hello from new member

[Pam C]

Hi. New member here.

I haven't been diagnosed yet, but have had night time nasal congestion, and dry nose for many years (30 maybe ) which led to probably unnecessary sinus surgery which started many years of frequent sinus infections. My ENT finally did some blood work 2 years ago and said my ANCA came back positive and referred me to a rheumatologist. ANCA has come back negative since then, but I had sub glottic stenosis a year ago in feburary and had laser surgery.

I guess my rheumatologist is waiting for some other organ involvement to diagnose me. I also have a saddle nose that happened over 20 years ago after a softball accident, and seemed to worsen after deviated septum surgery.

Some questions I have that I'm hoping some of you can help me with:

1. if I go to a vasculitis center for help with a diagnosis, do some specialize in Wegener's or will the closest one do? I am in South Carolina, so I think Emory is closest.
2. Has anyone been there and can recommend a specific doctor?
3. Can a nasal biopsy be accurate for a diagnosis, or do I have to wait for other organ involvement? Sounds like from what I've read about the meds, that only nasal involvement/sinus infections at this point might not be worth the side effects of the meds, anyway.
4. Also, has anyone had success with saddle nose repair surgery?
5. Also, I'm having lab work to check my kidneys done once a year. Is this often enough for someone with Wegener's, and should I be having yearly chest x rays?

I've read that using cartilage from ear/rib cage can be reabsorbed by the body, so doesn't last. But my ENT mentioned another material, and said he had only had one patient that had this material erode through. Makes me worry that with my luck, I'd be his second pt. to have that happen to, then where would I be? ( He was reluctant to discuss it, of course ). He also seemed reluctant to repair my nose if I possibly had Wegener's. So, I'm wondering if I should look for a plastic surgeon, and how does one go about finding one who really specializes in saddle nose repair ( not just advertises that he does ), as it is different from other types of nose jobs, when you are actually missing cartilage.

Should mention that my ENT now does facial reconstruction for cancer patients, so maybe I should just trust him, but have been burned twice now by 2 different ENT's doing sinus surgery that only made things worse. I definitely have trust issues when it comes to doctors and elective surgery. I'm also an RN, which only compounds this problem!

I'm just so tired of not being able to breathe through my nose well enough to sleep. End up sleeping in a recliner most nights, which I can't do if I want to travel. As you can tell, my rheumatologist hasn't given me much information, but after reading all of your posts, I have a lot more questions to ask her at my next visit, including how many Wegener's pt.'s she has. I hate to step on these doctor's toes, but am finally learning that I need to ask these things in order to find the best care for myself. Thanks for reading such a long post and for what y'all have taught me so far!

[andrew]

Hi Pam! Welcome!

Firstly I edited your post to isolate the questions so you get some answers. Everything you wrote is there, just formatted differently :) Hope you don't mind.

You definitely need a Wegener's Specialist. There's a list here although I don't see any in South Carolina. You may see one near you though: http://www.wegeners-granulomatosis.com/forum/general-wg-chat/4748-wegeners-granulomatosis-gpa-specialists.html

Don't be afraid of stepping on anyone's toes. It's your health and your body.

A biopsy of the affected area is a good contributor to a diagnosis along with blood/ANCA/C-reactive protein tests, urine tests and imaging to look for affected organs.

I'd be holding off on surgery until your diagnosis can be confirmed. Despite the fact it was caused by an accident it would be best to rule out Wegener's or any other disease you might have possibly affecting it prior to any rectification.

There are some people here that have had great success with saddle nose repair. Hopefully they will comment.

Well done on getting this far! You're on the right track and like I said, you need a decent specialist to get a diagnosis and move on with a treatment regime.

[Middlesista]

Hi Pam - I am sorry but live in Mass so have no specific knowledge about Emory but you should check out the vasculitis foundation since they have lists of docs and centers

Someone wrote about the importance of being proactive rather than reactive and that has stayed with me - why wait for organ involvement to occur? Close monitoring makes sense to prevent a flare.

