December 12,
2008
I’m at the
hospital today, but I’m only here for the day.
After improving significantly, I began to get sicker again. My liver functions skyrocketed, the pain got
worse and the itching became unbearable.
What we’re
looking at here girls is primary sclerosing cholangitis, a disease that normally runs its course
(that course being diagnosis to eventual total liver failure and transplant) in
about a decade. It’s an add-on to
Crohns disease (though more common with ulcerative colitis), and thought to be
the result of the same autoimmunity that triggers Crohns. Think of it as an
optional extra, like leather seats in a new car.
All things
considered, I’ve had a fairly easy go of it until now. I was diagnosed as a child and it was the
last thing they expected, given that the typical patient is male and 50 so
finding it in a 13-year-old girl surprised everyone. By the time the diagnosis had been made, considerable damage had
already been done.
In a nutshell,
it’s a degenerative liver disease. The
bile ducts (you’re born with 4) begin to scar and narrow. They become blocked
and then atrophy, obviously impeding and eventually preventing normal bile
flow. The only treatment is
transplant. The disease progresses
slowly – in my case, the initial diagnosis revealed that at that point, I had
only 3 functioning bile ducts. Off and
on during my teens I was pretty ill with the Crohns and the liver disease didn’t
really trouble me all that much. In fact, it just seemed to abate and I thought
no more about it.
Fast forward a
few years. I began to have upper right
quadrant pain and became so itchy, I felt like I was ready to claw my own skin
off. My bilirubin and other liver enzymes and functions were grossly
elevated. And although at that point I
was referred to a transplant surgeon, I responded well to the medication and it
never came down to that. When the
entire episode was behind me though (and it lasted well over a year), I was
down to 2 functioning bile ducts.
As I’ve said,
there’s no cure for this. I knew
eventually I’d need a transplant but I wanted to put that day off as long as I
could. I heal like an Immortal and
since I didn’t drink much or do street drugs, I figured I could buy myself a
bit more time.
But during that
long hospitalization, I had some surgery to remove neoplasms from my liver (to
simplify: nasty little cells that
shouldn’t be there and often become malignant) and an attempt was made to
unblock one of the two remaining bile ducts.
The surgery –
called a hepatectomy – was no big deal.
They go in through your belly button and as far as surgeries I’ve had
go, this was nothing. It’s a bit odd to
wake up and find your belly button sewn shut, but that was the most distressing
thing about it.
The ERCP was
another matter. Prior to the
development of a type of nuclear medicine test (a fancy xray for you
non-medical geeks out there) called an MRCP, diagnosis of primary sclerosing
cholangitis was made via an ERCP. Now it’s only used for therapeutic purposes,
as it’s dangerous, excruciating and invasive.
This is the
worst pain any human can endure and not die.
Hands down. I’ve had a lot of
physical pain in my life and as a result, I’ve got a ridiculously high pain
threshold – I’ve had stitches and staples put in my head with no anaesthetic or
painkiller (they lower my seizure threshold and seizures, given the violence of
mine, scare me more than anything).
An ERCP is
torture. That’s not hyperbole ladies –
it’s actual torture. You can’t
be out for it because you have to cooperate while it’s being done and the risks
of fatal internal injury during it are significant. Too much sedation will lower your blood pressure dangerously and
increase the risk of vomiting and this is the last place you want to start
throwing up. So the protocol is to give
only as much sedation as is required to “keep you comfortable” – which is a
fucking laugh.
Here’s how it
works: an instrument is placed in your
mouth to keep you from closing your mouth.
It’s made of hard plastic so even though you bite down as hard as you
can, your mouth stays open. A tube with
a camera is fed down your throat and pushed into the bile ducts of the liver
(and I’m really simplifying here). This
is the torture part. It is
excruciating. I always try to scream
and get away but I never quite manage it.
An instrument is then corkscrewed through the blocked bile ducts in an
attempt to open them up to permit bile flow.
I can’t even describe what this feels like: it’s something more primal than pain. If you have any conscious thought left at this point, you’re
praying to die.
It takes
forever.
After the ERCP I
had all those years ago, I swore I’d never have another. I would rather die.
I wasn’t given
that choice. During this most recent
hospitalization, I had to have another.
I remember the pain, I remember screaming and trying to crawl off the
table. I remember being held down.
I was on that
damn table for 45 minutes.
After it, I
started to bleed internally. I bled so
much, in fact, that I went into shock.
I developed pancreatitis as a result of the ERCP (the procedure causes
it) and for a day or so, I was very close to death.
I don’t remember
much, mercifully. I was being given
time released hydromorphone around the clock and tons of dilaudid every 2
hours. I was in and out of
consciousness – another mercy. The
trauma to my body was extensive.
When I came out
of that, it was to discover that I have only one bile duct left and it’s not in
good shape.
But between the
procedure and the drugs, I began to get better. My liver functions dropped until they were not as frightening as
they had been. They sent me home on
massive amounts of narcotic and I began to improve.
This lasted two
weeks.
Then the pain
and the itching began again and the bloodwork showed that my liver functions
were again deteriorating. Because I was
being so closely monitored, this was caught immediately. I stopped responding to the meds and within
a matter of days, was right back to where I’d started.
So today I’m
back in the hospital again, this time only to get baseline tests.
The consensus is
that the last bile duct I have is dangerously dilated and may well be on its
way to becoming obstructed. I’ve got
more neoplasms on my liver too.
I’ll need
another ERCP and I have to tell you, I don’t know if I have the courage to go
through that again. I know I have to –
I’m not healthy or stable enough for a transplant at the moment. If they don’t clear this duct, I could die.
To say this has
been a shitty year is putting it mildly.
As a result of my body’s treachery, first with the seizures, now with
this, I’ve been off work for a year. As
a result of that, I’ve lost everything I’ve had: my career, my independence, all my savings, my income. I never used to resent these unscheduled
time outs when I was a kid – they make me angry and hopeless now. I get very depressed and begin to believe
that perhaps I’d be better off dead.
I’m still not convinced that this isn’t true.
I know damn well
that if I lose the will to fight, it’s game over. But entre nous ladies, I’m getting pretty tired of this. I find it hard to imagine a light at the end
of the tunnel and the thought of starting over AGAIN just makes me weary. I don’t know if I have the heart for it and
I’m pretty sure I don’t have the energy.
It’s hard to
hurt this much for this long: at some
point, you just want it to stop.
Because PSC is so intertwined with Crohns (same autoimmunity,
remember?), that’s active again and I’ve got a gut bleed going on as well. My whole world has shrunk to one room in a
house and a town I don’t want to be in.
I’m isolating myself again – and that’s a bad sign. For me to cut myself off so completely from
other people is akin to crawling away to die.
I’m in a town I
loathe and I’m here on sufferance, at the mercy of things that are beyond my
control. I’m not eating. I’m not sleeping. I’m being given so much narcotic but it just amounts to bad drugs
that don’t work enough.
I’ve been in
this place before, but never for such a long time. I know this frame of mind too well to allow myself to dwell on my
current circumstances. For the time
being, I have to surrender and just let things be. Giving up control has always been difficult for me, so this is a
struggle.
And in the
meantime I’ll write. It’ll be drug
addled and likely incoherent but the feeling of fingers on the keyboard is so
soothing and these days, I’ll take my comfort where I can find it.
Till next time,
M.
PS - I want you to know that as hard as it is for me to accept help, I greatly appreciate all the donations, support, and well-wishes that have come in during this difficult time.
