New to WG.com from Houston TX

[TheMitchells]

Hello everyone,
To begin, I would like to say that this web site has been the most help for me and my family the last 5 days, since my wife was told that she has WG. I thank you for all of your stories. Y'all have helped us in a time that we really do need it.
Here is our story. Mary is my beautiful wife, I'm Brian, her lucky husband, and we have two boys. My wife has been having bad problems with her sinuses for a little over a year now. It first seemed that she was just having her allergies act up because of all of the pollen, ragweed, ect. in the air. Then about 6 months ago, she got a ear infection in her left ear. It had gotten so infected that it had gotten so swollen and cause her ear drum to form two holes on it. She was given medicine to help the infection, but it would still have fluid build up inside of it and never heal all the way. After this cycle happened for about 3 months, it spread to her right ear. Same problems, but the swelling never got as bad. I suggested to have a tube put in to prevent the liquid from building up in her ear. The doctor put the tube in her ear and thought it might prevent it as well. Two weeks later, there was no fluid in her ear, but two new holes had appeared on her right ear drum. That is when the ENT did more blood test, saw high numbers (ANCA 1:40H<1:20AI and Proteinase 3 antibody 5.9H<1.0AI). He suggested a rheumatologist and she went to see him for the first time last week (Tuesday). She said that he looked over the countless blood test, MRI and Cat scans that she has had to undertake the last several months and seemed to agree it was WG. She will not see him for another week. She has been taking 80mg pred. a day for 2 weeks until last Thursday when the RH took it down to 60 mg. I was not able to go to her first appointment with the RH and I can wait another 8 days to get some answers. I hope you all will take a minute or two to save me from feeling my head with the things that you can find, or can't, on the web.
Now here are my questions. From everything that I have read, I thought that the only way that you can for sure say that it is WG, you have to have a biopsy. Is that still the case? Neither doctor has suggested one yet. Next, I read that a lot of people have the sinus problems, but havent seen much about the holes in the ear drums and/or hearing problems. Has anyone else had their first symptoms similar to my wife? Does anyone have long term affects from the preds? What do y'all suggest about the treatment? Cytoxin just scares me to use. What should I ask the RH when I finally get the chance in 8 days? Does anyone know of a doctor that they know of in Houston or Texas that they see that they would suggest?
I could ask questions for another hour, but I will stop here. I'm sorry if I have said something or have been to blunt on anything that I might have said. I know that I'm not the one that has to live with WG the rest of my life, but I just want to help my wife in any way that I can. She is not the type of person to reach out for help like this, but I will. Everyone on this forum has some much more info than I can find on any other web site. I thank you all for your time and can't wait to read your valuable insight in the morning!

[renidrag]

Sorry you had to find us here but no better place to learn. We all can relate to your fears and wondering of the future. Although I do not have experience in ear involvement, lungs for me, the prednisone is one of the drugs used to ease symptoms however Cytoxan (ctx) is the heavy hitter in WG. It does come with a lot of risk, but also comes with rewards. That is what we have to decide. Methotrexate seems to be the drug of choice these days however. Biopsy is the best way to diagnose and Anca is really not an accurate measurement. Your story sounds very familiar as to many on here and others will be on soon to help you. My best to you and Mary and hope all works out well. There is hope.
Dale

[Psyborg]

Brian,

Biopsy is not really necessary, it is probably helpful, but those blood tests are pretty conclusive. Don't freak out based on internet based information. Much of what is out there is either dated, or more alarming than it should be. Mind you this is a serious disease, but if treated properly you will be a good spot. I strongly suggest you go to the Vasculitis Foundation website and see if there are any doctors within a reasonable distance to you. The biggest vasculitis centers are Mayo Clinic, Johns Hopkins, and the Cleveland Clinic, but those are all a bit distant for you.

CTX is a bit rough when taking it, but is the drug most likely to get the disease rapidly under control. At that point maintenance drugs like MTX and Imuran take over. RTX is another option and seems to be more tolerable than CTX for most.

[lag713]

Welcome! I'm glad that the forum has been helpful for you and your wife. I'll do my best to answer your questions based on my experience.

Biopsy for diagnosis - I have not had a biopsy but I was diagnosed using blood tests, CT scans, xrays, and symptoms. It was a process of ruling everything else out. No doctor has questioned the diagnosis. Like your wife, I had sinus symptoms (i.e. sinusitis, postnasal drip, rhinnitus, etc. for a long time).

Holes in eardrums - My ears have been relatively unaffected besides tinnitus. I can't speak to holes in eardrums.

First symptom in sinuses and ears - Yes, I had sinus problems but I thought it was just allergies. I also developed joint pain, malaise, fatigue, etc. Joint pain forced me to see a doctor and find a diagnosis.

Long term effects of prednisone - I have not taken prednisone but it seems like it doesn't have negative long term effects. However, many people with WG find that they need a very small maintenance dose to feel better. It changes the way your body produces certain hormones so it needs to be weaned off slowly so your body can start producing it again.

Treatment - There are a few options which are outlined nicely here (http://www.youtube.com/watch?v=j46fXp_aMiU&feature=youtu.be). It seems that she does not have any involvement in her lungs or kidneys so cytoxin would be an unlikely choice. I would assume her doctors will recommend methotrexate. I have sinus symptoms and that's what I'm taking currently.

Questions to ask - I would make a list. Write down a question whenever it comes to mind. They should be willing to answer any and all questions. Also, thinking prospectively, think about general questions about treatment and plans. E.g. Why do you recommend that form of treatment over the others? Side effects? When should I contact you (problematic symptoms)? What complementary treatments would you recommend to deal with her symptoms? etc.

