GPA AKA Wegeners granulomatosis

[Jools52]

I have had recurring high fevers, rigors, awful joint and bone pain and so much more. My blood tests showed me ANCA positive & it was not looked into. That was in 2006. In 07 I was diagnosed with ulcerative colitis so every time I then went to my GP with more and worse symptoms, it was ALL put down to the UC but I knew there was something more wrong. I suffered for over 10 years as a result. No one has the specific skills to get a diagnosis and it seems that auto immune diseases are now spiralling Into epidemic figures. 

For example, I was told that UC was 1 in every 100,000. When I went to clinics, I saw many more and just from the small place where I live. I see many more who have inflammatory bowel disease but the powers that be don't seem to want to publish the real figures. 

Now, I have been battling vasculitis, undiagnosed for 8 years, and was scared I was going to die and on many occasions. The fact that they use steroids and immune suppressing drugs, for IBD, probably has saved my life but not by design. 

I am now going through the long process of diagnosis and have had Bronchoscopy and lung biopsy, kidney and liver biopsy but the rheumatologist, who, by default, deal with these diseases, are not specialists and so it can be a very painful and long road to diagnosis and then real treatment. I am told they believe me to have GPA but that the lung tissue keeps changing from a glass like appearance and then goes clear. I am so frustrated as I am still suffering yet no one will give the green light for the cyclophosphamide ( chemo) to properly address this awful disease. 

I am now. Wing referred to a specialist centre, could be Addenbrookes or Birmingham, I don't know as yet. We really need to be able to get a referral much sooner, I am sure lives are lost due to the true specialists not being available to all. I hope that I get seen soon as this life is so hard when suffering from 3 chronic conditions. If I had listened to my GP and not kept on then I would still be being conned off. This is a killer and needs addressing much sooner. I am not being dramatic when I say I fear for my life. 

Please do badger tour GP and even go to A&E if you feel able. When I bet the attacks, I am unable to move from my bed. I mean, I can't even get to the loo. I'll leave the rest to your own imagination. It's a living nightmare.  

The single most frustrating thing is people often say " well, you look just fine" I knkw they mean well but it's so hard to explain and I now sometimes say nothing at all. 

[Chris-Bromsgrove]

Hi Jools52, Sounds like you have really been through the wringer. Sadly you tale is far from unique and whilst awareness of vasculitis is improving (thanks to the efforts of Vasculitis UK) there is still some way to go. The good news is you have definitely been referred to the right places in Addenbrookes and the QE Birmingham. I had my diagnosis and treatment at Birmingham and I have found them excellent. Keep pushing to get an appointment ASAP. Good luck. Chris

[Jools52 in reply to Chris-Bromsgrove]

Southmead hospital in Bristol & the rheumatologist is Dr Harsha Gunawardena. The biopsies and all other tests are done here. Not sure if this is decent or not as I have been to too many hospitals and they seem much of a muchness to me. 

I would just like to get properly diagnosed and then treated. I understand it's a big deal that I am getting seen. I try not to push my luck because I see others suffer

[Hidden in reply to Jools52]

I get to hear good things about hospital in Bristol and nearby.  If you live in the South West, Cambridge is a long way to go especially in your condition.  I go to London and it takes a while to recover from the long travel.  Thank you for sharing.  It's nice to hear the real "voice".

[Hidden]

Doctors don't know.  Especially, you look well enough and you sound well, "all is well" to them.  I suffered a lot over many years.  Are you on any treatments?  Where are you getting your lung biopsies?  Anywhere decent?  

[Jools52 in reply to Hidden]

Sorry, I replied to this but in the box above. Apologies for that 

[Marcus1988]

Hi Jools, I hope you're feeling as well as you can... 

I also have GPA and on my first flare up in 2015 it caused the cartilage in my nose to be eaten away... now have a 'saddle nose' deformity. I agree its not a nice disease and very difficult to diagnose (they thought I had a severe sinus infection for 2 months until my nose started disappearing!).

One thing I would suggest you asking for is Rituximab, I'm not sure if you've heard of it before? Its usually given as a second option by the NHS after Chyclophos. due to its cost! I had one shot of it last year along with daily steroids and it has sent me into 'remission'. I now feel perfectly normal (and have done for 6 months). One dose of rituximab lasts up to 18 months. 

Its unlikely that sufferers of this disease will never relapse again so my consultant at the Norfolk&Norwich (who is very knowledgeable about the condition) plans to keep my immune system suppressed with a daily dose of Azathioprine so that when I have another flare up it won't be as extreme. Its important that you're immune system isn't over-suppressed though as this can leave you open to some nasty infections (not just colds). 

I will also say that despite the fact Adenbrookes has a vasculitis clinic, its unlikely that you'll see one of the 'brains' on GPA. I'm very lucky that Dr Mukhtyar is at my local hospital, he is a saviour, literally.  

