Just diagnosed with Fibrillary Glomerulonephritis

[Trish]

I'm 35 years old and just received this dx. I'm scared to death. I feel like I have a great doctor. She has gotten several second opinions for me.

She is telling me that the only treatment is chemo type drugs and steriods. And there are many many side effects, and long term effects. And from what she has told me, only 1 in 5 respond to treatment.

I'm wanting to find out more information on this disease and this treatment. And hopefully get in touch with someone that has this disease, and maybe has had this treatment.

I don't have any other diagnosis. This just came on with no reason.

Help!

Trish
trishyo@yahoo.com

[DoriSchatell]

Hi Trish,

Sorry to take so long to get back to you, but fibrillary glomerulonephritis (FGN) isn't something we're very familiar with, so we needed to do some research. It's very normal to be scared when you're young, healthy, and out of the blue comes something unexpected and unknown. And there really doesn't seem to be a lot known about this condition. On the plus side, at least there are some treatments--there are many conditions that don't have any good options at all.

It really does sound like you have a good doctor, too. FGN is rare, and not everyone would have been able to make the diagnosis. She sounds like she's doing everything in her power to find you information. I'm sure she's no happier to give you this news than you are to hear it, but she's clearly in your corner.

Did your doctor tell you what level your kidneys are working now? Does the condition seem to be moving quickly or slowly? From what I've found in the medical literature, about half of people with FGN progress to kidney failure within a few years--which means that half don't.
-- In one study, a person was treated with prednisone (1 milligram per kilo of body weight per day) and cyclophosphamaide (2 mg/kg) for 6 months. The level of protein in her urine dropped, and she had a partial remission of her kidney disease. Her kidney function stayed normal. (Strebl P et al, Vnitr Lek. 2004 Aug;50(8):624-7)
-- In another study, 3 patients were treated with prednisone (1 mg/kg/day) and tapered off depending on how well they did, plus they received an ACE-inhibitor (blood pressure drug) and diuretic (water pill). After 9 & 12 months, 2 of the patients had no more protein in their urine and one had a reduction. All 3 had normal kidney function and at the time of the article, 2 were off steroids. (Dickenmann M et al, Am J Kidney Dis. 2002 Sep;40(3):E9.)
-- In one 10-year old girl with FGN, her proteinuria went away with NO therapy, and her kidney function stayed normal. (Bahrami D et al, Pediatr Nephrol. 2001 Nov;16(11):916-8.)

Since half of people with FGN have normal kidney function, it seems as if it would be in your best interests to protect your kidney function in the ways that anyone can:
-- If you have diabetes also (doesn't sound like you do), keep your blood sugar in the normal range
-- If you have high blood pressure (FGN can cause this), treat it to keep it in the normal range
-- If you smoke, quit--smoking has been proven to make kidney disease progress faster
-- If you need to have an X-ray dye test, like a CT scan or MRI, tell the radiologist that you have FGN and ask for the dye to be diluted. You may also want to ask about a drug called Mucomyst, that can help protect the kidneys from the effect of the dye, and see if they can use a less harmful dye (there is one, but it's more costly)
-- Avoid non-steroidal antiinflammatory (NSAID) drugs as much as possible. These pain-killers include ibuprofen (Advil) and naproxin (Aleve, Anaprox). Actually any drug with a generic name that ends in "profen" is an NSAID. If you aren't sure about a pain killer, ask a pharmacist.

[Trish]

I really appriciate your response! This is all new to me, and any information and/or support I can get is great.

I just got home from the hospital on Friday. I stared the Cytoxan/steroid treatment. My doc put in in the hosp for 2 days to monitor, then I'm continueing the treatment at home. So far it seems to going well. I guess by that I mean that I'm not throwing up. I'm just holding on to hope that this treatment works for me!

My doc told me that my kidneys were functioning at 25%. My last GFR was 39. And my creatnine (sp) is 1.6.

Thanks again,
Trish

[DoriSchatell]

Hi Trish,
If your GFR is 39, then your kidneys are functioning at 39%, not 25. That would put you at stage 3 chronic kidney disease. There are 5 stages. Your doctor is doing exactly the right thing for stage 3--keeping an eye on it and treating the underlying conditions. The other thing you can do (as I wrote above) is to protect your kidney function.

Let us know how the cytoxan/steroid therapy works for you--I'd love to see your GFR bounce back up, and hope that it does. I'm glad you're feeling well with the treatment.

[cindijean]

I see your note from 2006 - curious how/what has transpired in the last three years?? I have just been recently diagnosed and meet with my Doctor this week to find out what treatment he is going to use....
thanks, my email is cgolas001@twcny.rr.com