Not sure if I've got WG, but very concerned

[heartspun]

Hello, my name is Linnea and I am 35 years old, married with two kids, living in the SF Bay Area (California). I have been having a series of mysterious and seemingly unconnected medical issues for several years now, and I have begun to suspect that I may have Wegener's. I would love dearly to be incorrect - and my rheumy (whom I see for inflammatory arthritis, and whom I do not care for a great deal) says she "highly doubts" that I could have WG. All the same, she ordered the ANCA test for me and we should get the results of that early next week.

Here's a basic run-down of my issues that cause me to suspect WG:

2002 - Kidney attack. Possibly passed a stone; blood visible in urine.
2004 - 1st child born
2005 - joint pain flare-up, bladder infection
2006 - 2nd child born
2009 - severe joint pain flare; persistent cough (over 3 months); kidney colic; blood in urine; sinus congestion w/ clear runny nose.
2010 - kidney colic, blood visible in urine; terrible sinus infection, fever; kidney colic, blood in urine AGAIN; x-ray with "tiny pleural effusions".
2011 - "ear infection" - did not resolve with antibiotics - persisted 1 month; sinus infection with bloody nose; joint pain flare; second ear infection

Currently I am still experiencing the "ear infection" - have been on amoxicillin and ear drops for a week and have had absolutely no improvement in condition. There is little pain, but profound hearing loss, tinnitus, feeling of fullness on entire left side of head, some tingling and numbness in sinus area on left side. This is identical to the earlier "ear infection" except for the numbness and tingling on my face - this is new.

Since approx. 2007, whenever I get a seasonal respiratory infection, I *always* lose my voice, and experience a persistent deep cough that lasts weeks if not months. In addition, since that time, every urinalysis tests positive for blood (but never protein). I underwent a cystoscopy to look for causes, and the urologist found nothing, stating "some people just always have blood in their urine". Repeated episodes of kidney colic have never produced a stone.

I'm currently waiting on the ANCA results and a referral to an ENT specialist.

Any thoughts or advice?

[Psyborg]

I don't think they should rule out wg, but even an Anca test is not 100% guaranteed to come back positive. I would suggest attempting to see a specialist with Vasculitis who will likely be more knowledgable than your rheumy. Especially given you don't sound likebyou see eye to eye.

[Sangye]

I agree with Bob that a visit to a major center would be a good idea. I'm wondering why they haven't done a kidney biopsy yet. Surely there is sufficient reason.

[heartspun]

I will be requesting a referral to the UCSF Vasculitis Clinic after the ANCA test results come in... I guess until then, there's nothing to do but fret!

I never got a kidney biopsy because I had to change health insurance providers - and my new health care team is more concerned about the arthritis and less about the kidney/blood in urine problems I had been having previously. I gave them a copy of all my records, but I swear they didn't even read through them... I think I need to write up a "medical resume" to help them see the overall picture.

Just got my ENT appointment for Tuesday. Fingers crossed that I don't get brushed off.

[Sangye]

It will help you to write up a brief summary of your medical history just as you did above. Bring that to each doctor appointment. Ask them to look at it from a wider view instead of focusing on this symptom or that. Doctors should do that, but often don't. An ENT is likely only to focus on your ENT symptoms, but even there you can emphasize the larger picture.

[Psyborg]

I would suggest going to UCSF regardless of the results of Anca. Your symptoms certainly sound suspicious.

[norcalian]

Hey Heartspun...where are you seeing your rheumy? Dr. Chen at UCSF is the one that is suggested by the Vasculitis Foundation. There is also a guy from Stanford on the list...I don't remember his name. I was recently diagnosed with WG...so I have been meaning to get in to see Dr. Chen...but I have Kaiser and they will not pay for the consultation. So I'm not sure when/if I will get in to see her. Although it sounds like I have a bit more faith in my rheumy than you do...so I'm not in a huge hurry (especially since my treatments are coming along pretty well). I hope you get this figured out soon...and as you will hear on this forum, the sooner the better. WG can get really nasty really fast.

