Emphasis Spring 2022

SPRING 2022

MAKING MENOPAUSE MATTER

Advice and real experiences

NEWES

FEATUR FOR

2022

A special kind of support Spotlight on overcoming hospital chaplains anxiety Plus...

The National Audit of PH, real life stories, research news, expert interviews, and much more…

Our new self-help programme for people with pulmonary hypertension

Hello I’m very happy to welcome you to our first magazine of 2022. With spring starting to arrive, I hope this message finds you feeling optimistic about the lighter, brighter months ahead.

W

e’ve got a real mix of content in this issue, which is packed once again with real experiences, news, advice, and expert interviews.

As a PH community we’re privileged to benefit from such a skilled team of healthcare professionals working across the UK’s network of specialist centres, and it’s our pleasure to go ‘behind the name badges’ to introduce you to some of them in this issue.

On the subject of specialist centres, the findings of the most recent National Audit of Pulmonary Hypertension show they are continuing to meet the high standards set by the NHS – despite the challenges of the last couple of years. You can read our digest of the NHS report on page 57, which provides further reassurance that you are in safe hands at your centre.

As it’s a new year, we’ve given some regular Emphasis features a makeover and introduced a few new ones too. Another change for 2022 is a difference in the frequency we’ll be delivering this magazine.

It’s important to us that every issue is the best it can possibly be, as we know how much you value these pages.

With a small (but dynamic!) team, and many different projects on the go to help support the PH community, we have made the decision to publish three times a year instead of four.

This means your summer issue will land in July, and a combined autumn/winter edition will be published in November. In the meantime, we’ll continue to keep in touch via our monthly e-newsletters, so do make sure you’re signed up to receive them (see page 63 for details).

We look forward to bringing you another bumper issue of Emphasis soon!

Iain Armstrong Chair of the PHA UK media@phauk.org

IT’S IMPORTANT TO US THAT EVERY ISSUE IS THE BEST IT CAN POSSIBLY BE

If you have any feedback about this magazine, or ideas for future features, email media@phauk.org

SPRING 2022 emphasis 3

35

“With everything that’s happened, I’ve realised that the people around me are what really matters” Emma Sangar, living with CTEPH

22

A SPECIAL KIND OF SUPPORT Highlighting the important work of hospital chaplains

CONTENTS SPRING 2022 Emphasis magazine is produced by the PHA UK. This magazine is intended only to provide information and not medical advice on individual health matters. The PHA UK will not be responsible for readers’ actions taken as a result of their interpretation of this magazine. We encourage readers to always discuss their health with their doctors and medical team. Registered Charity Number: 1120756. © PHA UK.

This magazine is printed on paper from sustainably managed sources

27

LIVING BETTER WITH PH

The study aiming to test whether at-home supervised exercise can help people with pulmonary hypertension

Living with PH...

08

The pooped patient

20

My personal path with PH

30 43 44

Diane Kaylor on life with PH-associated fatigue Roy Dewar looks back on a year since diagnosis

From Canada with love

Hear from Tia, who lives with PH on the other side of the Atlantic

Over to you

Your tips on bathing and showering with PH

The peaks and troughs of exercising with PH Emma Ferguson shares her experiences

10

MAKING MENOPAUSE MATTER

52

It affects 50% of the population, and we need to talk about it more…

HELLO, MY NAME IS…

Meet Consultant Cardiologist Dr Dan Knight

57

THE NATIONAL AUDIT OF PH

New report: How specialist centres have fared against standards set by the NHS

Regular features...

The best of the rest…

06

Upfront

19

On the spot

28

Ask the experts

22

Overcoming worry and anxiety

Green Leaf Crew

34

32 39 51

News, views and what you’ve been saying on social media Your questions answered by professionals Word search fun and the latest column from Emily in our special pull-out for children

Kaylee’s column

Regular contributor Kaylee Mynot on transitioning to adult services

Fundraising Focus

The wonderful ways you’ve been supporting us over the last few months

40 46

Get to know Medicines Management Technician Mark Perry-Young Our new self-help programme for people with PH

Making the most of remote appointments

How to access our bespoke appointment planning form

Research spotlight

Dr Chinthaka Samaranayake on his prize-winning PH study

21 years of hope, influence and integrity

A look back at our charity’s story so far

SPRING 2022 emphasis 5

UPFRONT News, views, and what you’ve been saying online Green light for groundbreaking study A PHA UK-supported study using remote monitoring technology to help patients get the best results from their PH medication has received the go-ahead from funders.

The Phoenix study will use small implants to provide daily data similar to that gained from a right heart catheterisation. This data will be provided wirelessly to the hospital team via mobile phone, with the aim of ‘personalising’ medicine for the best outcomes for individuals.

Last year, the PHA UK conducted a survey amongst its members to ascertain whether there would be support for the cutting-edge study from the PH community.

The results, which can be viewed on our website, were overwhelmingly positive.

These findings were used as part of a funding bid for the study, which will begin with the first patient around September 2022. Patients from all the PH centres around the UK will have the opportunity to take part.

Driving with pulmonary hypertension

DVLA A guide to notifying the ion dit con lth of your hea

SPRING 2022 emphasis 6

Notifying the DVLA of your pulmonary hypertension A new guide to telling the Driver and Vehicle Licensing Association (DVLA) of your pulmonary hypertension diagnosis is now available to order for free.

Since 2018, people with PH have been required to notify the DVLA of their health condition.

At the PHA UK we have worked hard in collaboration with the organisation to ensure the process is simple and fair – doing the right thing by people with PH whilst also ensuring safety for all road users.

The aim of this publication is to

guide you through the process of notifying the DVLA of your PH, answer some of the common questions we hear, and help you understand what to expect along the way.

Order now at www.phauk.org or request a copy by calling 01709 761450. We have also made this publication available to all specialist centres, so feel free to ask for one at your next appointment.

MY R EV I EW

>Top Tweets

PH consultant @mark_toshner was keen to celebrate our nation’s network of specialist centres on Twitter. Reacting to the results of the National Audit of Pulmonary Hypertension, he wrote: “Specialist PH care in the UK is excellent…

Despite the pandemic, the national audit shows the remarkable resilience of our UK specialist system. Looking forward to 2022 I think we have lots of reasons to be proud and more optimistic about the future.”

Leap Fitness

PHA UK member Emma Ferguson reviews the Leap Fitness Group apps, a series of free online tools aimed at helping you fulfil your health and lifestyle needs.

Read about the National Audit of PH on page 57

Image : Ro ya l Papw it orth Hosp al

@RoyalPapworth shared this magazine’s interview with Consultant Surgeon Mr Steven Tsui with its 10,000 followers: “Being kind is one of the highest things you can do as a human.” Consultant surgeon Mr Steven Tsui talks to @PHA_UK about life as a surgeon, our world-renowned pulmonary endarterectomy service & how he was inspired by Papworth at school in 1979.”

Find the interview in our Winter 2021 issue, available to read online at www.phauk.org

Nurse Robert Campbell @rjcampb shared a message for the specialist PH centre in Glasgow: “Cannot thank the Scottish Pulmonary Vascular Unit enough for the amazing care of my sister.

So grateful ” Student Alex Ainscough @AinscoughAlex was part of a PHA UK-supported research event at the back end of last year:“Awesome to present my PhD work at the National PH Forum… Very grateful for the opportunity . Amazing venue, food and company ”

Follow us on Twitter at @PHA _ UK

"It was during the pandemic that I began using the ‘Leap Fitness’ apps. If you haven’t heard of them, I would really recommend checking out the variety wof options they have on your phone’s app store.

I downloaded apps for stretching, home workouts without equipment, full body workouts and many more. Each app offers combinations of exercises chosen to focus on a specific element of fitness like arms or legs, or flexibility or strength.

You then get a combination of about 20 exercises to complete, all with additional info about how to correctly assume the positions named. You can skip exercises that you dislike and swap them for others, or pause mid-workout to regain your breath.

One of my favourite elements is that no matter how many times I pause, skip or otherwise divert from the pre-planned session, you are always congratulated at the end. ‘Good Job!’, the app will tell you, ‘Well Done!’

If I was to offer one criticism of the apps it would be that a lot (but not all) of the exercise routines are part of a larger seven or 30-day collection of exercises made to build up a habit. This is great for most people, but I find it demoralising that I have only ever completed one of these, as ill health or exhaustion always gets in the way. My PH is unpredictable enough that regular exercise planning can feel impossible."

Leap Fitness Group apps are available on both Apple and Android devices. Choose from step trackers, stretch exercises, home workouts, fitness challenges and more. Always speak to your PH team before embarking on a new exercise routine.

Emma writes more about her experiences of exercising with PH on page 44.

Have you used a health-related app, read a book, or used a programme or website that you want to tell us about? Send us your review and you could see it in the next issue of this magazine! Email media@phauk.org SPRING 2022 emphasis 7

Diane Kaylor

The pooped

patient Diane Kaylor lives with pulmonary arterial hypertension, along with multiple autoimmune diseases, and finds writing helps her to cope with the challenges of her health conditions.

In the second of a series of musings for Emphasis, she reflects on life with the challenges of fatigue – and how sometimes, just sometimes, there are unexpected benefits to be found...

SPRING 2022 emphasis 8

Diane lives with her husband and an IV line in Derbyshire and takes oxygen 24 hours a day.

“Writing about how PAH affects my life gives me a different perspective. Rather than being totally immersed in my illness, if I reflect a bit more on what’s going on, I seem to gain a deeper or at least different understanding, and that helps me cope with it all a bit better.”

What I want to know is, who superglued my bum to this armchair?

That’s how it’s felt for more months than I care to remember. It’s not that I don’t want to move. I may be really thirsty, and I can hear the fresh orange juice in the fridge calling to me, but someone’s pulled my plug out and it’s just not happening.

It’s such a weird feeling. I say to myself ‘right, I’ll count to ten and then move’, and ten comes and goes as does another ten. And I just sit and stare into space wondering what it will actually take for me to stand up and just put one foot in front of the other.

Everything takes so long these days and it’s definitely not got anything to do with pacing – it’s more like grinding to a halt.

I’ve inherited one of those riser/recliner chairs from my dear old dad and it also tips you forward, but even that doesn’t do the job in terms of getting me to my feet. I just perch on the edge of the chair for a bit of a change of scenery and then return to recline mode. I wonder, can you get an ejector seat – or maybe I just need to be fired out of a cannon?

There are of course consequences to this immobility, including pressure sores and atrophied muscle. I once had the supposedly affectionate nickname of ‘thunder thighs’ but my legs now look like Pearl & Dean drapes that are badly in need of ironing.

Then there’s muscle weakness. If my toilet seat gets any higher, my head will be touching the ceiling and I’ll have to parachute off it – but let’s face it, without it I was in serious danger of pulling the bathroom sink off the wall.

Have I mentioned that my eyelashes feel like they’re strung

with miniature kettlebells forcing them closed at any given moment, and I find myself waking up some time later without any recollection of having decided to sleep? Then there’s lack of balance (my walk is now a stagger) and in recent weeks, oedema – which I really don’t recommend. It makes it harder still to walk when your thighs feel like they’re sloshing around as if they’ve had hot water bottle implants.

The vicious circle just keeps on turning.

I do know that I need to keep going and that muscle tone and gentle exercise would aid my breathing, and I’m trying, honestly, I really am. The spirit is willing, but the flesh is oh so weak. It’s like my mind’s going ‘c’mon you can do this’ and my body’s like ‘forget it!’ Ironically enough, this article very nearly didn’t get done because I’ve just been feeling too exhausted to write about fatigue!

I was wondering the other day why I enjoy being in bed so much, which incidentally is now downstairs. Although my husband Karl makes a really good human stairlift, I was getting to dread the effort required to make it up the stairs – or as I call them, K2.

I still go to bed around 8pm and rarely surface before 9am, and then only reluctantly. It’s not that I sleep for all that time, and in fact I went through a dispiriting period of insomnia – which as you can imagine, didn’t help at all with the fatigue.

But I’ve realised that I absolutely love my bed because it’s a place not only where I feel safe, but I also feel like a normal human being. When I wake up in the morning and I’m warm and comfortable and my breathing is easy, I can pretend I’m just like everyone else. It’s only when I force

*Joy of Missing Out versus Fear of Missing Out

myself out of this delightful zone and start having to undertake mammoth tasks, like walking to the bathroom and getting dressed, that I’m reminded what bloody hard work living is.

