Hello! :)

[Janish]

Hey Everyone! My name is Jane and I'm from Prince Edward Island, Canada.
I was diagnosed with GPA at the end of September. It started in August with what seemed like an exceptionally awful and painful ear infection with gradually worsening hearing loss, eventually ending up with near total loss. I was referred to an ENT, who decided to do surgery on my ears to drain the fluid and put in tubes - but when he tried to do the surgery, he found it was "like nothing he'd seen in 30 years of practice" and ended up taking a biopsy which led to my diagnosis. I met with a Respirologist, and I'm a little more than 2 weeks into treatment now (Methotrexate and Prednisone) and I've had improvement with my hearing in my left ear, and my ENT is confident that the other will get better once the fluid drains, which I'm very happy about.
Since my diagnosis, I am finding that I honestly have no idea what to expect. The doctor explained a bit about what GPA is, and what the medication is supposed to do - but I am at a loss as to what to expect in day to day life as someone living with it. I suppose that all I can do is carry on like I'm normal, and deal with things as they come. But are there things that I should be preparing for? Are there things that I need to know about that may not have been brought to my attention? I'm feeling really in the dark!

[Aneinu]

Sorry to hear about all this.
It is scary and confusing.
Many here go on to lead somewhat normal lives.
The disease is tricky so you need to be an educated consumer.
The group here is wonderful and provides tremendous assistance.
Obviously, good doctors are crucial.
Wishing you all the best.

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[Gab122]

Hi janish, your situation was very similar to mine. Tremendous ear pain and pressure, then ear tubes that revealed tremendous amounts of ear drainage. It took me 3 months and blood tests revealed high sed rate, c-rp and rheumatoid factor. That led to a positive ANCA test and lung biopsy. That was the first week of Aug that I got dx. Feels like a year ago. I'm in NH. Not far from you - well not worlds away. [emoji4] I'm on prednisone (60 mg at first, tapered to 30 now) and Rituxan infusions. My ear pair and drainage went away but I think I feel a little "activity" in my ears still. Everyone is different but I have shortness of breath when I exert myself at all. My treatment has proven very successful so the symptoms may be from the prednisone. I'm very new to this dx like you but there are many here in this forum with many years of experience. I'm sure they will chime in. In the meantime try to keep away from sick people. Are you on Prilosec to protect your stomach and calcium/with d to protect your bones (prednisone comes with side effects ).

Do you have a rheumatologist experienced with GPA? Have you had your lungs and kidneys ct scanned for disease? These are typical areas checked for GPA dx.

Welcome to the forum. Sorry you have this disease!!!
Wishing you the best!!
Gab

[Alias]

Hi Jane, your story is similar to mine. I started with sudden hearing loss, which was treated as an infection. I ultimately had three procedures culminating in a mastoidectomy. My next symptoms involved my nose and sinuses. Like you, I was diagnosed relatively quickly after someone suggested I get worked up for GPA. We can consider ourselves lucky, since many people go for quite a long time without being diagnosed. Also, it sounds like you have no vital organ involvement. (Confirming that usually involves a chest x-ray or CT to rule out lung involvement and a urinalysis to check for kidney involvement, I think.) Sounds like you are on a good track. It is a bit overwhelming to suddenly find yourself having to manage a rare, chronic disease though. You can find lots of good information through the website for the Vasculitis Foundation. If you email Joyce Kullman with VF, you can ask her for a copy of their free book on GPA and MPA. It has good, basic information on what the disease is, how it is treated, etc. It's also a good reference for family and friends who may want to know what is going on with you. It's probably too early to say how you will be affected in the long term. I ended up with permanent hearing loss and tinnitus in one ear, damaged nasal passages and eustachian tube issues. Now, more than two years after diagnosis, my symptoms are pretty minor (though still very annoying at times) and I continue with methotrexate as a maintenance medication. It is common practice to have periodic labs to monitor for disease activity and check for side effects of any meds you are on. I hope you are able to get adequate specialty evaluation there on PEI. A rheumatologist with experience in treating GPA is the specialist usually considered the best authority. If there's any question of you getting appropriate work up and getting set on a good course of treatment, you might consider travel to a larger medical community for another opinion, but from what you've said it sounds like you are doing well.

[Middlesista]

Hi Janish - some great suggestions here. Do make sure you have a Vasculitis Specalist overseeing your care. The Vasculitis Foundation has a good list for resources. As Alaskatom and Gab mentioned an RA usually oversees care.

