New Here - Possible GPA with Nasal involvement

Hi everyone, my name’s Rae. I'm currently having tests for possible Wegener's because of scabs on my septum that i had for years and some symptoms that I've been having for the past 6 months including night sweats, palpitations and a bounding pulse.
My nose has had a slight dip at the mid vault/internal valve area on one side for a couple of years but I've noticed recently that this is much more obvious now. Could this be my nose collapsing? There is no dip over the bridge or on the other side yet.
My nasal breathing isn't great. At times I feel like there's a loss of sensation of breathing in my nose and my automatic breathing had all but disappeared and I feel like I'm manually breathing. Other times it feels congested and hard to get air in. My nose inside is not very sensitive at all and I am able to covid swab my nose with absolutely no flinching or discomfort. I also jolt awake at night almost like apneas. I have had pain in my left trap muscle for the last 5 months and had a period of neck pain over the carotids which has now gone away. An ultrasound was fine.
Over the course of the months I've had several ESR tests and c-reactive protein tests and levels have never been raised. I'm awaiting results from specific GPA blood tests and have had CT scans requested. I have no problems lung/chest wise and had a 24 hour urine test done which came back fine. All full blood count levels were also fine. The nasal crusting has cleared up with Naseptin cream. Could this be GPA? Thank you

GPA can cause a variety of symptoms and can be very difficult to diagnose. It is important that you persist in your efforts to get an accurate diagnosis and treatment for whatever is creating your symptoms. If your symptoms are rather unusual you may have to resort to going to one of the few places in the world where they have considerable expertise at diagnosing are rare diseases such as GPA.

I did have crusting in my nose for several years before it eventually evolved into a full-blown serious flare of GPA which cause great damage to my body including kidney damage, lung damage loss of hearing, loss of balance, and increased neuropathy. I had various other symptoms which in hindsight were typical for GPA but none of the various specialists at different clinics ever put it together until I developed a very serious flare and it still took a consultation with Mayo to figure out what was attacking my body.

Good luck with sorting out a diagnosis for your situation and getting the care you need to address the situation and improve your health.

Thank you drz. In all honesty I’m praying that it turns out not to be GPA. I have been reading a lot of diagnosis stories on here and you are all so brave coping with all the terrible symptoms that can come along with GPA.

drz,

How did your hearing loss come about? Asking because all the ear problems I'm starting to have. How did it start and progress?
-Jeannette

Rae,
I agree with drz. Keep at it and don't give up. Try different doctors or clinics if you are not getting anywhere. Hopefully it's not Wegeners. Be persistent and you'll get to the root cause. Pay close attention to your body symptoms. Tracking your symptoms in a journal sometimes helps. I had to do that because I would forget after a while. Also, take pictures of any spots or signs of inflammation (edemas) on your skin. I had red blotches on my legs and edemas in my joints.
Good luck and let us know how we can help.

The journal is a great suggestion. It doesn't need to be elaborate either just brief notes help a lot later when memory starts to fade on how things happened.

A Google spreadsheet was my documentation of every symptom and doc visit. It has become my journal I can update from any place and share with any doctor when needed.

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Originally Posted by jmq

drz,

How did your hearing loss come about? Asking because all the ear problems I'm starting to have. How did it start and progress?
-Jeannette

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My first hearing loss began in childhood when a very bad Dentist blew out my eardrum by injecting Novocain into my eustachian tube. I went swimming with the perforated eardrum and got a serious ear infection. Recurrent infections due to a perforated eardrum led to a recurrent cholesteatoma that gradually dissolved all of the bones in my middle year and created a serious conductive hearing loss. This process took place over many years and there were several operations to try to repair the damage and restore the hearing without any luck.


The hearing in my other ear was normal until my GPA had a serious flare that almost killed me. It had attacked my kidneys and lungs so I had blood in my urine and was coughing up blood. Then one morning I woke up deaf. My balance also seemed impaired. At that point my GPA had not yet been diagnosed and it took over another week until I could get transferred to a bigger hospital where they had the experience and expertise to confirm the diagnosis of GPA. They tried to restore my hearing with mega dosage of steroids including direct injections into my eardrum but it was to no avail.


