Flareup

[Mark76]

Hi all well it has been 3 months since diagnosis and treatment now. Just to remind you all how I got diagnosed. I started with sinus pain in Sep last year which worsened to the point where I was spending 6 hrs a day in shower just to get some relief from the pain. Then I started bleeding from nose, lost partial hearing on left side and finally chest scan showed growing lung nodules. I lost 15kg and night sweats and cough and going downhill quickly in Jan when I was hospitalised. Had 2 lung biopsies and finally a diagnosis of WG.

Treatment was 3 high doses of IV methylpred, and 3 rounds of cyclophosphomide and rutiximab every 2 weeks. Prednisone daily starting at 50mg and tapering down.

I have monthly blood tests and am due to have a chest ct later this month to check on my nodules.

Today I feel pretty good and no longer have my cough, albeit occassional one after lying down, no sinus pain or bleeding whatsoever and regained my weight to what I was before disease. My pred dose currently 10mg. The only thing I have lingering is my partial hearing loss in left ear. When I lie down or when I jump I can hear clicking so Im assuming its more conductive hearing loss but who knows. Im getting less hopeful it will return given that with high pred dose hearing returned but then went back to partial again.

Anyway my question relates to flare ups. Whilst no one wants one I was curious to know if the stage of diagnosis eg early or late is a factor in having a relapse. Secondly I read that approx 50% of ppl relapse after going off maintenance. Would the fact that Im not on daily medication other than pred mean Im no longer on maintenance?

Thirdly, I also read that approx 30% have kidney involvement at first presentation. I thankfully didnt. But the report then goes on to say that approx 80% will have kidney involvement. Does this mean that its likely that kidney involvement will occur in a flareup even if didnt have it initially? I sure hope not. Or are they saying that if not diagnosed and treated that kidney involvement more likely?

Thanks for your support guys.



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[drz]

My take is that GPA is a lot like diabetes and many other chronic diseases in that the damage often tends to be progressive. Some times it can move very quickly like in a crisis or big noticeable flare and other times it might be slowly happening rather quietly behind the scenes like erosion going on in nose to eventually causing a saddle nose as the bridge collapses. Neuropathy in the feet is another slow moving progressive problem. This type of damage often seems to happen even when the patient often appears to be in good control of the illness or even in remission.

Some of the side effects might be an inherent part of the disease while others might be due more to side effects of meds we use for treatment and control of our GPA while others might be due to a combination of the two. Certain things like dermatology issues are more likely to appear eventually although not for certain. We know we are a much higher risk for many things because of our history and the required treatment but the best approach is to be responsive to any new symptoms but not to obsess about things that may never happen. Just hope you are in the percentage that do not develop the issue or problem until there is evidence it might happen and then get it checked out. Don't dawdle in doing so either. In my experience most of the times my worrisome symptoms have turned out NOT to be a serious problem. Bit some times they were things that required an active treatment response but once the problem was correctly diagnosed and treated it usually got better and my anxiety went down.

My Mayo at expert strongly advises me to do a urine dipstick test for blood in the urine ever week since kidney damage can happen quickly in short time and cause loss of kidney function like in did in our dear departed Jack, who was one of our patron sages here a few years back. He always advised people to be vigilant and monitor any signs of possible kidney problems. The one time my dipstick tests indicated blood in my urine I freaked out and got my regular lab work done as soon as possible. Then I learned from the urologist that the dipstick tests can create false positive for other reasons and my kidneys were still stable and not on verge of failing again. I did have substantial kidney damage from Wegs when first diagnosed thus the need to be more vigilant.

My impression is that once a organ has been attacked it is more likely to be damage by further attacks or on going Weg activity. BUT it can also attack a new area so one can never be complacent about Wegs since it can be very unpredictable but we learn to accept that and live with it.

[MaxD]

Mark, glad to hear you were treated and are feeling better. That's progress! Alas, once the mind is no longer focused on immediate pain, it starts to worry about longer-term issues. We've all been there. I remember feeling orphaned when my doc said I should see her every other month instead of every month and started reducing meds! Time will heal.

