Still in diagnosis limbo

[BookNut]

As you may know, I am a bit skeptical of my diagnosis. Well- now both my immunologists think it IS Wegner's. I had made a decision to go to the ENT and ask about a biopsy - just so I could be more certain. Now I am confused again. He is inclined to think along the lines I was thinking. I am not sick enough to have Wegener's. But…he says some folks do have a very limited version….

To make things even more confusing, my sinuses are quite clear right now - so he says there will most likely be nothing, or very little, to biopsy, which will still leave me in limbo. So….now he has referred me to a pulmonologist to see about a bronchoscopy. The referral has been sent and they will call me tomorrow to set up an appointment.

So - once again - I am on hold.

I must say, they are all good folks and interested. Yesterday I went to the allergist/immunulogist, who thought it was likely that I do have WG, but agreed that I should get a definitive test. At 8:30 this morning the phone rang, waking me up. It is the allergist. "Do I have any problems when I take NSAIDS (aspirin and ibuprofen")". When I told her no - she said "OK - that shoots that theory. I was looking into Samter's Triad - but if you can take NSaids - that is not the problem. Have a good day". So - at least I know she is thinking. She is such a nerd - in the most complimentary sense of that word. She is like a bull dog with a bone about this diagnosis.

Meanwhile - I continue my wheat-free life at the suggestion of my PCP. Not too bad so far…

Tune in again in the next 10 minutes. I am sure the diagnosis will change. This pulmonologist will be my 9th doctor!! At this point, I truly do not care. I want a firm diagnosis. If it is WG, I can take the medicine and deal with it. If it is not - then tell me what it IS, and I can deal with that too. I JUST WANT TO KNOW FOR SURE!!!! GRRRRRRR!

[Dirty Don]

Be patient...it takes time to get a solid diagnosis & a good team...unless you do it like I did...and you really don't want to go there!

[BookNut]

Be patient...it takes time to get a solid diagnosis & a good team...unless you do it like I did...and you really don't want to go there!

Well - at least I have a good team. I just want things to be settled so i can get on with dealing with whatever I need to deal with. Sigh.... Thanks as always for the support!

[NikkiNicole]

Hi book nut! I have just been given a tentative diagnosis of WG because of granulomas biopsied in my lung. My doc also says I'm not "sick" enough. I have rhinitis with what felt like sinus pain all last year, my hearing was muffled and I had to get tubes. Then they discovered a nodule in my lung. I had a swollen salivary gland removed, that was cancer and unrelated so they biopsied the nodule and found granulomas. Currently my sample is at Stanford Medical Center for a definitive diagnosis but I have started prednisone. I can't start my cytoxan until after radiation for the cancer. I'm not feeling any better yet, still coughing til I can't breathe. But, I hope after the cytoxan round, ill be good for a long time.
i hope they give you a final answer soon. I had my PCP, ENT, pulmonologist, rheumatologist, oncologist, ophthalmologist and saw a neurologist in the ER because the left eyebrow sank temporarily. It's been a short (to some) but long haul to me.
i also have one pupil smaller than the other. Not sure if that's related!!!

[BookNut]

i hope they give you a final answer soon. I had my PCP, ENT, pulmonologist, rheumatologist, oncologist, ophthalmologist and saw a neurologist in the ER because the left eyebrow sank temporarily. It's been a short (to some) but long haul to me.
i also have one pupil smaller than the other. Not sure if that's related!!!

What a time you are having! I hope you will soon be on the upswing. I also hope you don't mind my making a little joke. Believe me, I have every sympathy for your very challenging situation. But I just had to chuckle when I read about your "left eyebrow" (so glad THAT was only temporary!!). The reason I chuckled was because I just got off the phone with my PCP where I told her that I now have so many specialists that I expect any day to be referred to a "left-eyebrow specialist"! And don't I hang up, go on here to re-read the latest responses, and find someone with a left eyebrow issue!! What a disease. Weggies are nothing if not unique! Keep posting and reading. There is SO much expertise and empathy on this board!!

[annekat]

Yes, all the positivity and support on this forum continues to amaze me. I wonder if any other disease has this good a forum. We are so lucky!

Booknut, I was a little too terse in my statement about being "not sick enough to have Wegs for 2.5 years." It wasn't that anyone was hinting that I might have Wegs and not doing enough to find out if I did or not because I didn't seem "that sick". That would be even more frustrating, because I would be looking it up and finding out about it and not getting an answer. The way it was, no one even suspected it as Wegs, I just thought, like they did, that it was a never ending series of sinus infections and allergies, like a lot of people have who don't have Wegs. So it WAS frustrating, but not in the same way. Then when it hit my lungs and I thought I had pneumonia, things were bad enough for someone to suspect Wegs. And the appearance of my saddle nose, plus the biopsy, clinched it. That version of a delayed diagnosis is pretty common, unfortunately. I'm at least glad that you are not all that uncomfortable, that your sinuses are pretty clear, etc. but just hope that doesn't keep delaying the dx if in fact you have it.

