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cathy2330
09-18-2010, 10:22 AM
Hi everyone I have not written on this site for a while but I am always browsing and keeping up to date with everyone. I did post back in Aprilish on the new member post. Quick refresher is: About 8 years ago diagnosed with a narrowing in my bronchi. Got checked over years, always had some shortness of breath but managered. In Nov, Dec 2009 noticed a lot more shortness of breath could not even talk without gasping. Had bronch done in Jan. and doc saw the existing narrowing had closed sufficiently more and also had about 3 more areas, trachael stenosis and more in the brochis. Spent 5 weeks in hospital, had every test and about 5 balloon dialations and stent tried to put in unable to as area too narrow. Had anca test for wegs and was negative. Left hospital with imuran and pred. Could not tolerate imuran, extreme vomiting uncontrollable for hours, shaking sweats etc. Rheum. decided on cytoxan for 3months and pred. Did IV cytoxan 900 mg with mesna for three months last one was July 22. I am only on 5mg of pred. now. Have seen ENT in April and have severe nasal crusting, ulcers and he did a nasal biopsy , inconclusive for wegs, and nasal cleaning.

My problem now and question to you knowledgeable people is: Doc was going to go with Cellcept as maintenance as I cannot tolerate imuran, and she did not want methotrexate(she says complications of this med. can be lung and didn't want to compromise an already bad lung situation) so hence cellcept. Unfortunetly it was not on the approved med. for my extended medical and took until this week to finally hear they approved it. Could not afford to pay for unless approved. Problem is: I have noticed since cytoxan stopped my breathing is getting harder and harder, feeling the urge to always clear my throat, hoarse voice, gasping when talking, walking etc. Also have developed extreme stomache pains about 1 hour after eating lasting about 4 - 8 hours, nasal feels very hard and crusted again etc.

Rheuma wants to increase my pred up to about 30 mg again and believes I should do another 3 month protocol of cytoxan. I am seeing a gastro doc for an endoscopy on Wed. Seeing the ENT next thurs and have another bronch and dialation scheduled for Oct 5 to see what is going on with the lungs.

MY PROBLEM IS:::I DON'T OFFICIALLY KNOW WHAT I HAVE! They are going with ANCA negative Wegs, but have also mentioned Chaurg Strauss Syndrome, Scleraderma and relapsing polycondritis. The rheumy seems to say they are all autoimmune diseases , vasculitis, and treated generally the same, cytoxan / pred etc. they just effect different parts.

I DONOT want to do another 3 months of cytoxan, DO NOT want to increase the pred and I am so confused about what to do. It is very hard when I don't know exactly what is wrong , So therefore I am not sure what treatment is needed. If there is no new area of narrowing in the trachea , my thoracic doc said he could keep ballooning the old areas, problem is they seem to narrow back extremely quickly. The rheumy thinks cellcept will notbe strong enough and thinks the stomach problems, I am not really eating now drinking ensure, are vasculitis related also. I do not tolerate medications very well, the pred causing exteme heart racing, shakiness, insomnia. The cytoxan caused extreme naseau, fatigue etc. I don't know whats working or not working and feel like I am heading back to square one like in January.

Has anyone else NOT been officially diagnosed and being treated. I just want to get some opinions and comments. I know everyone will probably say you need the medication but for me my life is not any better while taking the meds in fact it is harder due to the side effects, and I have learned to live with the shortness of breath and if the doc can keep dialated I wonder if that is better.

I find I talk to friends , husband etc. but they cannot understand as they are not the ones suffering from the side effects or the breathing probs etc. Doc said to increase pred. today but I could not do. I have to think about what is best for me and I just don't know! Any suggestions or similar situations would be greatly appreciated.

pberggren1
09-18-2010, 11:50 AM
Hi Cathy:

Your symptoms sure sound like Wegs but with no positive ANCA either way and no positive biopsy it is hard to say.

It is proven that IV cytoxan is not as nearly as effective as oral. So I don't know why your doc would go with IV. It sounds like your doc does not know what they are doing.

If it were me I would ask for a consult with one of the VF docs.

elephant
09-18-2010, 11:51 AM
If you don't increase the prednisone you will continue with silent damage...you are already having shortness of breath. Have the done a CT scan of your lungs? I think you doctor is right about the cellcept not being strong enough...you need something stronger. Are you seeing a Wegeners specialist? I know you don't want to go up on the prednisone but I had to too....long story. Sounds like wegeners, yes wegeners can attack the stomach...i use to get abdominal pain and they could never figure out what the deal was. I am sorry you are going through this.

