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View Full Version : New to WG and scared to death...help



LisaMarie
06-19-2010, 04:43 AM
Hello everyone...I have just been given the diagnosis of WG.....I started a year ago with upper resp issues and it was chalked up to my hx of asthma...after several misdiagnosis a bronchoscopy was done in December 2009 and I had sub glot trach stenosis.....found and expert at KU Med to repair it..I was 80-90% occluded by the time he go in there in Feb 2010....we did laser with erythromycin and balloon dialtion...at that time no eitology was known...a month out I was healing slowly and 25% occluded...then all went crazy...I got a repir cold I cold not shake and by the time I got in to see the specialist I was already 50% occluded...and WG was noted ...It also effects my right pariod gland cause the right side of my face to swell...I am seeing an RA specialist and he started me on pred 20 mg daily and methotrexate 10 mg q week til we can get the rituxan approved....i am a whimp when it comes to meds...I am throwing up the 1st 24-48 after taking the methotrexate...so not sure what they rituxan will do...They started me on low doses to see how I handle it...They wanted to start me on 60 mg of pred daily...I am wanting to know if anyone has the same sites I have...my doctor tells me that where mh WG has chosen to settle can be hard to treat and is rare....I have 4 children under the age of 15 and hate feeling like crap all the time and too exhaust to do activities with them....Does it get better?...I use all my energy to go to work...I guess I am just fustrated...sorry and info on this disease would be greatly appreciated
Thanks
Have a great weekend

Jack
06-19-2010, 05:00 AM
Hi LisaMarie and welcome to the site. So sorry that you had to come and find us!

First thing I will say is that "scared!" is a perfectly normal reaction to contracting Wegs. It is a rare disease so you will know little about it and that is scary, but as you get to know the disease better you may become more at ease with it. We can help you there! :)

You need to be treated by a vasculitis specialist with experience of Wegener's. It is a tricky disease that does not follow any rule book and requires first hand experience from your doctors. Let us know where you are (perhaps include it in your profile) and someone will be along to help you to locate one. The treatment you are receiving is not unusual, but there are alternatives that may be more effective and suit you better.

There are no typical symptoms for Wegener's and everyone on the forum has a different story to tell. However, there are a couple of very dangerous ones involving lungs and kidney function that must be tested for. That is where your specialist comes in.

I'm sure lots of other members will be along with more comments, but in the meantime feel free to ask any questions and vent any feelings of anger or fear. We all do from time to time! ;)

JanW
06-19-2010, 05:46 AM
Hi, Lisa Marie - Sorry you had to find us.

I was diagnosed in January 2010 based on my subglottic/tracheal stenosis and my saddle nose, plus positive C-ANCA and P3. I had surgical repair of the stenosis in March with a balloon dilation as well and I was still 100 percent open six weeks ago (go for another check up this Friday). My doctor is Robert Lebovics in NYC and his number is 212.262.4444. I would suggest that anyone in the US call his offices for advice on this. He is one of only three docs in the world (sorry, don't know who the other two are) who do this surgery in his particular way, and he learned it at NIH while working Hoffman and Langford, who people here will tell you are two of the top WG experts in the country (I believe they are both at Cleveland Clinic now). Anyway, NIH pioneered a lot of things in the US in regards to WG. I'm a little concerned that it doesn't seem like you were also coated with chemo drugs (usually myomicin is used) as well as steroids in addition to antibiotics (which is what you had) at your surgical site. It is believed that the chemo may retard regrowth, and it has been proven that steroids reduce swelling. I"m also a little concerned about the lasers, as many docs do not do this any more because of the risk of additional scarring (my surgery was just with microscissors).

Your doc is right that the subglottis is a rare site (about 15 percent of WG patients) but WG is just about the most common cause for stenosis so docs usually think of that right away -- in fact that finding came back when I had a CT of my throat just because of the narrowing (it said: "WG should be explored"). Do you also have issues with your nose saddling (that is, the bridge collasping), because those two things often occur in the same patients. I have both and was also misdiagnosed with asthma for five years.

I'm sorry I can't be any help on the meds. I'm on no pred but 15 mg of mtx, and have no side effects at all.

