Patient Stories- CSS Around the World
In Our Own Words, a forum for folks affected by CSS to share their experiences, is one of the most popular features of the CSSA website. We are very pleased that three patients, from around the world, have shared their stories for this May, 2007 update.
Light at the End of the Tunnel By Graham Shore
Thirty-Five Years with Churg-Strauss (and counting.....) By David Moreland
Talk to me...
By Suzanne
March 2007
As I sit here attempting to write my story about being diagnosed
with Churg-Strauss 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… But 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’ll 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 pulmonologist. 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 appt with a cardiologist at the end of the week. My heart
just likes to race and make me short of breath. And the earliest
neuro appt I could get was in early May (numbness and tingeling
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 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 I 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 tinkeling 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
appt 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 retuned 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…)
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-Struass! 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 be 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. And 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...”
Suzanne
Light at the End of the Tunnel
By Graham Shore
May, 2007
My name is Graham Shore. I was born in Sheffield, UK, and this is an account of my experience of Churg-Strauss Syndrome (CSS). Note that not all incidences of CSS are anywhere near as severe as my particular experience.
I was born 27th December 1961. My earliest recollections of health
problems are the usual run of measles, chickenpox, mumps but also
severe anaemia. Apart from this, I was remarkably healthy and never
even suffered with hay fever, let alone asthma.
Around 1989/90 I started to have sinus problems; I put this down
to moving in with a girlfriend who had a dog and I guessed it was
some sort of allergic reaction. Anyhow, back in my own house and
things got worse; after walking home one evening in very cold air
I had my first real breathing problems, but these were gone in the
morning and I thought no more about them.
The breathing problems got much worse over the next few weeks and
I ended up not only with asthma but I also lost a lot of weight
(down from 82.5 Kg (13st) to about 63.5 Kg (10st) and was totally
without energy. It was 1992, and the doctors were perplexed.
Fortunately, I have lived in two great areas for UK hospitals, Sheffield
and latterly Redhill then Leatherhead, near London. At this time
in Sheffield I was the talk of the medical practice because nobody
had the slightest idea what the problem was!
I had a chest x-ray, and immediately ended up in out-patients ward
of the
Royal Hallamshire Hospital for an examination. The radiographer
thought the film was fogged and had to take the image again; the
consultant said it looked as though a bomb had gone off in my chest.
I was jaundiced too - bright yellow and my boss thought I had cancer.
I was admitted for a couple of nights, so that an exploratory bronchoscopy
could be carried out. They needed to know what was actually in the
lungs; they did this by removing a tiny bit of the inside of the
lung.
Now, the diagnosis for all this was a rare syndrome known as Loffler's syndrome, described on the net as 'mild'!! High doses of prednisone and some rest saw me make a full recovery. However the sinus problems came back again and in a couple of years I had to be admitted for an operation to remove nasal polyps.
So, operation over I thought that was the last of it for a good many years. Wrong. The asthma attacks were severe, sometimes requiring time off work, and of course very uncomfortable.
One evening while lying in bed after a very bad day of asthma, my heart stopped, or at least it felt that way. Both my arms felt totally dead, just like two lead pipes attached to my body. I juddered and came to, but it was very frightening. The next day my partner Sue took me to hospital and they admitted me straight away when I recounted the story. The staff at the hospital thought I had developed the initial stages of ischemic heart disease, and were sorry to ‘tell me the bad news’. But my ankles were not swollen, and I didn’t exhibit any other symptoms of heart failure. Odd. So I left the following day and started to eat more healthily and even to run a little, which did help until I sprained my ankle and that had to stop.
Another year down the line and its 2006. I started a diet around April time. I had had two weeks off work with a bad chest infection and asthma, and by now was getting royally p****d off with the situation (as were my employers). I took drastic action to loose weight; I went on a carbohydrate free diet (not Atkins) and got totally immersed in feeling better about myself by losing weight. That’s right, a 44 year old male with anorexic tendencies. I lost about 19Kg (3 st), but felt good, hardly any asthma at all. Then the asthma vanished completely. Pleased with myself, thinking I had conquered my condition single-handedly, I could have stopped dieting, but I didn't. Not only that, but I watched my weight begin to really plummet. This was getting silly - about 1Kg (2-3 pounds) lost each day. I was getting weaker too - I noticed I couldn't carry the shopping bags back to the office, and had to start using the lift again (breaking my new years resolution!). It was time for some more sick leave, and a visit to my GP. He didn't think that much was wrong, and was even patronising in my opinion. However I went back a few days later and demanded something be done. He could tell something was wrong this time, and got on the phone the local hospital (East Surrey in Redhill) and I went along to the A&E - they were primed as to what to expect. Fortunately, at the time I lived literally next to the hospital. I had a long wait sat on a hard plastic chair, then a chest x-ray and some blood was taken from an artery in my hand; the result came back that I had had a 'Silent MI' - a heart attack. I was admitted to the Coronary Care Unit (CCU) that evening.
Not feeling too bad by now, but mildly shocked by my predicament, I spent a day in hospital more or less recovering then saw several visitors the following day. My readings were good - sats were up, blood pressure ok too; so I was alright if a bit weak.
