New to the forum, and to Wegener's (I think...)

[godgirl]

My name is Jen, and I'm new to this forum. And to Wegener's.

My rheumatologist hasn't officially diagnosed me. I have symptoms, but my lungs and kidneys aren't affected at this point. I AM ANCA positive, however.

This all started just before Thanksgiving 2013. Woke up one morning to a very red eye. Didn't hurt, though. Didn't hurt for 5 days. Finally went to a specialist when the socket started hurting. Found out it was scleritis! I was a bit surprised, and so was the eye doc. He called my primary from his office to set up a workup for auto immune and connective tissue disorders.

After that, I stared seeing my rheumatologist. I was on taper after taper of prednisone, but anytime I went below 20mg, my eye would flare. The next symptom cropped up in January. My sister jokingly said to me, "are you going deaf as well as blind" as she turned up the TV. We started laughing and then stopped - because that might not be funny...

Had a hearing test and started seeing an ENT. Lo and behold, yeah, I had hearing loss and now have horrible tinnitus. Went to the rheumatologist and started methotrexate - and was keeping on my prednisone. Couple of days later - nosebleeds. AWESOME.

That's kind of it in a nutshell. There are other things going on that my docs know about - roving pains, facial pain, nose pain, tooth pain. My rheumatologist is super cool and she's been really thorough. I've been through a billion and a half tests, CTs, MRIs, labwork, x-rays - you name it. We're talking cytoxan at this point, but she is hesitant to put me on it until there's physical proof of the GPA. Up until last fall, I had been pretty healthy. Now, not so much. That's been pretty hard to deal with... No stamina, so much fatigue...and just not feeling good in general.

Anyway, glad to have found you all!

Jen

[Dirty Don]

Welcome Jen...what a familiar story. Glad you found us...a great group of experienced, caring, fun peeps! Seriously though...you can learn a lot from everyone on here. Many degrees of WG, which you may or may not have, but it sounds like it...really familiar story. Sounds like you have some docs on the ball, make sure they are getting first rate knowledge of how to deal with this, don't just accept 'more testing' if your body tells you different...there has to be a start to treating this as immediately and appropriately as possible, WG or not. Lots of peeps will check in soon with places, docs, experiences, etc. and you can glean from them. Best to you, stay patient and strong.

[MaxD]

Jen,

I'd strongly urge you to consult asap with a vasculitis expert (check out vasculitisfoundation.org) - based on your test results and history they may recommend against waiting for a tissue sample and they may also recommend Rituximab, which has fewer side-effects than Cytoxan and is at least as effective in a 3-year old study. My symptoms/history sound very similar to yours, and that is what my local rheumatologist as well as a vasculitis expert diagnosed and recommended for treatment. I was put on 1mg prednisone per Kg of body weight, with a slow taper once the Rituximab treatment started.

[Pete]

Hi Jen,

Welcome to our world. I agree with Don regarding treatment and managing your docs.

Cytoxan is one of the drugs used to induce remission and get the symptoms under control. If they prescribe it for you, be sure to stay well hydrated because this drug can be hard on the bladder.

Methotrexate is more of a maintenance drug - used after symptoms are controlled to keep you in a drug induced remission.

Prednisone dosage may increase also. Many of us started at 60 mg/day. Pred's common side effects at high dosages include weight gain (insatiable appetite), moon face, and buffalo hump.

Regardless of the side effects, it's possible to lead a nearly normal life. Your hearing may return in time. Not sure about what will happen with tinnitus. I had tinnitus before I got sick as well as some hearing loss. GPA clobbered my right ear. I wear hearing aids now. There's some improvement. At least I can listen to TV and car radio at the same volume level as my wife.

Keep us posted on your progress. Good luck and better health!

[godgirl]

Thanks, everyone.

Don. It does sound a LOT like a lot of stories here. Doc is pretty sure its Wegener's. Other than not having a biopsy, I'm not sure why the dx isn't there yet. I have no patience when it comes to my health. Guess that's gonna change! Ha!

I've been doing a lot of my own research since Christmas, and yeah, Pete, I thought mtx would be more maintenance. My biggest concern right now is that i don't have kidney or lung involvement at this point. If we don't catch this now, how long will it take for that to happen? I don't want to go on cytoxan because my kidneys and lungs finally were affected, you know? Why wait?

I'm meeting with my rheumatologist every two weeks at this point, and I see her Friday. We reassess every time.

MaxD, we're not sure insurance will pay for rtx at this point. Dr ofc is looking into it. I hope they will. Ctx sounds horrible. But if I have to take it, i'll suck it up.