Proper testing is vital - CT is better than CXR to find lung disease. My CXR was clear but my CT showed damage from GPA. Ongoing labs including inflammatory markers such as sed rate and CRP as well as renal labs such as BUN and creatinine as well as CBC are usually checked. ANCA markers are also commonly done although some folks have mentioned they can be inconclusive. A biopsy is felt by some to be the primary means for diagnosis although I have also read that sx as well as positive blood work can also be the basis to start treatment. The drugs used to treat our disease are serious business so most docs feel a definitive diagnosis is a must. With that said - I had a needle biopsy of one of the lesions in my lung, doc ended up accidently cutting an artery resulting in some serious complications and to add insult to injury the specimen was not useable due to all the blood. My docs went ahead with the treatment based off my blood work and symptoms.

You might want to keep a symptom diary and correlate with med changes and lab values. Even with a stellar medical team to oversee your care you have to be vigilant in your symptoms and self monitoring. I know some folks with visible signs of GPA activity take photos.

Again - I can not stress enough the importance of a team of doctors that are well versed in GPA. Every team needs a captain and your RA is usually that person. If you feel you need a second opinion seek one out. A good doc will support this

I am sorry that you are dealing with all this but I am glad you found the forum - lots of great info from some very knowledgable and supportive people and I truly feel blessed to have found them.

[Alias]

BTW Jane, you probably know this by now, but a common course of treatment involves a tapering course of prednisone to tamp down inflammation, and an immunosuppressant, such as methotrexate, which is what your doc has initiated. If you have "limited" or "non severe" (both terms are a little misleading) GPA, meaning no lung or kidney involvement, you may not need anything else. Prednisone side effects are a bummer, but as you taper things will improve. It is important that you have a trusted physician to monitor your progress. Again, based on what you've said in your opening post, it sounds like you are responding to treatment. Hopefully you will be in remission soon. Best wishes....

[annekat]

Welcome, Jane! Great advice has already been given. I also started out with a severe ear infection and hearing loss, followed by many sinus infections which were attributed to allergies. So I was not diagnosed until a couple of years later when it went into my lungs. You are lucky to be dx'd and treated so soon after your symptoms started, and it minimizes the chances of permanent damage, and you may get your hearing back. I'm doing OK now, it's well under control, but I'm still on meds, the same ones as you are at this point. I recently was found to have an inflamed esophagus and some ulcers due to acid reflux, when I should have been on an acid blocker all along, and I'm now taking one and doing a lot better. This situation led to bleeding in there, which made me anemic, and I'm taking iron pills and improving steadily. Just one aspect of the complications of the disease, which someone mentioned above. Everyone's story is a little different, everyone reacts a little differently to the meds, etc. Getting on this forum is about the best thing you could have done! I've been here since my dx, checking in faithfully every day, and know so much more than I ever would by just talking to doctors. The more you read and hang out here, the more of an overview you will get, and the less anxious you will feel. Feel free to vent when you want to, ask any question, go into any amount of detail on your experience, browse the archives with the Search function, and send private messages to anyone on the member list. Best wishes that things will fall into place and you'll feel better soon!

[drz]

My advice would be to carry on but be open to the idea that new symptoms or other health issues may relate to the side effects of your treatment. Ask the doctor what to watch for and what to report to them so you know what to report to your treatment team. They need your feedback to help plan and adjust your treatment so it is most helpful to you. Some side effects are to be expected and and others mean there is a need for some change. Only time can tell how the GPA and its treatment will affect you. Most things we worry about never happen so don't borrow trouble that may never happen to you.

[Janish]

Sorry to hear about all this.
It is scary and confusing.
Many here go on to lead somewhat normal lives.
The disease is tricky so you need to be an educated consumer.
The group here is wonderful and provides tremendous assistance.
Obviously, good doctors are crucial.
Wishing you all the best.

Sent from my SM-G920V using Tapatalk

Thank you, Aneinu.
I am already finding out what an amazing and supportive group is on here, and I'm blown away!
As far as having good doctors, how can I be confident that I'm working with good ones?

[Aneinu]

Thank you, Aneinu.
I am already finding out what an amazing and supportive group is on here, and I'm blown away!
As far as having good doctors, how can I be confident that I'm working with good ones?
You can find out if they are specialists in their field.
Google does a great job as well.
There are services that list and rate doctors.
References.
Mostly, you have to trust them, so do your research.
Best of luck and keep us posted.
We are always learning new things from this wonderful group.

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[Janish]

Hi janish, your situation was very similar to mine. Tremendous ear pain and pressure, then ear tubes that revealed tremendous amounts of ear drainage. It took me 3 months and blood tests revealed high sed rate, c-rp and rheumatoid factor. That led to a positive ANCA test and lung biopsy. That was the first week of Aug that I got dx. Feels like a year ago. I'm in NH. Not far from you - well not worlds away. [emoji4] I'm on prednisone (60 mg at first, tapered to 30 now) and Rituxan infusions. My ear pair and drainage went away but I think I feel a little "activity" in my ears still. Everyone is different but I have shortness of breath when I exert myself at all. My treatment has proven very successful so the symptoms may be from the prednisone. I'm very new to this dx like you but there are many here in this forum with many years of experience. I'm sure they will chime in. In the meantime try to keep away from sick people. Are you on Prilosec to protect your stomach and calcium/with d to protect your bones (prednisone comes with side effects ).

