Let's Talk About Pulmonary Hypertension

November is Pulmonary Hypertension Awareness Month and this week it is Pulmonary Hypertension Awareness Week in the UK. Pulmonary Hypertension (PH) is a rare, progressive and incurable disease. It is a serious condition that causes high blood pressure in the pulmonary arteries, which can severely damage the heart and lungs. 

Unfortunately not many people have heard of it, so if you suffer from it, the lack of awareness can add to all the stress of the diagnosis and the battles you have to cope with on a daily basis. When you suffer from a rare disease like Pulmonary Hypertension, it can feel very isolating, as people don't understand about the disease and you are often misunderstood. Only circa 7000 people are affected by PH in the UK and it can affect anyone regardless of age or ethnic background. It is more common in women than men. 

Pulmonary Hypertension is a very general term to describe this disease, but it is a very complex disease and really is much more than just 'pulmonary' and 'hypertension'. Indeed, it does very much involve 'hypertension', which is high blood pressure and with Pulmonary Hypertension it is a diagnosis of high blood pressure in the blood vessels of the lungs. When I suffered from Pulmonary Hypertension I must admit I used to become quite exasperated with this tag of 'hypertension', as people continually told me all about their high blood pressure problems and the tablets they were on for it, which is a totally different problem. Simple and straightforward high blood pressure can be extremely dangerous, but it can also be safely controlled by a few tablets and lifestyle changes and isn't usually a fatal problem unless it's undetected. 

Pulmonary Hypertension, however is high blood pressure in the lungs and although it can be treated to try and stabilise a patient, it cannot be cured and it becomes progressive, damaging the blood vessels in the lungs, causing severe breathing difficulties and the heart to overwork until it begins to fail and it can become fatal. Treatments can be harsh, with difficult side effects and can include: oral heart failure tablets such as diuretics; targeted oral treatments to stabilise the high pressure in the lungs; oxygen therapy; intravenous medications and major surgeries such as operations to remove blood clots from the lungs, lung transplantation and heart and lung transplantation. 

PH patients suffer with a range of symptoms such as breathlessness, extreme fatigue, dizziness, fainting, swollen ankles and legs, plus the side effects of varying levels of medications. 

Take a look at the PHAUK website to find out more about the causes, the symptoms and the treatment of PH, as they are explained well and in depth on here.

PHAUK WEBSITE

Pulmonary Hypertension is no 'ordinary' high blood pressure, in fact when I suffered from it, my 'ordinary' blood pressure was perfectly normal. I looked perfectly normal and well a lot of the time too, as heart and lung failure isn't something that anyone can see. It's something that's happening inside you, only you know how fatigued and unwell you feel, people cannot see it. It makes it difficult for others to understand when they cannot see you are sick and it is a disease that is unheard of. That is why we need to continue raising awareness to help others to understand more about the complexities of this disease and the impact on a Pulmonary Hypertension sufferer's life. 

My own PH journey was a difficult one, with many hospital admissions and emergencies, countless tests and monitoring; many complex medications including combinations of oral medications, inhalers and complicated intravenous medication; a two year wait for, and then undergoing a heart and double lung transplant. My transplant has given me such a better quality of life, but a transplant does entail a whole range of other medications and new medical problems to cope with. My transplant was because of PH and my transplant journey is very much a part of my PH journey. 

When people say, 'You haven't PH any more - why keep going on about PH?' It's because PH has been a big part of where and who I am now. It's in my past, but has shaped my future. It's because PH is so misunderstood. It's because so many of my friends still suffer with PH. It's because we still hope for a cure for PH. 

Part of the reason I wrote my book 'Life is for the Living' was to help raise awareness of Pulmonary Hypertension - to try and show others what it is like to live with a rare disease and undergo the varied steps of treatment as the disease progresses and the impact it has on a sufferer's life. Any proceeds from my book are for the charities that helped me through my journey and continue to do so, including the PHAUK, the charity that gave me advice, support and put me in touch with other Pulmonary Hypertension sufferers at a very desperate time in my life. 

PURCHASE 'LIFE IS FOR THE LIVING'

I will always campaign to raise awareness of Pulmonary Hypertension, because once I didn't know 

anything about it. It didn't really matter to me back in those days. Why take the time to learn a little about any rare disease? Well it may just save a life - your own or someone else's or help you to understand what someone else is dealing with and going through. 

I didn't know anything about PH and its symptoms once. Early diagnosis equals better prognosis. If only I had. 

JOIN THE PHAWARE GLOBAL ASSOCIATION

What a Difference a Day Makes

Sailing through October and I've been steadily embarking on the first few weeks of my fourth year post transplant. I'd been feeling fighting fit - well very fit. I'd just arrived back from a holiday in Whitstable, where we've walked miles every day in the fresh sea air and autumn sunshine. It'd been a whirl of wonderful times - I was cycling around a park in the middle of the Danube River in Budapest only the week before, laughing, happy and carefree.

What happened? I'm in shock more than anything. I try and trace things back a little. Why did I not spot the signs? Should I have worried when I didn't? 

