My Journey - EGPA Style

By Paul, EGPA, Congenital myasthenic syndrome, Australia, September 25, 2019

Hi I’m Paul and this is my story so far….

A long time ago in a galaxy far beyond the black stump lived a hermit trying to get by with his electrical contracting business. This hermit was supposed to have asthma but couldn’t seem to stay away from the prednisone as he could only work when on it.

The hermit tried so many things to get better, special diets, special doctors and a very special ingredient….friends with coffee! The not so typical hermit found that he could keep on when using the prednisone. Working and living and doing his favourite thing…Karate. You see this hermit was actually a pretty typical bloke…not wanting to be sick, he just kept on pushing and pushing and pushing until those times when his body pushed back.

Not really liking pleurisy and suspected Lupus he was able to check out of the hospital just after Christmas in December 2011. Looking at all the water in the area he found his way home by following a Gecko driving a yellow cab (by the way there really is a secret society of Gecko’s that drive yellow cabs and follow me around the country) through the swirling water and avoiding serious potholes the size of swimming pools. To his little home in the country. There with his lovely wife and five kids he managed to sit back and do nothing much at all apart from catching the seafood as it migrated across his driveway.

The bloke (so not actually a hermit) tried to do a few things on a weekly basis but just kept getting so tired, he would not sleep (and cleaned the house nightly) and frequently was up for days at a time. He sweated like the guy on the really bad deodorant ads, very often at night. Waking to believe that the floods hadn’t actually receded at all. He got so short of breath that he founded great lasting relationships with his local clinics where they provided him with course after course of his favourite pill. He was such a nice bloke that he actually, single handed, provided for the annuity payments of all the lovely people in the Sudafed company.

Then things went a little worse, he found that if he pushed, then his body pushed back harder. He found walking to be difficult, always painful and training In Karate led to days in bed (without any fun). Realising his options were becoming a bit limited the bloke decided to go for an office job, and actually found one. On that special day of testing and interviews he wasn’t well but managed to succeed. Later on he was informed that he would have to be relocated in order to be trained in his new job. So he packed his bag, jumped into his car and drove on down south to that most mysterious of places, talked about in hushed whispers, always on the tongue and never ready to be spoken about, National capital of Australia, Canberra.

There he was greeted with open arms by a family member with even more issues than himself, a black sheep (not a cute one) instrumental in divulging secrets of science. There he commenced his studies and missed his family. Fate however had other things in mind, his wife flew down to see him and attend an interview also in the south, a week later he had driven up to his old home, packed his old truck and drove it back down with the basics on board, oh and some kids as well.

Thereafter some adjustments were made, he went to work and learned. She went to work and learned and he got quietly sicker. He managed a few months at work before side effects of medications made themselves known by fracturing vertebras, developing a DVT, Pulmonary Emboli and some occasional hospital visits (only lasting a few weeks at a time). There lots of fun tests were made and he found out that Vampires are real, but they actually use needles. Thereafter becoming a seeker of truth he found a GP who would manage his new patient’s neurosis to an acceptable level by actually listening. (Now that’s special)

This professional bromance evolved well, with acceptable levels of intolerance and nice shirts only interrupted by the occasional need for payment. Until a fateful encounter with a pair of shorts enabled a specialist to see his legs (such mighty fine ones at that) where he was told to watch out for that vasculitis. (Oh well just something else right?)

This encounter paved the way forward for his loving GP to say, you bloody beauty! We may have a winner in the form of a diagnosis. Where he did then write and fax a referral to another Dr in that most specialist of fields a Rumourtologist. (well it is in Canberra) Requesting that Wegeners be investigated most urgently.

Where after he was without a confirmed diagnosis (as WG was apparently not the in thing). Months later after getting back on the bike rounds of hospital visits and specialists the bloke saw a web page for a different Dr in another field – Immunology and proscribed henceforth upon his veritable way. This oh rarity of the specialist fields provided an extra letter to the previous assumption of the GPA and Lo and Behold we have an EGPA.

So after starting on the suppressive and repressive meds this bloke came to wonder, miracle of miracles, where are other blokes with this disorder? In his indomitable fashion and after much diligent searching, he found his way onto the CSS Facebook arena, where he found that he may vent his spleen at the circumstances of camel dung in aircraft, Oh and the disease.

And that my friends is where she’s at…Waiting till the MTX becomes effective and I can reduce my dependence on the higher levels of prednisone. So here I leave you, until next time and like my favourite cartoon character I say unto thee most verily…..”You cannot harm me. My wings are like a shield of steel.”

Later on however…….

So my friends, the bloke is still waiting for the day that Pigs have flown faster than a speeding dumpster! Many years and many miles devoted to survival of the Bloke! In all of his native glory, (pictures not attached as they were contracted for use as props in various horror movies).

Lots of shinies! Yes! Serenity is the key to a long and prednisone filled existence! Lots of newer shinies to affect changes in the Bloke and his blokeness!

But seriously now….

A bit of a time line:

2000 – Not a good year (Quite stressful personally)

2001 to 2008 – Poorly controlled asthma, more fatigue, short courses of prednisone (50mg reducing over 5 days approximately every 2 months)

2008 to 2010 – Self employed as an Electrical Contractor, More stress, more fatigue, more general malaise and illnesses, more short courses of prednisone increasing in frequency every 4 to 6 weeks.

2010 to 2012 – Unable to work, financial stress, more illnesses / fatigue / asthma, prednisone short courses

2012 to 2015 – Relocate to Canberra, new job, claw back out of financial ruin, Diagnosis of diabetes, asthma, Cushing’s syndrome, osteoporosis and EGPA. Accompanied by Deep Vein Thrombosis (DVT), multiple recurrence of Pulmonary Embolism (PE), 5 different vertebrae fractures, pleurisy, enlarged heart and of course, fatigue. Oh and the bloke kept training as much as he could and earnt by Black Belt, not too, bad only took 11 years. (and he managed to break his hand at the grading).

2016 to 2019

Well the bloke had a new twist to the story after a bit of night driving, having a twitch he duly reacted, and the lights kept on bouncing. Very appropriate at a demolition derby, not so good on the freeway! Till his next appointment at the eye hospital, there for once he tested very well until the time came to ask a question “What about those bouncy lights?” thereafter he proceeded to MRI Land and Professors in Neurology – you ripper. So on that fateful day he was diagnosed with Myasthenia as well, (just something else right). So, fair suck of the sav, another medication has been added to the collection. The blokes respiratory guy (the one in the suit on the other side of the desk) managed to paper trail the evidence required for the next medication of the journey in the land of blokeness! Yay, there is now Mepolizumab on his feeding roster, great for the asthma but at a 1/3 of the dosage required for EGPA.

Have a nice day….

 

 

 

Share this

Share your photos, videos and your experiences!

Be part of Rare Disease Day by sharing your story with others and sending a message of solidarity! You can write about your story and also submit photos or a video testimonial.
X

Share your story




(To find the english name and more information about your disease, click here). Start typing the English name of your disease. Please choose from the suggestions that appear. If your disease is undiagnosed please write ‘undiagnosed’, or if you would like to list multiple diseases, please type in a list separated by commas.