*
It was a Sunday afternoon; my friend Lisa had been to visit for about an hour, then went off to see John’s flat, a mutual friend who lived in the same block of apartments as me. I can’t remember if there was any time in between, but Linda, the receptionist from work, came to visit me. We chatted and everything seemed fine; I was doing well as far as the readings on my monitors were concerned, and pretty soon the nurse came round (Keith I think) and declared that I was doing well and shortly after another nurse came round to say that I could actually go from the CCU ward onto the less dependant Holmwood ward, with patients who were mainly in for stent’s and so forth.
I couldn’t walk very well; I was shaky when I stood up and
Linda had to hold my arm and escort me into the other room (ward).
She was disgusted by the fact that my belongings were summarily
dumped on my bed and there was no help to move to the next ward.
Anyway, in the next ward I soon settled down and chatted to Linda
until Mac and Ralf came to visit. This was very pleasant and we
chatted for some time about lots of things; I remember Ralf recounting
what his arrhythmia experience was like and I hoped I didn’t
suffer a similar fate. One of the things he said, it might not have
been at this particular time, was that it was like the long fall
on a big dipper, but without the slowing down and stopping at the
end! He said you just kept on going, and it was terrifying. After
they left I had to wait a long time for some dinner (I think they
had forgotten me) and then I asked one of the nurses if I could
have a bath. This was the first time I had met Barry (Baz), a real
character but underneath it all a genuinely nice guy. He hummed
and arred for a while but eventually agreed and led me to the little
bathroom. The bath was some large hospital type bath, with very
easy access. Although I was weak and clearly not very well at all,
it was comforting to get clean and relax off the ward for a while.
When I was finished I felt better for the bath, but not better in
general; in fact I felt quite a bit worse. I didn’t sleep
at all well that night, and I had to get up in the middle of the
night and go for a walk to get some tea. I can remember not feeling
well at all; I could hardly walk and I had to lean heavily on the
nurse’s desk while I was asking for the tea. I went back to
bed and slept fitfully for a while; I was woken about 7:30 by the
general bustle and chatter in the ward. The guy across from me was
talking to someone at the opposite end of the ward so he had to
shout; I nearly complained but in the end decided to let it go.
However, a few minutes later I took a turn for the worse, and I
can remember sitting up on the side of the bed, crouched over. I
said to the noisy but kindly chap next to me that I wasn’t
feeling too well; he said “Shall I get the nurse?”,
to which I replied “I think so” and he put on his dressing
gown and went off to find one. The next thing I can remember is
that quite a few nursing staff were busily preparing to move me,
and one nurse asked if I would like some morphine to ‘take
the edge off things’. I said yes; I think it was 250ml. Barry,
the male nurse, said “I knew we shouldn’t have let you
have that bath”.
Then I was carted off to a side room and filled with some drugs, another nurse asked if my next of kin could be contacted; this was to become a familiar question in subsequent weeks. I always replied yes, and didn’t think any more of it; after all I didn’t have a clue what was happening. However I did say to one of the nurses “I’m poorly aren’t I?” and she replied, “Yes Mr Shore, you are very poorly indeed”.
I held onto the steel guard adjacent to the bed, and thought to
myself “well, I am at peace, and I am not in any (apparent)
pain. If this is it, then it’s not a bad way to go.”
At that point, I believe I was given the choice of life or death;
I can’t remember this as a conscious thought, but that’s
how I felt. There was no terror, no trauma; it was a concentrated
feeling of living in the moment, a nexus of life experiences; it
was almost beautiful.
Having survived this I found myself in intensive care and everything
had moved up a gear.
*
I was sent to the Intensive Therapy Unit (ITU) and given Cytoxin (cyclophosphamide) and put on a machine called a CPAP, which helps to relieve strain on the heart. This carried on for a while, perhaps a week. I was very weak, could hardly move, and the descriptions of life in Ben Watts book “Patient” fit me perfectly.
I was doing well-ish and ended up on the CCU again, but worse was to come, much worse. I arrested in my sleep and woke up to find several nurses and doctors buzzing around me. A central line was fitted into my neck. They didn't really know what to think, and were in constant consultation with Royal Brompton Hospital where the first suggestions that it might be CSS were mooted. The eosinophil count was sky high, and my temperature elevated. They were disappointed; they said I was "doing so well" and talk was of putting me on a ventilator. A few more days in ITU and I was put back into CCU, same bed, same spot near the window, quite nice really....
*
One day I felt very poorly. It was a Wednesday and some friends came to visit me. They were shocked. One of them said "Graham, I have had a full blood transfusion, if I can survive that you will get through this". I thanked him for his good-natured comments. Later, some more visitors; one chap held my hand but I was drifting off to sleep; the last thing I remember was he squeezed my hand and left. There were nurses and various medical staff lined up along the corridor; I was moving past them, slowly at first and they acknowledged me but no more than that; the pace crept up and I was moving faster and faster past them, until I was in a spin and the whole scene was out of control. Oh! I shouted and jumped upright from my bed. I had been shocked back to life by two pads stuck to my chest; the chest hair was burnt and I was breathing rapidly; several nurses and the doctor were at the foot of my bed. Back on the corridor, the staff lined up. I moved past them and again was acknowledged. I've been here before, and it started to get faster and faster and the room span and Ahrrh! I was jolted back to life again, same thing, doctors, nurses staring intently at the readings on the machines behind my bed. Back on the corridor, same story, medics lined up and moving past them ever faster till Arrrh - jolted back to life. This time I remained conscious.
