Egpa brain issues

[Cathywp]

Hi! I was searching the web and found this site. I live in Canada and got super sick in sept 2016. It has taken a long time but I now think I likely have ANCA negative egpa. Is there anyone else out there with that? My family doctor has been no help at all. I was hospitalized in sept 2016 after what I thought was three weeks with a bad flu...after four trips to emerg and two visits to my doctor someone finally checked my blood and my eucinophils were at 17,000 so they admitted me... by then I was really not conscious and had brain damage which I m still dealing with. What a long and bumpy road! I don't understand why it seems so difficult to get a diagnosis. When they released me from hospital they said I had idiopathetic hypereucinophilic syndrome and I was put in the care of a hemotologist. Although all bone marrow tests came back negative she kept insisting that I had some kind of mylioperiferative bone marrow thing and she wanted to start me on a chemo drug....I decided to go to the mayo clinic in the United States for another opinion and they said that i don't have that and to look further at my respiratory system.... six years before that my family doctor said I had copd....I always thought that was wrong too but whatever....so if I wanted to have any chance of getting better it seemed that I would have to figure this all out myself. I got my records and started educating myself.... I argued with my family doctor a lot. She insisted that since my anca tests were negative I could not have egpa....I believe that is simply not true and I probably should get a new family doctor but it's hard to find one here. After lots of confrontation I was able to get a referral to a rheumatologist who thinks I may have egpa and a respirologist who really thinks I have had egpa for a long time ....

But what the heck is going on with my brain? I don't understand how this is responsible for brain damage if it is a respiratory thing...they say my brain scans show that I had several stroke like attacks and permanent damage but the brain doctor I was sent to says no need to follow up any more and that's it... I am very worried it might come back since I don't understand what happened in the first place....if this is common in anca negative egpa then shouldn't all the specialists know about it?

[Hidden]

Hi,

Although EGPA affects the lungs in most patients it's not primarily a " respiratory " thing and can attack any tissue that has a blood supply ( e.g. anywhere ).

It's a very rare, complex disease ( 1 or 2 patients per 500, 000 ) and certainly brain involvement isn't the norm.

Only 40% of patients with EGPA are ANCA positive, there seems to be 2 different subsets of EGPA, Vasculitic ( with positive ANCA ) and allergic where asthma etc is more the problem. This makes the diagnostic process more fraught.

As with all rare disease the most important factor is to see a Consultant with experience in diagnosising and managing it, they are few and far between.

You may find this article useful, Dr Christian Pagnoux has written extensively about EGPA, might be worthwhile seeing if you can get an appt with him or asking your current Consultant to ask him for advice.

cssassociation.org/tl_files...

[Hidden in reply to Hidden]

This is the latest consensus statement re diagnosis and management of EGPA. Might be worthwhile asking your Rheumatologist if they have read it........

ejinme.com/article/S0953-62...

[Cathywp in reply to Hidden]

Hello Keyes

Thank you so much for your advice and explanation. I really appreciate the information. Perhaps because it has been so difficult to confirm my diagnosis I have never discussed autoimmune conditions with any of the doctors I have seen. I have only seen my rheumatologist twice and she likely has no idea how confused I am. I did not realize that vasculitis is the biggest part of egpa. Since reading your reply I have read about central nervous system vasculitis and perhaps that is what's wrong with my head? I worry that it is happening again as I am dizzy and weak and my vision seems weirder than usual and I have a headache again. I called my rheumatologist and asked the nurse to pass on my symptoms as I am not scheduled for a revisit for a few months. I guess I can't do much more. FYI last summer I tried to get an appointment with the specialist that you recommended but it was not possible. Hopefully I get better soon.

Thank you again for the information.

Cathy

[karens62]

Hi, I am also in Canada, near Toronto. I am anca negative too. I had what they labelled paranasal activity [middle ear infection, nasal polyps, sinus infections] for about 5 years before I ended up in hospital with heart and lung failure [2006]. The diagnosis relied on the presence of infiltrates in the lungs, the simultaneous problems with the heart [myocarditis) and previously un-diagnosed asthma. It's really hard to diagnose and so rare that I've found that there is a reluctance to diagnose it. I still have scars in my lungs but they say there is no reason to worry about it as the disease is not active now and the lungs are slowly repairing themselves. I hope you manage to connect with one of the vasculitis specialists near you as you should be able to get a better explanation of what happened.

Best wishes, Karen

On another note: I see Dr Rachel Shupak at St. Mikes. I've had EGPA for 12 years and have been in remission for 10 of those years. I'm still taking mycophenelate but have tapered to 500 gm and will do what I hope is the final taper in April.