I don't have any recommendations for your area but your local doctors can always consult with those at the Vasculitis Foundation. They're there to help create the best treatment plan for Weggies. We're uncommon and most doctors don't know how to treat us.

[MaxD]

Brian,

I had similar symptoms (horrible ear infections, but not the holes in the ear drum) starting August 2013, first in the ears and later roving joint pains until I was diagnosed with GPA (the current PC term for WG). In addition to my rheumatologist, I'm seeing two docs at the forefront of clinical trials and research in NYC. Based on my blood results and symptoms they were certain that the rheumatologist's diagnosis was correct and that I did not need a biopsy. Also, they strongly recommended Rituximab treatments - it is an advanced monoclonal antibody therapy that has been shown in clinical trials to be more effective than Cytoxan, and in a forthcoming report coming out this fall from another study also seems to be more effective specifically for sinus issues. Of course, what is true statistically over a population need not be true for every individual. However, the side effects of Rituximab (or Rituxan, the brand name) are extremely rare - I was apprehensive at first, but noticed no side effects at all in my 4 infusions. You should check about that treatment with your wife's doctors. The Rituxan web site will scare you at first, with all their obligatory listing of extremely rare, life-threatening possible side-effects, but talk with your docs and experts. Rituximab is an FDA approved treatment for GPA and is covered by many insurances - your docs/infusion center will check that for you.

As for the prednisone, yes there are likely long term effects, but the preds will be tapered down once the treatment starts. I started out at 75mg for a month, down to 60 when the treatments started and am now down to 15mg 3 months after treatments ended.

Check out the vasculitis foundation web site for much more information and for pointers to docs and clinics specializing in GPA. You can always consult with one of the docs for a second opinion or have your wife's docs consult with them.

Good luck to your wife and family.

[lag713]

Hey Max, Thanks for the info on rituxan. I asked my ENT about it as a possible treatment but he said that I shouldn't go on it with my sinus, ear, and joint symptoms. He explained that the long term effects are not known as it is still a relatively new drug. Your physician prescribed rituxan because you had nodules in your lungs in addition to sinus, ear, and joint symptoms, correct?

[Pete]

Hi Brian,

One of the questions you should ask your wife's rheumy is how many vasculitis patients s/he sees. If the number is small, you should ask him to consult with a vasculitis specialist. Here's the link to the VF consultant list: VF Medical Consultants

Good luck. Hope your wife is feeling better soon!!

[MaxD]

I did have a nodule in the lung, but that was a minor consideration as the symptoms were mainly in the sinuses and joints, plus one episode of Bell's palsy that was probably related. Rituxan has been used for over 10 years in the treatment of B-cell non-Hodgkins Lymphoma, but I haven't looked into longer term effects. I haven't come across any significant side effects that occur within 10 years.

Dr Spiera who I see for second opinions from time to time, and Dr Lebovics, the ENT who specializes in GPA are both coauthors on the upcoming report on the effectiveness of Rituxan for sinus problems in GPA patients. I'll post a note with a link to the report when I get it. You could bring it to the attention of your ENT if you wish.

[lag713]

Dr. Lebovics is my ENT. KimH and I recommended Dr. Lebovics and Dr. Spiera to you when you first joined the forum. I guess we look very different in person which merit different treatments. I look forward to reading their publication.

[annekat]

Welcome, Brian, and I'm glad you found us. With me, it was the big ear infection first, in both ears, including holes in the eardrums. That was followed by a couple years of sinus infections, attributed to allergies, which I was tested for and got shots for for a year and a half or so. Then it went into my lungs pretty badly and that's when I got diagnosed. The doc in the hospital suspected WG from the looks of my CT scan there, but my ANCA reading was low, and the actual dx was from a nasal biopsy done by my ENT. But I've read on here that nasal biopsies are often inconclusive, and that lung biopsies are better, but since your wife has no lung involvement at present, that doesn't seem like an option. I think a nasal biopsy would be a good idea, but if it doesn't test positive, WG should still not be ruled out, and docs will sometimes go ahead and dx WG without a positive biopsy, based on symptoms and other tests.

As for meds, I took CTX because I had lung involvement. That was cleared up, but the sinus involvement takes longer to clear up, largely because of permanent damage that may have been done to the tissues and bone structures. So now I'm on MTX for long term maintenance. At least they caught this a bit earlier for your wife than they did for me. That could help a lot. Anyway, it does seem more usual to use MTX when the involvement is mainly sinus, ear, and nose related and not in the lungs or kidneys. The heavier hitters like CTX or RTX can be saved for if things get worse in the future. CTX is indeed a very potent drug and there is limit on how much one should use over a lifetime. I did not find it all that problematical, though, in terms of side effects and such, and made sure to drink lots of water everyday to keep it from building up in the bladder. Hopefully, your wife won't have to deal with that because they will put her on MTX. It also has some side effects, which can be gotten used to, and liver function must be checked regularly. All the meds have some issues of possible side effects and toxicity. I can't talk much about RTX because I haven't used it but know that some here have been very happy with it and others have had some issues. It takes a little time to work, for most. It is very expensive and must be given by infusion; I like being able to just take a pill and not go anywhere. Since I've probably taken my lifetime quota of CTX, I'll save RTX for if things get really bad in the future.

I hope your wife can get started on some real treatment soon, in addition to the prednisone, and that you have or can find a doctor that you have faith in, who will agree to consult with an expert on the VF list if necessary: VF Medical Consultants . Keep us posted!