Before I could get a BAHA operation to restore some of my hearing I had to attain a drug induced remission from the GPA for at least a year so it was about two years after my GPA diagnosis was confirmed that I was able to regain some more adequate hearing. My only hearing is on the inside of the ear that has no conductive mechanism but the BAHA mechanism will pick up the sound and transmitted through my skull to the inner ear on the other side. I also have a behind the ear hearing aid on the ear with no conduction which does give me some limited hearing. My speech showed a significant deterioration before I regained some more adequate hearing and then it took several months to improve back to the level before the hearing loss.

Hearing loss is a very debilitating condition and many research studies indicate the isolation and depression created from hearing loss is even greater than from the loss of vision. Probably because people do not make jokes about blind people and are more willing to help them.

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Originally Posted by Rae82

Thank you drz. In all honesty I’m praying that it turns out not to be GPA. I have been reading a lot of diagnosis stories on here and you are all so brave coping with all the terrible symptoms that can come along with GPA.

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Welcome, Rae. I, too, hope you don't have GPA. But if you do, know that some have fairly mild, treatable cases, and you could be one of those. Also, it seems that you are getting it investigated relatively early, before it might start to involve the other typical areas such as lungs and kidneys, and cause damage there that would have been mitigated by treatment. I had ear and sinus involvement for a couple of years before diagnosis. Even though I had a good, experienced ENT, he has many cases of severe recurring sinus infection, often caused by allergies, and I seemed no different than those. So I was coasting along with allergy shots and repeated short courses of antibiotics and prednisone, which worked great to clear things up temporarily. But eventually it went into my lungs and I was diagnosed. It has been nearly 10 years since then, and it's pretty well under control, but I am still on a moderate dose of maintenance meds. I did get saddle nose right around diagnosis, which maybe you can avoid with prompt treatment, if they find you have GPA. My lungs were not as bad as some people's, but the damage caused early on is mostly still there and has affected my breathing permanently. With a prompt diagnosis, this might not have happened. I am lucky to have had no kidney involvement, and very little joint pain. I do have a fair amount of fatigue. New symptoms can crop up any time, but the maintenance meds will help prevent that. I must have regular CT scans and bloodwork to make sure things are on an even keel. All this is trying to be encouraging that if you do have GPA, you may be able to limit the severity with prompt treatment. It doesn't really go away, but I think people with early treatment will go into an earlier and longer lasting remission. It is manageable and may not affect your life to any great extent if you keep on top of it and stay in good health otherwise. Best of luck, and be sure to let us know how it goes. I still hope you don't have it, but if you do, you have come to the right place!

Thank you Anne.
I have had results from my specific vasculitis tests - are they called anca tests and they have come back negative. I’m still awaiting CT scan results. My ESR and C-reactive protein are also negative again. These have never been raised and I’ve had them done about 5 times now in the last 6 months. I’m thinking that it may not be GPA after all but am awaiting another appointment with the ENT consultant I am seeing.

Rae, I am one of the 20% who have GPA who have never had a positive result on an ANCA test. Or at least it is so minimal that it’s not considered positive. I don’t know the explanation, but it’s something to keep in mind. The other tests, though, should be showing something, though I think some of them can take some time to show up. I hope this means you don’t have GPA. If the ENT thinks there’s a place in your nose that would suggest a sample for a biopsy, he could do that. Mine did it right in his exam chair with a topical anesthetic, and it wasn’t bad. That’s how I got my dx, although they can give false negatives. Maybe you just have a deviated septum, which could explain the dip on one side. Those run in my family. Good luck!