A WONDERFUL nurse called Shoona, every inch the consummate professional, had taken hold of my hand and was gently rubbing it; it was so comforting I can't begin to express my gratitude.
The staff were watching my machines intently, looking for signs of stabilisation. "Ok Mr Shore, you've had an arrhythmia storm, but you seem to be stable now” the doctor said. A few seconds went by. Initial crises over; the heart had been so weakened by the CSS it had failed; it was "fat and floppy" as one cardiologist put it. “We are going to put a temporary pacing wire into your chest, so we will need to take you to the procedure room”. Great, I thought. Then came a long explanation about the dangers of the procedure, which made me snap back “JUST DO IT!”. I apologised, and the doctor said “Mr Shore it’s perfectly understandable under the circumstances”. Still, it’s never a good idea to upset the one person in the world who is about to save your life.
So, we went (my bed was being pushed) to the procedure room. I wasn’t scared or anything; I knew it was serious but I was looking forward to the pacing wire going in so that I would remain stable. We reached the room and I was prepared; I was placed under a large x-ray machine and some iodine was dabbed on my chest; the operation was carried out under local anaesthetic. The doctor remarked “you are almost the perfect patient – you just lay back and let it all happen”, to which I replied “I haven’t got any option”; not in a nasty way just as a matter of fact. She was brilliant, young and very, very good at her job; she even tutored a student doctor on the procedure while it was happening; he asked all the right questions and she told him it was a good skill to have (Good! Bloody marvelous I would have said!). So, the small operation over and I was wheeled back into the CCU. It must have been about 5 in the morning.
*
Two friends came to see me, Shaun and Kathleen.
Kathleen had been tremendous throughout my stay in hospital, a professional
nurse herself, she had run errands for me, checked the medical side
of things with the staff and had generally been an angel. Amazingly,
she was moving into new accommodation so was living with a friend
just around the corner from the hospital during my illness! The
great doctor who operated on me had got to know her well and had
phoned her for ‘tea and biscuits’ – a euphemism
that I was in imminent danger.
Shaun is a great friend and confidant; a hard man athlete and rugby
player, he always seems to know the right thing to say. They walked
in, one either side of my bed, both silent for a moment. Shaun knew
what to say: “Graham, you look scary”. I half smiled,
clearly seeing that he was concerned I said “Don’t worry
mate”, but it was too late for that, he was very upset. Kathleen
was too, but used to dealing with these situations she took a more
pragmatic approach. “GRAHAM! Enough already!” she half
shouted at me. Then she proceeded to plead with me to tell my sister
to make arrangements for my death, even though “God forbid
anything like that should happen.” It was true; I had purposely
kept my sister a little in the dark, and not told my elderly parents
anything at all, save that I had had a bad asthma attack. After
that the conversation was brief, and they left me to get some rest.
I thought it was early evening still, never thought to thank them
for getting up in the middle of the night.
A nurse appeared and asked if I wanted a sleeping tablet, Zopiclone,
which usually did the trick. I admitted I was frightened it might
spark off the arrhythmia but she assured me it wouldn’t, and
anyway I was in exactly the right place if anything did happen.
I drifted off till morning, all things considered not feeling too
bad when I woke up the next day. But I couldn’t know; I was
motionless in bed and any chance of me getting out of bed was nil.
More phone calls were made; they were desperately trying to get
me into either the Royal Brompton or Harefield hospital where I
could be treated by specialists. I think it was a couple of days
later that I finally got a bed at Harefield (it should really have
been the Brompton but there was ‘no room at the inn’).
I was taken by ambulance, flashing lights and siren when the traffic
got heavy. I remember looking up at the trees from the window (my
narrow field of view only allowing anything higher than 20 feet
to be in sight). I somehow felt much better just for getting out;
it was relief after being cooped up for so long.
I got to Harefield and was taken to the heart transplant ward; this was disconcerting, I hadn’t considered that as a possibility. I was placed in a room in the very high dependency section known as the ‘Square’, where all the nurses were kept busy by their monitors. The food was being prepared just outside my door; I was ravenous, being on large doses of intravenous steroids. Dinner was served and it was delicious – all the food at Harefield was great, come to think it was good at East Surrey too. Two friends dropped by (Kathleen and Olivia) with my favourite fruit juices and soya milk (I still had a reaction to cow’s milk) and a big box of chocolates and some books.
The specialist came in to discuss what was about to happen. I was informed the blood pressure was adequate, so I would not be put on any other machines. The treatment was basically to be a course of medication, to be changed over time until they found just the right combination; Ramipril and Prednisone were high on the list. So far so good. Later that evening, a consultant came in to see me and told me that I would need a pacemaker fitting, a special type known as an Implantable Cardioverter Defibrillator (ICD) “to prevent sudden death”. Hmmm. I was concerned about the operation for this, but over the next few days gradually forgot all about it.
