In the spirit of Vasculitis Awareness Month I am going to republish the story of my diagnosis with Churg Strauss Syndrome, a rare type of Vasculitis.
This is my CSS story. The one I wrote when my world was all confused and I was in both physical and emotional pain. I would like to say things have gotten all back to normal 8 years later but they haven’t. I don’t want to let this stupid illness rule my life but at times it does. It decides when I can be active and when I need to rest. It flares at the worst times and since I am labeled as highly refractory, I have never had the joy of remission. CSS may rule a major part of my life but it does not define me as a person. I still love to cook, and watch my daughter ride her horses. I love to snuggle with my son and have have the weirdest conversations with my eldest daughter. I am still trying to raise so rather co-dependent poultry and laugh and smile whenever I can, which is often!
Please read on, rather lengthy but a look inside myself , my pathway to diagnosis and this rather strange illness.
March – April, 2007
My Churg-Strauss Story
As I sit here attempting to write my story about being diagnosed with Churg Strauss Syndrome I am amazed of how this came to be. The last 7 months seem to be a blur. And my future is a haze. So somehow I sit here caught in the middle. Too scared at times to go backwards or forward. But I know I must go forward. So here is my look back.
Ten months ago (May 06) I was hiking the Grand Canyon with my family without any health concerns. Sure, I was slightly overweight (who doesn’t want to be one dress size less) and I had high blood pressure ever since the birth of my 3rd child 9 years ago. But that was all manageable; 1 tiny pill a day. I was an ex-teacher turned supermom and loving every moment of it.
After 9 years of living with a leaky roof I finally got my husband to agree to a remodel. The work began the 1st week of September 06 with a total roof tear off; lots of yucky black stuff fell inside the house.
My first signs of illness began by the end of that week. It started with hives – mean and ugly ones. Then came a cold with a cough but the cough never ended and I ended up at an urgent care center. I was diagnosed with reactive airways and sent home with my very first albuterol inhaler and prednisone (little was I to know that prednisone was soon to become my best friend). As the prednisone was tapered down my symptoms became stronger and now I was running a fever and having night sweats. So back to urgent care where after some chest x-rays (told they were mostly ‘normal’. I really want to know why doctors use words like ‘mostly’ normal.) I was put on more prednisone and some antibiotics.
Two more weeks passed and by now I was coughing so hard that I was throwing up. Back to urgent care and my first nebulizer treatment. WOW! I thought that was amazing. I could actually stop coughing for an hour. It was short lived.
Finally I broke down and went to my regular internist. I started crying in his office because I felt so bad. He listened, shook his head, patted me on the shoulder and stated I had acid reflux (GERD). Another round of prednisone and antibiotics were started and an acid reflux pill. I walked out of his office in disbelief. I was so upset and I coughed so hard, I threw up on his sidewalk (that will show him!).
By now the only way I could sleep was by sitting up and using the nebulizer every 3 hours. But if it was only acid reflux, then what was my problem? I got ANGRY! This was NOT all in my head. I called an asthma/allergy office and after crying on the phone obtained a same day appointment. It was here that I meet my savior (aka my asthma doctor). He walked in the room and even before meeting me put his hand on my shoulder and stated, “Man are you sick!” Then he sat down and stated, “Talk to me…” It was the most refreshing three words I think I had every heard. And he listened. Really listened! Never interrupting, never judging. I suddenly felt like I was going to get better.
The next weeks are a blur. CT scans of chest and sinus, bronchoscopy of my lungs, allergy testing that showed I had all these allergies (shocking since I had never had an allergy in my life), visits to an ENT (who recommended I go off all inhaled steroids!), visits to an eye doctor because suddenly I was getting blurry vision and my eye pressure was dramatically increasing, too many blood tests and once again an increase of prednisone to 60mg (by now I was gaining weight and began referring to prednisone as ‘evil candy’ – I hated it but needed it to breath). And what came of all this? A diagnosis of severe, persistent asthma that was hard to control. Sure they found a rather large amount of Eosinophils (EOS) in my lungs but I did have allergic asthma after all. I was now taking 10 different medications a day! But I was functioning – sure my resting heart rate was over 110 and my blood pressure was dangerously high; but at least I was functioning again! How bad could that be? My asthma doctor had nicknamed my cough as ‘the cough from hell’ and every time I went to see him he always started our conversation with those most comforting 3 words. “Talk to me….”
Then came the sinus infection. More antibiotics, more prednisone. I was having a bunch of other strange symptoms too (my one kidney was hurting all the time, I was breaking out in strange rashes, I had this funny pins and needles sensation in my left hand, my chest was hurting) but I kept them all to myself. I began to think that I was making this all up! I must be! It was all too strange. I mean, I must be a middle age, bored housewife that had nothing better to do but go to different doctor’s. I began to hate myself and my body.
