Driving down the M40 on the way to the Brompton hospital, I was adamant that I’d be returning without the steroids. Having suffered their side effects for the last two months, I had my fingers crossed that I’d be able to stop taking them now the trial period was over. However it didn’t quite work out like that!
I’m not a fan of hospitals. I’d rarely stepped inside one until I developed Pulmonary Hypertension three years ago. Since then, I have been a regular visitor. However appointments and stays have never got any easier; it is always a nervous drive down worrying about what the results will be. Until now! Strangely, this was the first appointment where I wasn’t too apprehensive about what they were going to say. In my last visit I’d been told that I have a second illness, Pulmonary Venous Occlusive Disease, and that there is nothing they can do about it. The PVOD is going to cause my lungs to get progressively worse. Therefore, having received this news last time, I went with the expectation that my lungs would have deteriorated. I accepted that I’d be receiving bad news, and in a way this made it easier as there was no second guessing what the results would be.
Despite the torrential rain in London, figuratively the sun was shining on us that day. We weren’t due at my first test until 11am, so Phil got a slight lie-in, and we had an easier drive without the rush hour London traffic. There was a vacant disabled space outside the hospital, so we didn’t need to drive around the side streets searching one out. For once, all of my tests were booked straight after one another so there was not as much waiting around in between. And we managed to squeeze in lunch and cuddles with my lovely friend Zoe and the gorgeous baby Agnes! 
And surprisingly, and incredibly, the sun was shining on my results too! 🙂 The Echocardiogram showed that my heart is still coping brilliantly despite the increased pulmonary arterial pressures and new lung problems. It is still not showing any signs of enlargement or heart failure. When my PH was this bad three years ago, my heart was incredibly enlarged and was starting to go in to heart failure; yet this time around, despite being at the same pulmonary pressure it is still coping! Yay for my heart! Then amazingly, the Lung Function tests showed that my lungs have not deteriorated any further! Despite PVOD being a rapidly progressive disease causing my lungs to nose dive for the first five months of the year, instead the results showed that they have stopped deteriorating and have stabilised so are still in the same condition as two months ago! My lungs are still in a poorly condition, and are struggling to get enough oxygen on their own, (hence why I now need supplementary oxygen for all activity), but I was incredibly surprised and relieved to be told that they hadn’t worsened in the last two months. Yay for my lungs! Finally, the cream on the cake: I managed to score my highest Six Minute Walk Test result all year, showing that my heart and lungs have currently found a way to allow me to move despite all of the problems!
In the “6 Minute Walk Test” I have to walk as fast as I can for six minutes, walking between cones set out at 10m intervals. The distance I walk is recorded, as is my oxygen saturation scores to ensure enough oxygen is getting around my body whilst I am doing it. My best ever score was 400m in August 2015, a few months before my PH started deteriorating and my PVOD was observed. In January I managed 180m, and by February, my poorliest period this year, I was unable to walk 30m let alone walk for 6 minutes. For the first time I completed this test with oxygen, as the test is supposed to represent how I would be walking around at home. Amazingly, with oxygen, I managed to walk for 330m in 6 minutes… not far off my all time best (though that was without oxygen)! And brilliantly, my body was able to maintain my oxygen saturation. I was gobsmacked at this result. I knew that my body was coping well since I’d been given oxygen, but was not expecting to walk as far. Obviously I would not be able to walk at all without the oxygen, but it is reassuring that we have found a medical intervention that is allowing my body to be active.
So in conclusion, the veins in my lungs are still pretty bad and finding it difficult to take oxygen from the air in to the bloodstream (PVOD). The pulmonary pressures in the arteries in my lungs and joining my heart are still high causing my heart to be working under increased strain (PH). However currently everything is stable and my heart and lungs are managing. And by using supplementary oxygen whenever I do anything, my body is still getting the right amount of oxygen in it and so is currently coping with it’s problems.
My final appointment of the day was with the respiratory registrar working with the professor who’d initially prescribed the steroids. He was confused by my results! He expected my lung function tests to show either a further deterioration (the steroids hadn’t helped) or a significant improvement (the steroids had helped). Instead, as my lungs had stayed the same, he was unsure of what to do! Although he was fairly certain that the steroids are not the reason for my stability, he was nervous to stop them -just in case! Therefore I am to remain on them for a further two months, before the tests will be repeated, which hopefully will give a better insight. I entered the appointment determined to stop taking the steroids, but the doctors apprehension to stop the medication made me nervous too! So I meekly went along with it! However he did agree to let me reduce the dosage, so hopefully the side effects will reduce also. Fingers crossed.
So as we drove back down the M40 towards home, I alternated between whoops and hi-fives at my surprising test results, and nervous glances in the mirror and pokes at my ever rounding moonface! Another two months on the dreaded steroids!
We Can Adjust The Sails 