Helping you plan for your 2024 break
The magazine for members of the PHA UK www.phauk.org
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As we step into the brighter months of spring, it’s my pleasure to bring you the first Emphasis edition of 2024.
Our recent feedback survey showed just how much you value this magazine, and I’d like to personally thank everyone who took the time to share their thoughts and suggestions. You can see what we’ve done with this feedback on page 10
Many of you told us how helpful you find reading about other people’s experiences of PH, and in this issue, you can hear from two PHA UK members ( pages 14 and 25 ) who are making the most of life. Your stories matter, and they really make a difference to others.
We started this year by taking the patient voice to a global research conference in London, bringing together scientists and academics working across many different countries to advance treatments for PH. Organised by the Pulmonary Vascular Research Institute, it was a heartening reminder of just how much work is taking place in this disease area – all over the world. Closer to home, you can read about a pioneering UK-based study into personalising PH medicine on page 20
Finally, I’d like to draw your attention to our report on page 42, which shows that standards of care in our specialist centres remain high – despite the pressures facing the NHS. As well as being Chair of the PHA UK, I am a Consultant Nurse within one of these centres, and I see first-hand every day just how hard my colleagues work for their patients. The findings of the National Audit of Pulmonary Hypertension, which measures standard of care, reflect this hard work. You should feel confident that you are in safe and committed hands with your PH team, whichever hospital you attend.
Enjoy the issue.
YOUR STORIES MATTER, AND THEY REALLY MAKE A DIFFERENCE TO OTHERS.
Iain Armstrong Chair of the PHA UK media@phauk.org
“One thing I’ve learnt on this journey is that the right person will come along”
Raya on relationships with PH, and the difference her girlfriend has made to her life
PHA UK Chair Dr Iain Armstrong has been shortlisted for two national awards in recognition of his commitment to nursing in pulmonary hypertension.
Iain, a Consultant Nurse within the Sheffield Pulmonary Vascular Disease Unit, is up for ‘Cardiovascular Nurse of the Year ’ and ‘Nurse of the Year ’ at the prestigious British Journal of Nursing (BJN) Awards.
The winners were due to be announced just two days after this magazine went to print – so take a look at our social media channels to see if he took home the gongs!
A new study has highlighted the benefits of EmPHasis-10, the quality of life measure developed by the PHA UK and used across the specialist centre network.
The questionnaire, used by doctors to understand the impact of PH on a patient, was compared against other disease-specific measures. The study concluded that EmPHasis-10 demonstrated ‘strong psychometric properties and superior feasibility for use in clinical practice due to its concise format’.
You can read more about the study, titled Clinical Utility of Patient-Reported Outcome Instruments in the Management of Pulmonary Hypertension: A Systematic Review, by visiting www.bit.ly/SystematicReview_E10
Recent design tweaks have been made to EmPHasis-10, making it easier to use and understand. The questions have not changed.
The national charity Carers UK has launched a new information phone service for those who prefer not to use the computer or internet.
Access information on caring by calling 0800 888 6999 and following the options provided. All calls are free.
Newly diagnosed with PH, my wife was given copies of PH and You and Medication and PH. I have naturally read them myself and am enormously impressed – not only by the detailed and helpful content but also by the thoughtful and skilful way in which it is presented.
I am an ancient and long-retired journalist and I was also a lecturer for many years, when much teaching time was spent getting young reporters to tackle often-complex information in ways that would enable readers to understand swiftly and accurately, preferably at one reading. I was reminded of these sessions two or three times (honestly!) as I read your booklets. The booklets are absolutely excellent. I’d certainly give them an ‘A’, or a ‘distinction’ even!
Thank you for the work you’re doing.
Gerry Kreibich www.teachingjournos.com
Order your free copies of PH & You and Medication & PH at www.bit.ly/PHAUK_Publications
February saw us take the patient voice to a very special global event held in London.
The annual Pulmonary Vascular Research Institute (PVRI) Congress celebrates innovation and progress in the field of pulmonary vascular research, and we were there to advocate for patients being central to all research – ensuring this type of illness is studied as a lived experience.
We presented the findings of our recent study into what it means to lives with PH today and made valuable connections with researchers from around the world. There is some outstanding work going on in this field, giving us all reason for hope.
We asked, you said…
Nic A wee afternoon nap always helps ��
Diane Listen to your body, pace yourself and if you need to rest do so. I try and have a ‘stay home day’ if I have been out and about a lot the previous day.
Candeece A nap throughout the day helps me ��
Jo Movement helps but it seems like it’s an impossible thing to get on top
HELPING YOU UNDERSTAND THE TESTS USED TO DIAGNOSE AND MONITOR PH
We asked our Facebook followers to share their tips on managing fatigue
of and really learn to manage. Every time I think I might have a routine and parameters sorted and it might be manageable there’s a plot twist!
Nicki Trying to limit myself to any activity and listening to my body and going for a sleep helps.
From walk tests to echocardiograms, and lung function tests to right heart catheters, you may experience a number of tests when you visit your specialist centre – used either to diagnose or monitor your PH. Our new series of short videos have been produced to help you understand what to expect from each.
Scan the code on the right to watch the full playlist or visit www.bit.ly/PH_tests
There was laughter alongside tears when we brought the patient perspective to a gathering of pharmaceutical company representatives in London in February.
The PHA UK's Iain and Mary were joined by experts by experience Julie and Kevin, PHA UK members who gave delegates an insight into what it really means to live with pulmonary hypertension.
The event was a powerful reminder of what really matters when it comes to drug development – the people who live with this disease every single day.
We're hugely grateful to Julie and Kevin for joining us for this valuable opportunity to make patient voices heard.
The production schedule for this magazine is changing this year, with issues now being published in April (this one!), August, and September. Each edition will continue to be packed with features, expert advice, real life stories and much more – keeping our PH community informed and connected.
Thank you to everyone who completed the feedback survey sent with the last issue of Emphasis.
It’s been wonderful to hear how much you value your member magazine, and how much we are doing right – and your suggestions for improvements were really helpful too.
We read and considered every comment and idea, and some changes have already been made to ensure Emphasis meets your needs and is the best it can be.
YOU TOLD US… YOU WANT MORE NEWS AND FEATURES ABOUT RESEARCH IN PH.
This issue includes three research features, putting the spotlight on studies around personalised treatments (page 20), walk tests (page 62) and genetics (page 64). We’ll continue to bring you research news in every edition.
YOU TOLD US…
YOU’D LIKE TO SEE THE RETURN OF A REGULAR SECTION FOR CHILDREN WITH PH
We’ve brought this section back in the form of Kids Corner – featuring a Q&A with a little phighter and a new young columnist. Turn to page 60 and enjoy!
YOU TOLD US…
YOU'D LIKE TO SEE THE MAGAZINE FEATURE MORE OF THE PEOPLE WHO WORK IN SPECIALIST CENTRES.
This issue brings you interviews with two healthcare professionals – a doctor from Sheffield’s Royal Hallamshire Hospital (page 24) and a pharmacist from the Golden Jubilee Hospital in Glasgow (page 50). We’ll continue to help you to get to know the people who work in PH through these pages.
YOU TOLD US…
YOU REALLY VALUE READING PERSONAL STORIES FROM OTHER PEOPLE AFFECTED BY PH.
We’ll continue to bring you these stories in every issue, covering a range of circumstances and experiences. This issue, hear from Mike Adams on page 14 and Kate Alsford on page 25. Personal experiences also form the basis of Raya’s column on page 36 and they are the inspiration for Sophie’s mental health column on page 18.
YOU TOLD US…
YOU ENJOY OUR NUTRITION FEATURES, BUT YOU’D LIKE TO SEE HEALTHY MEAL IDEAS AND RECIPE SUGGESTIONS TOO.
Our contributing dietician, Chermaine Kwant, will now explain how to make the meals she eats in a new regular feature. You’ll find the recipes for her protein banana pancakes and tuna patties with avocado smash on page 45.
We always welcome your feedback on this magazine. Please email media@phauk.org with any comments or suggestions that you have.
“Each time Emphasis arrives I realise I am not alone…The feeling of isolation disappears.”
“Thank you for producing a magazine that supports so many people to be able to raise their voices about life with PH”.
“It is such a good read and helps me enormously.”
“It is realistically informative but also upbeat.”
“It is presented in such a clever and eye-catching way and is easy to read.”
“When I read about people who have this condition - like me but of all ages - it gives me hope, courage, and determination.”
“Very well produced and a quality magazine in both content and appearance.”
“I look forward to every issue.”
“There is something for everyone in the magazine.”
“I use it as a reference tool for helping explain my condition to family and friends.”
