Hello from Rochester, NY. Just diagnosed with GPA and coming to grips

[bgifmo]

Hello everyone, I was just diagnosed with GPA 2 days ago, so I'm still in a state of shock. I had been ill for several months with bizarre symptoms (eardrum ruptures that didn't heal, flu-like aches, and eventually migrating joint pain) and I finally went to see my GPwho ran some tests that all came back negative, but got me on Prednisone. I immediately felt better, but was uncertain what lay ahead. It took awhile to get an appointment with a Rheumatologist who took little time in diagnosing me with Weggener's.
Needless to say, I was stunned. I didn't see it coming. We suspected an AI, but this was a pretty devastating diagnosis. He didn't really give me a lot of information during our session, so what I've learned from Google has been on my own. I'll tell ya, it's scary. (Obviously, I don't have to tell ya. You can all relate.)
So, I'm here to learn, hopefully laugh some, and to be part of a community that supports each other.
I have a wife and a young daughter and I'm not sure what the future will hold, and that is scary.
Right now, the Prednisone is doing it's thing, but I don't see my doc again for 6 weeks. As soon as I found out about the disease, I wondered if I should be waiting that long to get on the maintenance meds. He is leaning towards Cellcept, or as a second choice Methotrexate.
There is not a lot of "positive press" about GPA on Google, so it's been a pretty rough couple of days gathering information.
So, hello to all. Glad I found you.

Brian

[WG mom]

When my daughter was first diagnosed, I immediately checked the Mayo Clinic website which helped me to understand the basics. There is also much information available through the Vasculitis Foundation. We actually lived in Rochester for many years and suspect my daughter's ENT there wasn't well versed about WG because had he performed a biopsy after her initially surgery when she was 19, I believe it would have been positive. Her current ENT in Manhattan performed surgery at age 24 after symptoms for all those years were written off as allergies with sinus infections. Bottom line, make sure your doctors are highly knowledgeable about WG. The University of Rochester Medical Center should have excellent doctors so check their backgrounds.
With regard to medication, after her initial diagnosis just seven months ago, she was given a high dose of prednisone, as well as methotrexate and bactrim. Just recently started on rituxan, so its effectiveness will take some time to determine. She was never prescribed Cellcept.
Under the meds thread on this forum is a link to a webinar about meds. I watched that yesterday and it was very informative.
The more you know, the better you can advocate for yourself.
Best to you, Brian. Keep reading the posts from those who have first hand experience; they are a great resource.

[bgifmo]

Thanks WG mom, I appreciate it.
I'm working with Rochester General Hospital doctors, but I have been thinking about getting a second opinion, just for the heck of it. This rheumatologist came highly recommended by my GP, but with something this serious it might not be a bad idea to find doctor that you click with.
I agree with your advice about educating myself and being able to advocate for myself. I'll check out the meds thread, thanks.
I'm sorry to hear that it took so long for your daughter to get diagnosed! This seems to be a tough illness to figure out depending on what symptoms you experience and who you see. My ENT had no idea what was going on with me and said the last thing you want to hear from your doctor is that you are an "interesting case." He was right.
I'm fortunate right now to be responding so well to the Prednisone, but nervous about what the future holds.
Take care.

[Alias]

Hi Brian, I am a new member here, too. I was diagnosed about six months ago, after months of mysterious ear and nasal issues. I think initially one is relieved to have a diagnosis, so effective treatment can begin. But then, the implications of having a rare chronic disease start to overtake that feeling of relief pretty quickly. It is definitely scary. As you learn more about the disease and your particular case you will start to put it in perspective. Meanwhile, I hope you get a good treatment regimen established and just start to feel better. I agree with WG mom, there is great info available on the Vasculitis Foundation site, including a video series of lectures.

[Pete]

Hi Brian,

Welcome to the forum. Lots of good folks here.

You may want to either have your doc consult with a Vasculitis Foundation medical consultant/expert or take a trip to Cleveland Clinic for evaluation and establishment of a treatment plan. Drs Gary Hoffman, Carol Langford, and Alexandra Villa Forte are all excellent. I see Dr Villa Forte and am most pleased with her care.

Good luck!