You know what doesn't help? Working full time and school part time. Oh well. Done whining. :)

[windchime]

Thanks, everyone.

I've been doing a lot of my own research since Christmas, and yeah, Pete, I thought mtx would be more maintenance. My biggest concern right now is that i don't have kidney or lung involvement at this point. If we don't catch this now, how long will it take for that to happen? I don't want to go on cytoxan because my kidneys and lungs finally were affected, you know? Why wait?

Ctx sounds horrible. But if I have to take it, i'll suck it up.

You know what doesn't help? Working full time and school part time. Oh well. Done whining. :)

Cytoxan isn't that horrible. I was on it for five months, had some nausea in the beginning, but it went away. Important thing about it is to take all the med in the morning so it doesn't sit in your bladder overnight. When it was making me nauseous I would take half the dose at 9 and the other half at 1030. This seemed to help. Or some such time schedule. It's just important to get it down as early as you can. Eventually I just took it all at once with no problem.

Another concern is thinning hair. My hair has thinned and I've been assured by others on here it will return. I'm waiting for it's return. :rolleyes1: Make sure you drink plenty of liquid like Pete advised. This drug can be hard on the bladder, but not the kidneys to my knowledge. Cytoxan is also available as an infusion if that is something you think you'd better tolerate. There are always options. How severe your case is will dictate what your doc chooses for you. Everyone is different.

You will not necessarily ever have kidney or lung involvement. There are many on here that have had wegs for years and only have involvement in one location. The key will be listening to you body to catch your flares early. And there will most likely be flares.

[drz]

Some Weggies were diagnosed without any confirming biopsy. The most reliable biopsy are kidneys when kidney is affected, next the lungs if they hit an affected part but they can take larger samples in lungs so often have a high positive rate. Biopsies elsewhere tend to be very problematic and hit or miss due to problems in getting a good sample from an affected part where the Wegs is active.

It is true there are some risks to treatment for GPA but most of us think and know from our personal experience that untreated Wegs is usually far more dangerous and risky than our treatment cause Wegs can smolder for years and then turn deadly in a few weeks or even days. I believe a good Weg expert is comfortable starting treatment even without a confirming biopsy if all the signs point to GPA and they ruled out any other likely cause of symptoms.

[mishb]

Hi Jen and welcome.

I agree with what everyone above has said or recommended to you.
It certainly sounds like a familiar story to most of us.
I was lucky that my sinus biopsy confirmed WG, but all of the signs and blood tests basically confirmed it anyway.

I'm glad your Rheumatologist is on top of things.

Try not to stress to much about things and ask as many questions as need be, both to your doctor and on here.......someone will know the answers.

Take care and .........

[Alysia]

welcome to the Forum. I think you can ask about rtx instead of ctx. the side effects are less severe.

[Pete]

You know what doesn't help? Working full time and school part time. Oh well. Done whining. :)

I'm retired and know how much a flare depleted my energy level. I doff my chapeau to weggies who have to work while this damned disease ravages their bodies.

[annekat]

Hi, Jen, and welcome to the forum. I agree with Cindy that CTX is not that horrible. I had about the same experience with it as she did, and was able to get used to it at least as well as I'm now getting used to MTX as a maintenance drug. And for people who only have WG involvement in their nasal/sinus/ears area, I know of several on here who have successfully used MTX to get control of the disease, which is definitely milder than CTX in terms of long term risks. We know of a top WG doc who has recently prescribed MTX as an initial treatment for one of our members here. I would not push for using RTX if your case can be controlled easily with one of the others. It can be saved in case it's needed later. If your rheumy chooses CTX, I'd expect your issues to be taken care of fast, and then you can move on to the maintenance stage, which could mean MTX or Imuran plus probably a continuation of prednisone for quite awhile, with ongoing tapering. If you will tell us where you live, you might get a recommendation of a doc experienced with WG who can manage your care or work with your rheumy as a consultant, should you feel any doubt that you are getting the best care.

[jvilner]

Welcome Jen! I'm also pretty new to the forum.

[jvilner]

I was dxed with psoratic arthritis and took mtx for 2 yrs until I started with problems with my ears. Lots of fluid and hearing problems. My rheumi did not want to put me on meds until I had a biopsy from an affected organ. Looking back in retrospect it all took 2 months but it was decided to not wait for any other organ involvement and biopsy was done from ear to confirm wegs. I hope I'm not sorry that I waited 2 mos to start treatment and get this disease under control. But am happy that so far no other organ involvement. I am treated aggressively with prednisone imuran vitamins folic acid to slow progression and treat ears. Only time will tell and for sure my life has changed. Always at drs and doing tests. But someone once told me that the first yr after diagnosis is hard. Have to get used to yr new situation. You will with our support here. It's been very helpful for me.