My short term memory around this time was really bad; sometimes I would get a phone call from a friend and be asked what I had for lunch that day; no chance! It just wasn’t there, like the data had been erased. Apparently short-term memory is the first thing to go when you have heart attacks and the blood supply to the brain is restricted.
The staff at Harefield were wonderful; I am still under the care of Dr. Emma Birks and from day one I started my slow but sure recovery. I had lots of visitors, and Sue and my sister and brother in-law came to see me every weekend without fail. That was a round trip of 400 miles.
After a few days the central line was taken out; then another line and the catheter (thank God!) and I could actually lay on my side to sleep, a real luxury. I asked Kathleen on one of her visits if I was out of the woods yet, and she assured me that I was, which was a tremendous relief. I don’t know to this day if she believed it but it helped anyway.
Moved to another room, slightly less dependant and things began to improve rapidly. I was given telemetry so I could move around without the wires trailing back to the monitors, and eventually could walk to the loo and shower room by myself, fantastic! Some days were good, others great, some bad and a set-back usually took a few days to recover from, but all in all the progress was good. The pacemaker was fitted in late October; by now I was strong enough to appear ‘normal’ most of the time. I should mention that I woke up half way through the operation and asked the surgeon “Do you need a hand down there!”
I was released from hospital late October, and started my shaky recovery at home. An over-night stay at the Brompton, prompted by some strange blood results was needed early December 2006, but since then progress has been very rapid, and after Christmas I went back to work full-time. This was a bad mistake as I became very fatigued and now only work 15 hours a week, at least until my strength returns. I believe there are problems with fatigue for anyone with vasculitis.
So, there we have it, and to say that I am grateful and appreciative
of all the help and support I’ve been given is a massive understatement,
and I am only too willing to extend any help I can give to other
sufferers of this rare, serious but ultimately treatable disease.
Thirty-Five Years with Churg-Strauss (and counting.....)
By David Moreland, USA
May 2007
I recently found this site and was impressed by CSSA’s mission.
I was diagnosed with Churg-Strauss Syndrome in 1979, and hope that
my story will in some way help others to confront and manage their
condition. I’m counting my 35 years from when I first started
to show some of the many symptoms of CSS, and I’ve described
the various sequelae that have expanded its impact.
The usual childhood illnesses hit me very hard, and as a child
I had many colds and ear infections. By high school in the late
1960’s, however, I had put in many, many miles of vigorous
hiking and bicycling in the hills of Connecticut, and during my
last three years I was a halfback (midfielder) on the new soccer
team. As a junior in high school I was in extremely good health
with excellent stamina, with my only physical problems being nearsightedness
and a partial hearing loss in one ear from a childhood cholesteatoma
(a benign growth in the middle ear due to inflammation) and subsequent
mastoidectomy.
BEGININGS?
In retrospect, the likely first signs of CSS came early in my high
school senior year, 1970, when it became very difficult for me to
get into and stay in shape for the fall soccer season. I had developed
a persistent postnasal drip, which turned out to be the first appearance
of what became chronic rhinitis and sinusitis. I spent the season
enduring the taunts of my teammates and the wrath of my coach for
not staying in condition. In early 1971 I was diagnosed with fall
allergies and started treatment with allergy shots, which were ineffective,
and OTC medications, which offered some short-term relief. I have
had severe chronic sinusitis ever since.
ASTHMA
On to college, where I was still in very good shape in spite of
the annoying sinus problem, and where I stayed physically active
with backpacking, snowshoeing, and whitewater canoeing and kayaking
in the White Mountains of New Hampshire. The allergy shots were
continued, to no apparent benefit, and I used OTC nose sprays and
decongestants to try to manage the sinus symptoms.
I gradually developed a tightening in the back of my throat that
would appear episodically and would go away if I used an OTC epinephrine
inhaler. In the fall of 1972 I had my first actual asthma attack,
which was triggered by goose down while I was assembling a sleeping
bag kit. The attack was severe but was treated successfully with
corticosteroids. During the next year I developed steadily worsening
chronic asthma. Initially the symptoms were worse from mid-autumn
through mid-spring, but gradually became year-round.
The asthma symptoms were controlled poorly. From late September
through April or early May I would be awakened by significant –
often severe – asthma episodes every morning between 3:00
and 4:00 AM. In the days before inhaled steroids, the only effective
treatment for me was systemic corticosteroids, and the conservative
medical establishment at the time was extremely reluctant to prescribe
long-term steroid treatments. Instead I was given various preparations
of theophyllin and alternately encouraged to use or not use an OTC
epinephrine inhaler. I received corticosteroids only through emergency
room visits and hospitalizations. Initially I was given Tedral or
Elixophyllin, which is a rancid solution of theophyllin in ethanol
that burned as it was swallowed. It had an imitation tutti-frutti
flavor that utterly failed to cover theophyllin’s bitterness.