[lag713]

I agree that your doc should consult with those at the Vasculitis Foundation. It would be a mistake to wait 6 weeks to start taking medications to actually treat the disease given that many drugs take months to go into full effect. Prednisone makes you feel better by bringing down inflammation but it only treats the symptoms, not the disease. Given your symptoms, I think methotrexate would be most appropriate but that's something to discuss with your doctor. Cellcept has a higher relapse rate compared to methotrexate.

I would recommend that you get a CT scan to see if any damage has occurred in your lungs, urinalysis and blood work to check your kidneys, a hearing test to see if your hearing is intact, as well as an endoscopy of your sinuses, trachea, and upper lungs. I was just diagnosed in January and I've had the workup. Best thing to do at this point is establish a baseline and preserve the functioning you have by getting ahead of the disease - prevention is invaluable. Don't let the information on the internet scare you. Let this be an opportunity for you to take charge of your health and well-being. You can live a long and healthy life if you are proactive about treatment, checkups, treatments, etc. It is a chronic, lifelong illness so you will need to acknowledge that the disease can become active again at any time and you will want to be on top of it.

I know of Dr. Shapiro (rheumatologist) and Dr. Lebovics (ENT) in NYC but that would be a trek from Rochester. I make the trip from central Connecticut every 3 months to see Lebovics because of his expertise - he sees a lot of weggies every week whereas my local rheumatologist and pulmonologist have seen less than 5 each in their careers. Dr. Lebovics put me at ease because he is very knowledgable. Given your location, there may be docs in Canada that would be easier to access.

I recommend the resources collected as "stickies" in the General WG Chat forum. I'm sorry that you received this diagnosis and are floundering through the information online. You have support here. I know you must still be in shock. It took me a few weeks to really accept it as being real. It will take longer to come to terms with what it means. I still feel like I freak out whenever something goes wrong but I am learning to roll with the punches and accept what is out of my control.

[vdub]

Sorry to see you join our little elite club, Brian. I think it was Will Rogers who said "I wouldn't want to belong to a club that would have me". And, that holds true here. But, here we are. You'll go through ups and downs. Some people have some rather mild cases and continue on with life in a rather normal manner. While others, really have a bad go of it. I have been in the "bad go of it" area for the past 5 years. It was very depressing at times, but we kept plugging away and finally got the right mixture of drugs. For the past 5 months, life has been good. Lets hope you can reach karma real soon. Welcome to the forum..... vdub

[jlove]

A good doctor is definitely important, like everyone said. It does take some stress off, knowing you have a doctor you can really communicate with and one who knows what they're doing. It took me awhile (6 years) to finally be diagnosed so I was almost relieved when it finally came. Now, at least, you know what you have and you know what your options are. Like lag said above, the best thing you can do is be on top of your health and treatment and hope for the best. "Cautiously optimistic" is what I aim for and so far I've been pretty good with that. I try to minimize stress and take care of myself as best as I can so I don't put my body through more than it already has to. My diagnosis was a big change in my life but I have had a few positive things come from it so I try to focus on those things as much as I can. There's a lot of support and information on this forum so you're off to a good start!

[annekat]

Welcome, Brian. No, please don't get too caught up in what you read on the internet. Right here is pretty much the best source of info. There are some good sites such as Mayo and John's Hopkins, and the VF site, but even some of them are a bit out of date in places, regarding treatment meds and such. Here, many of us know a lot by virtue of reading about the many experiences and knowledge of other members, and get a feel for things over time. I have learned a lot more here than from any doctor or any other site on the web.

I agree with lag713 that once one is diagnosed, it is not standard procedure to wait to prescribe the immunosuppressant drug. Prednisone alone is not enough. And given the type of involvement you have, methotrexate seems to me to be the most usual choice, given what I've read here. There are stronger drugs, but they can be saved in case of more severe involvement, such as lung or kidney. A lot of people have done well on MTX. I don't know much about Cellcept, except that it doesn't seem to be commonly prescribed, especially as the initial drug, but even as a maintenance drug, that is, for when the disease has been brought pretty well under control by the initial treatment but needs continuing medication to stay that way. If someone can't tolerate MTX, or one of the others, they may be given an alternative.