I had a go at Karl the other day (nothing new there). It went along the lines of: ‘I really don’t begrudge you getting out and about and doing things with your mates, but how about we do something together? Why don’t you ever suggest we have a day out, or lunch out, or blah, blah, blah?’

Very soon after this conversation, and even discounting pandemic considerations, I realised that if Karl were to suggest whisking me out for a day at the seaside or a pub lunch, I’d more likely than not say ‘nah, it’s too much like hard work. I’ll give it a miss.’

Because sometimes it is more trouble than it’s worth. I’m more likely to get sucked into JOMO* as opposed to FOMO*.

And you know, for me there is some joy to be had in missing out.

Those interminable weddings (usually a second cousin who you see every five years and barely recognise) can be turned down with a perfect and mostly truthful excuse. I’m not well enough to put myself through that particular purgatory / your special day. So, I find my perks where I can.

Anyway, I’m going to end really abruptly at this point, as surprise surprise, I’ve run out of steam.

PS. Karl would like it duly noted that he too is exhausted. I do know that I’m high maintenance and inadvertently run him ragged.

Sorry Karl!

SPRING 2022 emphasis 9

menopause MAKING

MATTER

It’s something that 50% of the population will experience, but it isn’t always talked about enough. We take a closer look at menopause, and how it affects women with pulmonary hypertension.

SPRING 2022 emphasis 10

I

n simple terms, the menopause is when a woman stops having periods and is no longer able to get pregnant naturally. The process itself though can be complex, and just like pulmonary hypertension, it can affect people very differently.

A natural part of ageing, the menopause (sometimes referred to as ‘the change’) normally starts when a woman is aged between around 45 and 55 – but this can vary either side.

It is caused by a change in the balance of the body’s sex hormones, when your ovaries stop producing as much oestrogen and stop releasing an egg each month.

Symptoms can begin a long time (even years) before your periods stop, and this is known as the ‘perimenopause’. They then continue, on average, for around four years – although some women experience them for much longer.

If you’re reading this whilst going through the menopause, you probably don’t need to be told that symptoms can have a significant impact on your life. Women commonly experience hot flushes, night sweats, difficult sleeping, vaginal dryness, low mood or anxiety, reduced libido, heart palpitations, and problems with memory or concentration.

You might already be experiencing some of these symptoms because of your PH, which can make it harder to identify if you are starting to enter menopause – but a simple blood test, available with your GP, can provide the answer.

Dr Karen Selby is a Consultant Obstetrician and Gynaecologist who works closely with the specialist PH centre in Sheffield. She said: “The early signs of menopause can be very variable.

Some people may have no signs at all.

But things that may have been just put

What’s in a number?

The average age for a woman to reach the menopause is 51, but around 1% of women experience it before the age of 40. If you are experiencing menopausal symptoms under the age of 45, please see your GP, who will be able to run a blood test to confirm your hormone levels.

Source: nhs.uk

down to age before – such as loss of concentration, forgetfulness, and trouble sleeping - are now increasingly being recognised as perhaps being signs of early menopause.

“I think this increase in recognition and understanding is making it easier for people to access support but there is still a bit of stigma around it all, and a sense of ‘just getting on with it’.”

This stigma can be even stronger in certain communities. Wellbeing coach Meera Bhogal (pictured) runs guided menopause programmes and is passionate about raising awareness of menopause within South Asian communities. She said: “Menopause is seen as a ‘western disease’ and there is a real taboo around women’s health in general.

So many women don’t even know what menopause is, what the symptoms are, or how to get help.”

Meera believes this ‘mystery’ surrounding menopause is an issue across society in general, and that because some women feel unable to openly discuss the changes they are going through – and the impact on their lives – it makes it even more scary.

Despite these challenges, Dr Selby is keen to stress that if you are struggling with menopause, you shouldn’t suffer in silence.

“With a lot of gynaecological symptoms that women have, whether it’s menopause, incontinence, or heavy periods, they are still seen by many as ‘just part of being a woman’”, she added. “It’s only recently that women are starting to think ‘hang on a minute, why should l put up with this?’ The help is there; as women we just need to ask for it.”

Managing menopause Symptoms of menopause can be managed in several ways, including: Hormone replacement therapy (HRT): There are many different types of HRT. Most women take a combination of the hormones oestrogen and progestogen.

Interaction between oestrogen and PH is not fully understood, and it is not suitable for those with certain types of PH. Consultant Pharmacist Neil Hamilton explains more on page 14.

Oestrogen creams, lubricants or moisturisers can all help with vaginal dryness (which can also be a cause of painful sex during menopause).

Cognitive behavioural therapy (CBT): This is a type of talking therapy that can be helpful in managing low mood and anxiety.

Lifestyle modifications: Regular exercise (within your capabilities) and eating a healthy balanced diet can improve some menopausal symptoms.

If you are struggling with menopausal symptoms, do not be afraid to ask for help.

When things are heating up…

Hot flushes and night sweats can be frustrating and make some PH symptoms feel worse. www.nhs.uk recommends the following measures to cool down: • Wear light clothing • Keep your bedroom cool at night • Take a cool shower, use a fan or have a cold drink • Try to reduce your stress levels • Avoid potential triggers, such as spicy food, caffeine, smoking and alcohol

SPRING 2022 emphasis 11

The menopause

and me...

Four PHA UK members share their personal experiences

Zoe* is 49 and was diagnosed with idiopathic PAH just over three years ago. She has recently gone through tests to confirm the start of perimenopause.

painful periods “Around 18 months ago, my already time that I star ted e sam became worse. This was at the med it was assu I so , taking selexipag [for my PH] linked to the onset was it ise medication related. I didn’t real of early menopause. point where I experienced This progressively worsened to the ths and the frequency blood clots for two consecutive mon r week. The pain was of my periods increased to ever y othe , whilst driving, I had to making me physically sick and once half an hour. pull over to lay on the back seat for my GP who confirmed I recently had my bloods tested by perimenopausal. I feel that the results indicate that I am and hopefully gain relieved as I can now move forward the appropr iate help. e yet another medical I do feel disappointed too as I hav ing on and achieving condition that will hinder me gett of PAH in the ce what I would like to. My experien understanding, and ort workplace is one of lack of supp medical ther ano so I would be reluctant to mention k. wor condition that may impact my the same age, we all As most of my close friends are of opause, and support share stories and advice about men each other. 't wait as long as I did My adv ice to others would be don ate Facebook groups to seek help. Talk to others on priv ifically for women with [see page 16 for details of one spec PAH]. Never suffer alone.” *Name has been changed on requ

SPRING 2022 emphasis 12

est

Gail, 55, has idiopathic pulmonary arterial hypertension and has been living with menopause symptoms for around three years.

“I am classed a perimenopausal as I still have a monthly cycle. My symptoms include night sweats, hot flushes, disturbed sleep, dry vagina, bladder weakness and a low sex drive. I also get very moody and snappy, which is an increase from my normal PMT [pre -menstrual tension] .

These symptoms have affected my relationship with my husband, and this became more app arent during the pandemic as we have been together 24/7.

I decided to speak with my PH team in 2021, and they supported me to talk through my options with my GP. He was happy to get me star ted on trea tment after the meds were approved by my PH team.

We opted for a pessary to help with the bladder weakness and dryness, and that has helped significantly. I was also prescribed a low dose antidepr essant, which works by increasing the levels of serotoni n (mood enhancing chemicals) in my brain. My GP adv ised that it has been successf ully used to reduce the psyc hological lows and moodiness linked with perimenopa use and menopause, and it has worked for me.

I am pleased with the results so far but have decided now to try HRT to reduce the symptom s further (night sweats, itchy skin, restless sleep, mood swin gs and so on.) I star t two HRT medications this month; all have been approved by my PH team and I will have my blood pressure monitored monthly by my GP. It’s hard to say whether the peri menopause itself has negatively impacted my PH sym ptoms as some of the symptoms, especially the fatigue , are the same for both.

I know some women sail through the menopause. The trouble for me was because I have IPAH and am on IV therapy, the added symptoms from the perimenopause were pushing me into a place whe re I could not be my normal positive and proactive self.

It was pulling me down and it was becoming too much, so reaching out to my GP has meant I have had the med ical help I needed. I am feeling much more positive about the whole thing now.

I feel this subject is very important and not taken seriously.

Many women do nothing about it when they suffer for many months and years.”

Jayne, 52, has pulmonar y ar terial hypertension. She started experiencing the symptoms of menopause towards the end of her for ties.

s were very similar “Some of the menopause symptom cts of my effe side the or s to either PH symptom es. dach hea and pain medication, like joint gs interacting, but I haven’t had any issues with dru logist were keen both my PH team and my gynaeco d an HRT regime to discuss interactions and foun that was safe.

of 2020. I have I have been on HRT since the middle twice a week Estradot patches which are changed has helped This . wed rene was and my Mirena coil h under muc ty pret are s ptom massively. The sym d swings moo the h oug alth , time control most of the y that my GP are still there sometimes. I was luck t. star the listened to me from and asked if I was I spoke to my specialist centre first e never had any able to have HRT in general as I hav I spoke to my blood clots. This was agreed and then gynaecologist at my female GP who referred me to the logist and my PH local hospital. Between the gynaeco ard.” team, they agreed a safe path forw

Sue*, 50, has PAH and connective tissue dise ase and is in the early stag es of menopause.

“I experience significan t night sweats, generally in the days prior to when menstruation is due. My cycle has become much more irregular, with per iods around three weeks ap art.

My main issues are fro m fluid retention/bloa ting around the stomach are a, which makes my che st area feel tight and uncom for table too. I feel extremely drained and emotionally unbalanced , which is causing me to get anxious, irritable an d upset – sometimes sud denly and for seeming ly no good reason.

It has all definitely had a significant effect on my physical health. As I am only at an early stage in the menopause proces s, I have not yet taken any

medication like HRT, bu t I have discussed this with both my PA H spe cialist and my scleroder ma specialist.

My scleroderma specia list has arranged for me to have blood tests in relati on to menopause issues for which I await feedb ack. I am trying to lose the weight I put on during the lockdowns as this sho uld hopef ully help with the bloating/fluid retention issues if I am carrying a bit less weight.

When I discussed meno pause issues with my specialists, I found the m quite helpful and willing. My PH consul tant said that taking HR T would not be a problem for me if this was felt necessar y going for wa rd, although I am hesita nt to take any more medic ation. I feel like I must rat tle already!

Don’t be afraid to speak to your medical team if something is a cause for concern for you, either physically or em otionally. “

*Name has been change d

on request

SPRING 2022 emphasis 13

Still sweating over HRT? Dr Neil Hamilton is a Consultant Pharmacist at the Sheffield Pulmonary Vascular Disease Unit.

Here, he unpicks some of the advice and evidence around HRT treatment for women with PH.

H

Hormone Replacement Therapy (HRT) has been the subject of much discussion, controversy, and column inches in the press. Whilst the benefits to menopausal and post-menopausal women are well known, the potential for side effects has also been well described.

The guideline summarises very nicely the evidence for prescribing HRT along with a clear explanation of the risks and benefits. As with all NICE guidance, this was reviewed more recently in 2019 to ensure their advice remains up to date.

The menopause will affect all women at some point in their lives.

Approximately 80% of women experience some level of symptoms and current evidence shows that for some women these symptoms may

SPRING 2022 emphasis 14

last as long as 10-12 years. For some, the impact on their quality of life is huge. The symptoms of menopause can obviously affect all aspects of home, family, work and sex life. Hence this is a very significant problem to tackle and one of the key messages of the guideline is for women to seek advice from their GP in the first instance and receive the most appropriate treatment.

For women with pulmonary hypertension, the situation is further complicated. HRT treatments are either a combination of oestrogen and progesterone, oestrogen alone or a medicine called tibolone (more commonly started by hospital specialists if other options are not suitable or have not worked).

Progesterone alone is not prescribed to treat menopausal symptoms, but it

is used for endometrial protection in women with a uterus (either as part of a 'combined' HRT, via Mirena-IUS or as a progestogen capsule).

In addition to the range of medication contents, there are also different ways that the HRT is presented. HRT tablets used to be the most prescribed form, but the skin patches and gels are now preferred for most patients.

The skin patches are easy to use; they are replaced every few days, slowly releasing medicine which is absorbed through the skin. Gels are increasingly popular as they are so convenient; simply rub the correct amount onto the skin every day.