[Cathywp in reply to karens62]

Well nice to meet you Karen

How were you diagnosed? I only thought Chung Strauss because it fit some of my symptoms but because my ANCA was negative and I had no rash they all said it couldn't be....I have been so sick for the past ten years and I couldn't get any help until I had super high eucinophils and brain damage! My husband took me yet again to the emergency when I could not remember how to use a cell phone.... It looked interesting but I had no idea what to do with it! My lungs are damaged and will not get better...my brain is damaged and I hope it can get better but time will tell....I asked if they would send me for some kind of rehab but the blood doctor says it's not technically a stroke so no rehab.... no one can explain to me exactly what happened to my head but apparently the blood flow was cut off somehow... the blood doctor says maybe all those eucinophils plugged up the veins. I quit seeing her. Now I have a rheumatologist who thinks it may be churg Strauss but not sure. Somebody has mentioned lupus. My new respirologist (I quit the previous one because when my lungs started to get really bad ten years ago he just said I had COPD even though I don't smoke. I had bronchiastisies and a collapsed middle lobe and he said he had patients a lot worse off than me. Probably true but not helpful) anyway, my new lung doctor says he really believes it is churg Strauss . The lung thing fits as does all my sinus issues and years of ear infections but he also can't explain the brain issue or the huge fatigue and muscle weakness and tight elastic band feeling. And no one has any idea why I am super hot and then chilled feeling all day and night. They now tell me that I should not dwell on getting an actual diagnosis and forget about it because the treatment is the same regardless but I sure don't understand that. I am now on prednisone and azathioprine. I am trying to wean off the prednisone, currently down to only 3 mg daily, but I think my brain is getting loopy again and my eucinophils are on the rise again so I'm not sure what to do. Prednizone has given me glaucoma and I only have vision in one eye so I don't want to mess that up! Man I am a disaster! I live in Winnipeg and I tried to get myself referred to the one place in Canada that has expertise in ANCA negative egpa (mount sinai in toronto) but that didn't work either. Geez Karen you r likely sorry you answered this post! I'm really not a poor me type person at all. I just want to get this all figured out and hopefully before my poor husband cant take it any longer. He has been supportive but come on! No man wants to be a nursemaid to a wife who is constantly sick without any REAL good reason. Sighhhh thoughts???

Sorry I guess I am just sooooo amazed to find someone with perhaps this same thing I went on a bit!

Cathy

[PattyMPA in reply to Cathywp]

I read that Prednisone cataracts go . away after ending Pred if that us possuble.

[Hidden in reply to PattyMPA]

I don't think that's true.

I was on pred for 2 yrs and developed pred induced cataracts after coming off prednisolone.

[Cathywp in reply to Hidden]

Wow prednisone can cause lots of havoc! I have glaucoma from it....I probably can't get cataracts again since I already had that before steroids! Man! Getting old really sucks....but it's better than not getting old lol!

Cheers

Cathy

[Rarity1 in reply to Cathywp]

Hey Cathy, I have egpa Vasculitis, I’ve been to hell and back. Try methylene blue as it’s helped me massively. It’s so difficult because no one understands us x

[karens62]

Hi Cathy,

I thought it might be easier for us to talk via message instead, so I sent this reply to your messages as well. I was diagnosed in 2006 after a lengthy coughing spell, which I thought was caused by bronchitis. I went to emergency because my chest was hurting and I thought it was maybe asthma. I'd been told I had mild asthma a few years earlier. Anyway, I ended up waiting in emerg for 6 hours! Then they took a ecg and found that I was producing troponines which is a protein in the blood, released during myocardial infarction. I was having a heart attack and at the same time, the x-ray showed that I had pneumonia. Then the blood results showed elevated eiosiophils of 4,700. The on call doctor happened to be an internal medicine specialist who had heard of Churg-Strauss from a colleague 20 years earlier. He said that with my health history [no heart disease] that it didn't make sense to have two organs - both heart and lung - in failure.

Treatment started with 100mg prednisone [2.5 times more than the usual max. dose] and then added Cellcept [mycophenalate] after two months of tapering [to 60mg]. Then it took two years to finally taper off prednisone. Now, 10 years later, I have had no flares although the eiosinophils have been as high as .900. Mostly they are at .400 to .600, which seems stable for me. Also, I have tapered to 500 mg mycophenalte a day.

I responded to you other comments in another message to you directly.

Best wishes, Karen

[Cathywp in reply to karens62]

Hi Karen

Wow! You were really sick! It is great to hear that you have been able to be in remission for ten years. I think it was a lucky coincidence that you ended up in emergency that day with a doctor who remembered that he had heard about churg Strauss years earlier. So how is your health after ten years? How does egpa impact your life now?

I am not much of a computer person at all so I don't really understand different options for replying to you. I hope you are able to read this post.

Thanks

Cathy

[Rarity1 in reply to karens62]

You sound like exactly what I experienced too. The methylene blue harnesses your mitochondrial system and repairs it and eventually you start feeling better

[Rarity1]

sorry to hear about your experience. I have had similar experiences with doctors as our conditions are very hard to deal with and navigate through with the correct treatment. I have found my own way through a lot of my symptoms by using chlorine dioxide solution to cleanse my body of anything acidic and all the pathogens. Once I did that I started using methylene blue pharmaceutical grade. Don’t bother googling it as they lie about so many things. It’s transformed my life. It works on treating our mitochondrial dysfunction where all disease and viruses start then spread. I honestly believe it would help you. Try the book by Mark Sloan “a guide to methylene blue “ It’s available on Amazon. This will help you understand how it works on our cells.