Newbie

[Barbara H]

Hi
First let me say I am not sure whether I am here under false pretences. The renal specialists have not called my disease Wegeners, they called it pANCA vasculitis then something long and complicated beginning with g. trollong round the web I came to the conclusion it was the same. If I am wrong then throw me off by all means.
I started before Christmas 2009 feeling sick and retching when trying to eat, feeling breathless and exhausted, and coughing but not bringing anything up. The breathlessness I put down to chest infection, but it turns out it was "multiple" blood clots on both lungs. The GP said "you have been fighting a flu-like illness, your energy will come back in its own time" he fobbed me off twice like that, but then the practice nurse sent me to another doctor at the same practice, and he sent me for blood tests which showed kidney function down to 12%. then there was a lot of phoning to get me into Hope Hospital where there is a specialists kidney unit, and I was in there in May last year. Thank God! they immediately put me on a steroid drip, and then on oral steroids, and to my relief my kidney function began to improve.
when I left the hospital it was 25%, and I was told it would not increase more than that because of damage to my kidneys. Then after a bit it started to go up again and levelled out at 34%. Last reading it was 39% and I am hoping that is not just a flash in the pan.

I was still breathless and had a ct scan in June when the blood clots were discovered. I have been on warfarin ever since then, and am now breathing much better. My pANCAS which were 1/500 when I first went are now down to 1/32 the doctor says he is not too worried as this is quite low, and if we get rid, there will be a far less frisk of the vasculitis coming back. A big part of the treatment was cyclophosphomide infusions which have now finished, but next week I am going for an iron infusion. I am also to be put on azithiazine next week, so will see how things go with that.

I have not had severe pain in joints or muscles, which makes me think I might be on the wrong site. If this is so, forgiveme, and I will retire gracefully
all the best
Barbara Harrison :)

[Brooke]

Welcome to the group Barbara, I do not have any joint problems myselft, never had. So, I don't think all people with Wegener's have joint pain? If so, I would wonder about myself too!

[Daggar]

Hello Barbara,

Sorry to hear about your illness.

There are 3 "G's" to choose from:

Goodpastures
Wegener's Granulomatosis
ANCA Glomerulonephritis

You've either found the right site and your one of the rare WG patients (20% I believe) who test positive for pANCA rather than cANCA -- or -- they described your kidney issue as "ANCA Glomerulonephritis" caused by one of the forms of Vasculitis -- or -- Goodpasture's Syndrome (did you test positive for an antibody called anti-GBM?)

I'm not half as knowledgeable as some of the folks on here but my guess would lean toward WG because the Glomerulonephritis is basically limited to Kidneys and a negative test for anti-GBM (Goodpasture's) leaves WG.

My daughter Holly was admitted to ER back in November 2010 with a kidney function at about 25-30%. That climbed to 40-45% by the time she was treated for WG and released. We're now at the 3-4 month mark and she is sitting at about 65%. So there is hope that the kidney damage was not permanent. Did they do a kidney biopsy? That would give them a better overall picture of how much "permanent" damage was done.

This website could be very helpful even if you don't have WG because a lot of the info is "vasculitis-based" and the treatments/side effects can be much the same.

Hope you find the answers you need!!

Gary

[Barbara H]

Thanks for your replies! My memory seems to lean to the ANCA Glomerulonephritis. I will be pleased to be member of this site, though, if I am still welcome. Yes I did have a kidney biopsy, and the written report says "confirmation of necrotising Glomerulonephritis with some chronic scarring" So I suppose it is actually ANCA Glomerulonephritis. To me it still sounds very similar to wegeners, so would be glad to stay but its up to the other members.
love and best wishes to everyone on the site

[Daggar]

I'm not sure -- the cold-symptoms and lung activity may be WG but you should confirm the diagnosis. Glomerulonephritis could be the result of WG.

Welcome aboard!!

[Sangye]

Hi Barbara, yes please leave this group immediately! You can come back when you have proper symptoms like joint pain. Slacker! Imposter! LOL :laugh:

Okay seriously, welcome to the group. It sounds like you've "only" had kidney involvement and blood clots in your lungs. Wegener's makes people much more likely to develop blood clots, and I wonder if p-ANCA vasculitis does, too. I have no idea if people with p-ANCA glomerulonephritis have other symptoms like joint pain and fatigue. Not all Weggies have them, either. Actually, there isn't a single symptom that all Weggies share. It's highly variable.

Many vasculitides are treated the same as Wegs. It sounds like yours is. I hope you do well with the transition to imuran. :smile1:

[freakyschizogirl]

Hi Barbara, yes please leave this group immediately! You can come back when you have proper symptoms like joint pain. Slacker! Imposter! LOL :laugh:


I dont have joint pain, should i pack my bags too?? lol

[Sangye]

Outta here!! :flapper:

[Barbara H]

Hi thanks for all your replies. I can remember now how it was first described. I went for tests to the hospital, and as soon as I got back the hospital doctor was on the phone telling me to go straight back "The sooner we start treatment, the better the chance of saving your kidneys (that was a shock!) you have one of these rare auto-immune diseases, it affects the kidneys and lungs" I asked does the disease have a name and she said p-ANCA vasculitis glomerulonephritis. Quite a mouthful to sink in, when you are still reeling from finding you have a serious illness and your kidney are threatened! No wonder my brain went into a flat spin! Any way it apparently is not WG but it certainly seems similar enough for me to learn from people who have it.