One of the things about being a transplant patient is that you have to monitor yourself and note down daily observations. It can become a little tiresome over time doing this everyday as the years go by. I know some people give up on it, knowing they know their bodies well enough if something is amiss. I feel a little like doing this at times, so admittedly I don't managed to keep up every day, but I do ensure I take a run of recordings every week. I take just enough to check in each week, reassure myself of what is my normal - it's that standard to look back on if things don't feel right. It gives me evidence for the doctors. I can sit and tell a GP, 'But my temperature's elevated at 37 - it's been 36.4 for three years 

every day until now!' I make the effort to try and do it, as it makes sense that you may spot something untoward happening. My observations had all been fine - an odd statistic now and again - maybe a random temperature of 37, but no steady pattern; maybe an odd low lung function test, but I'm not the greatest with lung function technique. There were no observations throwing up alarming signals. 

I always have a cough. I have had the cough since my transplant. It stems from a join that healed in a funny way after my operation, it causes me to have to clear my lungs each day. I might have said a few times on our walks recently that I feel like I'm coughing a little more. But the air has gone suddenly colder, there'd been a cold wind blowing and I put it down to that, especially when I'd been walking along a chilly seafront early in the morning during normal prime coughing time. I can't really put a finger on it that it was getting worse, perhaps just marginally worse as I've felt the need to mention it. And then I recall mentioning an odd moment of breathlessness once of twice - clambering up a steep slope from the sea front to the flat terraces above with the wind in my face. Rob says, 'I'm breathless too, if you hadn't stopped for a minute, then I would have.' Similar thing again, this time being pulled up a hill by Ted at Wimpole Hall two days before my clinic appointment. I'd stopped and caught my breath, there had been a cold wind and I was a bit tired after already walking a good few miles. Nothing to worry, I put it out of my mind as soon as it's flashed into it. 

After Wimpole I'd woken in the night. I've kept having a tingle in my neck that shoots up my face to my ear. This night it's aching and feels stronger than normal, like my glands are swollen up or something. It's been doing this on and off from summer, but this feels more. Much worse. 'Should I phone for an appointment with the GP?' 'Will they just stand and stare at me?' 'What's is she on about - a tingle in her face?' 'Should I phone clinic?' 'But I'm there in another day,' I think. I decided to wait and see, if it becomes worse I'd phone the GP in the morning. It disappears and goes. Worry sorted. I might mention it at clinic, but then I don't want to seem like I'm a paronoid, self-obsessed transplant patient, so I might not. 

I jump in the car to go to clinic. I feel strong and well. Healthy. I say to Rob, 'I don't think I'll mention any of these silly little things. What will they say? Well they'll probably laugh at the tingling face bit; they'll probably say of course you might be a bit breathless climbing up steep hills with a dog pulling you on the lead with a cold wind on your face!' 

I've already mentioned to them about feeling fatigued and having to pace myself more, we've already discussed this at clinics and put it down to all the problems and ups and down that have gone on during the year following the serious attack of CMV virus at Christmas and all the medication tweaks that have come with it over the year. That's been an ongoing and continuous issue to keep the CMV virus at bay and having to pace myself and becoming fatigued was part of the course.

I felt breathless walking up the incline leading to clinic with a friend. Arriving in clinic I needed to sit down straight away. I'd no breath left. I sit there and think, 'Did that really happen? Did I actually lose my breath? Did I have no control on my breathing?' I recovered myself, sat and chatted with friends, had my tests done, felt fine. I think to myself, 'I'll just mention all things niggling things after all. I don't like what happened earlier.'

I see the consultant and we chat about how I am - I'm a bit reserved at first about the mismatch of what feels like 'nothings' going on. He turns and points to my lung X- ray. They are there, as always, my new lungs staring down from the computer screen and he tells me he's noticed some marked differences since my last X-ray. Things he's concerned about. I rattle off all those little nuances and nothings. I listen in surprise as I'm told I'm being admitted into hospital immediately and tests will be done straight away. It's all a shock - the swiftness of how this is all moving. 

Five days and numerous tests later, I return home a completely different person. I'd climbed in the car on Thursday morning thinking of myself as fighting fit and perhaps needing a little reassurance like all us transplant patients do when we visit clinic. I returned back home with the dreaded diagnosis of that 'R' word hanging over me. Rejection in my beautiful borrowed lungs. The 'R' word. And not only that, but there's another problem diagnosed in both lungs too, which needs specific treatment. Basically I'm not what I thought. 

So here I am home and we're catching our breath as a family. It's a wait and see the response to new medications. It's a wait and see the response to generously increased medications. It's a wait and see response to high doses of steroids. While I wait, I go back to those old tricks of recovery - things I do each day really anyway, but during times like this they come with a new intensity. They're tried and tested and with a fair wind can help work alongside the medications. Rest. Recuperation. Good diet. Excercise - well at least moving around within my limits as they are at the moment. Keeping the mind busy. Focus and resolve to fight, survive and overcome.  

Most of all, it's small positive steps, just one at a time each day to face whatever is happening on that particular day, be it for better or worse. It's better to tackle those than worry about the 'what ifs' that may never happen anyway. Just little steps. Little daily tasks. Small and positive accomplishments as we wait and see what unfolds next. 

The big positive at the moment is there is still hope - much of it - that things may be better again, even though I've been advised it's all going to take time whichever way things swing - they still might be better again if I am lucky.

What a difference a day makes. Fortunately for me it was a day when I just happened to be in the right place at the right time and fortunately the problems as they are forseen are being treated for now. I'm thankful for that, hopeful for this coming year still and always grateful for the extra wonderful years and time that I've been blessed with already.