[drz]

All your symptoms listed are consistent with having Wegs (now GPA) but it would help to have biopsy that could confirm it but biopsies are not !00% either unless the organ biopsied is infected. Unfortunately neither are ANCA tests !00% in yielding a diagnosis. Best case is a good review of everything by a Weg specialist who knows what to look for and how the symptoms are relate.

Good luck in sorting it out. Was reading Cindy's book today and she said her symptoms were present for over a decade before she got correctly diagnosed. Many of us went several years and can share your frustration and uncertainty. At least you now have Wegs on the radar which should help get it confirmed if present. Most of us never heard of it before our diagnosis.

[elephant]

Your symptoms sound like Wegeners, but you really need like what everyone said a Wegeners specialist soon, before you lose your hearing or other damage to your organs. Wegs can go in any organ and cause trouble, rare but it can. You are very proactive right now, just don't let your rheumatologist think there is nothing wrong. Push back and ask questions...or ask her to call one of the top wegeners specialist in the US. Good luck and keep us posted.

[vdub]

Most of us never heard of it before our diagnosis.
I'm kind of amazed that you have done enough research to come up with our obscure little disease. As everyone else has said "sounds suspicious, see a specialist".

[heartspun]

Thanks to all who have responded. I am currently being seen at Palo Alto Medical Foundation, right across the street from Stanford. Before that, I was at Kaiser. That is where I had the urologist shrug and tell me, "some people just always have blood in their urine". That never sat right with me, but I put my concerns aside after a while because the inflammatory arthritis had become especially troublesome. When these ear infections became a pattern, I started to wonder if they could be caused by an autoimmune issue as well, so I did a little searching and found the story of a WG patient which rang so many alarm bells, I was shocked. I had never heard of Wegener's before, except perhaps on an episode of House, M.D.! I'm still in a bit of shock at what the implications are if this is truly what I have... But I'm really glad to find this group and all of the information here.

[sue7211]

The vasculitis specialist (listed by the Vasculitis Foundation) in the rheumatology department at UCSF is Sharon Chung, MD. My son has been her patient for the last year. She is thorough, straight forward and pleasant. I would run, not walk, to set up a consultation with her now. Don't wait any longer as you might have to wait a while before you get to see her. My son had so many symptoms in various parts of his body that he had seen several specialists and no one put things together. His rheumatologist finally ordered the ANCA test 5 days before he entered the hospital in acute kidney failure. I feel that if one doctor had looked at the overall picture they might have figured out the puzzle but I agree when they just focus on one part of the body they miss it. In my son's case his symptoms kept migrating from one organ system to another. My best wishes for you!

[Sangye]

Thanks to all who have responded. I am currently being seen at Palo Alto Medical Foundation, right across the street from Stanford. Before that, I was at Kaiser. That is where I had the urologist shrug and tell me, "some people just always have blood in their urine".
That doesn't surprise me. Kaiser has had one of the worst reputations in medicine for a very long time.

[heartspun]

That doesn't surprise me. Kaiser has had one of the worst reputations in medicine for a very long time.

LOL! Richly deserved... I grew up in the Kaiser system and the best thing I can say about them is that they hand out Vicodin like after-dinner mints. :P

Very anxious about test results which should be arriving tomorrow or Tuesday. I've put in an appointment request with Dr. Chung at the UCSF Vasculitis Clinic as well. Not sure if my request will get shunted to the bottom of the pile since it's not an official referral, but I feel like I have to do *something* and otherwise have little control over the situation! :sad:

[heartspun]

Well! I wasn't expecting test results via email on a Sunday night, but the ANCA is negative. Not really sure what this means - will have to read more in the A.M.

[Sangye]

It doesn't mean much. Some people with definite Wegs continue to have a negative ANCA.