I would sit in near-strangulation with the dose measured out in
front of me for long periods before finally forcing myself to swallow
it. After a year or so I was switched to two grams per day of Slo-phyllin,
a time-release theophyllin preparation. It took quite some for me
to become acclimated to theophyllin and stop experiencing its side
effects of nausea and vomiting. The nightly asthma episodes continued,
however, and since theophyllin, ephedrine, and epinephrine are all
stimulants I rarely got a good night’s sleep. Sometimes the
episodes could be controlled within a few minutes, but usually it
took at least an hour and more often two hours to get back to sleep.
I would use an IPPB (Intermittent Positive Pressure Breathing) machine
to help force medication and saline into my lungs and to help clear
out some of the thick, sticky sputum. It was not unusual for me
to not get back to sleep at all, and I went through my last two
years in college in a fog of exhaustion. My friends made jokes about
heavy breathing, asserted that it was all psychosomatic, and gave
advice that it could all be cured with herbs or vitamins. A group
of them would break out into loud coughing whenever I entered the
room. I would not know from one day to the next what sort of condition
I would be in – would I be an athlete or an invalid? I became
cautious about making social commitments because I couldn’t
know more than a day in advance whether I would be physically able
to keep them.
I finished undergraduate studies and went to graduate school in
California, and hoped that the change in climate would improve my
health. It didn’t, and I had a couple of visits to the emergency
room, but I eventually did find an allergist who was comfortable
prescribing prednisone. He had me take 10-day tapering courses as
needed in addition to continuing the Slo-phyllin. I would run about
a five-week cycle: after a course of steroids my symptoms would
be very good for a couple of weeks, then gradually worsen and become
pretty severe before I’d start the next course. I struggled
through the first couple of years of graduate school in this mode.
In 1977 or so Vanceril, the first inhaled corticosteroid, became
available. I was started on it immediately and it stabilized my
symptoms greatly. I still needed the periodic systemic steroid courses,
but my symptoms were slower to deteriorate and did not fluctuate
as quickly or as widely. Improved beta-blockers also started appearing
around that time, and provided better short-term symptom relief
than the OTC epinephrine inhalers. Symptoms were still worse during
the winter months, but overall my asthma was much more stable and
I started to be able to sleep through the night again. The sinusitis
persisted, and I had nasal polyps removed in 1978.
Life seemed to be getting better, and during the summers I started
playing in pick-up games of soccer and riding my bicycle up into
the Berkeley and Oakland hills. My goal was to complete my thesis
work, start the job search, and get on with life.
VASCULITIS
With the 1979 fall job recruiting season approaching, I was putting
in a lot of hours to produce results on my thesis project. In mid-June
I was still keeping up my regular soccer matches, and during one
game suffered a split lip in a freak collision. A trip to the infirmary
and a tetanus shot patched me up, but that night I developed severe
abdominal cramping. The cramping had improved greatly by morning,
so I didn’t act on it. During the next few days I had some
diarrhea and nausea, and the cramping didn’t go away completely.
I passed it off as a summer flu, since there was a little bit of
something going around. Besides, I had to get those thesis results…
The cramping never really went away, and during July it began to
worsen – in retrospect, probably in sync with my steroid courses.
By the time I saw a doctor in mid-August, the pain was very significant
and I had lost my appetite completely. Blood work showed elevated
eosinophils, and the initial, tentative diagnosis was parasites.
The internist I was visiting said the tests were very ambiguous
and said they really weren’t sure what was going on. After
a couple more office visits and more blood tests, I was admitted
to the hospital on Sept 9 – nine days after my 26th birthday
– for observation and more tests.
In the hospital my asthma flared up because it was near the end
of my usual steroid-course cycle, and it took a few days to control
it. When the asthma stabilized I was given a battery of additional
tests, including barium scans which indicated intestinal blockage.
I was unable to eat, and hyperalimentation (intravenous feeding)
was begun to treat what had become malabsorption and malnourishment.
In the meantime there were more tests, while the abdominal pain
remained very significant and was poorly controlled with Vicodin.
The pain was generalized throughout the central and upper abdomen
and, somewhat, in the lower left quadrant. When I finally reported
that some pain had developed in my lower right quadrant, I was taken
immediately into surgery for an appendectomy – a relatively
minor procedure that, hopefully, would provide some information
about what was going on. The following day the pathology results
were available, showing vasculitis, and a rheumatologist was added
to the medical team. From him I first heard about systemic necrotizing
vasculitis and Churg-Strauss Syndrome. He diagnosed polyarteritis
nodosa or, because of my history of asthma, Churg-Strauss. He described
a paper by Anthony Fauci and co-workers that had just appeared in
the Aug. 2 issue of the New England Journal of Medicine reporting
the use of cyclophosphamide and high-dose prednisone for the treatment
of systemic necrotizing vasculitis. Treatment was begun that day,
Sept 18. I could not have been more fortunate that the paper was
published when it was, and even more so that the rheumatologist
was up on his reading.
At about this time my hands and feet first became numb, then painful
over the course of a day or two, with my left hand and arm eventually
becoming very painful while the other hand and my feet improved.
The thumb, index finger, and middle finger – serviced by the
median nerve – of my left hand became paralyzed and I lost
all feeling in them except for neuropathic pain. I developed significant
neuropathic pain in my left forearm and shooting pain in the whole
arm.