Once off the prednisone (again… can you say taper #8?) Things went downhill quickly. I called the asthma doctor again and asked him I thought it was funny how I always broke out in strange rashes a few days before a major asthma attack. He sent me for another set of blood work. He wanted it done when I was off prednisone.
He called me a few days later and wanted to see me in his office. It was at this appointment that I told him I really thought this was all in my head. I kept apologizing for wasting his time. He listened, I talked. He got me to ‘fess-up’ to some of my hidden symptoms. He was surprised that I had so many other symptoms. I explained to him that he was my asthma doctor and since my internist thought it was nothing (and prescribed anxiety pills for me) I didn’t want to bother him. At this point the asthma doctor showed me my blood work – he explained that my EOS level was at 25% and I was showing signs of a kidney infection. I begged not to go on prednisone again (the mood swings and the weight gain were getting to me). He used the words ‘organ failure’ and I unhappily accepted the pills. I would taper off prednisone once again and then we would do a skin biopsy and another blood test. I was scared! I knew something was not right. The asthma doctor spent an hour talking to me in his office when I told him I thought he should go because he was backing up other patients he answered, “If they don’t understand than they shouldn’t be patients of mine”
This was the first time I had an out of body experience. I realized when he was talking to me his voice became far away and I was an observer of the conversation. This would happen a lot to me in the coming weeks. I soon came to discover it was my coping mechanism.
The next time I tapered off prednisone we attempted to find the definitive diagnosis. It was at that time that I wrote this. I think it is best shared in its original format
1 day… 4 doctors.. long day…
Quest for diagnosis continues… Sometimes I think the doctors want
me to become REALLY sick in order to diagnose this! I’m not in the
hospital so I must be OK is the thought…
Today I got to see my asthma guy (who I love!) – and he basically set up a bunch of other specialist appt for me in the coming weeks. He still thinks it’s Churg-Strauss even though my ANCA tests come back within normal limits. Just came off a prednisone taper so we can run tests this week and my cough and my rash is taking over more body parts every day.
Did the blood test thing (note to self.. blood techs don’t like being
referred to as vampires…)
Went back to dermatologist to have him re-stitch one of my biopsy
sites. Although my husband offered to do it at home I just thought it was more sterile to go back to the dermatologist.
Visited my friendly neighbor eye doctor to check eye pressure. Still high but just shy of official glaucoma. Noticed he got new office furniture; asked if my frequent visits had helped pay for it. He laughed nervously.
Then it was off to the pulmanologist. We played the “what makes you think this is anything but normal asthma game”. He had copies of my latest blood work — did a chest x-ray and then came back into my exam room and said he thought it might be obscure auto immune thing called churg-strauss (no **** sherlock!). But he had to run to an off site appt. — as he was leaving he keep saying he needed to see me again. I told him to have his secretary call my secretary. He gave me one of those strange looks.
You know sometimes I think my strange sense of humor is the only thing keeping me sane. In between appointments I went to a book store to look at cookbooks — while there I started crying and couldn’t stop – very embarrassing. I think I am fine as long as I am busy. It’s those quiet moments that sneak up on you.
Have an appointment with a cardiologist at the end of the week. My heart just likes to race and make me short of breath. And the earliest neurology appointment I could get was in early May (numbness and tingling in left hand) Now they also want to send me to kidney specialist (darn kidney just wants some attention — I told the asthma guy that it just felt neglected and we should ignore it. He gave me one of those looks again.) and they also want a GI consult — Hey I thought it was a good thing that on this last prednisone taper I actually lost 10 pounds — I think it all averages out in the end.
It was at this time that I realized I needed to write things down. I suddenly felt the need to sort my feeling out. Too many things were happening.
Within a few days my diagnosis came in, the date was April 1st, 2007. The skin biopsies showed vasculitis. My EOS level was back up to 21%. My asthma was unstable again and the rash was taking over major parts of my body. When I got the call stating it was Churg-Strauss didn’t know what to do. I felt like I was drowning. I explained to my doctor I was waiting for him to say, “April Fools!” – Although I had emailed family and close friends preparing them for this diagnosis; I think in my logical, systematic approach I neglected to prepare myself. Would you believe I even sent a letter to my asthma doctor making him feel better about having to give me such life changing news? I am so busy trying to make others feel better about my illness that I forget that I am the one that needs the comforting.