Anyway, my intuition is also that a second opinion would be warranted, and perhaps you could get started sooner on your full treatment and minimize possible damage from stretching things out too long. Perhaps you could see one of the well known docs listed above, or others listed here: VF Medical Consultants (http://www.vasculitisfoundation.org/mcm_resources/medical-consultants/) Then if they are too far for frequent trips, maybe one of them could recommend a doc in your area, or your doc could consult with them, as they have made themselves available for that. Good luck! And don't get too scared, most of us do quite well for a long time with proper treatment. This may never have to affect your bringing up a family, having a career, and the other things we do in life.

[Alias]

I was put on prednisone as soon as the provisional diagnosis was made locally, based on the positive ANCA result, etc. After consulting with the rheumatologist I would be seeing in Seattle, my internist immediately ordered the chest CT and blood work to rule out lung and kidney involvement. If that has not been done in your case, I would be concerned and looking for explanation from your doc and/or another opinion. I ended up waiting two weeks to start immunosuppressant treatment, but the very day I saw the rheumatologist I was started on self administered injections of methotrexate and other meds. Six weeks does seem like a long wait, especially if the extent of disease in your case is still unknown.

[bgifmo]

Thanks everyone for saying hello and offering some good advice. I called my GP today and will see him Monday to discuss how my appointment went, and the idea of getting a second opinion. Rochester has quite a few AIR practices, and the University of Rochester is a very good and reputable hospital. Perhaps I'll find a better fit there. Lag713's point about finding a doctor with expertise in Wegener's is a good one. I don't have any idea how many people my doc has seen with this particular AI. There must have been a good reason for my GP to recommend him, in fact he had to fight to get me in to see him as he wasn't seeing other patients. But the fact that I left the appointment underinformed and unprepared does not sit well. Not to mention, I feel the clock ticking and wonder about getting on immunosuppressants.
Some of you mentioned getting baselines. I do have the blood tests (positive ANCA) and urinalysis baselines (kidney was involved), but nothing yet on the lungs. I'm going to request that a CT is done. While we were trying to figure out what the heck was going on with my ears, my ENT sent me to an audiologist who said I had fluid in my middle ear. With the prednisone, the ear finally did clear and the next pressure test showed my hearing was normal again, but the eustachian tube still isn't functioning properly as it clicks and crackles still.
I'm sure there are other threads on this, but I've just begun to wonder about insurance and the costs of treatment. My mother-in-law asked me if the drugs were "off-label" and I didn't know what that meant. But I think that most of the drugs were in fact developed for other purposes, so I need to start asking questions about coverage I guess.

[lag713]

Methotrexate is generic (brand name: Trexall). I pay a small copay based on my insurance. It's very affordable. The only issue I had was getting the injectable form of methotrexate with a preservative (provided about 10 doses of 25mg). It was not supplied by many of the local pharmacies but they had the type without preservative which was a one-time-use (so there was a lot left over but I couldn't use it the next week). They all have the same copay with my insurance (about $15) but I had to pay a little more on injectables because I had to purchase needles, etc.

Your GP can still talk with the docs at the Vasculitis Foundation as others have mentioned. Traveling a great distance doesn't always make the most sense (time, cost getting there, cost staying overnight, and missed work). I recommend asking your GP to consult with the Vasculitis Foundation. It's what they're there to do! They want to help us get the best care.

[bgifmo]

Lag713, that's a relief. My insurance is pretty good, so hopefully it will be similar. I'll look more into the Vasculitis Foundation.
I'm hoping to find a good team here in Rochester, but it's good to have some references in driving distance if that's what is required.

[BookNut]

I am also from the Rochester Ny area. I was briefly diagnosed with Wegeners...but got a second opinion and it was ruled out. I go to a group that is associated with Rochester General. The doctor who evidently misdiagnosed me was Dr. Arango. I also go to an asthma/allergy specialist in the same group....Dr. Bingemann. when I decided I wanted a second opinion before I started on Imuran, Dr. Bingemann referred me to Dr. Michael Nead, a pulmonologist who is in the URMC system. He ruled it out based on symptoms, lab tests and a bronchoscopy. I have continued to get worse as the months have progressed, so decided to make an appt at the Cleveland Clinic, which is near where my daughter lives. I have not been there yet. When I do get there, they will probably think I am a hypochondriac. My symptoms have greatly improved in the past three weeks. I feel almost normal again, after three years of getting progressively more short of breath. It might be because my GP prescribed a long term antibiotic. Or perhaps my last round of prednisone was particularly effective. At any rate, I think it is always best to get a second opinion, so you are certain before starting some serious drugs.