It sounds like you have good drs but stay proactive and best of luck!!

[godgirl]

Again, thanks everyone.

I'm very touched with your replies. I've felt so alone in this journey. My mother died 7 yrs ago of another rare autoimmune disease (inclusion body myositis), so I've been kind of waiting for this bullet to hit. But it was still hard to hear it all.

In particular, I'm VERY relieved to learn that CTX probably won't be as bad as I expected. I was SO worried about that and how much work I'd have to miss, and if I'd have to put off school. Whew...

So far, my only symptoms are in my eyes, ears and sinuses - and the sinuses aren't so bad. But I do have the roving pains in my joints and the facial/nose/tooth pain. And it's a different pain than when I have a sinus infection.

Question for everyone with ear problems/hearing loss. Was it fluid and infections that caused the hearing loss? Mine is NOT fluid nor is it otitis media. I have pressure behind my ears, but the ENT and rheumy say it's inflammation. In fact, my ENT suspects I also have Cogan's Syndrome. So I'm wondering if the Wegener's can be active in your ears without fluid or infection. Does that make sense?

Again, I really appreciate everyone here. I love being talk to people who know what I'M talking about! And realize that even though I don't look sick, I am.

Jen
PS I'm in the Madison, WI area.

[Pete]

Now that we know where you're from...

You live within reasonable driving distance from Mayo Clinic where there is an excellent group of wegs specialists. Might be worth a referral to get a definite diagnosis and state of the art treatment plan.

[godgirl]

Yeah, Pete, I have thought about it. And I've started talking with my insurance about it as well. I'm not sure they're keen on my going up to Mayo. I have an HMO plan thru work... And I understand medical billing.

I'll let you all know how that turns out. It kinda sucks that your insurance has to dictate your care. :-/

[BookNut]

Welcome to this wonderful group. I joined when I was mis-diagnosed with Wegener's. I have stayed on as an honorary member because many of my symptoms are similar, but mostly because I LOVE these people. They are so brave, helpful and generous with their time and wisdom. You will find SO much help here!

[MaxD]

You got that right! But Pete makes a good point - if you are able to, at least get an expert second opinion at Mayo and have your doc work with them. The hardest part is always the diagnosis and treatment plan. Once that plan is settled, it takes a lot of worry off ones mind, and you follow the ride with full confidence in your medical team while focusing on yourself instead of the disease.

Good luck to you, and to all of us!


Yeah, Pete, I have thought about it. And I've started talking with my insurance about it as well. I'm not sure they're keen on my going up to Mayo. I have an HMO plan thru work... And I understand medical billing.

I'll let you all know how that turns out. It kinda sucks that your insurance has to dictate your care. :-/

[annekat]

Question for everyone with ear problems/hearing loss. Was it fluid and infections that caused the hearing loss? Mine is NOT fluid nor is it otitis media. I have pressure behind my ears, but the ENT and rheumy say it's inflammation. In fact, my ENT suspects I also have Cogan's Syndrome. So I'm wondering if the Wegener's can be active in your ears without fluid or infection. Does that make sense?

About ears and hearing loss: there are various causes among Wegs patients. Others may know more about this than I, but i do have loss that looks like it is probably permanent. Some with hearing loss get over it eventually. I think the most common scenario is eustachian tube disfunction, caused by inflammation and tissue damage from the effect of Wegs on the whole sinus/nose/ear area. As in many cases, my tubes are swollen and damaged to the point where they will not open properly to let fluid out and let air in to balance the air pressure on both sides of the ear drum. My ear fluid has, in fact, probably turned hard and gummy to what they call "glue ear", which would not drain even if the tubes were open. Some people can be helped by tubes installed in the drums to let fluid out and air in. Others may report their tubes being open all the time, so there is a weird echo or roar which also affects hearing. Loss due to e-tube dysfunction is usually referred to as conductive hearing loss, and may or may not improve as inflammation is dealt with. Catching your Wegs early and being treated right away would help. The other major kind of hearing loss is neuro-sensory, which is caused by nerve damage in the inner ear which can happen during an infection, as it did with me in one ear. I don't know if there are other causes... possibly exposure to loud noises would be one. But I believe this type of loss is always considered permanent, though there are some great high tech devices for dealing with it. Mine is minor enough that I can get by with regular hearing aids for both types of loss. Anyone please feel free to correct me about any inaccuracies here!

