Emphasis Summer 2022 by phauk

Our BIGGEST ever issue!

SUMMER 2022

Experiences of PH around the world

Holiday season is here!

Your travel tips and special memories

Plus...

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Research updates, testing positive for covid, fundraising news, expert interviews and much more…

The magazine for members of the PHA UK www.phauk.org

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Hello Welcome to your summer edition of Emphasis! This is our biggest ever issue, and there’s something in it for everyone.

It's

been a busy few months behind the scenes at the PHA UK. We’ve published a new resource to help people understand more about palliative care (see page 18) and we’ve been working hard on a new project to gain a deeper understanding of how the pandemic has affected our PH community. You can find out more, and get involved yourself, on page 10. We’ve also been making plans for PH Day UK, our new awareness campaign set to take place this autumn. Pop Friday 4th November in your diary and keep an eye out for more details coming soon. Although we are called the PHA ‘UK’, we are proud that our influence goes beyond British shores. Our quality of life measure, EmPHasis-10, has now been translated into 41 languages, and our resources are used all over the world. Pulmonary hypertension doesn’t care where you call home, and this issue, we bring you the stories of those living with the condition in countries that are thousands of miles apart. Our bumper feature starts on page 37.

People from other countries make up an important part of our NHS workforce too, and on page 58, we hear from someone I have had the pleasure of calling a colleague for the last few years. Clinical Nurse Specialist in PH Amanuel Teklesenbet arrived in the UK from Eritrea as a refugee, and I hope you find his story as inspiring as I do. Finally, if you are lucky enough to be taking a holiday over the next couple of months, turn to page 54 for travel tips from the experts by experience – our members! Whatever you get up to this summer, I hope you enjoy it. Just don’t forget the sunscreen!

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

OUR INFLUENCE GOES BEYOND BRITISH SHORES

Iain will be ‘going the extra mile’ for people with PH by completing a 1600m outdoor swim in September. Find out more on page 8.

PS. If you’re an animal-lover, you’re sure to enjoy Kaylee’s column on page 48. She writes about how her gang of pets help her through the tough times with PH, and there are some very cute photos too.

SUMMER 2022 emphasis 3

“If you have questions about palliative care, don’t be scared about asking them” Dr John Wort

Co convnetinuing rsatio ns

Natalie looks back on ten years since her transplant

How your views will shape the future of clinical trials

A DECADE OF GRATITUDE

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

This magazine is printed on paper from sustainably managed sources

PUTTING PATIENTS FIRST Living with PH... 12

For my wife

Coping as a carer

24 32 52 62

Simon’s story of caring and family How to get involved in an important new study

Support for anxiety

Our self-help programme that’s making a difference

Keeping positive with PH

Maria Brook on what’s helped her over the years

Keeping the faith

Mother Jane’s unique experiences

Life-long, not life-limiting

Sarah Pyett’s personal journey with PH

Travel tips, pics and memories…

a n o g in o g ll a e ’r e W

58 R E M M SU HELLO MY HOLIDAY! NAME IS…

A special interview with PH nurse Amanuel Teklesenbet

37 Living with PH in America, Australia, Dubai and Turkey

Regular features...

The best of the rest…

Upfront

Testing positive for COVID-19

Creature comforts

On the spot

64 69

News, views and what you’ve been saying on social media Columnist Kaylee on how her pets have helped her cope with PH

Fundraising Focus

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

Green Leaf Crew

Car journey bingo and the latest column from Emily in our special tear-out for children

26 66

A range of your experiences

Meet Chito Gabutin, Lead Nurse within the Royal Free Hospital’s pulmonary hypertension service

Celebrating Sabbah

The fundraising event that brought two families together

Could anticancer drugs be used to treat PH?

The study hoping to find out

Get in touch: PHA UK Resource Centre, Unit 1, Newton Business Centre, Newton Chambers Road, Thorncliffe Park, Chapeltown, Sheffield, S35 2PH office@phauk.org 01709 761450 @PULHAUK @PHA_UK @pha_uk_insta @PHA ORG UK No part of this magazine may be reproduced without the prior permission of the PHA UK.

UPFRONT News, views, and what you’ve been saying online Save the date for PH Day UK

This year we’ll be dedicating Friday 4th November to raising awareness of pulmonary hypertension and there will be lots of ways for you to get involved. This is a fresh new annual campaign, to help us come together as a community and put the spotlight on this rare disease.

Make sure you’re signed up to our e-newsletters to hear more about this soon! Just fill in the short form at phauk.org or email media@phauk.org with ‘yes please’ as the subject line (there’s no need to add anything else – unless you want to!)

PH Y A D UK 2 202

VEM O N 4TH

BER

International interest in our self-help programme Pulmonary hypertension clinicians in Taiwan have approached our charity to seek permission to translate our self-help programme for use in their country. ‘Overcoming Worry & Anxiety’ is a series of workbooks to help people with PH manage anxiety and we are working with Taiwanese healthcare professionals to make the resource available to their patients. You can find out more about the programme and order it for yourself on page 24.

SUMMER 2022 emphasis 6

>Celebrating our nurses May’s International Nurses Day saw lots of people take to social media to celebrate the wonderful nurses in the UK’s pulmonary hypertension service. Here’s a selection of your comments on Facebook… Tracey “A ginormous thank you to the PH nurses at the Brompton and the PH nurse at Southampton General Hospital ” Fiona “All the nurses at SPVU, Golden Jubilee are fantastic. They are so friendly and helpful and nothing is too much trouble for them" Suzy “A massive thank you to all the PH nurses at Hammersmith who have cared for me on my PH journey x" Mandy “Papworth nurses are always happy to chat. I feel so supported. Thank you x" Gordon “Words can’t describe how grateful I am, every day, to the nurses at the Royal Free" Toni “Thank you to all the nurses working in PH units, especially in Sheffield… you are wonderful " Sharon “Thank you to Louise and Noreen at Belfast City Hospital for the support and care they give, I'm very lucky to have such dedicated nurses in my life "

Spreading the word about PH PHA UK member David Whittington (pictured) delivered an educational talk about pulmonary hypertension to a Christian congress in Derbyshire earlier this summer. David, who has had surgery for chronic thromboembolic pulmonary hypertension (CTEPH), addressed 70 delegates and gave out some of our publications. He spoke about his experiences of dealing with the condition during the pandemic and undergoing a pulmonary endarterectomy, and helped the audience understand more about how the condition affects people. David also plans to give out leaflets about PH whilst attending classic car shows over the summer, spreading awareness wherever he can.

Vicki “Thank you to Rachel and the other PH nurses at The Freeman. My mum is coincidentally in with you now, I'm so grateful for the fantastic job you do of caring for her! " Jim “Great Ormond Street nurses are incredible, they have given our family so much support over the last year or so"

More UPFRONT news overleaf SUMMER 2022 emphasis 7

UPFRONT News, views, and what you’ve been saying online

Swim challenge will see our Chair go the extra mile PHA UK Chair Dr Iain Armstrong is training hard for a special swim challenge that will see him complete a mile of the Serpentine Lake in London’s Hyde Park. Iain will take part in ‘Swim Serpentine’ on 17th September and he is using the event to celebrate kindness and raise money for the PHA UK.

He said: “During the pandemic a lot of people in the PH community have gone the extra mile for each other, and inspired by them, I want to do the same. I'll be swimming one mile (1600 metres) around the Serpentine Lake, which is the same lake that the 2012 Olympic triathletes competed

in. This will be very different from the warm indoor pools of Sheffield that I am used to! I'm aiming to raise £1 for every metre I swim, and by 'going the extra mile' I hope to celebrate the kindness of others and maybe even inspire some people along the way.

As a Nurse Consultant in PH I have seen the difference physical activity (within individual limitations) can make to patients, and this is something I have experienced personally over the last year or so too.

I've overhauled my lifestyle, losing almost 30kg, and swimming has become a big part of my routine. Whether you can move for a mile

You can sponsor Iain via his online fundraising page at bit.ly/IainArmstrongSwimSerpentine SUMMER 2022 emphasis 8

or a metre, getting active could help you too. I will be thinking of people with PH with every stroke of this open water swim.”

Another stamp of approval for our publications

Following a rigorous assessment, our information resources have been re-certified with a Patient Information Forum (PIF) quality mark. The ‘PIF tick’ is only awarded to organisations who follow high quality standards when producing health information and we have now achieved this for the second year running.

Knitting up Jubilee joy PHA UK member Mel Lee was part of a team of ladies who covered their hometown of Oundle in Northamptonshire in ‘yarn bombs’ to celebrate the Platinum Jubilee in June.

The team knitted coverings for trees, benches, bus shelters and bollards, and dressed them overnight as a surprise for residents. Their creativity made the local BBC News, and they were interviewed on the radio too. Mel can’t walk far due to her pulmonary hypertension and told us she finds knitting helps her to pass the time at home.

She is part of a ‘knit and natter’ group in her local community, and they have been yarn-bombing since 2016. Mel said the surprises make people smile, adding “we all need a bit of something nice at the moment, don’t we?”

Telling family and friends about PH: What would help you?

We’re planning a new printed resource to help people newly diagnosed with PH talk to their family and friends about the condition. Your own experiences are important, so if you have any thoughts about what would be useful to include, please let us know. You can email us at office@ phauk.org (type ‘new booklet’ in the subject line) or give us a ring on 01709 761450. SUMMER 2022 emphasis 9

A moment in history

This n e will b w website e laun ched in No v e m b to coi ncide er, PH Da with y UK

There is no doubt that the last couple of years will live on in our memories, and we are capturing them forever in a new website that will bring together people’s experiences of the COVID-19 pandemic.

More than just another website, this online time capsule will make sure the voices of the PH community are heard as we preserve history and dig deeper into the lived experiences of the crisis. Over the last couple of months, we have spoken to 30 PHA UK members who have shared their individual stories with us. We’ve interviewed both patients and caregivers and despite a shared diagnosis, everyone’s story has been different. And every one of them matters. Each story has given us new insight into the impact of the pandemic on people living with pulmonary hypertension. We hope this project will help to inform planning for future health emergencies and aid our understanding of life with PH as we move forward from the crisis. As well as sharing these stories on the new website, we will also be combining them into a printed anthology that will be available to order for free.

SUMMER 2022 emphasis 10

Production is also underway on a feature-length film that will bring these experiences to life in a new style that we have never explored before. And one of the most exciting elements of the project is our online exhibition – which we’re inviting you to get involved with now! As well as providing lasting evidence of this period of history, this work will also help to raise awareness of PH amongst the public. Please, play your part in this project if you can.

Remembering your pandemic, your way Our online exhibition will showcase your experiences of the pandemic in whichever way you choose. Please select from any of the categories opposite or submit something totally different if you wish. Your submission could be something you produced during the pandemic, or something you produce now, specifically for

the exhibition, which reflects your experiences over the last couple of years. This is not a competition or a talent contest; it’s a way of bringing together symbolic interpretations of the pandemic in a meaningful way. We understand that some submissions may be personal, so you can remain anonymous if you wish.

Get involved by sending us your...

Photographs

This could be anything from a snap on your phone to a carefully curated image using specialist equipment. Share your favourite place during lockdown, the pet that helped you through shielding, your first meeting with family in months, or anything at all that reflects your pandemic.

Drawings, paintings or crafts

We’re after all kinds of artwork, from sketches to crochet, watercolours to decoupage, and everything in-between. Please send us a photo of anything you produced.

Writing

Send us your poems, prose, short stories or articles. You can also send us anonymous diary entries or blog posts if you wish. If you’d like to reflect on your experiences of the pandemic by writing your story specifically for this exhibition, we’d love to include that too.

Music, singing and dance

You can also send us recordings or footage of musical recitals, singing, or dance performances that have a connection to the pandemic.

You can send us your submission for our online exhibition via the form at bit.ly/yourpandemic or via WhatsApp on 07375 354687 Please make sure it reaches us by 30th Sept 2022 If you have any questions, email media@phauk.org

Films and footage

We’d love to share any videos you took on your phone or edited together over the last couple of years.

Grea t for k i ds too !

Your pandemic, your word

Write down one word that you associate with the COVID-19 pandemic and decorate it with paint, crayons, glitter, or anything you like. You may even want to knit or crochet it or use graphic design software. Take a photo and send it to us for our online gallery. The word could describe how you felt, what you did most of during lockdowns, or anything else that sums up your experiences.

This word will be personal to you, but ideas might include:

Family...Scared...Quiet... Rollercoaster... FaceTime... Baking... Vulnerable... Hope... Gardening...Time... Can’t think of a word?