“It’s extremely informative.”
“I expect [this magazine] is a lifeline for many.”
“It’s colourful, professional, and it gives me encouragement that there is support, care and community available to me as a PH patient.”
“The ability to see others in a similar situation makes you feel less alone.”
Nothing beats a summer jaunt, and planning ahead is the key to enjoying a relaxing break – whether it’s in the UK or further afield. Here are some important things to consider when preparing for a trip away…
This is important for peace of mind if you are holidaying abroad. Unfortunately, people with health conditions like PH have to pay more for travel insurance, so consider this when budgeting for your break.
The following insurance companies have been recommended to us by PHA UK members who have had good experiences with them when travelling abroad:
Avanti
0800 888 619 avantitravelinsurance.co.uk
Staysure
0800 033 4902 staysure.co.uk
Able2travel
0845 839 934 able2travel.com
Freedom Travel
0870 774 3760 freedominsure.co.uk
Always keep a list of your medication and important telephone numbers on you, in case of emergency, and make sure the details of your specialist centre are included.
Even if you’re not normally on oxygen, you may need it to fly, especially if it’s a long-haul flight. Your specialist centre will be able to advise on this too.
You may need to hire a concentrator, so this needs to be factored into your holiday costings. If you own a portable oxygen concentrator, check with the airline to ensure they will allow your individual concentrator on the flight. If you are planning to cruise, it’s also important to check with the operator that your oxygen needs can be accommodated.
Keep your medication in a safe in your hotel room.
When taking out your cover, you must declare that you have pulmonary hypertension, even if it’s very stable. And you must declare any other pre-existing conditions too. It can be tempting not to, in order to keep the costs down, but it’s simply not worth the gamble.
This is important even when you’re holidaying in the UK. Always take more than you think you will need, in case of delays. And if you’re flying, split your medication between your hand luggage and hold luggage – just in case.
If you’re taking drugs or medical equipment through airport security, make sure you have a letter from your doctor that explains why you need them.
Check that you’re able to travel by plane – and request a fitness to fly letter in good time
Before you book a flight, check with your specialist centre that you can travel on a plane. You may need a ‘fitness to fly’ letter, and this will need to be issued by your specialist centre within 14 days of your flight. But please get in touch with them as soon as you know you’ll be flying, ideally at least six weeks beforehand. This is really important as it gives consultants time to prepare the letter and send it out at the appropriate time.
VICKI I'm off to Thailand and Ibiza this year with my husband. I'm a massive foodie so for me it will be all about sampling the local food, a cocktail or two at sunset and hopefully glorious sunshine.
EMILIA I'm going home to Menorca in July to visit my family. Cannot wait.
TONI My husband is retiring. We are looking forward to exploring the UK in our caravan. Cannot wait to get away in our tourer, I'm so excited.
NICKI My husband and I are heading to Florida and Disneyworld for a fabulous vacation later this year.
SAMANTHA Corfu in June for a week and Amsterdam for a rock concert, can't wait.
JAYNE Cornwall in November and then already booked next years cruise! Shame the insurance is sky high but that’s for another day...
RAJDA I may go to Qatar to see my son, he’s getting married in July and I can’t wait!
TOP ConsiderTIP...booking through a travel agent if you want one point of contact for questions about your holiday.
LYNN Back to beautiful Majorca...my second home Cala Bona the very end of April...and then Palma Nova later in the year...both very flat resorts. Great short haul flight and travelling from my local small and very easy and disabled friendly airport too x
JOYCE Hopefully if all is well, beautiful Scotland.
MANDY Off to Kefalonia in September as big birthday this year.
JONATHAN Last year we went to Malta. This year is looking like being Suffolk!
MELANIE Daisy (5) is going on her first caravan holiday to Porthmadog in Wales! Lots of sand, swimming and dancing at the children’s disco with her little brother and cousin!
JANE I’ve decided to make the most of the time I have whilst I am physically able so in May, a week in SW France; in June, 2 weeks plus in the Loire; in July, a week in The Lake District and Yorkshire. I have oxygen and will travel!
TOP TIP...
Take two copies of your most recent PH clinic letter with you on your travels.
With liver cirrhosis and pulmonary hypertension, Mike Adams has been through a difficult journey with his health. But despite the challenges, and a lot of unknowns, the 63-year-old is determined to stay positive. This is his story.
I've had
a problem with my liver for 23 years and four years ago, I went for a transplant assessment. You’ve got to be well enough to be able to get through the operation, but you also have to be sick enough to have the operation, so the decision was made to wait – and three years went by before everything went pear-shaped and I became very ill again.
Around then, doctors noticed that I had a problem with my heart and lungs– even though I had been telling them for over a year that I had been finding it really tough to breathe.
Eventually I spoke to one of the top consultants at the hospital and he said, ‘I’m sorry Mike, but you are now amongst the three per cent of people with liver problems who get problems with their lungs and heart as well’.
I couldn’t believe it. I’d spent all those years trying to get myself together with my liver, but now that
went on the backburner, and it was all about the pulmonary hypertension. But the medication is helping, and my pulmonary artery pressures have come down, so it looks like I’ll be put on the list for a liver transplant soon. It’s been a long time waiting, and a real journey.
I’ve had to be positive right from the start. When I was first hospitalised with my liver, I said to the doctor, ‘I’m not ready to die, so whatever you throw at me, I’ll do it.’
That’s the way I’ve got through life; being positive and making changes. I gave up smoking and drinking, and although there’s still a chance I won’t get a transplant, I’m not looking at it like that.
doomed’ because you’re not going to be able to do anything mentally if you’ve got that attitude.
The people around me help me to stay positive, especially my wife Sue, who has been absolutely fantastic. I’m not allowed to drive anymore, because some of my liver medication affects my brain, so she takes me everywhere, and she understands everything. She’s part of all my medical appointments because she can take it all in. She’s always been there for me.
When the PH was discovered a few years ago, a lot of people around me got very upset but I said, ‘I’m alright, I’m alive and they’re going to keep me alive. They’re doing their best to get me the right drugs and get me through all this and now it’s down to me’. I am a firm believer in not sitting on the sofa and thinking ‘my life is
I am a firm believer in not sitting on the sofa and thinking ‘my life is doomed’
I think it’s really important to have someone there to help you and to give you the odd nag, for example to remember to take your drugs. Sue and I ran a restaurant together until the PH made it too difficult, so now we have a part time gardening business together. We do a maximum of four hours a day, three days a week, and it works. It keeps me active, which helps me physically and mentally.
I’m feeling good about 2024. The idea of a liver transplant is scary as it’s major surgery, but I’ve been told that my recovery should be good because of the way I approach life. I’m feeling very positive about it all and I’m grateful for what I can do. I can work, I can get up and down the stairs, and I can go out for walks. At one point, I couldn’t walk 20 paces without having to rest for 15 seconds. My message to someone else struggling with their health is to accept help. There are always people out there who will help you, but you have to let them. Listen to your doctors, take the medication they prescribe, behave, and keep your head positive. You’ve got to get on with life and do what you can.
As the cost of living continues to rise, find out what financial support you may be entitled to.
We’ve teamed up with a group of specialist doctors to develop new information resources to help people with PH associated with congenital heart disease (CHD).
Some people born with heart problems (known as congenital heart disease) go on to develop PH, and at present, there are limited information resources available that are specifically aimed at these individuals.
At the end of 2023, we conducted a survey amongst both patients and caregivers to identify knowledge gaps and understand what is needed from new resources.
The questionnaire was promoted both online and within treatment centres, and it was completed by 177 patients and 33 caregivers.
Lots of helpful suggestions were given for the topics to be covered by new resources, and we are hugely grateful to everyone who took the time to complete this important survey.
You can see more of the findings at www.bit.ly/PHandCHDinfo
Key findings showed:
> Only 24 % of patients and 8% of caregivers were given specific educational material on PH and associated CHD, at the time of diagnosis.
> 88% of patients and 92 % of caregivers agree there is a need for new information resources about PH associated with CHD.
> 78 % of patients and 79 % of caregivers view CHD and PH as a combined, rather than separate, issue.
Our findings show there is a clear unmet need for information that focuses on the combined issues of PH and CHD. We’ll be gathering more insight to help us shape these resources by holding an online focus group with patients and caregivers, ensuring the materials we produce fully meet the needs of those affected by these conditions.
We’ll keep you updated on progress!
This work is being carried out by the PHA UK in collaboration with the CHAMPION group, which stands for Congenital Heart disease And pulMonary arterial hy Pertension: Improving Outcomes through education and research Networks. The group is made up of clinicians specialising in the management of pulmonary hypertension (PH) and of congenital heart disease (CHD) from a number of specialist centres in the UK. As the name suggests, their aim is to help improve the management of patients with PH due to CHD through research and education.