Yes this does concern me a little but with the septal scabs being something I have dealt with for years I would have expected one of the tests to show some elevation, but they are not even remotely raised. Blood screen all good otherwise as well. I don’t know what’s going on. My breathing is completely manual and I’m thinking about every breath because my nasal breathing just seems so wrong. If I just don’t breathe my body doesn’t seem to be doing it for me. I’ve had to start taking medication at night to sleep because I was jolting awake, almost like my body was breathing wrong in my sleep and my eyes were flicking back and forth when my eyes were closed. The nostril where there is the collapse near the valve seems strange to breathe through at night, almost like a loss of resistance. My nose gets so dry as well. One doctor has tried to palm it off as anxiety but I know my body and I know that my nasal breathing is so different now.
I’ve wondered about a paralysed diaphragm but then I’ve had about 4 chest x-rays done in the last 6 months and nothing has been picked up.

If you think your sleep is being affected, you might consider doing a sleep disorder test that might shed some light on what is going on in your body.

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Originally Posted by drz

If you think your sleep is being affected, you might consider doing a sleep disorder test that might shed some light on what is going on in your body.

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I asked my GP about a sleep study because I felt like I have apneas. He said I didn’t fit the bill because I’m not overweight and don’t snore chronically. Other doctors have said that’s rubbish and it’s not just overweight people who have apnea, but I’m getting so tired of trying to self advocate. I keep coming up against anxiety excuses but I know my body and this is not anxiety. My breathing is like I’m breathing through straws both feeling and in noise. My nose has collapsed in on one side, I have chronic shoulder/trap pain and my breathing is almost completely manual when I’m awake. I’m exhausted from the completely disordered breathing.

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Originally Posted by Rae82

I asked my GP about a sleep study because I felt like I have apneas. He said I didn’t fit the bill because I’m not overweight and don’t snore chronically. Other doctors have said that’s rubbish and it’s not just overweight people who have apnea, but I’m getting so tired of trying to self advocate. I keep coming up against anxiety excuses but I know my body and this is not anxiety. My breathing is like I’m breathing through straws both feeling and in noise. My nose has collapsed in on one side, I have chronic shoulder/trap pain and my breathing is almost completely manual when I’m awake. I’m exhausted from the completely disordered breathing.

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I think you might want to check it out with a sleep disorder specialist since many times doctors do make mistakes. Years ago I was told by a highly recommended internists that I did not have diabetes even though my Blood Glucose levels were elevated way beyond normal range. And to compound his error and display his amazing ignorance he told me not to worry about it. My mother had just died from it so I was naturally concerned about whether i had it. He then went on to say I not only did not have it when I definitively did but I would never get it cause I was too thin. I also ran into similar mistakes when trying for three years to get my GPA symptoms diagnosed correctly. The find a zebra web site can sometimes help you figure out uncommon disorders. When i put in all my symptoms it always came up with GPA as first or second possibility but this was only years after my diagnosis. If I had found it earlier I might have saved myself a lot of serious body damage.

Here’s the thing. Your specialist, no matter how good, is not living your life and experiences. If your sleep is being interrupted, and you have a collapsed nostril, there may be sinus issues involved, or, as one other member reported (my apologies for not remembering who it may be - if you’re out there, chime in) gastric reflux causing swelling and acid burns in the back of the throat. I know its a pain to be continually advocating for yourself, but sometimes it’s the only way to get something done, and considering how sneaky Wegs can sometimes be, it’s ALWAYS better to follow up symptoms rather than ignore them. Don’t let it lie, keep telling your specialist you need a sleep study, or have a good discussion about alternative causes. Keep a diary, get one of those respiration monitors, show them the results. Is it consistently bad, or only in certain sleep positions? Does it get better or worse dependant on your nightly meal? Does any medication affect it?

my breathing hasn’t been affected by Wegs yet, thank (insert deity here), but I know it’s always a distinct possibility in a flare. If it was me in your position, I’d be a right pain, I’d yell and scream at my docs until something is done.