Simply pick one of these to decorate:

• Coronavirus • Pandemic • Shielding • Lockdown

SUMMER 2022 emphasis 11

Simon Dawson has been married to Claire for 12 years, supporting her through her journey with pulmonary hypertension. He’s challenging himself for our charity by taking on his first marathon – and he’ll be thinking of her with every mile…

my wife For

SUMMER 2022 emphasis 12

familymatters

laire and I met online 17 years ago through the Friends Reunited dating website. She was born with transposition of the greater arteries and had open heart surgery at the age of two, with a pacemaker fitted at 15. In the year 2000, she beat all odds to become a mother of twins, but as the children have grown up, she has really struggled with fatigue and breathlessness. In 2008, she was told she had pulmonary hypertension – and the impact has been massive and progressive. Claire used to work full-time and gradually had to reduce her hours to a point where she had to give up work completely. Over the last few years, the PH has developed more aggressively, and it has reached a stage now where we are having to sell our family home because she can’t walk upstairs without feeling dizzy and having to sit down. We tend not to go out anywhere anymore as we can spend days planning something and then have to cancel at the last minute as she is having a bad day. Over the years, Claire has lost contact with most of her friends due to the last-minute cancelling of planned events because of her fatigue. The biggest impact of Claire’s PH on my life could be summed up as guilt, anger and frustration.

I don’t like the word ‘carer’ but I suppose you could put me in this category. We are a family, she is my world, and I would do anything to support her. Frustration because we don’t go out, I come home from work and the house is a mess because she has had a bad day and couldn’t get off the settee, and holidays we can’t go on. Guilt for feeling frustrated because it isn’t Claire’s fault, and anger at her disease as she is such an amazing, caring, beautiful person and she doesn’t deserve to have this happen to her. I don’t like the word ‘carer’ but I suppose you could put me in this category. We are a family, she is my world, and I would do anything to support her. What makes me happy is seeing her happy. If I have to iron her clothes, help her get ready, make meals, or clean and tidy, just to get a few more hours per day of her being mobile and communicative with me, then I would do this without thinking. The support I provide for Claire changes daily and is Simon and Claire with their children

never the same two days in a row. I have to work weekdays, and I am out of the house from 7.30am to 5.30pm. On a good day for Claire, I would get in from work and she would have tea ready for me, so after this, I would clean up, wash dishes, and do evening housework where required. On a bad day, I would get in and tidy the living room, make tea, clean up and do evening housework. Weekends would be similar. If Claire is having a bad weekend, which is more and more frequent now, I would pretty much do everything around the house to allow her to rest. It is very hard emotionally for Claire. I can see the pain in her face as she is a very intelligent woman, and she gets very forgetful, can’t get words out properly, and sometimes misses important events such as medical appointments as she cannot remember them. She wants to go out and visit places, go for walks with our dogs, and just do what ‘normal’ people would do, but she simply Cont...

SUMMER 2022 emphasis 13

familymatters

cannot. She doesn’t have the energy, stamina, or drive to do any of this. I am taking on the Newport Marathon for the PHA UK in October because I want to raise awareness of this condition as well as money for the charity. The PHA UK has been so supportive to my wife over the years, and anything I can do to help them help others with the same condition is the very least I can do. I find running boring and would much prefer to be in the gym, but I chose a marathon because I wanted to do something that requires months and months of dedicated training, and something that less than one per cent of the population has done. I want to try and raise awareness of PH as it is a misunderstood and poorly known disease, and the only people that really know about it are those that are either suffering from it themselves, or those who live with someone who is.

SUMMER 2022 emphasis 14

The PHA UK has been so supportive to my wife over the years' To be honest, I don’t even think a lot of close family members truly understand the debilitating effects of PH unless they are living with someone with the condition 24/7. Before covid, I would say I put work and my career too high on my agenda. When lockdown meant I worked solely from home, I really took notice of

how bad Claire and her condition had progressed. Being so caught up with my work and career, I had so much on my mind that the most important person in my life became second place. I have subsequently changed jobs and completely changed my work-life balance. Jobs come and go, but your loved ones do not.

You can sponsor Simon for his marathon challenge at justgiving.com/fundraising/dawson-s

Claire celebrating a birthday whilst shielding

Do you care for or support someone with PH? This is your chance to be part of an important new study into coping.

We’re working with researchers to investigate the impact of PH on individuals and their caregivers, and whether ‘self-compassion’ can be a helpful way of coping. The word self-compassion can mean different things to different people. For some, it means treating themselves with kindness, care and understanding. Self-compassion may be especially important when we are experiencing difficulties in our life. The results of this study will be used to help us better understand how people cope with the impact of PH and inform possible treatments in the future. Please note, the term ‘caregivers’ is used to describe anyone who supports someone living with PH. This could be a partner, family member or friend, and the support you provide could be emotional, physical, or practical. This research is being conducted in collaboration with Nottingham Trent University and Cardiff University and is being led by clinical psychologist Dr Gregg Rawlings. Dr Rawlings has worked with PHA UK on similar research studies, including developing the valuable selfhelp programme on page 24, which has now been made available to everyone.

This study into compassion and PH is for you if: • You are over 18 and live in the UK • You provide unpaid care for or support someone with pulmonary hypertension

What’s involved?

You will first be asked to read a Participant Information Sheet that will provide you with more information about the study. You will then be asked to complete a consent form before a series of questionnaires asking you about your demographics and experiences of anxiety, depression, quality of life, self-compassion, and ‘caregiving burden’. The questionnaires should take approximately 20 minutes to complete.

Find out more or volunteer to take part at bit.ly/compassion_PH Please sign up before 5pm on 15th August 2022.

If you have any questions please contact Dr Gregg Rawlings, at Gregg.Rawlings@NTU.ac.uk

T hi shoul s study more d take no an ho than half ur of time! your

SUMMER 2022 emphasis 15

Our experiences of having Earlier this year, we asked people with pulmonary hypertension to share their experiences of testing positive for covid, in response to requests from members who told us they would find this helpful. Thank you to everyone who told us what it was like for them. We have included a small selection in this feature, and you can read all of the accounts on our website at

bit.ly/CovidExperiences

SUMMER 2022 emphasis 16

COVID “

I had fever, headaches, body aches and coughing. I also felt very tearful and strangely very hungry first thing in the morning! I was petrified that I would end up on a ventilator if I became covid positive but after the two jabs and the booster, plus the weaker strains (hopefully), having had covid, I recovered quickly.”

Sindy, 56, tested positive in April 2022

“

It wasn’t as bad as I expected it to be. I had lived with the anxiety of catching it for such a long time, I didn’t realise how much that had hung over me. Catching it and knowing that I got through it actually gives me some comfort now, despite feeling really rough at the time.”

Jozee, 34, tested positive in February 2022

“

I had a tickly dry cough for a couple days, a headache, raised temperature and I felt very tired. I took paracetamol and slept more than usual. I felt anxious about how it would affect me as it ran its course. I was given the molnupiravir anti-viral treatment on the fifth day after my positive test. I was very worried about how and if I would be able to cope but to be honest it wasn’t as bad as I was expecting, probably thanks to the vaccines. It’s a relief now to know that my body can deal with it in spite of my PH, and it’s taken a lot of the dread and fear out of becoming infected should it happen again. I will still be very careful though to avoid it!”

Angela, 65, tested positive in January 2022

D-19 “

My main symptom was light-headedness and feeling dizzy when standing, aching and cold-like symptoms, cough, and I lost smell and taste. I now have long-term tiredness and am more breathless than before. I am exhausted after the smallest exertion.”

Irene, 67, tested positive in November 2021

“

I had a cough (coughing up blood), temperature, aches, headaches, exhaustion, and difficulty in breathing. I was admitted to hospital a week after testing positive with severe dehydration and covid pneumonia. I had oxygen treatment, antiviral drugs, antibiotics, steroids and paracetamol.”

Ria*, 55, tested positive in October 2021

“

I had diarrhoea, extreme tiredness, loss of taste and smell, achy muscles and a headache. I didn’t receive any medical treatment.”

Brian, 64, tested positive in April 2022

“

I had a sore throat, cough, lost my voice, headache, tired and breathless (but not excessively). I built it up to be such a huge thing and I was very scared about contracting it due to media and everything being conveyed as very scary but from my experience it was no worse than normal flu.”

Laura*, 23 tested positive in March 2022

“

My symptoms were loss of taste and smell, blocked nose, headache, dry cough (only for a day before testing positive) achy body and tiredness. Although it wasn’t nice to have, I was surprised that it didn’t actually have an impact on my PH.”

Amy, 28, tested positive in February 2022

“

I had what felt like a bad cold for two days then a really upset tummy for two days, then felt fine by day five. Although I went back to work as I felt well enough, I did realise very quickly that I was extremely tired by the end of the day. I do think this is still the effects of covid a month on as I’m still feeling this way.”

Deborah, 47 tested positive in March 2022

“

I had a high temperature for two days, with a headache and general aches. I also had a sore throat and irritated airways for a few days. A couple of stomach cramps for one day, and cold symptoms for about a week. It didn’t really affect my breathing and symptoms were pretty mild really. I had an infusion of sotrovimab on day five due to being immunosuppressed, but I felt I was getting better before I had it.”

Jill*, 46, tested positive in March 2022

Everyone who shared their experiences with us was up to date with their vaccinations. Many of them have other health conditions alongside pulmonary hypertension, which is why some received anti-viral treatments after testing positive. Visit bit.ly/CovidExperiences to read all of the accounts we received.

*Name has been changed on request

SUMMER 2022 emphasis 17

Helping you to live well with PH We have produced this new resource to fill a vital information gap and enable people affected by pulmonary hypertension to understand more about palliative care and how it might help them. • Information and expert advice • Real experiences • Useful contacts

...and much more.

MAKING

CHOICES Your Anticipatory Care Plan

Includes a separate planning booklet to help you prepare for the future, so you can live for the now

Order FREE at www.phauk.org or call 01709 761450

w e N E RESOURC

Continuing the conversations Our new publication aims to get people talking about palliative care, so that more patients and families can benefit. PH consultant Dr John Wort shares his thoughts on this important support that is so often misunderstood…. I like to describe palliative care to my patients as being part of symptom control, but it’s much more than that. It’s much more holistic and involves looking after emotional aspects, psychological aspects, financial aspects and more. Palliative care is not just for the patient; it can help with all these aspects for families, carers, and friends too. They are sharing their journey, emotions, and sometimes fears. Palliative care can happen at any stage of the patient’s journey, and it should be addressed right from the beginning. For instance, if a patient is newly diagnosed, the main issues may be breathlessness, anxiety, and fear – and palliative care is really important there.

If you have questions about palliative care, don’t be scared about asking them. I think having more information, like the new resource being provided by the PHA UK, should give patients confidence, and empower them to have those conversations. It’s important that people aren’t left with a negative feeling about palliative care, as there are so many negative connotations with it. Flip it over and see it as positive, as it’s about trying to help the patient live well and have a better quality of life. It’s about so much more than end-of-life care. Palliative care is for the whole patient journey, and for their loved ones too.

Dr John Wort is Clinical Lead for Pulmonary Hypertension at the Royal Brompton Hospital in London. You can hear him discuss more about palliative care by scanning the code to access our video.

SUMMER 2022 emphasis 19

“ We knew everything about each other. Apart from this.” Let’s give the people we love, the certainty they need. Talk to your loved ones about organ donation.

Visit organdonation.nhs.uk

SUMMER 2022 emphasis 18

Mother-of-two Natalie Kerr was diagnosed with PH at the same time she had her little girl, and ten years ago, she received a life-saving transplant that has enabled her to see her children grow up. Over the page, Natalie tells us what the last decade has meant for her family, and why she’ll always be grateful to the woman who gifted her lungs.

“I have probably lived more in the last ten years than a lot of people have lived in their lifetime”

A decade of

gratitude SUMMER 2022 emphasis 21

Natalie shortly after her transplant

started getting out of breath following the birth of my son Brandon, but I wasn’t diagnosed with PH until six years later, whilst I was in labour with my daughter Isabelle. I was heartbroken. I really suffered for the four years before my transplant. I needed a wheelchair and oxygen, and even on maximum medication I was still passing out. I had to limit how often I could hold my baby, because I was scared of blacking out with her in my arms. I couldn’t take her out in the pram, so I’d push her around the house instead. I missed so many experiences with her. I was on triple therapy, and I’d been assessed for transplant, but I didn’t feel ready until about my third year of being poorly. I had a right heart catheter which showed my pressures were through the roof and I realised then that I had had enough. I was running out of time.

"My children still have their mum because of my wonderful donor. She is a hero " I was placed on the list for a double lung transplant in October 2011 and that Christmas was hard. Isabelle was three and Brandon was nine, and every time the phone rang, he would ask if it was my new lungs. In the end, I was only waiting for 17 weeks. I expected some false alarms, but when the call came, I just knew it would work out. I was back home three weeks later and was a textbook patient, doing everything I was told.

