Diagnosis pending - new member from SD

[LindaKay]

Hi - I'm new from S.Cal. and likely have limited W's, at least according to 2 pulmonologists, and 1 of 2 rheumatologists, though the 1 rheumy who has the most experience is not convinced and 2 ENTs think it's a virus causing recurring tracheal stenosis. Any advice on getting a diagnosis? How long did it take for confirmation of W's? My pulm's keep telling me of patients who didn't get a positive ANCA for 7 years; it's been @4 yrs since first symptoms for me. ANCA and other blood tests normal, tracheal stenosis / inflammation comes and goes; not yet on MXT b/c last flexible bronch showed no signs of inflammation ("just" a paralyzed vocal chord attributed to, as usual, some mystery virus). Prednisone for several months didn't stop recurrance of tracheal stenosis after laser excision w/ or w/o stent (stent was a fiasco, even being on drug for CF [pulmozyme] 2/day + 4/day saline breathing treatments didn't stop mucus build-up). Still on the pulmozyme 1/day to deal w/ mucus, though docs seem to ignore that symptom; guess the tracheal stenosis and mostly paralyzed vocal chord are more interesting. Stenosis seems under control for now, after 2 dilations. Still not sure if it's W's or if I'll ever find out, so any advice is welcome.

[Jack]

Hi Linda, welcome to the forum.

It sounds like you desperately need to find a Wegener's specialist! Your current docs sound like they are out of their depth and they will kill you if you have the disease and do not receive the correct treatment.

Wegener's is not easy to diagnose unless there are one or two indicators showing that do not usually appear with other diseases. Tracheal Stenosis is quite a strong pointer as would be a biopsy showing the presence of granulomas. ANCA is not a reliable indicator, just another tool in the box. Diagnosis is usually made using observation of all your symptoms and scoring them according to the Birmingham Vasculitis Activity Score (BVAS).

Someone will be along soon to help you find the right specialist for the job.

[elephant]

Hi Linda, I agree with Jack. Need to see one real soon. Go to the Vasculitis foundation web site. I go to the Cleveland Clinic and see Dr Carol Langford, she is a Wegeners specialist. There are more very good Wegener specialist and some on this forum with be saying something soon. You want one that see's over 100 patients with WG. Someone on this forum recently died after three months being diagnosed with Wegeners disease....he did not see a Wegeners specialist..he was young...38 years old.

[JanW]

Also, the vasculitis foundation is having its conference in Long Beach on July 30-August 1 (patient conference). Would be a good place to see all the top docs in one place and potentially get a question or two answered. I'm going to that. I also have subglottic and tracheal stenosis, but mine isn't transient. The surgeon cut into the scar tissue as well as balloon dilated it and so far so good (had it done in March). I was reading on another board about someone with transient TS. I didn't even know that was possible.

[Sangye]

Linda, I agree with the others-- you need to get a confirmed diagnosis and be treated by a Wegs specialist. Elephant mentioned Mark, the member who died recently. He was diagnosed with sinus-only involvement but died 3 months after diagnosis due to silent, severe Wegs destruction of his heart. Please don't wait. Contact the Vasculitis Foundation for help finding a Wegs doc in your area or travel to a major center.

[LindaKay]

Thanks for the advice -- I will look into W's specialist, but I'm pretty sure I've already seen the specialist at (what I believe is a pretty well-respected teaching hospital) at UCSan Diego: the rheumotologist who said, essentially, "All the tests are negative for W's, I know W's, you don't have W's; maybe you did before but you don't now, and since the only real treatment consists of drugs that are basically poisons, I'm not putting you on anything." I'll have to push more about how many cases he's treated and others in the area.
Response to Jan - you prob'y don't need to be told this, but be sure to follow up more than the average time after the tracheal excision/dilation. I've had the laser excision a few times -- every time, I was fine for the typical 3-4 month recovery period; I didn't start having problems (the same area scarring over and narrowing) until after they pronounced me cured (well, the first time; after that, they knew better than to get too optimistic). Hopefully you will not have a similar reoccurance!