What are the results for
1) ESR/ Sedimentation Rate
2) CRP/ C-Reactive Protein

[heartspun]

Sangye, my ESR and SED are almost always normal, even in the middle of a bad arthritis flare-up! I am starting to think that perhaps the pod people came and switched my body out for one with normal labs? Actually, I just got my MRI results that do show inflammation in my sacral joints - the rheumatologist would like to trial me on Humira for that, since the NSAIDs have been chewing up my stomach. I'm going to see an ENT tomorrow about the ear, and hopefully will hear back from Dr. Chung this week.

Thanks for all the good advice here. I desperately need it at a time of great uncertainty!

[Sangye]

The pod people took my body, too! LOL

I have the same problem with normal labs. But thankfully my ANCA was off the charts when I was dx'ed, and I had a (+) biopsy on the first sample taken. I was very fortunate.

It could be a problem to do Humira before seeing Dr Chung. It isn't effective for Wegs and might interfere with a treatment she wants to do. Once you do Humira it continues having an effect for some time-- months, I believe.

[sue7211]

If they require a referral you might not get a response from Dr. Chung. I believe we had to get a referral in order to get a second opinion from her.

Call her office and ask what the status of your appointment is.

Keep plodding away it usually works. If you do have GPA (Wegener's) Humera would not be the correct drug to be on.

[heartspun]

Hmm. Any advice on what to say/how to approach the topic with the ENT doctor tomorrow? I have never seen this doctor before. I know that ear problems are not uncommon with WG, but I'm afraid he's going to think I'm crazy if I bring it up.

[Sangye]

You can explain that you have a variety of symptoms that no one seems to be able to diagnose. Out of frustration, you've done some research on your own and discovered that many of your signs and symptoms point to Wegs as a possibility.

Hard to argue with that, I think.

[heartspun]

Perhaps it will "help" that my other ear seems to be going the way of the first!

Sangye, that is very wise and skillful. Thank you for your advice.

[Sangye]

LOL Thank you-- it took me a long time to be able to communicate well with my doctors. :wink1:

[pberggren1]

LOL Thank you-- it took me a long time to be able to communicate well with my doctors. :wink1:

I think I communicated well with my docs from day 1. It is just that most of my docs did not communicate well with me. But now I have a really awesome Wegs doc that knows how to communicate properly.

[norcalian]

If they require a referral you might not get a response from Dr. Chung. I believe we had to get a referral in order to get a second opinion from her.

Call her office and ask what the status of your appointment is.

Keep plodding away it usually works. If you do have GPA (Wegener's) Humera would not be the correct drug to be on.

I spoke with Dr. Chung on the phone a couple of months ago. She told me that she needed a faxed request either from my HMO or from myself (if my HMO is unwilling to refer - which in my case they are). She said that in either case she would need a copy of my records...so if your HMO is unwilling to refer or pay for a consultation, you will still need to get a copy of your medical records to see her.

There is also Dr. Weyend who is also recommended by the Vascultiis Foundation. He is at Stanford...which might be in your network (since I believe you said that you were in the Palo Alto Group). He's suppose to be pretty good...I know my rheumy consults with him regularly.

[heartspun]

There is also Dr. Weyend who is also recommended by the Vascultiis Foundation. He is at Stanford...which might be in your network (since I believe you said that you were in the Palo Alto Group). He's suppose to be pretty good...I know my rheumy consults with him regularly.

Thank you for this, Norcalian! I looked up Dr. Weyend and she (!) is in my health insurance providers list. Hooray! I will be contacting her office later today.

Off to have my ears prodded at... hope this goes well! I've never seen an ENT before. Do they really wear those round reflector thingies on their heads? :biggrin1:

[delorisdoe]

yes they do

[heartspun]

Well, my ear popped open and drained a bunch of fluid last night, and now I can hear again! Yay for that. The ENT guy says that it's not bacterial or viral from what he can tell, and after scoping me (oh boy was that fun) he sees nothing unusual. He did say that my eardrum had ruptured but is healing itself.

