New here from Kentucky

[sadpixie]

Hello. My name is Valerie and I was just given a "pending" diagnosis of Wegener's. All of my symptoms and blood tests are pointing to it but they are waiting to get a final c-ANCA on me before giving me the official.
It all started with a sinus infection and ear infection last November. Things cleared up fairly fast but I was left with constant nosebleeds. Fast forward to August of this year and they returned with a vengeance. I now have very little hearing in my right ear and my left ear is constantly stuffy. I have suffered with extreme pain through my sinuses and now have saddle nose.
I just recently got out of the hospital because the pain and pressure in my face was too unbearable to take and I developed Bell's Palsy. I felt great while on steroids in the hospital and even on the small amount that they sent me home with but as soon as I was done with the taper packs everything came back with a vengeance.

Any advice and/or tips would be greatly appreciated. I am a mom of two young kiddos and I am anxious to get things treated the right way and soon.

[Jack]

Hi, :)

Your symptoms do sound fairly typical of Wegener's although it does present in many different forms. Even the suspicion of you having this disease should be prompting your docs into urgent action, things can develop very quickly and even become life threatening even when showing few symptoms. I managed to lose my kidneys that way!

The doctors that you will generally meet will know very little about the disease and its treatment and this can be a very complex area. You need to seek out the help of a vasculitis specialist and you may also have to fight your own battles in order to get the treatment you need. If you have active symptoms, waiting around is not an option.

I'm sure others will be along shortly to give more detailed advice. This is a good site for you to have found and is full of compassionate and knowledgeable people. Feel free to ask any question or just have a good vent about the situation you find yourself in.

[elephant]

Hi Valerie, it sounds like you have Wegeners. Like Jack said you need to see a Rhuematologist who sees hundreds of WG patients. You are close to Cleveland Clinic in Ohio, that is where I go to see Dr Carol Langford. There is Mayo in Minnesota too. I too suffer from the sinusitis and the ears being clogged up almost deaf in the left ear, eye pain and I could on and on. So getting the right treatment with a WG specialist will help tremendously get this disease in control.

[JanW]

Welcome aboard, Valerie. I agree with the others -- get to a specialist if you can. Most docs (even rheumys) will have seen one or two cases of WG, if any, in their practices, and don't have the experience you need if the going gets tough (and it may). I have the saddle nose also, and frankly, there are very few other diseases in the world that cause it, so your doc probably has a very strong suspicion that you have WG.

How is your breathing. I ask because saddle nose often runs together with subglottis stenosis -- that is narrowing and scarring of tissue in the windpipe -- I have both, Brooke on this site has both, and at least one other person I'm not recalling right now. Researchers don't know why this is but it's quite common. My ENT calls it the 'classic stigmata' on the disease that he sees (because he repairs both).

That's one more thing. I would suspect that if you go to an ENT, one thing that he will want to do is repair that deformity. Be very careful about this! Although people always want to get it done as quickly as possible (I know how that feels, believe me), we are not the typical plastic surgery case, and surgery can really spiral WG out of control, if you aren't careful. People do have them repaired, but it should be done by someone who understands the disease and the use of artificial fillers/tissue is right out for us (which means it can collapse again if you get sick again but it's a risk you take if you want it fixed).

I know that this is a ton to absorb, and you want to concentrate right now on getting well, but I thought I'd throw it out there -- aren't many of us around here with 'the nose.'

[sadpixie]

Thank you for all the information. It makes me extremely nervous to wait until Monday to get a final diagnosis. I am currently only showing signs of involvement in the sinus and ear area but I know that can change very quickly and kidney involvement can be silent up until it gets bad. How do you go about getting in with a specialist? Will I need a referral from the dr.'s here? I am feeling pretty lost as to the next step to take at this point, the waiting game is awful.

[Sangye]

Welcome to the group, Valerie. I see my friends have already done a great job of prodding you to see a Wegs specialist! I'll just add this link for good info. http://www.wegeners-granulomatosis.com/forum/general-wg-chat/986-what-newly-diagnosed-need-know.html

I'm assuming since you were hospitalized and did blood work for ANCA that they also did general blood work and a urinalysis. This is necessary to make sure your kidneys are functioning okay. ANCA takes about a week to get back. Meanwhile, I suggest calling Cleveland Clinic and arranging an appointment. Whether or not your ANCA comes back (+), you still have some classic signs of Wegs and need an expert to direct care.