SUMMER 2022 emphasis 22

It took a long time to get back on my feet properly, but I have probably lived more in the last ten years than a lot of people have lived in their lifetime. I’m so grateful to see every single day and be with my children, family, and friends. Everybody that knows me will tell you how happy I am, and how much I make the most of life. I’m not afraid to do anything; I want to see places and really soak everything in. I love to go to the beach and listen to the sound of the sea. I appreciate every little thing. I love celebrating birthdays and Christmas, and I’ve gone on so many holidays with the kids. We’ve been to Disney World, Venice, Lapland, Paris, and on so many adventures. I am just so happy to have been alive for the last ten years and every day is really special. It is such a gift. My children still have their mum because of my wonderful donor. She is a hero. She was 52 when she gave me her lungs, and she had a 17-year-old son. It does make me sad that I’m here with my children because she’s not with her boy. I am in touch with her brother, and he sends me a message every year on the anniversary of my transplant. He finds great comfort in knowing that I’m doing so well. I dedicate a lot of my time to raising awareness of organ donation, and a few years ago I started a petition to change the law to an ‘opt out’ system, which got over 15,000 signatures. I visit hospitals to speak to junior doctors and intensive care nurses about my story, and I’ve done lots of media interviews over the years. The kids and I even fronted a campaign for NHS Blood & Transplant, appearing on billboards around Manchester. I think of it all as my way of giving something back. I know life has not worked out how I imagined it to, but I think it has worked out for the better. I wouldn’t have experienced all the things I have, or made all the friends that I’ve made, if this hadn’t happened to me.” Natalie with her children

indness k f o n o i t a r b e l e Ac

and celebrate ten ay d th ir b th 40 er ed To mark h t, Natalie organis n la sp n a tr er h years since h in February. a big charity bas enjoyed el in Bolton, was ot h a at d el h t, reet The even ing Coronation St d u cl in – ts es u g o had by 270 nd the doctor wh a t, en m y h W y d actor An nosis. Natalie’s PH diag ed er iv el d y ll a origin ten years after ve li a s a w I if t a “I always said th g to go big with in go s a w I en th my transplant, e. “I gave a speech li a at N id sa , s” the celebration k everybody for n a th to t h ig n e at the end of th e, and the whole m p el h to e on d what they had r me. I have never fo ed p p a cl d n a room stood up efore.” thing like that b y n a d ce n ie er p ex split 000, which was 5, £ er ov ed is ra art The party ospital’s New St H e aw sh en th y between W PHA UK. Charity and the

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With M Grana el Barham prese da Repor ts , nter

SUMMER 2022 emphasis 23

ARE YOU STRUGGLING WITH 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

Request the programme at www.phauk.org or call 01709 761450 SUMMER 2022 emphasis 24

NEW

self-help programm e from the PHA UK

ou es, helping y ri se w e n r u O ore a little bit m w o n k to t e g rk ople who wo about the pe K’s specialist within the U twork PH centre ne

Behind th

job titlee

On the spot Meet Chito Gabutin, Lead Nurse within the Royal Free Hospital’s pulmonary hypertension service

What’s the most challenging aspect of your role? Getting things right the first time; caring for the patients is not a trial-and-error process for me. What’s the first thing you do when you get to work, and the last thing you do before you leave? The first thing is a coffee (vanilla latte) and the last thing I do is unfold my sleeves. Could you sum up your Royal Free colleagues in three words? Driven, patient-focused, compassionate. I lead a super team of nurses. What does lunch look like for you on a work day? A microwavable meal with an online meeting in the background. What’s your favourite vending machine snack? Ready salted crisps or a Snickers bar. Who or what inspires you at work? Complex patients and situations. What do you love about the NHS? The amount of work that has been put in to make the service better. How do you like to spend your time off? Reading work emails sleeping and baking brownies.

e a PH professional to If you’d like to nominat ail media@phauk.org be put on the spot, em some questions ;-) You can even suggest

What’s the best thing about your job? Being able to spend time with patients, even just for a chat and hearing they have improved.

C ri s

R SAELADY TE D SUMMER 2022 emphasis 25 19

spot lig Fundrai

Celebrating

sing

Sabbah

Friends and family of a young woman who lost her life to PH came together in a special way to raise money and awareness in her memory. Sabbah (lef t) and Naseem

d and rs an eers ntee lunt volu thee vo of th mee of Som the event of y da So e onthth ts an event ip e ic rt at pa s nt pa partic

abbah Wasim was just 41 when she died last year, and May’s event at Cannon Hill Park in Birmingham was a tender celebration of her life. The sun shone as family and friends walked or ran a 5km route around the grounds, and afterwards shared memories and stories of times spent together. The event was organised by Sähëlï Hub, a volunteer-led organisation that works to empower women in Birmingham by providing fitness activities and healthy lifestyle advice. Sabbah had spent 21 years as a volunteer Treasurer with the Hub, forming close friendships with her fellow Trustees. CEO Naseem Akhtar said: “Sabbah was the most committed and supportive Trustee, a cheerleader for Sähëlï Hub. She joined as a girl at 20 and blossomed into an amazing woman. She was fiercely loyal, totally independent, hardworking, and generous with her time and commitment to us, her family and friends. Sabbah will remain in our hearts and our minds, and we will miss her presence at all our activities and events in the future.” All entry fees were donated to the PHA UK and runners and walkers collected sponsorship too. Those who couldn’t make it made donations in Sabbah’s memory, and in total the celebration raised £2,157 for our charity. It also generated a huge amount of awareness of PH, thanks to conversations started in the park and media coverage in the lead-up to the event.

SUMMER 2022 emphasis 26

s, who Sabbah's parent of their s ie or shared mem ent ev e th at er daught the Sabbah and m a te ï ël Säh

We are very grateful to everyone involved in this event who supported our charity in memory of loved ones. It was an honour to be invited along and to capture the event on camera. Please scan this code with your phone to watch our short video of the day.

The event was made extra special by the coming together of two families who have experienced loss due to PH. Cadston Mason, who heard about the celebration when organiser Naseem Akhtar was interviewed about it on the radio, wanted to remember his mother Shena in this special way too. He walked with her sisters and his young daughter in her honour. Shena died in 2020, and Cadston shared memories of his mum with the group, taking comfort from being around others who understood. The family raised £256 in sponsorship, and writing on his fundraising page afterwards, Cadston said: “It's been a beautiful event despite the circumstances and it was nice to all come together and share stories of Sabbah and Shena. Sabbah has a beautiful family who you can tell absolutely adored her and are so proud of what she had accomplished. The weather was lovely, and I strongly believe that they were looking down on us.”

Cadston and his mother's sisters at the event

Cadston with his children and Shena

SUMMER 2022 emphasis 27

Putting patients

first

he PHA UK has an ongoing partnership with researchers from the University of Cambridge and Royal Papworth Hospital to ensure clinical trials consider the needs of patients and their loved ones, and earlier this year, we teamed up to conduct an online survey aimed at understanding what matters to this community. With implications for all future research too, the survey was conducted

By taking part in clinical trials, patients play a vital role in researching new treatments for pulmonary hypertension – and it’s important your voices are heard when it comes to designing them.

with the specific aim of shaping the design of a new trial called StratosPHere. This trial will investigate whether two drugs – phenylbutyrate and hydroxychloroquine – are beneficial to patients with pulmonary hypertension. These drugs are currently being used to treat other conditions. There are lots of new and innovative ways the team hope to carry out this trial, and this survey asked for opinions on these methods and much more.

A message from Dr Joseph Newman, Clinical Research Fellow, University of Cambridge and Royal Papworth Hospital: “The COVID-19 pandemic has changed the way that healthcare is delivered. It is also changing the way that research, and clinical trials, are designed and carried out. There are still many aspects that are just done ‘the way we've always done them’. We are keen to re-think this both from the medical and scientific perspective, but also by understanding what is important to you, the patient. We want to make clinical trials as straightforward for you to take part in as possible and measure aspects of your health that most reflect your day-to-day life, without compromising the quality of the results. Overall, the responses to this survey will help shape the StratosPHere trial, being launched later in 2022, but also other future research in PH and other heart and lung diseases. I would like to say a heartful thank you to all the patients with pulmonary hypertension who completed this survey.”

SUMMER 2022 emphasis 28

112

people wit comple h PH survey. ted the Th to each ank you and eve ry one of y ou !

The findings will now directly inform the protocol and delivery of this trial, as well as influencing the design of other PH trials in the future. Over the next few pages, we bring you some of the key findings and comments from the survey. Additional findings around topics including sleep, EmPHasis-10 and the use of technology are included in the full report, which can be viewed online at phauk.org. You can also access it by scanning the code on page 31.

Clinical trials What matters to you? The top 5 reasons

people gave for wanting to take part in clinical trials were:

of respondents said they had taken part in a clinical trial which involved taking a drug for pulmonary hypertension.

1. To help other people with PH in the future 2. To advance science 3. Because the drug treatment I would get in the trial may help me 4. As a patient with a rare condition, I am a useful ‘resource’ for science 5. So I can ‘give back’ for the treatment I have received

Respondents were also asked what might discourage them from taking part in a trial.

The top 5 reasons were:

of those who have taken part in a clinical trial that involved taking a drug for pulmonary hypertension said they would do so again.

1. I would be worried about the side effects from medication 2. I wouldn’t want to travel a long way, or frequently, to a specialist hospital 3. The cost that may be associated – eg. travel to hospital, parking, or time off work 4. I wouldn’t want extra invasive tests or procedures at hospital (like a right heart catheter) 5. I would be worried about taking a new drug More results from the survey SUMMER 2022 emphasis 29

Putting patients

first

“Even though I am self-employed I usually have to take at least 2 days off to go to the hospital.”

“I don’t feel stress about visiting my centre but I feel really stressful about getting there and back.”

82 % 70% of patients attended their PH centre at least once in the 12 months between March 2021 and March 2022 (compared to 92% pre-pandemic)

93% Respondents were asked: “If some things that normally take place during your visit could be done from home, such as answering questions about your symptoms, would you be happy for this to happen via telephone / video call instead?” If asked by a PH specialist to report symptoms, side effects or problems via an app, most respondents said they would be happy to do this: - Daily if it took 5 minutes or less - Weekly if it took 15 minutes or more SUMMER 2022 emphasis 30

of patients travel to their specialist centre by car and for 63% of patients, the journey takes more than an hour each way.

Almost all respondents said they would be happy for at least some aspects of their healthcare to be remote.

50% "Yes I would be happy" 43% "Yes, but I would still 7%

like face-to-face visits for other things" "No, I would not be happy about this"

“I think we need the visit to be reassured and seeing other patients is very beneficial to people with a rare disease as it gives you a sense of you’re not alone”

“I’ve been doing 6-Minute Walk Tests regularly at home during the pandemic and passing that info to my team at online or face to face clinic appointments”

82 %

of respondents said they have completed a 6-Minute Walk Test at their specialist hospital.

81 %

said that if they were trained to measure distance using an app on a smartphone or digital device, they would feel able to complete a 6-Minute Walk Test outside their house.

50% said they would be prepared to take phenylbutyrate as part of a clinical trial and 54% said they would be prepared to take hydroxychloroquine.

hydroxychloroquine

Respondents were asked to read short information sheets about the two drugs, phenylbutyrate and hydroxychloroquine, that will be used in the StratosPHere trial.

phenylbutyrate

Concerns included: “Finding somewhere accessible that is flat” “Interruptions from other people whilst outside” “Accurately trying to measure the distances”

50%

You can access more of the findings in our full report by scanning this code with your phone

54%

SUMMER 2022 emphasis 31

o t t o g e v ' u o Y “ d n a , t s u r t e v ha p e e k o t t o g e you'v ” g n i o g f l e s r you SUMMER 2022 emphasis 32

Maria with her youngest daughter and husband

After being diagnosed with idiopathic pulmonary hypertension in 2010, Maria Brook has found many ways to stay positive over the years. As part of her ongoing commitment to helping others, here she shares what she has learned.

hings weren’t easy when I first got my diagnosis. I had to give up my job in retail (I was a manager of a shoe shop), and I still find that hard to this day. I couldn’t even get myself to the bus stop back then, but life started changing for the better and these are some of the things that have made a difference along the way…

Setting goals and celebrating the small things

I work on goals and that’s how I live. When I was ill for the first time in intensive care, I had to learn to walk again, and I’ve been setting goals for myself ever since. I think it’s really important. I have some that are quite easy to achieve, some that are ‘medium’ and some that are hard. A small goal might be cleaning and wiping down my shower doors, because I need to bend down. A medium one would be managing to do half the shower, and a hard one would be doing the shower and the washing too. If I don’t achieve a particular goal one day, I try not to get deflated and instead I’ll try again the next day. I write things down and tick them off once I’ve achieved them, and if it’s a bigger goal, I might break it up into smaller steps. I’ve been talking to [PHA UK team member] Paul Sephton on the PHA UK’s Listening Line service and he has helped me a lot, as we set goals together and I can then tell him what I’ve achieved the next time we speak. He helps me to brush it off if I don’t achieve something first time, and encourages me to try again. One goal, after the covid lockdowns, was to go out for a meal with my family. The first time I went into a restaurant I froze and started shaking, but it’s getting better. One place I really missed through covid was IKEA and I made going again one of my biggest goals. I’m pleased to say I did it, in April, with the help of my friend who came with me.