At any rate, you have found an amazing group of people who will be a tremendous help to you. Good luck!

[drz]

The next time your doctor writes you a prescription, consider this: The medication (http://www.webmd.com/drugs/index-drugs.aspx) may not be approved for your specific condition or age group. Rituxan was widely used for treating GPA long before it became approved for this use.

But you probably shouldn't call the medical board. The practice, called"off-label" prescribing, is entirely legal and very common. More than one in five outpatient prescriptions (http://www.webmd.com/drugs/index-drugs.aspx) written in the U.S. are for off-label therapies.

"Off-label" means the medication is being used in a manner not specified in the FDA's approved packaging label, or insert. Every prescription drug marketed in the U.S. carries an individual, FDA-approved label. This label is a written report that provides detailed instructions regarding the approved uses and doses, which are based on the results of clinical studies that the drug maker submitted to the FDA.

“Many people may be surprised to know that the FDA regulates drug approval, not drug prescribing, and ... doctors are free to prescribe a drug for any[reason they think is medically appropriate],” says G. Caleb Alexander, MD, MS,a medical ethics advocate and assistant professor of medicine at the University of Chicago Medical Center. "Off-label use is so common, that virtually every drug is used off-label in some circumstances."

[mishb]

Hi Brian and welcome :mellow:

You are getting and will continue to receive excellent advice from everyone here.

Best of wishes to you for a journey that is not too long and becomes 'pain free'

[annekat]

All of my meds (CTX, MTX, prednisone, Bactrim) have been "generic" (same as off label, I guess?), and have not cost me much. I'm currently on a Medicare drug plan and paying $11.20 for MTX and nothing for pred or Bactrim (we may use the brand names on here even if using generics; the one for Bactrim is long and hard to type). If it were decided I needed rituximab, or RTX, that would be a different story, as it is given as infusion and horrendously expensive; if it were covered by Medicare, there would no doubt be a copay that I could not afford, and I might have to look into the company's program for low-income funding of the treatment. If you have good insurance and can afford whatever copay, this might not be a problem. At this stage, though, it sounds like you could get by with MTX or one of the other old standards and would likely do quite well.

[drz]

Off label does not mean generic. It refers to FDA approved uses for the medicine prescribed.

[annekat]

Thanks, drz.

[bgifmo]

Booknut, do you have a new diagnosis? I'm also seeing a doctor at RGH, in the same group as your docs. I'm anxious to see my GP tomorrow to hear his thoughts, and to see if he also recommends a second opinion. At the same time, I don't want to delay treatment for something that can progress quickly and be permanently damaging. But I don't know how long it will take to get to see a second doctor either. I was lucky to get in to see this doctor in 5 weeks instead of 3 months. It's all very scary and confusing. At the very least, I can ask for more testing as others have recommended here (chest CT), just to know what exactly I'm dealing with. I hope to get more answers and more clarity this week.

[WG mom]

Brian I sent you a PM, but don't think I sent it correctly. I am in Rochester this weekend for a wedding. My daughter's friend with whom I spoke last night has Lupus and sees a rhuematologist through U of Rochester Med Center ( who she says is fabulous). He does treat Wegeners as well as other AI diseases. His name is Dr.Richard Looney. I believe he is accepting new patients. I would seriously consider contacting him for a second opinion.

[bgifmo]

Hello WG mom,
Well, this is great news. I actually got an appointment with Dr. Looney last Friday for a second opinion, so this is very encouraging to hear.
The diagnosis was the same, but he did say that I had the "least bad" case of Wegener's he had seen. (Yay!?!?)
I felt comfortable with him and I'm switching my care to his practice.
Thanks for contacting me!