Psychotherapist
Sophie Papageorgis lives with pulmonary hypertension and is passionate about helping others with the condition. In her second column for Emphasis, she explains how being ‘present’ can make a difference to your mental health.
Understandably, our past experiences form a huge part of who we are today.
And many of us (with or without pulmonary hypertension) have probably had some frightening experiences that at times make us question ourselves, others, and the things that have happened to us.
As humans, we naturally worry about what could happen and what could go wrong, doing a lot of stressing and wondering ‘what if?’ Life events can feel overwhelming at times, and it makes sense that we would be concerned or worry about them.
But what happens when we find ourselves dwelling on the past, or constantly worrying about what the future may hold? At those times when we find ourselves wishing that things could have been different, having regrets, or fearing what might be around the corner, it can become all too easy to suddenly realise that you’re not really living, more just existing.
To quote the American writer Mark Twain, “I've lived through some terrible things in my life, some of which actually happened”. We’re probably all guilty of stressing ourselves about what may go wrong, and it invariably doesn’t happen. Even if it does, worrying doesn’t change the outcome, but takes away the joy of living in the moment. Getting sucked into the past or future is exhausting. But what can we do about it?
If you want to be happy, do not dwell in the past, do not worry about the future, focus on living fully in the present.Roy T. Bennett (author)
Mindfulness is a big one.
It is all about living in the moment; being aware of your surroundings, noticing and being aware of sensations, and just accepting them however they are and allowing them to ‘be’. It can take some practice to learn how to slow your mind down, and at first, it’s tricky when your brain wanders off. But you can learn to bring it back, stay with your breathing, and listen to what’s around you. Focus on things around you that attract your attention, like a pretty flower or lovely birdsong – things that make you feel gratitude or appreciative. You can even try things like mindful walking, mindful eating, even mindfully brushing your teeth! It’s all part of staying present in the here-and-now, slowing your mind, and ultimately improving your wellbeing.
Breathing exercises can be beneficial.
I can feel a bit of a twinge writing this as I know that breathing exercises can be a bit hit and miss when you have a lung condition! I use mindfulness apps on my phone and know that some of the ones they suggest, like breathing in for four and out for eight, do not work for me (how can you breathe out for eight when you’ve taken in half that much air?!) However, focusing on your breathing, slowing it down, and making it deeper, can all help with grounding and staying present. Find the rhythm that works for you, I reckon!
Make a deal with yourself that you won’t worry about things that are entirely out of your control, or past events that can’t be changed.
Because, frankly, what’s the point? It takes a LOT of energy to worry, and you can worry all day and all night long if you want, but it won’t change anything (other than making you tired and grumpy probably). As the Dalai Lama wisely said: “If a problem can be solved then there is no need for worry. If it cannot be solved there is no use worrying about it”.
However, we are all human and so we are going to worry. Asking yourself things like ‘will this be important next week?’ or ‘what would I tell a friend?’ can help give another perspective on concerns. You could try setting aside ‘worry time’ once a day for around half an hour (not too close to bedtime), and every time a worry comes up you tell yourself that you’ll worry about that later. When worry time arrives, if you’re still worried about those worries, then you spend those full 30 minutes worrying – and then you put them away for tomorrow.
Never underestimate the power of writing down your thoughts.
This is an excellent way of staying in the present. It gets them out of your head, creating distance from them.
Worry monsters also like to eat worries or hang onto them for another time! Journalling helps get in touch with how you might be feeling, giving yourself time and space to let your thoughts flow. And the more they come out, the more you can slow them down, pay attention to them, and give them a bit of thinking time – rather than having them rushing around your head like a whirlpool.
So next time you find yourself feeling sadness about the past, or panicky about the future, maybe have a go at slowing down, not constantly keeping busy or multitasking, but getting in touch with yourself and your feelings instead.
Visit www.bit.ly/SophiesExperiences to read about Sophie’s own experiences of PH. Look out for more of her advice in the next issue of this magazine.
‘PHoenix’ is an innovative new trial testing how well PAH drugs can be personalised for patients – and it’s set to roll out across more of the UK’s specialist centres soon. We spoke to Dr Frankie Varian of the Sheffield Pulmonary Vascular Disease Unit to find out more about the study’s aims, and how it’s been shaped by patients from the start…
The PHoenix trial, which opened for recruitment in June 2023, is a unique study using remote monitoring devices, placed into the lungs and over the heart, to help choose the best medications for people with pulmonary arterial hypertension (PAH).
It’s all about tailoring treatments to the individual by finding out which drugs someone responds best to, using advanced technology to monitor people from their own homes – without the need for them to keep travelling into hospitals.
The study aims to discover whether patients on ‘dual oral therapy’ (two kinds of tablets) can enjoy a better quality of life by adding one of two additional, already approved, drugs - riociguat or selexipag - to their daily routine.
To help guide treatment for patients from their own home and to help balance the benefit of therapies against side effects, the study uses cutting-edge, remote monitoring technology that provides daily data similar to that gained from a right heart catheterisation. This data is provided wirelessly to the hospital
team via mobile phone. One tiny device sits in the pulmonary artery and the other just under the skin on the chest. This technology monitors the pulmonary artery pressures and heart rate rhythm activity by sending readings directly to the clinical team and alerting them of any problems.
Patients involved in the study are asked to complete walk tests at home and to fill out weekly quality of life questionnaires using a mobile app, to enable the team to monitor how they are responding to the drugs.
They are monitored weekly during the trial for six months, but only four hospital visits - for MRI scans - are required throughout this time.
No study has ever put riociguat and selexipag ‘head-to-head’ in the UK in this way before, and Dr Varian believes the trial outcomes could have an impact on PAH care worldwide.
She said: “We really don’t know which drug is best. We feel like they might be equal in terms of certain parts of the way they improve PAH, but we may find that for certain individuals, one drug suits them better than the other.
We hope the results will really help inform all care in the future when we think about prescribing these two therapies.
“There’s lots involved in the trial but at the end, each patient who takes part gets a personalised therapy. We don’t have to wait for the results of the full study, as we can make a decision based on their responses to the drugs, and any side effects.
It’s a really unique trial in that way because normally when you go into a trial, you don’t find out the results until years later. But because all the drugs in this trial are already approved, it’s all about how we can ensure the best therapy for the patient.”
PHoenix is currently running at the Sheffield Pulmonary Vascular Disease Unit and at Hammersmith Hospital in London, with plans to open it up around other UK specialist centres over the next two years.
The study is focused on the treatment of PAH at this point, but there is significant potential for the findings to be used for patients with other forms of PH – and to influence the remote monitoring of patients in general.
“We’re in a fantastic era of change in terms of healthcare and there are so many new technologies that are coming through”, added Dr Varian.
“I think trials like this do offer a lot of hope and hopefully a better connection between the clinician and patient because of remote technology. I think all of these things are really exciting.”
For more details about the PHoenix trial, please contact either the clinical trial manager Jenn Dick via email at jennifer.dick@sheffield.ac.uk or Dr Varian via email at f.varian@sheffield.ac.uk
Dr Varian spoke about the PHoenix trial at our Together Sheffield event
PHA UK members played a key role in shaping the design of the PHoenix trial by completing an important questionnaire in 2021. The survey, conducted by the PHA UK in collaboration with Dr Varian and her colleagues, asked questions around the perceived importance of the study, and its aims and objectives.
The responses helped secure funding for the trial, as well as shaping its design – a powerful example of the very real difference it makes when patients share their thoughts in this way. PHA UK members are also part of the PHoenix steering committee.
Delivering a presentation at our Together Sheffield event in October ( pictured above), Dr Varian spoke to the gathered patients, and their family and friends, about the importance of delivering patient-oriented research in this way.
“The presentation was really a focus on how we can best design our research and clinical trials with the patient at the centre”, she said.
“That means using all of the evidence that we have that’s been delivered through the questionnaires patients have completed in the past, interviewing them before the trial, and working with them throughout the study.
“We want to really have a partnership with them, and I have found that with PHoenix, patients have helped every step of the way with making sure we’re asking about side effects, medication burden, and quality of life regularly.
“Without having that dialogue with them, I don’t think the trial would be as well designed as it is now. It has really enhanced our study and the PHA UK is such a brilliant network to be able to engage with patients who are interested in being involved.”
Dr Varian also delivered a presentation about patient-oriented research at the National PH Research Forum, a gathering of PH researchers and clinicians organised by the PHA UK and held at the end of last year.
