My PH Journey So Far – Getting the Diagnosis

I was a healthy child, a healthy teenager and a healthy early adult.  I rarely had to visit the doctor and was never allowed a day off from school for being ill!  Up until recently, I’d only had two hospital visits in my entire life- the most dramatic being a painful sprained ankle whilst jumping from a fence doing a muddy cross country run around Bosworth Park at school (I got out of cross country for a few weeks after that!).  Nothing extraordinary, nothing out the normal.  In fact I would have described myself as pretty active and pretty fit.  I loved playing in all the team sports at school, loved hill walking with the Duke of Edinburgh Award scheme as a teenager, and later found some insane thrill from canoeing down frightening rapids at university (screaming whenever it got scary!).  In fact I met my now husband, Phil, in our university canoe club many years ago (he was much much better at it than me, and didn’t scream!).  Phil loves exercise and is as fit as “a thousand gazelles” (his words).  We also both loved the outdoors, and so consequently our weekends and holidays were spent walking in the hills and countryside, and our evenings were spent playing badminton and squash.

Then around the age of 23 (2003), as I was nearing the end of my university life, I started to notice that I was getting out of breath after less exercise than normal.  I remember taking part in a charity netball tournament and finding it really difficult to run around quickly for very long.  However I concluded that I must not be as fit as I was when I played netball at school, and tried to exercise more as a consequence.  And that became the pattern for the next 8 years.  I exercised more, yet over time I continued to get more breathless at less activity.  I had a lovely group of friends who would meet up for weekends away walking in the mountains.  Originally we all walked at the same speed, however I first found it difficult to keep up on the uphills, then over the years I couldn’t keep up on the flat, and eventually by about 2009 I wasn’t even able to keep up with them on the downhills.  I joined a gym before our wedding, and despite doing 2 hours of cardio, three times a week, for 9 months, I still wasn’t getting any fitter.  Aside from a deterioration in my exercise tolerance, I felt fine and healthy.  I was however concerned enough to mention my exercise problem to the doctor, in fact I did so yearly for those eight years.  Unfortunately all of the doctors I saw blamed it on anxiety and ageing, and you don’t really argue with your doctor’s judgement.  However I always knew that something was not quite right, and used to joke that “one day it will kill me and then you’ll discover I only had one lung!”.

In 2010, Phil and I went to live and work in Ethiopia for a year (read our blog from that time here) with VSO.  During that time there were three separate periods when I was ill for about a week; symptoms- weakness and breathless when trying to walk.  Unfortunately we literally lived in “the middle of nowhere”, it was a two day drive down dirt roads to the nearest hospital, and there was no healthcare in our immediate vicinity.  Luckily I just rested and each time I recovered, and was able to carry on as normal afterwards.  On returning to the UK, I did go and visit the doctor about it, but was once again dismissed.  The following year we went travelling around the world (blog here and here) and spent a lot of time doing multi-day walks in the mountains in New Zealand.  By now we were so used to my slow walking (any quicker and I couldn’t breathe) that we had to adjust the walk times to accommodate my speed.  However I might have been slower, but we were still able to do all the walks we wanted to do, we just often returned in the dark, and I often needed a rest day afterwards!  However one memorable walk in China left me very ill, and caused us to cut our travels short.  Tiger Leaping Gorge is a spectacular two day walk, walking uphill along tiny cliff side tracks at an average altitude of about 2700m.  By the end of the first day I was so ill; I had a cracking headache, was struggling to put one step in front of the other and was very weak.  The next day we managed to get down off the mountain and returned to our hostel.  However I didn’t recover, and was displaying all of the symptoms of altitude sickness, despite only being at 2700m (it normally occurs higher than 3500m).  I spent three days in bed in the hostel, not recovering, before Phil managed to carry both our big backpacks and get me on a train to a town at a lower altitude.  After a week at this lower altitude, I slowly recovered, and we were able to carry on travelling for a little longer.  I suffered no long term effects from this incident, and life carried on.