There's a light at the end of the tunnel, if you receive the correct treatment you can live a normal existence (other than rattling with all the drugs!) 

All the best 

Marcus 

[Jools52 in reply to Marcus1988]

Thank you so much Marcus,

I really appreciate the reply and it gives me hope of maybe living a life that is less full of the awful symptoms. 

I went out last night and just started to pour with sweat and I mean it was drilling down my back. I had my shirt soak through to my jacket. As you'll Imagine, it really did get me some very odd glances. The lain levels were through the roof too. 

I will ask about this drug and see what the rheumatologist says. I am now a little more hopeful. I wish you all the very best In your own battle

Jools

[may-3-6]

Hi, i understand exactly how you are feeling throughout the eighties i suffered with ulcerative colitis i was told it was IBS and Over active Thyroid. I had most of my Thyroid gland removed in 1988 as i was allergic to the treatment.

I struggled along for years,in 1991 after having my third child my colitis worsened. Weeks were spent trying to control it but unfortunately i had to have an emergency operation to remove my toxic bowel. I was left with an ileostomy.

Many years past lots of ill health, in 2010 i was suffering with lots of symptoms back and forward to my GP luckily she knew i had a problem and had numerous tests. I have always been ANCA negative so that did not help with diagnosis. After having a CT scan i was found to have granulomas in both lungs, after many tests and eventually a Lung Biopsy at the Brompton Hospital was diagnosed with GPA in 2013.

I now have Type 2 Diabetes which again was untreated for 3 years whilst tests were going on to find out what the underlying problem was. I am now on Insulin.

I have also had multiple blood clots in both lungs both in 1991 after my colectomy and last year for no apparent reason.I am now on Wafarin for life.

I have had Rituximab, Prednisalone for treatment trying to reduce my steroids but having problems as my joint pain is so bad.

I have been told by certain specialists that colitis can be linked, or WG can be caused by colitis. I have recently thought that my auto immune health issues are connected.

[Jools52 in reply to may-3-6]

Thank you for your reply. It's really very pertinent as I keep being told that my waxing and waning of the symptoms are unique to the specialist and that he has never seen this in any other vasculitis patients. I will now look into it further as the research lab have said that ANCA can be positive in patients with UC BUT I do have these awful rigors and coughing up blood for some days after these attacks. What the rheumatologist said that normally the coughing of blood is persistent and doesn't stop, in his other GPA patients. I feel like I am being treated by people who are basically digging In the dark and the bone pain in particular is agony the like of I have never had before. 

Thanks for your reply and I hope things get better for you 

[Chris-Bromsgrove in reply to Jools52]

Just for the record, waxing and waning symptoms aptly describes how I was before diagnosis and treatment.

[Jools54 in reply to Chris-Bromsgrove]

Thanks Chris. 

Can I ask, did you ever have any feelings of your lungs being filled with some kind of liquid ? I ask as it seems that I get periods where I get woken by this and I cough up what looks like dark blood or also phlegm but I feel it gurgling in my chest & my Mrs, AKA She who MUST be obeyed, wakes me out of fear that I am suffocating. 

I get this mostly at night and clear my chest when I wake. I am waking with nostrils blocked every day now but this lung stuff is quite worrying and none of the specialists nor GP, have even seemed to have taken any real interest. I wonder if I may be steroid dependent? I was cut to 5mg but have gone back to 10 & it seems to have cleared up but then again, it would clear up during the day. 

I am at a loss with this, it really hinders my breathing 

[Chris-Bromsgrove in reply to Jools54]

JoolsI was lucky with my MPA as my lungs were not affected. It was mainly my kidneys that took the hit. The fluid on your lungs doesn't sound good and I am surprised your medics are not taking it more seriously. You need to try and find out if you can whether the consultant you are seeing also has some specialist knowledge of Vasculitis. Chris

[Jools52 in reply to Chris-Bromsgrove]

I really don't understand it either. I have chronic heartburn before this happens and I had this happen on the day I posted this. I went out, for an evening with some old heavyweight boxing legends, I started sweating profusely and had awful joint pain. I managed to hang it out and got home. I had awful heartburn so took my omeprazole. The night was a bad one and I woke to feeling of not being able to fill my lungs. It feels as if food has somehow managed to get onto my lungs. I then cough it up and it seems to be like an oily acid and has blood mixed in. 

I have told the specialist and GP but lung function tests have proved good and my chest has been X rayed and CT scanned a few times. Apparently my results have shown inflammation but this then rights itself. 

It really is a bizarre thing. 

