So close for dialysis (update on gilders)

[gilders]

Hi all,
I feel a bit guilty having a bit of a moan so soon after Phil's passing, but I know he'd want us all to still be here for each other.
To recap as VERY briefly as possible -
My latest relapse began approx 2 years ago. It was less aggressive than my usual relapses and the first time that it seems like it was not doing further damage to my kidneys. It was 1 year ago that the Drs finally confirmed it was a relapse (nasal biopsy instead of kidney biopsy this time) and treatment commenced. Treatment was just high dose Imuran and prednisolone which has always worked for me. This time it took 4 months before any improvement. I had a little improvement around end of February this year, but it didn't last long. Since then I've been roughly stable, i.e. I feel bad and don't feel like I'm in remission, but don't feel like WG is become more severe/active.
This is not usual for me. I usually feel worse and worse until treatment begins to work, then feel better and better until a point where I know I'm back in remission.

I'll fast forward past other tests and issues I've had these last few months to my latest Dr appointment and blood results.
My usual Dr is away until next April. The new Dr I saw thinks my WG is in remission, but I think he's relying on my ANCA result too much (it is good at the moment). My nose bleeds are back, I feel pretty terrible, rheumatism is getting worse and this new Dr has had a chest specialist look at my recent lung CT scan and they now believe it isn't pulmonary embolisms (blood clots), but damage (scarring) from WG.
His reasons for my symptoms not being proof of WG activity are - Nose bleeds (because I'm on Warfarin), feeling terrible (due to low blood pressure), worsening rheumatism (due to reduction in Prednisolone - I agree with that, but whats actually causing it?), WG lung damage (if it was active I'd be coughing up blood).
He maybe correct and I hope he is but one final thing occurred 24hs after my appointment and blood tests. He emailed me to tell me that my kidneys have deteriorated considerably. I won't bore you with all the results so will give you the most important/accurate one to do with kidney function. My GFR (filtration rate) is 17. I'm not sure if other countries use the same measurements, but to put it in perspective normal range is 90-120,

Stage 1	≥ 90
Stage 2	60 – 89
Stage 3	30 – 59
Stage 4	15 – 29
Stage 5	< 15

Stage 5 is end stage renal failure where transplant or dialysis is required. As I'm level 17 I'm borderline Stage 5.

The Dr has taken me off Furosemide (diuretic) and to drop my BP tablet (Lisinopril) to just 5mg/day.
He hopes this will take pressure off my kidneys and allow some function to return.
I will be having blood tests on the 27th which will hopefully show some improvement. If no improvement I personally think it is one result too many to ignore the possibility that I'm not in remission.

After Barbara's terrible experience with dialysis I'm a little concerned. I needed dialysis when I first started with WG and although I didn't feel better after it, I didn't find it as bad as what Barbara must have felt (although I was a fairly fit 17 year old back then).

Sorry for the ramble and moan. I know there's no useful information in it for anyone, I just wanted to get it off my chest and what better place?

[Alysia]

I am sorry for the though time you have. Its def sounds like wg activity to me. You can call it 'flaring' or 'smoldering'. Either are dangerous. You need to get serious treatment asap. Rtx for sure. Pred. Maybe also plasma exchange ? Igiv IV ? Those are the more 'heavy guns'. I learned when I was with Phil that plasma exchange can bring miracles when there is kidney involvement. If your doc is not serious enough, look for another one. Geoff has good one. Ask him. Keep on fighting. Love you.

[gilders]

Hi Alysia,
Hope you're coping as well as possible. Funny you should mention Geoff. I'm sure it was him who I contacted to get get his specialist's email (Dr Jayne I think). I didn't end up contacting him, but if my results aren't better on the 27th I'll give it a try.
I'm not sure in the NHS if you can change your Dr, especially if it's not within your home area??

[MikeG-2012]

I am a textbook case that ANCA is NOT the definitive test to show remission. My ANCA has been negative since April 2012, right after diagnosis and first round of treatment. But, last September, I had a nasty flare up. So don't let the docs rely on the ANCA, it is NOT that effective as a marker for remission!!!!