Friends, family and keeping active

Having a lot of friends around me to be able to talk to has helped throughout

the years, and my family have been important too. It really helped me see my eldest daughter Leanne-Elise go through university and get the job of her dreams. I’m very proud of her. I also want to see my youngest daughter get through her milestones and that motivates me to keep going. I wanted to see her get to senior school and see her through prom and her GCSEs. I wanted to see her get to university and now I want to see her graduate, get a job, and get married. It’s helped me an awful lot to concentrate my mind on her. My husband is wonderful, he’s my rock. He helped me take an allotment to grow seeds in, and he and my daughter did all the digging and planting. It was a team effort, and it’s helped me to feel like I’ve achieved something. Soon after I was diagnosed, I joined an exercise group and made friends with some of the elderly people there, who encouraged me to join the Women’s Institute (WI), and I became part of the committee. Friends would come to visit me too, as I like to talk to people, and all that really kept me going. The pandemic stopped all that, but I’m starting to get out and about a bit more now. Getting outside and doing some exercise is important to me. I try to do walking with my friend and if I’m at home, I follow light exercise videos on YouTube or do yoga on the Wii Fit console.

A helping hand

If I can help people that's the best thing, because I feel like I'm doing something then. I’ve got a friend who suffers very badly with depression, and she had to give up work too, so we’ve started doing lots of things together.

Out for a meal with family, one of Maria's post-covid goals

The long-awaited trip to Ikea, in April 2022

"No matter how ill I am, I still like to help people. " We encourage each other to go for walks and it gets us both out of the house. Once a week I cook a meal for an elderly lady who lives locally. I like cooking, so it helps me too, and I push myself to do it even when I’m not feeling great, so it’s a good goal to have. If I really can’t do it one week, my husband will do it for me. No matter how ill I am, I still like to help people.

Pulling out the positives

Sometimes I do get upset about things because I’m only 58. I’m on oxygen 24/7 and when I was diagnosed, I was only given six years, and that is often on my mind. I look at couples walking along the street holding hands, and I feel bad that my husband and I can’t do that because I’m in a wheelchair. I feel bad that we can’t do fun things as a family. But I have to pull myself together and remember that I’m still here, still seeing my daughters, and I’ve still done lots in my life. There are people who are far worse off who haven’t had that chance. My specialist centre has really helped me, which has made a big difference, and I have a lot of trust in them. You’ve got to have trust, and you’ve got to keep yourself going.

SUMMER 2022 emphasis 33

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 2000 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.facebook.com/groups/125342770826215

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.facebook.com/groups/402808577486104

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.facebook.com/groups/215307796620821

rofile in a Facebook p e v a h to d ee p at You do n roups. Sign u g e es th in jo order to and easy ! om – it’s free .c k o o b ce a .f www

Spread the word!

Not yet a member? Join our PH family for free today Be part of a 4,500-strong national support network

Are you living with PH, or do you have friends and family who are? We’re here to support people like you. Join today and benefit from: • Support and advice • Helpful printed information & 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 01709 761450 office@phauk.org

FREE

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Pulmonary hypertension affects people all over the world, and everyone has their own story to tell. In this special feature, Tegan, Dorothy, Mark, Erin and Kamil share their experiences from different corners of planet earth.

SUMMER 2022 emphasis 37

My life with PH in

America. Dorothy Watts lives in Seattle, Washington, and was diagnosed with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) in March 2020. The 31-year-old technology product manager has undergone numerous balloon pulmonary angioplasty (BPA) procedures and has found new ways to stay positive and active with her condition.

for a In hospital ure BPA proced

SUMMER 2022 emphasis 30 36

was diagnosed with deep vein thrombosis in June 2011 after starting birth control and in May 2019, I began experiencing leg pain, but I did not want to see a doctor due to upcoming trips. I travelled several times in the following months, including several overseas trips. During the summer of 2019 I became increasingly out of breath and dizzy; eventually, it got to the point where I could barely walk one block (this was very unlike me – I had just ran a half marathon earlier that year). However, I attributed my breathlessness to all of my travel,

a half Completing -diagnosis re marathon p

working out less, moving across the country, and starting a new job. Finally, I went to see a primary care doctor in Seattle who told me to go immediately to the ER [A&E]. I ignored her and instead got on a four-hour flight as I had a friend’s wedding in Chicago; however, while in the air my resting heart rate jumped above 120. I went to the ER upon landing and there was diagnosed with bilateral pulmonary embolisms. I went back on blood thinners and met with a pulmonologist, who ran more tests and finally diagnosed me with CTEPH in March 2020.

Balloon pulmonary angioplasty involves a very fine wire being inserted into blood vessels in the lungs, guiding a tiny balloon into position. The balloon is inflated, to around the size of a pea, for a few seconds to push the blockage aside and restore blood flow to the lung tissue. The balloon is then deflated and removed. This can be repeated several times in different parts of the lung during a single treatment session.

The healthcare system in America

Adapting to life with pulmonary hypertension I am on medications to prevent further blood clots and treat my PH. I also had six balloon pulmonary angioplasties in 2021, as I am not a candidate for a pulmonary endarterectomy. The BPAs helped reduce the pressures in my heart, and the medication has increased my exercise tolerance. The two biggest impacts of my CTEPH have been the changes to my lifestyle and exercise and the toll on my mental health, especially immediately after diagnosis. I went from being a very active young adult running half marathons to not being able to walk up a flight of stairs in a matter of weeks. Even after two years of treatment (including medication and BPAs), I still cannot run and have had to find new ways to exercise. There are a lot of outdoor activities in and around Seattle (such as hiking and skiing), but I have to be intentional about how much effort I can extend, and the altitude, which leads me to miss out on things. Adjusting to this new normal, especially as the pandemic set in, was difficult. I have found new ways to stay active, including yoga, walking, and recently, even low-impact Peloton [exercise bike] classes. I enjoy spending time with my husband, family, and friends, and travelling. My excellent team of doctors have also helped me stay positive, especially though the past year of treatment.

It can be extremely confusing and frustrating trying to navigate the US healthcare system. I am aware of four main ways to get health insurance in America: • Medicare, which is provided by the government once you turn 65, or if you have a disability • Medicaid, which is income-based insurance provided by the government • Coverage through the Affordable Care Act (aka ‘Obamacare’) • Private insurance through an employer Health insurance options are further broken down into different coverage types, and it is usually up to the patient to determine which plan is best for them based on how much they anticipate spending on healthcare in the next calendar year, and if their preferred providers will be within their approved healthcare network. Extra services such as dental, vision, and mental health services may not be covered in all plans. I feel extremely lucky to have access to private health insurance through my employer, and to be able to afford my deductibles and out-of-pocket expenses. When I was first diagnosed, I was on a lower plan since I did not anticipate I would have many medical expenses. Because of this, my deductible,out-of-pocket expenses, and copays [what you must pay towards costs] were high. During the first open enrolment period post-diagnosis, I changed my plan to the premium option; while the amount taken out of my paycheck each month has increased, my overall expenses are lower, and I am covered for nearly all expenses. There are hospitals around the US that are well-known for their expertise in PH and CTEPH diagnoses and treatment, the most well-known being University

Hiking w it husband h her last year

of California at San Diego (UCSD). While I was not treated at UCSD, many of the doctors at treatment centres across the US trained at UCSD, including several of my doctors at University of Washington. When I was first diagnosed, I connected with a Facebook support group managed by volunteers from the [American] Pulmonary Hypertension Association. There are several PHA groups, including groups dedicated to CTEPH and young adults. Through PHA, I have ‘met’ many other patients, gotten advice on different treatment plans, attended a virtual support group event, and heard others’ stories. I also follow some other PH organisations on social media to stay updated on PH research and events and to connect with patients. Finding a good support system is important, whether it be people in your life or internet strangers with PH.

Next, meet Mark in Dubai SUMMER 2022 emphasis 39

My life with PH in

Dubai. Originally from England, Mark Pincock has lived in Dubai for 11 years, and was diagnosed with PH out there in 2020. At the time of this interview, he was waiting for a liver transplant and plans to return to the UK when he is well enough. The 53-year-old, who moved to the United Arab Emirates (UAE) after leaving the army, told us his story…

Explorin mount g the UAE a wife M ins with his yr na an Completing bai in u D in n a m n Iro diagnosis re 2019, befo

have always been fit and active and at the time I got ill I was training and competing in some triathlons. I knew my body, my physical abilities, and importantly what felt right. In 2018 I gradually felt my sporting performance declining, dropping down the scale in competitions, and despite training harder, I was getting slower and struggling more with breathing control. I began regularly to feel dizzy when getting up from my chair, and breathless

Cycling wit h fr in the Duba iends i desert

Kayaking in H atta Dam with his wife

nd Clinic The Clevela ere Mark wh Ab u D ha b i atment e tr s e iv rece

walking up two flights of stairs at work. Finally, during a triathlon in October 2019, I felt very dizzy and faint, almost blacked out and held onto a wall for support. This scared me. In November 2019 I went to see a cardiologist at a hospital in Dubai and several tests indicated pulmonary arterial hypertension (PAH). I was then referred to a clinic in Abu Dhabi to see a PH specialist, where the diagnosis was confirmed.

A complicated health picture

At the same time, I was seeing a hematologist in Dubai about irregular blood results, which were first discovered in 2008 when I broke my leg in the UK. After many tests in Dubai the hematologist never found an underlying reason. Suspected liver cirrhosis was mentioned on an abdominal scan report (little did I know this was the underlying cause of my PH). Because this was a different private hospital in Dubai the information was unfortunately never shared with the clinic that diagnosed my PH, and I never thought anything of it as I hadn’t read anywhere that the liver could cause PH. Then in April 2021 I had an abdominal hernia, and a scan showed cirrhosis of the liver. This time, I mentioned it to my PH doctor, and he suspected at once that portal hypertension may be the cause of my PH. After further tests in January this year it was suggested I should have a liver transplant, and after what felt like a million more tests, including another right heart catheterisation, I was placed on the transplant list in February. Although this is pretty scary and was totally unexpected, I am at the same time very optimistic that this will cure me of liver cirrhosis/portal hypertension and hopefully reduce or eradicate my PAH. I see it as short-term pain for long-term gain and feel very fortunate that I may be curable, and live a longer healthier life than I initially expected on diagnosis of PH.

Healthcare in the UAE

The healthcare system here differs immensely from healthcare in the UK. The government hospitals are for free use by UAE citizens, but expatriates can use them free for emergency treatment, with other

care claimed back on their health insurance. I am most fortunate in working for a good company that provides top class medical cover, up to AED 2.5 million (£500,000) per year with a maximum co-payment of AED 100 (£20) for any medications which are supplied three-monthly. As an example of costs, a 30-tablet pack of Opsumit (macitentan) costs AED 17,000 (£3,500). I am being treated at Cleveland Clinic Abu Dhabi (CCAD) which is incredible. It is a large modern private hospital that not only looks like a five-star hotel, but it also has the most amazing professional healthcare staff recruited from around the world (with many British amongst them).

Staying active

PH has affected my physical and sporting activities. A few years before my diagnosis I had got quite fit again and took up triathlon which I enjoyed immensely. I enjoyed the planning of training, and loved the competition, but mainly I liked the community spirit that exists within those training and competing in the sport. So, whilst I have now given up triathlon I still keep as fit as possible and train within my limits, which for me means much less speed and distance when cycling. Unfortunately, I have recently stopped running as I find it almost impossible to control my breathing and actually found it quite frustrating and demoralising, so I walk instead. I do feel fortunate in that I am told I caught the condition quite early because of my physical activity. I feel very lucky that I am not affected that badly yet, my life hasn’t changed much at all, and I have fantastic medical facilities and staff available – who are taking great care of me. Being able to get outside and do stuff keeps me positive and focused and so far, I’ve been able to continue doing most things I like doing.

Building a support system

I live with my wonderful, supportive, and caring wife Myrna. She comes to many of my appointments with me and understands most of what is going on. I’d be lost without her and sometimes I forget what a mental worry it is for her. Obviously as I live abroad, I’m not surrounded by immediate family, but I

Just a week after this interview, Mark received the life-changing phone call that a liver was available for transplant, after less than two months on the waiting list. He is pictured here with a nurse two days after the operation.

speak to them regularly by video call and they are all aware of what is going on and are always supportive. My close friends here in UAE are always on hand to help if I ask and our good friends in Abu Dhabi put us up at theirs when I can’t travel home to Dubai after some procedures. There are no PH charities over here that I am aware of and I’m sure my consultant would have put me in touch with one if there was. This is one of the main reasons I looked up the PHA UK and it has been a great help and source of information.

Changing plans

My wife and I were expecting to retire to Spain later this year. We bought a beautiful place in the Alicante region, had it completely refurbished ready to move into and were highly excited about our future life there. Unfortunately, it quickly became clear that I would not get medical insurance to cover the extremely high costs of pre-existing PH medication. In the end we sold up and are currently buying a home close to my daughter in the Lake District instead. I look forward to hopefully spending time walking in the fells of the Lake District, walking a dog, and picking up my camera again. I am very optimistic about the future post-transplant and hope that it all works out ok. I think my biggest driver of all now is to watch my granddaughter Florence grow up.