[sadpixie]

They did do general bloodwork but did not do a urinalysis.

[Sangye]

Hmm... I suggest you call your PCP and ask if you can swing by for a urine dipstick test. It only takes minutes, is inexpensive and will put your mind to rest while you wait for the ANCA.

[Thakator]

Hi Valerie,
My welcome to you as well. And, please do visit this forum often as it contains a wealth of information that will serve you well.

I too live in Kentucky and second the opinions you see on here regarding your need to be working with an up to snuff specialist on this disease. Trouble is, where are they? My experience was that I couldn't get an initial appointment with either the Cleveland Clinic or Mayo in Rochester for two months. I made the Mayo appointment and proceeded to try and get some answers here in Ky. in the interim - - answers and help, since I was clearly going downhill at a fairly rapid pace. Like many of us that you will find on here, my experience was also that things can happen quite quickly. Unlike many of us, I was fortunate in that I was able to find help close by and was able to get a definitive dx. by a rheumotologist who was on to the suspicion of WG from the very outset. Because she knows about the need for speed in our situation, she got me through the process of multiple blood tests, scans, examinations by an ENT as well as an Opthomologist (there can be signs of WG in the eyes), an open lung biopsy and into treatment in a fairly rapid manner - - before my Mayo appointment date came around. Didn't go to Mayo then since I didn't want to waste their time by going simply for a second opinion. May try again, however, if things don't seem to go well here. But, so far I'm quite satisfied with the care here.

If you are at all near Lexington and find yourself unable to go to Mayo or CC for whatever reasons or that they can't get to you for a couple of months, I would strongly recommend that you try the UK Clinic in Lexington. Specifically the Rheumotology Clinic. It can take two months to get an initial appointment there as well, so I would advise that you call directly and ask for an appointment with Dr. Hanaoka - - being sure to let whoever you speak with that there is good reason to suspect Wegener's and thus you feel the need to see her (the doctor) as soon as possible. I don't know if they will make you go the route of having your personal doc call to make the referal, but I'd try going the direct route first and most definately mentioning the WG. Dr. Hanaoka is very much aware of the necessity of acting quickly in these matters and if she hears of your plight I'm confident she will try to see you soon. And, she's good. Clinic # (859)-323-3900.

Please feel free to e-mail me if you desire any further insights as to my experiences in dealing with the UK Clinic. And, if you try to get in and are given a long wait time, please let me know and I will personally speak with Dr. Hanaoka to see if she can speed it up. Take care, Ron

[Sangye]

I know the other long-standing members will agree with me that going to a major Vasculitis center like Mayo is not just to confirm the diagnosis. I wish I could convey the difference between going to a non-Wegs specialist vs a Wegs specialist.

[JanW]

It may be that Valerie's blood work covered creatinine, which would also be indicative of kidney health. If a doc saw something out of line with those values, I assume they'd jump on it pretty fast. But yeah, generally, bloods and urine go hand in hand in the world of wegs.

[sadpixie]

I just dropped by my PCP and she got the labs faxed to her from my ENT group and the hospital that I was at. The bloodwork did cover Creatinine and it looked fine. She also had chest xrays done and they are clear for now. I found out that she used to work for a nephrologist out of St Louis and had at least some knowledge of Wegener's and was a bit appalled by how long it has taken then ENT group to get the ball rolling. She was also able to get me into a local rheumatologist on Monday. I will be asking him to contact the Vasculitis Foundation until I can get into a specialist.

I will most likely be contacting the Cleveland Clinic to get in to a specialist there. How long does it generally take to get an appt?

[Thakator]

Hi Sanjye,
As usual, I agree with what you have to say regarding this matter. I've written it here in this forum before - - I agree with you that the best course of action is to get in with a WG specialist and that going to either Mayo, CC or JHU would be optimal. Agreed. That's the top tier, the best way to go. But, the facts of life are such that for many of us the ability to get to one of those places is thwarted by either family concerns, work obligations, inability to travel, distance from where they live or financial limitations. We can't all access that top tier and that's why I am responding to your note - - for those who can't.


For the long-standing members and for the many visitors who peruse these messages, for those who are seeking an answer as to where to go for help when they can't go to the big three, to all of you I would say that there is a tier two that is much better than it used to be several years ago (before the internet). A tier which is apt to be more easily accessable and more quuickly accessable. A tier which has many (even if nowhere enough) physicians who can get us through the Wegener's minefield.

