What to Expect...

[Pmarsh]

Hello. I was recently diagnosed (Oct 2010). I'm 32 and have always been in excellent health (before all this). I've made a pretty quick recovery after an initial visit to the ICU for lung issues, and chromic sinus issues. It didn't affect my kidneys however. I'm now on Imuran and 7.5mg of Pred. Finished cyclophosphamide last month.

I'm just interested in hearing from some people who have had Wegener's for a while or who were diagnosed later in life. I'm really just fishing for an idea of what to expect down the road. Are many people in a drug free remission? Are there people who have had none or very few relapses? Are relapses typically less serious than the initial onset (now that you know the symptoms)? I know everyone will be different to a degree. I'm just trying to be optimistic that my life (and active lifestyle) can go on with only minor changes - like taking drugs and closely monitoring my condition.

Thanks for any advice!

[Jack]

I was diagnosed at the age of 30 and I am 56 now. As you already know, Wegener's affects everyone in a different way and this variation can be quite radical. All the way form Hardly Affected at All through to Dead! :scared:
I had major kidney and sinus involvement and my diagnosis was quite late. Also, although my treatment was the best available from a research centre it was early days and things have now moved on. But taking these things into account, my own experience, for what its worth was this -

First 5 years - multiple relapses which were nowhere near as bad as the initial symptoms because the close monitoring allowed quick treatment. I also lost my kidneys, but I was still able to work with quite a lot of time off sick.

Next 15 years - a fairly normal life with the aid of a transplant and lots of pills. Some minor issues with infections and drug side effects, but I held down a good job and raised a family.

From then until now - the side effects start to catch up with me and become more serious. Prednisolone is by far the worst! I eventually had to retire from work (I had ensured that I had a good pension and my finances were sound) and continued to deteriorate. I am now using a wheelchair for much of the time.

Please don't take on board that your story will follow this pattern, I can guarantee that it won't. Just be prepared to change direction when the Wegener's decides to do something unexpected and hope that you are one of those who maintain drug free good health. :thumbsup:

[delorisdoe]

I am going to respond to your comment "now that you know the symptoms". This does not make it easier unfortunately. The symptoms are often those of a cold or sinus infection etc. I spend 3/4 of my day trying to decide if it is the wg or if it is a cold. should I mention it or not. I spent most of december with undiagnosed pneumonia because I knew I had a specialist appointment early january and that my cold would be gone or it would be the wg. I suffered alot for nothing as it was pnuemonia. It does not get easier. not for me anyway.

leigh

[JanW]

I think it's all over the map. I was dx in January 2010, have been medicated since March 2010 and am now attempting to reduce my mtx to see if I can maintain drug free remission. If not, I will likely move to rituxan, since I need to be largely medication free to have my saddle nose repaired. Of course, if I come out of remission, one of the things that can happen is that my nose can collapse again (they replace it with your own cartilage since it would be unwise to put foreign substances in a Weggie) so there's that.

I was not very sick at diagnosis (could still function, work, not in hospital, mainly had mobility issues which led to the dx -- severe ankle pain), and really have been pretty well since being treated. What I heard at the the Vasculitis Symposium is that the figures you see on the internet about 50 percent of patients having at least one relapse or flare, is incorrect, in fact the researchers said that most people can expect to flare at least once. I met people who only flared once in 25 or 30 years though, so there's that. Unfortunately, the flares ranged from not very seriously and easily treated, to one woman who spent several days in a coma (but was still at the conference and was older -- at least mid 70s, and she had had the disease going back 30 years or so, so she was also really treated with all of the rough stuff -- ctx for many years, never off high dose pred, had never been seen at a vasculitis center -- so it was actually amazing that she was as healthy as she was).

[Pmarsh]

Many people on this site mention getting a WG specialist or going to a Vasculitis center. I currently see an ENT who seems very good. He says he has several patients with WG. I saw another ENT before him who immediated wanted to operate on my sinuses. This ENT wants to try other options first (like the Nelimed rinse). I like this approach. I also see a respirologist and a nephrologist (even though my kidneys were not affected). The nephrologist actually started my treatment. He came to see me the night I was admitted to hospital and arranged the transfer to another hospital where the could start plasmaphoresis immediately. I have been monitored by an Opthamologist and saw a Urologist after leaving the hospital. I think this team is doing a good job at the moment. Would anyone suggest anything different? I live in Vancouver (Can).

Thanks for your stories or advice. It is really appreciated.

[vdub]

Tomorrow, 21Apr, will be my 1st anniversary for removal of my pituitary and diagnosis of WG. I was 59, retired, and having fun. WG totally interrupted life for about 6 months, but slowly things started to even out. Life will never be the same, but I have leveled out and generally feel good from day-to-day. I have some limitations on me, like no visits to MX or other locations that don't have US/Canada quality medical care. Germany, France, England, Japan, Australia, NZ, are all ok and probably some places I'm not remembering at the moment. Africa is out. And I can't backpack very deep into the mountains like we used to do. But, I was getting to old for that anyway.