Do you have a rheumatologist experienced with GPA? Have you had your lungs and kidneys ct scanned for disease? These are typical areas checked for GPA dx.

Welcome to the forum. Sorry you have this disease!!!
Wishing you the best!!
Gab


Hi Gab!

Thank you so much for the warm welcome and well wishes! Wishing you all the best as well with your own treatment and journey!
I am not taking Prilosec. My doctor didn't prescribe anything for my stomach at this point. I am taking Calcium and Vitamin D though. I've had my chest X-rayed and I have an appointment booked for December for a CT Scan of my sinuses. I had some blood and a urine test immediately upon diagnosis, so I believe that's how she checked my kidneys. I'm also having blood tests weekly for the next few weeks, and then bi-weekly for a while.
I don't currently have a rheumatologist - but it's sounding like that's someone I need to be seeing. Definitely have to speak to my doctor about being referred to one.

[Janish]

Hi Jane, your story is similar to mine. I started with sudden hearing loss, which was treated as an infection. I ultimately had three procedures culminating in a mastoidectomy. My next symptoms involved my nose and sinuses. Like you, I was diagnosed relatively quickly after someone suggested I get worked up for GPA. We can consider ourselves lucky, since many people go for quite a long time without being diagnosed. Also, it sounds like you have no vital organ involvement. (Confirming that usually involves a chest x-ray or CT to rule out lung involvement and a urinalysis to check for kidney involvement, I think.) Sounds like you are on a good track. It is a bit overwhelming to suddenly find yourself having to manage a rare, chronic disease though. You can find lots of good information through the website for the Vasculitis Foundation. If you email Joyce Kullman with VF, you can ask her for a copy of their free book on GPA and MPA. It has good, basic information on what the disease is, how it is treated, etc. It's also a good reference for family and friends who may want to know what is going on with you. It's probably too early to say how you will be affected in the long term. I ended up with permanent hearing loss and tinnitus in one ear, damaged nasal passages and eustachian tube issues. Now, more than two years after diagnosis, my symptoms are pretty minor (though still very annoying at times) and I continue with methotrexate as a maintenance medication. It is common practice to have periodic labs to monitor for disease activity and check for side effects of any meds you are on. I hope you are able to get adequate specialty evaluation there on PEI. A rheumatologist with experience in treating GPA is the specialist usually considered the best authority. If there's any question of you getting appropriate work up and getting set on a good course of treatment, you might consider travel to a larger medical community for another opinion, but from what you've said it sounds like you are doing well.

Hey Alaskatom,
I definitely do consider myself lucky to have been diagnosed so quickly. I had at points felt a little silly making trips to the emergency room for an ear infection (I don't have a family doctor), but never again! lol I'm thankful that I was taken seriously.
I appreciate the recommendation for the book. I will definitely look into that.
Did you hearing improve at all after treatment compared to its worst point?

[Janish]

Welcome, Jane! Great advice has already been given. I also started out with a severe ear infection and hearing loss, followed by many sinus infections which were attributed to allergies. So I was not diagnosed until a couple of years later when it went into my lungs. You are lucky to be dx'd and treated so soon after your symptoms started, and it minimizes the chances of permanent damage, and you may get your hearing back. I'm doing OK now, it's well under control, but I'm still on meds, the same ones as you are at this point. I recently was found to have an inflamed esophagus and some ulcers due to acid reflux, when I should have been on an acid blocker all along, and I'm now taking one and doing a lot better. This situation led to bleeding in there, which made me anemic, and I'm taking iron pills and improving steadily. Just one aspect of the complications of the disease, which someone mentioned above. Everyone's story is a little different, everyone reacts a little differently to the meds, etc. Getting on this forum is about the best thing you could have done! I've been here since my dx, checking in faithfully every day, and know so much more than I ever would by just talking to doctors. The more you read and hang out here, the more of an overview you will get, and the less anxious you will feel. Feel free to vent when you want to, ask any question, go into any amount of detail on your experience, browse the archives with the Search function, and send private messages to anyone on the member list. Best wishes that things will fall into place and you'll feel better soon!