Aiming to inspire other researchers to keep patients at the centre of all studies, her presentation was voted the best of the conference, securing a £1,000 grant to enable her to further her work in this area.
We
Duncan and Rory Morrison are now at the start of a year-long fundraising challenge, running a half marathon each month to support the PHA UK in memory of their aunt. We caught up with Duncan in February, as the brothers prepared to embark on race number one…
We’re a very small family, and Lesley lived with her sister Linda who has a mild learning disability. So she had caring responsibilities before she became ill, and once she was diagnosed with PH, it was a case of them caring for each other.
It was fortunate that her PH was picked up by the Golden Jubilee Hospital in Glasgow quite early, as it meant she got started on treatment quickly. That gave her a decade of really good quality of life, before we lost her a year ago.
Lesley had a great sense of humour, despite all the challenges, and she was always positive. That was massive; her ability to get out of bed every day and just tackle everything. She was incredibly caring, and always thinking about other people, rather than being solely focused on the illness.
I moved away to study in Edinburgh from 2018 until 2022, which is when my younger brother Rory became more involved in caring for Lesley. As well as providing emotional support he learned how to make up her drug cartridges, as she was on a Hickman line, and that all had a massive impact on his decision to train to be a pharmacist – which he’s doing now at university.
Lesley passed away on 27th February
2023. Rory and I had already decided to do the Edinburgh Half Marathon that summer, with the hope that she would have been there to cheer us on. But it wasn’t meant to be.
After chatting through some ideas over Christmas, we decided to do something really positive in her memory by running a half marathon every month for a year. We started with the Inverness Half Marathon on 10th March – the day she would have turned 57. We know she’ll be there with us in spirit for each event.
As well as raising money, it’s important for us to raise awareness of PH through doing this too. It’s misrepresented, it takes too long to diagnose, and it’s essential more than ever to spread the word. I want more people to talk about it, and to have PH in their vocabulary, and to understand that anyone can be impacted by it. You can be a top athlete, and you could get hit with an illness like this, and it could just wipe you off your feet.
I miss Lesley a lot and as a family we’re all doing our best to be there for each other. If she was here, I know she’d be at the start and finish line of every event we do, as that’s just the kind of person she was.
Rory has been training hard whilst at university
As well as the monthly half marathon, I’ll also be cycling 100km each month to add an extra element to the challenge. I’ll be putting my body under stress, to highlight the stress that people with PH have to go through every day of their lives.
Rory is doing his training up at university in Aberdeen, and I’m doing it here in Fife. We spur each other on as although we are very close, we are also quite competitive.
We’re almost at our fundraising total already, and I’m hoping we’ll smash that soon. We’ve got a whole year to keep asking for sponsorship, and that’s the beauty of the longevity of the challenge, as it will keep the conversations about PH going too.
JudithHurdman
Judith is a Consultant Respiratory Physician at the Royal Hallamshire Hospital, working with PH patients as part of the Sheffield Pulmonary Vascular Disease Unit (SPVDU) team. She returned to the PH service in 2022, after nine years in a different role.
How did your PH journey in Sheffield begin?
"I started working at the SPVDU as part of my respiratory registrar training rotation in 2008, and then did research work there, plus a quality improvement project, until 2013.
My research work involved setting up the ASPIRE database, the research database in Sheffield, as part of our work looking at PH with lung disease. This was a huge job, going through 2,500 sets of medical notes.
Then I moved to the other hospital in Sheffield (The Northern General) where I focused on pulmonary embolism, and the acute side of that. Obviously, there’s a big link as doing pulmonary
embolism follow-up clinics does relate to chronic thromboembolic pulmonary hypertension (CTEPH).
In 2022, the role of Consultant Respiratory Physician at the SPVDU became available, so I returned to PH. I now do a split role, with some pulmonary embolism work at the Northern General Hospital and some at the SPVDU. I move a lot between the hospitals, and my days are very varied."
How does it feel to be back working in PH?
"It’s nice because I know a lot of the SPVDU team from a long time ago, and it’s quite a small team, with everyone
"A lot of the standout moments ofmycareersofar areaboutpatient journeys"
really focused on what they’re doing. They’re very dedicated and caring so it is quite an easy team to settle back into. Things have changed a bit since I was away, for example new drug treatments coming in, and the launch of the national balloon pulmonary angioplasty service for CTEPH [carried out at Royal Papworth Hospital]. A lot of things are still very similar though. It’s nice to see patients that I knew from before; for example, someone saw me the other day and remembered me from her first visit to the unit in 2012. "
"There are people I’ve worked with or trained with along the way, and you gain different things from different colleagues, but it’s got to be the patients. A lot of the standout moments of my career so far are about patient journeys, especially when you see someone reach an improvement." .
It’s been two years since Kate Alsford was diagnosed with idiopathic pulmonary arterial hypertension, and she’s learning to adapt to life with the disease. Here she shares her challenges, her gratitude, and her advice to others at the start of their PH journey.
Iwas working on a beauty counter when my symptoms began. There was a lot of standing up and moving around and I had to climb three flights of stairs to get to the toilet and staffroom.
I started noticing I was having to stop halfway, while other people I worked with passed by. I’d be so tired, I’d have to sit down on the stairs, and it got really embarrassing. I just assumed I’d become unfit.
Things started to get really bad and I noticed my mental health was really declining. I kept thinking, why can't I breathe properly? What is happening to my body? I went to see my doctor a few times, and at first they thought it was anxiety, so we tried a talking therapy and they suggested antidepressants. Then they said it was probably asthma, and gave me an inhaler, but things just got worse.
Continued overleaf...
Getting appointments was hard through the COVID-19 pandemic, and I couldn’t believe how much the symptoms were affecting my life. They were affecting my relationship, my friendships, and my ability to be a mum to my children. Sometimes the breathlessness made me feel like I was choking to death. Things were becoming increasingly hard.
In 2021, my doctor finally did an ECG, and he could see my heart rate was very high, even though I was just sitting down. He told me to go to A&E, and I started panicking.
They did every test under the sun there, and I was sent home with a referral to the cardiology department. I went back and forth a few times through that winter of 2021, because they didn’t know what it was.
I just felt awful. As soon as I’d dropped my daughter at school, I would have to lay in bed all day, and my mental health was so bad. It sounds very dramatic, but I actually felt like I was dying. I was so depressed.
Luckily, I was eventually transferred to the PH service at Royal Papworth Hospital, who did a right heart catheterisation which showed my pressures were really high.
It was all a bit of a rush then, and I was told I had pulmonary hypertension and a massive bubble of fluid around my heart. I’d gone into heart failure, and I felt instant panic.
I kept saying, why me? I’ve never smoked in my life, I’ve always been
into fitness, and I just couldn’t believe it. I was expecting the worst.
It’s been two years now and I’m slowly coming to terms with it. I’m getting there.
I'm so, so lucky and grateful to have such an amazing family. It was such a worry for them when I was diagnosed, and they still worry now. I’ve got a really nice group of friends who have all been really understanding and I’m so lucky to have them too.
It is still tough though, and there are still times now that I need to cancel plans. I’m at home a lot, and if we go on little trips I have to make sure I’ve got my mobility scooter. So life’s really had to change, and I’ve had to adapt to what I’ve got, but I’ve been lucky in terms of the support I’ve had.
I’ve got two children, aged 13 and 18, and I do feel my role with them has changed massively through my illness. I’m not able to do the things I used to do with them, but I do also feel lucky that because I had them when I was so young, they’d grown out of being toddlers by the time I became ill. I’d never be able to run around after toddlers now.
My children are very supportive, and my daughter especially helps me all the time. But it’s hard not to think about when they are older. It’s scary and it all does impact my mental health.
If you look at me, you wouldn’t think there was anything wrong with me, and that’s the problem with invisible disabilities. It’s a daily task looking fine, but knowing that inside, you’re not.
If you look at me, you wouldn’t think there was anything wrong with me, and that ’s the problem with invisible disabilities.
But I get lots of support from my specialist PH team and palliative care team, who are unbelievable.
Based on my experiences, the first thing I would say to someone newly diagnosed with PH is that the internet is not your friend. Don’t Google, and don’t overthink things too much. Put your trust in your PH team as they will help you through everything.
PH is rare, and that’s scary, but I’ve made friends with a lot of people in the PH community through the PHA UK Facebook group ( www.bit.ly/OfficialPHAUKFacebookGroup). I don’t care as much about the little things anymore, and I really appreciate life. You only get one, and you need to live it.
There is so much advice and support out there. It does seem really scary and daunting at first, but have trust, have hope, and take every day as it comes.
Scan here to watch Kate talk about her experiences
“It is not the strength of the body that counts, but the strength of the spirit”
Robbie Saul-Georgel grew up with the PHA UK, as we joined the charity almost at the beginning, in 2001.