Oh. BTW I am Jools52 and jools54 as this site somehow would not let me sign in. Sorry for any confusion 

[Chris-Bromsgrove in reply to Jools52]

That sounds like bad acid re-flux. I experienced something similar when I was on a high dose of Pred. It didn't happen too often thankfully but it was very unpleasant. The stomach acid comes right up your oesophagus when you are asleep and you end up inhaling it. However I didn't experience any blood in it. Chris

[Jools52 in reply to Chris-Bromsgrove]

I kind of hope this is the case & I haven't had it happen so badly since I started taking the pantoprazole again. I have had acid reflux before but not to the extent where it had got onto my lungs. I guess I best start taking the PPI drugs again but I have read about a link to dementia with them but needs must. 

It's crazy that I now have not only the 2 auto immune diseases but now have to worry about the side effects of the immune suppressive drugs as they can sometimes be worse than the actual disease that they treat. I guess that 9-10 years of prednisolone will have caused damage. 

Thanks for the reply. I know my 3 brothers & mum all suffered hiatus hernia and my mum telling me she felt like some oily stuff was stuck in the back of her throat and the doctor advising her to sleep more upright 

[Essex-jill]

Hi Jools.

I'm so sorry to read that your still suffering and I'm afraid your not alone in having to battle to get a diagnosis as well as dealing each day with the illness. 

I was diagnosed over 17 years ago and attend Addenbrookes. If you are referred there you will see one of the many specialist doctors there. All the team will sit and listen to your symptoms and their aim is to improve your life and control this condition. They will also refer you if necessary to other specilaist teams , rheumatology, respiratory and ENT. All who have so much knowledge and expertise in our condition. 

Sending best wishes and hope you get answers and treatment very soon. 

Jill 

[Jools52]

Thank you. I just get frustrated when I am suffering the awful fatigue and feel very much like a prisoner in my own home. I don't want to come across all dramatic as I know others are having a rough time but it's all relative. 

I am not sure whether a referral will mean I definitely get to go to one of these places as I've not been through this kind of thing before. I was allowed a second opinion on the IBD & was very lucky that I got into a professior led clinic and so drugs cou,d be used off label. It has probably saved me from having my colon removed. 

The bone pain is the most painful plus the visual disturbance, that scares me most 

[azeyes in reply to Jools52]

Jools tell me about the visual disturbance.  Vasculitis can go into the eyes. I am an ophthalmology  Technician.  Still trying to get a diagnosis.  Suspect polyarteritis  nodosa.  Extreme pain legs. All the time gastro issues mostly from pancreatis.  Fatigue is bad kidneys function is not good. Bedridden most days . Worst part all the doctors that have no idea test this, biopsy that and come back with "inflammation markers are not high enough to cause pain or any issues maybe you need a psychological exam. 

[Jools54 in reply to azeyes]

Hi Azeyes,

At first i had what I can only describe as hazing. When you get a hot day then you see a heat haze & I would get that and sometimes an headache along with it. When I got put on the high dose steroid this all disappeared but recently I have had a more alarming thing where I had what seemed like an "L" shape in one eye and a semicircle in the other where I had total loss of sight and it scared me badly.

I was put onto azathioprine in 07 when I was diagnosed with ulcerative colitis and I did ok on it until they wanted me to up the dosage from 100 mg to 200 mg & I had 6 hours of almost total blindness. It terrified me. 

I had a brain MRI and they found only what they call normal wear & tear for a 52 year old. I am on 15 of prednisolone and 100 of azathioprine right now and have had no repeat of this eye problem for now. I knew it was not right as none of the IBD sufferers had the same symptom. I knew, even as far back as 07, that I had more wrong with me

I hope this helps and good luck in getting treatment. I find that the injections of methylprednisilone really help with the bone pain so please ask your GP about a Depomedrone shot. I call it my angel injection as it really helps me so very much

[azeyes in reply to Jools54]

Any black out of vision is serious. Blood flow to the optic nerve is blocked . Look up retinal vasculitis . Should have a retina specialist checking your eyes

[Jools52 in reply to azeyes]

I did have an appointment with emergency in Bristol but I found they had only 2 doctors and a huge queue. I was told to have my MRI done in the meantime. I got rushed in the MRI suite and was not given time to adjust to the restrictive headphones and headgear they put on you for a brain scan. I basically freaked out. I habe had brain MRI before but not rushed like this. I had been to hospital so many times in the last 6 months and I guess I just broke down that day. I ran out of the hospital and sat in my car just shaking with tears running down my face. 

I am ex Royal Marines and not quick to show emotion. This was a very emasculating experience and it made me feel shame and embarrassment but, luckily, my sight has not been affected since but I do get very sore eyes and they can sting very badly. 

I just need a couple of weeks break from hospital. I take full responsibility for what could happen. It's been non stop hospitals since 07 and I am very tired of suffering. The tests just add more worry. 

Thank you for your reply 

[azeyes]

Yes I have had 5 MRI ,EEG,emg , in the last two weeks . Don't feel bad when you are in pain for a long time everything is difficult. Add a bit of frustration and depression to it. They could have taken a bit of care with you the noise alone is enough to send anyone.