This year I turn 30 and that number has been in my head since the day I was diagnosed. I was told at that time, people with my type of PH, which is PH with Eisenmenger's have a life span to 30 years old. I didn't enter 2020 thinking oh God, this is my final year on the planet, but it makes your mind go rather crazy. I think how have I gotten this far and lived such a wonderful life so far, but on the other hand, I think, I'm reaching 30, how much more life is in me? Yes it sucks having to think this, but when you live with a deteriorating illness, you are given statistics and facts about living with that illness and it wouldn't be human if I had these thoughts boggling around in my mind.
I know there are a few PHighters (people who fight PH!) that are doing very well with their PH and Eisenmenger's and are living life to the full and are over 50.. so I know since I was diagnosed the life span has increased and I am very hopeful that I will be one of those people.
So back to my PH appointment, we discussed how I was getting on at the moment. PH test wise, I have improved and I am stronger, but right now I'm feeling rather crappy. We've put it down to the cold weather as this really affects my breathing and I'm back to struggling to walk any distances without getting very breathless. It also means I've been on the O2 a lot more than usual to get through these cold days and nights. I say, bring on the Summer!! Also I'm low on iron again, which also makes my symptoms worsen, so I'm booked for another iron infusion this month.
We discussed future medication and found that there a few medications which aren't suitable for my type of PH, so that's less options for me in the future. However my consultant told us they're working in the backgrounds to bring out more meds that should suit me and they're hoping they'll be ready within the next 10 years.
I am currently on Sildenfil, which I've been on for the past 12 years, and most recently I moved to dual therapy and went on to Macitentan last year. Fortunately, I have taken to this therapy very well, which is why PH test results have improved.
My consultant told me that when my body stops taking to this therapy, my only medication option is Epoprostenol through a Hickman Line, which is a long, flexible plastic tube that is inserted underneath the chest wall skin and into the large vein draining into the heart. This also means I will be put onto the transplant list at the same time, to hopefully receive a double lung transplant.
Extract from PHA UK - Epoprostenol is given intravenously (injected directly into a vein) via a permanent catheter (a thin, flexible tube that is inserted into a vein). This catheter is called a Hickman line or a Groshong line. Prostanoids are continuously pumped into the body by a portable pump. It must be given this way because the medicine only lasts for a few minutes in the bloodstream. The pump attaches to the Hickman line via a thin plastic tube. The pump is worn attached to a belt around the waist or carried in a small shoulder pack. Smaller children usually use a secure backpack.
But the good news is that my consultant believes that I will be on my current dual therapy for around 10 years, yay! So another 10 years has been added onto my lifespan!! All in all, it was very good to talk about my future plans, yes it's scary having to have these talks, but it's good to know that a plan is in place, even if life doesn't always follow them! Howevever, I am ever so grateful that I've got such a fantastic PH team at the Royal Brompton, who are looking after me and making sure I am able to live the best quality life I can. So cheers to another 10 years of keeping positive and keeping up the PHight! Also with the love and support from my biggest supporters, I know they will make sure I continue to live life to the full.
Thanks for reading!
Thanks for reading!