It is worth bearing in mind that if any patient prescribed an oestrogenonly gel or patch has not either had a hysterectomy or has a Mirena coil in place, the doctor will also prescribe a progesterone tablet to reduce the risk of womb cancer.

The 2015 NICE guideline has answered some long-standing concerns and clearly described the pros and cons of treatment. However, as with other chronic conditions, the interaction between oestrogen and PH is not fully understood. Firstly, there are valid reasons why oestrogen may be beneficial, not least because it has a protective effect on the bones (it protects against osteoporosis). It can also provide relief from the flushes and psychological issues such as mood-swings.

However, oestrogens also have pro-thrombotic properties (i.e. they increase the risk of blood clots).

This will have a varying degree of importance depending on the type of PH that you have. For example, if you have Chronic Thromboembolic Pulmonary Hypertension (CTEPH), any increase in the chance of having further clots could be life-threatening.

As a result of the higher risk of clots, oral HRT is not recommended as a first choice anymore. Therefore, patients with PH would be offered the patches as a good option.

There is a lot of research work underway looking at all aspects of

oestrogen in PH. Such work includes looking at whether oestrogen may cause PH, whether high levels may be involved in making a diagnosis of PH more likely, and even whether treatment with oestrogen-like compounds could be beneficial. If the investigators are able to draw some useful conclusions from their work, the picture may be clearer.

However, at the moment the evidence for taking oestrogens in any form remains very conflicting.

The NICE guideline does a great job of raising the importance of appropriate management of menopausal symptoms. It goes a long way to clarifying and describing the long-term risks of taking HRT, which will allay some commonly held misconceptions.

The 2019 review brought this up to date by looking at more recent studies to see if the advice concerning risks of long-term complications such as heart disease, stroke, diabetes, osteoporosis,

different cancers and blood clots needed to be changed. Interestingly the best new evidence was often mixed; either showing that HRT did not increase the risk of long-term problems, or was shown to have the effects that were already described. As such, the review did not change the 2015 guidelines, so their recommendations remain in place.

In summary, HRT offers an effective treatment for menopausal symptoms.

HRT patches or gel do not appear to be associated with an increased risk of blood clots and may provide an attractive route of administration for patients with PH. If you think you are suffering from symptoms which may be related to the menopause then it would be sensible to discuss with your GP, who can fully assess whether HRT is the correct treatment for you. Your specialist centre will be happy to give your GP advice if needed.

Over-the-counter HRT

"A type of HRT to treat vaginal dryness could soon be available from pharmacies. The UK medicines regulator, the MHRA, is proposing to reclassify vaginal oestrogen tablets called Gina10 (estradiol) to make them available as a pharmacy medicine. This means you will be able to buy a supply without a prescription from your doctor after discussion with a pharmacist. The consultation closed on 23rd February, and as this magazine went to print, an outcome was yet to be announced. Making HRT easier to access is great news for the general population, but I would urge women with pulmonary hypertension to ensure that whoever they discuss this treatment with is aware of their other health conditions, for the reasons outlined in this article."

SPRING 2022 emphasis 15

e ’r u o y f i t r o p p u s e Onlin e s u a p o n e m h g u o r h going t provide information The following websites y for women affected and resources specificall by menopause:

arity.org www.themenopausech t.co.uk www.menopausesuppor www.daisynetwork.org opause) (dedicated to early men rs.co.uk www.menopausematte

en Chat with other wom who understand

other women You can talk directly to ted by menopause in a with PH who are affec which now has over private Facebook group d search ‘UK Women 160 members. Log on an ial Hypertension’.

With Pulmonary Arter ndently of the was set up indepe Please note, this group ated or managed by us.

PHA UK and is not moder

Recommended follow

McCall talks very TV personality Davina riences of menopause, honestly about her expe sed awareness and and campaigns for increa l tically acclaimed Channe understanding. Her cri d an McCall: Sex, Myths 4 programme, Davina nd able to watch on dema the Menopause is avail w. no t enopausing’ is ou online, and her book, ‘M ook @davinamccall, Follow Davina on Faceb ccall, and Twitter Instagram @davinalpm @thisisdavina

W hen someone you care for is experiencing menopause…

If someone you kn ow or love is goin g through perimenopause or menopause, you may not be sure how best to suppor t them.

We asked people with PH for their advice :

“Being supportiv e and offering a lis tening ear is the best thin g you can do as a relative or friend. partner, It’s important to realise that going through th e menopause is a significant change, both phys ically and emotio nally, which can make an indi vidual feel very di fferent about themselves.”

“Do not dismiss or belittle what they are going through. PH ca n be very demoral and additional iss ising ues can really push you, so help them to seek supp ort – especially if they don’t get it straight away.”

“It isn't always po ssible to relate to somebody else's sit uation and how th so we all need to ey feel, make an extra ef fort to try to be understanding. M aking it easy for so mebody to be open about what they are experienc in g will help immensely. If som ebody can share w he n they are feeling awful, this will help all round as it will also explain their beha viour too at that time.”

Do you want to share your experiences of menopause with PH ?

s to We’re planning supportive resource al son per and help people living with PH ers. oth experiences are very helpful for you Please email media @phauk.org if . lved would like to get invo

SPRING 2022 emphasis 16

We’ve taken this magazine to the Inspired by these pages, EmPHAsis on Air is our new podcast bringing you chat, advice, interviews and analysis with the PHA UK team.

It’s available on Spotify, Apple Podcasts, Amazon Music, Audible and more – where you can listen to previous episodes too. Plus, you’ll always find the latest episode on our website.

Don’t forget to subscribe!

Got some feedback, or an idea for future episodes? Contact the team at office@phauk.org

des episeor ev gyht !

SPRING 2022 emphasis 17 fortni

PHA UK website rated best in the world for information A global study has named the PHA UK website the best quality PH association site in the world for information about pulmonary arterial hypertension.

T

SPRING 2022 emphasis 18

Dr Weatherald, Assistant Professor at the Libin Cardiovascular Institute and University of Calgary in Canada, now directs his patients to www.phauk.org alongside the country’s own PH association website.

“I was very impressed with how well the quality-of-life aspects were covered by the PHA UK website; for example, the sections on intimacy and relationships, caregiver support, and extra resources being mailed to patients at no cost.

The other really strong aspect was the emphasis on shared decision-making, which was lacking on other websites.

This includes encouraging patients to take an active role in managing their care, but also presenting them with accurate and evidence-based information that can be helpful when they are discussing their points or concerns with healthcare providers. The website scored really highly on these things. I was very impressed with how comprehensive the website’s focus was on quality of life and all the non-medical aspects as well.”

Dr Weatherald

Dr Saleh

itled ‘Quality of online information on pulmonary arterial hypertension’, the study was led by researchers in Canada.

It analysed 117 unique English-language websites about PAH, including those from healthcare providers, pharmaceutical companies, and other patient organisations. www.phauk.org came out second overall, beating the NHS website, the USA’s Mayo Clinic website, Medical News Today, and more. It was the highest scoring website from a pulmonary hypertension association.

The study scored websites using two widely-used assessment tools.

The JAMA benchmarks, which focus on transparency and lack of bias in online health information, are a series of four criteria established by the Journal of the American Medical Association.

The DISCERN instrument analyses whether consumer information is reliable and trustworthy, providing users with a valid and reliable way of assessing the quality of written information on treatment choices.

The research was led by Canadian PH physicians Dr Dana Saleh and Dr Jason Weatherald, and the findings were presented at a major European medical conference. They were also published in respected medical journal ‘The Annals of the American Thoracic Society’.

Dr Saleh, a Respirology Resident at the University of Calgary, said:

He said: “As I looked at all these websites, phauk.org really stood out as one of the most thorough and comprehensive among all societies.

It scored very highly for both quality and content, with a nice balance of both elements – reliable, unbiased information, and thoroughness of information too.”

The team behind the study are now planning further research into online information about pulmonary hypertension.

.

“The PHA UK website is fully deserving of this recognition. It is a valuable, trusted source of information for our patients, and I regularly direct them to both that and their lifestyle website during my consultations. It has also been a vital source of trusted information and advice, for both patients and PH professionals, through the COVID-19 pandemic.”

Carl Harries, Lead Clinical Nurse in the Royal Brompton Hospital’s PH service

e first of our th to e m o lc e W here we get to new series, w ut bit more abo le tt li a w o n k in o works with h w e n o e m so alist PH the UK’s speci rk. centre netwo

Behind th

job titlee

On the spot This issue, meet Mark Perry-Young, the new(ish!) Medicines Management Technician at the Sheffield Pulmonary Vascular Disease Unit.

What’s the first thing you do when you get to work?

Fill up my water bottle, check in with the rest of the pharmacy team and plan my jobs for the day.

What do you enjoy most about your job?

I really enjoy being part of the wider ward team. The role is already providing me with lots of great learning opportunities.

Tea or coffee?

Decaf coffee with milk and one sugar.

Which snacks fuel your working day?

Anything and everything. If it's offered, I will probably indulge!

Who or what inspires you at work?

The level of dedication and expertise I have already seen from the Sheffield Pulmonary Vascular Disease Unit team.

What’s on your desk?

An Incredible Hulk toy, a soft pink polka dot dragon and a scary clown head (all not mine).

be put e a PH professional to If you’d like to nominat n even ia@phauk.org. You ca on the spot, email med ;-) suggest some questions

Can you sum up your job in a nutshell?

Very very briefly, I help assist with the clinical, cost-effective and safe use of medications.

What do you enjoy outside of work?

Running, music, art, good food and travelling.

SPRING 2022 emphasis 19

My personal path with PH

Roy Dewar was diagnosed with idiopathic pulmonary arterial hypertension in January 2020 and has spent the last year adjusting to life with the condition. Here, the 77-year-old Scot looks back on an ‘interesting’ 12 months – and explains how he stays positive in the face of change.

I

was always in quite good health before the PH, aside from a couple of illnesses I picked up whilst travelling around the world for work.

Around three years ago though, I started noticing breathlessness.

I used to enjoy walking a lot, but suddenly even walking to the bus would leave me out of breath.

I was initially diagnosed with atrial fibrillation [a heart condition that causes an irregular and often abnormally fast heart rate] but after the breathlessness got worse, I had an echocardiogram at a hospital in Edinburgh. It showed that my heart was slightly enlarged, but there wasn’t cause for concern.

In October 2019, my symptoms took a turn for the worse and I blacked out whilst walking up the steps at a train station. The fall caused me to lose three teeth and I broke every bone in my face.

SPRING 2022 emphasis 20

That’s when I started pushing for more tests, but even after being referred to a consultant cardiologist, I was told nothing was wrong.

“Being diagnosed was a relief” It was only in September of 2020, when I saw another cardiologist, that pulmonary hypertension was mentioned as a possibility.

I was referred to the Scottish Pulmonary Vascular Unit at the Golden Jubilee Hospital down in Glasgow, where the consultant confirmed that it looked like I had PH and referred me for further tests to finally confirm it.

The very next day, back in my hometown of Cupar in Fife, I had another fall. This was again as a result of a blackout but it did not do any damage this time.

After a series of tests, I was officially

diagnosed with idiopathic pulmonary hypertension - something I had never heard of - in January 2021.

To be honest, being diagnosed was a relief to me, because I finally knew what was wrong.

During the course of my PH diagnosis, doctors also discovered a lesion in my lower left lung, and this continues to be monitored. It doesn’t seem to be growing, which is good news, but of course it has been an additional worry.

Treatment for my PH started the day I got my diagnosis. I’m on Sildenafil and Ambrisentan, plus 16 hours of oxygen a day. I split the oxygen into eight hours during the day and eight hours at night, and I take mobile canisters with me in a rucksack if I’m going out. I’ve got used to the oxygen quickly and it hasn’t been nearly as bad as I expected. It seems to work well.

Roy and Ann on their wedding day I am a big football and rugby supporter, and I had a season ticket for Rangers. I used to go to every game, but I haven't been able to do that now unfortunately. I’m still a huge sports fan, but I just watch it on TV now instead.

I don’t attend big events anymore, and that’s more because of the PH than covid worries, but I still try to go out for lunch and I have felt better in the last six months. Now when I get up, instead of feeling exhausted, I am able to walk for 25 minutes every morning – mainly around the house. My six-minute walk test results are getting better as a result.

“I have felt better in the last six months”

“Confidence is still a bit of an issue”

My attitude to all of this has been to stay positive because there’s not a lot else I can do about it. I just try and do what I can and get on with things.