Great advice Peter. I might have been one of the reflux people you are thinking of here. I had it for many many months and saw a bunch of doctors and did a bunch of tests. I had one doctor look at me briefly and tell me it wasn't really too bad. Really, I'm literally inhaling acid in my sleep and coughing to the point of breaking ribs, not able to sleep for weeks and 'its' not really too bad'.
The pain and suffering alone was so bad I contemplated putting an end to it- but hey, at least I had someone let me know it 'wasn't really too bad'. That always helps.
Its a long story but I consider myself a miracle that now days I have zero reflux problems and have no restrictions around it. So GOOD things can happen too.
I came within ONE week of getting very invasive surgery to deal with it and delayed it as things were improving for some reason.
So, the point of me bringing this up is to reinforce your comment about the need to advocate hard for yourself. Trust what you know, insist on timely action to figure things out and pursue treatment. Even my old time doctor from way back told me "No one is going to care as much about what happens to you as you do" He was actually encouraging me to advocate as I tended to not really stick up for myself.

I have recent experience with an otherwise good doctor dragging his feet and dropping the ball on getting me timely treatment. This has resulted in several months of suffering that I know from experience could have been avoided. As a bonus he found a way to blame ME for being sick. I still like him and respect him but no one is perfect all of the time.
I pushed and pushed hard because no one else was going to.

Thanks me2, I thought it might have been you, but couldn’t find the thread. Hopefully it never comes to surgery.

Thanks Peter. Things are really amazing for me in the reflux department. I literally thank god almost daily that the problem is gone and I am enjoying perfect non/reflux health.

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Originally Posted by Peter Marsham

Here’s the thing. Your specialist, no matter how good, is not living your life and experiences. If your sleep is being interrupted, and you have a collapsed nostril, there may be sinus issues involved, or, as one other member reported (my apologies for not remembering who it may be - if you’re out there, chime in) gastric reflux causing swelling and acid burns in the back of the throat. I know its a pain to be continually advocating for yourself, but sometimes it’s the only way to get something done, and considering how sneaky Wegs can sometimes be, it’s ALWAYS better to follow up symptoms rather than ignore them. Don’t let it lie, keep telling your specialist you need a sleep study, or have a good discussion about alternative causes. Keep a diary, get one of those respiration monitors, show them the results. Is it consistently bad, or only in certain sleep positions? Does it get better or worse dependant on your nightly meal? Does any medication affect it?

my breathing hasn’t been affected by Wegs yet, thank (insert deity here), but I know it’s always a distinct possibility in a flare. If it was me in your position, I’d be a right pain, I’d yell and scream at my docs until something is done.

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The problem I have is that the docs don’t seem to be listening to me. My GP has written me off as being anxious. I totally agree that whatever is affecting my breathing has caused anxiety. I’d say that’s pretty understandable seeing that breathing is pretty important to staying alive. I went to an ENT with my breathing problems and because I mentioned the scabbing on my septum, he wanted to test for GPA. All tests have come back negative. However, I don’t really think they’re listening to me about the structural changes to my nose. I can see that my right nostril is now noticeably thinner than my left. There is now an obvious dip in the mid vault area of the nose and the internal valve inside feels like it’s buckled/split and is not now one smooth piece like the other side. The NHS is on absolute go slow because of Covid and just because it’s underfunded. I had a CT scan on the 4th March and I still have not received results. When I chase with the consultant’s secretary I get responses back about how they’re dealing with Covid and they can’t speed things up. Meanwhile I’m suffering every day with not being able to breathe right, breathing manually and feeling dizzy and lightheaded from it. Advocating for myself just seems exhausting and futile.

My heart goes out to you Rae. I know how it feels to be overwhelmed by serious symptoms and a lack of proper response to them. I don't know if it's an option for you but over here there is a 'new to me' option of getting a medical advocate. I recently talked to one myself and found it to be very helpful. Of course they charge for continued help and I know that's not option for everybody. Still, even the initial conversation was for me helpful and encouraging. In my case I got some ideas that had not occurred to me on my own. It's good to have even a little help.

The pandemic has impacted healthcare almost everywhere. I remember last year when in my area of rural Minnesota only emergency care was being offered for a while since all the hospitals and clinics felt overwhelmed with the large number of virus cases. Most also experience staff shortages as staff members became infected. Right now in our area the virus has showing a rapid significant increase which is probably due to one of the mutations that is more contagious and more infectious.