Then one day in March 2013, I got up to get ready to go to work, when my legs suddenly collapsed beneath me in the bathroom.  My feet were blue, my lips were blue, I felt weak, and I could barely talk as I was out of breath.  We rang NHS111 for advise, and they sent us an ambulance.  Three days in hospital later, the doctors were none the wiser what was wrong with me, and I was sent home with a sick note, and a hope that rest might solve the problem.  Unfortunately I never recovered.  Since then I began to suffer from breathlessness with minimal exercise, weakness and fatigue.  For the following six months I was seen by cardiologists, neurologists and an infectious diseases consultant.  There was no answer, and so eventually I was diagnosed with Chronic Fatigue Syndrome.  Those six months were incredibly difficult.  I felt so ill and needed so much help doing everything.  My Father-in-law came to live with us for the first three months; as I couldn’t really leave my chair, he would bring me lunch and cups of tea, and I just read, meditated and listened to calming music.  Phil had to help me shower, and I lived in comfy pj bottoms as they were easy to put on!  I could barely walk around my house, relied on Phil to push me in a wheelchair if we went out, and often needed carrying up the stairs at the end of the day as they were too tiring.  Visitors exhausted me, and they were limited to an hours visit, which was really difficult when you live so far away from your nearest and dearest.  If people came for longer, or I had a hospital appointment that took longer, then I would spend the next day barely able to move, barely able to breathe and feeling achy.  Phil and my Mum took over everything that needed doing in the house; my Mum making the hour journey to visit me every weekend for the first few months to do our housework!  As the six months went on I began to learn what I was able to do without ill effect, and got slightly stronger, and was even able to be  bridesmaid at one of my best friend’s wedding, albeit in a wheelchair!

However in October 2013 I went dramatically downhill again.  One day I was home alone whilst my husband was working, and felt very breathless and weak.  Another ambulance and another two days spent in my local hospital, with doctors scratching their heads, and making me walk so they could watch my oxygen levels fall.  But still no answers.  However the cardiologist had noticed that my heart was enlarged- the right ventricle was almost double its normal size.  When they performed a bubble Echiocardiogram, they noticed that there was deoxygenated blood in a part of my heart where it was supposed to be oxygenated.  He eventually suspected Pulmonary Hypertension (PH) and I was transferred from my local hospital to the John Radcliffe in Oxford, as it has a large heart centre.

Once again I was put through many more tests; CT scans, Perfusion scans, ECGs, blood tests, liver scans, before eventually having a Right Heart Catheter to measure the level of blood pressure in my pulmonary artery.  By then I was so exhausted from being taken from one test to another; it had made me dramatically worse.  I was barely able to walk, my legs were weak and shaky and my breathing was awful; during the 6 Minute Walk Test (6MWT) I was only able to walk for 23m, even though I was trying to walk for the whole 6 minutes.  Therefore getting the diagnosis was actually a relief.  I was relieved all the testing was over, I was relieved there was now a chance I could have meds, I was relieved it had a name.  I didn’t really think about what they were saying- it’s progressive, it’s terminal, you can’t have children.  It took many months before my head allowed myself time to process all of that.

I stayed at the JR hospital for a further week whilst they were waiting to transfer me to The Royal Brompton Hospital in Chelsea, London, one of only a handful of hospitals in the UK that was able to treat the condition.  During that week I got dramatically worse and increasingly terrified.  I relied on Phil to do everything; from passing me things out of hand’s grasp, to helping me dress, wash and brush my hair.  He was with me every day, all day.  I was wheeled everywhere (even to the toilet 3 metres away).  I struggled to sit up in a seat for longer than 5 minutes, so spent all day in bed.  Sometimes I felt too exhausted to even speak, just trying to mumble one word in the hope they would understand.  The worst were the points when I was too tired and too weak to even breathe.  My body felt too exhausted to move even a muscle.  It felt like my conscious was burrowing inside myself, and for an hour I didn’t move anything, I simply concentrated on breathing, in and out.

Arriving at the Brompton on a Friday and watching the stern faces of the doctors and nurses as they examined me, I have never been so scared.  I honestly thought I was going to die that weekend.  I was at my weakest point ever.  I asked the doctor if I was going to die in the next couple of days, and he said no.  I remember the relief.  Suddenly there was a little glimmer of hope inside myself that maybe I could get better.  There was some debate about what medicine to start me on: my PH pressures were rated as moderate, yet my symptoms were rated as severe (WHO level 4).  Eventually they decided to see if I would improve on oral therapy, thus leaving IV therapy as a backup if I didn’t improve in a few months.  So I was started on dual oral therapy- Sildenafil and Ambrisentan (PH targeted drugs), as well as Spironlactone (a diuretic), Ferrous Sulphate (iron tablets) and Warfarin (a blood thinner).