I find that that doing a search of the forums is helpful for specific questions and usually folks will chime in.

I am also an RN -

[vdub]

You do need to be your own advocate in many cases. I think the average time for a diagnosis of gpa is about 24 months, because gpa is still a relatively rare disease, but 30 years is a little much. :-) I would be hesitant to think your sinus surgery was a waste. I had sinus surgery to open up the channels before I was diagnosed and it has been great to be able to breathe. I'm sorry your's hasn't turned out that way, but maybe you would have been worse without it.

Did your rheumy mention weg (gpa) or did any of your other doctors mention it? Also, did they put you on any medications such as prednisone. The reason I ask is because if they put you on pred, then they are thinking of an autoimmune disease.

If you don't have a vasculitis specific rheumy and you want a cost and time-saving assessment, then call the local rheumys and simply ask them (or their nurse), if they have any wegeners patients. Its not uncommon for most rheumy's to have a half dozen or more wegs patients. If they do have several wegs patients, that would be enough for me to go to them for an initial assessment and, then, depending on what he says, you could check out a specialist. It might be faster, if you don't have a vasc specilist close by.

[Debbie C]

Hi Pam, you definitely need a dr. that specializes in WG. Check out the VF website to find one by you. Also like everyone else says don't be afraid to speak up, this is YOUR life ....not theirs and if they don't like it then find another. Do NOT wait for this to spread somewhere else.. any good wg dr would know that anca isn't a good marker to prove wg. Mine came back neg 3 or 4 times before it was positive. You should also get MONTHLY blood and urine checks and it wouldn't hurt to get a ct scan of your lungs, since this can spread all over, and x-rays only show so much. A biopsy would be the best proof..find a good ent to do this. I just got my VF newsletter and there is a lady her name is Judy Brown phone 803-324-1331, email [email protected] that has 3 different support groups in S.C. Maybe she can tell you the names of someone in your area that can help. I also agree with Andrew,don't do the surgery until you know whats going on. Take care and keep us posted and hopefully someone else with sinus issues or saddle nose will chime in. Good luck

[renidrag]

I would certainly try to get a diagnosis as quick as possible. What Doctor would want to wait to see if it spread to other organs? Anca is not the tell all to WG. I agree with Debra C that you need your bloods done monthly with special attention to PR3, CRP, Sed rate, Creatinine and maybe a biannual ANCA. I have never had a positive ANCA.
Dale

[vdub]

I have never had a positive ANCA.
I should have mentioned that, as well.... I've never had a positive ANCA, either.

I also get my blood work frequently (every 6 wks), but I revolted a couple months ago. I was tired of so much continuous doctoring over the past 5 years, so I took a self-imposed break a couple months ago. I rescheduled all my doc appts for May and don't intend to get a blood test until a week before the appts. But, then, I know in May I will have to hit the ground running because I have a surgery scheduled to help correct my lungs. Wegs is sometimes difficult to deal with mentally.

[Alysia]

3. Can a nasal biopsy be accurate for a diagnosis, or do I have to wait for other organ involvement? Sounds like from what I've read about the meds, that only nasal involvement/sinus infections at this point might not be worth the side effects of the meds, anyway.


Hi & welcome. nose biopsy can be false negative, meaning that you have wg but the biopsy will not show it. I had 2 false negative nose biopsies, in each they took about 6 pieces from my poor nose.
C anca positive, even in the past, is something to tell the docs about, when they look for diagnosis.
do you have other symptoms that you might think that are not related, like red eyes ? bleeding gums ? joint pains ? rash ? ears issues ? vertigo ? stomach issues ? fatigue ? anemia ?
sometimes we think that other issues are not related when they actually are and when you connect all the dots you can see more.

[annekat]

Welcome to the forum, Pam! It is true that nasal biopsies do not always give conclusive results, but I did get a positive one on the first try, so it is possible and could be worth a shot if you are suspected of having WG. It would help if it was done when things are quite active in there, like crusting and bleeding, so they'd have a better chance of getting a good sample. The procedure, for me, was not difficult, done with only local anesthetic, right in my ENT's exam room chair, and not planned in advance, so he stayed late to do it on the spur of the moment. There was some pain and blood, but it wasn't too bad.

