Please explain what Wegeners really does.

[pberggren1]

As far as cyclophosphamide goes the typical treatment time for that is 3 months but no longer than 6 months unless absolutely neccessary. But with azathioprine and methotrexate they can be used for much longer periods of time because they are much less toxic and are considered maintenance drugs.

Remissions vary greatly from patient to patient.

[vdub]

How long does usually a patient have to take the chemo? After the chemo is over do you usually go into remission for a long time?

Yeap, like Phil said. I will add, tho, that while on one of the immuno-suppressants, you are much more susceptible to infection. I've been on mtx for 14 months and I try to avoid anyone who is sick or places where you are likely to get sick (nursing homes for instance). I might be in remission now, but won't know for a couple more weeks. If I am, then I will slowly taper off the mtx. The taper will take about 3 months. No one knows how long someone might stay in remission or what my trigger it to reappear. The disease is really strange and affects everyone differently.

[marta]

Ok, Wegeners is an Auto Immune disorder which the small vessels get inflamed and prevent blood flow from reaching the organs. Can someone explain why the patient feels so week , why the body pains. the loose of muscles etc. After reading some stories here I see Wagner's affect more than organs.Ive read about deaf, why? Why the weight lost if the person is eating normally. Please help me understand this disease. Its so much more complicated!

I wanted to see what others say before I said something because I am going to guess at the answers from just experience and some of the stuff I learned in school many many many years ago.

I think the initial pain that we get when we first get the disease (before we start taking all the drugs post diagnosis) is the inflamatory response which is the normal body response to either trauma or a viral or bacteria infection. It's just that our immune system is confused and is responding to nothing real. Normally a headache is (be it primary or secondary) due to some issues with blood flow. In the case of migrane headaches it is a reduced blood flow to the cerebral cortex. Cluster headaches are also linked to altered blood flow in the head. And since one of the medications used to treat normal headaches is Ibuprofane - and anti inflamatory, the cause is at least partially an issue of inflamation. I don't think that Wegener's causes infections, it mimics an infection. This explains the many failed attempts of many of our doctors to treat us with numerous antibiotics before diagnosis. I think that most of our problems are primarily inflamatory, and secondarily the deposit of granulomas which impedes the effectiveness of whatever it has attached to and eventually destroys it and leaves behind useless scar tissue. The granuloma deposit is also an immune response to trauma (loosing healthy tissue is replaced with granulomas which in normal circumstanses eventually develops into new healthy tissue). That is the reason for the pain before diagnosis and why normally high doses of pred seem to relive the pain so quickly - pred = the world's best anti inflamatory.

As Sangye said, the body uses up a ton of energy trying to recover hense the weakness.

There are two main reasons for the deafness (I have one of them and Phil is an expert in the other). Mine is because of blocked eustachian tubes (inflamed and shut closed) thus creating a vacuum in my middle/inner ear cavity which sucks in fluid from adjoining cells and fills the cavity with fluid which prevents sound waves from reaching their final destination properly. I just had tubes put in three days ago and can now hear better than I have in the last six months. I was very very deaf until three days ago - little things I hear are putting a smile on my face today. Phils issue is sensory nerves. I think (and I could be speaking out of turn here) that his nerves were damaged from the disease, probably lack of blood supply to the nerves during active disease state. Phils is a lot tougher to deal with which is why he is going to give cochlear implants a try.


I also think that part of the reason that it affects us different and some people get ear issues while others don't, and the sinuses, and the lungs and the nerves, and kidneys, skin (also an organ) etc., etc., etc.... has to do with the natural variation within the human species. Wegener's affects a certain size of blood vessels - the really small ones, and there is a range of variation with almost every detail in the human body so why not the size of our blood vessels that lead to various organs....That's my theory, not sure how right it is.

Once we are diagnosed I think that usually the hospital stay and dealing with the disease when it's fully active drains a lot from the body. I was very very very strong before I got sick and the short time I spent in hospital (lung biopsy, new drug regimen, laying in bed and not doing much and eating the horrible hospital food) rendered me useless. It took weeks before I had enough strength to walk around the block. Taking a shower was THE chore of the day and sucked all of the energy I had saved up for the entire 24 hour period. I also continued to loose weight after the hospital for another two weeks, and ate like a horse when I got home and was popping the pred like it was going out of style. I didn't start to get the puffyness for at least two to three months after starting the treatment. The meds also suck out any of the good stuff (i.e. muscles) that you've built up over the years. So like Sangye said, more fat, less muscle - hence our super awesome Weggie look.