My condition was stable for eight days following the appendectomy,
but there was no improvement. On the ninth day the daily blood tests
showed that my liver function had become severely impaired and late
in the afternoon I was taken into surgery for a general abdominal
exploration and an attempt to determine what the problem was with
the liver.
When I awoke in the recovery room late that night the surgeon described
their findings in great detail. They had removed 18 inches of small
intestine and my gall bladder, both of which were necrosed and gangrenous.
My hepatic artery was thrombosed and my liver appeared to be completely
necrosed. They were unable to restore circulation to the liver,
and the damage to it was so severe that, after consulting with all
the available surgeons in the operating complex who were scrubbed
up, they terminated the surgery without attempting to address either
the liver damage or the remaining necrosed portions of my intestines.
In the recovery room the surgeon told me that my injuries were fatal
– and should have been fatal long before they ever had a chance
to see them – that they hadn’t expected me to regain
consciousness, and that they were not ordering my evening medications
because “it would be pointless”. I was moved to Intensive
Care, where I was isolated from the rest of the ward and the nursing
staff checked in every 15 minutes or so to record my blood pressure
and pulse, both of which were extremely high.
I spent the night performing relaxation exercises and building
a rapport with the nursing staff. In the dark morning hours I first
became chilled, then went hypothermic, and the nurses took the initiative
to contact the key medical team members at home to get permission
to treat it the hypothermia. My medications were resumed in the
morning, and from that point on I received very attentive care.
During 72 days in the hospital I weathered a partial failure of
the surgery, a systemic Candida infection that was treated with
amphotericin B, a gastrojejunostomy to bypass a section of intestine
that apparently had been too severely damaged to recover, and several
other complications before I was discharged. My liver function returned
spontaneously. I had reached a low of 129 lbs, down from my normal
175, which I carried on a 6’ frame. I went home from the hospital
on the day before Thanksgiving in 1979.
After being discharged I continued oral cyclophosphamide for four
months to complete the six-month course, then was put on chlorambucil
(Leukeran) for another six months. The fingernails on the paralyzed
fingers of my left hand fell out over the course of a month or so
after my discharge, with new nails growing in beneath them.
I continued Vanceril and Slo-phyllin for asthma, but after the
high-dose corticosteroid was tapered down, started alternate-day
corticosteroids instead of the periodic courses I had used previously.
Initially my asthma symptoms were improved enormously compared to
before the chemotherapy and I was able to taper off the systemic
steroids completely. After two years, however, the asthma symptoms
worsened and I had to resume alternate-day steroids, arriving at
the basic regimen of medications that I used for the next 17 years.
I returned to graduate school determined to complete my degree
after a three-month hiatus. The three fingers in my left hand remained
paralyzed and it was very painful for me to touch anything with
them, so I changed the focus of my studies from laboratory work,
which required careful manual manipulations, to computational work,
for which I could at least hunt and peck with seven fingers on the
keyboard. As it became less painful to touch things I sewed together
a parka, which I got as a kit from Frostline to help exercise my
hand. I used a sewing machine in their local store and for the duration
of the project had to grasp and pull the fabric, and guide it carefully
through the machine. It turned out to be a good level of exercise.
Feeling and function in the left hand improved gradually over several
years, and after many years became near-normal, even though there
had been no detectable transmission in the left median nerve at
the time of my discharge. My left hand remained weak, however, and
cramped up badly with prolonged use.
After completing my degree in 1984 I entered the corporate world
in Ann Arbor, Michigan. I was referred to a top-notch pulmonary
specialist at a local private hospital, and he recommended a rheumatologist
to keep an eye on the vasculitis. I remained active with volleyball,
bicycling, and cross-country skiing.
In the late 80s I suffered two seizures and was started on anticonvulsants,
which I tolerated poorly, experiencing numerous side effects and
depression from several successive therapies. Eventually the seizures
were linked to functional reactive hypoglycemia, caused by gastric
dumping as a result of the gastrojejunostomy. Ultimately, all I
need to do to avoid seizures is to not have sweets on an empty stomach
shortly before getting vigorous exercise under stressful conditions.
By managing my diet and exercise I no longer need to be on anticonvulsants.
RELAPSE
In the early- to mid 1990’s I began to have some vague urinary
tract symptoms – soreness in my kidney area, periods of difficulty
urinating, painful urinations and ejaculations, and occasional blood
in my urine and semen. My blood tests were unremarkable, and the
internist, rheumatologist, and urologists were not terribly concerned
about the symptoms. In the mid-90s I started treatment for mild
depression brought on by building frustrations with my work situation
and with my health.
During these years I periodically developed itchy, leathery nodules
on my elbows. These would come and go with no apparent pattern and
were unrecognized as potentially related to vasculitis.
When Accolate became available for asthma in 1996 it was added
to my therapy and I was able to stop the alternate-day systemic
steroids, but had to retain the inhaled steroid (by then it was
Flovent 220). Upon stopping the systemic steroids I had a brief
episode of soreness in my kidney area, which resolved itself over
a day or two. I began going to a nephrologist, who determined that
my kidney function was mildly impaired, but also that I had hepatitis
C – probably contracted from blood transfusions during the
1979 surgeries. He suggested that my urological symptoms might be
due to the virus.