Regarding the thought of waiting for another organ to become involved, I don't think that is a good idea. I went undiagnosed for 2.5 years with just nasal, sinus, and ear stuff, which was chalked up to recurring sinus infections caused by allergies. I did have allergies, and even got shots for them. But I had WG, and no one thought of it until it went into my lungs and caused permanent damage there... not nearly as bad as some people have had, but my lung capacity is reduced and my breathing will never be as good as it was. If they had considered WG earlier with the sinus stuff, and gotten a biopsy there during a flare up, perhaps I would have been treated in time to avoid the lung involvement. Although WG does what it wants and it might have happened anyway. But the earlier the dx and treatment, the better, and the less chance of permanent damage.

So, yes, get regular blood work, as it can take awhile for some of the results to show fully, and compare from one time to the next. If you feel your current rheumy is moving too slow or hasn't enough knowledge, definitely seek out someone who is more expert. A good university teaching hospital (which I think Emory is?) would be a good place to start. I don't know east coast geography well enough to know which of the really well known specialists is closest to you, but I think there is one in N. Carolina (Dr. Falk?) Check the VF website list of specialists here: Vasculitis Foundation » VF Medical Consultants (http://www.vasculitisfoundation.org/mcm_resources/medical-consultants/) . And here another site where a list of docs recommended by patients may be found (in addition to the one posted by Andrew above) : http://www.vasculitispan.org/ . A doc well versed in WG may be able to dx you without a positive biopsy. Best of luck to you in getting this resolved.

[deanna]

Hi, I am a new member also and have found this website to be so helpful to me. I am seeing my second doctor next week but I really will say it is my first. The one last week was inappropriate in so many ways. I have already looked at Vasculitis Centers in the US. Because I am a retired nurse I have knowledge of these places. My appointment next week is where I used to work. I will be going to the Rheumatology Department. I do plan to talk to the doctor about the centers, clinical trials and does she have access to them. How do they compare to the other Vascular Centers. I learned as a nurse who took care of cancer patients that a 2nd opinion should never upset the doctor. The doctor I worked with had many patients ask for a 2nd and sometimes 3rd and he was never offended. We were happy to assist the patient. This is all very scary and I want the best for me. This is my life and arming myself with information is the only control I have at this time. My husband has health issues also so this has been a very overwhelming time for both of us. As I said I am in a large city with a teaching hospital, very well known, that has people come from other places to get diagnosed. I will keep you posted on what is going on.
Again thanks for the help and support from everyone.

[Alias]

The one regret I have about my treatment is that I submitted to a nasal biopsy. In my case it was a quite invasive procedure done under general anesthesia, and it resulted in additional scarring and obstruction in my already damaged passages. The biopsy was negative, and I learned later that nasal biopsies are often negative in patients who are otherwise known or suspected to have WG. My labs, including positive C-ANCA and PR3, plus my symptoms, were sufficient for diagnosis. I know others have had different experience, but I would caution anyone to ask carefully about what is to be gained from a nasal biopsy and if the result would make any difference in the treatment that would be offered. I think sometimes doctors order or perform procedures just because they can....

[Debbie C]

When I was first dxed..my pulmy had me do a lung biopsy and it came back positive but my rheumy, at the time, did not have knowledge of wg and did not believe that was the problem since the anca test came back neg. He thought I had a thing called 'Boop" and I was in the hosp. so he wanted my ent to do a nose biopsy and thank goodness he refused to since it was already dxed. And to this day he still thinks my old rheumy is an idiot !

[Pam C]

alaskatom,
thanks for that information. I have such night time congestion at this point, that i can't imagine doing something that will make it worse. Maybe i just need to ask for more frequent urine and blood checks, and maybe referral to a vasculitis center to get a diagnosis.

[Pam C]

Alysia,
No other symptoms, except the nasal crusting, which i forgot to mention.