I get tired more easily, but my strength is slowly coming back. If I wanted to work, I am healthy enough to do so. I think I'm on the track to remission, but won't know for a few more months and I don't know if it will stick once they taper me off the mtx. At the moment, I'm on 25mg mtx, plus all the pit replacements and I feel great. My weight is back down to within 10 pounds of pre-diag.

[Sangye]

PMarsh, I have had great difficulty getting the Wegs under control and have had tons of complications and damage--quite the opposite of your experience. It's been 5 years since I was dx'ed and have not gotten much of my former life back. Like you I was extremely healthy and strong when this happened.

Like the others have said there is no predicting which way Wegs will go. Also, you've learned some of the symptoms. Wegs can flare in other areas and give new symptoms or mimic other illnesses. I had hemorrhaging lungs at diagnosis and thought I knew the symptoms well. Nope-- 3 years later I thought it was pneumonia and so did my Wegs specialist until I started coughing up blood. Wegs is often extremely sneaky.

Here's my best advice: Wegs is unpredictable. That can be crazy-making when things are going wrong. But it also means that you can be surprised by sudden improvements or problems that appear and disappear on their own. If things are going well today, then rejoice in that and don't look down the road. If they're not going well, then have faith that the unpredictability of Wegs often works in your favor and things can easily turn around.

[Sangye]

Also... as far as getting a Wegs specialist involved. Wegs can turn on a dime. I wish I could convey this to people. Mine literally went from fairly straightforward to "Holy smokes we have no idea what to do" in a matter of hours.

It might sound odd, but the best time to get a Wegs specialist is when things are going well. You might not be able to do it if you get into trouble. No matter how much you love your docs, no matter how well they've done up until now, it makes a huge difference to have the security of the docs who know Wegs better than anyone in the world. Travel to a center and become their patient. You will never regret it.

[JanW]

The key member I see missing on your team, PMarsh, is a rheumatologist. This person is (generally) here in the states, the 'quarterback' on your care, and mine acts quite like a primary, in referring me to other specialists with WG experience. My secondary doc is an ENT, but he would not prescribe immunosuppressants or much of anything else for me -- he would kick it back to the rheumy. My ENT treats me surgically -- I have had surgery on my windpipe, and will have it on my own. He is also the person who reports back to my rheumy that there are no signs of active disease in my throat or sinuses -- my rheumy doesn't even look at these areas (I pretty much have the top WG ENT in the country in my case -- many people here and other places have been to him). I try to see them in the same week -- about every six weeks to two months now.

[Jack]

If you are still being bothered by sinus issues, don't even consider surgery unless you have explored every other option. Most sinus problems should subside to manageable levels once the Wegener's has been brought under control and surgery could result in serious complications.

I had terrible trouble with my nose and sinuses before diagnosis and underwent a great deal of involved and extensive surgery, but since hitting the Wegener's on the head it has been little more than just a runny nose.

[Daggar]

Hello PMarsh -- sorry to hear about your illness.

You were diagnosed a month prior to our daughter Holly (nov 2010). She had severe kidney involvement at the time of diagnosis but has recovered fairly well. Most of her current complications are due to treatment moreso than WG.

As for health care.... it sounds like the Nephrologist took control early which is great! As JanW said a "rheumatologist" is usually a big part of the medical team -- especially if you have no kidney involvement. Your other doctors are pretty much the same that Holly has (ie Opthamologist and Urologist). I'm not sure how the Canadian system works for adults with WG (Phil or Marta would be best in that regard) but for kids it becomes a team approach where they have regular clinics to go over disease activity, test results, treatment side effects and any changes in their original plan/schedule. The Nephrologist/Rheumatologist team at the Alberta Children's Hospital treat 6-8 kids per year with WG but there are a number of other Vasculitis disorders as well.

It appears as though you did the 6 month cyclo which would indicate that you progressed well from initial treatment . Holly is due to switch to Imuran in May/June if she continues to progress as she has without any WG activity and negative lab results.

How long have you been on the Imuran and did you notice any difficulty making the switch? Did they increase your lab work frequency while changing the meds?

[Pmarsh]

Hi Daggar. Yes, I did the 6 cyclo treatments. My last one was at the end of March. I started Imuran a week or so after the last treatment. So far no issues with the change in meds. I get blood work done every 2 weeks, and fortunately I get to see the results almost immediately after. My blood work has been all normal for a couple months now. I understand they do add a few tests to the usual work when you go on Imuran because 20% of people don't tolerate it.

Glad to hear your daughter is making a speedy recover. I went from the Hospital in early November, to limping around in December, to full time work in January. Now I'm trying to get back into shape (skiing, running, and biking) and looking forward to a more typical summer.

I see my Nephrologist today, so I will ask about a Rheumatologist.

[Daggar]

Sounds like you're doing good .... the blood work is a good indicator of how you're doing but from what I've been told it can also be late in picking up issues. Just be aware of anything unusual (sinus, lung, joint, urine, etc) - especially since you are active -- it may mask itself as a muscle/joint strain from activity. All the more reason to have a Rheumy involved! Keep up with the lab work even though you may feel great -- it could save you a lot of grief! Keep that Neprhologist in your corner -- he may prove invaluable if anything does pop up.