Hello Anne,

I'm sorry to hear about the complications you've been experiencing, but I'm glad that you're steadily improving! I appreciate the tips on how to get the most of this forum! I am hoping to get myself well-informed so that I can keep on top of this - while trying to maintain a good amount of optimism! I am happy to be on this forum with you, and all these wonderful people, who are so helpful and welcoming from the start! It's unfortunate that we have to go through this, but it's nice that we have each other. :)

[annekat]

Hello Anne,

I'm sorry to hear about the complications you've been experiencing, but I'm glad that you're steadily improving! I appreciate the tips on how to get the most of this forum! I am hoping to get myself well-informed so that I can keep on top of this - while trying to maintain a good amount of optimism! I am happy to be on this forum with you, and all these wonderful people, who are so helpful and welcoming from the start! It's unfortunate that we have to go through this, but it's nice that we have each other. :) Very true that new friendships are a benefit of having WG and being on the forum. As for my complications, they aren't nearly as bad as what some others go through with WG. I just mentioned the digestive tract problems and resulting anemia because it is something to be aware of when using prednisone long term, and some are not adequately informed about the need to take acid reducers regularly. I hope you are one of those with a less severe case, and that you are on the road to remission.

[Gab122]

Janish another place to check for good doctors is http://m.vasculitisfoundation.org/
In case it hasn't been mentioned yet. Also if you follow the "contact us" tab on their site they'll invite you to join their Facebook group and connect you with others that have types of vasculitis. They will also send you a resource book about Weg GPA. I'm glad you found us and know you are not alone!

[Alias]

Not in my case, but every case is different. The conductive hearing loss in my right ear is quite severe, and unfortunately a conventional hearing aid does not work very well for me. I have seen a top notch otologist in Seattle and he says it is very unlikely I will get improvement with time. A bone anchored hearing aid is probably in order but I am not up for the invasive procedure at the moment.

[drz]

Not in my case, but every case is different. The conductive hearing loss in my right ear is quite severe, and unfortunately a conventional hearing aid does not work very well for me. I have seen a top notch otologist in Seattle and he says it is very unlikely I will get improvement with time. A bone anchored hearing aid is probably in order but I am not up for the invasive procedure at the moment.

The newer procedures for installing a BAHA are a bit easier and not as noticeable as old school versions and they are are also easier to maintain the site afterward. I got the the old school version five years ago but it dramatically improved my hearing and quality of life even though it was not done perfectly so I had extra problems with infections afterwards. I still ma very glad to have it.

Go try out one by wearing the aid held on your head with a rubber band and see how it works for you. I couldn't believe how well it worked when I first tried it years ago. A good audiologist that works with BAHA's can help you evaluate if it would help you?

[annekat]

Alaskatom, I have the same hearing configuration as you, left ear easily correctable with an aid, right ear deteriorated to where the aid doesn't help. Yet if I hold a phone right up to it, I can hear it without the aid, though not well. So I haven't given up on it. Like drz, I tried out the BAHA by having it strapped to my head, and thought it seemed pretty satisfactory. I was told that Medicare covers it because it is a prosthetic device. But I'm pretty sure there'd be a copay that I'd have trouble affording. I'm also put off by the invasiveness of it. So I'm putting off any decision about that for now and getting by with one ear.

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[Alias]

Thanks for that Titus. I should look into it further. I am slightly spooked by the procedure and potential to make things worse than they are, though the risks are probably very small. I think I'm just not ready emotionally, but physically I've probably been stable on my meds long enough that the docs would be OK with going ahead.

[Alias]

My left ear is more or less normal, but for the annoying sensations caused by eustachian tube issues. I have reasonable "residual" hearing on the right, i.e. it could be restored to pretty good function with enough amplification. The problem for me is that I have congenitally small ear canals, and I make an unusually large amount of wax. So... getting the device to stay in and be effective is literally a pain, and it tends to compact wax, causing additional discomfort and muting the device. Kind of a no win situation there. In most environments I am OK, but crowds cause problems, and I have to take care as to where I sit when I'm in a group in order to direct the most sound possible to my good ear. Another annoyance is that, even at home, I cannot figure out where sounds are coming from. Like, if my cell rings, I run around frantically trying to find it; or if a dog is barking I can't tell which direction it's coming from, etc. I'd really like to have more normal hearing but am just not quite ready to move forward on the BAHA thing yet. I'm still enjoying just being more or less stable!

[Mrs.M]

Hi Jane! Welcome. In glad that you found us. Everyone has had great advice. My husband had required aggressive treatment to keep his WG in check. The best thing to do is to get a doctor who has treated the disease. In my experience, it is not enough to know about the disease. They need the experience of treating it. That doctor should have other experts that they feel comfortable working with, such as ENTs, etc.

Are you on an acid reducer? How about bactrum?

The biggest thing to remember is that you are immune compromised. Be exceptionally cautious about germs. Wash your hands - often. Do not touch your face. The best method I use is to immediately wash my hands when I get to my house. My family does the same.

Keep your appointments. Keep track of the symptoms. Have someone be your advocate. You're the patient. Sometimes you need someone to advocate for you - especially when you don't feel well.

Let me know if I can help in any way!!

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