After being so ill when first diagnosed with Pulmonary Hypertension, my biggest fear in the world is being that poorly again. Unfortunately PH is a progressive disease, so at some point it is inevitable that my PH will worsen. The hope, therefore, is to spot this change before it damages my heart too much and makes me so ill again. As a consequence, I’ve found that I have a little part of my brain that is keeping a watchful eye on me, checking my daily progress. A good thing about having a daily routine is that this makes it easy to compare one day with another. When I’m able to do something for the first time, then there is internal cheering. When I can’t do all my daily exercises, then there is slight nervousness. In the past couple of years, there have been a handful of points where I’ve suddenly become very symptomatic, unwell, and not able to undertake my daily routine, yet there is no obvious reason for this. The first time this happened, I was immediately panicky and ended up at A&E. In fact, Phil was running a marathon that day for our organ donation campaign (see here), and crossed the finishing line to hear I was now in hospital. He then had to spend the rest of the afternoon in sweaty post-marathon clothes as he came straight to see me!
However over time, I’ve learnt that these unexplained blips are likely to be a feature of my PH, and I have to wait a few weeks for my body to fully recover from whatever caused it to be ill (One of the issues with having a rare illness, is that a lot of things cannot yet be explained). The shortest ill period has been a few days, the longest 5 weeks; thankfully I have always come bouncing back to the level I was before. I now recognise that my body is going to do this occasionally; I’m usually able to not worry too much in these situations, but instead try to just patiently wait for it to mend itself (plus positive thinking works wonders). I think my Mum finds these periods most stressful; I can hear her panicking on the phone when I tell her, therefore I generally don’t like to mention if I’m feeling worse (love you Mum!). I also now wait a few weeks before contacting my consultant, and having a quick check up.
At the end of October (2015) I was feeling really pleased with my walking- on a couple of occasions I had managed to walk the 500m length of one of our local footpaths. However one evening as I was walking along with Phil (with Lottie running around madly next to us), I found that I was feeling slightly breathless after about 300m. Having achieved the 500m route the day before, I, probably foolishly, tired to push myself further, as I desperately wanted to complete that distance again. (It is important to push yourself else you’ll never get better, just not too much!). Anyway as a consequence (yippee) I managed to walk the whole distance, but (boooo) I felt awful for it. I felt light headed and weak. I was breathless: my bag of air felt very small, which meant my breathing felt more shallow and I was unable to take deep breaths. I scootered home, and laid down and did nothing for the next couple of hours, whilst my body tried to recover. The next two days I felt awful (this is normal when you’ve had a busy day, you spend the next day ill) but by the third day I was able to start doing parts of my normal routine again.
Since then I began to notice that I was struggling more with my breathing, and was finding it harder to do everything in my daily routine. I could do the basics still – washing up, cooking, but now Phil had to start doing the clothes washing and other jobs. I was finding that I felt more tired and was no longer able to be out of the house for more than a few hours. At the start of November my best friends had planned a weekend away full of baby shower fun, as one of them had a little one due at the end of November. However, disappointingly I could only manage a few hours before I had to be taken home; I was very tired and breathless. At this point I still wasn’t really worried as it had been going on for only a few weeks, and I was pretty hopeful that it would sort itself again. I also bought myself a couple of light boxes (daylight lamps), as I was now rarely leaving the house, and these did help me to feel more energised.
However 5 weeks later I was still feeling more breathless and not able to achieve as much activity as normal. Luckily I had an appointment with my Oxford consultant (Dr Dwight) and my Brompton consultant (Dr Kostas). An echo showed my heart looked ok, but my walking test was slightly down (360m compared to 400m in August). As I’d still not naturally bounced back up, they decided to double my sildenafil (PH targeted drug) dosage to 50mg, and booked me in for a right heart catheter in January, to get an accurate assessment of my heart condition and pressures. I was feeling pretty optimistic that this would sort it out.
