wegeners granular matosis anyone?

[smartiejake]

My mum has been suffering terribly from this horrible rare disease for the last 10 months. She has regular cyclophosphamide and now is on immunoglobuline as well, in addition to lots of other various drugs. She is very deaf, blind in one eye, has lost 4 stone in weight, has bells palsy, a fistula in her gum through to her sinus and is asleep for about 4 hours a day.
Previous to this she was the picture of health and swam 5 miles a week!
She is only 62 and I am heartbroken for her. Anyone else got experience of this bastard of a disesase who can give us some hope?

Not direct experience but do you think it would help if you could go wth her to the hospital for her next appointment? Is she well enough to tell them how she feels? would it help if you wrote it down with her so that she could be sure to get some of her problems addressed?
Hope it works out for her

I have a friend who was diagnosed with this disease 10 years ago. He had pretty aggressive treatment for it but he also has long periods of remission (years) so its not all doom and gloom. My sympathies though, it is a horrible disease

Yes I have heard that there is often long periods of remission and that treatment can be very successful but it just seems to be taking such a long time to have any significant impact.Initially she did amazingly but had a relapse in July( when she lost the sight in one eye) and she has been so poorly since. She is so down about it and my poor dad is struggling to cope even though my sister and I help alot.

smartiejake, I'm so sorry to hear about your mum. Mine has Churg Strauss syndrome which is related as it is another form of vasculitis and she has had a range of bizarre and seemingly unconnected problems including peritonitis, a collapsed lung and double vision. She's had it about 15 years. However, she doesn't suffer anywhere near as badly as your poor mum - it sounds really awful.

Her condition has more or less stabilised and once they got the treatment levels right (immune suppressants among other things) she learned to manage it quite well. It sounds like your mum is not stabilised and I hope it's possible with the right treatment to get her to a place where it's manageable but I don't really know the disease so it may be more difficult than with churg strauss.

I found the most useful thing was to sign up to a yahoogroup on churg strauss and there's bound to be one for this disease too. The CS one is contributed to by patients around the world (it is also incredibly rare) so you get other people's experience. I log in for my mum as she's not very technically adept and I just let her know if there's anything interesting in there that she should raise with her specialist.

Also, make sure she is seeing a top specialist. A rare and serious disease warrants more than a bog-standard consultant. My mum goes to St George's in Tooting where I think they have a lot of knowledge on auto immune diseases.

hope you all make some progress and your mum gets some better days soon.

My mum is seeing all the top specialists (ENT, oral surgery, audiology, rheumatology, eye specialist)and can't fault the hospital she is seen at.

She is having a small op next week and the top oral surgeon and the top ent surgeon are doing it one after another so she only has to have one anasthetic. They have both changed their entire schedules to make this possible. They always manage to get her in or CAT and MRI scans very quickly (helps to monitor the disease)

My dad only has to call with a query and he is always put straight through to the consultant and if not available they always call back. I suppose she is a bit of a medical celebrity there and perhaps that's why she is so well cared for. It's a disease that is only diagnosed in 8 people per million! Our GP hadn't even heard of it!

Hi,

Nasty condition, try this site:
www.vasculitisfoundation.org/wegenersgranulomatosis

Hope things stabilise with your Mum.

Thanks have looked at quite a few bits on line. Not sure I have seen this one.
Problem is that most people respond very well to the treatments. She isn't, so lots of the stuff on the websites doesn't seem to apply to her. I have suggested St. Georges in Tooting to my dad and I think he's getting so desperate that he might consider it although as I sad before, she is extremely well looked after where she is at the mo.

Unfortunatley things are going down hill at the mo. She had blurred vision in her "good" eye yesterday and was rushed to moorfields. They sent her back to the local hosp where she has been found to have an infection of some sort. So she is back in having ABs. Just hope it means she can still have her op next week as feel this will help.