It is a crazy disease, but once we're on the meds and keep as safe as possible (without turning all Howard Hughes) from infections and other opportunistic invaders, we have a good chance of getting some semblance of normalcy back in our lives. I haven't gotten the full sense of normal, but I'm not giving up hope that it's possible. Getting my hearing back this week, and my sense of smell back in the last month, I am sure enjoying these seemingly insignificant sensory experiences, so baby steps forward.

One thing is for sure - this too shall pass.

[pberggren1]

Thanks for this nice post Marta. But then again all your posts are nice.

I feel like punching someone in the face right now. The pred is really getting to me. The last couple of weeks the slightest thing sets me off. I wish I had a nice gun to let off some steam with. I know that would be good therapy for me. Or maybe some rope and a bridge and a...... LOL

Anyway, Marta is mostly right about my hearing loss.

When the Wegs first came along for me in 2003 my loss was conductive like Marta's and some nerve damage but to what extent we will never know for sure. I had 3 sets of ventilation tubes put in my ear drums with no success in the first year. My ENT said it was like digging in concrete. He aslo said that my eustachian tubes were most likely shot and not functioning due to damage from the Wegs like granulomas and scar tissue and inflamation.

Then in 2004 I was flaring and did not know it. I was on mtx at the time as well. I had 3 weird episodes like almost fainting and then coming to with less hearing in one ear or the other and increases tinnitus. By the third episode I was totally deaf in the left ear and my right ear had about 50% hearing left. My ENT said that it could be the Wegs that did this or the mtx or a combination of the both, but there is no way of knowing for sure.

Then in 2005 I got hearing aids and they helped a lot. I have what is called a Bi-Cross set up where the left hearing aid is a microphone with a transmitter that transmits the sound to the right hearing aid. I did fairly well with these up until December of 2010.

In December of 2010 an antibiotic I am taking wrecked my right hearing nerve completely and left me totally deaf in my right ear as well. But in March my ENT put another set of tubes in because the cochlear implant people said it looked like I have fluid in my ears and if that is the case then I cannot have cochlear implants. So he put the tubes in and 2 days later a bit of hearing came back but was gone again by the end of the month. The ENT said most of the loss was due to the Amikacin but that obviously there was some conductive loss as well but because the eustachian tubes are shot they do not allow normal air movement within the middle ear and cause the ear drum to cave in.

So this is where I sit now, completely deaf.

I see the cochlear implant surgeon on August 10. I hope that I will be able to get the implants. I have been communicating with a lady in town here that had both sides done back in 2007 with great success. So hearing her story gives me hope.

[Sangye]

Stephanie, if you feel it's appropriate and you have the chance, would you be willing to post your last reply on this thread? I think it would really help newbies.
http://www.wegeners-granulomatosis.c...need-know.html

[chrisTIn@]

I think this still is a good thread.
Every now and then the question pops up again.
What is Wegeners?

Well. most answers are right here...

[renidrag]

It is good you brought this up as it seems people signing in are looking for info. Pay special attention to Sangye's post about What the Newly diagnosed need to know just above.

[Barbara N]

That is one of the better descriptions I have ever gotten.

[Barbara N]

Good luck with that Phil. I am seeing me ENT doctor in Madrid in May when I go to the ophthalmologist. Last time they said I wasn't a good candidate for the implant.

[Barbara N]

I hope there isn't an exam afterwards because I don't think I have the whole thing figured out yet.You should all get together and put out and owners guide on how to let Wegener's share your body. We are lucky to find out so much here but there is still so much more everyone could add to It is so complicated especially if you are living in an area where WG is not well known. Moving is not always a possibility. I am still knew to this sight and everyday I am learning new things about my condition and hearing stories that affirm my belief that you can go into remission for a long time and the importance of staying strong, positive and healthy Good health to every one, Barbara