Next, meet Tegan & Erin in Australia

SUMMER 2022 emphasis 41

Our lives with PH in

Australia. Erin Boustani, 35, lives in Sydney with her husband and two cats. Her mother had pulmonary arterial hypertension, and she was diagnosed herself at 17.

've had PH for so long that it really doesn’t concern me as much anymore. I’m used to considering it during every decision I make, and to the daily medication and the three-weekly injections. I used to let it overrun my life, especially when I wasn’t feeling well, but I don’t anymore. I would have to say the biggest kick in the guts is not being able to have children. I feel I was diagnosed early enough in life that kids weren’t really on my radar, so I was able to come to terms with it. Seeing other people with their babies or announcing pregnancies sometimes gets to me, but I’m pretty good about it now. I focus on what I can do, not what I can’t do. For example, I feel sad sometimes about not being able to have children, but I reframe it and know I will be the best aunty to my nieces. Perhaps having children was not what I was put on this planet for.

I love spending time with my husband, family and friends. I love to work out and am always trying new programmes to get stronger and stay as fit and healthy as possible. I love learning new things and am always looking to level up in some way. In terms of how temperatures affect people with PH over here in Australia, it’s a mixed bag. I hear some people have more trouble with the cold temperature and equally people have trouble with the hot temperature. For me, the icy cold wind of winter messes with my breathing. In summer, the heat itself is fine but I get swelling in my ankles which is very uncomfortable. The nice neutral spring or autumn days are perfect; not too hot, not too cold! I feel truly blessed to be in Australia with the healthcare system that we have. My PH care has been phenomenal, and I haven’t had to pay for any of it.

With husba

SUMMER 2022 emphasis 42

nd Jason

Out with fr

iends

a so n Wi t h J t h e on b ea c h

H o r se

My medications are all covered or subsidised by Medicare, which allows all citizens access to free healthcare and emergency care in our hospitals. We also have the Pharmaceutical Benefits Scheme (PBS) that significantly subsidises many medications for people. I count my blessings for this system every day. I am part of the Pulmonary Hypertension Association Australia (PHAA) group on Facebook. It is a nice group, and everyone is very supportive of each other. I enjoy the group as I know I’m not alone. I also tend to connect individually with people on Instagram to get more of a personal connection when I’m feeling down or need help. I’ve built quite a few lovely friendships that way.

E ri n a n t he i r d Ja so n o n we d d i ng d a y

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wi t h

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With one

of her cats

Erin’s advice for other people with PH “Focus on what you can do, rather than what you can’t do. When your body feels like it has failed you, tap into your mind. Having a positive mindset works wonders. Always be your own advocate; if something doesn’t seem or feel right, speak up. I have also found connecting with others very therapeutic, so feel free to reach out to chat any time.” You can chat to Erin on Instagram, where she posts as @erinboustani

Tegan also lives with PH in Australia

SUMMER 2022 emphasis 43

Our lives with PH in

Australia continued... Tegan Dunmall was diagnosed with PH as a child. Now 32, she lives near Melbourne and works as a Clinical Nurse Consultant.

was five when doctors found I had pulmonary arterial hypertension, and my mum was told my heart was the size of a male athlete’s. By the time I was 11, I was in right- sided heart failure, liver failure, and the other organs not far behind. I was on 30-40 different medications to manage fluid and breathing and seizures, but none were preventing me from heading towards a double lung and heart transplant.

oot g a photosh Tegan durin reness wa for a PAH a risbane B in n campaig

SUMMER 2022 emphasis 44

However, going onto intravenous epoprostenol is the one key thing that saved me as it is the sole reason I am here today. I am now on triple therapy, with sildenafil and macitentan too. I think PAH has impacted every facet of my life. Because I was diagnosed so young, it changed the trajectory that my life may have been on. My hobbies today are skills that I used to pass time in hospital, or while home sick. For example, I love to craft; I enjoy all sorts, but currently mostly enjoy crocheting. My job as a nurse was entirely decided as I grew up in hospitals around nurses and doctors. Everything I do has PAH in mind, from when I leave the house in the morning, to international travel. However, I don’t let it rule my life. It's always there, but never the focus. I think my family is the main reason for my positive outlook. They support me 100% and I can count on them if I need to share my burdens. My family and a few friends also support me by coming to appointments, and debriefing if I get bad news or new information. My dog Raffy is also a

r ting he Celebra th birthday 0 dad's 6

brilliant support. He keeps me active and outdoors often. The weather over here doesn’t affect me too much luckily, however, I do think living in Melbourne does help as we are lucky to have very little humidity. I find the Northern Territory and Queensland have higher humidity and this affects me a little. We have a few specialist PH centres in Australia, more than when I was first diagnosed. Each state has their own, and I have attended two in Melbourne. The first one was when I was a child, and the second one as an adult. There weren’t any children to transition before me, so my transfer was a little rocky. One day I was a child, and the next suddenly an adult.

Tegan’s advice for other people with PH

rds of In the Fjo the in N o r wa y w f inter middle o

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“Surrounding yourself with hope and positivity can do wonders. Things may be hard, but the support of your family, friends and medical team will be invaluable. Medical advances have come a long way in the 28 years since my diagnosis – from no treatment-specific medications, to being managed on three concurrently. Hope has kept me afloat in-between.” You can follow Tegan on Instagram @itsalungshot

r sisters

at With her mum a in Ch d, an yl Disne

In Barcelon

Exploring an art expo in Melbourne a, Spain

SUMMER 2022 emphasis 45

to raise at an event n g in g n si il Kam natio of organ do awareness

Standing up for patients in

Turkey. In 1991, Kamil Hamidullah’s brother was the first person to be diagnosed with PH in Turkey – and four years later, he was given the same diagnosis himself. Trading in his dreams of being a pilot, Kamil now heads up the Pulmonary Hypertension and Scleroderma Patient Association (PAHSSc), the Turkish equivalent of the PHA UK. We caught up with him to find out more. How does your story begin?

"My brother was a military officer and although his case was the first to be diagnosed in Turkey, we knew he was not the only one with the disease. At the time, Mum and Dad started organising awareness activities with their own means and tried to reach other patients. So that was how the first PH patient

SUMMER 2022 emphasis 46

solidarity group was founded in our capital, Ankara, in 1991. Back in those days, the illness was neither mentioned in books nor was there any information about its treatment methods. We lost my brother rather quickly in 1993. In 1995, it was my turn to be diagnosed with pulmonary arterial hypertension (PAH). Experts who were conducting research

on the genetic mutation of PH were looking for PAH patients with no other conditions, which was a long shot at the time. All of a sudden, I became their pop star. As a family we supported these researchers over the following years, taking part in their research study on mutation at universities in America. Together we strived against the bureaucracy of our country to get epoprostenol treatments approved in Turkey and I became the first patient

We are all tired warriors here to receive a PAH-specific treatment. After all these struggles and fights, I found myself as the leader of this disease within our patient community. When I was young, I dreamt of becoming a pilot – but one who flies an airplane, not in the context of his flight forward in his disease."

association but thanks to our significant activities throughout the year, we are the only lung patient association in Turkey to have its voice heard in Europe through the European Lung Foundation."

How is the association funded?

How did the association evolve, and what does it aim to do?

"In Turkey finding funds is very challenging so it is difficult to plan, but every year we try to donate a portable oxygen concentrator to a patient in need. We organise online interviews with patients and their relatives to find out about their experiences of every aspect of the illness. We set up other online resources to provide them with information on PH and we also organise symposiums, not only about illnesses we represent but on different issues such as lung-heart transplantation. For example, we are trying to raise awareness of the ‘opt out’ organ donation system that is in place in Britain. We hope this will be implemented one day in Turkey. We have a quarterly e-magazine called ‘Nefes’ (which means ‘Breath’ in English) in which we share with our readers what is going on throughout the world via interviews with expert physicians and associations like the PHA UK. We are a reliable information source, especially for those newly diagnosed. Also, for the sake of awareness, we have created a 6-Minute Walk game app for mobile phones. Needless to say, it was produced on a low budget and at the moment it is only on Google Play: bit.ly/3LxwEE0 We have set up a Rare Diseases Network by joining forces with nine other patient associations to find shared solutions to similar problems. In Turkey, we lack a national policy concerning rare diseases. We have accordingly presented all our issues together with our solution suggestions to the Grand National Assembly of Turkey. We may be a small rare disease

"We cannot raise funds like you do in the UK. We can’t get support from large-scale industries; we rely only on individual donations. We are grateful and wish to thank all our donors. We mostly live off with what we can do with our own resources. Once we step outside to seek help, our dependency on others increases. Our association is run totally by the efforts of its patients. At times, we even tend to neglect our health so as not to delay our operation. We are all tired warriors. Our first association didn’t survive; we do not wish to lose this one too and that is why we try to do our best. People with PH in Britain are very lucky to have an organisation like the PHA UK."

What is access to PH drugs / treatments like in Turkey?

no lung transplantation surgery can be performed on patients younger than 14. Organ donation here is so limited that patients in need of heart-lung transplantations are desperate. We have a very large population, and our healthcare system is going through difficulties. PH treatments

People with PH in Britain are very lucky to have an organisation like the PHA UK

are being continued with the devoted efforts of doctors. There are nearly 15 specialist PH treatment centres here in Turkey and families have to plan their trips and accommodation needs. As most people live on a minimum wage, the diseases naturally become a weary burden for those who live close to the poverty line. Patients do not pay for the treatment itself, but they need to spend at least six times more than the amount healthy people spend for their health in general. Families who depend on medical equipment, like oxygen concentrators, suffer from high electricity bills. In these hard times, people even must pay to get to breathe."

"As far as reimbursement of PAH medications are concerned, we are well ahead of most European countries, as nearly all our medications are reimbursed [paid for] by the Social Security Institution. We express our gratitude to relevant authorities and to those who have fought for this issue. On the other hand, organ donation figures are very low in Turkey. Prior to the COVID-19 pandemic, only 500 deceased donations were made each year and as we all know, not every organ is suitable for transplantation. Statistically, approximately 50 lungs were donated, and 22-30 patients could get a lung transplant. During the The PAHS pandemic, there Sc t e a m was a sharp decrease. Plus,

SUMMER 2022 emphasis 47

Having grown up with pulm onary hypertension, 21-year-old Kaylee Mynot kn ows a thing or two about life with a rare disea se. In her latest column for Emphasis, she reflects on some of the surprising ways that her pets have be nefitted her wellbeing.

e r u t a e Cr forts m o c I have been a massive animal lover my whole life and have always had pets growing up. But as I’ve got older, I’ve begun to realise how much of a positive impact they’ve had on my physical and mental health, especially during the pandemic.

So let me introduce you to all my pets! First there’s my corn snake e Sweetcorn, who I’ve had sinc I was ten. It was actually a t PHA UK family weekend tha made me want a snake, as they had reptiles there one year that we could hold and

learn about. Then there’s Star our Border Collie; she’s my best friend and means so much to me. Then we have Mateo our Bengal cross cat. We got him

SUMMER 2022 emphasis 48

during a break in lockdowns and he’s honestly changed my life. We do everything together and he really does mean the world to me. Mateo has always been a well-travelled cat, he loves car rides, visiting family, and s going out on little adventure etc. sts fore and nts to viewpoi s Mateo definitely encourage be and re mo me to go out more active. He’s also the best nap buddy when I’m not feeling well!

Last but not least, we , have my hamsters! Aspynn Axxie, Alaiya, Aloona, Alivia, Alizah, Aries, Aleksis and our foster hamsters Violet and Oliver. Believe it or not, my hamsters have given me so much confidence. When I star ted owning them, I had no idea about proper care (like lots of other people). But when I learnt how big their enclosures needed to be and how much they actually

er need to thrive, I wanted oth n. lear and w kno to people So, I decided to start Instagram and TikTok accounts to advocate for proper care. I was not expecting to get so many followers, my , accounts grew pretty quickly 00 and I now have over 25,0 followers on TikTok. This has also enabled me to meet and talk to so many like-minded people across the world.

Me and my friend Charlie d. now rescue hamsters in nee , ing It has been so reward seeing the hamsters go to amazing homes after being in such bad conditions.

Star

I met my best friends though hamster owning too, and I can’t imagine life without them now.

Sweetco rn

Mate o

Unfortunately, not many people know about the specific needs of a hamster, so it’s been such an amazing experience being able to educate people through social media.

Quite honestly, I don’t think I would have got through lockdowns without the animals. I haven’t ever really had many friends so having so many pets has been such a comfort for me, they really are like mini best friends. Owning pets has really made me enjoy ever ything more, I think I’d be lost without them. Pet ownership has really helped me not to dwell on the fact I have PH, as it gives me something else to focus on. It’s nice to be able to look after something, as usually it’s everyone looking after me!

Pets really do have a massive impact on our lives, and I can’t thank my beautiful animals enough for everything they have helped me through.