He wants me to do inhalable steroids daily for three months. He also said that it could be related to my auto-immune issues, but he basically told me to go back to my rheumy for that. So, medical ping-pong! Fun! I'll put in a call to the Stanford doc tomorrow.

[elephant]

I agree with your ENT, so glad you can hear. Did you have a CT scan of your sinuses or mastoids ( by your ear)? When my ears felt full and I could hardly hear, my mastoid cells were filled with fluid and my sinuses were swollen. Glad you are calling Stanford. Hope you get in soon.

[norcalian]

Thank you for this, Norcalian! I looked up Dr. Weyend and she (!) is in my health insurance providers list.

ooops...sorry, I guess I assumed Dr. Weyend was a dude...my bad :blushing:

I'm glad she's in your network though...what a lucky break!

I'm glad things went relatively well with your ENT and that you can hear again. I started with ear problems right before my Wegs went into full effect (lungs, kidneys, etc.). I was given some prednisone which cleared up the hearing problem and probably saved my hearing since the Wegs was causing damage to my nerves. But after I stopped the prednisone for my ears the fevers started...

Good luck with your diagnosis. Hopefully Dr. Weyend can help figure things out for you!

[sue7211]

Norcalian can you please let me know publicly or privately who your rheumatologist is? I need one not for Wegener's but for other issues and I live in Berkeley. Thanks!

[heartspun]

So, both of the Weg's specialists in my area require a referral, and my primary care doctor is on sabbatical until the end of next week. Fooey. I haven't asked my rheumy for a referral, because I'm really not sure she'd be willing to do it. Also, I'm just plain tired of doctors at this point. :flapper:

Ear is still painful/congested/oozing... ENT guy put me on inhaled steroids and said to come back in three months. Rheumy put me on a Medrol dosepack, which has gotten my arthritis inflammation under control. Aaaand, the kicker is, my husband just accepted a new job, which means our current insurance is only good until the end of the month and then we have a week gap with no coverage, and we don't know yet which doctors are accepted under the new plan. Rargh!!

[Sangye]

Can't you get COBRA coverage for that week?

[heartspun]

Can't you get COBRA coverage for that week?

Yes, I am sure that we will be able to buy gap coverage for that time. I'm just not looking forward to learning a whole new set of ropes with a different insurance provider. Hopefully I will be able to get my referral from my primary doc next week and then just have to choose the best plan option that will allow me to actually go SEE the specialist!

I have been wondering, if they need to take a biopsy to look for signs of Weg's, how does that work if it's primarily affecting your ears/sinuses? I suspect that a kidney biopsy is in my future, if/when the kidney issues flare up again.

[Sangye]

I hear you on the insurance. Very scary.

Sinus biopsies are famously inaccurate. It's hard to get a good sized sample and there are lots of false (-). If you don't have involvement in other places, they might have to make the diagnosis based on labs, other diagnostic tests and signs/symptoms. That's fairly common.

[elephant]

Hey I had Four maxiallry sinus surgeries/biopsies that showed "NADA" pertaining to finding WG. The docs confirmed WG in a open lung ( took a chunck out of my lung) to comfirm WG, which we knew I had before the lung biopsy.

[mama2005]

My ANAC tests always show negative but the ENT did two sinus biopsys to figure out that I had WG.

[Chadwyck]

Hello heartspun. Welcome to the forum. This is my first day able to use the forum as I am new here as well. After reading your post, it reminded me of me saying the exact same thing 2 years ago. As for the ACNA test, I have done about 8 of them from it was suspected until confirmed Wegeners. Every one of those ANCA tests came back negative! But, it isn t sufficient to rule out Wegeners from what I was told. By the time I got the 9th c-ANCA test done, I had the nasal biopsy done. Both test results came back one day apart from each other. The ANCA result showed I did not have Wegeners and the biopsy proved I did had Wegeners!!! So please don t let a negative c-ANCA test give you the wrong idea that you are ok. Hope this helps.

