Wednesday, 12 April 2017

It's hard not to listen to the numbers

Dear world,

It’s been a while since I last put pen to paper, ok, finger to keyboard, but I’ve really been struggling for inspiration of what to write next. I’m sure those who know me, know I can wax lyrical when there is not so good news, but when I’m feeling good, apart from saying I’m good and all is well, it’s hard to write a whole blog about that. Talking of good things, I am feeling really good and if I look back over the last year I have done amazingly well. Granted I have my down days where my body demands a duvet day as I have way over done it, but you don’t have to have PH to have those days!

I’ve been fighting off the demons and keeping up with my exercise regime. I’ve even started properly getting into running too. I’ve found a great app on the NHS website, which is a running podcast called Couch to 5k. 
It’s a 9 week running program that’s been designed to get just about anyone from the couch to running 5km or 30 minutes in just 9 weeks. I’m currently on week 2, so no not Paula Radcliffe just yet, but its great inspiration for me to keep going. I’ve never been a runner so I had no clue where to start, well apart from trying to make sure I put one foot in front of the other and go slightly quicker than walking. But I found after 2 minutes I was literally about to keel over and die as I had way over done it and completely out of breathe! But this podcast breaks it down into little consistent running and walking breaks. Totally takes the stress out of thinking when 60 seconds is up and am I meant to run or walk now. It’s a total no brainer. In week 1 I did 60 seconds of running, 90 seconds of brisk walking and so on for 20 minutes. Laura (the running commentator) tells you when to start and stop and along the way gives you a few tricks on posture and breathing etc. It’s genuinely been the best treasure I could find.

As well as this I’ve been going to a cardiac rehab clinic twice a week at the local hospital. I knew I was going to be the youngest there by a good 30 years but the memo hadn’t quite got round to the other people in clinic that day. Their faces were a picture when I arrived in my gym gear all pumped and ready to go. I think they thought I was the new gym instructor. So when I started to follow them and copy what they were doing they were quite baffled and when they heard my accent I think they were even more bemused. It’s been great though, I do an hour twice a week working my way around different gym equipment rating how tired and breathless I am feeling and checking on my heart rate and oxygen levels throughout.

I had an appointment at the end of February to see my new PH doctor. I was a little apprehensive to say the least as I have only ever been seen by the doctors at Sheffield. They have seen me at my worst saving me from death (not to be dramatic) and got me to a stable point, so for me they are pretty important people who I have a lot of trust in. In a way I felt like I was cheating on them by going to see the new doctor! Daft isn’t it!! Anyway I looked at it that it was only a consultation and what was the worst that could happen.

When we met her I could tell she had a good grasp of my medical history with her tone and what she was saying, so she eased my initial worries. Then she started to focus on the fact that I was a nitric oxide responder when I was first diagnosed. We knew from my diagnosis 3 years ago I was a responder which is a very positive thing to have when you have PH. 3 years ago Sheffield tried me on high dosages of calcium channel blockers which are meant to help a very very small amount of people with IPAH if you are a responder. If successful to the drugs it means my prognosis could be a lot better; hopefully live longer than I will on current medication, reduces the chance of rejection of the drugs unlike the ones I am currently on and the drugs are a lot less damaging to my vital organs than the current ones I am taking, therefore meaning I can cut out the regular trips to the doctors I currently have to. Which I would welcome as who wants to go to hospital regularly?!

At the time 3 years ago, the doctors tried to get me on high dosages of calcium channel blockers but my body didn’t respond the way it should and as I seemed to be declining quickly the doctors wanted to put me on more aggressive medication. This worked and due to being stable the doctors at Sheffield have been keen to keep me on the current regime so I could continue as I have been.

The downside with this medication regime is one day it will stop working. My body will stop responding to the medication and therefore I will go back into decline unless they find other medications to work.  This has always been at the back of my mind, especially the last year thinking I am good now but will I just wake up one day and find the drugs no longer work and that unless there is a cure or new medication then we are looking again at transplant or worse, death. It’s something that keeps my anxiety up there I can tell you!

It turns out that this new doctor’s specialism within PH is people with IPAH who are nitric oxide responders and getting them onto high dosages of calcium channel blockers. Lucky for me I have IPAH and am a nitric oxide responder! So her plan of attack is to try me again on the high dosages of calcium channel blockers.

The odds are 50/50. I have been one of the lucky ones so far by being a nitric oxide responder. To put it in context the statistics are, 1 person in 1 million every year is diagnosed IPAH in the US and Europe. Take the UK for example with a population of 60 million, this means there are 60 people each year diagnosed IPAH. Out of those 60 people, half will be nitric oxide responders. Of which I am one. Out of those 30 people, half again will actually respond to being on high dosages of calcium channel blockers. Of which I am hoping to be! So I need to be one of those 15/60!!

If my body will respond to them this time, the plan is over time to reduce the dosages of my current medication, maybe even taking me off these altogether and keep me on calcium channel blockers. It might be a complete pipe dream that doesn’t work, but it also might. It’s some hope. And I need hope.

I came away from the appointment completely out of sorts to be honest. It didn’t feel real. I wasn’t sure if I was to scream ecstatically from the rooftops that someone might have an answer to get me as close to being cured as humanly possible or whether I should keep it a secret in case by saying it out loud it might stop it from happening. I told family and a few close friends but apart from them I wanted it to be my secret, and suppose let it all sink in. 

I also wanted to wait to share anything as I needed to have it confirmed I was still a responder. As if I wasn’t then it would be a complete non-starter. All hopes would be completely dashed and it would just be carry on as you are for now and wait for your body one day to stop reacting to the medication. But I had it confirmed last week after having the right heart catheterisation that I was still a responder. So we are good to go and start on the high dosages of calcium channel blockers. I next see the doctor at the end of May so hopefully then we can discuss when I will start them.

I did receive some slightly unsettling news whilst having the right heart catheterisation. It showed that my pressures have increased, which does suggest deterioration. As my pressures were last checked December 2015, I’m not sure when in the last 18 month period they have increased so it’s hard not to be able to pinpoint when this happened. I hope when I see the new doctor she can give me comfort that this isn’t the start of the decline. Having PH is such a mental disease. I feel really well and am able to do more cardio exercise than ever before but yet inside the pressures say a different story. Overall though it’s still extremely positive and I have to focus on how I am feeling each day. I am beating the statistics by being alive now and I hope to continue beating more statistics, and being one of those 15 who can be treated by being on the calcium channel blockers.

As always thanks for reading.


Gemsy xx


  1. Hi Gemma, just caught this post which was lovely as I'd been thinking of you as well as the other folks I 'know' via the heart and ph blogs. I'm so pleased to hear you're feeling so well. Hands down the best sign of how things are surely? I share with you that continual sense of foreboding that has you looking for the beginning of the end too often. I'm just getting over a horrible chest infection which put me into hospital last weeks. It's put my body and mind in turmoil again and I'm hoping to get back on solid ground too. I'd been starting a little jogging - well shuffling more like �� Pedestrians pass me! ��. Now I feel so crappy I'm not doing anything. Few days and hopefully I'll be up and at 'em again. Heck I'd love to get to a point where I could try start of the couch to 5k. My husband loves it. Fingers crossed.

    Just wanted to say hi and send best wishes. With you in spirit and totally admire your courage in confronting all this.

    Love Jill xxx

    1. HI Jill,

      Thank you so much for your comment.

      Hope you are feeling better and your chest infection has gone now. Great news on the jogging, don't worry about people over taking you, it's not a race against anyone else. Just do it for you no one else.

      Thanks again,

      Gemma xx