Kaylee has four social media accounts dedicated to her pe ts.

• Follow @theacrew_hamste rs on Instagram and TikTok for hamster updates (and very cute photos!) • Follow @truff _and_muff s_ hamster_rescue on Instagram for rescue centre news • Follow @mateo_thekitten on Instagram for feline adventures

Kaylee's column

We’d love to celebrate yo ur pets too! Send us a photo an d tell us what they mean to yo u, and you could see them in the next issue of Emphasis. Em ail your photos and words to media @phauk.org

SUMMER 2022 emphasis 49

PHAUK

Specialist support from someone who understands

LISTENING

LINE

Our Listening Line support service gives you dedicated time with a PH professional to discuss your worries or concerns. • Free and confidential • An impartial listening ear • Available to all PHA UK members, family and friends too Talking to someone who already understands PH means you don’t have to explain your condition first.

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

Calls take place with Paul Sephton, a former clinical nurse specialist in PH. Paul has many years of experience supporting people affected by pulmonary hypertension, both in a clinical environment and as part of the PHA UK team. “Paul’s support lifted me. He was so understanding and supportive, and it was helpful to chat with someone who knows about this disease.”

PHA UK member

SUMMER 2022 emphasis 59

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

COMMUNICATION

IS KEY

Consultant Pharmacist Dr Neil Hamilton on why we all have a part to play when it comes to smooth medication delivery…

s you will be only too aware, medication to treat PH can be complex and expensive. We can prescribe a range of different treatments and combinations depending on the nature of your condition if this is in line with the NHS treatment guidelines. One of the complicating factors is that GPs and hospital doctors outside the network of PH expert centres are not authorised to prescribe these treatments. I am sure it might seem to be more convenient to get all your tablets on a repeat prescription locally. However, your local team may not have the knowledge and experience in this rare condition. We, as prescribers, must make sure not only that you are getting maximum benefit from treatments, but also oversee any side effects you may have. This may include the blood tests that you need, for example with bosentan, ambrisentan or macitentan. As you may live a long way from your centre, the NHS has agreements in place with a number of homecare delivery companies to get PH treatments sent out to you. We will write, then send, your prescription to one of these companies, who will call and arrange a delivery slot convenient to you. As such, the homecare companies are a vital part of the UK PH service as the hospital teams would not be able to co-ordinate nationwide deliveries. In addition to the medicines themselves, homecare companies supply some patients with blood test

kits, and for patients on more complex treatments, they send pumps, lines and other consumable items. Crucially, there are 24-hour emergency support services for our patients on intravenous prostanoids in the event of issues with the pumps. In general, the homecare companies provide a very good service and I have been particularly impressed with how they all coped through the pandemic, rapidly changing their processes in unprecedented times to ensure patients did not run out. More recently there have been shortages of medications, and also consumables used by our IV patients, which have needed very careful management and even rationing. As a result of careful planning, patients have avoided being without. You should all have one to two weeks’ ‘buffer stock’ at all times so that if there is a problem with a delivery, you are not at risk of running out. If you have not got this much buffer, please discuss that before your next delivery so it can be corrected. At holiday times or for extended stays overseas, it is especially important to be organised with your stock levels. I would suggest that you have an additional two weeks more than your planned time away. This is a good idea to cover contingencies where you are delayed for any reason. I would strongly advise to have this stock (along with a copy of your clinic letter or up-to-date prescription) with you in your cabin bags to avoid any problems with hold luggage going astray.

There are several ways that you can help your deliveries run smoothly, and all of these highlight the need for good communication: • Make sure you are in at the agreed time slot to receive a delivery. The NHS has to pay for each delivery slot, so it is crucial these are not wasted. • They may use a number which shows on your phone as ‘unknown/withheld number’. This is also true of the hospital. Please do not ignore these calls. • If you miss a call from homecare, please return their voicemail message. • The NHS contract requires homecare companies to ask you for a regular stock check. If this is not possible when they call, please do this as soon as you can and call them back. • If you get into your last two weeks of supply and you have not heard from your homecare company, please call them. Last-minute or emergency delivery runs are difficult for everyone and costly to the NHS. These can and should be avoided.

If you have any queries regarding your stock levels or delivery dates, your PH centre is unlikely to have the information you need, so the best people to contact are the delivery companies directly. You can find their details below:

Sciensus (formerly known as Healthcare at Home)

0333 103 9499

Polar Speed 0800 783 3178 (+ option 3) Pharmaxo 01225 302188 (+ option 1) Healthnet 0800 083 3060 Lloyds Pharmacy Clinical Homecare 0800 090 2056

SUMMER 2022 emphasis 51

Keeping the

faith West Bromwich Priest Jane Dicker (known as Mother Jane) has pulmonary hypertension, has worked with hospices, and is under palliative care services herself. Opposite, she shares her unique perspective on this type of care and explains how her faith helps guide her through illness. SUMMER 2022 emphasis 52

Mother Jane has PH in association with brittle asthma (a rare form of severe asthma), which she has lived with since the age of 17. She was diagnosed with PH in 2013. She has worked within parishes all over the country, with adult and children hospices, and as a university / higher education chaplain.

work with hospices is varied. Sometimes it can just be talking with patients, and an opportunity for them to have a laugh and see the human side of what it means to be ordained. It can be playing a game of cards or reading a book – and not necessarily the Bible! I’ve helped lots of people who are not religious. Church of England priests are for everybody; it can be a misconception that priests are only there for people who have faith. It has to be trodden very lightly, because some people can be concerned that you have come to ‘talk them into’ faith, and that needs lots of reassurance. For that reason, I’ve often gone into hospices wearing just a badge instead of a dog collar. I hope that through my work with hospices, people found some peace, and were able to feel a little bit calmer about things. I hope they didn’t feel quite so overwhelmed, as we were able to break things down.

It’s very reassuring that the care is there for me if I need it

It’s vital for people to realise that palliative care doesn’t mean it’s ‘the end’. It’s about having the best quality of life that we can, given all our limitations. I think that some misconceptions about hospices are historical. People have memories of ‘half-truths’ that live on with them, so then it can be like a mountain to climb for people to understand that things have really changed. I believe hospices play a very important role in helping people with life-limiting diseases and it’s very important to start the conversations early. I was first referred to connected palliative care when I was diagnosed with suspected cancer, and I have to admit I did panic initially, so I understand how some people feel. But then the palliative care nurses came out and saw me at home and explained it all properly. I had a hysterectomy, which was cancer-free, but I’ve been kept ‘on the books’ because of my brittle asthma and pulmonary hypertension. It’s very reassuring that the care is there for me if I need it. My faith has helped me deal with ill health in several ways. One of the strands is the people that you meet, and the friendships that are built up over the years. And I think also just

having God’s presence there at times has been the thing that has helped me move forward; knowing he is there in it with me. It’s hope, and hope in itself is a very important part of what my faith is about. Hope in the things that are seen, and hope in the things that aren’t seen. Don’t get me wrong, there are bad times, and like anyone I have my moments. Things can get overwhelming, and faith doesn’t mean I am ‘shielded’ from any of those things, but it does help me to get back on my feet again. I get a lot of support as a priest and I’m lucky in that respect. I have a Spiritual Director and I belong to a community where I get support from a Religious Sister, so I have that network set up. My health conditions have affected my ability to work at times. I’ve spent a lot of time in hospital, for either admissions or appointments, but colleagues are very kind in terms of stepping up and helping. My work as a priest is a big part of my identity and it’s difficult knowing that I will eventually have to retire on health grounds. That will be a big adjustment and I’m trying to prepare myself for that. My parishioners know I have health issues, but they don’t know all the ins and outs. Some of them are really good about it, and some are not so good. That’s the way life is; either people ‘get’ and understand your health, or they don’t.

Mother Jane has also kindly contributed some words to our new palliative care publication. For full details, please see page 16. SUMMER 2022 emphasis 53

a n o g n i o g l l a e r ’ e W

R E M SUM ! Y A D I L O H ncy foreign fa a or e m o h t a s day Whether it’s a few ld le of years we cou p u o c st la e th r te vacation, af ed our members sk a e W k. a re b a all do with r rite pics from thei u vo fa d n a s p ti p for their to lidaying with PH … o h f o s e c n ie er p ex hing in everyt “Bo o k u can e, s o y o advanc re are f lights he ask if t etc. This s ir a t l i sh , of s s in Daw re a w o t ph o . Th e in 2017 Devon eral nice ev were s nts in the ra u a t ps” re s out ste h t i w a a re rs

ete Carol P

“Tak e y o u r y o u r t i me body an d l ,b is you c an do ut also if ten to you f Th e p it, do ee n h son o oto is wit ’t be scar l li ke h my ed! n h ol i da you Lisa M arsh y in Norfol ngest k”

“Plan ahead and try to remain ca lm”

Daniel Redm ond

“If you’re st aying in a ho tel, make sure be forehand eit h er that there is a lift or that th ey can accomm odate you on the ground floor ”

Patricia Har rie

“I have flown to New Zealand with oxygen and provided you plan early, nothing is impossible. The photo is of Hobbiton there and it was taken just before lockdown”

Kathy Davis

SUMMER 2022 emphasis 54

“My top tip for a ni ce holiday is not to tick boxes. It does n’t matter if you on ly visit one place in a day, as long as you ta ke your time an d enjoy yourself. If you get too tired , stop and ta ke a re st. This photo is my hu sband and myself in Dublin in 2016”

Linda Donn

pre pa re d le but be ib s s o p if e of in advanc e photo is "Plan well t short notice. Th ic r t c an ele ea ee p for chang in Kent – I needed w n t h e st o d e s m t ir o a t g Broads hough it up!” ir and alt re k b a c k wheelcha ach it was a slow t be hill to the

a Phill Rich

rd s

“Be organised with medication and try to go somewhere that is fla t for walking. We love our holidays abroad and we have a favourite place in Corfu. The photo is fr om Sidari in Corfu in 2019”

h a ve u re y o u “Ma ke s ion medicat all your gen, if and oxy . in place needed, relax, stay at After th d enjoy an positive e can liday. W your ho t e a gr e a still hav e long as w s” time as ie d our bo listen to

a Roy Dew

“Ma ke s u re y o u are f it t o travel a n d ge t advice f ro m y o ur PH team”

J oy c e H

Lou Chadburn

cations to look for lo “Always try places k but also pic that you li ke s so e h nty of benc hile that have ple w own and rest hoto you can sit d p e h T ur holiday. enjoying yo e st ie il 2022 in Tr is from Apr go into 't n a c u o y ril (Italy). In Ap re too e ause ther a the water bec .but they are fun sh.. many jellyfi to watch”

ession

ack. They “Invest in a fold-up backp for day trips, are lightweight and great sandwiches, short breaks, shopping, cs and water bott les, glasses, ma in your fit n't wo t tha all that stuff m The Algarve pockets. The photo is fro d with PH the June 2015 : I was diagnose six-week trip week before we left on a dog Roxy and in our van with our litt le es” we had the time of our liv

Janice Lyons

10 Sarah Sebti, her brother) h it w d e r (pictu

“If you are flying, pack all you r meds, including IV pump stuff, in your hand luggage. You are allowed a bigger hand luggage allowance when fly ing to carry your meds. Be prepared for security to dissect all your meds, esp ecially IV meds, and chuck it all out of boxes when it’s taken you hours to pack. This is their job so try not to get stressed about it!”

Andrea Bown

oo d e a l ly g “Get r surance in om travel tter fr t e l a d an an on s u l t your c ur yo a bout n an d io t i c on d a t i on. c me d i joy!” An d e n

tha Saman gham n Cu n n i SUMMER 2022 emphasis 55

GETTING AWAY...

r o f g n i Plann l u f s s a succe

R E M M SU BREAK s go in y a w l a oesn’t s d g n i l l and it’ – n o Trave i t erail t direc d h g n i a r c e t th PH tha s y a w l e Royle i l u J n ot a r e M em b o l i da y ! h s t g n n e i c th ow a re which h , l i s a n i t e a d e xp l n to the d e n n u t wh e a l o p e s s o a l w didn’t e h s t n me a d play. e p p o t covid s

wanted to tell everyone about my partner Kevin and I’s recent experience of attempting to travel abroad and getting covid. Earlier this year, after more than two years, we finally decided we should get back out there and start living again, instead of existing at home. We're in our sixties and seventies and it matters that we still have time to travel as we've always done. When the first lockdown came, I didn't renew my travel insurance, as we had no need for it. Little did I know it would be two years later when we could even

SUMMER 2022 emphasis 56

consider going abroad again. But with three vaccines, we decided to take the plunge. For anyone who knows me, I am very organised and thorough when I do anything. So, as soon as we agreed a date to visit France for a week, I started to plan our break:

Travel, hotels and insurance

We booked our ferry with Brittany Ferries because we always travel by car rather than flying. We find having our own car with everything we need with us

a relaxing way to travel; we go at our own pace and enjoy everything. We arranged three different B&Bs which is part of our travelling so we get to explore more places. One of my first tasks was to check with the ferry company about their covid rules, and our rights if we had to cancel. For the B&Bs we used reputable sites, booking.com and airbnb.co.uk, as they have free cancellation policies up to 24 hours beforehand with full refunds. For my travel insurance, I went back to Staysure as they've always covered me for annual multi-trips since my

diagnosis of IPAH. I spoke to a rep rather than doing it online as I wanted to ensure we had maximum covid cover for all eventualities, either at home or abroad. They fulfilled my needs, and my cover didn’t cost much more than it did two years ago.