Robbie was by then five. He was diagnosed with severe pulmonary arterial hypertension associated with his complete atrioventricular septal defect (ASD) at the age of 16 months – too late for surgical repair, and his prognosis was that he could need a heart and lung transplant by the age of two.
He later developed Eisenmenger’s Syndrome and a coarctation of the aorta. Life seemed to be a round of hospital visits, oxygen, and medications. PHA UK conferences and family weekends [which ran up until 2019] became features of our year and we made some great friends along the way, as well as appreciating the tremendous support and strength of the organisation.
With his strong magnetic personality Robbie made friends with anyone and many young PHA UK members were in his network, both
Fran Saul-Georgel lost her son Robbie a year ago and remembers him as a man who never let PH define him – using his own journey to help others and leaving a very special legacy. These are her words.
patients and their siblings. He was less interested in the clinical information than socialising, and how great it was for us to be amongst others who understood without explanation what living with this condition is like!
He became known as someone who would not let his condition define him, whether pushing his boundaries participating in musical theatre or speeding down a ski slope. At one family weekend in Sheffield, he loved showing off his skiing skills, as well as helping the novices, and he went on to qualify as an instructor.
At one conference he received an award for spreading awareness about PH. From a young age Robbie had spoken openly in class and assemblies
about his condition and its impact, and fundraised both at Scouts and at school. On one occasion it was with a spelling competition, using the long complex words of his conditions as tie-breakers!
Attending Parliamentary Committees twice in his formative pre-teen years, in support of a PHA UK campaign, certainly awakened an interest in campaigning. He went on to study Economics and Politics at Exeter University, gaining First Class Honours.
During his time at Exeter, he travelled around the country with the university ski team, but also became involved with the organisation ‘UpReach’ which exists to support students disadvantaged economically, physically or mentally, in gaining top graduate jobs. Following graduation, he became an ambassador for UpReach and continued to advocate for them.
Likewise, he was a passionate volunteer and committee member for the charity
“Robbie truly showed us that limits are those you impose upon yourself, and that life, however short or long, should be lived 100% to the full”
‘Over the Wall’ and attended camps for sick children from the age of nine, both as a camper and then as a helper.
After completing an undergraduate placement with Deloitte, they offered him a prestigious graduate job, to start in September 2020. Following graduation in 2019, and much to his family’s trepidation, Robbie embarked upon an ambitious solo trip to Australia, gaining massive confidence and independence that those close to him would never have imagined could have been possible.
On his way home he stayed with a family friend in Hong Kong, narrowly avoiding being caught up in the riots. After a short period at home, he set off for Canada to spend a winter season in a resort near Vancouver.
He was hugely indignant that COVID-19 brought his season to an abrupt end in mid-March 2020 and he came home to lockdown and shielding for three months. However, he did not waste his time, and when his start at Deloitte was delayed to 2021, he sought a new post and took a role as a business analyst with international management consultancy Nous.
At Nous, Robbie was in his element.
He was hungry for success and quickly made an impact, being promoted three times in the 32 months he was there. He was renowned for his friendly approachability, willingness to join in any fun, ability at the microphone either in karaoke or as a DJ, and his big green headphones!
In his last year, simultaneous with being delivered the devastating news that, after years of stability, his disease had progressed and he should now be assessed for transplant, he set up and coordinated a disability and wellbeing network for the whole of Nous. It was available to 500 employees and across three continents.
Through sharing his own journey, he encouraged and empowered others also to share theirs, fostering an openness and mutual supportiveness in the workplace. This is undoubtedly his greatest legacy, and one of which he was immensely proud.
Robbie delayed being assessed for transplant so that he could complete a two-year work placement for Nous in Toronto. He was due to return to the UK in July 2023 but passed away suddenly in April after taking a short flight from
Toronto to Nova Scotia.
He was so very excited that he was finally going to be delivering workshops in person after all the lockdowns and remote working.
He was 27 years old and is greatly missed by his partner Jordyn, family, and many friends. Robbie truly showed us that limits are those you impose upon yourself, and that life -however short or long - should be lived 100% to the full. To quote Tolkien: ‘It is not the strength of the body that counts, but the strength of the spirit’. Robbie had strength of spirit by the bucketload.
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.
This is our original forum, which now has almost 2500 members. It’s a very active group, with responsive members who offer advice and support at all times of the day and night.
www.bit.ly/OfficialPHAUKFacebookGroup
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.
www.bit.ly/PHAUKCarersGroup
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.
www.bit.ly/PHightingOn_LifeAfterLossToPH
This has been set up independently of the PHA UK and is run by a group of parents. You can join
www.bit.ly/PHParentsUKat
You need to have a Facebook profile in order to join these groups.
Sign up at
www.facebook.com
It’s free and easy!
This four-week self-help programme consists of a series of workbooks based on Cognitive Behavioural Therapy (CBT), to help you take control of problematic worry and anxiety.
✓ Developed with a team of clinical psychologists
✓ Tried and tested by people with PH
✓ Complete in your own time at home
✓ Totally free of charge
100 % of people with PH who tested the programme said it helped with their anxiety.
“When I received the booklets, I was determined to get the best out of them. I cannot express my gratitude enough. The clear explanations and easy-to-use strategies to cope with anxiety have been life-changing.”
PHA UK member
Order the free programme by scanning this code, visiting www.bit.ly/OrderTheProgramme or
A bespoke piece of artwork, created exclusively for the PHA UK, aims to reassure people affected by pulmonary hypertension of the support network available to them.
Created by artist Kevin McAllister, who lives with PH himself, 'Acceptance' was painted in acrylics onto MDF board. It incorporates part of the PHA UK logo in the form of colourful arrows travelling forwards, and hands reaching up and down in a show of mutual support.
The nails of the single hand are painted with his wife’s nail polish, recognising the unwavering understanding she has shown to him over the years.
Newcastle-based Kevin, who has PH connected to congenital heart disease, said: “The painting has two meanings to it. You could see the single hand as someone asking for help, because they have
and friends, and the PHA UK. So the support goes both ways. It’s showing there are always people reaching out to help you, but you have to put your hand up to accept it.”
Although the painting has been inspired by Kevin’s own lived experiences of PH, he said the meaning is representative of many of life’s challenges – and shows the importance of making connections.
“The arrows taken from the PHA UK logo are all about moving forward in life, and travelling onwards, taking each step at a time. It was important to me to make them bright and uplifting.
This piece of artwork is about celebrating the everyday heroes who provide support for people with PH. I want people to look at this painting
and be reassured of the support that’s around them – even if they don’t realise it. And I want this to make them feel hopeful.”
Kevin has recently completed a fine art degree and artistic design is a huge part of his life, boosting his mental wellbeing and helping him cope with the challenges of chronic illness.
Describing art as a ‘total escape’, he added: “From the moment I pick up a pencil or a paintbrush, or start designing on my computer, everything else just switches off around me. I don’t think about my health conditions, about being a parent, or a husband – I just get engrossed in creation.
It doesn’t matter what is being created; it’s the feeling of making something myself, from scratch, that sends me into another world. It’s almost like a kind of meditation.”
You can see more of Kevin’s work on his website, www.wheesydesigns.com
‘Acceptance’
Kevin McAllister, living with PH by Words: PHA UK member
“Thanksforbeing
“One
person will come
Raya Mynot has lived with PH for most of her 23 years, and worried she’d never find someone to accept her for who she was – until she met Jade. Now celebrating a year together, Raya reflects on the difference their relationship has made to her life and explains how they make it together.work
Being in a new relationship can be daunting as it is, however, when having something like PH it can be even more nerve-wracking. Having to tell that person about having PH and letting them know you find some day-to-day activities challenging can be pretty hard – especially when that person is someone that you hope will like you, or that you want to make a good first impression with.
One thing I’ve learnt on this journey is that the right person will come along, and when they do, they will love you for exactly who you are, no matter what.
Me and Jade have now been together for a year. Over that time, we’ve learnt to manage my PH really well and now it actually doesn’t affect our relationship in many ways at all. Jade has been amazing with everything.
I learnt that education is key. I’ve educated her on everything I possibly could, and I even brought her along to a PH appointment day at my specialist centre pretty early on in our relationship.
“Having Jade around has given me a lot more independence”
Jade really took everything in and wasn’t afraid to ask any questions. Even now after a year she will still sometimes randomly turn around and ask a question, and I love that she feels so comfortable to do that. I will always be happy to answer any of her questions.
