Coping With Surprising Results

It was supposed to be an uneventful stay at the Brompton.  A routine biannual checkup.  I was expecting four days of tests and procedures and assessments.  Four days of poking and puffing and prodding.  Four days of Netflix films and tea machines and yummy three course meals.  I wasn’t expecting a ride on an emotional roller-coaster. 🙂

There was a full timetable of planned tests.  Most of them were regular and routine; repeated every few months at my different hospitals.  CT Scans, Lung Function Tests, ECGS, Echos and Bloods.  Tiring but very familiar.  However I hadn’t had one procedure -the biggie- for two years… and was nervous.  Although it would be my fourth ‘Right Heart Catheter’, the others had all been completed by my old consultant at Oxford.  I was anxious about having such an invasive procedure performed by an unknown surgeon in an unfamiliar theatre.  But as the only accurate way to assess PH, it was essential I completed it.

A Brompton RHC is like a mini operation.  My warfarin was stopped in the days proceeding, and replaced with a horrid daily stomach injection!  The doctor visited the night before, warned me of my chances of dying (less than 1 in a 1000 😉), then asked me to sign all the consent and disclaimer forms.  At ridiculous o-clock on the morning of, I showered using pink operation antibacterial wash, got dressed in a gown, took some calming diazepam, and had a cannula placed in the awkward crook of my arm.  Then I endured three hours of stomach rumbling and toast envy, as was nil-by-mouth!  Finally I was wheeled in my bed, through the hospital (so all the visitors could play the ‘guess the operation game’ 😉 ) to the freezing ‘Cath Lab’.

It was only a 40 minute procedure, but felt longer.  I was awake and very aware!  A tiny wire was inserted -via the artery in my arm- into my heart… a giant X-ray screen showed it snaking its way inside my body.  Hordes of people wandered around doing important doctor jobs, or used technical machines to calculate measurements behind the glass screen.  There were shouts of “pause, test now”, “pause, test now”… with wires vigorously wiggled and manoeuvred between each go.  Tubing pumped blood out to be tested, and syringes of saline were injected back in.  It was odd.  For the most part it was fascinating; I enjoyed observing the action and watching the X-ray and beeping machines.  But then ever-so-often my stomach dropped as I reminded myself that they were fiddling around in my heart!  It was 40 minutes of fascination, interest, panic, fascination, interest, panic… and trying to keep very very still!  But afterwards I was rewarded with multiple cups of tea and a long sleep!

The results were good.  Amazingly, wonderfully, thankfully… the results were great! 🙂  My heart is continuing to work brilliantly; it’s normal sized; pumping efficiently and correctly.  The shunting and branching problems in my lungs caused by PVOD remain unchanged, however there is slight lessening of interlobular septal thickening so blood flow is a little easier.  The gaseous diffusion rate of my lungs has further increased; they’re now functioning at 33%.  But the best news of all, the biggest surprise, the shock… is that my Pulmonary Hypertension has significantly improved.  Massively improved!  When last measured in 2016 (during PVOD decline hell), the pressures in my pulmonary artery were at the high end of ‘moderate‘.  Now they’ve reduced so dramatically, the PH pressures are very mild.  They’ve halved.  They’re now really low!  Over the past two years, my powerful daily drugs have reduced the abnormally high blood pressure in the heart and lungs.  My PH is currently under control. 🙂

In fact, the positive changes in my heart and pulmonary pressure and lungs over the past two years, is so unusual considering I have PVOD as well… that my consultant has asked to write a medical paper on my case!  Apparently, “rarely do people stabilise when they have PVOD, never do they improve”. 😛  ae96e1599045c951df7dee7c246acd9cActually it took a while before I could smile at these results.  For the first few days I was an emotional whirlpool.  When the surgeon excitedly informed me of my pressures, I challenged him: “Then why am I still disabled?”.  I couldn’t comprehend how they could be my PH readings, when I was unwell.  Utter confusion.  Complete puzzlement.  Some fellow PHers with similar pressure readings can live relatively normal lives… yet I can barely look after myself.  It didn’t make sense.  Gently the surgeon reminded me that I have more than one problem.  I also have PVOD.  My lungs are failing.  Stomach drop. 😦

It was a tricky few days in hospital.  Normally I’m so proud of my wonderful battling body, so appreciative of it’s progress, so thankful of every small victory.  Normally I don’t compare myself to others, understand that my version of Pulmonary Hypertension is super rare, focus only on my own journey.  But this time, I really struggled with everything.  My PH team were delighted with my lowered pressures.  But the more they tried to cajole me into smiling, or exclaimed that I “should be happy”… the more I screamed inside.  I’m still ill!  I’m still ill!  Nothing has changed!  I was poorly before they knew the RHC results, I was still poorly afterwards.  I couldn’t understand their rejoicing.  I ranted that “these results don’t make sense” and “why am I worse than other patients?”.  I was blinded by my negativity so refused to acknowledge any personal functional improvement from two years ago when my PH had been worse.  I felt the results meant nothing.  I was still poorly, disabled, and awaiting a lung transplant… there was nothing to celebrate.

I had a pity party.  A cascade of anger and envy and guilt and sadness engulfed me.  I hated my lungs, I hated the PVOD, I hated the void of medicines to reverse the devastating effects of my second condition.  I felt that by also having failing lungs, I was being cheated out of living a less symptomatic life, a better life, a more normal life.  With just PH alone, my lowered pressures might have seen me walking, driving, hoovering… but with PVOD as well, no chance.  Hours of comparing myself to other PHers who don’t also have dying lungs, then absurdly berating myself for not being as able as them.  Worry that the doctors and nurses thought I was lazy for not being more able.  Irrational guilt that if only I exercised more or prayed more or ignored the symptoms more, I would be able to function better.  Was it my fault?  Illogical and desperate plans to fight through the fatigue and breathlessness, in the hope of becoming more like others with mild PH alone.  Sadness and sorrow, as I mourned my dream of further recovery.  With PH pressures nearing normality, no chance of curing the struggling lungs due to lack of PVOD medicines… I faced the reality that I’ll never improve from the me here now.  Sarah today is the best I’m ever going to be, until transplant.  Hope shattered.  Sadness.

52ce1419f67ed2502c8c67af53f7d5e2But back home, after a few Lottie and Phil cuddles, the haze of negativity lifted.  I stopped comparing myself to others, and instead started to rationally compare myself now to when I last had my pulmonary pressures measured.  The me of two years ago had high pressures and a dodgy heart; I couldn’t walk to the kitchen, hadn’t cooked in months, struggled to function.  Now with lower pressures and a happy heart, I’m less breathless, less fatigued, more independent.  I walk further, cook regularly, take day trips.  Of course I’ve improved over these past two years!  Yes I’m still ill and symptomatic as I also have failing lungs… but I’m in a much better place than when my PH was worse.  I thought back to PVOD diagnosis day, when my prognosis was poor and my future looked bleak.  I was expected to get worse: my pressures were supposed to increase further.  But instead the opposite has happened. 🙂  I also began to realise that lower PH pressures mean my battling body will be facing fewer problems.  Less strain on my heart and vital organs.  A reduced chance of heart attack.  A stronger and healthier position for transplant.  It took a few days to recognise the positives, but at last I was able to smile.  I loved my lungs again, I loved my body again.  I was thankful.     photo 1 (20)It was supposed to be an uneventful stay at the Brompton.  Four days of Netflix films and tea machines and yummy three course meals.  But instead it was four days of relearning lessons and reaffirming self-love and chocolate.  Plenty of chocolate!  🙂


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