The first tier would be that of our PCP and the network of private practice specialists to which s/he refers us. The least effective tier, the most protracted one, the one with the most ignorance of WG. The second tier, though, is considerably different - - members here are like the big three in that they are structured in the clinic mode and that they have a large body of physicians gathered in one spot working more as an entity as contrasted to those in the private parctices. This tier is made up largely of University Medical complexes of one sort or the other and most staes will have one or more. My whole point in writing this is simply to point out that there may be sufficient assistance closer to you than you think and to encourage you not to think that all is lost if you can't get to that top tier. Other folks on this forum have referenced various places in what I have described as tier two and I hope that we continue to do so for the sake of all who are looking. That's what I was doing in my note to Valerie.

Personally, I am fortunate enough to be able to go to Mayo and, as I said in my previous post in this thread, I had already made the appointment. I do recognize their place in this environment; I agree with you. My deciding not to go there for a second opinion was simply a reflection of the fact that I by then had moved beyond the opinion seeking stage; I had my dx which was based upon histrory, symptoms, blood tests, ANCA positive results, multuiple lung nodules and a WG positive biopsy obtained via the removal of a golf-ball sized nodule during an open lung/web resection proceedure. I no longer needed an opinion as top what was wrong; I had all the confirmation needed. What I needed then was treatment. The treatment here will be the same as it would be at the hands of the Mayo specialists as far as I can tell. If I should find otherwise or find my care here to be less than enlightened, then I shall move on to them. In the meantime, I'm choosing to have faith in my local tier two facility and hope that you would encourage those who can't go that optimal route for reasons mentioned above to do the same and not to feel like they don't stand much of a chance if they can't get to Mayo, CC or JHU.

Keep up the good work, Sanjye, you are a fount of knoweledge herein. Ron

[Sangye]

I just dropped by my PCP and she got the labs faxed to her from my ENT group and the hospital that I was at. The bloodwork did cover Creatinine and it looked fine. She also had chest xrays done and they are clear for now. I found out that she used to work for a nephrologist out of St Louis and had at least some knowledge of Wegener's and was a bit appalled by how long it has taken then ENT group to get the ball rolling. She was also able to get me into a local rheumatologist on Monday. I will be asking him to contact the Vasculitis Foundation until I can get into a specialist.

I will most likely be contacting the Cleveland Clinic to get in to a specialist there. How long does it generally take to get an appt?
Make sure they don't do unnecessary x-rays. I can't remember if you had shortness of breath or other indications of lung involvement. If not, there is absolutely NO reason to go hunting around. Wegs is a lifelong disease and you have to limit your radiation exposure. I've had massive radiation exposure due to my original docs (non-Wegs specialists) and ER docs. Literally 3 times the radiation of Hiroshima, in 4.5 yrs.

[Sangye]

Ron, I agree that not everyone can get to a VF center. However, everyone can have a VF doc consulting on their case-even Weggies living outside the US (I checked with the VF).

There are so many subtleties and decisions along the road with Wegs. Even though one's treatment is working well, the info gleaned from a Wegs doc goes far beyond which drug to use.

When I was treated at Mayo Arizona I thought it was amazing care. I had no idea about VF docs. It wasn't until I got to JHU that I realized how inadequate and just plain uninformed my care at Mayo AZ was. Had I agreed to that doctor's plan I would have been put back on 80 mg pred!

Even excellent rheumys who don't specialize in Wegs can really mess up our care. Last year I was hospitalized for a week at JHU. My own Wegs doc couldn't treat me but was advising the other rheumys. Much was lost in the communication. Most of the rheumys work in the same office as him, and are truly excellent docs. Even so, one thought I had viral bronchitis, one thought a mild Wegs flare, and one thought it was a severe Wegs flare. The last one was correct, but he wanted to do 80mg pred immediately and taper over months. When I saw Dr Seo he put me on ctx and 5 mg pred.

Besides my own experiences, every member we've lost this year was due to not having a Wegs specialist. This is why I'm admittedly pushy and annoying when it comes to Weggies consulting with Wegs docs. There are all kinds of ways to make it happen-- going only once or twice a year, getting flight assistance from pilot philanthropists, etc.... Every time we lose a member to Wegs I get a bit more annoying.