Sangye
09-18-2010, 12:51 PM
Cathy, it does sound like Wegs. I also think Cellcept wouldn't be strong enough, though it's hard to say. It sounds like you tolerated the ctx fine and they only did 3 months. Like Phil, I'm wondering why they didn't use oral ctx, which has a better success rate. Have your docs have considered rituximab?

The side effects you listed from pred happen to everyone. If the heart racing is bad enough you might have to take a drug for it until you taper the pred down to a lower dose.

I think you might be in a little denial about Wegs. You can try to tough out the symptoms and avoid treatment, but the disease marches on and does permanent damage. You can't get that chance back if you change your mind down the road. Also, the longer you postpone getting proper treatment, the more likely it is you'll wind up on the stronger drugs and higher doses.

cathy2330
09-18-2010, 01:33 PM
Hi Guys thanks for your replies. As far as I know here in British Columbia there is no Wegs specialist so it makes things very difficult. The rheumatologist wanted to do the IV cytoxan because she wants to use the mesna to help protect the bladder. I guess what I really struggle with is I cannot get a FIRM diagnosis. It is only best guess scenerio. They have taken biopsies from my nasal done the anca test numerous times even all my blood work back intitially did not indicate this. I did tolerate the cytoxan fine but my worry is about the toxicity of it and long term effects. We did talk about rituxan but because I have tried the cytoxan and it did seem to help my breathing during treatment she thinks it is best to go with what we know. Do you think if I do another 3 months of cytoxan that will be enough. The big concern really seems that within 2 weeks of the cytoxan finishing my breathing was noticibaly worse. What happens if after another 3 months it gets worse quickly again. I guess my thinking was having the bronchocopy so we can see exactly what is happening. The docs seem to think because some of the narrowings are so many many years old that as soon as treatment stops the narrowing starts shrinking back. If the bronchoscopy doesn't show any new areas of stenosis then that would be good. I don't think I am in denial, I know something is definetly wrong but it is so hard to not know with confidence what it is and whether I am doing a treatment that is unnecessary.

Does anybody know any other diseases that have the same symptoms as mine? I guess since the cytoxan seemed to work for the 3 months I should be doing more but I am scared of the long term effects of this. I guess everybody has the same concern , what if after another 3 months things aren't better? It is very selfish of me but I just want my life back. Since January nothing is what it was and sometimes I wonder if it is worth it. I know there is alot of people out there that have it a lot worse than I do but sometimes I don't feel like life will ever be the same. I have not been working since Jan. and feel that this illness defines my life I carry on and truly if you knew me I care about others so much and always put on a happy face and try not to let people know what is really going on and pretend I am happy but this whole year has practicly passed by me and all I have done is hospital stays, doctors, lying in bed, feeling sick and generally not who I really am. Does it get better???Sorry for the big sob fest but this is probably the best outlet for me and it feels better to actually say this stuff rather than hold it in. I am truly not a whiner or complainer but feel that sometimes it is just too much.

Thanks for listening and letting me vent.

jola57
09-18-2010, 05:46 PM
Hi Cathy, as you can see I'm from Mission, but I work in Maple Ridge. i would be very happy to have tea at Tim Hortons in Maple Ridge some time next week during the day. Call me at 604-818-0078. We can chat about Wegs. I was diagnosed 4 years ago and after two cytoxan and pred cycles I am on methotrexate and tapering from pred. Call me and leave a message if i don't answer. Looking forward to meeting you.

Jack
09-18-2010, 07:58 PM
Hi Cathy,
You could say that everyone on this site who has not had a positive biopsy result is undiagnosed, but it is well known that Wegener's is hard to pin down and that is why diagnosis is made using the BVAS symptom scoring system. This gives a probability to whether you have the disease or not. However, in your case, I think you should be receiving some fairly aggressive treatment anyway because it seems obvious that you have an active auto immune disease of some sort.
We all have our fears about the use of Cytoxan and its potential for bad side effects in the future, but don't forget that Wegener's can kill you now and we have indeed lost several members of the Forum. Your disease needs to be stopped in its tracks now before you suffer serious harm.
Once the disease is brought under control, life can get much better. It took many years to achieve, but I eventually became well enough to hold down a good job and raise a family while keeping the Wegs at bay. The price I paid was heavy duty treatment with steroids and immuno-suppressants which are now showing serious side effects, but on the whole it was worth it for the 20 good years.
As everyone else has said - what you really need is the care of a vasculitis specialist.