The next week or so was awful. My body didn’t seem to like the drugs increase, and made me incredibly weak (very reminiscent of being weak before drugs- thus very scary). I was determined to go to our best friends’ son’s first birthday party, but felt incredibly weak. We made it to the edge of our village before we had to pull over in a farm driveway and reassess. I couldn’t contemplate the journey, never mind the room full of people! With me seemingly reacting to the drugs increase, Phil had to take over everything again, though I could still get washed and dressed on my own, it just took longer. However a fortnight later and I was feeling a lot better, although still not as fit as in the summer. My condition seemed to be going up and down, up and down. Luckily we were approaching Christmas and I contented myself with sewing Christmas bunting and watching Christmas films! If you’re going to be ill at any point, better for it to happen in the winter when it is cold outside! Actually I was still having occasional good days, when I almost felt like I had in the summer, so we were able to have some lovely pre-Christmas celebrations with my family.
Christmas was sadly awful. My in-laws live on the Isle of Man and we stayed with them for a week. We were incredibly excited to be going over, especially as it was to be our niece’s first Christmas. Unfortunately the 4 hour ferry crossing was during a horrid severe gale (Beaufort level 9), and I never really recovered from this. Then Christmas Eve night was spent googling “what to do when your dog eats a whole bowl of nuts and raisins”, and waking periodically to check the dog was still snoring away next to us. Consequently the poor nights sleep probably didn’t help the situation, and although I awoke feeling very symptomatic, I forced myself to try and join in on Christmas day. I apparently looked “really awful” (thanks husband!). I spent the next three days in bed, having food brought to me, and best of all, my little niece brought to me to play with for a while. But it was a scary time; the hardest part was knowing I was on the island, and so many miles and a rough sea away from my normal healthcare. If I’m ill in the UK, there are a number of hospitals that specialise in PH. However, unfortunately the hospital on he island has an A&E but it is just the equivalent of a small town hospital. On the day we left, I thankfully started to feel more human, and we thankfully had a better crossing (Beaufort 7).
I had kept my PH nurse (Emma) updated about my condition, and by the middle of January as I appeared to still be quite ill, she called me in for an emergency check up. I was now feeling increasingly breathless; every little activity would leave me struggling to breathe. My oxygen saturation levels (sats) were constantly falling every time I did anything. Normal people’s oxygen levels are 99-100%, and they stay like this always. A symptom of PH is that your oxygen levels fall with any exertion. Since being on medication, my sats hadn’t been falling as much (e.g. 92 after a 5 minute walk). However now if I went to the porch to get the mail, they would be on 82. If I made a cup of tea they would be on 80. I’d walk up the stairs and they’d be on 74. My fingernail beds were now permanently blue, my lips kept going purple, and I was constantly cold (but it is Winter!). I was doing the 6 Minute Walk Test at home most days, yet I could only manage on average 150m (down from 360m 6 weeks earlier). I was also struggling to cope with visits and visitors. One of my best friends had had a baby girl at the end of November, but I’d been too ill since then to make the journey to London to see her. However living life is important, and sometimes you just have to do things even though you know you’ll feel bad the next day. Therefore I pushed myself to have a wonderful post-Christmas get together with my best friends and their gorgeous babies. I had a really lovely time and some fantastic baby cuddles, but unfortunately spent the next day feeling ill and with increased breathlessness.
The NHS is amazing. Within half an hour of Emma ringing me for an update she had spoken to the PH doctors, and had arranged for me to have a check up and some tests straight away. Phil came home from work to take me into the Oxford Heart Centre. An ECG and Echo showed that my heart was still looking ok, and from the echo they estimated that there was no change in my pulmonary pressure. However my oxygen sats were falling with activity; after a 20m walk they fell to 92, after talking for about 20 seconds they fell to 87. In the summer, they would have remained at 97 during both of these activities. At least this supported what I’d been experiencing at home; the last thing you want is your body on its best behaviour when someone is watching! I was due in for a Right Heart Catheter procedure a few days later, so a decision about the next step was postponed until after that. The doctors were pretty hopeful that it was just another blip, but by this stage I was beginning to worry that it was my PH progressing. We would find out in three days time.