Driving plans

Next was the car. I have a Motability car which has had the lease extended to five years from three. So, I contacted them and was told to check my MOT and service if not already done. I asked them what I needed to drive to France post-Brexit, as it's all changed since our last visit. We needed a UK sticker not a GB one, and a current breathalyser kit for the glovebox. We also needed a breakdown kit, high vis jackets, triangle etc, which I bought at Halfords. As I was driving the Motability car, we needed to have a form called VE103 to prove I was able to drive it abroad.

I normally take enough meds for the period we are away plus a week either side in case of delays etc. I packed all our meds in my insulated picnic bag I use to keep the meds at an even temperature and all together. Lastly, we updated our domestic and travel covid passes on our phones and put them into our wallets. We also printed off hard copies of all our documentation, including our covid passes in case of WiFi failure. We changed our currency and so all was set.

AN UNWELCOME SURPRISE ...

The week before our holiday, we started to do daily testing as we had to be covid-free within 48 hours of travelling on the ferry. Plus, we had included a short trip to stay with our family friend for a couple of days before sailing on the Sunday. We tested Thursday morning and were both negative, so we drove to Portsmouth. By the time we had arrived at our friend’s house, Kevin felt poorly with fever, aches, coughing and sneezing, so he went to bed. On Friday morning, we tested again and he was positive. I tested positive by the Sunday, so our trip had to be cancelled. Can you imagine, after all that time in preparation?! My friend suggested we stay a few days until we felt better as it was too far for me to drive initially, even though I was still negative. So, we stayed and rested as we were both pretty poorly.

Tests, medication, and covid passes

I collected extra lateral flow test kits, so we could test whilst away as it's important to be negative to travel on the ferry both ways. I contacted my drug company, Sciensus, to ensure I'd have enough medication for before, during, and immediately after our break. They arranged my delivery before we were due to leave.

CANCELLING OUR PLANS

The morning Kevin tested positive, I started the process of cancelling the trip. I called the ferry company first, which was a good idea because if I'd have cancelled online, we'd have lost our money. Instead, the rep I spoke to arranged a new date in the future which we can change to suit, and we didn't lose the booking. So, it’s a good tip to call and wait in a queue as it's worth it. I went online for the B&Bs and was able

sidering If you're con lease travelling, p ects of check all asp don't your trip and shortcuts take risks or ostly. as it can be c

to cancel with a full refund as it was more than 24 hours before we were due to stay. If we hadn't have chosen these methods, we could have used the travel insurance, who wanted proof of positive PCR tests, which we did locally for free. But we didn't need them as our companies had reimbursed us, so all was good. When we felt well enough, a few days later, we travelled home without stopping as we were still positive and remained so for ten days each. It was difficult driving all the way up to Manchester with no stops, but luckily we timed it well and got home safe.

WHAT WE’VE LEARNED

I wanted to share our experience with you all with PH as we won't hesitate to try to travel abroad again soon. We've looked at our lives and ages, and our breaks matter to us when we can afford them. My insurance covered all eventualities of covid, whether home or away, so there was reassurance at all times that we were okay. As I had prepared and checked everything the whole experience has been positive for us. If you're considering travelling, please check all aspects of your trip and don't take risks or shortcuts as it can be costly. Kevin and I have only lost our holiday time, but no money, and we will attempt it again as we have peace of mind with planning. I do hope you enjoyed reading about our experience. Good luck and happy holidays!

SUMMER 2022 emphasis 57

Behind the

job title

Amanuel Teklesenbet did not take a well-trodden path into NHS nursing. Arriving in the UK from Africa as a 22-year-old refugee, he didn’t speak a word of English and dedicated his life to learning the language, gaining GCSEs and training as a nurse. Proving that anything is possible, he now works as a Clinical Nurse Specialist at the Sheffield Pulmonary Vascular Disease Unit. Amanuel told us his extraordinary story of overcoming adversity and finding a career in pulmonary hypertension that he loves.

“I will tell my daughter that her daddy has been through a lot”

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l e u n a Am lesenbet Tek Can you tell us a bit about coming to England?

There are lots of reasons that I wanted to leave Eritrea, mainly political and religious persecution. I was fearful for my safety. I arrived in the UK on 30th October 2008 with a friend after a long journey which took a year from leaving my home. First, we travelled to Sudan, a neighbouring country of Eritrea, where we stayed for nine months. Then we had to travel across the Sahara Desert, with 24 of us packed in a little truck for nine days. We then crossed Italy and France before arriving in the UK. The journey was difficult. We heard of people being locked in refrigerators in lorries, and people dying. We arrived in Birmingham and went into police custody before going to a detention centre for processing near London, where I stayed for a couple of months. I was then moved to a centre in Barnsley, and then Sheffield, which is where I have stayed ever since.

What led you to nursing once you’d arrived?

In Eritrea I really wanted to become a footballer, but at the age of 18 I had to

go into military service and become a first-aider. When I arrived in the UK, communicating was my first priority as I didn’t speak any English. In September 2009 I started college and explained about my background in first aid and said I’d like to be a nurse. As a Christian I believe in helping people, that’s a very simple thing for me, so it felt right. I knew I wanted to be a nurse, but my English was rock bottom, so I worked very hard on it. I spent three years learning the language and working hard on my reading and writing, which I am very proud of. I got my GCSEs in English, maths and science and was able to complete my nurse training at university.

How did you end up caring for people with PH?

After qualifying I started work at Sheffield’s Royal Hallamshire Hospital in the Ear, Nose and Throat (ENT) department, and was seconded to the city’s other hospital, the Northern General, to ease winter pressures. This is where I met a nurse that had also been seconded, from the Sheffield Pulmonary

Vascular Disease Unit (SPVDU) at the Royal Hallamshire Hospital. He told me about the unit’s work with people with pulmonary hypertension and it felt like somewhere I wanted to be. I knew nothing about PH at that point, but I completed extra training, including a medical education programme run by the PHA UK, and I joined the SPVDU as a Staff Nurse in May 2017. I remember when it was first suggested to me that I should then apply for the role of Clinical Nurse Specialist in PH. I honestly didn’t think I’d be able to do it, because English is my second language, so I decided I’d wait a few more years. But then I changed my mind and I thought ‘why shouldn’t I apply’? I’m so proud that I did it. In my previous role in ENT we’d treat patients and then they would leave. But I’m able to develop relationships with PH patients; they come back to you, and they know you. You get to know them, and get to know their family members, and build up that relationship and trust. It’s very rewarding. During the early stages of the pandemic, I was moved to work on the covid ward and that was another challenge. As nurses we were working through the pandemic and then going home to live through the pandemic. You couldn’t get away from it. I had a newborn baby, and it was all very worrying, but I’m proud of the part I played in helping patients during this critical time. Continued overleaf >>>

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“I hope my story helps change some misconceptions about refugees” Have you felt welcomed by the UK?

Amanuel with SPVDU colleagues during the Sheffield Half Marathon

People in the UK, and in Sheffield particularly, have been so welcoming to me. I did worry about whether they would be. But I have lived here nearly 13 years and I can honestly tell you that I have never met anyone who has been unwelcoming or racist. I can gladly say that Sheffield people are the nicest! Patients ask me about my history and my heritage, and I am very happy to tell them my story. Many of them are surprised to hear I came over as a refugee. It’s nice to hear them say ‘well done’ and I hope my story helps change some misconceptions about refugees.

How does the UK healthcare system compare to healthcare in Eritrea?

In my country we are so poor. If you need a big operation, you must go abroad, and people ask for help on social media to raise the money. I know of someone from Eritrea who came over to the UK and she wasn’t accepted as a refugee, but she had serious heart problems. She needed an urgent operation and she had only been here a few weeks, but the NHS still saved her life. Everyone knows how wonderful the NHS is. It is the best in the world.

What do you like to do away from work?

I enjoy running, and earlier this year I was part of an SPVDU team that

“I’ve had the pleasure of working with Amanuel since he joined the team in Sheffield in 2017. He has worked incredibly hard from arriving on these shores with nothing, and his tale is a humbling reminder that overseas nurses are a crucial part of our healthcare system. The word ‘refugee’ can conjure negative connotations and Amanuel

SUMMER 2022 emphasis 60

completed the Sheffield Half Marathon for the PHA UK. I also enjoy football and I go to Sheffield United matches sometimes with colleagues from the SPVDU. I play with an Eritrean community team, and I really enjoy cooking – especially breakfasts. I also enjoy spending time with my family. I met my wife in London, and we married in 2019, and our daughter Peniel was born in 2020. My brother lives in London but my parents are back in Eritrea. I try and go back there every three or four years, but it can be difficult. You can see people struggling, and it brings back memories.

How do you feel when you look back on your journey?

I’m so proud of what I’ve done since coming to the UK with nothing. I had no English and no experience, but to come out of my comfort zone and learn and speak and express, and develop my communication, is something I am very proud of. Besides all of this, my proudest moment is my daughter being born. I’m absolutely looking forward to telling her my story. I will tell her she is here in England because her daddy had to come over all this way, and there are hundreds of stories I can tell her about my journey. I will tell my daughter that her daddy has been through a lot, but anything is possible, and she can do anything she wants to – even if that’s working for NASA!

is proof that we need to open our minds and look beyond stereotypes. He is an asset to our hospital, to the PH community, and to the NHS as a whole. I hope you find his story as inspiring as I do.” Dr Iain Armstrong, Chair, PHA UK, and Nurse Consultant, Sheffield Pulmonary Vascular Disease Unit

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✓ Blogs and videos

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

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’PHA UK Official Carers Group’ For family members, friends and loved ones ‘PHighting on: Life after loss to PH’ For those who have lost someone You’ll find direct links to these forums at www.phauk.org

MEMBER

BLOG

tI 's a life –long condition, but it's not I always life –limiting

Pulmonary hypertension affects everyone differently, and PHA UK member Sarah Pyett is keen to show that the condition can sometimes have positive outcomes. She wrote this blog for our sister website, www.phocusonlifestyle.org n July of 2013 I was in London to run the British 10k. In September of the same year, I returned to the city to attend a friend's wedding. I clearly remember getting off the tube at Tower Hill station, walking up the steps to the street with my suitcase, and feeling a pressure in my chest when I reached the top. The feeling passed within a few seconds, I shrugged it off and carried on enjoying my weekend of celebrations. Over the next month or so, I experienced that feeling of pressure more and more often. I’d get it when I walked up the stairs at home, when I got out of the car to walk into the supermarket, and any time I went from a relatively relaxed state to exerting myself in the slightest.

SUMMER 2022 emphasis 62

If you would like to write a blog for our lifestyle website, please email media@phauk.org

One evening at home, I walked upstairs and sat down at the desk in my study. I remember feeling the pressure in my chest, followed by dizziness, and I blacked out in the chair. I knew it was time to get seen by a doctor, so I took myself to A&E the next day. After X-rays, blood tests and a CT scan, they sent me home with heparin injections after suspecting a blood clot on my lung. When the symptoms didn’t disappear, I went to my GP who referred me to a cardiology team, and over the course of the next few months, I had multiple heart monitors, alongside more scans and blood tests. When the teams at my local hospital had exhausted every avenue, I was referred to the cardiology team at Royal Papworth Hospital in Cambridge. During the time between referrals, I continued to experience chest pressure, and I passed out a few more times after climbing the stairs at home. These were my only real symptoms; I didn’t experience any of the other common symptoms such as breathlessness, fatigue or ankle swelling.