I think one thing that Jade needed to get used to was doing things at a ‘Raya pace’. When we are walking together, sometimes I have to remind her that she’s doing ‘Jade steps’, and she’s practically pulling me along behind her. We always have a giggle about things like that.
Jade has also taken on all of the medical things so well. When I’m not well she
knows exactly what to do and that was something she picked up so quickly. I always say she’s lucky because she gets to be more then a regular girlfriend – she gets to be medically trained too!
Jade knows I struggle with the cold weather too, so recently we haven’t been doing a lot. She bought me an electric hot water bottle, so I’ve been using that non-stop. With the cold weather, we've been doing movie nights with lots of blankets and cuddles.
Having Jade around has given me a lot more independence. She is insured to drive my car (I’m still doing my driving lessons) so it allows us to be able to go out when mum is at work. I rely on mum a lot less with Jade. We love going to the cinema for a little date night, and it's perfect for me because there’s no walking involved and it's always warm in the cinema.
It has been really nice to be able to have that time with Jade. We always make sure that we have the oxygen with us in case I need it. It can obviously get quite heavy, so Jade is more then happy to carry it.
When we are out, most of the time we park in a disabled bay so I don’t have to walk very far and it’s easy to get the wheelchair out if I need it, but if we park too far away, Jade gives me a piggyback, and it just looks like we’re joking around together. And because I’m really small it’s so easy for her. She will also always push me in my wheelchair too which she loves doing!
Jade has really helped me as a person too. With PH I get quite anxious to go places or be out for a long time, but when
“It takes such a special person to be able to understand PH”
doing it together it's so nice to know that I’m safe when I’m with her, and she’s there to get me out of a situation if I’m feeling unwell or anxious. It’s also really nice because like me, Jade prefers a cozy night in over going out any day!
So we always have some special ‘home date nights’ where we watch a movie with our favourite snacks, we make homemade pizza, and we sometimes do little crafts together like diamond painting, embroidery or crochet. These have actually been some of our favourite date nights which is so nice because is not what you would call a typical ‘date night’!
I honestly can’t thank Jade enough. It takes such a special person to be able to understand PH and handle things amazingly when in a difficult situation, and Jade does it so perfectly.
I always used to stress that I wouldn’t find anyone that I would want to spend my life with, but honestly those type of people come around when you least expect it, and it's all worth the wait when you do find your person.
Don’t be afraid to show people the real you, because that person will love you for exactly who you are when you find them.
Hi, Zoe! Why did you join the PHA UK?
Hospital consultants advised me to join as they said informationthewould be helpful and it would connect me to people with my condition. I joined in January last year.
What's the best thing about being a member?
Having informationup-to-date and reading Iphighters’otherstories. have also met a really good friend through the website. Sometimes it can feel
isolating but scrolling through the website and Emphasis makes me remember I’m not alone.
Which specialist centre do you attend?
I am cared for Hammersmithby Hospital in London. Thank you to the whole of the PH team there. Without them I wouldn’t be here today… words could never express how grateful I am.
This issue, we are introducing you to Zoe and Lee…
Zoe Bresnihan is 46. She lives in Kent with her partner and three children and was diagnosed with pulmonary arterial hypertension in November 2022.
What advice would you give someone newly diagnosed with PH?
Never give up hope. No matter how dark some days are, there will be another day that will be brighter. Your medical team are your anchor. Always accept help from friends or family members to make your life a little easier.
Who (or what) makes you happy?
My children and my family unit, and socialising with friends, makes me happy. Being able to continue working keeps my mind occupied.
And finally, what does being a 'phighter' mean to you?
Being a phighter means that I now have a better quality of life. Even though I experience pain and fatigue, I try to appreciate each day and live my life to the fullest. It puts it into perspective how important my children, relationship and family are.
Hi, Lee! Why did you join the PHA UK?
I joined shortly after diagnosis after my wife recommended I do so, and because of the information you were sharing.
What's the best thing about being a member?
The realisation that I’m not alone in my journey, and that other people are having the same experiences that I am having.
Lee Cuthbertson, 52, was told he had pulmonary arterial hypertension in March 2023 and is awaiting a lung transplant. He lives in Staffordshire with his wife Lisa and 16-year-old daughter Emily.
Which specialist centre do you attend?
The Royal Hallamshire Hospital in Sheffield. All of the staff there are extremely friendly… with a special shoutout to Dr Charlie Elliot. He was very thorough with his explanation of my diagnosis and very supportive.
What advice would you give someone newly diagnosed with PH?
Find out as much as you can about the condition and possible treatment plans…and don’t be scared to ask for a second opinion if you feel it necessary.
Who (or what) makes you happy?
seriousness of the position that I am in, but somehow, we manage to find the humour in the situation. I have a very good support network of a few really close friends which is vital in helping to maintain my mental health. I do my best to be as positive as I can.
You have to try to find happiness in everything. My daughter is my world and we are constantly laughing at the daftest things. She understands the
And finally, what does being a 'phighter' mean to you?
Being a phighter means never giving up. Taking each day
as it comes and just keeping going. I’d like to give a special shoutout to my amazing wife Lisa. I’m not sure how I would have coped without her constant support. I am not known for expressing how I feel particularly well and can keep things bottled up sometimes…but she has always been there, especially though the darker times.
Poetry books and a disco are the latest fantastic fundraising initiatives that have helped our charity in memory of Will Acres.
Will’s grandma Joan Hammond has written hundreds of poems, and the family compiled them into books to sell, alongside ceramic robin tree decorations made by Will’s mum Lea, in the run-up to Christmas.
January saw family friend DJ Mark Covell organise a disco event at Emsworth Sailing Club, where Will used to sail, and well over £4,000 was raised in his name. Lea told us: "It was an amazing evening with lots of dancing and love for Will. Although it was tough, we felt very humbled that so many friends and family came together to raise so much for PHA UK in memory of gorgeous Will. He’d have been chuffed!"
Will’s family and friends have fundraised tirelessly for our charity since his death and we will always be grateful for this kindness.
A snapshot of some of your support over the last monthsfew
Talented PHA UK member Corinne Taylor made all of these knitted, sewn, and quilted crafts, and sold them at fairs in the run-up to Christmas – raising over £173!
Will was a talented DJ and musician
The Dean family walked a total of 500 miles during January to raise over £1,000. A huge thank you to Andy, Naomi, Rueben, Florence, and Elsie for this team effort in memory of Kirsty.
Kind-hearted Alicia McInnes is gearing up for two huge running challenges in support of her friend Sophie Gloag, who she describes as “one of the strongest people I have ever met”. She'll be taking on the Paris Marathon in April, followed by the Edinburgh Marathon in May.
Aidan Lewis will be striding the streets of the capital on 21st April, as he takes part in the world-famous TCS London Marathon. Aidan is raising money for us in support of his wife Kate (and he’ll be cheered on from home by their dog Dexter!)
Your makeskindness a difference. We are an independent charity with no government funding, so we rely on the kindness of our fundraisers to help us support people affected by pulmonary hypertension.
From bake sales to bike rides, marathons to motor racing - and everything you’ve seen in this feature - we’re constantly amazed by the lengths our supporters go to in our name.
We are so grateful for every penny raised, and as we come into the warmer months, there couldn’t be a better time to support us.
Find out more, get inspired, or request your free fundraising kit, by scanning the code on the right.
Nurses and doctors from specialist centres north and south have been lacing up their trainers to pound the pavements for the PHA UK.
Dr Joe Newman of Royal Papworth Hospital completed the Cambridge Half Marathon in March, raising awareness of PH alongside funds in the run-up to his event.
Describing himself as ‘absolutely not a natural born runner’, he went on to conquer the 13.1 miles in less than two hours.
Further north, nurses John Harrington, Charlotte Peirson and Sammy Abraham, tackle a very hilly challenge. The trio, colleagues at the Sheffield Pulmonary Vascular Disease Unit, took on the undulating Sheffield Half Marathon at the beginning of April.
We have a very special PH community here in the UK and it’s really heartening to see healthcare professionals choose our charity, in support of their patients, when it comes to fundraising activities. And many PHA UK members sponsored Joe, John, Charlotte and Sammy, making it a real team effort for our cause.
Thank you to everyone involved!
Are you planning to do something fantastic in 2024?
Our free fundraising toolkit contains everything you need! Order now at www.bit.ly/PHAFundraisingKit
The latest National Audit of Pulmonary Hypertension sends a reassuring message that standards of PH care remain high – despite huge pressures on the NHS.
The National Audit of Pulmonary Hypertension (NAPH) sets out to measure the quality of care provided to people who are referred to pulmonary hypertension services in Great Britain. Data is collected from all the centres in England, and from the Scottish Pulmonary Vascular Unit in Glasgow.