[WG mom]

I am so glad you were able to get an appointment with Dr. Looney. He comes highly recommended so that should be of some comfort. Not sure what he means by "least bad," but I know right now my daughter's is localized in her sinuses which I'm told is good for an early remission. She'll have her last rtx drip this week and I'm hoping she will soon experience some relief.
Best wishes to you, Brian. Please keep us all updated on your treatment progress. And keep checking in with all the great folks here on the forum. They have been a fantastic source of information for me and know they will be for you as well.
Keep moving forward...

[keegan55]

It depends on the severty of your gpa. Some are lucky and it is limited to one area. This group has been so helpful with so much info. I read but dont reply to much hate typing lol. I hope you have engaged a kidney dr and an ENT a good one that has had dealings with GPA.There has been so many advancements with GPA that we can expect longer lived etc.I hope your rhuemy is a specialist in GPA .If in lungs You also need pulmonary dr stay strong you eill do very well

[JeanMarie]

Welcome & sorry you are here but hope we can help!!
I'm in agreement with some of the other folks here--as soon as I saw only prednisone & not seeing your rheumy again for six weeks my hackles went up. There is a Vasculitis support group in upstate NY--listed on the foundation support site--vasculitisfoundation.org :rolleyes1:

[Jayne 14]

Welcome to the gang
Best place for help is right here
I had a short wait for the specialist to prescribe MTX so initially started with pred : that's just how it was , being referred from 1 guy to the next : hold on in .
I think the waiting is tough
You just want to get going but you will
And any treatment we get takes a while to get working & all the support is here
Good luck
Big love to u & our whole gang from the U K 👍


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[Velma]

Hello from WV.. I am a week in on this support group and am very pleased to have found a home to vent, give and get support as well. I was diagnosed when I was 23 years old and have battled it for 20 years now. The main advice I would give you would be the following: #1 fight for yourself and quality care--ask questions and never let interns work on you .. you are not a guinea pig, #2 be prepared to interview doctors and ask them what they know about WG (you want some one who is willing to work with a team as this disease usually effects ears and lungs and/or kidneys.. you need a good team).. anybody can look to see what is generally done for us with this disease.. you want someone with experience dealing with it and who will fight to help you. #3 be prepared for flares.. it will come and go into remission.. don't think any one medication is a cure all.. reality is you may go years just fine and then night sweats start or a cough begins.. medications may have to be changed or adjusted.. this is "normal" #4.. follow your instincts. You will begin to become your own doctor and keeping a journal or calendar to write down dosages of meds and symptoms will help you better help your doctors and the details will help more than you think. #5 communicate with your wife and doctors and family about how you are feeling. Prednisone may make you snappy (especially higher doses).. look up meds they want you to take and know the side effects. But most of all.. know that we are here for you and your family. The medications have come a long way in 20 years and I am still doing good. I have ups and downs as we all do.. but I am still alive. You will be okay. It will be hard at times.. but you will be okay. Prayers for you and your family.. and if you have any questions please feel free to ask. Someone here has surely experienced what you are feeling and going through. Good luck and keep us posted.

[Miranda]

Welcome! And sorry you are having to join us. I think you will like it here. I have found so much helpful information and real true experiences. Don't look too much into the internet or it will drive you crazy and scare you even more. There are a lot of people on here who have been through it all and give lots of good advice. On the doctor thing, If I were you I would get a doctor you are comfortable with and know you can trust. Second opinions are good. I got my second opinion a few months ago after having it for 6 years and things started turning on me. Best decision I made so far. Best of luck to you!

[BookNut]

Booknut, do you have a new diagnosis? I'm also seeing a doctor at RGH, in the same group as your docs. I'm anxious to see my GP tomorrow to hear his thoughts, and to see if he also recommends a second opinion. At the same time, I don't want to delay treatment for something that can progress quickly and be permanently damaging. But I don't know how long it will take to get to see a second doctor either. I was lucky to get in to see this doctor in 5 weeks instead of 3 months. It's all very scary and confusing. At the very least, I can ask for more testing as others have recommended here (chest CT), just to know what exactly I'm dealing with. I hope to get more answers and more clarity this week.

