone who is world-renowned in his particular (very tiny) medical specialty (throat and nose surgery on vasculitis patients, with a specialty in WG patients), says that he will not operate on anyone who is not in medication-free remission and I think it's really off base then to suggest that this surgeon must not have faith in his own capabilities with this surgery. I didn't get it wrong, because I asked specifically whether he had ever operated on anyone who was on medication for WG (after he announced that the number one qualification was six months med free remission) and he said once in his career he operated on someone who was on 2.5 mg of pred because it was clear that she would never be off pred. When you consider that a small portion of WG patients have this condition (less than 20 percent), and a large chunk of those have it with my severity (not very severe, totally functional nose with an intact tip and collasped bridge), it's just not that far-fetched that a guy who has done 150+ over the course of more than 20 years has only operated on people who are off meds. If he does mine while I'm still on, you all will be the first to know, but that would involve my rheumy moving him off of a position he's pretty firm with.

And I agree with the others that people who are in a medication-free remission are much less likely to post of support boards, particularly if they didn't form relationships here. But that doesn't mean that they don't exist in equal numbers to people who are extremely sick with this disease.

[Sangye]

Many people can't get off low doses of pred simply because their adrenals are permanently dependent on it, not because it would cause a Wegs flare. So they have truly inactive disease but the pred is necessary to stay alive. Such a scenario is not that unusual.

It doesn't make me question his skill or capabilities. I just don't understand the science behind such a decision.

[me2]

When I was about twenty I was diagnosed with Wegener's. I had suffered for a long time and was extremely ill. I was treated with Cytoxan and prednisone for about two years. I was then in complete drug free remission for the next twenty years. I then relapsed and have been struggleing ever since.
So, I am mentioning this so Jack can say he knows of one guy who had a long period of drug free remission.
In spite of years of recent battle with the disease I still have hopes of getting remission again. I think it could happen for anyone. This disease comes and goes in mysterious fashion and doctors are getting better and better at dealing with it. So are we patients.
I had the illness before the internet was created. I didn't meet or talk to another person with Wegener's for 17 years. Talk about lonely.
The first person I talked to was Marilyn Samson the founder of what is now the Vasculitus Foundation. It was so amazing to talk to someone who had the same illness. God bless her for her work on behalf of others with this disease.
We are so lucky now that we here all have each other.

[Lightwarrior]

I agree that we are lucky to have each other. I can't imagine getting through this without support, you were very strong.

[pberggren1]

me2 and Litghtwarroir, that is so true.

We owe Andrew so much for getting this site going and keeping it going. I have found so much support and comfort in this site because of people like you and Andrew. I am going to cry now - I am a little emotional at times. Especially lately not knowing what is really going on with me and planing to go the the Mayo Clinic.

Thanks to everyone on this site for encouraging me and keeping my spirits up.

[Jack]

I am going to cry now - I am a little emotional at times.
Things affects me like that sometimes. It is a combination of the Pred and the continuous underlying stress of the situation. Give yourself permission to just go along with it. :)

[Sangye]

I can't imagine going 17 years without knowing another Weggie! That's amazing. I went 2.5 yrs "alone" and it was awful.

It's so inspiring to hear of any long-term remission-- drug-maintained or drug-free. Twenty years, wow.

Jack and Phil, I'm constantly in danger of electrocuting myself by crying at this keyboard.... :)

[JanW]

That's truly the wonder of the internet - - connecting people who would be very rare indeed within their local communities.

Always good to hear of others maintaining long-term drug free remissions. My rheumy has several patients like this also, although he cautions that this should not be the goal.

[elephant]

I will do what my wg doctors says to stay in remission. Yes, I am not perfect...still get sinus crap and rinse nose out once a day...I still have night sweats and insomia....but i am in a medicine induced remission. I should be in bed, but I am visinting my sister in Illinous and using her computer. So I have not been able to come on here. Good night.