[gilders]

Mike, I also know from first hand experience that ANCA isn't a good marker. The first relapse I had, I picked up on it whilst ANCA was negative. My original Dr agreed with me and started treament again. About 2 weeks into treatment ANCA did then show positive. I've almost always known my body well enough to know if I'm just at the beginning of a relapse, whether things are improving or deteriorating and when I'm in remission. This time I just don't know because I've got so many other illnesses going on at the moment.

[mrtmeo]

Hi Gilders,
If there are any rbc casts in the urine, vasculitis is live and active.
Lung activity can be in the form of nodules ((clots) which they can't positively tell the difference unless they surgically go into the lungs) that get bigger and smaller. My mom has always had this activity without coughing up blood.
As far as dialysis, Nocturnal at-home (5-6 nights per week) is the best and provides a quality of life that also, allows a better variety of foods in the diet.

I pray your kidney function comes back and they treat quickly.

[drz]

Sorry to hear about loss of kidney function. But that alone can account for lot of fatigue and feeling crappy. My nephrologist reduced my blood pressure meds last year to try protect my kidneys from further loss of function. She said the latest research indicates a normal blood pressure is best for maintaining kidney function. Before it was going lower than normal guideline limits.

I was also a stage four at my worst but improved to stage three over the years since my initial DX. I hope yours also improves.

[Rose]

Hi Gilders

Although all those symptoms could have been caused by what the doctor thought, I would have thought a WG flare should have been ruled out first especially when all these symptoms have been happening at the same time. I never had any lung symptoms at all when first admitted to hospital before WG diagnosis but a routine x-ray as part of their investigation as to what ails me showed quite a few golf ball sized nodules. This is such a sneaky disease.

Rose

[gilders]

If there are any rbc casts in the urine, vasculitis is live and active.
Lung activity can be in the form of nodules ((clots) which they can't positively tell the difference unless they surgically go into the lungs) that get bigger and smaller. My mom has always had this activity without coughing up blood.
As far as dialysis, Nocturnal at-home (5-6 nights per week) is the best and provides a quality of life that also, allows a better variety of foods in the diet.

I pray your kidney function comes back and they treat quickly.

Hi mrtmeo,
Thanks for the advice.
By rbc I guess that's red blood cells? If so then yes I've had plenty of blood in my urine since my kidneys failed completely in '95. The blood has been present for the last 19 years even when I'm in remission, so I don't personally think it proves "vasculitis is live and active", but like many things, it can be an indicator.
I understand what you're saying about lungs. The CT scan wasn't very clear especially as it couldn't be done with contrast dye (due to poor kidney function). I know I really need a kidney biopsy, but I've not had a good experience with these previously. I've been struggling to get my INR (blood "thiness") level stable after I had to stop taking warfarin after my bone and bone marrow biopsy. Another biopsy would mean going back on daily injections for a few weeks and struggling to get the correct warfarin dose.
It worries me a little that your mum has activity in the lungs without coughing up blood. I'd previously (years ago) coughed up LOTS of blood when WG was very active. I hoped that my Dr was correct with his "no blood - no activity" diagnosis.

I've known for many years that I would eventually be back on dialysis so have researched it a bit. the nighttime one does have it's advantages, although the literature in the UK recommends it to be done EVERY night rather than 5-6 nights per week. There was one thing I read that made me smile - it can be "paused" half way though to allow toilet visits (fair enough) or sex! I can't imagine anything much worse to kill the mood then "hang on a second love, I just need to unplug myself"

[gilders]

Sorry to hear about loss of kidney function. But that alone can account for lot of fatigue and feeling crappy. My nephrologist reduced my blood pressure meds last year to try protect my kidneys from further loss of function. She said the latest research indicates a normal blood pressure is best for maintaining kidney function. Before it was going lower than normal guideline limits.

I was also a stage four at my worst but improved to stage three over the years since my initial DX. I hope yours also improves.

Thanks for your kind words. It gives me some encouragement that I might still not be "flaring" and that my kidney function my improve. I thought Wegeners was hard enough to control, but kidney function and blood pressure now seems just as confusing. I.e. poor kidney function has given me high blood pressure and that's why I was put on Lisinopril. Now my kidney function has got even worse, my BP has gone the opposite way (high to low) and I now need to cut down on Lisinopril.