I am sorry it has taken so long to get back to you. I have been away....trying to find answers at the Cleveland clinic. Still do not have a firm diagnosis. I am not impressed with my treatment here. Too long a story to,get into. Dr. Bingemann keeps referring to my condition as my "asthma-like" illness. The doctor I am going to here does not really seem to care what happens to me. The test he lined up for me required me to go off my meds. I ended ip with an epic asthma attack that landed me in the hospital before I could even take the test. You would have thought this would have motovated him to see that I got prompt treatment. Not so. -10 days later I am still having to call every day to get him to call me back. Supposedly the new test....a methacholine challenge test....will be rescheduled very soon. I am feeling much better because of large doses of prednisone, so I should be able to tolerate the test this time. He also told me today that he thinks Ishould go to the Asthma Center. Passing me off I guess...bit It actually looks good. If only he had suggested it at the beginning I might have avoided a lot of grief. Anyway, I plan to see what results from the test, and then I am going to give it all up and go back to my other doctors. Maybe go to the Asthma Center in the future. I. am just really tired of being short of breath almost all the time. It has certainly messed with my quality of life. Up till three hears ago, I had no breathing problems at all. Frustrating.

[Velma]

Do you think the care is better at Cleveland? I live in WV and have exhausted all the docs here and went to Pittsburgh but they have me in a bigger mess so now looking at Cleveland. What is your opinion?

[Allisonquast]

I
I agree that your doc should consult with those at the Vasculitis Foundation. It would be a mistake to wait 6 weeks to start taking medications to actually treat the disease given that many drugs take months to go into full effect. Prednisone makes you feel better by bringing down inflammation but it only treats the symptoms, not the disease. Given your symptoms, I think methotrexate would be most appropriate but that's something to discuss with your doctor. Cellcept has a higher relapse rate compared to methotrexate.

I would recommend that you get a CT scan to see if any damage has occurred in your lungs, urinalysis and blood work to check your kidneys, a hearing test to see if your hearing is intact, as well as an endoscopy of your sinuses, trachea, and upper lungs. I was just diagnosed in January and I've had the workup. Best thing to do at this point is establish a baseline and preserve the functioning you have by getting ahead of the disease - prevention is invaluable. Don't let the information on the internet scare you. Let this be an opportunity for you to take charge of your health and well-being. You can live a long and healthy life if you are proactive about treatment, checkups, treatments, etc. It is a chronic, lifelong illness so you will need to acknowledge that the disease can become active again at any time and you will want to be on top of it.

I know of Dr. Shapiro (rheumatologist) and Dr. Lebovics (ENT) in NYC but that would be a trek from Rochester. I make the trip from central Connecticut every 3 months to see Lebovics because of his expertise - he sees a lot of weggies every week whereas my local rheumatologist and pulmonologist have seen less than 5 each in their careers. Dr. Lebovics put me at ease because he is very knowledgable. Given your location, there may be docs in Canada that would be easier to access.

I recommend the resources collected as "stickies" in the General WG Chat forum. I'm sorry that you received this diagnosis and are floundering through the information online. You have support here. I know you must still be in shock. It took me a few weeks to really accept it as being real. It will take longer to come to terms with what it means. I still feel like I freak out whenever something goes wrong but I am learning to roll with the punches and accept what is out of my control.

Welcome to the forum, you will get such good support and information on this forum, this is great advice. When my husband was diagnosed we put blind faith in our doctors, with this disease that's not always the best approach. You have to be knowledgeable and advocate ( sometimes) quite fiercely for yourself! read, read, read and ask questions of your medical team. Read paragraph two above again - it's great succinct advice!

[BookNut]

If you read my post above you will see how discouraged I am with Cleveland Clinic. However, there are MANY on this forum who are very impressed with them. The Dr. I have seen most often recommended is a Dr. Ville Forte. Not entirely sure of spelling. She is a wg expert. I do not have WG. Just garden-variety asthma, albeit a life changing version. No one here seems at all interested in my case. Fortunately, I have great doctors in Rochester, NY. I just thought that because I was going to be near Cleveland this summer for the birth of our granddaughter, it would be a good idea to see if I could get a different perspective. My biggest lesson was that my home town doctors are better. But again....the WG care appears to be excellent.

[drz]

Do you think the care is better at Cleveland? I live in WV and have exhausted all the docs here and went to Pittsburgh but they have me in a bigger mess so now looking at Cleveland. What is your opinion?

How about Philadelphia where Merkel is located? He is considered top shelf in Wegs.