We Can Adjust The Sails

This week marked the third anniversary of my initial dramatic collapse, which turned out to be Pulmonary Arterial Hypertension (PAH).  In a strange coincidence, this week will now also be the date when I found out the name of a second lung condition I am also suffering from.  At least it makes anniversaries easy to remember!

I spent the start of the week at the Brompton.  They’d been deep cleaning the ward so had kept in less female patients, thus amazingly I was given a whole bay of six beds to myself! Last time I had a whole side room to myself, now a whole bay, should I be expecting my own personal ward next time?!  I had a couple of visits from London friends, which was lovely, and I completed some tests, including being injected with, and breathing in, radioactive material (VQ scan).  Luckily baby Agnes came before this test, as apparently you’re not allowed in contact with little ones or pregnant people for 24 hours afterwards.  The main reason for my visit was to meet the respiratory professor who had been looking in to my case, and to have a final conclusion conversation with my PH team.

Oxygen on a trolley- much easier to move around

Baby Agnes was a very welcome visitor

A whole bay to myself!

Over the last few months, my Brompton PH team have been running tests and slowly whittling down the possibilities for why my lungs are currently struggling.  My symptoms have always been more dramatic, and different to what they would normally expect from my variety of PH.  In the proceeding weeks, the doctors had starting dropping in the names of possible diseases.  I’d done my best to avoid googling suggestions, but when one name became increasingly mentioned, I did finally give in and see what the internet had to say about it.  However amazingly I was able to stay calm and positive, and refused to allow myself to worry until I was officially told that was their conclusion.

So finally there is a second diagnosis.  I have a second related condition called Pulmonary Venous Occlusive Disease (PVOD).  Simply put, in Pulmonary Arterial Hypertension (PAH), the arteries in my lungs have become narrowed and calcified, which means the blood is struggling to travel through them, causing a backlog of pressure in the heart, which damages it.  In PVOD, the veins in my lungs are also blocked up.  In my case, the build up of pressure is causing some of the blood to bypass the capillaries in the lungs, so is not being oxygenated.  This process is exacerbated when I am doing exercise or activity.  So both the arteries and veins in my lungs are blocked.  Poor lungs, no wonder they are struggling at the moment.

The only definitive test for POVD is a lung biopsy, a dangerous and painful procedure.  Their diagnosis is therefore based upon best fit, I am showing all the signs and test results to indicate it, but the final definitive test will have to be reserved for autopsy.

PVOD is incredibly incredibly rare.  According to my consultant, every year there is one case diagnosed per 10-100 million people.  By my calculations that’s about one new case in the UK each year.  There are better odds of winning the lottery than developing PVOD!  You can’t say I haven’t got ill in style!

To be honest, it’s not great news.  Like PAH it is progressive.  Like PAH it is terminal.  Like PAH there is no cure.  However unlike PAH there are no further medicines to help try and slow down the progression of the disease, or help abate some of the symptoms.  In fact, some of the PAH medicines can have detrimental effects, which may have been why this condition has worsened since I had a recent meds increase.  It is now going to be a delicate balancing act, giving my body the correct medicines to help the PAH, and so stop me from developing heart failure, yet also being mindful of not worsening the PVOD by doing so.  Due to the lack of medicines to slow the progression, and the lack of research into it, the prognosis is bad.  Consequently there is absolutely nothing the doctors can do and so my only chance now is a lung transplant.

It’s a strange situation when you find out news like this.  If it was a movie, there would be dramatic music playing, rain pounding on the windows and some dark and moody lighting.  The reality is about 6 PH doctors and nurses crammed in to my tiny ward cubicle, all staring at me, as they already knew what was about to be said.  They drew the curtains to give the pretence of privacy (however the fellow patients in the next beds are listening in their boredom!).  The sun was streaming through the windows.  There was only the sound of children playing in the park across the way.  And then they told me what they thought I have.  I batted a few questions to them, namely can we do anything about it, what happens now?  And then I burst out crying.  And that’s it.  I suddenly have a new disease.  I suddenly have a different future, a different plan, new fears, new hopes.  All the alternative treatments have suddenly disappeared, and instead I have one egg left (transplant).  And then after 10 mins of crying, I got over the shock and I began to accept it.  In a way, getting a second diagnosis was a relief, as it explained why I’ve been more symptomatic than expected, and ended the speculation in my head, the run-through of possible causes.  Even bad news is better than the waiting and wondering.  Instead I am left with the facts; I know where I am at, and am now able to work out the plan of action for the future.  The next day at home, I needed a day to cry, to stay in bed and to feel sorry for myself.  And then the following day, like medicine, the sun streamed through the window, and awoke me with a smile.  And I got on with it.  For surely there is no other way.