[Al]

Yes, I am sure that we will be able to buy gap coverage for that time. I'm just not looking forward to learning a whole new set of ropes with a different insurance provider. Hopefully I will be able to get my referral from my primary doc next week and then just have to choose the best plan option that will allow me to actually go SEE the specialist!

I have been wondering, if they need to take a biopsy to look for signs of Weg's, how does that work if it's primarily affecting your ears/sinuses? I suspect that a kidney biopsy is in my future, if/when the kidney issues flare up again.

A few notes: 1) In the US, access to COBRA plans is required. 2) Your primary must give a referral if you need one. Not to do so would be a breach of professional ethics, though he or she may not give the specific referral you want. 3) Kidney biopsies are not exactly a barrel of laughs, but they are relatively accurate, without a lot of false positives or negatives. In the sinuses and ears, they usually look for granulomas, but there is a lot of art to this.

Al

[drz]

A few notes: 1) In the US, access to COBRA plans is required. 2) Your primary must give a referral if you need one. Not to do so would be a breach of professional ethics, though he or she may not give the specific referral you want. 3) Kidney biopsies are not exactly a barrel of laughs, but they are relatively accurate, without a lot of false positives or negatives. In the sinuses and ears, they usually look for granulomas, but there is a lot of art to this.

Al

I remember my kidney biopsy was relatively painless and I slept through most of it. When I woke and I asked them how it was coming along they said it was done. I have felt more discomfort from a having a tooth filled. I think like all medical procedures it depends mainly upon upon the skill of the practitioner, like blood draws, transfusions etc are relatively painless when done by a skilled person but a painful experience when done poorly.

My kidney biopsy was "full of Wegener's" so no questions about my diagnosis along with extremely high ANCA score.

[Al]

I remember my kidney biopsy was relatively painless and I slept through most of it. When I woke and I asked them how it was coming along they said it was done.

Good story. My whole deal is to get the medical people off script. I had the radiologist laughing so hard she nearly missed her mark. For me this was the simple part. Worse was being required to be tied to the bed for the next 15 hours or so. Along about half way through that purgatory, they gave me a roommate--"Poor Vern", who had had prostate surgery and had a mis-installed catheter. He was in serious pain, but it turns out the surgeon was playing tennis and couldn't be found. Then Poor Vern lost his call button. I called into the nurse's station on his behalf, announcing myself as his "cellmate". Poor Vern and I became good friends. Anyway, it was fantastic to be allowed to get up and walk around a bit about 2:00 in the morning....

Al

[maria garcia]

Vern my aughters Biopsy has taken two weeks and yet nothing Maybe her pathologist is also playing tennis. I have to laugh if not I will definetly CRY!

[heartspun]

Thank you all, for the replies and especially urging me to not let this go - it's so easy for me to convince myself that I am "not sick enough" even though I have no hearing in my left ear. It seems as if I won't get any serious medical attention unless I wind up in the hospital. I spend every day ridden with fear and anxiety about what horrible thing might happen to my body any minute now, or perhaps I will just be this way for the rest of my life. It's a bad place, not liking this at all. :(

[Chris G]

Heartspun - What kind of progress have you made toward seeing a rheumatologist who specializes in wg? I did the whole ENT/Opthalmologist/rheumatologist circuit too (many of us did). Until you find a rheumy who knows wg well, you're not likely to get a diagnosis. They don't know enough about it, and will tell you that your symptoms don't fit, even when you know damn well they do! By the time you find this forum, you know more about wg than most garden variety rheumies & ENTs! Yes, a complete list of your past and present symptoms is needed. When you find the right doc, it will give him/her the background necessary to diagnose you.

Negative ANCA doesn't necssarily mean anything either. When I'm flaring my ANCA happens to be positive, but my ESR and CRP are usually normal.

