Ent visit

[onatreetop]

Went to the ENTand got my biopsy results no WG in the sinus or septum!!! That was a relief. Still a bit sore from the surgery 8 days ago. Also am going for fasting blood work this morning and than to the dentist. Have a bad tooth that is killing me that I have to take fixed. Other wise I am back to work and jumping all the hurtles that are in front of me. Nice to have the kids back to school after almost a week off. Weather. so I get to see the RA in about three weeks and see whats next.

[elephant]

Wow, that is good. How are you feeling these days?

[JanW]

That is indeed great news, Onatreetop! Onward and upward!

[Sangye]

When do you go back to JHU, Onatreetop?

Not to be a party-pooper, but biopsy results are not 100% accurate. It's not uncommon for them to come back (-) even when there's disease. I hope your (-) is accurate, though.

Also, it's a good idea to let JHU look at the pathology slides of the biopsy. I've known people who had clear path reports from one doc but experts found disease. It's not as clear-cut as we'd like it to be.

[JanW]

Wow. Learning something new everyday from Sangye. I thought that biopsy results actually were close to 100 percent, hence the term 'gold standard.' I thought that blood work, specificially ANCAs, had much less veracity, even in the presence of clinical disease, somewhere in the 90s, I believe I was told...

[Sangye]

I didn't know that about biopsies before I got Wegs, either. But with Wegs it happens all the time.

When I first got diagnosed my lungs were hemorrhaging-- had been for at least 3 months. My ANCA, Sed rate and CRP were off the chart and I had numerous signs/symptoms of active Wegs. My pulmy wanted to do a bronchoscopy for a biopsy sample. I was in critical condition and unlikely to survive any procedure like that. He said even if we did it, it was common to get a (-) biopsy because the tissue sample is so small.

The preferred biopsy with lung involvement is an open lung biopsy, where they take a big chunk o' lung.

I don't know the numbers for ANCA accuracy. It's fairly high for disease dx, but completely unreliable to chart disease progression and response to treatment.

[JanW]

Hmmm. I always thought that too small a sample would show up as a -- I don't know what -- not a negative but a "too little tissue to be analyzed" or something like that. Indeed, the ENT told me that all of the areas of his concern that have been affected by WG have much too little current inflammation to effectively biopsy, and the nodule in my lung is also too small as well. I definitely know that the rheumo said the preference is lung or kidney biopsy vs. throat or sinus biopsy.

He is actually treating a woman with WG (for over a decade) who has never had positive ANCA, CRP or ESR results except after a miscarriage, when her blood values shot sky high. They came back down a couple of month later. Ironically she was diagnosed with a sinus biopsy, after multiple severe nosebleeds.

How do the P3 numbers relate to all this in terms of another marker to confirm diagnosis without biopsy?

[Sangye]

That's an incredible case. My ESR and CRP don't go up until I'm ready for the ICU. That was a big reason for the delay in diagnosing this current flare.

As far as I know the PR3 is the same as the ANCA as far as accuracy.

[JanW]

On the other hand, my ESR and CRP are pretty high but I had no real painful systemic systems -- mine was doing all of its dirty work on the inside (and on my nose). Sorry for all of the questions, but, I know you've never been in remission, so I would have thought that you would always be having a 'flare.' I assume a flare for you is the worsening of symptoms you always have and the addition of new ones?

[Jack]

As far as biopsy being the Gold standard - I think it probably is when you get a +ve result, but a -ve result still leaves the door open.

[JanW]

That sounds like everything with Wegs! Negative ANCA doesn't mean you don't have it, etc., etc.!

[Sangye]

As far as biopsy being the Gold standard - I think it probably is when you get a +ve result, but a -ve result still leaves the door open.
Yes, that's my understanding as well.

[elephant]

I had four sinus surgeries/biopsies and non of them could say it was from Wegener's just a possible Wegener's. Taking the lung nodule out of my lung showed Wegener's. That was done doing video assist lung surgery. Ouch!

[Sangye]

That sounds like everything with Wegs! Negative ANCA doesn't mean you don't have it, etc., etc.!
That's right. I can't think of anything absolute about Wegs, other than if left untreated it's fatal.

And also that it totally sucks. There are much better diseases to choose from.

[JanW]

You know, I've always wondered about that Sangye -- is it always fatal in all cases if left untreated? Do we not know about cases of spontaneous remission because those people never seek treatment (maybe never even know they have Wegs?) There are certainly people whose cases remain limited and very dormant for long periods of time (that we know of), just as there are people who find themselves fighting with the disease all the time once they are aware of what it is.

Of course once you are aware, you have to monitor and treat as appropriate -- I would never try to suggest that anyone on here ignore this very serious condition, but, like the patient of my rheumy who has never been treated---not so much as a steroid or antibiotic-- in a decade (only followed by him), I'm wondering if there are any others that were never counted and eventually died of something else. It's like a have a hard time believing that 98 percent of "victims" are white -- I think it's more likely that other people aren't being counted, for a multitude of reasons.

[Sangye]

I know you've never been in remission, so I would have thought that you would always be having a 'flare.' I assume a flare for you is the worsening of symptoms you always have and the addition of new ones?
Sometimes with Wegs you can only tell what was going on in retrospect. I knew I was never in remission, or even close. My docs knew that, too. But we also knew it wasn't highly active (eg lung hemorrhage) for a couple years. I was sort of in moderately-active phase.

Last year it increasingly turned into full-blown active disease. It took a few months to crank up to that level. In retrospect we could see that the "flare" began in Feb/Mar even though major signs didn't show up until about May.

I now see that having 5 yrs of active disease has been a major part of why I haven't bounced back. (I was dx'ed in 2006 but had had active disease for at least a year by then) Active Wegs-- whether moderate or severe-- takes a huge amount of energy and a big toll on the body. No wonder I feel like poo. It's really just occurring to me lately, odd as that may seem.