LisaMarie
06-19-2010, 07:14 AM
thanks I see Dr Garnette at Ku for my stenosis and Dr huston at at likes for my WG he is part of Dr Abdo group .My stenosis repair was with a cutting of the tissue into 4 like a pie then emycin injected then balloon dilated. My labs r negative at this time for WG so i was delaided in a diagnosis but fron my little k.owledge don't have to hv
ave positive labs

JanW
06-19-2010, 07:23 AM
If your rheumy is part of Abdou's group, is he consulting directly with Abdou, because that doc is on the list of medical consultants on the Vasculitis Foundation's website (a very important resource: Front Page | Vasculitis Foundation (http://www.vasculitisfoundation.org)). I see that Garnett wrote a paper about SS surgery that's up on emedicine, so he's considered some kind of expert in treating that condition, though not necessarily in patients with WG. Cutting into four like a pie sounds like my surgery, but it wasn't done with lasers -- I'm not clear why yours closed back up so quickly either.

LisaMarie
06-19-2010, 09:21 AM
i guess it is because I was not treated for wegener's...garnett only treated the symptom not the disease....was told without remission i have a poor prognosis so i am hoping for remission....it is really scary to read any info on the internet

pberggren1
06-19-2010, 11:14 AM
I'm glad to hear that you are in good hands LisaMarie!

LisaMarie
06-19-2010, 12:41 PM
so does anyone also have a parotid gland and lower jaw gland affected too...my gland by my ear and under my jaw swell and r painful...it feels like my lower jaw bone is deformed

pberggren1
06-19-2010, 04:15 PM
I have had some damage to my saliva glands due to Wegs because I have decreased saliva production. But which ones I don't know. I know that the parotid gland is the largest saliva gland.

I have some pain in my jaw, teeth, and ears sometimes but I am sure it is related to what is going on in my sinuses.

Jack
06-19-2010, 07:21 PM
Random symptoms seem to be quite common with Wegener's and many of them are not documented. Certainly there are lots of problems on the forum with ear and eye plumbing so problems with saliva glands would be no great surprise. As with most Wegener's symptoms, there may be attempts from doctors to treat the symptoms that they see rather than the underlying condition. If it is the cause, you should find that things improve when they get the Wegener's under control, which they will! :)

katarzena
06-20-2010, 03:06 AM
It is scarry but not that much. Most people achieve drug free remission after approximately a year of treatment (that's what I've been told) but there are some that have to be on low doses medicines forever to stay in remission (these are rare). So just keep the positive thinking, it is extremely important and you'll get through this!
I am on rituximab IV and I have non side effects so keep the positive thinking that you wouldn't have any either! ;)

edit: yes, it does get better. For me it was rough in the beginning but now, 4 months later, I feel perfectly fine, go to school, study and am doing my best to achieve remission. the only thing is I have those stupid side effects of medicines but everything else is normal (for me).

So.. Keep your chin up and ask everything you want know, remember there are no silly questions on this forum :)

Sangye
06-20-2010, 11:00 AM
Lisa, welcome to the group. Yes, Wegs is scary business. It's full of unknowns and that's the hardest part. Keep asking questions, though. This group is amazing.

Katarzena, some of that is not accurate. Most people achieve remission after about a year of treatment, but we don't have statistics on how many of them are in drug-free remission. I don't think drug-maintained remission is rare. I think it's quite common, actually.

Lisa, how you do with treatment is all over the map and it's impossible to predict how it will go for you. Some people who were on life support were back to work in a few months. Others who were in terrible shape but not bad enough to be on life support are never able to work again. My best advice is to focus on the present moment. I'll let you know when I'm able to follow my own advice for a solid day (or hour. Ahem.) :D

katarzena
06-21-2010, 02:12 AM
I guess I was wrong. I think the oney year treament for most people is correct but are you trying to say the you think it is quite common for wg patients to stay on drugs forever? If so that sucks.

JanW
06-21-2010, 02:42 AM
That's absolutely true, Katarzena. My doc has told me more than once that a drug-free remission shouldn't even necessarily be a "goal" of the WG patient (and I put that in quotes because goal implies something that is within someone's control) but we can live very well on drugs for the rest of your life.

Jack
06-21-2010, 03:49 AM
I did not like to say, but I was under the impression that drug free remission was very much in the minority. Sorry, but I can't find any statistics to support.

katarzena
06-21-2010, 06:27 AM
I still don't think so or I don't want to believe it's true? I don't know. All I know is that a friend of mine has another autoimmune disorder and is now off medicines and in remission after about a year of treatment and that keeps my spirits high!