In 1997 I developed inflammation in both of my shoulders, for no
obvious reason. Any attempt to move my arms at the shoulders became
very painful and my shoulders quickly froze up so I couldn’t
raise my arms from my sides. It took over six months of painful
physical therapy and exercise to return them to full function. This
doesn’t seem to be something that typically happens with CSS,
but I note it because it is another manifestation of inflammation.
I have experienced ear infections fairly frequently in my adult
life, and during the mid-90s they would occur several times a year,
especially in my left ear. They resulted in ruptured eardrums many
times, so were treated aggressively with various antibiotics. In
May 1998 I was put on a two-week course of ciprofloxacin, a fluoroquinolone
antibiotic, to treat an ear infection. I had been on Cipro once
before and had experienced abdominal discomfort that resolved quickly
once the course was over. As this second course progressed I began
to feel fatigue and discomfort, muscle pain, then joint pain, and
I stopped the course on my own a few days early. Shortly thereafter
I noticed a “floppy foot” condition in my left foot,
which also started to go numb. I saw the rheumatologist within a
day of this, but he was not concerned by the symptoms. Two days
later both hands and both feet started going numb much as they had
in 1979. I drove myself to the emergency room, where they diagnosed
polyneuritis multiplex and sent me home, in spite of my protestations
about having a history of peripheral neuropathy from systemic necrotizing
vasculitis. The next day my hands and feet became very painful and
I returned to the ER, where, after triage and a brief exam, I was
left unattended overnight. I was admitted to the hospital in the
morning and after two or three days of the rheumatologist, nephrologists,
and the neurologists debating what to do, a kidney biopsy was performed
and they decided that it was consistent with the presence of vasculitis.
During this time the symptoms worsened, affecting mainly the ulnar
nerve in my left arm and the sciatic nerves in both legs, the left
being worse. The outside of my left foot became paralyzed.
A six-month course of cyclophosphamide was initiated, this time
given intravenously once a month, and a high-dose course of prednisone
was begun. The combination of Vicodin, oxycontin, amitryptillin,
and Neurontin did not fully alleviate the nerve pain, and I developed
significant edema in my lower legs. I was sent home after a week,
initially with home nursing to administer IV medications. I was
unable to walk without crutches because of the pain in my left foot,
and needed a home care worker to shop and to do some light food
preparation. I could put in only short days at work before the pain
and swelling in my foot would become too severe for me to concentrate.
Fortunately I was able to do quite a bit of work at home.
By the end of the six-month cyclophosphamide course I was able
to walk with considerable discomfort without crutches but was able
to function more or less normally. I had stopped all of the painkillers
except Neurontin, which I continued for a year or so. I had also
gained 20 lbs from inactivity and during the next couple of years
worked back down to my original weight of 170. The pain in my foot
and thighs gradually diminished over several years to near-normal,
but the paralysis in my left foot remained. The ulnar side of my
left hand improved to the level of the median side, with nearly
full function and sensation.
TODAY
My condition is now being managed by a rheumatologist at the University
of Michigan Hospital who specializes in autoimmune conditions and
vasculitis. Following the 1998 cyclophosphamide course he initiated
methotrexate at 20 mg/wk with alternate-day prednisone. I have been
unable to taper the prednisone dose below 10 mg, with pulmonary
function being significantly better at 12.5 mg alternate-day. My
asthma deteriorates with any reduction in either the inhaled or
the systemic steroid. I continue with Flovent and albuterol for
asthma and with Nasonex, ipatropium bromide, fexofenidine, gentomycin
flushes, and warm saline flushes with a Hydropulse Irrigation System
for the sinusitis.
Sinusitis is still a significant problem for me. I’ve had
tinnitus as long as I can remember, especially in my left ear, and
it fluctuates in severity. I have had two surgeries to remove sinus
tissue and polyps, and for the past several years have had a tube
in my left eardrum. Ear infections are now rare, though I have constant
sinus drainage.
My pulmonary function is quite good. In spite of the asthma my
lung volume is large for my age and size. The efficiency of gas
exchange in my lungs, however, is only 60% of what it should be.
CT scans show some irregularities that appear to be transient and
that biopsies show are not infectious, vasculitic, or cancerous.
I have residual effects from the vasculitic damage. I have some
difficulty fastening and unfastening shirt buttons and tend to use
my middle and ring fingers of my left hand with my thumb rather
than my index finger. My left hand still cramps up occasionally,
making it difficult to perform tasks around the house that require
fine manipulations for extended periods. The thumb gets stuck against
the palm and I have to massage it and pull it open again. The edema
in my lower legs comes and goes, and the left side of my left foot
is still paralyzed.
My liver and kidney functions are stable and biopsies have shown
the liver to be in very good shape, in spite of what it went through
in 1979 and in spite of the hepatitis C and the methotrexate. We
have not attempted to treat the HVC for fear of exacerbating the
vasculitis.
The condition of my stomach is now being monitored carefully. The
1979 gastrojejunostomy altered its functioning enough that the stomach
lining becomes irritated by bile and endoscopies have shown dysplasia
– abnormal cells that can lead to cancer – and polyps.
