Hello and very nervous

[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.

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[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.