Jack
06-21-2010, 07:25 AM
Over the years I've read a lot of papers about Wegener's and it is difficult to find any exact statistics about drug free remission, but my impression is -

For many people, as their medication is reduced the Wegener's symptoms start to return and they have to be treated with a maintainance dose to keep them stable. They are in remission, but not drug free.

Others are able to reduce their medication to zero without symptoms reoccurring and can be considered to be in complete remission, but the relapse rate amongst this group is very high.

A minority manage to remain drug free and in remission for a significant length of time - 10 years?

I'm afraid that the truth of the matter is that this is not yet a disease with a cure.

Sangye
06-21-2010, 07:54 AM
Jack has it right. Really the only statistic we see about Wegs is that after a year of treatment, about 80-90% will go into remission but 50% of them will have a flare again. That doesn't distinguish between drug-free and drug-maintained remission. And it doesn't say what percentage will flare over and over.

The main goals of the Wegs specialists are to:
1) Keep you alive
2) Minimize damage to organs/ tissues
3) Treat flares with the mildest drugs and/or the lowest doses as much as possible
4) Get you off pred or at least to 5mg or below
5) Help you adjust to a new normal

It took me awhile to realize that my goals were somewhat different than my doctors':
1) Stay alive
2) Avoid damage to organs/ tissues
3) Never have a flare
4) Be off pred and all drugs forever
5) Get my body completely back

I bet a lot of you can relate.

LisaMarie
06-22-2010, 01:01 AM
Yes I will agree that my goals are different then the doctor's from what I can gather......I am trying not to read too much on the internet as it is scarey and I want to live a long life and enjoy my kids....I have a habit of pushing it when i should be resting and have been told I need to listen to my body and rest when it needs it......I am trying to do that ....I guess I can not expect miracles if I won't play by the rules...When my trach was 90% occluded I still worked 12 hour shifts 3-4 days a week wirh a heart rate of 130-160 as a nurse on a medsurg floor....Doc's are sure how I did it ...I told them by the Grace Of God...bills have to be paid and kids need food...but I have been told I need to stop and rest or they may not be able to achieve remission...I have taken a desk job doing quality review . I was able to wait the 1 1/2 months for the surgery by changing jobs...so it was a blessing the job came open....when they first found my stenosis it was documented unknown eitology...it was not til about a month ago when I started having breathing problems and they scoped me did they see active WG in my throat ...trach and Right sinus ...so now I am waiting again for the power to be to make a decesion....I saw dr Abdou group and was put on methotrexate 10 mg weekly and started on 20 of pred daily...The physicain want to start me on 60 mg of pred but I usuually have funny reactions to meds so we started on a lower dose....I am sure he will increase it after I go back...trying to get rituxen approved....I appreciate all of you all's knowledge and understanding....the 2 people I have known persoanlly that had WG have passed ..so there is really no one to ask my questions too......I have also learned that beening a nurse with an illness is not always a good thing...we either know too much or think we do ....The statics I found on line was 5 yrs if remission not achieved ...I like the data here better...hope you all have a great week ...my nausea is starting to subside from the methotrex....yippy maybe I will have 2 good days before I start loosing my cookies again......

brocky99
06-22-2010, 02:44 AM
When I was 1st diagnosed I also pushed myself to the limit everyday thinking the drs didnt know what they were talking about, what with a pub to run, 2 kids plus my other half and 2 dogs I never stopped but when I was back in hospital 2 days after being discharged I realised that I couldnt run round as much. Now my kidneys have nearly failed and I have no energy but I do try to do as much as I can but I also know when to stop and rest which is a lot more often these days.

LisaMarie
06-22-2010, 06:25 AM
I have another question...Does anyone get Left shoulder joint, neck , jaw and chest pain all at the same time that decreases if you hold your breathe....2 yrs ago the cardiologist said it was arterial spasms...wondering if it could be WG...had an episode on Sunday that lasted 20 minutes...Should I ask my WG doc?...It is scary to experience but the cardio said it was not a heart attack...so I tend to deal with them ...they happen about ever 3 -6 months for no reason.....