[Daggar]

Your story sounds a lot like Holly's -- we were unhappy with our GP writing up prescriptions and signing her up for pyhsio so we took her to the Children's Hospital ER to have her checked. Luckily we did because they admitted her right away, diagnosed her within 48 hours and started treatment at that point. It probably saved her life! Her kidneys were in a mess at that point. They checked her for upper respiratory involvement but that came back negative. She's been on the treatment now for nearly 4 months and all signs of WG have cleared up, however, the kidneys were damaged and she's had some side effects from the drugs that we are now dealing with. The lung and kidney involvement can happen with WG as well -- one or the other, or both

[Barbara H]

Hi Gary
I am sorry to hear about our daughter Holly, it seems a lot for a young child to go through, but at least it appears that things are improving for her. I am not knwolegable about these things, but wonder if maybe as she grows, her organs may grow with her body and become more efficient. I hope so.
I do not have youth on my side , I am 65 but remain optimistic that the improvement I have had will continue
best wishes
Barbara

[elephant]

Hi Barbara H, It certainly sounds like you have Wegeners disease. Wegeners loves the sinuses, kidney and lungs! Welcome and love to have you stay!

[Daggar]

Sadly the kidneys are not like the liver -- they do not regenerate. If she has 65% kidney function at age 15 that will only decrease as she ages and if she is hit with a flare-up that isn't caught in time it can lower quickly. 60-65% kidney function at the age of 50-60 is not so bad.... but at age 15 it is of great concern.

This is why her Nephrologists are "all over it" at this time to make sure they stop any possibility of further damage. They have told us that she could stay at 65% and lead a normal life as long as she sticks to "healthy living" (exercise and a low-sodium, low-fat diet). They have started to use a blood pressure med to keep her bp down -- a result of lowered kidney function and the prednisone. High blood pressure will also do further damage to the kidneys.

A bit of a balancing act....:unsure:

[Sangye]

Still, never underestimate the ability of a young body to heal. I've seen them do miraculous things.

[Daggar]

Yes Sangye we're hoping for that!

She has surprised the kidney doctors so far with the last couple of tests (creatinine levels - could be in the 65-70% kidney function now).

If "attitude" has anything to do with it Holly will do very well!

[Barbara H]

power to her!

[Daggar]

And to you as well! The "little weggie", as Marta calls her, says thanks!!

[Daggar]

Barbara.... I've just had a fellow contact me through another WG service that was diagnosed with pANCA Vasculitis and an auto immune disorder called Polyarteritis Nodosa.

He's been trying to find others with the pANCA related disorder -- I've given him this sites address because I believe there are a few on here that fit the description.

Does that Polyarteritis Nodosa sound like something they told you at the time of diagnosis?

[elephant]

My sister had tha PAN, it can affect every organ because it inflames the vascular system. She was only on high does of prednisone for 6 months and it went into remission and hasn't come back.

[Barbara H]

Hi
It probably is similar. My doctors seem concerned about the vasculitis and dont seem to bother about expanding on the particular name of the variations. sounds very much the same. I am under a vasculitis specialist and am very satisfied with my treatment. My pANCAS titre is now down to 16, they dont measure it any lower than that, so if it goes down again it is negative. Is prednisone the same as prednisolone? I am still on that but down to 7.5 mls now. I have finished having cyclophosphamide infusions, now I have started on azathioprine instead, we will see how it goes, but considering my kidney function was down to 12 when I went in, and they though they might have to put me on dilaysis, I am pleased to say the last reading was 39%.
Would be pleased to hear from the gentleman you mentioned

[Sangye]

Barbara, I've also heard Wegs specialists say that many vasculitides require the same treatment and it doesn't matter too much if they can't pin down the exact diagnosis.

[Daggar]

Good to hear that you're doing better! Prednisolone is the iv-pulse (liquid) vs. the oral pill form - prednisone.

There were a few replies on the other site to the fellow I mentioned -- it appears pANCA vasculitis is more common than first thought and there are a few that have had nearly "complete" recovery!

Kidney function with auto-immune disorders is a tough thing to determine. The Nephrologists told us that Holly's damage appeared to be substantial but she has surprised them so if you're at 39% now it could level off there or could show further improvement if the treatments have stopped any inflammation. It's such a tough thing to determine.

As Sangaye said... although the vasculitis disorders are different the treatments are interchangeable for the most part minus side effect complications.

Let us know how you do on the azathioprine....

[Barbara H]

that's odd1 I am taking prendisolone in pill form! Not to worry, maybe terminlolgy is a bit different in the UK from in America. I will let you know how I go on, I have only just started them today. I also heard of a 15 year old boy, his mum said the vasculitis has completely gone a few years ago, and his kidney function is almost back to normal. Here's hoping!

[Daggar]

sorry barbara i got false info online.... imagine that!!

I guess this is the correct comparison from the doctors:

Though prednisone and prednisolone are used in the same manner and equally as effective, they should not be confused with each other. Prednisone is activated by the liver into prednisolone. For this reason and because it is more easily absorbed, prednisolone is the drug of choice when hepatic disease or insufficiency is present.

Both prednisone and prednisolone are readily absorbed and distributed into muscle, liver, intestines, and kidneys. They are both metabolized in the liver, and excreted in the urine.