[godgirl]

Thanks, Anne.

I have neuro sensory hearing loss. ENT says it's probably due to inflammation of the eighth cranial nerve... When I had my first hearing test, the audiologist had no idea what was going on - she said the pattern was odd and something she hadn't seen. When the ENT and I met, he said he looked at the pattern and knew it was auto-immune. Specifically because all of my low tone hearing is gone. He also said it was permanent loss. And since then, all my hearing tests look the same. They test for fluid every time, and look for infections, and I have none, nor any indication there have been fluid or infections. I used to get ear infections about 10-15 yrs ago, and now very rarely. And the pressure I mention isn't fluid... It's way inside my head.

Hmmm... Well, hopefully, my docs and I will eventually get this worked out.

[annekat]

Thanks, godgirl. The ears and all the mechanisms involved with them are still pretty mysterious to me, so it helps to hear explanations from others and their docs. I'm sorry you have permanent loss. Mine probably is, too... I guess I can live with it, it isn't as bad as some have to deal with, yet, anyway.... It's funny how something like wearing hearing aids can be one of the worst things imaginable, but then when it happens it really isn't that big a deal. I'm thankful to have them.

[drz]

Thanks, godgirl. The ears and all the mechanisms involved with them are still pretty mysterious to me, so it helps to hear explanations from others and their docs. I'm sorry you have permanent loss. Mine probably is, too... I guess I can live with it, it isn't as bad as some have to deal with, yet, anyway.... It's funny how something like wearing hearing aids can be one of the worst things imaginable, but then when it happens it really isn't that big a deal. I'm thankful to have them.

Four years ago when I went deaf from Wegs I wondered how I would adjust to life without hearing. But then I got my BAHA and a regular aid for other side and great improvement in my hearing again. Last night I enjoyed a concert of classical music with lot variation in notes and volume. The only big down side for me was needing to often adjust volume to minimize feedback at times as certain pitches seem to over load aids at louder volumes. But it sure beats being deaf and going without any music.

[annekat]

Four years ago when I went deaf from Wegs I wondered how I would adjust to life without hearing. But then I got my BAHA and a regular aid for other side and great improvement in my hearing again. Last night I enjoyed a concert of classical music with lot variation in notes and volume. The only big down side for me was needing to often adjust volume to minimize feedback at times as certain pitches seem to over load aids at louder volumes. But it sure beats being deaf and going without any music. I remember trying to enjoy a music concert with no aids, before I ever knew I had WG, and was having all kinds of fluctuations and weirdnesses in my hearing. I thought it was just continuing repercussions of the horrible bilateral ear infection I had had, holes in drums not healed, etc. I could generally hear the music but it didn't sound right. I don't think I've been to a concert since getting my aids but actually expect it to sound pretty normal. Guess I should buy some tickets and find out.

[pberggren1]

drz, that's awesome your setup works so good for you.

Anne, what does music from your stereo system sound like?

[annekat]

Phil, I don't have a real stereo system set up, just a good sized boom box, and don't know how my aids would do with a real system. When I do listen to it, it sounds good enough for the way I use it, often just as background noise, not cranking it way up or anything. When I'm selling at the farmers market and there are lots of noises from different directions, then I will occasionally misjudge what direction a voice is coming from. But not often. I do know I need another hearing test and the aids probably need adjusting, but my problem isn't severe enough for it to be a big deal. The best thing that happened lately was that my right hearing aid stopped squealing after my ENT pulled out a huge chunk of ear wax.

[drz]

Phil, I don't have a real stereo system set up, just a good sized boom box, and don't know how my aids would do with a real system. When I do listen to it, it sounds good enough for the way I use it, often just as background noise, not cranking it way up or anything. When I'm selling at the farmers market and there are lots of noises from different directions, then I will occasionally misjudge what direction a voice is coming from. But not often. I do know I need another hearing test and the aids probably need adjusting, but my problem isn't severe enough for it to be a big deal. The best thing that happened lately was that my right hearing aid stopped squealing after my ENT pulled out a huge chunk of ear wax.

My hearing also improved after getting my ears and hearing aid cleaned up with new tube. It is strange how a bit of wax or gunk can really impact your hearing in a negative sense.

[annekat]

Yep, and it's important to get the tubes replaced every so often. My audiologist will do it for free every 6 months and recommends that interval. I waited too long and one of the tubes broke, because they get brittle with time. And it was harder for the technician to get the old tube out of the ear mold, and the ear mold got a little damaged. But it still functions. I'll try to be a little more diligent about aid cleanings and tube replacements, as well as having ear wax buildups removed.