Within days I started to feel better.  I remember a red-haired nurse who gave me the encouragement to walk for the first time in weeks, a few steps around my bed.  I had daily physiotherapy sessions for the fortnight I was there, as on top of the PH, my body had become deconditioned, thus the weakness.  Everyday I tried to walk further (I fondly remember my fellow ward patients clapping me when I was able to walk across the ward for the first time).  I started to dress myself again and brush my own hair.  Before I was discharged I was made to do the 6MWT again, and this time managed 75m.  I was very happy.  After being in hospital for a month, I was eventually allowed home, to start my new life with Idiopathic Pulmonary Arterial Hypertension (IPAH).

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21 thoughts on “My PH Journey So Far – Getting the Diagnosis

  1. Please keep writing Sarah. Having known you since we were 5 years old, and knowing how active you’ve always been, I just cannot believe how or why you have developed this horrible condition. I am constantly amazed at how positive you are and how well you cope with it every single day. Sending my love xxxx

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  2. Great read. Thank you for sharing. Just remember.. living with IPAH is still living! I am on the same medications as you are plus sub q Remodulin. You may never get fully used to it but you will adjust! I hated it at first but now its just part of my life. New and better medications are coming out all the time. In time, I know there will be a cure! Best wishes!

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    1. Thanks Jim. I’m glad you’re getting on well with your medication. I agree that living with PH is still living! 🙂 And we’ll get our cure one day! x

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  3. I have PAH as well I was diagnosed in 2013 too, i loved reading your story, I dont think anyone else is blogging on this condition. There are stories, but not daily or weekly struggles on a continuous basis. PLease keep it up.
    Your Phriend in Florida USA Jennie

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    1. Hi Jennifer, a fellow PH-er! I have a few friends who also blog about PH, at some point I’ll try and work out how to link their blogs up so you can see theirs too. I hope you’re well. x

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  4. Sarah I LOVED this blog. All the symptoms for years mirrored mine, though I didn’t go walking around the world, just Spain! Looking forward to the next one very much indeed. Xxx

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  5. You are a very beautiful lady with a very beautiful and amazing story to tell. I hung on your every word. Please keep writing. Sweetie, do you have any idea that you are someone’s light in a very dark storm? Please allow the Lord to use you and open your heart to what God has in store for you Kate. He’s not finished with you yet. Norma J. Miller/USA

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    1. Thanks Norma, very kind words. I hope my story might be useful to someone at some point. When first diagnosed, I wanted to know as much as I could about the illness, so hopefully this might explain to someone about it. x

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  6. You have written so beautifully, and remembering your active lifestyle all the way from school I can only imagine the intense shock this was to you.

    You are doing so well, and thank you for allowing us to read about your journey. Much love little Ewe.

    Kel x

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  7. Oh Sarah wot an interesting, amazing and well written blog.. I loved it 😉… and very close to my heart being a fellow PH gal xx

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  8. Thank you for sharing. I was mid-diagnosed as well. I was also very fit prior to diagnosis, and I also tried working out harder to improve my stamina with no improvement. I’m on IV Veletri (and O2) which has been a life saver. I sincerely hope things improve for you!

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    1. Hi Colleen. Yeah it’s srange when you’re fit and then suddenly not, but nevermind. I’m glad you’ve got on well with your meds. x

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  9. Similar story here as well. Was a very active child (but did have some health problems and congenital abnormalities), an active teenage life, got married at 19 and life carried on. Had always been involved with Scouting so was camping, hiking and cycling regularly etc… In 2003 my life came to an abrupt standstill. I was suddenly taken ill at the end of May and went downhill very quickly. Was seen at the Sheffield PH centre in the September and referred onto a ventilation specialist in October 2003. This was the turning point. Relatively stable but with many up and downs since then. Keep going…I do, just a little slower than most.

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  10. Hi Sarah
    I am reading the blog from the start. You have a skill at capturing life with PAH/PH. It got me thinking about writing one too! Keep writing and those pics are fantastic x

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