The ear infections you're suffering can be very painful - I know them well. I think they can also be very dangerous. By the time I was diagnosed, my eardrum had erupted, my mastoid bone was full of fluid as well. It took over 10 days for the fluid to drain, and I was in agony for at least 4 of those days..... even with 3 different pain pills. Your ear infections were not viral or bacterial because (if they're similar to mine) they are caused by the eustachian tube being closed for so long. This causes a vaccuum inside the middle ear, which pulls fluid from the surrounding tissues. That fluid sits in there, continues to fill (overflows to the mastoid), and gets infected -or not - but the resulting pain and pressure can be unbearable. I lost much of the hearing in my right ear last year as well, but it returned to normal after about 3 months of treatment with antibiotics, methotrexate and pred.

You asked about nasal/sinus and ear biopsies. To my knowledge, they don't do ear biopsies. As Sangye said, sinus biopsies are notoriously unreliable. When I first saw the ENT (wg specialist), the inside of my nose was a mess, and he said he could see the typical "cobblestone" appearance of wg inside my nose. At that time, he confirmed my rheumatologists wg diagnoses, via that observation. Later when the rheumies asked for a sinus biopsy for absolute confirmation, I had been on mtx and pred for a couple of months, and my symptoms were much less obvious. The ENT told me he'd basically be taking (large) random samples, hoping to find something. No thanks. I refused.

The moral of this very long story, is that you MUST find a doc who specializes in wg. Regular rheumies, and ENT's will not put the pieces together for you.

[heartspun]

My primary care doctor, bless her, has agreed to submit an urgent referral for me as soon as my new insurance takes over, next Monday. It may still take a number of weeks to get in to see a specialist. There are two; one at UCSF and one at Stanford. I have as many of my records together as possible, including a sort of "timeline" that I wrote up which summarizes everything.

Chris - the ear issues sound identical. The fluid has leaked out and crusted up the outer ear, although that seems to be dwindling. Did/do you have problems with both ears? I think the twinges are starting in my right ear as well, but so far it's still clear.

I am so glad I found this board. :wub:

[Sangye]

Wow, I am SO relieved to hear that you're going to get to a Wegs specialist. Whew.

[Chris G]

My primary care doctor, bless her, has agreed to submit an urgent referral for me as soon as my new insurance takes over, next Monday. It may still take a number of weeks to get in to see a specialist. There are two; one at UCSF and one at Stanford. I have as many of my records together as possible, including a sort of "timeline" that I wrote up which summarizes everything.

Chris - the ear issues sound identical. The fluid has leaked out and crusted up the outer ear, although that seems to be dwindling. Did/do you have problems with both ears? I think the twinges are starting in my right ear as well, but so far it's still clear.

I am so glad I found this board. :wub:

Yes, I have had trouble with both ears. With my first known flare, around 07/08, my LEFT ear was affected, both eyes, sinuses, throat. And like you, the plugged ear has been one of my first and most reliable symptoms. In 08, the wg went to sleep on its own before I could find a proper doc, and get a diagnosis.

When I flared again in the spring of 2010, it was my RIGHT ear that was affected, along with throat, sinuses, and nose (no eyes this time) - oh and also pain on my sternum (I think it's my body's version of joint pain). So each time, it has been a little different - sounds like it has been for you too. Anyhow, last year's flare was the whopper that was needed in order for someone to realize that I do have wg, because the right side of my head damn near exploded, and my nose was a bloody mess (literally and figuratively!).

Were you ever on prednisone for any length of time? I think I read that you had tried a medrol dose pack. If you felt any improvement in your symptoms while taking pred or the medrol pack, it's important to include that in the information on your time line. I was given 2 medrol packs by my PCP last summer, and each time, my ear miraculously popped open within 2 days, and the second I finished the pack, the ear closed right back down again. It was a big clue pointing us in the right direction.

Oh, regarding that crusting in your outer ear......try to clean that out if you can - warm water and some q-tips (carefully!). For me it caused a lot of irritation, and eventually created an infection in the outer canal (sort of like swimmer's ear I think). Then I had to use the drops again to clear that up.