elephant
09-18-2010, 08:58 PM
Cathy, I am so glad you came on this forum to vent and let us know what is going on with you. I want to let you know I had this disease for over 30 years. I was diagnosed in 2008....I started to get sick when I was 12 and it got worse at age 14..1980. My first symptoms were arthritis,fever, lower back pain and pneumonia ....then it got so bad...I looked bad and was admitted...well my kidneys were failing. The doctors at " Children's Memorial hospital in Chicago new I had a autoimmune disease but didn't know what.....I was in the hospital for a solid two months. They gave me IV pulses of prednisone and maybe cytoxan....I do remember them giving me a bottle of pills of cytoxan and explaining that my hair would fall out.
So what I am getting at is that like Jack said you do have a autoimmune disease, and you need to be treated regardless of what the diagnosis is. I am 45 and have so much damage from the wegeners disease because no one new I had it until the doctors finaly put the pieces together in 2008. I am doing fine now and feel blessed that I see a specialist in Cleveland Ohio....
Cathy you will get throught this, I understand how you feel.." life passing you by." I have felt like that many times, but I also have learned to appreciate my family and dog more. Wishing you the best! Keep us updated.

Sangye
09-18-2010, 11:01 PM
Cathy, it's natural to feel the way you do. You can come on here and vent any time you want. I'm not the best one to cheer you up about Wegs, since I've had over 4 years of non-stop problems. I haven't worked since I was dx'ed in June, 2006. I haven't even come close to functioning as a regular person, much less the very healthy and strong person I was before this happened. Before that depresses you further, know that much of that happened because I didn't have a Wegs specialist until 2.5 yrs into it. By that time a great deal of damage had been done.

I called the VF last week. Canada has several VF docs who will consult with your rheumy for free. They also said the VF docs in the US will consult with your docs for free. So there is absolutely no reason why you can't have access to a Wegs specialist.

Your lung involvement is very unusual. There's no way a regular rheumy could know how to treat it. Basically he's just guessing and that's not okay. The notion of using IV ctx--less efficacious than oral-- merely to protect the bladder is just plain odd. You can protect your bladder quite well on oral ctx by drinking tons of water and peeing it out frequently. Most people have no problems when they do this. There's no way to get around the toxicity of these drugs. I understand your concerns about taking them if you don't really need them, but you've already seen that ctx made a difference. My guess (and I do mean "guess") is that you just needed ctx longer to really get the Wegs under control. But what you really need is expert advice. You can limp along without it, but I think you'll suffer for it--physically and emotionally.

JanW
09-19-2010, 12:15 AM
Hi, Cathy -- you can certainly have WG without a positive c-ANCA and many of us have never had positive biopsies. I too, have stenosis, although mine is luckily in my subglottis (around the true vocal chords) as opposed to lower in the trachea or in the bronchi, which is more difficult to get to. This is a rare (15% or so) complication of WG, and also a complication of sarcoidosis (which, as I understand it, is treated mainly with pred). It is a always a complication of relaspsing poly. It is not a complication of Churg-Strauss syndrome, which almost always presents as asthma and can easily be detected by testing for the presence of eosinophils in your blood (I was tested for this).

I can only tell you what my ENT told me, many times, as waited for my surgery (and to make sure that I didn't cancel my surgery)...'breathing is not overrated.' Relatively few of us here know exactly how you feel -- to always be short of breath and gasping doing simple things like walking and talking...clearing your through hundreds of times a day, not being able to sing or scream without losing your breath, not being able to read aloud or run for the bus. Been there, done that and there is not a day that I don't think about getting my breath back. My quality of life was severly compromised and I didn't even know it until I had gotten back what I had lost.

My other question is this -- whether there is a surgical solution for you beyond dilation? I had my scar tissue surgically opened with micro-scissors, and haven't had to have a second surgery yet (had surgery in March, dx in Jan.). I'm still open 7 mm (normal is 9 or 10), so it's worked out well for me. Not knowing anatomy well, and having it only affect the first two rings of my trachea, I don't know if surgery is possible lower down in the lungs. I was basically told that medication was not going to affect this problem in any way -- neither make it better, nor worse, nor stop its advancement. I have never been on pred for WG or for stenosis. I needed to be treated medically because I have WG, and WG is fatal if not treated, full stop. So I'm on mtx and in a mtx induced remission right now.

If you doc wants to call my doc, his name is Robert Lebovics and he practices in NYC. The number is 212.262.4444. and he is a VF consultants and one of the world's foremost experts on stenosis. I know that he works with a thoracic surgeon on cases that involve the subglottis and trachea (for surgical intervention; he's an ENT, not a rheumy).