The audit is performed by NHS England, and supported by NHS Scotland, NHS Wales, the PH Professionals Group, and the National Pulmonary Hypertension Centres of United Kingdom and Ireland Physicians’ Committee.
Here at the PHA UK we continue to support the audit on behalf of the patient community, after funding its first year in 2009. And every year we bring our PH community the key findings in a clear and accessible way, after members told us which of the measures are most important to them.
The Audit measures the quality of care by assessing whether patients receive the right treatment in a timely manner, whether the services are appropriate, and what the outcomes are for patients. It reports on performance against agreed national standards.
The 14th Annual Report demonstrates that the national network of PH centres continues to provide high quality care, despite more patients being evaluated and commenced on PH therapies than at any time since its inception. This should provide reassurance to people affected by PH, commissioners, and healthcare professionals, at a time when the NHS is experiencing unprecedented pressures.
Professor David Kiely Director of the Sheffield Pulmonary Vascular Disease Unit and Lead Clinician, National Audit of P
Opposite are some of the findings from the 14th National Audit of Pulmonary Hypertension, based on data gathered between 1st April 2022 and 31st March 2023. All eight specialist centres covering England, Scotland, and Wales took part.
More patients than ever are being seen by specialist centres:
The number of new referrals to PH centres has increased year-on-year, except in 2020-21 (duringthe COVID-19 pandemic). The number of new referrals has almost doubled in 14 years – from 1,861 in 2009-10 (the first year of the Audit) to 3,585 in 2022-23.
The number of patients starting PH treatment is the highest it has been since the Audit began:
1,049 people started therapy in 2022-23, increasing from 743 people in 2009-10, the first year of the Audit.
10 of the 15 national standards were met at national level in 2022-23
99% of patients referred to a specialist were diagnosed within 6 months. (95% target) This represents a 3% improvement from the previous year!
Created in 2009, the NAPH is the largest audit of hypertensionpulmonary in the world and its findings are used to inform futureplanning.service
97% of patients received a right heart catheterisation before being given drugs to treat PH. (95% target) This represents a 4% improvement from the previous year!
95% of patients who have had at least one consultation in the last year have had an emPHasis-10 quality of life score recorded (90% target). Here at the PHA UK we championed the introduction of this standard because quality of life is so important.
97% of patients taking PH medication have had at least one consultation within the last 13 months. (95% target) All consultation types, including those not in-person, are included in this standard and statistic.
The figures above represent the national picture, and are the key findings our members told us they were most interested in. You can view the full findings of the Audit at www.bit.ly/NAPHreport
Chermaine Kwant (pictured) is a registered dietician who lived with pulmonary hypertension before undergoing a lung transplant. Here, she shares what a day of food looks like for her – and how you can recreate her meals for yourself…
One of my main pillars is fresh cooking and consuming as much unprocessed food as possible. Opposite is what I ate in one day recently...
Do you have a question about food or nutrition?
Email nutrition@phauk.org and our experts will be happy to help.
I often start the day with my super protein banana pancakes.
This is a delicious dish rich in protein (which is good for your muscles and immune system), minerals (such as magnesium and potassium, important for various functions in your body like muscle contraction and heart health), and cinnamon (which stabilises your blood sugar, contributing to better energy levels throughout the day).
Here's how you make them (serves two people):
Blend 2 ripe bananas with 3 eggs, a tsp of baking powder, 2 tbsp of unflavoured protein powder, 2 tbsp of cottage cheese, and ½ tsp of vanilla extract. Now add some flour until you get a nice thickness for baking.
Fry some pancakes in butter. I like to stack them up a bit to get thicker pancakes. Make sure not to fry them for too long so they remain moist inside.
Serve them with some butter and cinnamon powder.
On this particular day, I had some leftover pumpkin soup from the day before with two boiled eggs.
Last year, we asked our PH community to tell us about their eating habits, diet challenges, and what areas they need help with – and over 600 of you responded. The insight gained through this questionnaire is
This was a regular quick meal for me of tuna patties with avocado smash. This is truly an easy meal with plenty of good fats from the avocado, which are very beneficial for your heart, brain, and skin. Tuna also provides a good amount of protein.
Here's how you make them (serves two people):
Drain 2 cans of tuna (in olive oil) and mix with 2 eggs, a finely chopped onion, some chopped tomato, freshly ground pepper, and 2 tbsp of flour.
Heat some butter in a pan and scoop heaps of the tuna mixture into it, then fry them on a medium heat until brown on both sides. Meanwhile, mash an avocado with a chopped clove of garlic and some salt and pepper. Serve this with the tuna. Feel free to experiment and add some fresh herbs like cilantro, parsley, or chives.
Do you have a favourite recipe you’d like to tell us about?
Email nutrition@phauk.org and we’ll share it with our members!
already shaping our work, and resources will be produced throughout the year to support you in the ways you told us matter.
The key findings from the survey - the first of its kindwere included as a separate booklet with this magazine.
You can also view the summary report, and the full findings too, by scanning the code on the right or visiting www.bit.ly/PHandNutritionFindings
Thank you to everyone who took the time to share their thoughts and experiences in this way.
By Shaun Clayton, a certified nutritionist and Operations and Finance Director at the PHA UK
When coping with modern life, finding the time and energy to prepare nutritious meals daily can be a Herculean task, especially for those among us navigating the challenges of pulmonary hypertension. Enter the humble ready meal: often misunderstood, and yet, it can be a potential ally in our quest for balanced nutrition. Let me explain why! The notion of ready meals typically conjures images of preservative-laden, sodium-saturated boxes. However, nestled within the freezer aisles or the refrigerated sections of Tescos or Asda, there exists a variety of options that defy this stereotype. The key? Look for meals that boast a balance of nutrients – fats, carbohydrates, and, crucially, proteins.
Protein is the unsung hero of satiety, keeping you feeling fuller for longer and providing the sustained energy needed to navigate the day. For individuals managing PH, this nutrient is particularly invaluable. Fatigue can be a frequent visitor, making the task of cooking seem daunting.
High-protein ready meals can offer a convenient and satisfying solution.
While protein takes centre stage, it's the balance with fats and carbohydrates that completes the performance. Go for meals that offer a good mix of all three, ensuring they come from quality sources. Look for things like wholegrains, lean proteins such as turkey, low fat mince or chicken (plant-based options include tofu and tempeh), and healthy fats from things like avocados, nuts, or hummus. These are your allies here. This balanced approach not only supports general health but can also help in maintaining your energy levels throughout the day.
Ready meals have long been stigmatised as the opposite of healthy eating. Does “I thought you were trying to eat well, put that box down!” sound familiar? But as our understanding of nutrition evolves, so too does the landscape
of prepared foods. Today, many options are designed with health in mind, catering to a variety of dietary needs and preferences. They can be a practical tool for those times when fatigue sets in, and the thought of standing over a stove or chopping by the worktop is overwhelming.
The reality is, unfortunately, nutritionally complete ready meals do tend to cost more than cheap, highly-processed versions. But it's always worth keeping an eye out for special offers, or if you find one you like, checking the pricing across different supermarkets.
It's true, homemade meals where you control the ingredients and portions are often ideal. However, ‘ideal’ doesn't always align with the realities of living with PH. There are days when ‘good’ is not just good enough; it's just right. On these days, a well-chosen ready meal can provide the nutritional balance needed to fuel your body without depleting your energy reserves.
When selecting a ready meal, glance at the nutritional label. Look for options high in protein but be mindful of sodium levels, which can be particularly high in prepared foods. Seek out meals with a colourful variety of vegetables, lean proteins, and complex carbohydrates. Remember, variety is not just the spice of life; it's the foundation of a balanced diet.
For those living with pulmonary hypertension, managing energy levels is crucial. Ready meals can offer a convenient, nutritious solution for those days when cooking is a mountain too high to climb. By choosing wisely, you can enjoy the convenience without compromising on nutrition.
In the end, ready meals are not a one-size-fits-all solution, but they can be a valuable part of a balanced diet, especially for those managing chronic conditions like PH. So next time you find yourself reaching for a ready meal, do so with confidence, knowing that with the right choices, you're not just opting for convenience – you're embracing a practical, nutritious option tailored to your needs.
✓ Keep this checklist handy, perhaps on your smartphone or as a small card in your wallet, when shopping.
✓ Remember, it's about finding the best option available that fits within these guidelines, not seeking perfection.
✓ Consider your personal nutritional needs and preferences, adjusting the checklist as necessary.
■ High protein: Aim for meals with at least 10-20 grams of protein per serving to help you stay fuller longer.
■ Balanced carbohydrates: Look for wholegrain or complex carbohydrates rather than refined ones for sustained energy.