I'm not familiar with your healthcare system the but I have in the past had insurance plans that function like an HMO which meant we were assigned a primary care physician who was essentially a gatekeeper to limit our access to other specialties. I was usually pretty fortunate about being able to get referrals to specialists I wanted to see but the backup plan was to go to the emergency room or the walk in clinic for urgent care.

I have in the past been a patient at the world-famous Mayo Clinic in Rochester Minnesota. I remember when my local physicians had recommended I go see them for care since they were uncertain how to proceed with my GPA symptoms and suppressed immune system for the issues I was experiencing. I had trouble getting an appointment to be seen by my regular doctor at the Mayo Clinic and was advised to go to their hospital emergency room which would get me into the Mayo Clinic care system

Maybe trying to use the emergency options in your area might help you move up the ladder to a higher priority. If nothing else you could probably justify such contact as having an anxiety attack from the lack of necessary care.

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Originally Posted by Rae82

The problem I have is that the docs don’t seem to be listening to me. My GP has written me off as being anxious. I totally agree that whatever is affecting my breathing has caused anxiety. I’d say that’s pretty understandable seeing that breathing is pretty important to staying alive. I went to an ENT with my breathing problems and because I mentioned the scabbing on my septum, he wanted to test for GPA. All tests have come back negative. However, I don’t really think they’re listening to me about the structural changes to my nose. I can see that my right nostril is now noticeably thinner than my left. There is now an obvious dip in the mid vault area of the nose and the internal valve inside feels like it’s buckled/split and is not now one smooth piece like the other side. The NHS is on absolute go slow because of Covid and just because it’s underfunded. I had a CT scan on the 4th March and I still have not received results. When I chase with the consultant’s secretary I get responses back about how they’re dealing with Covid and they can’t speed things up. Meanwhile I’m suffering every day with not being able to breathe right, breathing manually and feeling dizzy and lightheaded from it. Advocating for myself just seems exhausting and futile.

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Yes our GPs act very much as a gatekeeper and they refer on to the services that they think can help. I have called my GP today and he is going to write in response to my previous ENT appt to see if I can get seen quicker. I will go private for my next appointment if needs be, I just need someone to properly listen to my concerns and investigate from there. My GP was actually surprised today when I told him that nobody had actually looked up my right nostril properly. The ENT consultant scoped my left nostril and didn’t bother with my right. Just had a little look at the septum.

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Originally Posted by drz

The pandemic has impacted healthcare almost everywhere. I remember last year when in my area of rural Minnesota only emergency care was being offered for a while since all the hospitals and clinics felt overwhelmed with the large number of virus cases. Most also experience staff shortages as staff members became infected. Right now in our area the virus has showing a rapid significant increase which is probably due to one of the mutations that is more contagious and more infectious.

I'm not familiar with your healthcare system the but I have in the past had insurance plans that function like an HMO which meant we were assigned a primary care physician who was essentially a gatekeeper to limit our access to other specialties. I was usually pretty fortunate about being able to get referrals to specialists I wanted to see but the backup plan was to go to the emergency room or the walk in clinic for urgent care.

I have in the past been a patient at the world-famous Mayo Clinic in Rochester Minnesota. I remember when my local physicians had recommended I go see them for care since they were uncertain how to proceed with my GPA symptoms and suppressed immune system for the issues I was experiencing. I had trouble getting an appointment to be seen by my regular doctor at the Mayo Clinic and was advised to go to their hospital emergency room which would get me into the Mayo Clinic care system

Maybe trying to use the emergency options in your area might help you move up the ladder to a higher priority. If nothing else you could probably justify such contact as having an anxiety attack from the lack of necessary care.

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If it is possible it might be good to call the ENT's office and ask to talk to the ENT's nurse and explain your situation and why you need to be seen soon. I found I'm somehow able to get appointments sooner when I do this in my own situation. Ask to be on the waiting list for any cancellations too. I've actually gotten moved up a bunch of times with just that one trick.