Hope it is well under control!!

and... I never thought I'd ever say this ... go Canucks!!

[Pmarsh]

Yeah thats what I like to hear. Go Canucks Go! We just wanted to finish 'em off at home...

[delorisdoe]

Caucks? Is that hockey or baseball?

[pberggren1]

Caucks? Is that hockey or baseball?

I think it is football or soccer. LOL

[JanW]

I think what the rheumy is also helpful for is if, for instance, you suddenly break out in a bad rash (as I did) he can examine you and pretty much tell you that it's not from your meds (which, your neph or pulmo would be unlikely to do -- at least here, anyway, because they don't really deal with the whole body that way) and then immediately refer you to a derm who has probably seen some WG before for treatment. Also, like Daggar says, as a very active person, you could put a lot of things down to pain from overactivity, and the rheumy is likely to ask some leading questions as well as perform simple tests that might show things are more inflammatory in nature.

[delorisdoe]

I think what the rheumy is also helpful for is if, for instance, you suddenly break out in a bad rash (as I did) he can examine you and pretty much tell you that it's not from your meds (which, your neph or pulmo would be unlikely to do -- at least here, anyway, because they don't really deal with the whole body that way) and then immediately refer you to a derm who has probably seen some WG before for treatment. Also, like Daggar says, as a very active person, you could put a lot of things down to pain from overactivity, and the rheumy is likely to ask some leading questions as well as perform simple tests that might show things are more inflammatory in nature.

I just don't think it is so cut and dry. It was a pulanologist who took one look at the rash on my legs and frst tested for wegners because of the rash.

[delorisdoe]

I think it is football or soccer. LOL

For our european friends these are the same sport

[JanW]

Not saying it is cut and dried, but here, most doctors really rely on the referral system, and aren't quick to go outside of their specialties. Also rheumys dealing with autoimmune diseases may be more common here than in other countries I don't know. It's pretty standard here that if you have anything from RA, to WG, to Lupus, that who your primary doc would be.

[pberggren1]

For our european friends these are the same sport

Technically not! I was just joking before, Cannucks are a hockey team.

[delorisdoe]

Of course. There does seem to be differences between countries and in canada even between provinces. The rhumatologist sent me back to the pulminologist as soon as I was diagnosed

[pberggren1]

I am treated by a Nephrologist and have no plans of leaving him because of his vast experience with Wegs and other Vasculitides.

[Daggar]

......It's pretty standard here that if you have anything from RA, to WG, to Lupus, that's (Rheumy) who your primary doc would be.

That's the protocol at the Children's Hospital in Calgary as well. Holly received a very detailed resource binder after diagnosis and that did come from Rheumatology. It appears WG falls under the Rheumatology umbrella for the most part except when there is risk to organs and then they transfer the patient to the proper clinic (ie Nephrology - kidney or Pulmonology - lungs). They still oversee the WG concerns/treatments but refer back to the respective clinic for any threat to the organs involved.

[pberggren1]

My last Rheumy seemed to be a little put off by me going with this new doc that is a Nephrologist and has much more experience with Wegs.

[Daggar]

My last Rheumy seemed to be a little put off by me going with this new doc that is a Nephrologist and has much more experience with Wegs.

Too bad you couldn't remind him of how you're "put off" fighting the disease and treatments every day. He may of learned something from the Nephrologist that could of helped future patients.

Happy Easter Phil......

[Pmarsh]

Interesting discussion - bot about hockey, football and soccer, and various approaches with doctors. I saw my Nephrologist today as he was basically transferring my primary care to my Respirologist. My kidneys were not affected, but he will continue to monitor my blood work. He didn't think there was any need to see a Rheumy as well. All of the MD's on "my team" seem to have a good amount of experience with WG.

Here's another question... Has anyone who only had sinus or lung issues ever flared up with kidney issues? Or has anyone with "limited" WG ever flared to full blown WG? Not sure exactly how to state that question?

[vdub]

My rheumy (the specialist) said he didn't like the term "limited". He said that was an old european term that really didn't convery the right idea. He said he preferred to describe "limited" as "localized", meaning that it is only attacking a specific organ.

When does it move from "localized" to general [my term]? I think it will vary greatly from one person to another. WG can be so different that I think it would be very difficult to say.

[delorisdoe]

Interesting discussion - bot about hockey, football and soccer, and various approaches with doctors. I saw my Nephrologist today as he was basically transferring my primary care to my Respirologist. My kidneys were not affected, but he will continue to monitor my blood work. He didn't think there was any need to see a Rheumy as well. All of the MD's on "my team" seem to have a good amount of experience with WG.

Here's another question... Has anyone who only had sinus or lung issues ever flared up with kidney issues? Or has anyone with "limited" WG ever flared to full blown WG? Not sure exactly how to state that question?

it seems to be going the other way for me. I have only ever had lung issues, severe lung issues at times but never any other involvment. Now it seems to be heading up that way. I picture these little furry wg creatures chomping their way up from my lungs to my sinuses.

[elephant]

The wegener hit my kidneys first, then 30 years later it hit my sinuses/lungs and other damages that I can live with.