I tried to explain it to my husband that I feel like I am running and if I stop this illness, known as Churg Strauss, is going to run over me like a freight train. I just keep moving. Currently I am at the stage that we are trying to figure out what organ systems have been affected. We know it got to my lungs and skin. We have a strong suspicion it has affected my heart (the uncontrollable blood pressure and the tachycardia are all symptoms). I still have to visit a neurologist to see about that tingling in my hand. There are questions about my kidneys (you mean one shouldn’t be able to feel their left kidney?) Since I have lost a lot of weight (18 pounds in 3 weeks but I look great in jeans now!) they are now questioning my GI tract. It is overwhelming! I keep running from doctor to doctor in hopes that the train will not catch up to me.
I am scared. Who wouldn’t be? I have these unseen cells in my body attacking. I even went through a stage where I refused to eat for a week, deciding that if I didn’t feed these cells they wouldn’t multiply and harm me (dumb! I know!). I am grieving. A good friend helped me understand that having an illness like this makes you grieve. Grieve for what you lost, that carefree feeling; the not worrying about every ache and pain. I know that will settle down in time too. Logically I know – emotionally I am in turmoil.
A few days ago I crashed. Emotionally. I went to a doctor’s appointment only to forget that it was rescheduled for another day. After leaving the office I began crying – I felt like that juggler that has all those balls up in the air. This was my first ball to come crashing down – I panicked, thinking that once one ball came down they were all going to fall. I got in my car and began driving – to nowhere in particular – the important thing was that I was ‘running’ – I needed to keep moving. I drove, and drove and drove. Then I got tired and stopped. I fantasized about going to the airport and getting on the first plane to anywhere; just to run away. I knew this was neither logical or sensible but it was emotional. It was at this time that my phone rang and it was my asthma doctor, he uttered those three words I used to find so comforting, “Talk to me ” but this time I answered NO! I think I scared him! He was trying to figure out if I should increase my prednisone again, since my rheumatologist appointment was still 3 days off. I was running and he was interrupting. He told me to, “hang in there” ; I said it was easy for him to say. He apologized. I hung up after agreeing to another 10mg of prednisone increase – he kept stating that he didn’t want to visit me in the hospital. After a good cry I returned home to my family. I knew that is where I belong (but a big part of me still wants to run away from this illness).
We will be throwing this thing into remission in the coming weeks/months and then it is all about being vigilant with weekly/monthly blood tests to make sure it stays in remission. The drug mix they use for remission is not always a pleasant one but it’s better than the alternative.
Everything will work out — I will be limiting my activities. (what no skydiving?) The annual camping trip is definitely a no-go this summer. The only camping I should be doing involves room service and a butler. And it’s a bummer that I will have to hire house cleaners two days a week now (no downside here) I have to remember to rest a whole lot more. A tired body is ripe for the overtaking by these cells.
There are humorous moments during all this. When the dermatologist grabbed me by the arm and keep patting my hand as he lead me to a chair to sit down (note to doctors, don’t ever do this! You freak the heck out of your patients!) He then asked me if I knew what vasculitis meant? I was so tempted to say “No, is that something bad?” but I couldn’t I let him off the hook and let him know I realized the seriousness of my illness. There are those days of getting blood taken where you have that hidden set of plastic vampire teeth in your purse – not daring to take them out but it makes you smile as you get poked one more time. There are those well meaning nurses that hand me a glass of water to drink when I am in the middle of an asthma attack (I cough rather than wheeze so this confuses them) – I say thank-you politely. There was my dentist with a sad look in her eyes when I told her about my illness – she asked if there was anything her office could do for me. I mentioned my house needed to be painted. I need humor to get through my days – sometimes it allows me to stop running for a little while.
I found myself stating to people how ‘lucky’ I was that my illness was caught early. But darn it—I am NOT lucky! I hate that word! I find myself using it as a defensive mechanism
ie: I am so lucky I have a doctor that kept testing things.
I am so lucky in was caught early (wait I have vasculitis already and am in stage 3)
I am lucky I can afford this illness (who in their right mind can afford an illness?)
I am lucky I have kept myself out of the hospital (that’s not luck; that’s pure stubborn woman!)
There is nothing LUCKY about having Churg Strauss Syndrome! It STINKS! But with time and patience I will learn how to adapt my lifestyle. I will stop running and the freight train will not run me down. And when my wonderful asthma doctor says, “Talk to me” I will! I need to talk and to cry and to scream.
I have learned that life is a precious thing — we get caught in our daily activities and forget that. My new message tagline is, “When life gives you lemons, say they are from a small independent organic farmer and sell them for three times as much.”
Go to Karen in Wonderland CSS / EPGA pages
Return to Karen in Wonderland blog posts