cytoxan question

[cathy2330]

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]

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]

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]

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]

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]

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]

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.