The blackouts I had before diagnosis affected my confidence, and even though I am still ‘allowed’ to drive, I have chosen not to. My wife Ann does the driving instead.

The confidence issue has been a big thing as I do worry that I’m going to fall again. In the last couple of months, I’ve been on a bus twice and a train twice, and most of the time my wife has been with me – although I did do one trip on my own.

My wife has been a great support and I rely on her a lot. I’ve got also got three children and seven grandchildren, and they all help me stay positive. I try to see them as much as possible.

I have a big family, and lots of good friends across the UK, who I keep in touch with and see as much as I can. I try to do as much as I am able to, and I think the most important thing is being positive.

I read a lot of posts on the PHA UK Facebook group and some people are a lot worse than me, so I guess I consider myself to be quite lucky.

I try and do as much as I can for myself, but I have to be careful I don’t do too

much. I’m getting to the stage now where I do feel I want to go out and do things, with the support of my wife, but confidence is still a bit of an issue.

It’s been an interesting 12 months.

Weird, not wonderful – but ultimately not that bad either.

Roy and his wife Ann during his banking days in Hong Kong

Roy’s advice for other people newly diagnosed with PH

Roy says his grandchildren help keep him positive

“Don’t Google it! Get proper information and advice from your specialist team. Accept the fact that maybe you can’t do what you used to do, but don’t think it’s closing your life down completely.

It’s about adapting. Do what you can, without overdoing it. Ultimately, don’t let PH rule your life.”

SPRING 2022 emphasis 21

ARE YOU STRUGGLING WITH ANXIETY? This four-week self-help programme consists of a series of workbooks based on Cognitive Behavoiural Therapy (CBT), to help you take control of problematic worry and anxiety. ✓ Developed with a team of clinical psychologists ✓ Tried and tested by people with PH ✓ Complete in your own time at home ✓ Totally free of charge 100% of people with PH who tested the programme said it helped with their anxiety.

“When I received the booklets, I was determined to get the best out of them.

I cannot express my gratitude enough.

The clear explanations and easy-to-use strategies to cope with anxiety have been life-changing.” PHA UK member

Request the programme at www.phauk.org or call 01709 761450 SPRING 2022 emphasis 20

NEW

self-help programm e from the PHA UK

Behind the

job title

A spec al kindof support

Amongst the doctors, nurses and other medical staff at hospitals around the UK, a unique group of professionals spend their time supporting people in an important - but often misunderstood - way.

Mary Ferguson spoke to hospital chaplain Fran Kissack to find out more about how she and her colleagues care for patients and their families.

SPRING 2022 emphasis 23

"Sometimes people just need to talk, so we help them to process their thoughts and feelings."

F

ran has been a chaplain for nearly 20 years and has spent the last five at the Royal Hallamshire Hospital in Sheffield, where she supports the specialist pulmonary hypertension unit alongside other departments.

Hospital chaplains are salaried NHS employees. Their role is to provide emotional and spiritual support, or simply a listening ear, to patients, their relatives and friends, and hospital staff too.

This support is for anyone, whether they have a faith or not. “It is a common misconception that we are only there for religious people”, says Fran. “We are there for everybody, and we try to support them in whatever way they need us to.

Sometimes people just need to talk, so we help them to process their thoughts and feelings.

“Some people do want to pray with us, but very many of those we support don’t have a faith.

With chaplaincy, it’s about holistic care. When you’re in hospital, your wellbeing needs to be supported as well as your physical health needs.”

Fran is also keen to stress that the role of a chaplain is to support someone in the way that they need, and that it’s not about imposing views upon them.

“What we think isn’t important, it is all about what they think is important. And the support is more about listening than talking; we are not there to be trained counsellors and deliver therapy.”

This type of support is known as ‘active listening’ and it’s something that chaplains are well-placed to do, especially when busy hospitals may limit the time of others.

Anther common misconception is that chaplains are there only to support people nearing the end of life, but Fran insists this simply isn’t the case.

“I understand that some of the early NHS hospital chaplains were given a list of everyone who was dying, and their job was to go round and see them. Our role has changed a lot in the last 70 years, but unfortunately, some people still think that is what we do. Often, they think our role is to deliver bad news, but that’s not our job. We are there to help and support people in whatever way they need us to.”

For some patients, this help simply involves having access to an independent and impartial ear – and the chaplaincy service gives them the opportunity to discuss things they feel unable to with family and friends.

“You might not want to talk to your close relatives about how

SPRING 2022 emphasis 24

you really feel because it might upset them, and vice-versa”, says Fran. “This can create a conspiracy of silence about what’s happening, but as chaplains we can support both parties to be able to talk to each other.”

For other people, a chaplain’s value may lie in providing a guiding light during spiritual distress.

“Our strapline in Sheffield is ‘presence, understanding, hope’. It may be that people want to talk about their hopes and fears, or how what’s happening to them fits with their worldview. We are there to support them if they have a sense of not knowing what’s happening to them, and where they are going. You could sum it up as a ‘search for meaning’ but it’s so much more than that.”

All chaplains dress differently, but Fran chooses to wear a polo shirt – and she believes it helps that as a chaplain you are an alternative face in a medical environment.

“I don’t know the answers to the medical questions, and I don’t pretend to know”, she says. “We are different from medical care, and that’s important.”

Patients can choose to see a chaplain as often as they like during their stay, and Fran never gets offended if that is only once – or perhaps not at all.

“It is entirely your choice whether you see myself or my colleagues. You can say ‘not today’ or ‘never again’, although some patients do see us regularly. If they are religious, we will support their religion, for instance by saying prayers with them.”

Whilst end of life support is just a small part of what hospital chaplains offer, it is nevertheless an important element of their role. At Sheffield Teaching Hospitals NHS Trust, Fran’s team is on call 24/7 to be with people in their last moments or in other emergency situations – and this can be a huge comfort for family members too.

Ultimately, chaplaincy forms part of an holistic model of care that addresses more than just the physical – and Fran says there is even evidence that if your general wellbeing is supported, you will get back home sooner.

“When you go into hospital it can be a scary experience”, she adds. “You can often lose your privacy, dignity, and the ability to be in touch with your family – so having somebody to support you with that is really important.”

.

If you would like to speak to a chaplain whilst you’re in hospital, and it hasn’t already been offered to you, then do not be afraid to ask staff on the ward to arrange this for you. You can also contact hospital chaplaincy teams through the hospital switchboard.

Although all UK hospitals have a chaplaincy service, the size of the provision may vary.

Patients are often able to request chaplaincy in line with their religion, and if that is not possible, connections may be made with a faith group who can provide support.

UNDERSTANDING MORE ABOUT HOPE

chaplains we can support them to find that

Fran is currently undertaking academic

hope”, she says. “I believe that you live better

of illness, and her PhD will focus on hospital

people better, then we can help them more.”

will be interviewing both staff and patients

PHA UK Chair Dr Iain Armstrong, whose own

Unit, with the ultimate aim of improving

in pulmonary hypertension.

is, what gives people hope, and how as

of this magazine.

research into the role of hope in the context

if you are hopeful and if we can understand

patients with pulmonary hypertension. She

Fran is being supported in her research by

at the Sheffield Pulmonary Vascular Disease

PhD in 2014 focused on understanding hope

holistic care. “I am interested in what hope

Look out for updates in future issues

SPRING 2022 emphasis 25

Specialist support from someone who understands

PHAUK LISTENING

LINE

Our Listening Line support service gives you dedicated time with a PH professional to discuss your worries or concerns.

• Free and confidential • An impartial listening ear • Available to all PHA UK members, family and friends too Talking to someone who already understands PH means you don’t have to explain your condition first.

Find out more or book an appointment by emailing listeningline@phauk.org or calling 01709 761450 Appointments can also be booked via our website at www.phauk.org

This is not a formal counselling service and it is not designed to replace clinical advice from your specialist centre team.

Calls take place with Paul Sephton, a former clinical nurse specialist in PH. Paul has many years of experience supporting people affected by pulmonary hypertension, both in a clinical environment and as part of the PHA UK team.

“Paul’s support lifted me. He was so understanding and supportive, and it was helpful to chat with someone who knows about this disease.”

PHA UK member

Living better with PH

An important study aims to test whether at-home supervised exercise can help people with pulmonary hypertension – and this is your chance to take part.

L

ike many things, the SPHERe trial suffered a set-back due to COVID-19. But it’s now back, and just as determined to discover more about the benefits of physical activity in PH. The trial is being led by clinical exercise rehabilitation experts and researchers from the University Hospitals Coventry and Warwickshire NHS Trust, and the University of Warwick Clinical Trials Unit. It initially set out to launch at the start of 2020, and after delays due to the pandemic, it’s now begun recruitment once again.

Funded by a £1.3m grant from the National Institute for Health Research (NIHR), the trial aims to find out if exercise rehabilitation can be done at home with online (remote) supervision from trained exercise specialists.

Who can take part?

Researchers are looking for 352 people with pulmonary hypertension from anywhere in the UK. Fitness levels are unimportant, but to take part you must have access to the internet and be able to follow exercise sessions online.

What will be involved?

Researchers will allocate people randomly (using a computer) into two groups. Group one will be invited to take part in the remote supervised exercise programme for eight weeks, and group two will receive general advice on physical activity.

Those in group one will need to travel to assessment sites in either Coventry, Walsall, Birmingham or

Glasgow* for an initial assessment involving an incremental shuttle walk test. They will then be asked to follow an eight-week individual online exercise programme at home, with a weekly catch-up call with an exercise specialist and a weekly online-led group exercise and support session.

After four months and 12 months, participants will be asked to return to the assessment site for further tests.

Travel costs will be reimbursed.

Why does this study matter?

Existing research shows that exercise rehabilitation may be helpful for people with certain types of pulmonary hypertension, by helping to reduce symptoms like breathlessness and fatigue, and making it easier to carry out day-to-day activities.

But these studies looked at giving exercise to people, under supervision, whilst they were in hospital.

Until now, no research has looked at whether exercise can be prescribed outside of the hospital setting, for people living with pulmonary hypertension in the community.

The findings of this study will help researchers understand how effective this type of supervised exercise is, so that similar programmes can be used by the NHS to help more people with PH.

*Work is taking place to identify more sites around the UK

How do I find out more or sign up?

More information about the trial can be found at www.warwick.ac.uk/ sphere. You can contact the researchers directly with any questions, or put yourself forward for the study, by emailing SPHERe@warwick.ac.uk or calling 024 76234570.

Scan this code with your mobile phone to watch our interview with Stuart Ennis, one of the exercise specialists working on the trial. You can also find the video on our YouTube channel @PHA ORG UK

SPRING 2022 emphasis 27

Every is we put sue, questio your selectio ns to a no UK’s top f the profess PH ionals

Paul Sephton

Former Clinical Nurse Specialist in PH

Research Physiotherapist, Golden Jubilee National Hospital

“My wife was diagnosed with pulmonary hypertension nearly a year ago and while she seems to be coping well, I’m still struggling to get my head around it all. Does it get easier?”

A diagnosis like this is often a big shock to the whole family, and it can take a long time to adjust.

It’s important not to be hard on yourself, and to allow yourself space and time to come to terms with what’s changed.

Communication is also key; talk to each other about how you are feeling, and how you may be able to support each other.

Equipping yourself with as much knowledge as possible about the condition can also help, but make sure this information comes from trusted sources (be careful with Google!) The specialist centre treating your wife will be very happy to

SPRING 2022 emphasis 28

Shaun Clayton

Joanna Ford

Director of Membership Support at the PHA UK

answer your questions, as they recognise how important it is to involve family members in care too. You might find it helpful to talk to other spouses / partners of people with PH. At the PHA UK we have a private Facebook group exclusively for family members and loved ones. Type this into your web browser to join in the chat: www.facebook.com/ groups/402808577486104

We also operate a Listening Line service for anyone affected by PH, including family members. Turn to page 26 for more details and do get in touch if you’d like to talk anything through.

You’re definitely not on your own.

Paul

“I keep reading that people with PH should exercise, but how do we know it’s safe? It wasn’t so long ago that we were told we shouldn’t” Historically it has been common practice to advise people with PH to avoid exercise. This was due to concerns about increasing the strain on the heart, worsening the condition. However, exercise therapy can be of great benefit in many conditions affecting the heart and lungs and PH is no exception. There is now strong evidence to show that supervised exercise delivered by a specialist centre is safe and effective for patients who are medically stable and on optimal PH medication. Research shows that individually tailored rehabilitation can deliver significant

“I am in the process of applying for various benefits. What choice of cars would I have through the Motability scheme?”

This is a question I hear a lot. Motability is a scheme that allows you to lease a car at a heavily subsidised rate if you are in receipt of the higher rate mobility component of Personal independence Payments (PIP).

Your choice of car depends on how much you are willing to spend. There are three spending structures; each one gets slightly more expensive, but each step brings a wider selection of cars.

1. Cars that cost less than your mobility allowance.

According to the Motablity website there are around 45 cars that cost less than your mobility allowance.

That means that they’d take the agreed weekly amount from your mobility allowance but give you the remaining money back to spend/save. A key difference between this option and the next is that these cars are available at a fixed weekly amount for the whole of your lease agreement. The remainder of your allowance, including any annual increases, will be paid directly to you by your benefit payment agency. The kind of cars that are available on this option are small ones like the Peugeot 108, Kia Rio and Toyota Aygo.

improvements in exercise capacity, breathing function, mental health and quality of life.

The benefit of being reviewed by an experienced PH physiotherapist is that exercise Find out ho w activity is prescribed at a safe level you can g for you. The rehabilitation programme involved in et a into the be study is tailored for people who experience nefits of e xercise wit shortness of breath and fatigue. Each h PH o n page 27 session helps to improve your knowledge about the condition and educates you on how to exercise safely and cope better with symptoms. This gives you the tools to be able to independently manage your condition with confidence and help make lifelong changes to your activity levels. Joanna

2. Cars that cost all of your mobility allowance. There is a larger selection of cars available to you if you go for this option. Again, according to the website there are around 135 cars that cost the same as your allowance, which means you swap the whole of your weekly mobility allowance for the car of your choice. Unlike the previous option, this payment will include any increases in your allowance during your lease. The cars available include the Citreon C3, Mini Cooper and Toyota Corolla.

3. Cars that cost all of your mobility allowance and have an Advance Payment. The last option is where you can increase your choice further by paying a one-off, non-refundable upfront payment which they call an ‘Advance Payment’. The Advance Payment covers the cost between your higher rate mobility allowance and the total cost of the lease. The kind of cars that are available on this option are the Ford Mondeo Saloon, Jeep Compass and Toyota C-HR. You can find more information at www.motability.co.uk

Shaun

Got a question for the next issue?

Email media@phauk.org and we’ll find the right expert to answer it!

SPRING 2022 emphasis 29

I

live in Ontario, about 15 minutes from Niagara Falls, with my fiancée Erik and my children Kason (7) and Kylie (2).

During my pregnancy with my daughter, I could not walk up one flight of stairs without having to sit and take a break, and I frequently took naps throughout the day – meaning I had a very hard time keeping up with my son.

When Kylie was born, I began experiencing severe shortness of breath, but due to breathlessness being a symptom of COVID-19 I was waiting for it to ‘blow over’ before going to the doctor.

In August 2020, I was walking to my car when I had what I thought was a heart attack. I had pain in my left arm, jaw, neck, and back; as well as tightness in my chest, nausea and shortness of breath.

When I arrived at the hospital they checked my bloodwork, which initially was normal. It wasn’t until the second round of blood tests (used to assess if someone has had a heart attack) that my troponin levels (a type of protein found in the muscles of the heart) were elevated.

SPRING 2022 emphasis 30

Upon doing an echocardiogram, the doctor noticed a large atrial septal defect [hole in the heart] which I was not previously aware of. The doctors in my hometown send me to a hospital for a right heart catheter, which also showed pulmonary arterial hypertension.

It was during my first appointment with a specialist, in October 2020, that I was officially told my diagnosis. Until that point, all I had seen was a note on a nurse’s clipboard with the words ‘pulmonary hypertension’.

What I thought was a heart attack was actually an artery tearing on the inside of my heart (known as Spontaneous Coronary Artery Dissection, or SCAD).

It was a blessing in disguise, as it meant I had gotten an early diagnosis of PAH, and I know others aren’t that lucky.

Treatment on the other side of the Atlantic

I am fortunate that I’m able to manage on oral medication. I take drugs called Adcirca (which is like sildenafil) and Opsumit (macitentan) and they

have helped me significantly.

They have brought the pressures in my lungs down low enough that I now qualify to have my atrial septal defect closed within the next coming months (prior to treatment, an open-heart procedure wasn’t deemed to be safe enough).

Since beginning the treatment, although I have noticed improvements, I still experience the fatigue and some shortness of breath - which we hope will improve after the closure.

The healthcare system in Canada is a universal system that is publicly funded, primarily by each province and territory. In Ontario, we have a healthcare plan called ‘OHIP’, which is basic insurance coverage for things such as required surgeries (not cosmetic procedures), childbirth and the like.

We also receive benefits from workplaces which help cover the ‘extras’, for lack of a better word. For example, giving birth in hospital is free, however staying in a private room afterwards is not. OHIP only covers a ward, or a room with other patients.

In Canada we have 17 Pulmonary Hypertension Specialized Centres across the country. We also have the charity The Pulmonary Hypertension

Tia has a blog, where she shares her experiences of living with PAH. You can follow her posts at tiaatkins45.wixsite.com/ phandtia

Association of Canada [the equivalent of the PHA UK] and they have a website with a ton of information and resources.

Life with PH in Canada

Pulmonary hypertension has affected my life in a few ways. Firstly, my ability to keep up with my children, and take them on hikes or for a swim has been impacted. These are things I cannot do with them alone and would need someone to help me keep up.

The increased fatigue is also an issue as my children tend to not want to nap as much as my body needs me too.

PAH has also affected my work life.

Prior to my symptoms, I was able to keep a job full-time, but unfortunately now, once the fatigue kicks in, I am unable to stay awake for the usual 9-5.

PAH has affected my mental health too. Being told that I had an incurable disease was extremely hard and distressing, not only for myself but also for my family. But as we approach a year since my diagnosis, we are getting used to the fact that this is our lives now.

Having young children with this condition is quite tiring to say the least.

However, I am thankful every day for my children. They help me to push through my day, and through the heartache and mental health issues this has caused.

Because of them I am stronger.

I am slowly starting to adapt to life with PAH. As I write, the cold weather has hit Canada and I realise it is much harder to breathe and to do things than it is in the warmer months. I am learning the activities I can and cannot do and when I’ve hit my limits.

As time goes on, I am learning that some days are good days, and I can get through them – and some are bad and go on for weeks. Most importantly, I am learning that all of this is OK.

SPRING 2022 emphasis 31

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SPRING 2022 emphasis 32

DID YOU

KNOW..?

Your eyes blink around 20 times a minute (try and count them for the next 60 seconds!)

Enjoying hobbies with PH

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to come up my end.

I’m definitely not the best at football but nevertheless, I enjoy it. being I sometimes might prefer any running in goal as there is hardly to stop the ball involved, you just need going into the net. lly I love to dance, especia e at Stagecoach [part-tim lots th wi ] ren child performing arts school for hard lly rea it lly, I find of other people, but usua ve ha t times I migh with all the cardio. Some my PH makes it too to take a break because do it and still have fun. hard. Despite this, I still do actions and dances Sometimes, we have to be challenging, even if with singing which can ill, I do it anyway and they are little actions! St en in lots of Stagecoach always enjoy it. I have be d dancing. shows, singing acting an

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FUN ..

FACT.

The Green Leaf Crew are named after the leaf in the PHA UK logo!

SPRING 2022 emphasis 33

Making the most of your

FREE !

remote appointments Plan for your telephone or video appointment with our bespoke preparation form, designed specifically for people with pulmonary hypertension

✓ Plan the questions you want to ask ✓ Pre-fill your EmPHasis-10 questionnaire Download the form at www.phauk.org No printer? No problem!

Call 01709 761450 or email office@phauk.org and we’ll happily post you a free copy SPRING 2022 emphasis 34

ead

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I’VE NEVER

WANTED PH

to define me

Emma with her sons Joe, Sam, and Rory, and husband Jon

Emma Sanger lives in Herefordshire and was diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) in 2008.

Over the page, the 57-year-old describes the twists and turns of her journey, the support of her family, and the hope and gratitude her experiences have left her with.

SPRING 2022 emphasis 35

A

t the end of 2007 I distinctly remember starting to feel breathless on walks.

We live in the countryside, so we walk regularly, and anything uphill started to become a struggle. I began to notice that even walking from my car to the office was hard.

I got a cold, which developed into a chest infection, and things just got worse. I was back and forth to the GP, told I had pneumonia, given antibiotics, and then I would fall ill again. This went on for six months before I was eventually able to see a respiratory consultant.

Scans showed pressures were high on the right side of my heart and it was at this point that pulmonary hypertension was first mentioned as a possibility.

I had never heard of it, and it was only when I looked it up online (which nobody should do, but everybody does!) that I realised how serious it was.

I was referred to a specialist centre in Sheffield for diagnostic tests in July 2008, which confirmed I had chronic thromboembolic pulmonary hypertension (CTEPH).

It was a very frightening time because I didn’t understand it all and wanted to find out more. I would always rather have the facts because I think if you know what you’re dealing with, it’s a starting point to managing or understanding things.

Of course, I had loads of questions about what had caused it. Why would my blood clot and then cause this serious and rare disease? Was there something in my medical history? My family are

SPRING 2022 emphasis 36

generally healthy, and I’ve always been fit and healthy, so it was a shock. I was told I was just ‘unlucky’.

Hopes dashed

Now the process of dealing with it and managing it began. A pulmonary endarterectomy [surgery to remove the clots] was mentioned and I remember it being held up as the thing that would save me. It was possible that an operation could remove the clots, and I would be fine.

two months after my diagnosis, and I spent the day at Royal Papworth. Here, I was told that my scan results showed the risk of me having the operation was too high, and therefore I was unsuitable for surgery. Jon and I were stunned; it was devastating.

We went back home, and it felt like the end of the road. In those early days I really believed I had a matter of years, and I was advised it would be wise to organise my affairs. All I could think about was the effect on my children.

“When you’re ill and you’re tired, your priorities shift” Whilst I waited for my assessment at Royal Papworth Hospital [the only hospital in the UK that carries out the surgery], I started on medication and spent my time at home just sitting.

It was too much effort to go upstairs, and I felt useless.

My family were great. My three children were only young then (the youngest was eight and the others were going into their teens), but they did what they could to be helpful. They could see I was unwell, but I didn’t want their lives to be disrupted, so my husband Jon and I were quite selective with what we told them. Jon was brilliant, and everyone rallied round.

My surgery assessment came round

This was definitely the lowest point during my whole PH experience.

Adjusting to a new ‘normal’

I went back under the care of Sheffield, and other medication options were discussed. The team were really comforting and reassuring.

My medication was increased, and I started to feel a bit better, although I did have to give up my job at the Tourist Information Centre. I did the odd couple of hours for them here and there, on a voluntary basis – but I would be exhausted.

The next big hurdle was the benefits system. Nobody seemed to understand the disease and the process went on for

“It’s hard to express in words how grateful I am to the teams at Sheffield and Royal Papworth for their kindness and expertise.” months, but eventually, after going to a tribunal, I got what I was entitled to.

It was hard work, and I wouldn’t have been able to jump through all those hoops without Jon’s support. It was exhausting.

I started getting into the routine of re-managing my life; changing, adapting, and accepting this was the way it was going to be.

Everything was exhausting, so if I needed to do something or go somewhere, I would have to plan it all carefully. I got a blue badge and that really helped me, but if I went to the shops, I would have to rest the next day to get over it.

Life went on, in this different way of living. It was quite boring, but I think when you’re ill and you’re tired, your priorities shift. Going out and seeing people became less important; the most important thing was to feel well.

Another chance

I just carried on, and over the years, I started to feel a lot better. In March 2014, I had a new set of scans, which showed how much things had improved.

The balance of risk had now shifted, and I was told that although this was unusual, I was now a suitable candidate for the pulmonary endarterectomy. This was another shock, but of a different kind!

It was an easy decision for me to make, and I had the operation in October 2014.

The surgery went well, but I do have some residual PH, so it hasn’t been a ‘cure’.

But the size of my heart reduced straight away and although I didn’t wake up for

three days, and the recovery was quite long, I do think it was worse for my family than me at that time.

Our home is over three hours’ drive from Royal Papworth and Jon did a lot of journeys back and forth over the fortnight I was there.

I experienced paranoia and psychosis [known as ‘ICU psychosis’] which I now know is quite common. I had a couple of appointments with a clinical psychiatrist but once the drugs wore off, I was fine.

I don’t think I was the easiest of patients!

It was quite an experience, and there was a lot of pain and discomfort, but I did all the things I was supposed to do, and gradually, I recovered. It’s hard to express in words how grateful I am to the teams at Sheffield and Royal Papworth for their kindness and expertise. I’m very lucky.

Life beyond surgery

Because I have residual PH, I still see a specialist once a year, down at Royal Papworth. We have six-monthly telephone consultations too, and in some ways, it’s quite nice to have that contact and reassurance.

I am aware the PH is still there and if I ever feel a bit run down, I always wonder if it will become a problem – but I’ve learnt how to manage things.

I know now that if I need to sit down and have a rest that I’m not wasting time, I’m investing in energy for later on. It takes a long time to get used to that though!

It took me a good year to properly

Emma and her son Rory, who completed a sponsored walking marathon for the PHA UK in 2020 recover after the surgery but I’m now working again part-time.

My confidence had plummeted [as a result of everything that happened] and it took 12 months of interviews, but I ended up getting a really nice job working in a customer service role at an historical monument.

Going to work gave me a bit of confidence back. It gives me something to focus on away from home and a sense of purpose.

I’ve never wanted PH to define me.

I’ve still got a bit of disease, I’m still on medication, and I get seen by medical professionals, but it’s integrated into my life now.

I’ve noticed that people don’t ask how I’m feeling as much anymore, because I’m seen as ‘just getting on with life’, which is quite liberating.

With everything that’s happened, I’ve realised that the people around you are what really matters. I feel very grateful, and very lucky, and I want other people to know - in a realistic sense - that there is always hope.

You never know what is around the corner.

SPRING 2022 emphasis 37

Wondering what financial support you might be entitled to?

Benefits Calculator and Grants Search tools now available at www.phauk.org Find out if you could access welfare benefits, charitable grants and other support that you might be missing out on.

In association with Turn2Us www.turn2us.org.uk

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Kaylee's column

Having grown up with pulmonary hypertension, 21-year-old Kaylee Mynot knows a thing or two about life with a rare disease. In her latest column for Emphasis, she reflects on transitioning to adult PH care services, and offers her advice for young people about to embark on the change.

Kaylee is happy to chat to any young person who may be nervous about moving up to adult care services. Please email media@phauk.org and we will pass your message on.

SPRING 2022 2018 emphasis 39

Behind the

job title

“ It truly is an amazing time to

be involved in PH research” research” A presentation about heart rate and energetic function won Dr Chinthaka Samaranayake a PHA UK-sponsored prize at last year’s National PH Research Forum. Emphasis caught up with him to find out more about the findings of his study, and the implications of wider research in the field of pulmonary hypertension.

Can you tell us more about your study and what it involved?

The research study investigated what factors determine the way the right ventricle [one of the chambers of the heart] responds in terms of its energetic function. Specifically, we studied whether reducing the heart rate using a commonly used drug (beta-blockers) brings about a favourable response in the energy the heart uses during each heartbeat.

The patients who took part in the study were given a short infusion of a beta-blocker medication while exercising on bicycle pedals during a right heart catheter procedure, and we measured the changes in the heart function with a special catheter before and after the medication infusion.

SPRING 2022 emphasis 40

What were the findings and how significant are they? How will your research influence diagnosis or management of PAH?

We showed that heart rate moderation during exercise improved right ventricle relaxation and therefore its function.

This was a small study designed to test the concept, but hopefully the results will lead to better understanding of the heart function during exercise in patients with pulmonary hypertension.

The results will ultimately help in the selection of patients who are likely to benefit most from treatments for pulmonary hypertension in the future, and lead to new therapies specifically targeting the right heart in pulmonary hypertension. This project allowed us to establish a very nice platform for doing

“Research in PH is moving forward on a lot of fronts, with multiple different treatment options being tested in clinical trials” other similar studies, and the team at the Royal Brompton Hospital has been working on two other novel ideas, translating some of the laboratory-based research into clinical trials. I have been very lucky to be involved in some of this work.

How enthused are you by the research that’s taking place across the board in PH at the moment?

Research in PH is moving forward on a lot of fronts, with multiple different treatment options being tested in clinical trials.

Moving the focus away from pulmonary vasodilatation alone and focusing more on disease modifying therapies is very exciting.

The results from the early phase clinical trials of these treatments are extremely encouraging and hopefully will translate into a lot of treatment options for our patients in the near future. There is also a lot of amazing basic science research work in PH carried out both in the UK and overseas which will significantly advance our knowledge and understanding of this condition.

The recent advances in the procedural interventions and monitoring techniques have also been incredible.

It truly is an amazing time to be involved in PH research.

Dr Samaranayake is a thoracic physician with special interests in pulmonary hypertension, pulmonary embolisms and pulmonary vascular physiology. He completed medical training in New Zealand and specialist respiratory physician training in Australia, and is now working as a Senior Clinical Fellow at the Royal Brompton Hospital’s pulmonary hypertension unit. He is completing several research projects towards a PhD in risk stratification and early interventions in pulmonary vascular disease.

Why is medical research in PH so important?

PH is a relatively ‘young’ disease, so most of the treatment options have only been available in the last 30 years.

The major advances seen in PH in recent years is driven entirely by the incredible medical research that has been conducted over the last few decades.

There is so much more to understand about PH and the key to further developing our knowledge is through research. We need to think broadly in the clinic and in the laboratory to advance research in PH to continue to help our patients. The incredible collaborations established through the PH network and the PHA UK have meant that UK researchers have been world-leaders in driving research.

How did you feel to win the PHA UK prize at the research forum?

It was a huge honour to receive the PHA UK prize. It is a reflection of all the hard work the PH team at the Royal Brompton Hospital has done over the years to establish platforms for translational and clinical research

in PH. I feel very privileged to have had an opportunity to make a small contribution.

I want to express my sincere thanks to the whole PH team at the Royal Brompton, and in particular Dr Colm McCabe, for providing guidance and support for my PhD research. I also want to acknowledge and thank all the patients who participated in the project – we couldn’t have done it without the amazing patients who are always willing and engaged to advance PH research.

The National PH Research Forum takes place annually at the Chelsea Physic Garden in London.

It brings together physicians, scientists and academics from all over the country and the PHA UK has provided sponsorship since 2012. The annual forum is an opportunity for those involved in developing better treatment for people with PH to share research, experience and ideas.

After a forced break due to the COVID-19 pandemic, the forum returned in November last year.

SPRING 2022 emphasis 41

“ We knew everything about each other.

Apart from this.”

Let’s give the people we love, the certainty they need.

Talk to your loved ones about organ donation.

Visit organdonation.nhs.uk

O ve r t o

you...

In our new regular feat ure, we ask for your top tip s on different aspects of man aging everyday life with PH. This issue, we asked our Fa cebo followers for their advic ok e on bathing and showering…

“I have a bath board to show er with. It ’s a w with a small h hite board andle on the si de that fits ac [which has a sh ro ss my bath ow use it, but if I’m er head]. If I’m having a bad day I can having a good day and I can I don’t tend to stand, then use it.” Debbie Taylor

t on when “I use a stool to si ashing my hair. showering and w so I make sure I have an IV pump, l wrapped with I have the line wel place the pump clingfilm, and then , outside of the on a chair nearby ilding curtain.” Judith W

my back ower, I stand with back to sh I n he w d an e e top of my “I have an IV lin recting that at th di y, y ra sp er ow sh est and wetting m y to the ch y m n w do g in nn spra avoid the water ru ower head by hand to direct the sh ower e sh th pe ld dressing. I ho verhead rainfall’ ty my ‘o an e us r ve ne I ater, and if to rinse myself. ntrol the flow of w d that’s co ly si ea t n’ ca I an head as s to be changed ed ne it et w ts ge dressing t do.” Cas Lawson no er th ra I’d b jo a just an extr “I only very occ asionally take a bath as I find my heart races for quite a while af terwards. If I do, I only take a shallow one and keep the door open so there’s no steam, and so I need it. We hav can call for help if I e changed our shower to a double-hea ded one, with a fixed overhead and a handheld on e, so I can just stand under the spra y or hold it to shower if I’m sitting dow n . I no longer have an IV line, but wh en I did the handheld version was es sential so I could keep the water away from my line as mu ch as possible .

I u se Press’n Seal [a d brand of food wrap] to keep the line d ry, which was easier to remove than o rdinar y cling fi lm .”

Julie Royle

“I have a thick towelling robe, which I put on wh en I get out of the shower, then go and lay on the bed. Once I’v e had a rest, I take it of f, and he y presto, I’m all dr y without any ef for t.”

Paula Joanne Smitham

“I find baths and steam make me bre athless, so instead I stick to showers and hav e the door open to remove steam. I also have a shower chair so I'm not wasting my energy standing up. And I have the shower head han ging up and pointing down, so I don't nee d to hold it and can just sit under. Using 2in1 shampoo and conditioner, instead of separate ones, reduces activity. And I dry myself on the shower chair, so I'm sitting down. Then I lay down immediately afterwards, to rest, before get ting dressed.

Finally, showering in the evening is my top tip – if I shower before bed at night, I can rest and sleep straight afterwards.”

Sarah Marshall

ework r carr ying out hous fo ks ic tr or s tip y t us know have an ks well for you, le or w Next time: Do you at th ng hi et found som phauk.org with PH? If you’ve ur tips to media @ yo nd Se . rs he ot r fo and we’ll share it

SPRING 2022 emphasis 43

a s k n a e d tr p e o u g s h h T Emma Ferguson lives in Northern Ireland and was diagnosed with idiopathic PAH at the age of 16.

The English Literature graduate enjoys writing about different aspects of life with pulmonary hypertension, and here, she explains how she makes exercise work for her.

T

his week, and really this month, I’ve been exhausted.

For me, PH exhaustion has a creeping feeling.

It starts with a cold or a particularly tiring few days. The longer the days, the deeper it sets in – so that it’s going to require more and more rest to get rid of it.

So often things don’t stop soon enough. Pressure to meet deadlines and outside forces means that other things slide in their place, usually things in my personal life; pleasures like reading or meeting friends.

Work-life balance is difficult at the best of times, but when unpredictable health is added to the mix, prioritisation becomes even harder but also even more essential. Another thing that gets pushed aside when I’m getting exhausted is exercise, although unlike my other examples, exercise has never really constituted ‘pleasure’ for me.

When I was at primary school (and undiagnosed) I was simply the sickly kid who couldn’t do sports and was regularly

SPRING 2022 emphasis 44

off school. By secondary school, aside from mandatory PE classes, I’d written off regular exercise entirely.

The Duke of Edinburgh’s (DofE) Award was eye opening for me. An enlightened attitude to participation espoused by the DofE charity meant that I was able to avoid the heavy rucksack-wearing, incline-walking expeditions mostly associated with the award – and instead travel in a canoe.

This required a lot of additional training for safety, but it was something that I could do.

Over the course of four years, I completed my bronze, silver and gold awards in a sport that allowed me

to keep up with my peers. The upper body and core strength required to manoeuvre the canoe was something I was able to build up without becoming too breathless. Completing DofE still relied upon me being relatively well before I set out, but the actual exertion was manageable.

By the time I was at university, walking had become my best friend.

We all know the advice about PH and exercise; walking is perfect for people with PH and ideally, we should walk while breathless so long as we can maintain a conversation. Walking everywhere to save money improved my fitness like nothing else. I walked

“Exercise and I will never be best friends, as we don’t meet regularly enough”

x

e f

o

s i i ng w c r e ith

everywhere; if it was 40 minutes or less, I would walk it. This was great, but only while I was well.

While at university I tried lots of different sport societies from ultimate frisbee where I couldn’t keep up with the running, to Brazilian Ju Jitsu where the intensive warmups at the start of the session were too much. I also tried Park Run [nationwide weekly mass participation 5km events] where well-meaning people not aware of my hidden disability encouraged me to keep going – when really all I needed was to stop.

I’ve noticed, as an impact of PE at school while undiagnosed, that high intensity exercise classes aren’t really for me. Even though class teachers are usually very understanding about my disability when I inform them of it, I still

Emma and her mum

Emma writes regular blogs for our lifestyle website, www.phocusonlifestyle.org

If you would like to share any aspect of life with PH for our magazine or websites, please email media@phauk.org

feel an undue pressure to keep going and match the pace of the class.

Yoga classes are the exception to this rule. I got to attend a series of free sessions while at uni, aimed at improving mental wellbeing. They were gentle, focused on breathing and holding stretches. While I found the breathing elements the hardest, there was something about the more contemplative setting of a yoga class that meant I wasn’t as concerned about not doing things properly or stopping if necessary.

When COVID-19 arrived all these classes stopped and my general predisposition against video calling meant that online classes weren’t for me. So, I started using an app called Leap Fitness instead [see page 7 for Emma’s review of this app].

PH Read Emm a’s review of th e exercise ap p ‘Leap fitne ss’ on page 7.

Emma taking a break on one of her walks I don’t know if regular exercise planning will ever be possible for me, but I have learnt that much of what I’ve mentioned relies upon me making the right calls early on.

It often feels like with PH the best thing you can do is pre-planning; prepare for all eventualities.

Exercise and I will never be best friends, as we don’t meet regularly enough, but better overall awareness about my exhaustion can make us better than casual acquaintances.

Last month I climbed the Stairway to Heaven trail in County Fermanagh and after regular breaks, made it to the top.

This month, while I’ve been exhausted, walking has been the limit for me.

If I can shake off the old ideas I had at school about what ‘good’ exercise looks like, and tailor what I can do to fit my condition better, then hopefully there are more peaks than troughs ahead.

SPRING 2022 emphasis 45

21 years of influence, hope and integrity As well as looking to the future, it’s also important to reflect on just how far we have come with the help of our members, our supporters, and our unique pulmonary hypertension community. This is a snapshot of the PHA UK’s journey over the last 21 years…

Our story begins...

Thank you for the excellent support I have received since my diagnosis

2000

The PHA UK is registered with the Charity Commission and a helpline staffed by volunteers is introduced

You have always been my ‘go to’ reliable source for any concerns and questions

SPRING 2022 emphasis 46

It is lovely to have such a charity and our own community

2001

The first national PHA UK conference is held in Derby

2011

EmPHAsis-10, the world’s first PH-specific quality of life measure, is developed by the PHA UK alongside the University of Manchester. It is now used around the globe over 30 different languages.

Also this year, The first students enrol onto the PHA UK’s Medical Education Programme.

2013

The PHA UK partners with the University of Manchester to begin an 18-month longitudinal ImPAHct research study exploring exactly what it’s like to live with PH, both for patients and their kinship

2004

The first PHA UK family weekend takes place in Chester

You unite people to support each other

The PHA UK has been my blessing. I'm forever grateful

2006

The PHA UK hits 1000 members!

(Today, we have over 4,500)

Thank you for advocating on behalf of the PH community”

This year, we also visit 10 Downing Street to ask for political support against potential funding cuts for PH drug therapies

2007

Thank you so much for being there”

A PHA UK campaign leads to NICE (National Institute of Clinical Excellence) overturning a decision to cut funding for all but one PH drugs. This groundbreaking U-turn (the first of its kind) meant we helped safeguard the future of drug therapies for people with PH.

2010

The PHA UK supported the formation of the National Audit of Pulmonary Hypertension, designed to uphold the high standards of care at specialist centres

Always great resources and support

We are supported from every angle

2014

The PHA UK is the only UK health charity to take a stand at the European Respiratory Society’s Congress in Barcelona, which attracts 20,000 healthcare professionals

You are the lifeblood of our community… you have our backs when no-one else does

SPRING 2022 emphasis 47

21 years of influence, hope and integrity

Thanks to you, I feel at last that I'm not in this nightmare alone

2016

The PHA UK forms PHocus2021, a political advocacy group to influence national decisions. This is also the year the charity invests into the development of rehabilitation in PH, and launches its first PH Awareness Week.

2018

By harnessing the patient voice, the PHA UK helps overturn commissioning decisions to enable the new drug selexipag to be made available across the UK. The charity launches a second website, phocusonlifetsyle.org, to help people along their journey with PH.

2015

EmPHAsis undergoes a major rebrand, becoming the glossy magazine you’re reading today!

You provide excellent support and a wealth of information for phighters and carers

The PHA UK has been my blessing. I'm forever grateful

2017

The PHA UK invests into important research into palliative care in PH

WINTER 2021

YOUR VOICES

Real-life experiences shared SPRING 2022 emphasis 48

PH & the pandemic

Research findin gs

FU

Be The imp hyp

Hello

2022

What we’re all looking for

2019

*Shielded Voices, November 2020

The PHA UK publishes groundbreaking research into the financial and emotional impact of PH. This work is the first of its kind in pulmonary hypertension.

Here’s next 2 to the With o 1 years. ur wo nderfu PH com l m u n it b e hi n y d u s, a n is poss ything i b le !

2020

The PHA UK responds to the COVID-19 pandemic by mobilising all available resources to support members through the crisis.

Website visits increased by 200% and 98% of members surveyed said information provided during this time was ‘extremely’ or ‘very’ useful*

2021

The unwavering support and information you provide to patients and those affected by the disease is priceless!”

A major report into the impact of the pandemic on the PH community is published by the PHA UK. ‘1000 voices’ captures the experiences of patients and loved ones and attracts the highest number of survey responses in the history of the charity.

It’s comforting to know you are always there”

ph + the

ULL RE PO

pandem c RT | M arch 20 19

eneat h the surfa e true ce pact o financial f per te pulmon nsion ary .

1000 VO CES The lived experien ces of the

SPRING 2022 emphasis 49

g n i s i a r d n Fu

FOCUS

Please let u you decid s know if e to do som fundraisin e g f o r u s :Email offi ) c call us on e @phauk.org, 0 or tag us o 1709 761450 n Twitter or Facebook, Instagram

On your Marque... get set...go!

This year will see petrol turn to pounds as members of the Classic Marque Sports Car Club fundraise through their race series. The club has chosen us as their charity partner for the next three years and we’re privileged to be supported in this way. As well as raising money, the club is raising awareness by donning PHA UK stickers on the cars, and they delivered a presentation about PH at their annual awards dinner in November.

Superstar shoutouts

Celebrating Erika Every penny makes a difference to our charity, and we were touched to receive a £60 donation with a very special reason behind it. Five years ago, Erika Cowie passed away with PH at the age of just 18, and her grandparents have saved up her pocket money ever since. Every year the money is donated to charity, or for a cause close to their hearts, and this year they chose to give half to us, as ‘a little help towards making a difference to the lives of others’. Erika is pictured with her nanny and grandad on her 18th birthday.

K TH A N !

U O Y

Thank you to these fantastic fundraisers…

Gary Gledhill, who lives with pulmonary hypertension, took on a 10km walk in January to raise over £300. He said: “It has been an amazingly rewarding journey. I am not usually into things like this, but I will certainly do something again!”

Staff at SGC, an inspection, verification, testing and certification company in Ellesmere Port, donned their favourite sweaters to raise over £500 from a Christmas Jumper Day in December.

A group of families joined forces to run every day of January in memory of Kirsty Phillips. The Deans, Attards, Garretts, Hanlons, and Bramleys covered at least a mile a day for 31 days, raising over £1000.

At the end of March, a team of healthcare staff from the Sheffield Pulmonary Vascular Disease will take on the Sheffield Half Marathon. The Hallamshire Heroes will be ‘puffing for pulmonary hypertension’ for 13.1 miles.

Look out for photos in the next issue!

GOO LUCKD! SPRING 2022 emphasis 51

Behind the

job title

D

t h g i n K n r Da

Dr Dan Knight is a Consultant Cardiologist, working within the Royal Free Hospital’s National Pulmonary Hypertension Service.

He is also Honorary Associate Professor at University College London’s Institute of Cardiovascular Science, and an Arsenal season ticket holder who is trying hard to get back into running. We spoke to the father-of-two to find out more about his work, his motivations, and his predictions about the future management of PH. Was working in medicine always part of your plan?

No, before my teens I wanted to be an architect! During my GCSEs I realised I liked science, but I wanted to work with people too, which is how my path took me to medicine. Plus, my stepfather trained as a doctor and ultimately went into research, so he was an inspiration.

I completed my medical school training at University College London (UCL) in 2004 and have boomeranged back there ever since, coming back to do my research degree when I was a cardiology registrar. I’m now a consultant at

SPRING 2022 emphasis 52

the Royal Free but I’m also carrying on my research through UCL, so I’m continuing to work with the people at both institutions who were originally my teachers and mentors.

Did you find PH, or did PH find you?

The interest kicked off in 2008, which was my first year as a Cardiology Trainee Registrar. That year I was placed at the Royal Free, which is where I met Gerry Coghlan [the founder of the hospital’s PH service] and learned more

about the condition.

I came back to do my research in cardiac imaging and pulmonary hypertension, so I never really went away. During that time, I met Vivek Muthurangu at UCL, who has been a leader in the field of developing better imaging techniques for patients with PH using cardiac magnetic resonance imaging (MRI). Since then, my interest in PH and cardiac imaging has continued to grow and I never looked back! I think PH is an area that has huge potential in terms of developing new treatments and new ways to investigate and manage it.

What does the job of a Consultant Cardiologist involve?

My days are very varied which keeps things interesting – and keeps me on my toes!As a Consultant Cardiologist I specialise in pulmonary hypertension and cardiac magnetic resonance imaging (MRI) and I combine both of those interests into my clinical and research work.

I could meet patients at any point along their journeys – right at the start when they first get referred to the service as well as during their follow-up clinic visits or when they attend for their tests.

The Royal Free also operates shared care PH clinics at several other centres across the country, so in my job I regularly travel to see patients in clinics based in Plymouth and Portsmouth too.

Whilst the travel can be a little tiring, I really enjoy this hands-on patient-facing work, and being able

“What I enjoy most is helping people feel better” to interact with other healthcare professionals outside of your ‘bubble’ is interesting, refreshing and rewarding.

Many of our patients can’t be trekking long distances to London every few months for follow-ups or tests, it just isn’t fair on them, so I think it is important that we bring the service closer to them as best we can. It gives our patients better access to care and our service, but also invests in their region as a whole by reducing the variation in awareness of PH, improving access to our service, and lessening delays in diagnosis.

Can you tell us about any achievements you are particularly proud of?

I developed a new routine clinical procedure in pulmonary hypertension, which involves combining the right heart catheter [the ‘gold standard’ diagnostic test for PH] with a cardiac MRI scan, so that both tests are rolled into one. [Known as the MR-guided right heart catheterisation (MR-RHC) programme].

Instead of having two separate investigation procedures, this means we now have a combined procedure that is more comprehensive – and there is no radiation.

It is a better platform for understanding and investigating PH further and it’s now used routinely at the Royal Free.

We have had several other specialist centres from Europe come here to train in this technique, and the majority have successfully taken the programme back to their countries. It has been really satisfying to see this and my long-term aim is to establish a network of centres adopting the technique so that we can look towards better ways to assess our patients and the effects of novel therapies. Because of this work, in April last year I was awarded a British Heart Foundation Fellowship to carry out more research into pulmonary hypertension and cardiac MRI using MR-RHC.

What do you enjoy most about your clinical role?

It sounds clichéd but it is true; what I enjoy most is helping people feel better. As a medical professional, that’s fundamentally the reason for being here and I don’t think it ever leaves you.

The teaching I have had has always encouraged me to do whatever is most important for the patient. If you can try and see things from the patient’s perspective, then that can really help you make decisions.

Where would you like to see yourself - and the role of research - in ten years' time?

My long-term goal is to try and positively change the way we manage PH for the better.

With the different aspects of research that I am doing, all of them ultimately are trying to find ways of better measuring and understanding what is going on with our patients.

I think the direction of research needs to change in this country in the way that it is set up. I would like to see it more as a hybrid clinical/academic service.

I look at other centres internationally, in both PH and in other specialities, and the boundaries that can act as barriers between research and clinical care aren’t so clearcut.

We know that if a patient takes part in research, they tend to have better outcomes, and at the most basic level this is simply because they are so closely engaged with the service.

There is also a misconception that research participants are ‘guinea pigs’, which can put people off, along with extra visits that might be involved.

Improving accessibility to trials and improving our patients’ understanding of them will help.

And what about the future of treatment and management of PH?

There are some promising therapies that are in more advanced trials at the moment. I hope we can look back in five to ten years’ time and say, ‘do you remember the days before we had that drug?’ I think we will also see a more precision-based approach to understanding and treating people.

I don’t see it as a ‘one size fits all’ drug-based approach in the future.

I think we will understand patients more as individuals, rather than as a uniform group of people with elevated pressures in their lung arteries.

I think that approach will make a big difference in understanding how we can select treatments that work best for patients as individuals.

Finally, when you do get some time off, what do you do to relax?

Most of what I do outside of work is enjoyable, but not necessarily relaxing!

I have two boys aged six and three, and I am a season ticket holder at Arsenal – which hasn’t been very relaxing for the last few years.

I am trying to get back into running. I have completed a few marathons in the past but I’m about 10 kilograms away from that now, in the wrong direction! I have also just taken on a role as a primary school governor which is rewarding in a very different way.

Life isn’t quiet – I’ve always got something to keep me out of trouble!

.

SPRING 2022 emphasis 53

Our online hubs are for every step of your journey with PH www.phocusonlifestyle.org ✓ Real-life stories and experiences ✓ Advice on nutrition, exercise and mental wellbeing

✓ Dedicated sections on pregnancy, intimacy, and relationships

✓ Spotlights on employment, travel, and hobbies

✓ Blogs and videos

…and much more to help with all aspects of living with PH

www.phauk.org

✓ Information about pulmonary

hypertension, including diagnostic tests and treatment

✓ Financial support and benefits ✓ Driving with PH ✓ Fundraising support and resources ✓ Our online shop – including free booklets and guides

✓ All back issues of Emphasis (available to read online)

✓ Research reports ✓ COVID-19 support ✓ Our latest news

Online forums

Our private Facebook groups are a valuable source of support and advice from people with similar lived experiences.

Search:

‘PHA UK Official Facebook Group’ For those living with PH

’PHA UK Official Carers Group’ For family members, friends and loved ones ‘PHighting on: Life after loss to PH’ For those who have lost someone

SPRING 2022 emphasis 54

You’ll find direct links to these forums at www.phauk.org

w o d n i A w world to our

or of t c e r i D , n o t y a l

By Shaun C HA UK P e h t t a t r o p p u Membership S

n e PHA UK offices, Shau th om fr te da up r la gu In his re supportive resources … w ne e m so on s te da shares up

T) and we’ve been working (CB w ne gs in br s ay d, A new year alw th the Universities of Sheffiel wi t en sp ’ve we d an opportunities Cardiff and Nottingham to g in rk wo 22 20 of rt pa the first get it ready. We wanted to be of ys wa w ne lop ve de to hard n that we’ve thought of tai cer . ys gu d suppor ting you everything, dotted every I an s The first resource is available crossed every T – because it’ t now and is aimed at helping important we give you the bes people with PH manage their possible tools that we can. lem ob pr a anxiety. We know it is Turn to page 22 to find out d for a lot of our members, an more, or visit www.phauk.org work has been going on for A resource around palliative a lop ve de to w no ar over a ye care will also be available t en em ag an f-m sel eek four-w soon. We’ve invested heavily u yo wer po em to e mm ra og pr to research around this type in f. sel ll to help your of care, and this material wi e We’ve featured the programm be the first of many work s in previous issues of this to suppor t people to access , magazine as part of a what is a hugely beneficial controlled trial to test how yet underused, part of the effective it is - and we’re healthcare system. delighted that it’s now This first resource will e. on ery ev to le availab consist of two booklets, which on The programme is based will come as a pair. One is py Cognitive Behavioural Thera

d dedicated to in formation an debunking myths (I always naively thought palliative care was only something we ng received when we were comi towards the end of life, but that ’s just not true!)

The second is a practical ink planning tool to help you th en about what matters to you wh it comes to your care – both now and in the future.

We hope you find all of these resources help ful. As always, if you have any feedback, or ideas for further suppor t materials, do let us know.

You can email us at office@phauk.org or call 01709 761450.

Until next time!

Shaun

SPRING 2022 emphasis 53 55

Join our PH family for free today Be part of a 4,500-strong national support network.

Are you living with PH, or do you have friends and family who are?

We’re here to support people like you.

Join today and benefit from: • Support and advice • Helpful printed information and resources

Being part of the PHA UK also enables you to participate in important research, and our friendly office staff are just one call away when you need advice. Join our PH family and you’ll be joining 4,500 members in a unique network of support and inspiration.

• Emphasis magazine delivered to your door four times a year

Join FREE today at www.phauk.org Call us on 01709 761450 Email us at office@phauk.org Or simply fill in the form below and return to us.

• Fundraising ideas and guidance

• Free access to financial advice from qualified professionals via our partnership with Turn2Us

YES! I’d like to join the PHA UK for FREE Name: Address: Postcode: Email:

Telephone:

Are you a Patient n Carer n Parent n Medical professional n Other (please state)

Are you willing to take part in PHA UK surveys? Yes n No n Which specialist treatment centre do you attend?

Pop your completed form into an envelope and return to us for free to FREEPOST, PHA UK (no stamp needed).

Specialist centre standards

remain high

The latest data from the National Audit of Pulmonary Hypertension shows specialist treatment centres in the UK have maintained high standards through the COVID-19 pandemic.

The National Audit of Pulmonary Hypertension (NAPH) is the largest audit of pulmonary hypertension in the world.

It was created in 2009 to measure quality of care by collecting and reporting on data across the UK’s specialist centres, and the findings are used to inform future service planning.

Centres are measured against agreed national standards, with set targets – and despite the challenges posed by the pandemic, the latest findings show centres continued to perform well.

“Once again, this national audit has demonstrated the high levels of care delivered by our network of specialist centres. Patients and their families should feel reassured by the data, which is reflective of the hard work of teams and individuals across the NHS.” Professor David Kiely, Lead Clinician, National Audit of PH

✓

✓ ✓ ✓ ✓

See the findings over the page…

SPRING 2022 emphasis 57

Key findsirenpregsenst

How many patients, who have had at least one consultation in the last year, have had a quality of life questionnaire recorded during that time?

90 %

stic The below stati f standards at the evaluation o Each centre a national level. ed with their id v ro p n e e b s a h cuss with own data to dis at a local level. commissioners

76%

How many patients taking PH specific dru gs have had a least one annual consultation at a specialist PH centre?

94%

(83% of patients have an EmPHasis-10 score recorded)

90 %

TARG E

How many patients referred to a specialist centre were seen, assessed and diagnosed within six months?

9

97%

SPRING 2022 emphasis 58

TARGET

T

5%

TARGE T

How many patients received a right heart catheterisation befor e being given drugs to treat PH?

80%

95%

TARG E

T

A word of reassurance about your data NHS Digital collects information about you and your treatment to track your care wherever you receive it. The data within the published audit is anonymised, which means that a patient cannot be identified because their personal information is removed. If you do not want your information to be used in future audits, please contact NHS Digital by emailing enquiries@nhsdigital.nhs.uk or calling 0300 303 5678.

They will talk you through the process of having your data removed. Please be assured that this will not affect your treatment and care in any way.

At the PHA UK we played an important part in the creation of the audit by funding its first year.

Since then, we have continued to provide support and advice on behalf of the patient community.

Every year we bring you the key findings in a clear and understandable way, focusing on the statistics you told us were most important to you. We also provide anonymous patient quotes about some of the areas being audited for the NHS’s own report – another way we ensure your voices are heard.

PHA UK Chair Dr Iain Armstrong was asked to provide the foreword for this year’s report into the National Audit of PH. He said: “The pandemic has had an impact on new referrals, diagnosis and commencement of treatment – but despite the hurdles faced by the specialist centres, they have still managed to meet or exceed the majority of the standards set.

I am very reassured that the service has continued to deliver high quality care throughout the pandemic.

It is also commendable that despite having the opportunity to ‘opt out’ of this year’s audit, all of the centres chose to submit data.

Keeping patients at the centre of healthcare is vital as we move out of the pandemic. And as we start to look ahead, this report gives me great confidence in our network of specialist centres – which truly are the envy of the world.”

The audit is delivered by NHS Digital and commissioned by NHS England. It is also supported

by NHS Scotland, NHS Wales (GIG Cymru), and the National Pulmonary Hypertension Centres of United Kingdom and Ireland Physicians’ Committee.

✓ The full National Audit of Pulmonary Hypertension, including all the findings, is available by visiting www.bit.ly/nationalaudit

✓ ✓ ✓ ✓

What did the audit reveal?

All eight specialist PH centres covering England, Scotland and Wales took part in the latest audit, which looked at performance between 1st April 2020 and 31st March 2021.

At a national level, eight of the 14 established National Standards were met. Failure to meet certain standards was not unexpected due to the disruption caused by the national emergency of the COVID-19 pandemic and its impact on clinical services and patient access to them.

You can see the key findings opposite.

Some of the additional findings Standard

Result

Target

New patients should begin drug therapy within 12 weeks of referral

83%

80%

Patients receiving a PH drug should have had a PH diagnosis recorded

100%

99%

PH centres should record patient participation in research

27%

20%

76%

50%

New patients should be seen or discharged within 30 days

This data shows that between 2020 and 2021, patients were reviewed quicker than prior to the pandemic!

SPRING 2022 emphasis 59

Play your part in the

fight against PH Clinical trials have transformed medical treatment for people with pulmonary hypertension – and more patients are needed to take part in research.

In the UK, before a new drug can be given to patients, it must be shown to be safe and effective in clinical trials, and must be approved by the Medicines and Healthcare Products Regulatory Agency (MHRA).

Before there were therapies available to treat PH, people diagnosed with the condition had an average life expectancy of about three years. Now, thanks to the approval of various new drugs, this life expectancy is much improved.

Without clinical trials, and the patients who agreed to be involved in them, these drugs would not be available.

All of the specialist PH centres in the UK are involved in research, so you may be asked if you would like to take part in a clinical trial. If you can, please think about getting involved and playing your part in the fight against PH.

Interested in taking part in clinical research?

Let your specialist centre know!

I intend to continue to take part in any research, study or trial in the hope that one day we can all be cured of this disease.

Carole Ayrton PHA UK member

PHA UK member Carl Burgess lives in West Yorkshire and has been participating in studies and trials since being diagnosed with idiopathic pulmonary arterial hypertension in 2015.

I

remember research opportunities being mentioned to me pretty early on, and I saw lots of advantages, with not many drawbacks – apart from a little bit of time on my part. The motivation was the opportunity to almost be a ’fly on the wall’ and benefit from all that knowledge out there. I wanted to learn as much as I could about the condition.

I also wanted to know about any new developments as soon as they happened, and I thought that by getting involved in research I’d really be at the forefront.

I’ve been involved in a couple of studies over the years.

One was around Magnetic Resonance Imaging (MRI) scanning and how it can be used to improve diagnosis and prognosis. It involved me having additional scans and I saw that as a benefit to myself as it’s more information on my own condition. It was fascinating to see the scans of the heart pumping and see exactly what’s going on.

I’m currently involved in a study that looks at ‘machine learning’ and the use of ‘artificial intelligence’ in interpreting scans and measurements.

And I’ve also had extra bloods taken before, to be stored as part of a major study down in Cambridge. I was having blood taken anyway, so it was just a case of them taking a bit extra, and me giving permission for that data being used. That was a no-brainer really.

I’m still thinking about whether to take part in a new study that involves having a monitoring device implanted in my chest. It may be that this one is a step too far for me, as it’s more invasive, so I’m still undecided. But I don’t feel

(

t l e f s y a "I've alw d n a d e t r o su pp ” d e m r o f we ll -in Carl

rushed by the team into making a decision; there is never any pressure.

I’ve always felt supported and well-informed by the medical teams when I’ve been taking part in studies, and they always take the time to explain things to me.

In many ways, I feel I’ve gained more than I’ve contributed, in terms of what I’ve been able to learn and understand about the condition. It’s been a real education for me.

There is a possibility, even if it’s slim, that those taking part in research studies will live long enough to benefit from the outcomes. And just as my treatment has benefitted from other people’s involvement in past studies, I thought this would be an opportunity to contribute in some way for the future.

There are collective benefits in that respect.

Life expectancy and understanding of PH has increased so much over the last couple of decades and that progress wouldn’t have happened without people volunteering for studies and trials.

When you are diagnosed with a condition like PH, I suppose you either lay down and accept the cards you’ve been dealt with, or you try and fight it a little bit – and getting involved in research is my way of doing that.

SPRING 2022 emphasis 61

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