[Sangye]

The JHU Vasculitis website says "Untreated Wegener's granulomatosis is fatal." That's all I've ever heard as well.

I think of people like Elephant who went for so many years without being diagnosed, even though she lost a kidney. The kidney transplant drugs happened to keep the Wegs from progressing to a fatal degree. So many Weggies with mild disease might be on pred for "asthma" or other immune-suppressants and could inadvertently be treating the Wegs. Considering how many Americans are on immune-suppressing drugs, it could be a likely explanation.

[JanW]

So I guess another way to describe it would be that sometimes you are in an acute phase, Sangye, and at other times you're just more in a chronic symptomatic phase. It's so horrible that you just can't seem to get cut a break, although I guess that there are others on here as well who have had active disease for some time...but it's clear that yours has just been really delibitating because you post about it. I'm sure that since the JHU docs think that they can get you to remission, though, you will!

[JanW]

I'm sure that once you hit the point where you are symptomatic, yes, you do have to put on drugs, and even if the docs guess wrong (like they did in my case), the class of drug was still somewhat treating the wegs. It would just be interesting -- like the way that they find that some people had cancers that never grew and they died of something else -- whether that is also true of very mild cases of wegs that never rose to the even seeking medical attention. Would be interesting to know if such a thing even exists.

[Sangye]

So I guess another way to describe it would be that sometimes you are in an acute phase, Sangye, and at other times you're just more in a chronic symptomatic phase.
Sort of. During the less active phase the Wegs was still progressing and doing damage. I developed lung nodules (never had them before) and nasal symptoms.

It's more like "Sneaky Active" vs "Obvious Active."

[Jack]

is it always fatal in all cases if left untreated? Do we not know about cases of spontaneous remission because those people never seek treatment (maybe never even know they have Wegs?)
The disease was first identified in 1936, but the treatment of it with the combination of Cylophosphamide and Prednisolone did not come until 1975. Before that time, all identified cases were fatal, mostly within two years, but I expect that diagnosis was restricted to some of the more obvious cases so undiagnosed remission may have existed.

[elephant]

I am sure there are cases around the world that people have died from Wegener's and no one new it. Some of them could of been diagnosed wrong ( ulcer's on the leg and have sinus problems) and then one thing leads to another and they die of complications of unknown origin.
My Aunt died at age 35, of a stroke. Young and pretty healthy except high blood pressure. There no family history of high blood pressure. Now I wonder if she was going into kidnye failure and all along had Wegeners disease or Vasculitis ( other). She was probably misdiagnosed too.

[Jack]

From the number of late diagnoses (almost the norm) I'm sure there are plenty of cases that are never identified and the victims just die. I was very nearly one of them!

[JanW]

That we can definitely agree on. Just wondering about the flip side -- mild cases that never develop into anything requiring treatment.

It remains a mystery until more research is done, I guess!

[Jack]

I think the problem will remain with the diagnosis until we have a simple test that can give a definite answer. What we need is a machine that can take a blood (or saliva?) sample and test it for every known disease.

[moyan]

Exactly my thinking, Jack. And when that is in effect, we will have an epidemic!! Such is my belief.

[Sangye]

Aw, takes all the fun out of the Big Surprise: "Guess what hideous disease you have?"

[onatreetop]

Go back when i decide to to JHU. Hellmann sent the results to all my docs including Soloway. We all get to decide what to do from here. I am feeling well................no different really. Still on the meds and now more. managed to get an absessed rootcanaled tooth on top of all of this. It was pulled today and now on my second week of anti biot. at 1700mg this time. Soit was a long day....fasting test for lab, then dentist to make appt for oral surg. then work for 5 hours, then pulled the @#$%^^&%$# out and got stiched up after an hour of pulling. And then drove home with one eye working because they gave me a really good shot that numbed the whole side of my face including my eye!!!!! I taped it such and drove home fine Thank god. Never ate today and can only do liquids for the night til morning. What a rollar coaster today. Decided to drink wine for dinner. Here come the carbs!!!! Foot is ok but not great. Face pain still present only eight days after surgery.I begged the oral surg. not to touch it the whole time today. he was good about it but used my lower jaw for levrage instead. ouch. Otherwise a bit touched or crazy laughing out load still when I am not trying to stay asleep. Napped off and on when I got home. Now i am awake. for a bit anyway.

I am happy, confused, bewildered, and moving forward snow or no snow. Have a lot of work to catch up on the next two weeks. So ................here I go!!!!!

[elephant]

Take care of yourself. You are a busy little bee. You on Amoxicillin? Five years ago I had a abscess tooth and I was on antibiotics for two whole months! It is painful. Hope you heal and feel better soon!

[onatreetop]

Thank you I will try. You all have a lot going on too! Yes its is amoxicillin that I am taking again. Now that the dead tooth is out hopefully it wont hurt so bad. Just sore from the yanking and tugging. I was waiting for the surg to put his foot on my chest to pull it. It was a stuburn one!!
I have two small houses today so it shouldn't be that bad. Thebig one is Friday. I havea feeling it might be an eight hour visit since I didnt go last week. job security with lazy people you know.

Hope you all hanging in there. I might get a brake from the doc visits for the first time in weeks. None scheduled for next week Yeah!!!!!! Then they start again the next week. Had to reschedule the second sleep study because it was to soon after the sinus surg. One of the gel packing tubes came out HOLY POOP!!! I couldnt beleive that thing was in my nose and still have one to go!!!!! It had to be an inch and a half long!!! Thought it was lung tissue or something else!! Scared the poop out of me. Have a good one! Post more later!!