More to your points: without maintenance therapy the chance of relapse within the first two years after remission in sinus-related cases is extremely high (over 70%). However, since you were treated with Rituximab, it generally stays in the system for an average of 8-10 months. My docs check my B-cells with every blood test. For at least the first two years after remission, I got one infusion of Rituximab when the B cells showed up at 1%. The chance of relapse with this is lower than 3% according to studies. Some docs prefer one infusion every 6 months regardless. Other maintenance therapies use methotrexate and other meds. I like RTX because it has few obvious side effects and has worked for me. (Beyond two years whether to continue maintenance therapy, or to forgo it entirely, is something my docs will ponder soon - they tell me long-term care is an art, so we'll see what happens.)

Now, you were treated only 3 months back, so it's unlikely you are in remission - most likely the B-cells are still getting zapped. I was told I was in remission when I stopped my prednisone and all my blood tests were normal. So, relax - you're doing well and getting better.

Finally, as long as you get regular blood and urine tests, you should be fine. Not everyone develops kidney failure (interestingly, people first diagnosed with kidney involvement have less chance of relapse than those with sinus/respiratory issues - go figure!).

Keep us posted, and feel free to vent any concerns. The next set of issues you're going to worry about will start as you taper the prednisone down below 10mg. Aches, tiredness etc. It took me a good 5-6 months to recover from that. But now I feel better than I did years before I was diagnosed. Good luck to you.

[Mark76]

Very informative thanks Max...out of interest did you have ear involvement? Im having an auditory assessment today to determine if conductive or not.

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[MaxD]

Very informative thanks Max...out of interest did you have ear involvement? Im having an auditory assessment today to determine if conductive or not.

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Oh boy, did I ever have ear involvement! It all started with my ears filling up with fluids and recurrent infections - low doses of prednisone were all that helped. After that it progressed to Bell's palsy (presumably disease impinging on the facial nerve bundle that goes through the ear), and finally lung nodules and joint pains. After the treatments I'm in remission - but my ears are constantly ringing (one gets used to it, but absolute silence is a distant memory). I also suffer from some hearing loss (but not conductive I don't think) and eustachian tube dysfunction (essentially, often all I can hear is my own breathing and voice and I have to snort hard or lie down to normalize, it has to do with a valve not functioning properly, but I'm told there's nothing to be done about that).

My docs do have me see an ENT/GPA specialist who checks the trachea every 6 months - that's another unfortunately common site for granulomas to develop. So far that's been clean and I hope it stays that way!

[Alias]

My first symptoms, or at least the first symptoms that drove me to seek treatment, were ear related. I was treated for an ear infection with antibiotics and two surgeries before being properly diagnosed. Mine started suddenly, seemingly out of nowhere. I took a short plane ride and had ear pain and hearing loss, and things were never the same. The hearing on one side never returned to normal. More than two years later, I have some residual hearing on that side according to audiograms, but practically speaking I am deaf on that side without a hearing aid. It is conductive hearing loss, no evidence of nerve involvement in my case. I also have the tinnitus and eustachian tube dysfunction described by Max, varying in severity from day to day, but never absent.

[Mark76]

Hmm thats interesting...so do you still get pain in ears when flying? Yes I suspect conductive with eustachian tube dysfunction too...ive heard varying success with a device called ear popper that is supposed to treat eustachian tube dysfunction. Its non invasive and delivers air to sinuses and eustachian tube...might be worth a go??

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[Alias]

I don't get pain but I do feel extra plugged for a day or two after flying. Actually, my "good" ear seems to clear when we reach cruising altitude, but then plugs badly after landing. If I have a multi leg flight it's worse. Very weird. Just looked at ear popper on Amazon. Never heard of it before, but it looks worth trying. Has anyone tried this?

[MaxD]

Interestingly, all my past problems (long before the disease) with sinus pain and ear pressure when flying disappeared after treatment. Now when I fly, I don't sense any issues!

[MikeG-2012]

Interestingly, all my past problems (long before the disease) with sinus pain and ear pressure when flying disappeared after treatment. Now when I fly, I don't sense any issues!

Looking back, I had REALLY BAD issues with flying. Now, no issues.