[JanW]

There are certainly cases where treatment may be completely identical at a 'Tier Two' facility (and in fact I will define my own hospital as such -- while it has as WG specialist, and one of the lead researchers in the RAVE study, and is the largest rheumy center in Manhattan, it is NOT a vasculitis center), I think that's only fine if you have the most better-than-textbook case of the disease (which I'm fortunate to this point to have had). Any time there's a little monkey wrench (e.g., you can't get off pred in the now expected 3-6 month time frame, you are having trouble reaching remission within a year), your treatment might be very different at a vasculitis center.

I met a woman during my focus group on WG last night (which I will write up on a separate thread) who is being treated by a pulmo at a top teachinig hospital in NYC. She's been dx a little more than a year, bounces around on pred, gets through the day ok, but still has nodules in her lungs. She also happens to see my beloved Dr. Lebovics for her sinuses. Guess where she's going next month? Cleveland Clinic to see Langford. Guess who told her she should do that? Dr. Lebovics (who I can guarantee also knows her pulmo because he's a top guy). As I always say, the guys who have seen the suffering and death that this disease can cause, don't mess around. For the top physical problem that I have (nose and throat) I see Lebovics because he really is considered one of the two or three best in the world (I'm sure he'd be in Cleveland with his former colleagues if he wasn't born in NYC).

So I'd say that the visit to a vasculitis center or specialist really isn't even a second opinion about your diagnosis, but, rather it could be an entirely new opinion about how you can best get into, and mainitain, remission.

[Sangye]

Exactly. And it's the "little monkey wrenches" that have gotten me into trouble much of the time. Like when I tapered pred too quickly last spring-- Dr Seo took one look at me and knew exactly what was wrong.

[jtausan]

Hello,

I like Sangyes quote that Mayo Arizona was amazing but JHU was even better. I feel bad now because about a year ago I went through the hoops and scheduled at JHU in Baltimore. Before I was to go I let family, work, financial obligations etc change my mind. After I read that sentence from sangye I realized I have made a hugh mistake and need to schedule again with JHU. This is a hardship for me and I know Mayo (rochester) is closer but I just think JHU would be amazing! I want to live and my quality of life sucks. (I know you all know what its like) I soooo appreciate all of your comments and suggestions and I learn so much from all of you. I do have a new Rhuemy here in town but want to have the WG specialists treat me and have the dr's here just follow the directions. That would be the ideal situation. I hope it works.
Jtausan

[Sangye]

Jtausan, Mayo Rochester is as good as JHU. Both are major Vasculitis Centers. If Rochester is close, it might be best for you to go there.

To clarify, Mayo Arizona is not a Vasculitis Center, and my rheumy there was not a Wegs specialist. I thought it was amazing care, but it wasn't. My stubbornness saved me from being seriously overtreated, as I refused to go back on massive pred. I didn't realize how inadequate the care was until I got to JHU. Mayo Arizona is a leading facility for so many conditions, just not Wegs.

[JanW]

Honestly, I've never heard anyone on these board differentiate between one vasculitis center and another. People in Boston love Merkel, people who go to JHU love Seo, people who go to CC love Langford and so on. All of these people (and others in other vasculitis centers) are highly knowledgeable, and I don't think you'd go wrong with any of them.

[Sangye]

Very true! All the Wegs docs are amazing. :smile1:

[pberggren1]

And you can always come to Swift Current as well to see my doc.

[sadpixie]

I see that there is a doctor listed on the VF website in Kansas City. It is a bit closer to me and I would be able to go sooner than other options, would it be worth it to go to him or just save a bit more to travel farther to one of the major centers?

Also just found out my liver enzymes are quite high. Ultrasound of the liver showed no abnormalities. Dr. said this will affect my ability to take methotrexate. Is it possible for Wegs to be affecting my liver as well? Thank you all for the input!

[Sangye]

The advantage of going to a vasculitis center is that you can see other specialists who also know Wegs well. I wonder if you could find out if the Kansas doc has a good team like that.

Wegs can affect the liver but it's very rare. It's more likely that the drugs you're on are stressing the liver. Mtx and imuran are hard on the liver.

[sadpixie]

Thank you, I will give the dr. in KC a call tomorrow. Today was my first appt with my local rheumy, I was able to call and get it bumped up from Monday. I have requested that he consult with a specialist and since I have no current signs of activity other than the sinuses he is waiting on that to put me on meds aside from prednisone. He would really like to do rituxan if we can get my insurance to cover it. I will see him and my ENT again next week.

