Why I am grateful to have Wegener's

[Minneapolismark]

Still 75% mortality at ten years...

[JanW]

It is incurable but I would bet that the rate of death from true WG (ex co-morbidities), is probably in the less than 15 percent range, and that there are comparable numbers in the 'single flare and never again' category. Both are fairly unusual to encounter.

What I've never been able to suss out is the number of people actually disabled by the disease. That might be a scarier, larger number.

It's actually a little worrisome to me that your doc referred to it that way (although, I know, you said he's a sweetheart of a guy). That's just not the modern thinking at all. Expert docs think that with good, effective and (probably) quick treatment, this is a manageable, chronic illness. They routinely refer to patients who have gone on to have children, live normal lives, etc., etc. -- all while being seen a few times a year or occasionally having a surgery. Even when my doc was showing pictures of people who had suffered the ill effects of pred and ctx (in the context of showing people for whom he had corrected a saddle nose) they were 15 year old pics and those people were very much alive and doing well.

[Minneapolismark]

But the Journal of Rheumatology still puts mortality at 25% at five years and 75% at ten years. (please see my posts under "Wegener's Statistics.")

Whether mortality is from the disease or from co-morbidity seems moot to me. This is important to acknowledge for several reasons:
1. although we don't want to scare anybody, this is NOT a disease you can take lightly.
2. just because you can possibly struggle back to work does NOT mean you should do so. Resting isnt just a convenience it is a necessity.
3. getting the right physician is critical. Yes I know I need to heed my own advice on this, and am doing so.
4. don't ignore symptoms or other illnesses that pop up
5. own your own disease and its treatment
6. be realistic about planning your day, your week, your month and your future

7. the seriousness of the disease may convince the IRS, your creditors, your insurance company and other organizations to assist you more than you would imagine. This has been true for me. The IRS was super helpful when they realized how serious my situation was.

I don't plan to die from this, at least not anytime soon. At the same time I can't take my eye off my recovery either.

[Minneapolismark]

At the same time fear is useless. The fact is that if you are on this list then you are probably taking your disease seriously. In the healthcare industry (my former career) we categorized patients by level of engagement. This meant the degree to which they interacted, learned, participated and drove their treatment and recovery. Level 5 (the highest level of engagement) patients do very well. Level 1 (lowest engagement) patients do very poorly.

So if you are on this list I am willing to bet you are NOT one of the people who will die from this or its co-morbidity diseases!!!!!!!!

On the other hand patients are way more likely to become fully engaged if they understand whatever facts are available and face the seriousness of their disease. Patients in denial are way less likely to engage at level 5 and much more likely to die.

Now please understand that I am not saying anyone here is low engagement. Quite the contrary.

But much of my strategic planning work was aimed at changing healthcare to more fully engage the patient in the care process. And now I have a perfect test of my own convictions.

BE A LEVEL 5

[JanW]

What year are those stats from? I still think 25% in five years is high, particularly if someone is diagnosed in midlife or younger.

[Sangye]

There is no way the 75% mortality at 10 years is true. The VF docs don't treat Wegs as a death sentence, as regular rheumys often do. (When I got to JHU Dr Seo had to drive it into my head that it wasn't a death sentence, as I had been told.)

I will say that every single death in this group this year was of a patient who did not have a Wegs specialist and was not receiving adequate care.

Whether someone dies from Wegs or a co-morbidity is HUGE as it indicates very different efforts need to be made in managing the disease. For example, because so many Weggies die from infection and cancer and not Wegs, the Wegs specialists are learning to get patients off immunosuppressants as soon as possible. This is an enormous change in protocol. Most of Dr Seo's treatment decisions for me are made based on the fact that he considers me (47) young, and doesn't want me on these drugs for the next 40 years.

The only death sentence with Wegs is not being treated when necessary.

[Minneapolismark]

Could well be. 2006 was the date of the article. I have been unable to find more recent stats.

Doctors who are not "in the know" about Wegener's will tend to GREATLY under-estimate the seriousness of the disease, the criticality of the treatment protocol and the need to pay attention to random symptoms and details. In short, the uninitiated doctor will not tend to take the Weggie seriously, and this could very easily prove fatal. I do know that mortality is closely associated with the competence of the care provider more than anything else. Number two would probably be the engagement of the patient in the treatment process. FACTS are our best weapon against ignorance. Stats from a prestigious journal are a great tool in sharpening the attention of our care providers. If someone has different stats from a doctor then by all means share them. For right now these are the best documented stats I can find.

Again I am willing to bet that the degree of engagement on the part of the patient strongly influences outcomes. engagement is usually driven by a fair assessment of the seriousness of the situation. Not exaggerated but informed

One of the respondents to the survey on this site listed their only Weggies medication as an anti-biotic. If I was that person I'd want to know that it is imperative that I immediately get to a competent doctor and find out if that is an adequate protocol.

[JanW]

As to someone being only on an antibiotic, that person could be on bactrim as some kind of maintenance dose for sinus issues and in fact be off all immunosuppression. S/he could certainly be being adequately treated because s/he is in remission. Or, if I had answered the questionnaire while I was waiting for surgery, I would have answered "none" to the medication question, because my rheumotologist didn't want to start immunosuppression prior to surgery (I had had issues with liver enzyme issues on a test dose of mtx). Yet I was still being adequately treated.

I hope that I can answer a similar questionnaire within a year with the answer: none. Because while I will always be a weggie, I will also always hope for a medication free remission. As Dr. Seo would say, I do deserve a shot at that.

[JanW]

To your other point, I think that docs not in the know would tend to in fact UNDERTREAT the disease while simultaneously OVERESTIMATING the destruction that the disease must enact upon the patient. So you get docs who really do believe that the disease is a death sentence giving people tons of pred and keeping them on ctx for a year and yet not really understanding that the right protocol will likely get the person into remission. I met people at the Symposium who had been fired by their doctors for complaining about things like debilitating joint pain because, essentially that was to be expected with this horrible disease. People that were called whiners because they were going deaf and that's just what "wegener's does." In fact, Dr. Merkel of Boston said that even by experts, he believes loss of hearing has not been taken as seriously as it should be because in the scheme of things that could happen, it's not the worst. Funny, the one thing that I found that everyone took seriously was the saddle nose (you wouldn't believe how often patients will have an unknowing ENT offer to 'fix that.'). My theory is because looking at that nose really bothers THEM. Other than loss of self-image/esteem, it's rarely the worse of a weggies problems (particularly lots of us also have windpipe issues).

[Minneapolismark]

I do think we are in violent agreement! 25% at five years and 75% at ten is from a variety of sources, most notably the Journal of Rheumatology. This stat is four years old and includes everyone everywhere, including those treated by ineffective doctors.

Though no stats are presently known, we have good reason to believe that effective specialist care, an appropriate life style, and staying actively engaged in the therapeutic process means we are probably going to live a long time.

But still the stats are what they are.

I would be OVERJOYED to have someone offer better and more recent statistics, but until then these are the best concrete facts i have on mortality.