One other thing -- do you by any chance have a saddle nose or have you noticed any depression starting in the bridge of your nose (which will look as though you have sort of developed a bump on your nose). That's a saddle nose and another marker towards WG. Typically if you have saddle nose you have stenosis, although, if you have stenosis you do't always have the nose. I have both, and I'd say it's about 50-50 around here that people have both. It also makes docs take you very seriously because it's prominent, unusual, deforming and a sign that tissue is dying in your body.

Good luck.

Sangye
09-19-2010, 12:29 AM
Jan, I wouldn't say that few people on here understand what it's like not being able to breathe. Lung involvement is very common with Wegs. I don't have stenosis but I've had lungs full of blood, clots, and both at the same time. I was on oxygen for 19 months.

Even when my lungs are not bleeding, my breathing is difficult. I can walk about 25 ft before overall weakness and gasping for breath force me to sit.

JanW
09-19-2010, 12:32 AM
Point taken Sangye.

I only meant that stenosis is by no means a common complaint of patients on this board.

cathy2330
09-19-2010, 01:46 AM
Hello everyone and thank you all for your responses. I know that all of us have it bad and I am not the only one. Jack I found your words and I have read about your story and you are very inspirational. I think what happened is I had done my 3 months of cytoxan and was really hoping that it would be the miracle worker and all would be great and I would be back to work and normalcy. When that did not seem to be happening I had a little breakdown. I am coming to terms with it, it could be worse, and will move forward with what needs to be done.

Jan I do not have saddlenose. I did talk to my thoracic surgeon last week when I saw him and asked him about using mitocylin C?? on the stenosis's. He said he will look into. I see him again Oct. 5 for bronch and dialation. My stenosis is upper trachea around vocal cords and lower trachea but real problems are in my bronchi's upper and also very low in the brochi's which are extremely hard to deal with as hard to get at. They need to remain open because once closed off cannot reopen. They are in both right and left brochi's.

I am seeing my rheumatologist on thurs. and I am going to talk to her about calling the Wegs specialist in US to confer. She did do this initially I believe she spoke to a Dr. Lebovics about my case and they discussed treatments and that is what I beleive they came up with, cytoxan iv with pred.

Interestingly, my last two bloodworks I have high eosinophils which is when my stomach problems started. This is why she is wondering also about this strauss chaurg syndrome. I will be going for bloodwork again on Monday but for over a month they have been high.

I will be moving forward today with a much better and more positive attitude, sometimes you just need some time feeling sorry for yourself then you need to pick yourself up and think about all the people that are worse off then yourself and move forward. Everyone has a story and goes through bad things at some point in there lives and sometimes I get wrapped up in my own problems and forget about others. Enough of that and onto moving forward and getting through the next stage.

Thank you all for everything and your words.

Jack
09-19-2010, 02:41 AM
sometimes you just need some time feeling sorry for yourself then you need to pick yourself up
I don't think there will be a single person on the Forum who does not relate to that.
Good luck with pushing things forward - there is a good life to be had with Wegener's, it's just a case of persisting until you find it.

JanW
09-19-2010, 03:45 AM
If she talked to Lebovics then you are in good hands as far as the upper airway stenosis goes, anyway. I did have myomicin C applied to the surgical area after my surgery.

drz
09-19-2010, 07:56 AM
Jan, I wouldn't say that few people on here understand what it's like not being able to breathe. Lung involvement is very common with Wegs. I don't have stenosis but I've had lungs full of blood, clots, and both at the same time. I was on oxygen for 19 months.

Even when my lungs are not bleeding, my breathing is difficult. I can walk about 25 ft before overall weakness and gasping for breath force me to sit.

I can make it 50 and sometimes 100 feet. They said my lungs would recover some. Have you got any improvement from worst stages?

Sangye
09-19-2010, 10:42 PM
Yes, I'm not on oxygen anymore, so that's a big improvement. If you haven't lived on supplemental O2, it's hard to describe how awful (and exhausting) it is.

I also lived at high altitude (7,000 ft) for the first 1.5 yrs with Wegs, then moved to lower altitude (3,000 ft). When I moved to Maryland (sea level) I really noticed the difference.

Oddly enough, my lung function is decent on PFT's. But they sound tight when I cough or laugh, and they don't function like my old lungs did. They've had quite the assault-- full of blood for 3 months before diagnosis, full of blood clots immediately after dx, pneumonitis infiltrates from mtx, possible pneumonia, ctx toxicity (twice!), bleeding again last year, and on/off pred (makes them more sensitive to pollutants, etc...). I also came into Wegs having had pneumonia 3 times before.