[drz]

Not sick enough to have Wegs?? Don't doctors realize that most diseases tend to tend to come in various degrees of severity from very mild to quickly fatal. Wegs used to be quickly fatal till they found some treatment for it, but I suspect that even back then people limped along for months or years before it became severe enough to get diagnosed. I bet many died from it without it being correctly diagnosed.

Limited does not mean not serious. In my opinion it is an archaic term meaning no kidney involvement but being present in respiratory system. I had generalized Wegs but my Wegs in lungs was a pretty serious problem, not a mild one, but without the kidney involvement it would have been called limited Wegs.

Biopsy of upper respiratory system according to John Hopkins web site are not as reliable as biopsies from lung and kidneys.

Wegener's Granulomatosis - Types of Vasculitis

"Because Wegener’s so often involves the upper respiratory tract (sinuses, nose, ears, and trachea [“windpipe”]) and because biopsy of these tissues is a relatively non–invasive procedure, these sites are frequently biopsied in patients suspected of Wegener’s. Unfortunately, the yield of biopsies from these sites is rather low: probably less than 50%. Therefore, sometimes more invasive procedures are required to make the diagnosis.
Lung biopsy (either open or thoracoscopic) is often the best way of diagnosing Wegener’s. The ample amount of tissue obtainable through these procedures usually permits confirmation of the Wegener’s diagnosis. Similarly, although the amount of tissue obtained through a kidney biopsy is usually much smaller, the finding of certain pathologic features in the context of a patient’s overall symptoms, signs, and laboratory tests is frequently diagnostic."

[drz]

More important info from the above web site: "Wegener’s granulomatosis is a disease involving granulomatous inflammation, necrosis and vasculitis that most frequently targets the upper respiratory tract, lower respiratory tract, and kidneys. Although Wegener’s granulomatosis can begin at any age, the average age of onset is about 40 years. Other organs frequently affected by Wegener’s granulomatosis include the eye (proptosis and double-vision from retro-orbital pseudotumor, scleritis), skin (ulcers, purpura). or peripheral nerve (mononeuritis multiplex). Wegener’s granulomatosis may be limited to one site for many months or years before disseminating. Systemic symptoms (fever, fatigue, weight loss) are also common. Anemia, mild leukocytosis, and elevated Erythrocyte sedimentation rate (ESR) are nonspecific laboratory findings. Chest radiographs often show infiltrates, nodules, masses, or cavities; only hilar adenopathy is incompatible with the diagnosis of Wegener’s granulomatosis. CT of the chest is more sensitive than chest radiography and can be abnormal when the chest radiograph is negative. Glomerulonephritis causes hematuria, erythrocyte casts, and proteinuria.

Untreated Wegener’s granulomatosis is fatal. Prednisone may slow progression of the disease but by itself is insufficient to arrest the disease. Respiratory tract disease usually progresses slowly, but renal disease can progress rapidly and therefore warrants urgent evaluation and treatment. With the traditional treatment of prednisone (initiated at 1 mg/kg daily for 1 to 2 months. then tapered) and cyclophosphamide (2mg/kg daily for at least 12 months), more than 90% of patients improve and 75% remit. However, 50% of the patients who later remit also relapse, and oral daily cyclophosphamide causes serious toxicity. Short-term toxicity includes cytopenia, infection, and hemorrhagic cystitis. Long-term use of cyclophosphamide in patients with Wegener’s granulomatosis more than doubles the risk of cancer overall, increases the risk of bladder cancer 33-fold and the risk of lymphoma 11-fold. Monthly intravenous cyclophosphamide appears less toxic but also less effective. Weekly, methotrexate appears to be an effective alternative for Wegener’s granulomatosis that is not immediately life-threatening, and it also appears to be beneficial in maintaining remission. The role of trimethoprim-sulfamethoxazole in treating active disease is controversial, with some finding it effective for Wegener’s granulomatosis limited to the respiratory tract, and others not. In patients who have achieved remission, trimethoprim-sulfamethoxazole reduces the relapse rate."

[annekat]

Thanks, drz. Your post made me realize I'd mis-worded mine so I edited it. Yes, a lot of people limp along for years and probably always have. Then things get worse, it gets diagnosed, we get treated, and we usually get better. Where before, things got worse, it didn't get diagnosed, or it did, but either way, people always died. So we are lucky indeed to be born when we were, if we were going to get Wegs.

One bad effect of more doctors learning about Wegs is that they could think they can treat it themselves and fall into some of the patterns we've seen here, calling it "limited", not being aggressive in getting a dx, etc. It seems some docs don't realize the seriousness of it and play around with treating it themselves when they should be helping the patient find a specialist or one to consult with.

[rebekah]

I understand how you feel... my doctors had no clue what was causing everything until I was finally diagnosed with WG. At least they think that's what it is for you. It sounds like you have good doctors working around the clock to help. Hopefully they have a definitive answer for you soon, so you can start treatment and start to feel better soon.