[Sangye]

I forgot you're not on other meds yet. Did your doc offer any explanation for your elevated liver enzymes? The most common causes are alcohol, drugs, other toxins, hepatitis and obesity. It's very important that they find the cause. Elevated liver enzymes indicate liver stress. If it continues it can do permanent damage to the liver. Poor liver function will really complicate treatment options for you, too. Not just Wegs drugs, but drugs they have to use to deal with complications.

[sadpixie]

It's currently a mystery, my liver looks perfectly healthy on scan and he is reluctant at this point to have a biopsy done. We are hoping that it could have been all the antibiotics that I was on prior to diagnosis (endless round of zithromax, avelox, clindamycin and augmentin). I have stopped taking them and we will retest a few days before I see him again. I don't drink, etc and though still somewhat overweight i recently lost 42lbs and had not been overweight for long. Developed hypothyroid after my four year old son was born so it was a bit difficult to lose that pregnancy weight from him.

[Sangye]

I hope that's all it is.

But hey, no wonder you developed hypothyroidism. Carrying around a 4-year old inside of you had to be a lot of stress on your body. :w00t::tongue1:

[sadpixie]

lol! That would be quite taxing on the body! I have been reading your post about beating some of the side effects of prednisone and I think I have my husband on board to help me follow some of your advice, it has been very interesting!

[BrianR]

I see that there is a doctor listed on the VF website in Kansas City. It is a bit closer to me and I would be able to go sooner than other options, would it be worth it to go to him or just save a bit more to travel farther to one of the major centers?

Also just found out my liver enzymes are quite high. Ultrasound of the liver showed no abnormalities. Dr. said this will affect my ability to take methotrexate. Is it possible for Wegs to be affecting my liver as well? Thank you all for the input!

Some antobiotics, including Dapsone (used for those who cannot take Bactrim), can raise liver enzymes.

[marta]

Hey sadpixie,

I too have the same liver issues. LFT's were sky high when I just had my flare in October/early November (GGT was at over 350, ALT was 150+) and this is the reason they kept taking me off the cytoxin. I also had elevated liver enzymes when I first got diagnosed (now that you mentioned it in your post, I remember also being on a whole swack of antibiotics before diagnosis trying to beat down the seemingly infected ears I had). I don't drink, do drugs, have hepititis and was 142lbs before getting sick (I too had some pregnancy chub I couldn't get rid of - was about 132lbs before havnig Hana four years ago) but I can't see that throwing off my liver. My scans all look normal too. I've been under the impression that although 'exceedingly rare' that in my case my liver might be getting involved when the disease is active. I've been off the dapsone for a few weeks now, and now that the cytoxin is doing it's thing and things seem to have stabalized ever so slightly and my liver enzymes are going down (on Wednesday ALT was 57 and GGT was 174) I talked my doc into trying out the dapsone again and see if it does anything. This time our experiment is more controlled and we're not removing and adding cytox to the mix (which takes about a week to kick in and a week to leave your system - it was a very flawed experiment a few weeks ago). So I will report any findings within a week. I'll go in for bloodwork on Monday and see what happens after three days on dapsone.

Hope things stabalize for you. uggghhhh, what a crazy Rubic's Cube this goofy disease is.

Incidentally, I had no issues with my liver throughout the summer when I was taking both cytox (cyclophosphamide) and dapsone. Things were normal and steady. Only when the thing kicks into high gear.

[sadpixie]

Bah got a call from rheumy this evening saying that my urinalysis showed a small amount of blood. Creatinine was still normal, I am to go in Monday morning for more testing. I hate waiting over the weekend.

[Jack]

I very often have a trace of blood in my urine, not enough to see, but it shows on the tests. This is very common and almost certainly nothing to worry about. Just needs further checks to make absolutely sure.

Don't let it spoil your weekend! ;)

[sadpixie]

Thank you, with just being diagnosed and learning about the disease my mind is still a bit in panic mode. I will definitely try not to let it ruin my weekend!

[Sangye]

Even though I have no kidney involvement I often have trace blood, too. It's not that uncommon for people in general and is nothing to worry about. I'm not sure if yours was "trace" or a bit more, but I agree with Jack-- don't worry about it and just get it rechecked on Monday.

[jola57]

sadpixie, do not worry it seems to be quite normal to have a "trace" blood once in a while.

[sadpixie]

Rechecked and everything was ok. Ready to start treatment next week!

[Jack]

Another crisis avoided! :biggrin1: