Emphasis Magazine - Winter 2023

WINTER 2023

The doctors who went the extra mile

Treatment updates The latest on sotatercept

RAISING THE PROFILE OF PH

How PH Day UK put pulmonary hypertension in the spotlight The magazine for members of the PHA UK www.phauk.org

Event news, fundraising updates, research, expert advice, and more…

Also inside

Pedalling for patients

Self-care & chronic illness

Advice from lived experience

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Hello Welcome to the final issue of Emphasis in 2023. I hope it’s been a positive year for you and your family.

The

Thank you to everyone who has shown support for our charity during 2023.

From fundraising to sharing your stories, filling out our surveys, and backing our awareness campaigns, it’s all made a huge difference to our work.

I believe there is lots to feel hopeful about as we come to the end of the year.

There are new drug developments (page 18) and a wealth of valuable research is being carried out in our disease area (page 32). Theres also huge comfort to be found in the strength of our community, so let’s look ahead to 2024 together.

last few months have been a very busy time for us here at the PHA UK, and you can read about lots of our activity in this magazine.

October saw us bring patients together in Sheffield for a special gathering featuring expert talks, with plenty of opportunity to meet others affected

by PH (see page 14). Our plan is to roll these events out with specialist centres around the UK – so do keep an eye out for something happening near you.

In November, we all came together to raise the profile of PH through our annual awareness initiative, PH Day UK. A huge number of you got involved, and you’ll find lots of photos on page 20.

To coincide with the initiative, we launched the findings of our major piece of research into what it’s like to live with PH today. The results show that people are still waiting too long to be diagnosed with PH, and our new series of films highlight the devastating impact this can have.

Turn to pages 26 and 27 to find out more.

A supplement containing an overview of the full findings of the research has been included with this magazine.

I BELIEVE THERE IS LOTS TO FEEL HOPEFUL ABOUT AS WE COME TO THE END OF THE YEAR

Best wishes for the festive season.

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

S DINISG N I F KEYIAGNOS AS JUST H /D P ING

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WINTER 2023 EMPHASIS 03

14 Our special event for patients

The day we got together in Sheffield PH DAY UK 20

16

Celebrating Rory How the Williams’ stay hopeful for their son

WHAT’S INSIDE? WINTER 2023

Raising the profile of pulmonary hypertension

Looking back on PH Day UK

LIVING WITH PH

10 “Self-care is far from selfish” 25

Looking after your mind with PH

More than a diagnosis

Andrea Hampshire on why her daughter’s PH doesn’t define her

54 Teen Talk 61

Emily Gravenstede shares how she keeps busy whilst waiting around at hospital

Accessing support from your centre Advice from Clinical Nurse Specialists in PH

This magazine is printed on paper from sustainably managed sources 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.

28

Not all heroes wear capes Some of them wear lycra!

26 WHY ARE WE STILL WAITING?

56 Scaling summits

The problem with delayed diagnosis

REGULAR FEATURES

Vicki and Kay’s epic trek for our charity

THE BEST OF THE REST

6

Upfront

18

An update on sotatercept

41

Meet the member

32

Science, medicine, hope

42 Fundraising Focus

33

46 Food for thought

40 Winter warmers

News, musings and more Say hello to Kate Lewis A snapshot of your support Expert advice on nutrition and PH

The PH drug that works differently The annual PH Research Forum

Support for depression and low mood Your chance to help yourself and others Stocking fillers from the PHA UK

Get in touch: PHA UK Resource Centre, Unit 1, Newton Business Centre, Newton Chambers Road, Thorncliffe Park, Chapeltown, Sheffield, S35 2PH office@phauk.org 0300 373 5367 @PULHAUK @PHA_UK @pha_uk_insta @pha_uk No part of this magazine may be reproduced without the prior permission of the PHA UK.

UPFRONT News, musings and more...

A deserving award for Joanna PH expert Joanna Pepke-Zaba, consultant respiratory physician at Royal Papworth Hospital, has been recognised with a prestigious European award.

In the Autumn, Joanna was named as the recipient of the European Respiratory Society (ERS) Lifetime Achievement Award in Pulmonary Vascular Diseases, recognising the huge contribution she has made in the field of PH over the years.

She was director of Royal Papworth's Pulmonary Vascular Diseases Unit from 2003-2019 and was instrumental in the organisation of the National Chronic Thromboembolic Pulmonary Hypertension (CTEPH) programme. Her research concentrates on CTEPH and PAH and she has published over 200 papers and trained generations of research fellows.

Partnership aims to help people with PH enjoy a better quality of life Moving more - within your capabilities - is proven to be safe if you have pulmonary hypertension, and it can make a big difference to your quality of life.

2024 will see the PHA UK partner with clinical exercise physiologist Dr Ciara McCormack (pictured) to produce information and resources to help you get more active, answering common questions and concerns and guiding you through your journey to increased wellbeing.

Ciara works with pulmonary hypertension patients in Dublin and has extensive experience of helping people with the condition benefit from physical activity.

WINTER 2023 EMPHASIS 6

"Educating, encouraging and supporting people with pulmonary hypertension to engage in physical activity is something I am very passionate about. I am really looking forward to working alongside the PHA UK to help those living with PH have access to information and resources to assist them on their journey to becoming more active."

Ciara McCormack

Your letters

>Dear PHA UK...

I am attaching a picture of my son Dominik, age 7. Here he is after getting his green and white belt in Jujitsu. He is pictured with one of the leaders, Sensi Kirsty.

He has only been recently diagnosed with pulmonary hypertension and it’s been a bit of a whirlwind journey since then, getting used to all the hospital appointments.

He has had two appointments now at Great

I want to let you know about my CTEPH story. Last year (2022) I was out walking and became breathless, and after a short time in A&E I was diagnosed with blood clots in both lungs.

A few months later I was referred to the specialist centre in Glasgow where I was diagnosed with Chronic Thromboembolic Pulmonary Hypertension (CTEPH).

This was pretty scary at first but the team at the Golden Jubilee Hospital were brilliant and gave me lots of information.

I was later called back to see them for some tests, and I was advised that the blood clots

Ormond Street Children’s Hospital who have confirmed his PH is a result of his underdeveloped lungs (due to a right-hand side congenital hernia he was born with).

He would love to appear in your magazine, as he faces other challenges when doing sports, as well as his PH. Laura Pavlikova

were causing damage to my heart, that my arteries were clogged, and I would require an operation called a Pulmonary Endarterectomy (PEA).

I agreed to have the surgery and it went ahead in July. The worst bit was getting woken up at 5.30am to have my chest shaved!

I’ve now had my three-month post-operation check and the good news is my surgery was a success and I can now return to normal activities.

I feel so much better. A big thank you to everyone in the NHS! Euan McMurtrie

From the minute I arrived at Royal Papworth Hospital for the PEA the whole team, from the surgical team to the nurses, physios and catering staff were absolutely brilliant. I was really well looked after and I want to say a big thank you to everyone who has been involved in my care.

Got something to say?

Email us at media@phauk.org or write to: Emphasis editor, PHA UK Resource Centre, Unit 1, Newton Business Centre, Thorncliffe Park, Sheffield S35 2PH. You could see your words published in the next issue of this magazine!

More UPFRONT WINTER 2023 EMPHASIS 7

UPFRONT News, musings and more...

We asked, you said…

We asked our Facebook followers for their highlights of 2023

Lauren John

Julie Greenway

My son Noah aged 3 made me happy this year. Even with my IV line 24/7 I can still get out and enjoy my time with my son I’m looking forward to marrying my partner next August! 2024 is going to be a happy year x

My highlight was raising over £200 for the PHA UK

☺

Melanie Hatton

Nicki Ross

I managed to have two trips away this year, in Aviemore and central Scotland. I got to go to a concert to see my favourite band, went with my husband Jamie to see one of his guitar heroes performing, and on a cinema trip. All in all I’d say 2023 has been an incredibly successful year for me, especially given I only got the PH diagnosis in October 2022. The support from Golden Jubilee Hospital staff, the PHA UK and their Facebook groups has been amazing, and makes dealing with this condition so much easier x

WINTER 2023 EMPHASIS 8

My daughter Daisy’s highlight would most definitely be meeting Mirabel from Encanto And I think my personal highlight is seeing Daisy start school. When we were first given the diagnosis of Daisy having PH there were many dark days when I didn’t think I’d ever get to see her in a school uniform - and now she’s absolutely loving school and even received Star of the Week for her balancing skills in PE! She’s a superstar every day

Your voices heard in Europe

People with PH went through a lot during the COVID-19 pandemic, and many of you shared your experiences as part of our 2022 research project.

The findings of this survey were displayed at a prestigious conference in Milan in September, putting your voices in front of hundreds of delegates at the European Respiratory Society (ERS) congress.

You can access all the findings, and stories from over 30 people affected by PH during the pandemic, by visiting our dedicated website www.mypandemic.co.uk

IN BRIEF NEW LEAFLET TO HELP PEOPLE DISCOVER OUR CHARITY

A compact new leaflet has been published to help people find out more about the PHA UK. Order your free copy at www.bit.ly/GetToKnowPHAUK

STAY INFORMED WITH OUR MONTHLY E-NEWSLETTER

Get the latest news from the PHA UK direct to your inbox each month! Sign up for our monthly email digest at

Kind words on social media Produced by

the PHA UK

@bump_tobaby21 Forgot I had CHILDREN ordered these online from the PHA site! Some information booklets about pulmonary hypertension arrived today! They are so well written with some great info included from the consultant and nurses at Great Ormond Street. I’d highly recommend looking at their resources if you know someone affected by PH!

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Help Contacts

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www.bit.ly/PHAUKmonthlydigest

DON’T FORGET YOUR VACCINATIONS!

If you have PH and you haven’t already received your flu and COVID-19 vaccinations, it’s important to arrange to have them as soon as possible.

It’s the best way to protect against winter viruses and stay as well as possible through the colder months.

@Firegoddess_8 The PHA UK supports patients living with Pulmonary Hypertension together with their families. I’ve been living with this incurable, life-limiting condition personally for nearly 8 years and have been helped immensely by the work of this amazing charity.

WINTER 2023 EMPHASIS 9

After her own struggles following her PH diagnosis, Sophie Papageorgis now works as a psychotherapist and is passionate about helping people with their mental wellbeing.

We caught up with her to hear about her pulmonary hypertension journey, and why she wants to share her advice for others with this rare disease.

Self-care is far from selfish WINTER 2023 EMPHASIS 10

Bodies and minds are so incredibly linked How long has PH been part of your life?

When I was five, tests showed the pressures in my lungs were really high because I was developing Eisenmenger’s syndrome as a result of being born with a hole in my heart. I was 16 before I started getting symptoms though.

I was breathless, fainting, and generally feeling awful. There was a long period of repeatedly going to my local A&E and being told I was having panic attacks because I was doing my AS Levels and I was a teenage girl.

Eventually I was diagnosed with pulmonary hypertension and although I didn’t want to hear it, there was relief that at least it wasn’t in my head. I was started on treatment at this point, and as it was 20 years ago, there weren’t so many drugs available.

What impact did the diagnosis have on you?

It all had an awful effect on my life.

My GCSEs were out of the way, but the symptoms started when I was in my first AS Level year, and I got really depressed – to the point where I had a psychotic episode.

I did take my AS Levels, and then my A Levels, but it was difficult as I’d missed so much school. After my exams I took a year out and did nothing, because of how traumatic everything had been.

I received some help from a therapist (although I was reluctant at first), and I then saw a psychiatrist and spent some time on medication. Things got better but it wasn’t the easiest time back then. It’s horrible when you’re not in control and your body isn’t doing what you want it to do.

What led you to your chosen career path?

My own experiences of my mental health, especially during that time, impacted my desire to become a psychotherapist.

Looking back, I do wish I had been more open to the psychological support that was made available to me. It would have made a huge difference.

I was working in customer services for an online retailer when I started volunteering for Samaritans as a listening volunteer, and that’s what inspired me to finally make the change. I trained for three years and qualified as a psychotherapist 18 months ago.

Psychotherapy is so rewarding because I feel like I’m doing something worthwhile.

It’s amazing seeing how people change.

Some may have very low self-esteem, or are very traumatised, and working through it can make such a huge difference.

I’m constantly learning, and I learn so much from all my clients; they are amazing.

How do you balance being a psychotherapist with the challenges of PH?

These days I get breathless when I walk, and I get so many chest infections. I also get a lot of headaches, because of the oxygen levels, but I generally push on through as I enjoy working. I do recognise that I need to take some of my own advice and practice self-care!

It’s important not to self-disclose with clients, but everything I’ve been through does help me to empathise with people, especially those facing health challenges.

My personal background gives me a unique insight into what people are feeling, including all the anxiety and the existential worries.

Continued...

WINTER 2023 EMPHASIS 11

Dealing with the emotional impact of PH is huge Has your work as a psychotherapist helped you too?

I think it does help to know I’m not the only one who has experienced these things.

I’m still not very good at showing when I’m sad about things but I’m getting better at voicing it.

I’ve had a lot of psychological support as an adult. When you’re training to be a counsellor you have to be in therapy yourself, so I’ve had years of therapy, and it’s still ongoing. I’m still working on myself in that respect, and I understand that it’s a lifelong journey.

Why is it so important to take care of mental wellbeing when you have a physical illness?

Bodies and minds are so incredibly linked. An illness like PH can take so much out of you physically that you need to build yourself up however you can, so practicing self-care and showing yourself compassion is really important. PH is hard, and people don’t always get it.

The phrase ‘self-care’ gets thrown around a lot. It means prioritising yourself, and making sure you’re looking after yourself holistically (all of you). It’s about doing things that make you feel that you’re important, and that you’re worth doing things for.

It could be looking after what you eat, taking some exercise if you can, or having a day in bed because you’ve run out of energy. That’s ok too.

Self-care is far from selfish. You can still think about other people whilst thinking about yourself and you do need to take care of yourself, especially when you have a condition like PH that other people can’t see.

WINTER 2023 EMPHASIS 12

You’ll be sharing your advice for people with PH over the next few issues of this magazine. What made you want to do this?

I got in touch with the PHA UK to offer my help because it’s a cause very close to my heart, and I think what the charity does is amazing. I’ve had some fantastic support from them in the past.

The mental and physical aspects of chronic illness are so intertwined. Dealing with the emotional impact of PH is huge and I think it’s great that it’s incorporated with everything the PHA UK does.

I know that many people with this condition feel worried or anxious and have struggles with their mental health.

Although I can’t change anything about their personal circumstances, I’d really like to do what I can to help.

Sophie lives in Surrey and provides counselling and psychotherapy services for adults, children, and couples. She is a registered member of the British Association for Counselling and Psychotherapy.

Read Sophie’s top tips on taking care of your mental wellbeing with PH opposite.

If you have a question you’d like her to answer in the next issue of this magazine, please send it to media@phauk.org

SOPHIE'S TIPS

Taking care of your mind with PH Practice self-care Self-care is vital for everyone. It not only helps to keep us as healthy as possible, but it boosts mood, shows us we’re worth taking care of, and reduces anxiety.

Self-care can be anything from sitting quietly for five minutes in nature, to reading a book, focusing on spirituality or religion, taking a short walk, doing artwork, having coffee with a friend, keeping a gratitude journal, or playing Tomb Raider (just me?!).

It can be anything which nourishes you physically, mentally or emotionally.

Listen to your body It can be hard when you’ve got a to-do list in front of you that you need to work through, and although your mind is willing, your body may not be. Or the associated brain-fog may be getting in the way. Let’s face it, having pulmonary hypertension is not always a bed of roses.

If your exhaustion levels are high or you’re

struggling with your breathing, your body may well be telling you it’s time to take a break.

And that can be deeply frustrating. Try to hold self-compassion, know that you’re doing your best, and accept that to-do list can wait for another day.

Don’t be afraid to say no Other people don’t always get what it’s like living with a long-term condition which impacts us day-to-day. I know I find myself wanting to keep up with others, not ‘letting them down’ or not showing them that I’m struggling at times.

As a self-confessed 'people pleaser', I understand how hard it is to sometimes say no to others.

But there’s only so much that we can do, and although it’s difficult to admit, we can’t always keep up with others around us.

Everyone is entitled to say no to things they don’t want to do, and sometimes we need to do a little bit of work on our assertiveness skills to be able to prioritise ourselves.

Focus on your goals

Get emotional support

However big or small, goals keep us focused, motivated, and often make us pretty proud when we complete them (tell me I’m not the only one who adds things I’ve already done to the bottom of my list, purely to cross them off and celebrate?!)

Goals can be anything, but they might include something like learning a new skill, making a new recipe, increasing walking steps, or finding a new career path.

Even at times when things feel rough, if we’re working towards our goals then we’re on course to making ourselves happier and more fulfilled.

As we all know, living with a long-term condition, and coming to terms with its impact, is not always easy.

It’s hard going at times, and it can feel isolating.

If you find yourself struggling, feeling sad, anxious, or depressed, maybe it’s time to reach out for support. Talking to a friend or relative, someone on the PHA UK Facebook group (see page 59), or even a counsellor, can help things feel a little easier.

Sharing the burden and the worries, and knowing that you’re not alone, is a huge support with mental health and wellbeing.

Look out for more advice from Sophie in the next issue...

WINTER 2023 EMPHASIS 13

The day we got together in Sheffield

“I came away with a good feeling for the future”

After years of being kept apart due to COVID-19, it felt extra special to welcome patients, loved ones, and healthcare professionals to our first ever ‘Together’ event in October

Held in partnership with the Sheffield Pulmonary Vascular Disease Unit, the gathering was open to anyone cared for by the team at the Royal Hallamshire Hospital.

Expert talks throughout the day covered topics including treatments, research, nutrition, and physical activity – with a focus on empowering patients and their loved ones through developing knowledge and understanding.

Those in attendance were also able to give feedback to their specialist centre and discuss improvements and plans for the future.

The event was a valuable opportunity to meet others affected by PH, and many new friendships were formed.

With Storm Babet battering Sheffield the weather may have been against us, but the rain failed to dampen spirits during a day of learning, connecting, and finding strength amongst others.

We’ll be holding more of these free gatherings in collaboration with specialist centres around the UK, so look out for a Together event near you soon.

“Very informative and it was nice to see other people living with the condition”

“A big thank you to everyone… it was lovely to meet PH friends” WINTER 2023 EMPHASIS 14

“It was a brilliant day… nice to all get together”

“Thank you for a brilliant day”

The expert talks delivered at the event were captured on film to enable as many people as possible to benefit. Watch the talks, along with highlights from the day, by scanning this code or visiting www.bit.ly/TogetherSheffield2023

“I loved it, everything was so easy to understand”

“Well organised, fun, informative… fantastic to see lots of other people in the same boat”

“It gave me hope and showed you are part of an army, not a standalone soldier” WINTER 2023 EMPHASIS 15

Celebrating Ro As a happy and adventurous toddler, you’d never know that three-year-old Rory Williams has pulmonary hypertension. His father Dan shares their family’s story and explains why it’s important not to lose hope.

W

"

e had some scans in the later stages of pregnancy, and they showed that Rory had a low heart rate.

The original diagnosis we got was a heart block (congenital) which is completely unrelated to his PH. We were told his heart wasn’t communicating the way it should have done and after being born by c-section, his heart rate dropped considerably.

At one point it was just 19bpm and he was fitted with a pacemaker the day after being born.

About six months later, Rory was down for a nap and my wife Becky went to get him up. He had a high-pitched cry, blue lips, and wasn’t very responsive. She called 999 and that’s when the craziness started.

Rory was in hospital in Newcastle for seven weeks having tests. Eventually he had a right heart catheterisation and they had to administer nitric oxide before they could take measurements as he quickly became unstable. The measurements showed his pressures were through the roof, so at that point the staff knew they were dealing with pulmonary hypertension.

Newcastle has a great link with Great Ormond Street Children’s Hospital so one of the lead consultants there was able to explain everything. We were advised not to Google it, but obviously we did, and it was overwhelming.

It felt like we’d finally figured out the pacemaker bit, and now we had this to deal with. It felt like the end of the world.

WINTER 2023 EMPHASIS 16

We then stumbled across the PHA UK website, and joined the charity and received their information pack.

That was so useful in helping us explain everything to family, including our parents. Becky and I were still in the process of dealing with it ourselves, so the booklets enabled us to help everyone else get their heads around it.

It was too raw for us to have to describe it to everyone else, so the PHA UK booklets were the perfect remedy for that.

Life as a toddler

Rory is currently on two different oral medications, and he is doing so well at the moment. If you looked at him, you would not think he had this condition.

He is just a typical toddler, and we feel so lucky.

At this moment in time his body is very accepting of this medication.

We hold our breath before every medical appointment but every time so far, we’ve been told he’s doing really well.

He loves anything to do with Spiderman, and animals, and annoying

his big sister Phoebe. She is six and she’s very patient and understanding of everything. She knows she can’t go full tilt with him (even though he wants to!).

We talk about the medication with them both. If you had a conversation with Rory, he would tell you about the ‘box in his belly’ so he understands that he’s slightly different. It’s so normal for him that he will come to me and say, “daddy it’s time for medicine”.

Because he doesn’t look or act like there is anything wrong, we haven’t had to explain the PH to many people.

He’s starting nursery in January, so maybe there will be some questions then. I think it will come to a stage when he will realise he is a lot more different from other kids his age, and we’ll cross that bridge when we come to it.

How does Rory inspire us? He just plows on, and he’s fully committed to doing everything he does. Nothing stops him getting on with life. He likes to climb sofas and jump off them, he has no fear about life, and for me that is really inspiring.

The importance of support

We’ve had so much support from both sets of our parents. Becky was in hospital with Rory for seven weeks, breastfeeding and unable to get home to Phoebe, who was only three. It was the hardest time.

This was spring 2021, and some covid restrictions were still in place, so it was a few weeks before Phoebe was allowed to go and see her new baby brother.

We’d spent the first six months of 2020 in lockdown as a family together every day, so to have the family torn apart like this was so difficult.

The amount of strength Becky showed was incredible. At that time, we could never have imagined things being as they are now.

We thought things would never be the same again, and we’d never be able to

< Rory at one day old, just before his pacemaker was fitted

ory "How does Rory inspire us? He just plows on..."

“I was overwhelmed when I heard the kids had chosen the PHA UK in support of Rory”

enjoy being a family. But now, we just feel ‘normal’, and at the moment, we’re feeling good about the future.

Our advice to other parents facing a new diagnosis of PH is simply not to lose hope. Things can change, medicine is advancing all of the time, and every single case is different.

There are trials going on all the time, and some of the results are looking promising. We know we are in a lucky position with Rory at the moment, but everyone is an individual. Lean into the support that’s available.

"

Rory’s father Dan is a computer science teacher at The Grammar School Leeds, and in a huge show of support, the whole of year ten chose to raise money for our charity by taking on the Yorkshire Three Peaks challenge.

Aiming to scale the peaks of Pen-y-Ghent, Whernside, and Ingleborough, the youngsters and 30 staff generated more than £11,500 in sponsorship. Unfortunately, multiple attempts to complete the challenge were thwarted by unsafe weather conditions – but the funds and awareness raised is truly an achievement to be proud of.

Dan said the students often ask about his son, after his condition was discussed during a biology lesson. “I was overwhelmed when I heard the kids had chosen the PHA UK in support of Rory”, he said.

“To launch the walk, we had a special assembly and I spent half of it talking about PH and the PHA UK, using the resources and videos from the PHA UK website. I shared them along with photos of Rory to show that PH is a hidden disease, as you wouldn’t know he has it just from looking at him.”

We are hugely grateful to the pupils and staff at The Grammar School Leeds for this incredible support of our charity.

WINTER 2023 EMPHASIS 17

SOTATERCEPT: An update Earlier this year, we brought you news of the PAH therapy sotatercept – which is the first drug to target the root cause of the disease. But as 2023 draws to a close, are we any closer to it being made available?

We spoke to Dr Mark Toshner, PH Specialist at Royal Papworth Hospital and Associate Professor at the University Q. What excites you about this treatment? of Cambridge, to find out.

Mark: This drug is a really big step Q. Thanks for chatting to us about this, Mark. First of all, can you remind us what makes this drug different?

Mark: Sotatercept doesn’t work like the other drugs that are licensed for pulmonary hypertension - known as vasodilators - which all work by opening up the blood vessels.

Sotatercept arose from some of the work done in genetic forms of the disease, and in particular in a pathway known as the bone morphogenetic protein type 2 receptor (or ‘BMPR2’ pathway).

Essentially, the drug works by changing the expression of proteins that we know underly the familial [genetic] forms of the disease.

So, rather than just opening up vessels, it works more directly on the underlying ‘problem proteins’ that are causing the disease in the first place.

Sotatercept is also different because it’s taken in the form of nearly-monthly injections

WINTER 2023 EMPHASIS 18

forward because although the last 20-30 years has seen a lot of changes in the treatment options that we have available for pulmonary hypertension, all those drugs work in the same sort of way, so the benefits have been incremental.

This appears to be a different mechanism, and the results [of the trials] are as good as everything we’ve seen in the last 20-30 years.

Since I last spoke to you at the PHA UK a year ago, the results of the full big trials of sotatercept have been released and we’ve been able to comb over them. The good news is that the drug seems to be having a very significant effect in a very wide group of patients with pulmonary arterial hypertension.

The majority of patients who were in the clinical trials were already on a significant therapy package, so this appears to add additional benefit on top of the therapies we already have, which is what’s exciting me the most at the moment.

Q. It’s encouraging to hear how promising the trial results have been. The question everyone has, is how long might we be waiting for it to become available?

Mark: Sotatercept has not been licensed anywhere around the world yet, and there is likely to be a bit of a delay before it’s licensed in the UK and is in widespread use.

What comes next is that NICE (the national body that makes treatment recommendations) will do an appraisal of the drug, starting in March next year (2024) and that will take a number of months.

This will involve them deciding what they think the added benefit of the drug is, balanced against its cost. So, we’re not there yet, but we would expect some decisions to start to be made in 2024.

Q. Who will be eligible for this treatment if it’s made available?

Mark: We don’t know yet what the decision of the regulator and the reimbursing authorities will be, so we don’t know exactly where it will fit.

However, it will be used within a group of patients that are similar to those the drug was trialled in - so those with group one PAH. It will likely be available for patients who are already on a significant amount of vasodilator drugs, most likely at least dual therapies. It will almost certainly be an ‘add-on therapy’ in that context.

Beyond that, we don’t know what the specific limitations will be and where it will fit in the treatment guidelines.

It’s important to remember that there are still some open questions, for example who the drug works best in, and how it should be administered. As a result of that, there are still ongoing trials across the UK that most of the specialist centres are signed up to.

If you are interested in being part of these trials, please speak to your PH team to see if you are eligible for them.

than all the vasodilators. It tells us that we can alter disease, beyond just opening the vessels up.

I expect there will be a subsequent generation of new drugs coming on the back of this. So, this will be the start of the next phase, or evolution, of how we treat patients.

I think we can be pretty optimistic that the last 20 years have seen a change in life expectancy and quality of life for patients,

and in the next ten years and beyond we should be seeing further gains still.

I think what we’re talking about here is the capacity to do something different in this disease area and that is definitely a message of hope. It’s a really exciting time.

As we come to the end of this year, and the end of a difficult few years [with the pandemic], I think we’re in a much more hopeful position than we’ve been in for some time.

.

Scan this code or visit www.bit.ly/SotaterceptUpdate to watch PHA UK Chair Dr Iain Armstrong explain how the NICE appraisal process works.

“It’s important to remember that there are still some open questions”

Q. It sounds like there is a lot to feel positive about. Can you sum up why developments with this treatment should give people hope?

Mark: This is the first drug really that we’ve had in pulmonary hypertension that works by a different mechanism

WINTER 2023 EMPHASIS 19

E H T G N I S I RA PROFILE OF Y R A N O M L PU ION

Naomi Bell covered 6.5 miles in memory of her sister

HYPERTENS

e for r community unit ou w sa r be em ov N Friday 3rd s campaign to put es en ar aw al nu PH Day UK, our an light.

PH under the spot

Walking & Wheeling for Hope...

Walk for Hope

Adam jogged a mile in memory of his granny

d Little Archie walke e th ith w for his cousin r lle help of his stro

Helen Keith and her friends covered 14 miles in support of Helen’s son Charlie

Sally Parker's dogs walked with her in memory of son Josh WINTER 2023 EMPHASIS 20

Matthew Calve rt and his trike were joined by his classmat es

Chris Wiltshire walked a full marathon for his wife

Lou Chadb u took almo rn st 27,000 ste ps

11 :29

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@lou.chadburn WINTER 2023 EMPHASIS 21

What else you got up to…

Our newly-launched awareness packs went down a storm, with well over 100 orders and many of you using the contents to spread the word about PH far and wide. Posters went up on shop doors and noticeboards, factsheets were shared, and there were many letters sent to GP practices. Cakes were crafted, biscuits were baked, and member Myra Morrison even produced some artwork.

Healthcare professionals from across the UK's specialist centres also showed their support in different ways.

WINTER 2023 EMPHASIS 22

PH DAY UKNOV 3r d

2023

A huge thank y to every ou on who go e involve t d!

n a h t e r o M s i s o n g a i ad pshire’s had to am H e th s, er th o Like so many have the tests to ie b b A r te h g au push for their d ad to a pulmonary le ly al tu en ev ld u ort that wo part of their supp s A s. si o n g ia d n o hypertensi wrote this blog a re d n A m u m , K for PH Day U ld daughter’s o rea -y n ve se er h to explain why ever define her. n ill w s n io it d n co health

We

are believers of the benefits of having a diagnosis. It lifts a weight of worry for everyone who has spent so much time and effort looking for help and can provide focus for a pathway of support.

It is both life-changing and reassuring at the same time.

You know that you have a challenge to face, life might change, but it can provide hope and the determination to keep going. Whether that’s to find the best treatment, a cure, or the goal of living your best life possible.

This is what having a diagnosis means to us, but it doesn’t define everything we do. She will always be Abbie first. A child first, with the right to aim high in all areas of her life that make her happy.

A diagnosis will always come second to that.

Her many conditions are most certainly not what our daughter means to our family. She is surrounded by people who are of the same mindset: that she has many strengths, and together we

will celebrate her achievements in life for as long as we can.

Abbie was born with congenital scoliosis, and it was when she was 18 months old that we raised a concern as she was struggling to lift herself from the floor, she couldn’t walk, breathing seemed such an effort, and she appeared so poorly.

It was explained away that it was probably due to wearing the plaster spinal casts full time, but they would do a routine echocardiogram to check.

Pressures in her lungs showed some elevation but there didn’t seem to be any major concern.

So, we carried on with life and continued to watch her lay horizontal most of the day, pale, and quite frankly lifeless, while other children her age were enjoying achieving those wonderful early years milestones.

Instinctively, we knew this was something more serious. It broke our hearts to see her unable to enjoy her early years and everything became so overwhelming.

By this time, we

decided to raise our voices and keep asking for a second echocardiogram.

We stopped worrying about upsetting the system. We decided as long as we were being respectful, just keep asking!

By the time she had her second echocardiogram, they found the pressures in her lungs were now dangerously high and her heart was enlarged. The professional in the room was so concerned, she escalated the results that day and early the next week Abbie had a heart catheterisation procedure. We received the diagnosis of pulmonary hypertension and she was transferred to the care of Great Ormond Street Children's hospital. Continued...

WINTER 2023 EMPHASIS 23

Thankfully, her journey since diagnosis of PH has been stable since she started her medication. Although she still gets breathless and very easily fatigued, she can do so much more than laying quietly on the floor watching everyone else enjoy all that life has to offer.

Abbie’s PH is a result of her restricted lung development, which is in turn, a result of her new diagnosis of progressive kyphoscoliosis. We have been told the risks outweigh the benefits of any spinal operation now, so PH will be part of her life for as long as we can foresee. But with the diagnosis, treatment and support in place, Abbie can do so much more and feel like a little girl with dreams, interests and strengths to pursue.

In her powered wheelchair she can be more independent on family days out, keep up with her two big brothers, or arrive with a smile to the ward when we go to the hospital for appointments.

She has the best distraction techniques I have ever heard and knows just how to delay professionals from their tasks.

“What does that do?” she will say.

“I am not ready yet” and, “just one more minute” are her very best lines.

She loves music and dancing, and with her walking frame so she can now join in with her Saturday morning ballet class.

Even if only for a few minutes, it doesn’t matter, as she feels like a real ballerina.

Her stairlift at home means she can go upstairs to play in her room or shut her door when she needs a bit of space. She has oxygen therapy set up at home, school and on the go so when she needs a break, she can have one. This helps her get through the day better, conserving her energy and allowing her to attend school full time. With an adapted downstairs toilet, she can get ready at her own speed and arrive at school with all her bags and equipment, ready to learn.

After watching her struggle to participate in her early years, she is now flourishing at school.

She enjoys reading and is writing much more independently without getting tired. Only this week she

She has made so many friends at school who love her for who she is Abbie is now seven years old and is most certainly living life to the fullest in her own unique way. She is always smiling or being funny, and she even has a stubborn streak that pops out every now and then. She is incredibly determined and will stand up for what she believes in, even if it causes a few turn-taking or sharing issues. She is wary of strangers, but she builds trust and compassion in the most amazing way.

WINTER 2023 EMPHASIS 24

wrote a letter to the Prime Minister voicing her views on sea pollution.

She has made so many friends at school who love her for who she is and have been so accepting of all her equipment and tiredness. She loves to stand up in ‘celebration assembly’ and achieve star awards for having courage to keep going with her learning.

She is joining in with her first outdoor adventure trip with school this year and wants to join the school choir one day.

She has the best nurturing relationship with all her teachers, and they really do know her well.

They make every effort possible to ensure she is included and able to try everything on offer, with adjustments

Walk for Hope

Wheeling for Hope

Abbie showed just how determined she is when she completed a 10km Wheel for Hope in support of PH Day UK last month.

With her trusty powered chair Ruby - decorated in PHA UK balloons - she smashed the challenge to raise almost £1,500 for our charity. specific to her needs. She has, however, earned a couple of speeding tickets when in her powered chair ‘Ruby’ in the playground because she doesn’t like to go slow!

This is all worlds away from that heartbreaking time before her diagnosis when we thought life would just pass her by.

A previous life where we thought all our time would be focused on trying to figure out what was wrong so we could get her the help she needed. But now we can focus on ensuring she lives life to the fullest.

Today, she is happy and thriving and we are so thankful that she is supported by so many wonderful people and professionals who see Abbie, the child, first.

So, with hope and determination always in our minds for Abbie, she has the potential to achieve so much now, and she is certainly more than her diagnosis.

Mum Andrea said: “The sun was shining, the sky was blue and we felt very blessed to feel the positive atmosphere around her. It was such a special moment to watch as groups of friends walked a lap (or more) of hope with her.”

The family appeared on BBC Radio York to talk about the challenge and raise awareness of pulmonary hypertension.

WINTER 2023 EMPHASIS 25

LIVING WITH PH TODAY

WHY ARE WE STILL WAITING? PH Day UK saw us share the findings of our major research into what it means to live with PH today.

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A supplement containing an overview of the findings of the survey was included with this magazine.

of you completed the survey, and some of the key findings showed that people are still waiting too long for a diagnosis of pulmonary hypertension.

A third of respondents said they waited over three years to be told they had the disease, compared to 10% of those surveyed in 2016. This means the figure has trebled in the last seven years.

The findings also showed that the number of patients waiting over a year to be referred to a hospital by their GP - after reporting symptoms - has doubled. In 2016 this represented 21% of patients, compared to 40% in 2023.

Almost a third of patients had to see four or more doctors before receiving their diagnosis, and for 51%, it took over a year to be diagnosed with PH after first noticing symptoms.

The PHA UK has conducted a survey into the lived experiences of the disease every few years since 2007, and the results consistently show that despite significant advances in treatment, the long waits for diagnosis have remained.

The 2023 survey was completed by 859 people with PH in the UK – making it the charity’s largest ever study into the lived experiences of pulmonary hypertension. As well as experiences of diagnosis, it asked patients about the impact of treatment, satisfaction with the specialist PH service provided by the NHS, and how the disease affects different areas of their lives.

The results of this research will help us set our strategic priorities for the next five years, underpinning our charitable activity and determining the direction of the PHA UK. The findings will also be used as an evidence base to campaign for access to new treatments or policy changes, and to protect specialist service provision.

Thank you to everyone who responded to this survey, proving once again that people with PH may be breathless, but they’re certainly not voiceless.

Scan this code to watch Dr Iain Armstrong, Chair of the PHA UK, discuss the problems with delayed diagnosis.

WINTER 2023 EMPHASIS 26

You can read the full report by scanning this code or visiting

www.bit.ly/BreathlessNotVoiceless

KEY FINDIN GS /LIVING WITH PH PH IM

PACT OF PEOPLE S MANY AREAS ’S LIVES

96 84 79 %

of people with PH say the conditi has an imp on on their oveact quality of rall life

%

say it has an impact on their mental or emotional wellbeing

%

say it has an impact on their abil ity to work or attend education

78 6 0 %

say it has an impact on their relationship or family s

say it has an impact on their financial situation

%

DELAYED DIAGNOSIS: WHY CHANGE MATTERS A powerful film released by the PHA UK has highlighted the problem with delayed diagnosis and why change is so vital.

Released on PH Day UK, the video tells the stories of nine PHA UK members, who all faced significant challenges to get their pulmonary hypertension diagnosed and start treatment.

Watch the film now by scanning this code or visiting bit.ly/WhyAreWeStillWaiting

HEAR THEIR VOICES

You can watch each individual story by scanning this code or visiting bit.ly/WaitingForMyDiagnosis.

Subtitled versions of all the videos can be found here too.

Thank you to everyone who helped us make this valuable series of films, which are already having an impact.

WINTER 2023 EMPHASIS 27

s e o r e h l l a

) t ! a r o c y l N(some wear

On Irish soil

on embarbklin o t g n i Prepalarst leg to Du the

belfast Cycling from Sheffield to Dublin is no easy task, but a group of PH consultants from specialist centres across the UK and Ireland made light work of it at the end of September.

They covered over 250 miles, starting from the Royal Hallamshire Hospital in Sheffield before heading over the Pennines to Liverpool. After an overnight ferry to Belfast, the determined doctors wheeled down the country - through characteristic Irish rain before reaching the Mater Hospital in Dublin.

Pedalling for patients over three days, the group raised almost £8,000 for the PHA UK and PHA Ireland combined. And when the going got tough, they were inspired to push on by thinking of people with pulmonary hypertension.

WINTER 2023 EMPHASIS 28

Dublinline finish g PHA UK ge thankin dliffe said : a s s e m t oin , Dr Con In a mid-p ir support e th r and fo rs membe er ending ups

ay of nev h “It's been a bds and descents). Althougo downs (clim of a PH patient may alsd each the journey ps and downs, we pulle too." have some u as we hope to help you other along

Scan this code to watch nge footage from the challe

wear cape s

A HU THA GE YOU NK US AFROM LL!

Meet the cyclists: Dr Martin Johnson

Golden Jubilee Hospital (Glasgow)

est arn e d r e l l e w A

Dr David Welsh

break

Caledonian University (Glasgow)

Dr Jim Lordan

Freeman Hospital (Newcastle)

Dr John Cannon

Royal Papworth Hospital (Cambridge)

Prof Robin Condliffe

Royal Hallamshire Hospital (Sheffield)

Prof Sean Gaine & Dr Brian McCullough Mater Hospital (Dublin)

Dr Emmett Major

Liverpool

Liverpool the e Crossiinnges on day on n Pe n

Belfast City Hospital (Belfast)

The t off freoamm before set Hallams the Royal ting hire Hos pital

sheffiellidne start Prof Condliffe’s The group was joined by Consultant and friend Steve Saunders, on of the Sheffield Pharmacist Neil Hamilt e Unit drove a as Pulmonary Vascular Dise em. th support van alongside WINTER 2023 EMPHASIS 29

My mum,

her stren Lucinda loved her granddaughter Rebecca WINTER 2023 EMPHASIS 30

Jason Smith lost his mum Lucinda Duxbury-Smith in July, but he’ll always remember how she made the most of life with PH. These are his reflections.

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lovely; you wouldn’t have thought there was anything wrong with her.

I went to all of mum’s early hospital appointments with her, driving her up and down to London from Kent. I used up my entire annual leave allowance doing that during the first year she was diagnosed. When she was more comfortable with things, she started attending the appointments on her own, using patient transport.

Mum carried on swimming, which she loved, and in the years before her death she joined a local rock choir.

M

um had just turned 70 when she died. She was diagnosed with PH in 2004, so almost 20 years ago, and in 2021 she was told she had lung cancer and was given a year to live. But she continued to do everything she wanted to, and I felt I could accept the diagnosis too because she carried on like there was nothing wrong. She was amazing.

I was in my late twenties when she was told she had pulmonary hypertension. I remember going round to her house and she was upstairs crying. I asked what was happening, and she told me about the illness. It was quite a shock.

Mum dealt with the news by deciding that she was going to have a really good holiday first, and then decide where to go from there. We went on a cruise together and it was

familymatters

Jason and Lucinda on a cruise just after her diagnosis She also took a college course in silver smithing, gaining her a qualification to get her own hallmark. She was an antiques collector, and she loved her little silver campervan.

She adored my daughter, her granddaughter Rebecca, and would often take her out. She also loved my partner Heidi, who I’ve been with throughout mum’s illness. Heidi understood her frustrations really well.

Mum had a lot of friends in the PH community and the support she received from people in the PHA UK Facebook group really helped her.

She believed in spirituality and healing, and I think that gave her the strength to carry on. Things did get her down at times, but she’d get straight back up and have another go. Over time we did see her deteriorate, but she would never give up.

She always had respect for herself and others and she supported me during difficult times. She always had time for people, even when she was ill, and she was very caring and very loving.

Even when she was in hospital at the end, she was courageous. The night before she passed away, she stepped up out of her chair - because she refused to lay in her bed - gave me a hug and said, ‘I really love you’. That was the last time I saw her.

I think mum’s sheer will, determination, and her positive outlook is what kept her going all these years. And I think that spirit will stay with me.

I’m sharing this story because I hope the experiences we’ve had as a family might help those who are recently diagnosed and worrying. Mum had 19 years of good life. She was an absolute hero, and I am so proud of her.

J a s on

WINTER 2023 EMPHASIS 31

PH Research Forum 2023

Science, medicine, hope

November’s annual PH Research Forum saw researchers and clinicians share their latest study findings and learn from each other’s work.

A

ttended by representatives from all of the specialist centres in the UK and Ireland, the event at London’s Chelsea Physic Garden was a valuable opportunity to look towards the future.

The conference has been supported by the PHA UK for many years, but this year saw us manage its organisation – playing a key role in encouraging collaboration and the sharing of knowledge.

As part of the line-up of speakers, our Chair, Dr Iain Armstrong, presented the findings of our Living with PH survey (see page 26 for more details) – ensuring patients were at the centre of everyone’s thoughts.

A £1,000 grant was awarded to the presentation voted ‘best of the event’ by delegates. Provided by the PHA UK each year, the prize aims to accelerate the reach of the research for the ultimate benefit of patients.

This year it was won by Dr Frances Varian of the Royal Hallamshire Hospital for her presentation titled: A patient-clinician partnership in remote healthcare delivery through a decentralised clinical trial design in PAH. Dr Varian will be sharing an overview of her research with us for the next issue of this magazine.

The diverse range of topics covered by the research presentations included:

• Remote healthcare delivery • The role of hope in hospitals • Redefining ‘idiopathic’ disease artificial intelligence (AI) might speed up referrals • How for specialist care

• The digital health landscape in Scotland • Changing trends in PH associated with congenital heart disease • Combination therapies and add-on therapies • Long-term outcomes in CTEPH • Genetics and children with PAH • Robotic self-guided right heart catheterisation WINTER 2023 EMPHASIS 32

LAST E CHANC E K A T O T PART!

Do you struggle with low mood or depression?

Here at the PHA UK we’ve partnered with clinical psychologists to trial a new self-help programme aimed at supporting people with pulmonary hypertension to manage their mental health.

We’re looking for adults with PH who feel they have low mood or depression to take part in this pioneering study to test the effectiveness of a series of self-help booklets, based on cognitive behavioural therapy (CBT).

The programme will then be made available to everyone with PH, so this is a chance to help both yourself and others.

SIGN UP BEFORE 31s JANUAtR 2024 Y You can find out more or apply to take part at www.bit.ly/SelfHelpStudy or scan this code with your mobile phone.

WINTER 2023 EMPHASIS 33

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l a c i z z i u q s! a m t s i r h C this ber Rob m e m K U A H P f o o Welcome to part tw ith loved w y la p t o n y h W . iz Owen’s family qu stive season? fe e th r e v o s d n ie fr ones or

? . .. s n o to r a C ?

1. What colour is Noddy’s hat?

2. Who was always trying to

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catch Tweetie Pie? 3. What is the name of Popeye’s black bearded enemy?

4. What is the name of the skunk in the film Bambi?

5. What is the name of the toad who is Danger Mouse’s enemy?

6. Who is Count Duckula’s housekeeper?

WINTER 2023 EMPHASIS 34

7. ‘By the power of Grayskull’

was who’s catchphrase?

8. Muskie is the sidekick of which character?

9. What’s the punchline to this joke? Why do elephants have big ears ?

10. ‘Mirror mirror on the wall’ is a phrase from which film?

?

?

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s son) has been Rob (pictured with hi iends since he writing quizzes for fr ing a diagnosis gave up work follow ension. of pulmonary hypert ite one with us, He shared his favour e first two rounds and we published th is magazine. in the last issue of th ing your This time, we’re test and cartoons. knowledge on music

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7. Sharleen S 8. Pauline Black gton n 9. Chester Benni 10. Nina Persson

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Missed part one?

You’ll find the full quiz at www.bit.ly/Robs_Quiz

WINTER 2023 EMPHASIS 35

Bluto e c at 3 .

ONS CARTO 2. Sylvester th n Greenback g 1. Blue er 5 . Baron Vo . Deput y Daw 4 . Flow y 7. He Man 8 n’t pay the 6. Nannuse Noddy wo hite 9. Beca :- ) 10. Snow W ransom . AC DC GE RS . Slade 4 LEAD SINie 2. James 3 ranberries rk 1. Blond cript 6. The C r 9. Linkin Pa 5 . The S 8. The Selec to 7. Texas Cardigans 10. The

PH Professionals: Together again at last Neil Hamilton,Consultant Pharmacist at the Sheffield Pulmonary Vascular Disease Unit, shares an insight into a special group that works together to benefit patients.

T

here is also an established set of ‘shared care’ centres, each of which is linked closely to one or more of the specialist centres. Shared care is established to reduce travelling time where appropriate quality care can be offered nearer home.

The teams at the centres may work ‘locally’ in one unit, but they also have input to the UK-wide system through either the UK Physicians Group (for the medical staff) or the PH Professionals Group (for everyone else). Readers of this magazine may already be aware that the glue holding all of this together and ensuring that the patients are central to everything we do, is the PHA UK. I have the honour of being the current Chair of the PH Professionals Group. Prior to the COVID-19 pandemic, we would meet twice a year in London.

The first national lockdown in March 2020 meant that our spring meeting that year had to be rearranged to a virtual

WINTER 2023 EMPHASIS 36

Patients with PH in the UK have access to a great network of specialist centres, rich with expertise across so many healthcare professions. This extends beyond doctors and nurses to pharmacists, physiotherapists, radiologists, psychologists, and many more. event, hosted by the PHA UK online. The virtual meetings continued, along with the emails between times, until we were able to safely meet up again last Autumn.

Following a very successful re-launch meeting in Birmingham last September, the group returned to London and have had two great meetings this year. As with the congresses and conferences

which have restarted face-to-face in the past year, it is unanimous that being able to meet up in-person is so valuable to all concerned.

As you would expect, the COVID-19 pandemic brought change for so many, and we have seen a number of personnel changes within our PH Professionals group membership. We are sorry to have

lost those who have moved out of PH but we are excited to see some new faces too.

It was especially pleasing to welcome one of the nurses from the Dublin unit to our October get-together.

This meeting comprised a great blend of sharing best practice, learning from each other, sharing exciting new treatment developments, and getting a preview of the PHA UK’s work in preparation for PH Day UK. If you were involved in an event for this awareness day, I sincerely hope it was successful. And if you raised money, thank you – I know what a difference every donation makes.

It was a pleasure to be involved with the recent ‘Together Sheffield’ event held by the PHA UK (see page 14).

It brought together staff from the Sheffield Pulmonary Vascular Disease Unit, the PHA UK team, and most importantly, the patients. This was a very positive day, and it was great to see so many who braved the bad weather to attend.

.

The PH Professionals Group showed their support for PH Day UK at October's meeting

Neil has many years of experience in the field of PH, both in his clinical role and through his long-standing association with the PHA UK.

There has never been a dull moment in my years involved in PH and there is no sign of that changing soon. The teams are working at a very challenging time in the NHS with all the strikes and staff shortages. However, it also an exciting time in terms of new treatments coming soon and a global PH research conference coming to London in January. Throughout all this, I am reminded how the UK PH community is so united. This is a huge strength and it’s the envy of other clinical areas here in the NHS, and of PH services around the world.

WINTER 2023 EMPHASIS 37

d n i m My H P d n a

s lived with Raya Mynot, 23, ha sion since pulmonary hyperten er latest she was a baby. In h is, column for Emphas she explains how her nal mental and emotio health has been impacted over the years.

WINTER 2023 EMPHASIS 38

“Never be a fraid to ask for suppor t...

Don’t let negative emotions control you r days”

PH is a condition that can limit what you do in your day-to-day life, and it can affect the simplest of tasks, which can become very frustrating. For me personally, it really affected me when going through high school and college.

During high school there were a lot of tasks and activities that I couldn’t take part in due to my health conditions, and this definitely had a negative impact.

I had to miss out on certain lessons and activities (PE, sports day, and some school trips) which made it quite difficult for my peers to understand, meaning I wasn’t included as much. This left me feeling pretty lonely.

It wasn’t until the last couple of years of high school that I had a friendship group that tried to understand, which was really nice. Our favourite thing to do was go and have dinner at the pub right next to school, so it was easy for me to walk to.

“Throughout school and college I learnt how to cope by myself a lot, wh ich in tu rn made me a stronger person and gave me confidence to advocate for myself” When I moved up to college some of those friends went to the same one as me, so it was really nice to have people there who already knew about me. However, getting some of the new teachers to understand was a little tricky.

In college I had to complete a certain number of hours of work experience, and this was really difficult for me since I had only done half a day at the nursery I was getting my experience at, due to me being too tired to complete a full day.

The nursery was so supportive of me and helped out wherever they could,

however the teacher who monitored it all was very pushy when it came to me completing my hours and didn’t understand why I had to have so much time off if I was tired or had appointments.

I think this is what frustrated me the most, and it got me really down as I felt I wasn’t going to be able to pass my course. My mum was a massive help in helping make sure I got my pass, which I did in the end!

It definitely gets me down a lot when people don’t try to understand PH or why I can't do certain things.

Throughout school and college I learnt how to cope by myself a lot, which in turn made me a stronger person and gave me confidence to advocate for myself.

Now I’m an adult my low days are definitely a lot less because I make a conscious effort to fill my days with things I enjoy doing and can do independently, and this has helped to improve my mental health so much.

I also started therapy after college which really helped me to work through all my negative emotions and frustration.

It’s always okay to reach out for help when you need it, and it was definitely one of the best things I have done.

Sometimes our emotions can become very overwhelming, and it can be a lot for our brains to work out and cope with.

So never be afraid to ask for support. I also get so much support from the people around me; my family and my girlfriend are honestly amazing when I have my down days.

Don’t let negative emotions control your days. Fill your days with things that make you happy, and people that make you smile. Don’t focus on what you can’t do, put all your focus on what you CAN do instead!

Raya's column

In life everybody definitely has their own struggles and highs and lows. However, living with something as serious as PH can really have an effect on emotional wellbeing and mental health. I myself have experienced low points in life when having PH.

WINTER 2023 EMPHASIS 39

R E T N WI S R E M R A W E

H T M F RO K U A H P E N I L N O SHOP

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WINTER 2023 EMPHASIS 41

g n i s i a r d n Fu

FOCUS

Nikki’s crafty Christmas Talented PHA UK member Nikki Ross has been raising money for us all year from her knitting, and her range of Christmas creations have gone down a storm. Nikki sells her items to friends and family, as well as taking stalls at local events. She also has a website, where you can browse her products and place an order.

Visit www.nickis-knitting.webador.co.uk

A snapshot of some of you support ov r er the last few months The sky’s the limit for Philip and Erica Erica Jones celebrated her 21st birthday by jumping 15,000ft from a plane to raise almost £1,500. Erica, who made the leap in memory of her father-in-law Keith Pratten, said: “Because it’s a big milestone I wanted to do something a little bit out of my comfort zone, but at the same time raising money for a good cause.”

75% of everything Nikki sells gets donated to the PHA UK!

email us at ur 2024 wish list, If a skydive is on yo we can help ! k.org to see how au ph g@ in is ra nd fu

A very Scottish stroll The Dewars managed to beat the northern rain to stay dry during the Edinburgh Kiltwalk in September. Fiona Dewar was joined by Rachel and Lesley for the 21-mile trek around the Scottish capital, raising £800. They chose to raise money for our charity in support of Fiona's father-in-law Roy, who is pictured with them here.

WINTER 2023 EMPHASIS 42

Phillip Barrett is another fundraiser who took to the sky over the summer. Completing the jump in memory of his father Alan Barrett, his sponsorship for us reached almost £1,700. He told us: “There were 50 seconds of free-fall before the parachute opened, then around five minutes descending under the canopy. Oddly, while I was really nervous in the run-up, the actual descent was surprisingly unscary and enjoyable.”

More super supporters… draisers Not all fun physical! have to be ughty Farah Feno ‘sober embraced raise October’ to eing by b over £450 to give up sponsored a month. alcohol for

Gilpin and Sarah Chris Took the to complete p u d e m a te ing rathon, rais Dublin Ma ry of m e 0 in m o 0 4 1, £ t s o alm John Took.

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Led by Chris Wiltshire, colleagues from Intuit Quickbooks walked 13.1 miles around London to raise over £1,300. It was the second half marathon walk this year from the team, who were raising money for us in support of Chris’s wife Kelly.

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WINTER 2023 EMPHASIS 43

New research will aim to help people get the most from IV treatments Researchers from the Sheffield Pulmonary Vascular Disease Unit are planning a new study to help PH patients on intravenous (IV) prostanoid therapy to get the best outcomes from their treatment.

To help access funding to enable this research to take place, the team needed to know whether it would be valued by the PH community. We asked for your thoughts in the autumn and we’re grateful to everyone who completed the short survey.

THE FINDINGS SHOWED THAT:

100%

of people with PH surveyed think this type of study is important (95% think it’s very important)

97%

would be interested in the results of the study (72% would be very interested)

76%

would be happy to take part in the study in various ways

Thank you to everyone who shared their thoughts in this survey. This information is now helping to secure funding for the study, and we’ll keep you updated on progress. Once again, your voices are making a difference!

WINTER 2023 EMPHASIS 44

WHO RESPONDED? PEOPLE

92 COMPLETED

THE SURVEY HAVE

53% IDIOPATHIC PAH

42%

ARE CURRENTLY ON IV THERAPY OR HAVE BEEN ON IT IN THE PAST

PH medications delivered intravenously are called prostanoids, and they include iloprost and epoprostenol.

This type of treatment involves patients wearing a small pump that delivers the therapy directly into their veins via a line.

Although these drugs are very effective, comprehensive training is needed to ensure the pump and line can be managed safely and effectively at home.

Support ASK THE

nurses

PHA UK Support Nurses Paul Sephton and Sian Richardson are former Clinical Nurse Specialists in PH, with long careers at the Sheffield Pulmonary Vascular Disease Unit. Still registered nurses, they now work part-time with our charity to offer support to our members.

“I’ve recently been diagnosed and I feel better knowing there is a specialist team to support me. But I’m not sure how often I’m able to contact the nurses, and what’s appropriate?”

Never feel like you are ‘bothering’ the Clinical Nurse Specialists (CNSs) and never feel embarrassed about asking a question. It’s what they are there for, and they want to help.

If you don’t feel comfortable calling, or you want to ask something on a Sunday afternoon when it’s on your mind, then you could email instead. Most centres have a dedicated central email address that you can reach the nursing team on.

Your email will always be answered as soon as possible, and if you call and leave a message, that will be returned in a timely manner too. Nurses are always busy - it’s all part of the job! - but they want to help you.

It may be your family and loved ones who have questions, and it’s absolutely fine for you to contact your nurses to ask these questions too.

Your CNSs work as a team, so it may not always be the same person you speak to when you call, or the same person who answers your email each time. But rest

assured that everyone will deliver the same high standards of care.

It’s important to remember that in an emergency, or if you need urgent advice or immediate action, you need to call 999 or go through your GP service or local hospital.

For anything that can wait 24 to 48 hours, contact the CNS team.

The key message here is that you’re not on your own. CNSs are there for you when you need them and they provide supportive advice and care at all stages of your journey.

We used to be Clinical Nurse Specialists and since retiring from the NHS, we now work as part-time support nurses here at the PHA UK. That means we can help with general questions about PH, and as registered nurses, we’d be very happy to hear from you. You can email us at support@phauk.org.

Please be aware though that because we work for a charity, not a hospital, we don’t have access to test results, clinical letters or medical notes – so specific questions about your circumstances will need to be directed to your specialist team.

Paul & Sian x

WINTER 2023 EMPHASIS 45

Food for. thought..

OUR REGULAR NUTRITION FEATURE TO HELP YOU MAKE THE MOST OF LIFE

EATING TO MANAGE

the side-effects

OF PH MEDICATION Constipation, obstipation, and diarrhea can all be side effects of some medications (and Christmas indulgence too!) but symptoms can be eased through the right nutrition.

Chermaine Kwant, a registered dietician with lived experience of PH, explains… iarrhea is a very common side effect of some PH medication, and constipation and obstipation (difficulty passing an accumulation of dry hard feaces) can occur too. Eating well, however, can help you have better intestinal health.

D

DEALING WITH DIARRHEA Diarrhea can be caused by the diuretics (water tablets) that many people with PH take. Prostacyclins - the drugs that some patients take intravenously via a pump - are also known to accelerate bowel movements (known as defecation, or ‘having a pooh’), which can cause diarrhea.

When you have diarrhea, this can affect how much nutrition you absorb from the food you eat. This can result in less energy, and it can also affect how much medication you absorb.

KEEP A DIARY

It can help to keep a basic diary, to keep a log of which foods make things worse for you. For example, some people have problems with onions or spicy food, or they find it difficult to digest dairy products. If you keep a diary of what you eat and when you experience problems with diarrhea, this will help you learn which foods can make things worse.

WINTER 2023 EMPHASIS 46

AVOID TOO MUCH FIBRE

When you have diarrhea, your intestines are over-stimulated, so it’s important not to stimulate them even more. Fibre can stimulate them and encourage defecation, so it’s a good idea to avoid having too much of this if you are experiencing diarrhea.

Choose white bread or rice instead of brown or wholegrain, for example, and avoid raw vegetables. Ensure they are cooked well and consider blending them into a soup.

Chicken broth can also be very beneficial.

However, when you have medication that causes diarrhea as a side effect, you have to live with it – and you can’t live forever on chicken broth! So, my advice is to listen to your body, and keep a diary, to discover what works for you and what doesn’t.

USE REHYDRATION DRINKS

Diarrhea means you are passing lots of water through your body, so rehydrating in the right way is important.

It’s a good idea to visit your local chemist to ask them about rehydration drinks, which are a combination of minerals and sugars, to help replenish what’s being lost through diarrhea.

Alternatively, you can easily make these types of drinks yourself. Take a glass of water, add in a pinch of sugar and a pinch of salt, and shake it up. Simple!

IF YOU LOSE YOUR APPETITE..

If you feel nauseous or lose your appetite whilst experiencing diarrhea, it’s not a problem if you don’t feel like eating much for a day or two.

You must keep on drinking though, to ensure your body stays hydrated.

This will help ensure that the medication you are taking is being absorbed into your body.

MY ADVICE FOR GENERAL DIGESTIVE HEALTH...

Eating foods rich in probiotics, or taking a probiotic supplement, can be very beneficial for digestion.

Probiotics can be found in fermented foods, such as some yogurts and cheeses, kefir, and sauerkraut. The fermented drink ‘kombucha’ is available in lots of places too. Feeding the bacteria inside your intestines in this way will help.

If you are going to eat more healthily and try to fix your intestinal health, for the first two weeks, your complaints may get even worse, before things improve.

But keep going, as eventually it will get better.

Remember that digestion starts when you chew your food. Eat slowly and avoid drinking fluids whilst you are eating a meal. This is because drinking will thin the stomach acid, which digests the food you are taking in. Some people with pulmonary hypertension experience reflux because the stomach is already irritated by medications, so I would never advise to drink with your meal.

Ultimately, everyone is different and when it comes to digestive issues, there is no answer that works for everyone. Listen to your body.

My day

on a plate Ever wondered what a dietician eats?

Here’s what Chermaine dined on the day before we spoke to her for this feature:

COPING WITH CONSTIPATION If your problems are at the other end of the scale, and you’re experiencing constipation or obstipation, drinking lots of water is definitely one of the best pieces of advice in this case. This will help to soften the stools to encourage them out of your system.

High fibre foods are also helpful here. Something like vegetable soup is a good idea as you get the fibre and the fluids in one.

Warm foods can also help when you have problems with your digestion or intestines and bowels.

It’s important to remember that most people with pulmonary hypertension will have a fluid restriction. Therefore, you need to ensure your fibre intake and fluid intake is balanced.

That’s because if you have a lot of fibre but don’t increase the fluid, your obstipation and constipation will worsen. It’s always about balance.

Exercise can also help if you have constipation or obstipation.

Even a small walk will help your intestines move, to push the waste through. It’s important to keep moving if you can.

Massaging your abdomen can also help, but this should be done in a counter-clockwise direction.

Chermaine lived with pulmonary hypertension before undergoing a lung transplant, and she is passionate about how nutrition can help people enjoy a better quality of life with chronic illness.

BREAKFAST Crackers with cream cheese, bell pepper and cucumber

DINNER

LUNCH

A cupcake at the theatre with my niece (it’s ok not to eat perfectly 100% of the time, and it is the festive season after all!)

Roasted pumpkin salad with herbs and spices, baba ghanoush (dip made with aubergine and yogurt), turkey sausages, and pitta bread.

WINTER 2023 EMPHASIS 47

Food for. thought..

VITAMIN K-RICH FOODS AND WARFARIN:

WHAT YOU NEED TO KNOW S

ome people with PH take a blood-thinning medication called warfarin, which works by interfering with the body’s ability to use vitamin K to form clotting factors (substances in the blood plasma which are involved in clotting processes). As some foods are rich in this vitamin, it’s important to know which to avoid.

Disclaimer: The information I am about to give you about warfarin and its interaction with vitamin K-rich foods is based on general knowledge around the subject. But remember, medical advice can change as new research comes in.

I want to stress that the information I’ve provided isn’t a replacement for talking to your specialist team. They’re the ones who can give you the best advice tailored to you and your situation. So, before you make any changes to your diet, especially when it could impact any of your medication, it’s important to seek advice from them first.

They’ll be up-to-date with the latest guidelines and can give you advice that’s right for you.

Your health situation is unique, so always follow the guidance and recommendations your team suggests

WINTER 2023 EMPHASIS 48

when it comes to taking warfarin or making any adjustments to your diet.

WHAT WE KNOW

Warfarin is a blood-thinning medication used to prevent blood clots. It works by interfering with the body’s ability to use vitamin K to form clotting factors. Therefore, it’s important to be mindful of your vitamin K intake when taking warfarin, as too much or too little vitamin K can impact the medication’s effectiveness.

FOODS TO LIMIT YOUR INTAKE OF: LEAFY GREENS Spinach, kale, collard greens, Swiss chard, turnip greens, and mustard greens are rich in vitamin K.

BRASSICA VEGETABLES Brussels sprouts, cabbage, cauliflower, and broccoli are high in vitamin K.

HERBS Parsley, coriander, basil, and other green herbs contain significant amounts of vitamin K.

GREEN TEA Green tea contains vitamin K and can potentially interfere with warfarin.

SOY PRODUCTS Soybeans, tofu, and soy-based products may have moderate levels of vitamin K.

BOOK RECOMMENDATION

By Shaun Clayton, a certified nutritionist and Operations and Finance Director at the PHA UK

UltraProcessed People

THE IMPORTANCE OF CONSISTENT VITAMIN K INTAKE

The key with warfarin is to maintain a consistent vitamin K intake. This means that if you’re consuming foods rich in vitamin K, try to do so in a consistent manner.

If you suddenly increase or decrease your vitamin K intake, it can impact your medication’s effectiveness.

ALCOHOL AND WARFARIN

Alcohol can also interact with warfarin and may increase the risk of bleeding. If you consume alcohol, it’s important to do so in moderation and discuss it with your healthcare provider.

Remember: The goal is not to completely avoid vitamin K-rich foods, but rather to maintain a steady intake so that your healthcare provider can adjust your medication dosage accordingly. It’s crucial to communicate with your doctor or healthcare provider about your diet, as they can provide personalised recommendations based on your specific health situation and medication dosage. Always follow your healthcare provider’s guidance and recommendations while taking warfarin.

by Chris Van Tulleken

FOODS TO CONSUME IN MODERATION...

FRUITS While most fruits

have minimal vitamin K content, some, like blueberries, blackberries, and raspberries have slightly higher levels.

AVOCADO Avocado has a moderate amount of vitamin K.

FATS & OILS Oils like

canola, olive, and soybean oil contain small amounts of vitamin K.

This column was inspired by a query from a PHA UK member. If you have a question about food and PH, email nutrition@phauk.org and Shaun will happily provide some guidance.

Why do we all eat stuff that isn’t real food.. and why can’t we stop? This is the question explored in this hugely popular title, which lifts the lid on ultra-processed food (UPF) and why it’s such a problem for so many of us.

If you read dietician Chermaine Kwant’s column in the last issue of this magazine, you’ll know that avoiding UPF is her biggest piece of advice for anyone living with a health condition.

Understanding why it’s such a large part of our diets could be the first step to making a change, so give this eye-opening book a chance this winter.

It’s widely available from all book retailers now.

This book has been recommended by Mary Ferguson, editor of Emphasis.

If you have a health-related recommendation you’d like to share, please let her know by emailing media@phauk.org WINTER 2023 EMPHASIS 49

An introduction to

mindfulness You may have already heard of ‘mindfulness’ – a type of meditation in which you focus on being intensely aware of what you're sensing and feeling in the moment, without interpretation or judgment.

It can help you feel calmer and more centred when worries take hold, making it a valuable tool when you live with a condition like PH. Kyle Clayton explains more.

WINTER 2023 EMPHASIS 50

Mindfulness can be found in anything once you find yourself practicing it consistently. It’s all about what works for you, and what you enjoy.

A

s the mental health charity Mind explains, ‘Mindfulness is a technique you can learn which involves noticing what's happening in the present moment, without judgement. You might take notice and be aware of your mind, body or surroundings. The technique has roots in Buddhism and meditation, but you don't have to be spiritual, or have any particular beliefs, to try it’.

Essentially, mindfulness is a form of meditation in which you train your mind to lose focus of worries, stress, and concerns, and focus on something else. It’s all about not getting overwhelmed and living for the now.

Mindfulness aims to help you cope with difficult thoughts and have more freedom in how you think and what you feel.

It can help you to feel generally calmer and less stressed, and to have kinder thoughts about yourself.

Ways to practice mindfulness The aim of practicing any form of mindfulness is to clear your mind and focus only on what is around you. Could you give some of these mindfulness practices a try?

Mindful breathing Sit down, relax, and focus on your breathing. Try to focus on every aspect of each breath; the inhaling, exhaling, and the way your belly and chest rises and falls.

Mindful eating This can be done while cooking or eating. Focus on every detail; the smell, the sounds, the texture, and the taste. This could also help if weight loss is your goal, as it helps you to feel fuller more quickly.

Walking meditation Walk slowly and intentionally, paying attention to each step,

The importance of acknowledging your bad thoughts

It may sound simple, but while achievable, learning mindfulness is not the easiest thing in the world.

The beginning of the process is the hardest part, as it starts with acknowledging what troubles you.

In doing this, you will know what you need to let go of, and what to put to the back of your mind. This will free your mind to focus mindfully on something else, which could be the air, sounds, or even the food you’re eating.

It will allow you to notice the good in every day.

The negative thoughts will never fully disappear while practicing mindfulness, but it’s about putting them to the back of your mind and acknowledging them as mental events that come and swiftly go.

the movement of your body, and your surroundings.

Mindful observations Choose an object and observe it closely, noticing its details, colours, shapes, and textures. This helps enhance your ability to focus.

Mindful colouring Engage in colouring or drawing, paying attention to the colours, shapes, and movements. There are various books available to purchase that provide ready-made patterns to work with.

Journalling Write down your thoughts and feelings without judgment. Reflect on your experiences and explore your emotions.

Mindful daily activities Bring mindfulness to everyday tasks like brushing your teeth, washing the pots, or taking a shower. Focus on the sensations and movements involved.

Learn more about mindfulness via these websites: www.bit.ly/MindfulOrg www.bit.ly/MindWebsite www.bit.ly/MentalHealthOrgWebsite WINTER 2023 EMPHASIS 51

Don't miss out on what's yours As the cost of living continues to rise, find out what financial support you may 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

Log on for support and information www.phauk.org

✓ Information about PH – including tests, diagnosis and treatment

✓ Downloadable resources ✓ Research opportunities and reports ✓ Our online shop – including free booklets and guides

✓ Latest news

www.phocusonlifestyle.org ✓ Real-life stories and experiences ✓ Blogs and videos ✓ Lifestyle and wellbeing advice ✓ Relationships and family ✓ Work and travel …and much more to help with all aspects of living with PH

Accessible to all We’ve invested in technology that allows people to access our online content in a way that works for them. Assistive toolbars will read text aloud, translate into 99 languages, magnify text size, convert content to MP3 files, and much more.

Simply tap on this button at the bottom of each web page to access all of the assistive features.

WINTER 2023 EMPHASIS 51

Nfe aEtuWr e

e e t

k l a t n

Wit h E mil y Gr ave ns t ede

PAH since Emily is 13 and has had she’s had she was a baby, meaning ond Street many visits to Great Orm e, she shares over the years. This issu rself busy all the ways she keeps he and how she whilst waiting around – g in London… makes the most of bein

Probably the worst part of going to the hospital is all the waiting I have to do, so here are some things I do to occupy myself:

Gaming

Reading

Puzzle books

I really, really like reading as it almost takes me to another world. It is a really good thing to do in hospital as for me it takes away all concept of time, so waiting goes really fast, so I always remember to take my current book with me.

I find it is always so handy to have a puzzle book on hand as a lot of the time when I get bored, puzzle books get rid of my boredom quickly. They get my brain working plus they are so fun to do!

WINTER 2023 EMPHASIS 54

Emily's favourite knitting project - Hedwig from Harry Potter

I will always take my iPad with me so when I get extremely bored I can just start playing. I do have games on my phone, but I find I am more entertained by the games I have on my iPad.

Knitting Knitting is one of my favourite hobbies and I enjoy finding different projects to work on.

One of my favourite and biggest projects was knitting Hedwig from Harry Potter, it took me several months and a fair bit of it was done in hospital.

Enjoying dinner in a restaurant before seeing a show whilst in London

When I was younger, I used to have lots of sticker books and the hospital would sometimes give me things that they had on the ward to play with during my stay (and it still happens now!). At GOSH I have been given trays full of craft materials to create things with and lent a PlayStation. Also, in some rooms I can watch TV.

With her mum in London during a visit to GOSH

In the Blue Lagoon at GOSH Emily likes to see West End shows when she stays in London

When we go to GOSH if we are there for a whole day or overnight then there is often time to do things outside of the hospital.

Some of my favourite things are to visit Coram’s Fields Park (which is close by), visit the British Museum, go to nice places to eat, and if we can, see a West End Show in the evening.

Inside the hospital, there is the Blue Lagoon where you can see lots of big Disney statues and sometimes they have things to do in the Lagoon cafeteria.

You never know who you might bump into at GOSH – we’ve seen therapy dogs, life size GOSH teddies and even a celebrity chef (Gordon Ramsey)!

.

WINTER 2023 EMPHASIS 55

Scaling summits for people with PH September saw friends Vicki Gibson and Kay Gracey trek across the Alps in support of the PHA UK; a gruelling four-day challenge that raised almost £2,000.

Here, Vicki explains what it all meant to her.

Vicki (right) and Kay

WINTER 2023 EMPHASIS 56

The whole experience has been fantastic

was at least a three-four hour descent.

We spent the final night in a gorgeous village and on the fourth day came the last climb, which seemed to take forever. In reality it was about five hours and when we reached the top there was a beautiful restaurant, with stunning views of Mont Blanc and the mountain ranges.

When things got tough on the trek I thought of my mam, who couldn’t even walk when she first diagnosed with PH, and of all the other PH sufferers going through the same thing with the disease. I did it for them.

I dug deep and channelled my mam. She went through so much and was such a trooper. She had sepsis and was cared for in intensive care, and she then received her PH diagnosis. She was also a breast cancer survivor.

K

The group who took the challenge together

ay and I wanted to do something to challenge ourselves both mentally and physically, and I was keen to honour my mam Ethel, who had PH and sadly passed away in 2021 as a result of contracting Covid. It was important for me to do something in her name, that would help other people with the condition.

I’d never done anything like this before, but we paid our deposit to the company organising the expedition, and that was it – no backing out now.

And so, 18 months of training to cross the highest mountain range in Europe began.

Suffolk, where we live, is the flattest county in Britain so we spent many weekends trekking in places like the Peak District and Wales to prepare for the challenge. We did 28-mile hikes, we lived on the Stairmaster at the gym, and I even bought an exercise machine that mimics mountain climbing for my dining room.

We trained hard, and then suddenly it was time to go. We flew to Geneva, picked up the rest of the group we’d be walking with, and started the trek on the Italian side of Mont Blanc. It was a non-stop climb up to a hillside refuge where we would spend our first night.

There were 14 of us, all strangers, in beds side-by-side. But we quickly bonded, and everyone was just so lovely.

Day two was the hardest thing I have ever done. It was so steep, a six-hour ascent, in temperatures reaching 30 degrees. Days three and four were the same and each day there

Vicki's mum, Ethel She was so strong and courageous for dealing with everything she had. She still kept all her feistiness and strength, yet she was so caring. My mam was under the care of the Freeman Hospital in Newcastle for her PH, and I know she thought the world of her team there.

When the rest of the group doing the trek with us found out we were fundraising for the PHA UK, they were all interested in PH and what it was. They even asked Kay and I to lead the last mile of the challenge in honour of my mam, leading the pack up to the finish. It was lovely.

I’m so pleased with how much we raised. We initially had a target of £350 so to get to almost £2,000 including Gift Aid is amazing. In short, the whole experience has been fantastic, and I know my mam would have been so proud.

WINTER 2023 EMPHASIS 57

We provide support in the ways that work for YOU. If you like to talk…

Our Listening Line support service gives you dedicated time with a PH professional to talk through any worries or concerns you have relating to your emotional or mental wellbeing.

PHTAENUINKG LIS

LINE

Speaking to someone who already understands PH means you don’t have to explain the condition first, and this service is available to family and friends too.

Find out more or book a telephone chat by emailing listeningline@phauk.org or visiting www.bit.ly/ListeningLine

“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

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If you prefer support by email…

Our e-support service is as valuable as a helpline, but delivered via email instead. We can help with ongoing emails to support you through a difficult time, or with one-off enquiries or problem-solving.

Contact us at support@phauk.org at any time and we’ll get back to you within two working days.

“I'm so grateful to have this chance to email as I'm hearing impaired”

ail us at

E-support service user

Please note: These are not formal counselling services and are not designed to replace clinical advice from your specialist centre team.

WINTER 2023 EMPHASIS 58

Safe spaces to talk to others affected by PH

Our private Facebook groups are a valuable source of support and advice from people who truly understand.

Each group has a strict screening process to ensure those requesting to join are genuine, so you can be confident of privacy. They are safe spaces to talk to other people with similar lived experiences.

PHA UK Official Facebook group

This is our original forum, which now has almost 2500 members.

It’s a very active group, with responsive members who offer advice and support at all times of the day and night.

Join here: www.bit.ly/OfficialPHAUKFacebookGroup

PHA UK Official Carers Group

This forum is exclusively for family members, loved ones, and anyone who provides care or support for someone with pulmonary hypertension. Set up in 2021, it is growing to become a valuable source of connection.

Join here: www.bit.ly/PHAUKCarersGroup

PHighting On: Life after loss to PH

This group was set up by PHA UK members with our support.

It is a safe space for families and friends to support each other, share their experiences, feelings and memories – and connect with others that have experienced loss due to PH.

Join here: www.bit.ly/PHightingOn_LifeAfterLossToPH

PH Par This ha ents UK s

been indepe ndently set up of the P UK and H i of pare s run by a gr A oup nts. Yo uc www.b it.ly/PH an join at Parent sUK

ave a You need to h file in order Facebook pro roups. to join these g Sign up at ok.com www.facebo sy !

It’s free and ea WINTER 2023 EMPHASIS 59

A spotlight on the Motability scheme

If you have a disability and want to lease a new car, but you don’t really know how to go about doing it, then there’s a charity just for you. Kyle Clayton explains…

L

et’s talk about Motability, a charity which covers the UK and aims to help disabled people acquire leases on a new car, scooter, or powered wheelchair, using their disability benefit.

Motability help as much as possible by arranging your insurance, covering your servicing and MOT, providing RAC breakdown cover, and should the car be electric, it will arrange and cover the cost of a home ChargePoint and standard installation.

To enlist the help of Motability however, you must be on the higher rate mobility part of either the Personal Independence Payment (PIP), or the Disability Living Allowance (DLA).

Motability will help you choose a car, Wheelchair Accessible Vehicle (WAV), scooter or powered wheelchair, and the payments will be taken straight from your mobility allowance, with however much you pay depending on however much you receive.

There will likely be a fixed advance payment on the vehicle depending on

WINTER 2023 EMPHASIS 60

which one you choose. The advance payment is generally required if your mobility allowance doesn’t cover the full cost of the larger or more expensive vehicles. However, if you’re unable to afford the advance payment but require a certain type of car or WAV, then you may be eligible for a grant.

You can find out more about this at www.motabilityfoundation.org.uk Once you’re ready to go ahead, visit any dealer and let them know your situation and that you’re looking for a car, scooter, or powered wheelchair, and they’ll help you order. If you’re searching for a WAV supplier or adaptations installer, don’t worry about travelling, they’ll come to you to help you order.

Once you’ve had a test drive and like the feel of a vehicle, visit a Motability scheme dealer, and they’ll order it for you. If it’s a car, you should be able to pick it up from your local dealer. If it’s a different vehicle, then it will be delivered to your home.

Find out more about the Motability scheme at www.motability.co.uk

Motability will help you choose a car, Wheelchair Accessible Vehicle (WAV), scooter or powered wheelchair

Research spotlight

PAH and genetic research: Navigating the diagnostic odyssey Dr Emilia Swietlik of the University of Cambridge is passionate about using research to improve both diagnosis and outcomes for people with pulmonary hypertension. In her latest article for Emphasis, she discusses the results of a 2023 study into the diagnostic journey of PAH patients who participated in genetic research.

I

n the intricate world of medicine, the path to diagnosis is often a journey full of twists and turns, especially when dealing with rare diseases.

These conditions, individually affecting a small segment of the population, pose unique challenges. Globally, they number between 6,000 and 8,000, together impacting a staggering 300 million individuals. The quest for timely diagnosis is a mission that goes beyond the boundaries of current medical knowledge.

Discovering the genetic roots of rare diseases is a crucial step towards improving patient care. Not only does genetic diagnosis often lead to better disease management, but it also offers valuable insights for prognosis, genetic counselling, and predictive testing.

While rare disease patients commonly face diagnostic challenges, certain conditions, such as PAH, introduce unique complexities in terms of family dynamics and ethical considerations.

This prompted the launch of the RAPID-PAH study, a comprehensive investigation into the diagnostic journey of PAH patients who participated in genetic research. This study aimed to unravel the impact on patients' lives, particularly on family dynamics and ethical aspects.

Our collaborative effort involved interviews and focus groups with a total of 63 patients, caregivers, relatives, clinical teams, and researchers in the UK. We gathered their invaluable perspectives and experiences related to both clinical and genetic aspects of the diagnostic process.

Continued...

“Your participation in genetic research is like shining a light on the road ahead” Dr Emilia Swietlik

WINTER 2023 EMPHASIS 61

Stage 1

Stage 3

Health complaints and seeking GP help

Correct diagnosis and relief

Stage 2

?

Misdiagnosis and frustration

Challenges along the way: The themes unveiled The study showed that during their diagnostic odyssey, patients experience several distinct phases.

Seeking help from GPs: At the outset, patients reach out to their family doctors (GPs) in pursuit of answers to their ongoing health concerns. This initial step sometimes leaves them misunderstood or dismissed and with more questions than solutions.

Misdiagnosis and frustration: Progressing further, many encounter a

Family dynamics in focus: A rare disease's ripple effect Genetic diseases, such as heritable pulmonary arterial hypertension (HPAH), have ripple effects that extend beyond the patient. They not only impact the individual, but also ripple through their families. These diagnoses can either strengthen the bonds within a family or test them to their limits.

Empowering family bonds The presence of a rare disease can strengthen family relationships and this is particularly evident in paediatric centres where parents are actively engaged in the diagnostic process. This involvement

WINTER2023 WINTER 2023EMPHASIS emphasis62 58

series of incorrect diagnoses, leading to mounting frustration and confusion.

These misdiagnoses not only delay their quest for answers but also add to their physical and emotional distress.

Correct diagnosis, shock, and relief: Finally, they receive an accurate diagnosis, bringing clarity and a sense of relief. This validates their experiences and opens the door to proper treatment and management, marking a turning point from uncertainty to newfound hope.

Genetic diagnosis: When it comes to genetic diagnosis, patients often feel that clinicians exercise excessive caution.

fosters unity and active participation.

"I cherished my son, and I still do...

We have a fantastic relationship. I don't know if I would have had that relationship with him without [the disease]... It makes you appreciate life more because he could have been snatched away from me at any point."

– study participant Shaping the future: Parents and children Among adult patients, parents sharing their disease experience with their children often marks a pivotal moment in their relationship. Parents wait for their children to reach emotional maturity before disclosing the disease.

Stage 4 Breaking genetic diagnosis

They desire clearer answers and earlier discussions about genetic aspects.

This process may bring disappointment, uncertainty, or relief, with concerns about passing on the disease to future generations (this will be discussed more later on).

Adapting to life with the disease: With a diagnosis, patients embark on a journey of shaping their outlook on life and their condition. They embrace opportunities, including treatments, clinical trials, and support networks – demonstrating resilience in the face of adversity.

"When she got to about 19, she got to the, 'I'm an adult.' And I do remember having a conversation with her then, 'Right, sweetheart. This is me treating you like an adult. Pulmonary hypertension is life-shortening...’

And she burst into tears. But it was a changing point." – study participant Genetic testing and family dynamics Informing relatives about the possibility of predictive DNA testing often falls on the person known as the ‘proband’ – the first person or individual in a family or group who is identified as having a particular medical condition or genetic trait that is being studied or researched.

Stage 5

Life with the disease

This process can sometimes exclude potential cases from genetic testing.

Patients can be categorised into two groups – those who advocate for testing and those who prefer a cautious ‘watch and wait’ approach.

Empowering the next generation Among supporters of testing, especially patients with biological children, discussions focus on how much information to share with their children, and when to share it.

Parents of underage children or young adults adopt a sensitive approach, emphasising the need to balance transparency and safeguarding.

Genetic diagnosis is seen as crucial for children with PH: "One of the reasons people say 'yes' [to genetic research] is because when your child is diagnosed with idiopathic PH... even the best care at the moment is not what you would want to accept for your child, and the only way out of that trap is research." – a clinician who was interviewed as part of the study Respecting autonomy When it comes to adult children, parents respect their children's choices, influenced by factors like family planning and relationship status.

"I'd leave it up to them... It wouldn't be a decision that I could make for them. I could only ever outline the situation to them, and if they decided to go ahead with testing, then that would be their choice."

– study participant Some parents wanted their children to undergo genetic testing quickly to address the risk of the disease.

"I felt compelled to get her tested because there was a 50% chance that she could carry the same defective gene." – study participant Others opted for vigilant clinical monitoring without genetic testing.

These decisions were often shaped by the strength of family connections and feelings of guilt of having passed on a faulty gene.

Michaela Fay (pictured) played a key role in the study discussed in this article by conducting interviews with patients.

The impact on women: Navigating complex decisions

The journey of diagnosis poses unique challenges for women, particularly around family planning.

Discussions may require patients to consider abortion, adoption, and surrogacy. Personal experiences and emotional attachments significantly influence these decisions.

"If they'd said to me, 'Yes, it’s hereditary, yes, he could have it,' I would not have had an abortion, no.

Certainly not after losing my baby."

– study participant Others struggle with moral dilemmas and the impact on

maternal health and family responsibilities.

But despite challenges, adoption and surrogacy can provide options.

“It was horrendous going through the adoption with the pulmonary hypertension. I did have to say I fought so hard. But I did it.”

– study participant While some contemplated surrogacy, only a few pursued this option.

"We were lucky enough to do traditional surrogacy and had two children with the same lovely surrogate mum... That's been brilliant." – study participant

Conclusion: Empowering your journey In the world of rare diseases, getting a diagnosis isn't just a medical thing; it's like finding a guiding light for patients and their families. The path to diagnosis can be tough, but it shows how strong people can be when they make informed choices.

As we keep learning about PAH, as researchers we invite patients and their families to help us. Your participation in genetic research is like shining a light on the road ahead.

We're all in this together, creating a story of strength and hope.

If you receive an invitation to take part in research, and you decide to help us, you're helping to uncover the secrets of PAH. This means we can find better treatments and improve the lives of those with this rare condition.

Your involvement is crucial, and it can make a big difference.

Look for another article by Dr Swietlik in the next issue of this magazine WINTER 2023 EMPHASIS 63

Self-help programme from the PHA UK

HELP TO OVERCOME WORRY & ANXIETY

This four-week self-help programme consists of a series of workbooks based on Cognitive Behavioural 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

Order the free programme by scanning this code, visiting www.bit.ly/OrderTheProgramme or calling 0300 373 5367

Join our panels and shape our work...

Members of our Feedback Forum and Research Forum panels play a key role in helping to guide PHA UK activities and contribute to important research work.

We contact all forum members with opportunities via email, and it is up to you whether you respond. We appreciate that PH comes with bad days so we understand if you need to give some of our invitations a miss. If it’s not for you at that time, simply ignore the email and look out for the next!

FEEDBACK FORUM Whether it’s a booklet, poster, video, or web page, we need to be sure that it meets the needs of the PH community.

That’s why we’re building a mix of people affected

by PH - patients, and their loved ones too - to give us feedback on new materials before they are launched.

Your opinions are really important, and they help us make sure we’re getting things right.

As part of our Feedback Forum, we’ll ask you to spend a little time reviewing our materials before they are launched. This may be in the form of reading a leaflet, watching a short video, or having a look at a web page.

You will then be asked to fill in an online form with your thoughts. Sometimes, we may ask your thoughts on an idea for a campaign or project during the planning stages, to ensure we are taking things in the right direction.

Join at www.bit.ly/PHAUKfeedbackforum

RESEARCH FORUM

volved e, n i t e G tim r own fort u o y in he com n t m o r f r ow of you e. hom

There’s never been a more important time to lend your voice and experiences to PH research. As well as conducting our own studies to help people affected

by PH live better lives, we are regularly approached by researchers from healthcare and academia, who recognise the importance of the patient voice.

Our Research Forum is for patients and loved ones or carers who would be willing to help with these studies when possible.

They often involve completing surveys or questionnaires, and sometimes they may involve online or telephone chats, or focus groups. Occasionally, you may receive an invitation to be involved in a different type of study, but it is unlikely you will be asked to take part in a clinical trial in this way.

Join at www.bit.ly/ResearchForum_PHAUK

Share this magazine with family & friends

Stronger together Be part of a 4,500-strong national support network We’re here to support people like you.

Join the PHA UK today and benefit from: • Support and advice • Helpful printed information & resources • Emphasis magazine delivered to your door • Free access to financial advice from qualified professionals via our partnership with Turn2Us • Fundraising ideas and guidance

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.

Join FREE today www.phauk.org 0300 373 5367 office@phauk.org

Get involved with Emphasis!

Andlly... fina

This is your magazine Share your PH story

Tell our readers about any aspect of your journey with PH. We like to include experiences from both loved ones and carers.

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Contact us with your recommendations, findings, and nuggets of advice, so we can share them with others via these pages.

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Want to comment on something you’ve seen in Emphasis, or share an opinion or feedback for print? Write us a letter or send us an email – you’ll find the addresses at the bottom of the page.

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We love to celebrate the talents and achievements of our readers. Send us something that has made you smile, something you’re proud of, or something you want to share with others. This is a great way for children to get involved and see themselves or their work in print too.

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MOVING HOUSE ?

Don' t fo rget to g iv us your new add e res to ensur e you ke s ep receivin g your c opy of Emph asis!

Get in touch!

Contact us here at Emphasis by emailing media@phauk.org or writing to Emphasis, PHA UK Resource Centre, Unit 1, Newton Chambers Road, Thorncliffe Park, Chapeltown, Sheffield S35 2PH WINTER 2023 EMPHASIS 67

FREE PHA UK PUBLICATIONS

Helping you to live well with PH Also available: • Driving with pulmonary hypertension • Your benefits assessment toolkit • Your pocket guide to PH See the full range of titles and order for free by scanning this code or visiting www.bit.ly/PHAUK_Publications

• Words of wisdom ...and more!