While the dysplasia was resolved with a more careful diet, the stomach
lining remains irritated and polyps appear occasionally and must
be removed.
I have long had stretch marks on my torso from the two times I
was bedridden and taking high-dose corticosteroids. Since the mid-90s
other effects of taking corticosteroids for 30 or so years have
become more apparent. My blood pressure has become slightly elevated
but is treated very effectively with lisinopril. My skin –
especially on my shins – is fragile, breaks easily, and heals
slowly, so I wear my old soccer shin guards for activities, like
clearing brush, that present a risk. I always have a collection
of small bruises from bumping into things or from carrying something
heavy against my legs. Several years ago my bone density was found
to have decreased significantly, but it largely has been restored
by taking calcium and Fosamax. My face is perhaps slightly more
rounded than it might be otherwise, but that is one place where
the steroids have had little effect.
In all, I am still able to be very active. I participate in a volunteer
group in town and serve as a guide for blind cross-country skiers
and kayakers at events in the state. During the summer I in-line
skate two or three times a week at an aerobic level, kayak once
a week or so – also at an aerobic level, and play volleyball
occasionally. I work at strengthening the calf muscle in my left
leg, but still cannot lift my own weight with it. During the winter
I work out at a gym and cross-country ski when I can. I seem to
be limited in the level of conditioning and muscle strength I can
attain, and it’s not clear whether this is related to the
autoimmune condition, the medications, the virus, or just age. My
lung’s inability to absorb oxygen normally limits the intensity
of my activity. My digestive system does not handle fat very well
and I need to be careful of my sugar intake, so I maintain a high-fiber,
low-sugar, low-fat diet, which is a good thing. The problem is that
I have difficulty maintaining my weight above 160 lbs during the
summer, since I seem unable to digest enough food fast enough to
keep up with the exercise.
I have found that I am generally the most unusual case that’s
walked into a given physician’s office in a long time, and
I have encountered physicians who want to take on my case whether
they are qualified to or not. It has at times been difficult finding
appropriate care. I am fortunate now to be near a large teaching
hospital. The primary physician, the rheumatologist, the pulmonary
specialist, the nephrologist, and the gastroenterologists with whom
I work now have varying levels of experience with autoimmune conditions,
but they are all very good in their fields, communicate with each
other well, and are frank about what they do and don’t know.
Their focus is to prevent more relapses rather than just to react
to them.
I often think back to a guy, Don, who was in my junior- and senior
high schools. He was an Eagle Scout in the same troop I was in,
and was driven to complete all of the challenges he undertook. Don
had cerebral palsy. He had to struggle fiercely to get his muscles
to do his will, even for the simplest motion. Every movement he
made was an excruciating effort, his own body fighting every step
and every attempt to communicate. Yet walk and communicate he did.
He could carry a backpack just as heavy as mine, and would force
himself along the same paths and up the same hills. If he fell he
wouldn’t accept help getting up, but would do it on his own.
In school he pecked away on the electric typewriter the school provided,
holding himself to the same standards as everyone else. The memory
of his determined efforts puts things in perspective when I find
myself drifting towards self-pity.
During college I spent a summer as a counselor at a camp for diabetic
boys. The camp’s theme was that as long as the boys managed
their diabetes there was no activity they couldn’t do, and
the program centered on their being very active and athletic. Each
of the campers had their ups and downs and there were always challenges
balancing diet, insulin, and exercise, but they kept at it and found
they were no more limited than anyone else, and could enjoy very
rich and varied lives. I had just developed asthma, and the camp’s
message resonated with my new situation.
I am thankful to have these role models from my past. There’s
no question that a major chronic illness is a burden, and takes
time and energy – physical, mental, and emotional energy –
that would otherwise be applied to other aspects of life. My energy
level and mood fluctuate widely, but slowly. I wrestle with the
question, to what degree do I use my health as an excuse, and when
is it an actual reason for doing or not doing something? Some of
the effects are obvious – I can’t do significant exercise
on the days I take methotrexate, for instance, because it makes
my strength and stamina very low. Other effects are more subtle
and I’m sure that I’m only aware of some of them. If
I’m feeling listless or fatigued, is it because of medications,
because of having been too active over the past week, because I
need to rest up from using mental and emotional energy for coping,
or is it the mild depression back again? In any case, is it a reason
not to go out dancing tonight? It often is. The burden clearly has
an influence on my level of motivation, my concentration, and my
confidence. It’s not easy to follow the example of Don the
Eagle Scout and find a way to forge ahead in spite of one’s
burdens. But, it’s either do that or miss out on many of life’s
offerings.
It is clear that Churg-Strauss Syndrome, vasculitis, and autoimmunity
are below the radar for much of the medical community At the time
of my original diagnosis my brother-in-law physician observed that
medical professionals are trained to look for a horse when they
hear the sound of hoof beats, but it my case it turned out to be
a zebra. I hope that the CSSA and this web site will help raise
awareness to a level where future patients can obtain prompt and
accurate diagnoses and receive the most effective treatment possible
in a timely manner.
|
