We Can Adjust The Sails

When I was first diagnosed with ‘Pulmonary Venous Occlusive Disease’, I asked my consultant how much time I had left.  He screwed his mouth up, scrunched his forehead, and pulled a ‘bad news’ face.  In that one reaction, I knew it would be distressing.  And I also knew I wasn’t ready to hear that.  So when he slowly opened his mouth to tell me… I jumped in quickly and stopped him.  Instead we talked about how the deterioration would continue, how there was nothing further to help me, how a rapid decline was a feature of the disease… but we didn’t talk figures or timelines.  And we didn’t put a date of expiry on my head.

A few weeks later, in the midst of transplant assessment, it was repeatedly confirmed that my prognosis was bad.  The look of sympathy and pity on the transplant consultants faces when they heard my diagnosis.  The widened eyes of the ICU doctor, when I told him the name of my disease.  The urgency in which I was rushed through the assessment process.  Instead of the normal 6 months to a year, from referral to getting on the list- I was all done and dusted, with my signature signed, by two months.  They all knew more about the disease than me, and were worried.  So I needed to know too.

Therefore a few weeks later, when I’d processed and thought and come to terms with the latest diagnosis, when guessing was worse than not knowing, when I’d psyched myself to be ready for the truth… I decided to research my fate.  How much time did I have left?  What was life expectancy with my disease?  What was my prognosis?  I turned to google.  There was practically nothing- it’s too rare- but I did find one relatively recent research study.  It followed a group of patients newly diagnosed with PVOD.  At the one year anniversary of their diagnosis, 90% were dead.  Yep dead.  90% were finished, ended, gone.  90% were passed away, residents of heaven, living with the angels.  Only 10% were alive to celebrate their joyous anniversary of being diagnosed with PVOD.  And as there was no follow up study at the two year date- it is a safe bet that none made that milestone.  Ta Da!  There was my prognosis.  The odds were in favour of me not surviving the year.

So I cried, and I cried, and I cried.  I told Phil, but his optimism, and scepticism, rejected the study.  “It was a small sample size”… “they weren’t on steroids”… “PVOD is so little understood”.  So I cried some more that he didn’t understand my fate, that he was being too positive and brave.  And I didn’t tell anyone else really.  I didn’t want others to be freaking out, or worrying.  And I didn’t want others to be believing it.  Expecting me to go… ’cause I wasn’t ready to go.I have now been diagnosed with PVOD for sixteen months, or one year and four months, or 488 days.  I have already beaten the odds of that terrifying study.  I am a lucky, lucky, lucky member of the 10% that survived the year.

I have a strange relationship with that PVOD research project.  On one hand I accept it’s findings… and on one hand I don’t.  On one hand I believe it’s my prognosis… and on one hand I don’t.  Classic double-think.  I urgently cram my weekends with living now, yet still make mental plans for 2018.  I recognise the strengths of the research project, yet remind myself of the flaws.  I occasionally sob that “I’m supposed to die this year”, yet regularly joke about my short expiry date.  I accept a doctor’s prognosis would be based upon the same study, yet belittle it as it’s google sourced.  I periodically worry I’m in bonus time, yet more often use beating the odds as strength that I’m surviving.  Double-thinking allows me to cope.

For the past year, there has been many occasions where I could have asked my consultants outright for an accurate prognosis.  Finally had that dreaded conversation that was halted on the day of diagnosis.  But I’m still not brave enough.  Still not ready to know the truth.  Because the whole time, my prognosis is based upon a google search, I can still continue to double-think.  When it comes from a doctor’s mouth, it suddenly bears more weight.  It seems more credible, plausible, accurate: a truer prediction.  The same research study when self-sourced can be mentally discredited, but when doctor quoted has to be 100% accepted.  No more double-thinking.  And if you take away my double-thinking, you take away my ability to carry on living normally.  You take away my hope.  You take away my fight.

Last week at my routine ‘Pre-Transplant’ appointment at Harefield hospital, my poor prognosis was once again discussed.  The same consultant -with the sympathetic and pitying eyes a year ago- now looked at me with bewilderment.  He wasn’t expecting me to still be here and well, one year on.  He knew my disease, he knew my prognosis, he’d rushed me through assessment to give me the best chance.  Yet I was sitting across from him, looking relatively well for a dying person.  In fact, instead of being in a worse physical condition, my clinical scores had actually improved over the year.  My heart is now coping and not expected to imminently fail.  And although my lungs are only functioning at 30%, they are stable and no longer free-falling.  With these clinical results, other patients might be discussing being suspended from the list temporarily.  If there is a chance they can survive another year with their own lungs, then that is substantially better odds than facing a risky transplant.  Other patients… but not me.  I have PVOD.  Although I am well and stable today- I may not be next month.. or next week.. or tomorrow even.  He reminded me that my condition can plummet dramatically; losing vast lung function overnight.  He reminded me that although it’s amazing that I’m stable now, it is unexpected.  He reminded me that my disease is bad; my prognosis is poor.  Therefore my best chance of being alive next year is to still have the risky operation before the decline comes.  So I’m being kept on the list.  Still waiting.

I left the appointment beaming pride- at his bewilderment, my stability, beating the odds, my coping body.  Only later did I process his words and realise that he still thinks I’m going to die soon.  I’m not invincible.  Even though I’ve been very very lucky- the end is coming anyway.  It shook me up.  Really shook me up.  He was still supporting the scary prognosis- the ‘you’re supposed to die in the first year’… the ‘no-one makes two years’ research project.  So I cried and sulked and moped around in PJ’s for three days.

It’s horrid to have a disease where everyone thinks your time should have been up long ago.  Awful to have an illness where everyone is amazed to see you alive and well from one appointment to the next.  Terrifying to think where I would be now, if I hadn’t randomly been given steroids.  Sickening to think about what is coming- what everyone tells me is coming.

And then on the forth day… I got on with it again.  I can’t change my disease, I can’t change my prognosis… but I can change my reaction.  So I plastered a smile on my face, I changed out of my pyjamas, I moved off the sofa.  Yes I know my future is dicey, my prognosis is poor, my expiration date is nearing.  I accept that.  I truly accept that.  But I’m once again allowing myself to laugh at the absurdity of the situation.  I’m once again allowing myself to gain strength and hope from beating-the-odds so far.  I’m once again allowing myself to think that the outcome for the study patients, might not be my prognosis.  I’m putting my faith in those miraculous drugs that are keeping me stable and planning for the future.  Classic double-think.  Live, hope, fight.

And, as no one in the scary study made the two year anniversary- that’s now my new target!  🙂