Finally getting answers

A few months later, I went to Papworth for a procedure to test the electrical pathways in my heart, and it was during this investigation that they discovered my arterial pressures were much higher than normal. I got referred to yet another team, but this time a team that I would come to know very well. Over the course of two days in December 2014, I attended the Pulmonary Vascular Disease Unit at Papworth, and underwent blood tests, a 6-minute walk test, pulmonary function tests, X-rays, an ECG, echocardiogram, and a right heart catheterisation. From the date I first took myself to A&E, it was 13 months until I finally got

my diagnosis, Idiopathic Pulmonary Arterial Hypertension, the kind that has no underlying cause. Whilst my lung function was good, the right side of my heart was enlarged and the walls were stretched thin. Fortunately, during my right heart catheterisation the team had tested my response to nitric oxide, and I was deemed ‘vasoreactive’. This meant that I was suitable for a type of medication called a vasodilator, which the doctors described to me as being like the very first rung on the long ladder of treatment options. They assured me this was a positive thing, despite the diagnosis I had just been handed. The pharmacist prescribed me nifedipine, and since I took that first tablet while still on the hospital ward in 2014, I have experienced absolutely no PH symptoms whatsoever.

for a healthy heart. Plus, there is always fake tan! I’ve been able to continue to work full time, which is great. My two-day stay at Papworth actually came just one month into a new job, and my employer couldn’t have been any more supportive. I was office-based prior to the pandemic, but am now based at home, with the occasional visit into the office. My partner, Fiona, lives in beautiful North Wales. We met at a concert in 2014, before I was diagnosed, so she has known about my condition since day one. As a couple, we love travelling, anything to do with animals, and we

Enjoying life with PH

Despite the doctors telling me not to Google the condition, that’s the way of the world these days, and I did research it when I was initially diagnosed. The life expectancy data that you see is frightening, and it did consume me for the first couple of months until I realised just how historical it was, and how many more successful treatments exist today. When I went back to Papworth for my check-up six months after diagnosis, my test results showed that my arterial pressures were much lower, my lung function was normal, and my heart had returned to the size and shape that it should be. This has continued to be the case at every check-up since. I am now seven years post-diagnosis, and doing really, really well. I even had extra staff wanting to witness my exercise test a couple of years ago, as they couldn’t believe how well I am. I am very active day-to-day, I swim at least 1km most evenings, I try to get out walking as much as possible, and I’m a keen golfer. I’m not allowed to lift anything heavy due to the strain it could put on my heart, but aside from that I have no real restrictions in my day-to-day life. The only side effect I get from my medication is slight swelling/discolouration on my ankles and lower legs. It did bother me for a while initially, but I’ve learned to live with it now and it’s such a small price to pay

Join the PHA UK Facebook group @PULHAUK

absolutely love going to concerts. My PH doesn’t hinder me when it comes to any of this, the only additional thing I have to do is declare my condition on my travel insurance to ensure I am covered abroad. For anyone newly diagnosed, I would say to try to stay positive (as hard as it can be sometimes), and keep in mind that there are many, many different treatment options available to you. For so many, PH isn’t the sentence that it once was. Remember that Google is not your friend, but the PHA UK Facebook group is. It’s full of other people going through the same as you, people who can give you advice, support, and just be there if you’re having a bad day and need to let off some steam. I know I am a rare case, and I am incredibly lucky that my life with PH isn’t much different to life before, but I wanted to write this blog to show that PH can sometimes have positive outcomes. It’s a life-long condition, but it’s not always life-limiting.

SUMMER 2022 emphasis 63

g n i s i a r d n Fu

Your suppo rt over the last few months has been am azing ! This is just a small selection o f what you’ve been up to for our cha rity…

S U C O F

Celebrating in style These supporters turned special occasions into fundraising opportunities… Suzanne Darbyshire (right) marked her 60th birthday with a luncheon at a local hotel, and a raffle and donations raised £3,635! Suzanne and her husband also gave out leaflets about PH to help raise awareness too. Caroline Artt (below) celebrated her 50th birthday with a party that raised over £1300 thanks to the kindness of her family and friends.

Suzanne and Tony Darbyshire

TS ! A R G N CO

Big thanks also go to Julie Royle, who asked for donations instead of presents for her big day earlier this year.

Suzanne's luncheon

Caroline with some of her birthday cards

Enjoying her 50th birthday party

WINTER 2019 SUMMER 2022emphasis emphasis2264

Something a little bit different... When a Virgin Hot Air balloon landed in a farmer’s field in North Yorkshire, it sparked a generous donation to our charity. Pilot David Sutcliffe set down on land belonging to Tom Coast-Brown, and as is customary, offered a gift as a thank you. Because Tom has family connections to PH, he asked for a donation to be made to our charity instead. Pictured: David’s balloon

Pushing through the pain Daring duo Patricia O’Donnell and Gary Peart took on a 10-hour endurance run that raised £1,148 in support of their niece Alex Charlesworth. During the ‘Fortitude Ultra’, the pair had to start a three, four or five mile loop every hour for ten hours – covering well over 60 miles between them. Despite having run marathons in the past, Patricia told us the event was much harder than expected. She said: “My husband is always signing up for horrendous physical challenges and talked me into joining him on this one! The hills were brutal, and I was totally out of my comfort zone. By the eighth loop, eight hours in, it was just pure pain. Gary and I were thrilled when our fundraising went over £1000, and if there is ever a breakthrough to make Alex’s life easier then it will be worth all the miles we can ever cover.”

GREARTT! EFFO

Cycling for strength and support

GOOD! LUCK

PHA UK member Jo Dunbar has set herself a virtual cycling target to clock up donations as well as miles. Jo, who is recovering from pulmonary endarterectomy surgery, is using an e-bike to cycle the equivalent distance between the hospitals that treat her in Wrexham, Liverpool, Sheffield and Cambridge. She aims to complete the 450-mile round trip total by the end of the year and will support both the PHA UK and a hospital charity with her fundraising. Jo said: “As I'm recovering from PTE surgery, consistent appropriate exercise is important, and I do that best with a challenge. I'm splitting sponsorship between Liverpool University Hospitals Charity because of the excellent care I receive at the Roald Dahl Centre, and Pulmonary Hypertension Association UK because of all they do on behalf of patients with PH and the incredible resource they have been since my diagnosis with CTEPH.” You can sponsor Jo at justgiving.com/fundraising/cycleforpha

me fundraising so o d to e d ci e d w if you s on 01709 761450 Please let us kno u ll ca , rg .o k u a h ffice @p for us :-) Email o or Instagram book, Twitter or tag us on Face

SUMMER 2022 emphasis 65

Could anticancer drugs be used to treat pulmonary arterial hypertension Researchers hope to access funding for a new study that will investigate whether a therapy to treat cancer could be repurposed for use in PAH. Here, we tell you more about what the team from University College London are trying to find out.

ulmonary hypertension (PH) is caused by the small lung arteries becoming thicker, making it harder for blood to pass through these narrow channels. A specific type of pulmonary hypertension in which the disease process occurs in the pulmonary arteries is pulmonary arterial hypertension (PAH). The condition causes the heart to struggle to pump blood through the lungs and causes high blood pressure in the lung arteries. Currently, treatments for PH are based on drugs that relax the artery muscles to make it easier for blood to pass through. However, this doesn't address the thickening of the blood vessels – so up until now, conventional therapies have had limited impact on prolonging survival.

SUMMER 2022 emphasis 66

The potential to repurpose well-known medication

Blood vessel dilators, known as ‘vasodilators’ are medications that open (dilate) blood vessels. There are several new treatments being developed and tested in combination with vasodilators to improve the disease process. Some of these drugs are approved and being used to treat other diseases such as cancer. However, anticancer drugs cannot differentiate healthy cells from diseased cells, which leads to severe side effects for the patient. This has therefore limited their use and the problem remains a challenge for doctors and researchers. A strategy is needed to tailor the delivery of a drug to exactly where the disease is in the lung blood vessels, to avoid the targeting of healthy cells that causes these side

(LEFT) DR MARYAM PARHIZKAR

Lecturer in Pharmaceutical Engineering at University College London

(RIGHT) DR BENJI SCHREIBER

Consultant Rheumatologist, Royal Free Hospital

effects. And as part of this, the ability to monitor the progress of the treatment in a non-invasive way is really important. If clinicians can identify the drug is reaching the target area and evaluate this through medical imaging (for example medical resonance imaging, known as MRI scanning), they would be able to predict the response of specific tissue. This would then allow them to select the right amount of drug to be used, that would treat the disease effectively without the unwanted side effects.

Exploring a solution

Researchers believe a solution to this clinical problem is to package anticancer drugs into tiny nano-sized particles, which will help concentrate the treatment on the diseased tissue and decrease the side effects. The study sets out to develop drug delivery strategies based on small particles (nanoparticles) that can be injected into patients. These nanoparticles will specifically concentrate in the lung arteries through a targeting molecule, that will be incorporated on the surface of the particles. The strategies will also use medical imaging to improve and guide the treatment. This means that nanoparticles will also contain a radioactive molecule that allows them to be seen by special imaging methods – specifically a positron emission tomography (PET) scan. This will allow doctors to evaluate how effective different doses of the drug are, so the right amount for each individual can be found. This is what’s known as ‘personalised treatment’. The study sets out to address the challenges in treatment of PH by introducing alternative therapy, guided by imaging, through targeted delivery specific to the lung arteries. The targeting is non-invasive and medical imaging will ensure that the therapy is delivered to the thickened blood vessels only, and not to other areas of the body. It normally takes around 10-15 years from the point of research for a concept like this to be used routinely for patients. However, because PH is a rare disease and researchers are seeking to repurpose drugs that already have regulatory approval, the team are hopeful that the process can be accelerated. The study is due to begin in October this year and a funding decision from UK Research and Innovation will be made before then. Keep an eye on our social media channels for updates.

The team of engineers, physicists and clinicians seeking funding for the study include Dr Maryam Parhizkar and Dr Benji Schreiber. They told us: “The outcomes of this study will have a substantial impact on our understanding of how this new nano-size drug delivery system can be further translated into the clinic, and how patients can benefit from more personalised treatment options. We are very excited about this, as we are experts from different disciplines working together to develop a highly specific therapy for PH. We believe this locally-delivered therapy for pulmonary hypertension would be a real advance for patients and will certainly be a great step towards finding a cure for this disease.”

Research studies into treatments for all types of PH are ongoing, and at the PHA UK we support these projects however we can. Although we cannot report on every piece of research, we believe it’s important to keep our readers informed of potential new treatments where possible – and our goal is that one day, we can bring you news of a cure.

SUMMER 2022 emphasis 67

Wondering what financial support you might be entitled to?

Benefits Calculator and Grants Search tools now available at www.phauk.org Find out if you could access welfare benefits, charitable grants and other support that you might be missing out on. In association with Turn2Us www.turn2us.org.uk

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SUMMER 2022 emphasis 69

mily lumnist E d o c w e r C af has ha Green Le and she w ension ld o s r a e is 11 y l hypert ry arteria 3. Here, she a n o lm u p s e ce she wa (PAH) sin about the tests sh ll tells us a t her clinic visits… sa undergoe

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spital visits over I have been to many ho Basingstoke, the years, with trips to t Ormond Street Southampton and Grea gstoke is the (GOSH) hospitals. Basin ill quite a drive. closest to us, but it is st doctors doing my Whilst I have different same people on the tests, I usually see the nic, at GOSH and in PH team at every PH cli Southampton. I’ve known the GOSH I quite enjoy PH clinics. now and so I know PH team for many years SH is quite a nice them really well. Also, GO s of things to see place to visit. There are lot ing any medical tests. and do when I am not do

s There are a variety of test that I do as part of my clude: hospital visits, these in ram and it

ardiog ECG ECG stands for electroc This is easy and just

m. records the heart’s rhyth my chest and feet. on involves some stickers ing, except for peeling I don’t have to do anyth the stickers off. rdiogram and it Echo Echo stands for echoancad nearby blood art is used to look at the he . an. It doesn’t hurt at all sc of d kin a vessels. It is , I often watch TV while I just have to be patient this is being done. test I literally just 6 Minute Walk For this, us ually while wearing have to walk for 6 minutes ine on my finger. a little oxygen SATs mach

SUMMER 2022 emphasis 70

Emily er doing h st walk te

When I was squeezy thing! little I called this the arm

Blood pressure

c Resonance MRI stands for Magneti uses a magnetic field Imaging. It is a test that microwaves that take and computer-generated in part of your body detailed images of a certa ile I go in the MRI (for PH it is the heart). Wh tch my choice of DVD. scanner at GOSH I can wa but I don’t mind it You have to lay very still, od tests at a few Blood tests I only havegoblo , y well but sometimes of the visits. They mostl ey can be a little bit if my veins don’t show th ays manage to get tricky, but the nurses alw me numbing cream so blood. I always have so es in I can hardly feel it. that when the needle go

MRI

s the consultant lets At the end of the PH clinic me (my Mum or Dad) me and whoever is with am doing. Occasionally know how they think I se of one of my they might change the do out adding a new medicines or even talk ab any questions I have. medicine. I can ask them

And

finally...

Have you heard the

PHA UK podcast?

EmPHAsis on Air is hosted by PHA UK team members Shaun, John and Mary, with regular special guests. It brings the pages of this magazine to the airwaves with advice, interviews and analysis – and a fair few laughs too!

Listen now on Spotify, Apple Podcasts, Amazon Music, Audible and more. You’ll also find all our episodes on our website.

Tell us what you think of Emphasis...

SPRING 2022 emphasis 17

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This is your mag az want to make it ine, and we the best it can be . Is there somethin g see more (or less you’d like to ) of? Perhaps you have an idea for a fe to comment on so ature, or want mething you’ve read in this issu e? Your feedback is really import ant to us, so plea se share your thou ghts. You can email us at media @ phauk. org (pop ‘Emphasis fe ed subject line) or w back’ in the rite to us at : PHA UK

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SUMMER 2022 emphasis 71