LisaMarie
06-22-2010, 06:29 AM
Look like we both come from strong stubborn stock as my grandmother use to say....I will try to slow down and listen...and will pray you can too......Thanks for letting me know I am not alone

Sangye
06-22-2010, 06:52 AM
I have another question...Does anyone get Left shoulder joint, neck , jaw and chest pain all at the same time that decreases if you hold your breathe....2 yrs ago the cardiologist said it was arterial spasms...wondering if it could be WG...had an episode on Sunday that lasted 20 minutes...Should I ask my WG doc?...It is scary to experience but the cardio said it was not a heart attack...so I tend to deal with them ...they happen about ever 3 -6 months for no reason.....

Has your Wegs doc done an echocardiogram recently? Heart involvement with Wegs is not common, but it is silent. We just lost a member to it a couple weeks ago. He supposedly only had sinus involvement but Wegs was destroying his heart. You should have an echo at least annually, and more often if you have unusual symptoms or concerns.

LisaMarie
06-22-2010, 07:22 AM
Echo done last fall...had both a regular and a bubble echo....will see if they can do it again.....thanks
I feel like I need to know more than I do...and at the same time trying not to over analysis things ...ughh

talentx7
06-22-2010, 07:32 AM
I really hope that the stat about living 5 years with WG is VERY VERY old. I wonder if WG is not all that uncommon anymore. There seems to be alot of us out there, and more and more are being diagnoised. Hopefully that will mean better treatment and (holding my breathe) a cure.

Sangye
06-22-2010, 07:42 AM
The 5-year survival rate is a standard way of describing serious illnesses. It doesn't mean that no one survives after 5 years.
Five-year survival rate - Wikipedia, the free encyclopedia (http://en.wikipedia.org/wiki/Five-year_survival_rate)

I wish we could get good data on the real prevalence of Wegs. The number I've usually seen is about 500 new cases each year in the US.

ranpar01
06-22-2010, 09:41 AM
The 5-year survival rate is a standard way of describing serious illnesses. It doesn't mean that no one survives after 5 years.
Five-year survival rate - Wikipedia, the free encyclopedia (http://en.wikipedia.org/wiki/Five-year_survival_rate)

I wish we could get good data on the real prevalence of Wegs. The number I've usually seen is about 500 new cases each year in the US.


I've seen the 5 year figure as well and agree that it's a stock number that get's thrown around for a lot of serious diseases. I've also read that sympton free remission stretches can be up to 20 years. My Dad was symptom free for many years before this last flare-up he had. As for prevalance, I'd love to see those figures as well but that might be very tricky because WG is (in my opinion) not as rare as people think - it's just RARELY DIAGNOSED. That's why forums like this and others that try to raise awareness are so important.

LisaMarie - I'm glad you found this forum and are getting some answers. This is a great group of people.

LisaMarie
06-22-2010, 11:56 AM
Trust me I am praising God I found this web site/support group.....It is keeping me sane for now....I just want answers and am learning I may not get them and learn to accept it....Got to watch my youngest play ball tonight....He did great and they won....yeah!!!!.i don't want to miss any of this and it just gets fustrating ...He wanted to swim with me afterwards but i was too wiped out from work and watching the game in the heat and all...so he gave me a rain check....okay enough complaining it could be so much worse....have a great night

Nancy
06-27-2010, 05:34 PM
Hey Lisa Marie -
read your post here - I saw you mention KU Med... I live in Topeka, KS and see Dr Abdou.... See if anyone has any issues with changing to him... He is THE one that you want to see.. I am also a nurse practitioner - so yes, understand the work to maintain kids and bills... I have had Wegener's for 14+ years and would be happy to help in any way that I can.... Waiting with crossed fingers for the RTX same as you.... Dr Abdou specializes in Wegener's and actually pursues the information and attends all conferences, etc.... Talk with you soon...

LisaMarie
06-27-2010, 09:47 PM
i was told he is not accepting any new patients....plan on asking again on my next visit.Dr Scott assured me he will consult on my case. Genentech denied me even though i make less money now due to declining required a change in positions at mu hospital...working on an appeal..just pray everyday God gives me strength and patience....talk to u soon..glad to know someone close to home see Dr Abdou

Sangye
06-28-2010, 12:41 AM
LisaMarie, docs will often make a personal call to ask for an exception. It always works.

I had a great PCP in Arizona who was closing her office. I was beyond terrified. At that time my rheumy was awful and had nearly killed me several times. There wasn't another rheumy in all of northern AZ, and my PCP was a lifeline for me. The PCP my doc wanted me to see was quite popular and wasn't taking new patients. My doc called her and she made an exception for me.