■ Moderate fats: Look for meals with a healthy balance of fats, preferably with more unsaturated fats. Avoid trans fats entirely if you can.
■ Lower sodium: Choose meals with less than 600mg of sodium per serving to help manage blood pressure.
■ Microwave-safe packaging: Ensure the packaging is safe for microwave use to make preparation easy and safe.
■ Quick and easy preparation: Choose meals that can be prepared quickly, catering to times of fatigue.
■ Diverse cuisine: Select a variety of cuisines to keep meals interesting and appealing.
■ Vegetable inclusion: Ensure there's a good portion of vegetables for balanced nutrition.
■ Appropriate serving size: Check the serving size to ensure it meets your dietary needs without encouraging overeating. Sometimes ready meals are designed for two people, so be careful of this.
■ Whole, real ingredients: Prioritise meals with ingredients you recognise and can pronounce.
■ Limited additives: Be on the lookout for meals with the fewest preservatives and artificial ingredients.
■ Dietary restrictions: If applicable, check for gluten-free, dairy-free, vegetarian, or other dietary specifications.
■ Long shelf life: Find meals with a longer shelf life to have a convenient option available without frequent shopping trips.
■ Clear nutritional information: Ensure the meal has clear and comprehensive nutritional labelling.
■ Fibre-rich: Look for meals with at least 5 grams of fibre per serving to promote digestive health and satiety.
■ Serving suggestions: Look for any serving suggestions to enhance the meal, such as adding fresh vegetables or a protein source.
Grab your furry friend for Paws for PH and walk with us on World PH Day SUNDAY 5TH MAY
Let’s walk together, wherever we are, to raise much-needed funds for the PHA UK this spring…
Paws for PH is our brand-new initiative, spearheaded by PHA UK Chairman Iain Armstrong, to raise money and awareness on World PH Day (Sunday 5th May).
Our charity receives no government funding, so we rely on the kindness of our supporters to help us continue our work. This is a brilliant opportunity to come together as a community and walk our own routes, and our own distances, knowing that others around the UK will be doing the same.
Collecting sponsorship for your dog walk (or dog jog!) will really help our charity, and because asking for donations sparks
conversations about PH, it’s a great way of raising awareness too.
We understand that pulmonary hypertension affects everyone differently, so choose a distance that works for you. Whether it’s a couple of laps of your garden, or ten miles along the river…every step and every pawprint matters. If you can’t take part yourself, perhaps a friend or family member might like to take up the challenge?
World PH Day is an annual global event to raise awareness of PH. Find out more at www.worldphday.org
Everyone who takes part gets a PHA UK medal, certificate and some delicious dog treats. Raise over £50 in sponsorship and we’ll also send you a free bandana for your pooch!
A round appaws!of CONGRATULATIONS AND THANK YOU forleapingtothechallenge andsupportingthePHAUK
Treat Time!
You can take on our Chairman’s Challenge alone, or with friends or family.
Want to step up the challenge? Jog with your dog instead!
“ This challenge combines two things we know make a big difference to quality of life – movement and animals! Many of you know that I’m a huge dog lover, having been a volunteer with the charity Guide Dogs for many years alongside the PHA UK and my work as a Nurse Consultant in PH.
After what’s felt like a long winter, the better weather of spring makes it the ideal time to get moving - within your limitations - and I hope this challenge will be a good incentive to get out walking with your four-legged friends.
Don’t worry if you don’t have a dog though; you can still take part without one! And you don’t have to walk – if you normally take your dog out whilst on a scooter on in a chair, this challenge is still for you.
World PH Day is another great opportunity to raise awareness of this rare condition, so we’re encouraging as many people as possible to complete their walk on Sunday 5th May.
This challenge is as much about encouraging togetherness and physical activity as it is about generating income. But raising even just a small amount in sponsorship will make a big difference to our work, and we are truly grateful for every penny.
Thank you in advance for supporting this special challenge. ”
Iain Armstrong Chair, PHA UK
5 Advanced Pharmacist at the Scottish Pulmonary Vascular Unit (SPVU), Golden Jubilee Hospital.
5
WHO OR WHAT INSPIRES YOU IN YOUR WORK?
"As cliche as it sounds, the patients. PH patients go through so much and to see and witness their tenacity, determination and willingness, all with a smile (usually!), it makes work very worthwhile."
WHAT’S GREAT ABOUT WORKING IN THE NHS?
Josh has been part of the Scottish PH service for six months, covering a colleague’s maternity leave. We caught up with him to talk teamwork, typical days, and crispy M&Ms…
WHAT DOES AN ADVANCED PHARMACIST DO?
"My job covers lots of different aspects. The mainstay is making sure that we are using the most effective medicines and ensuring that this is done safely for every patient. We usually say, “right patient, right drug, right time”. A lot of my work is clinical – on ward rounds and outpatient clinics where I may be actively prescribing and discussing treatment with patients. Other parts of my job include managing homecare and repeat prescriptions. Other aspects include governance and medicines information, especially responding to patient and other healthcare professional queries."
WHAT DOES YOUR DAY NORMALLY LOOK LIKE AT THE SPVU?
"Most healthcare professionals will say no two days are the same, and that is certainly the case within our team. However most 'typical' days will include an early ward round, reviewing the patients admitted to the ward with our consultant, clinical fellow and specialist nurses. As we cover the whole country of Scotland it can become busy! Clinics also run in the afternoon and here I will see patients face to face. It is always lovely to see them."
COMPLETE THIS SENTENCE: THE BEST PART OF MY JOB IS…
"Working with the incredible SPVU team – they have all been so friendly and welcoming and we all get on so well. We are a very tight-knit group, and they make work so much more enjoyable!"
"Teamwork – over the past few years particularly with the COVID-19 pandemic, it was evident to see just how adaptable and amenable we are when we work as a team. I don't think other organisations or companies could have succeeded in the way we did, and I suspect that is down to the teamwork of all involved."
WHAT’S ON YOUR DESK?
"Currently a big pile of epoprostenol prescriptions to check. A large water bottle (a new year resolution to drink more water), and a snowy Christmas Santa decoration gifted by a colleague, who has somehow become the office mascot!"
HOW DO YOU THINK YOUR COLLEAGUES WOULD DESCRIBE YOU, IN JUST THREE WORDS?
"Witty, helpful (I hope), quick-replier (I know that's two!)"
WHAT DOES TIME AWAY FROM WORK INVOLVE FOR YOU?
"I enjoy finding new places to have brunch or dinner. I am a fair-weather paddle-boarder – so I can often be found in a loch somewhere, (hopefully dry, but I have been known to fall in once or twice)."
"M&Ms (crispy) will win my heart every
I’ve had a bit of bad luck recently. I injured my knee when I slipped doing dance at school. Unfortunately, I have a small fracture to my femur and some cartilage and ligament damage.
These past three-and-a-half weeks, I haven’t been able to do anything very active – in fact I have not even been able to go to any upstairs lessons at school. Being limited in how I can get around has meant I have had to find different ways of keeping myself occupied.
Here are some of the things I’ve been doing to keep myself busy…
I have learned to crochet. I was lucky because I had been given some crochet kits for Christmas and I could use the instructions and online videos to help me learn. I’ve made a few Harry Potter themed toys. Crochet is easier to do than knitting while waiting for appointments because you only need one hook – not two needles.
I’ve been playing more Mario Karts on our Switch. This has been brilliant because we sometimes play as a whole family and thanks to all my practice, I am now doing much better when racing against everyone else, which is fun.
I have had to find different ways of keeping myself occupied
I have also had a go at some origami – I’ve been making some flowers. I worked out how to do that online and it’s a lot of fun. Of course, I do also still have school homework to do. I can also still play my violin and join in with singing and drama at Stagecoach [a performing arts academy]. The other things I now have are physiotherapy exercises.
The physio is not too bad and I’m hoping that if I do my exercises every day I will be back to being able to dance, play netball, go to cheerleading club and join in with PE again before I write my next column.
X
Emilywrote thiscolumnin February2024. Wewishherthebest ofluckforafastand fullrecovery!Our Listening Line support service gives you dedicated time with a PH professional to talk through any worries or concerns you have relating to your emotional or mental wellbeing.
Speaking to someone who already understands PH means you don’t have to explain the condition first, and this service is available to family and friends too.
Find out more or book a telephone chat by emailing listeningline@phauk.org or visiting www.bit.ly/ListeningLine
“Paul’s support lifted me. He was so understanding and supportive, and it was helpful to chat with someone who knows about this disease.”
PHA UK member
Our e-support service is as valuable as a helpline, but delivered via email instead. We can help with ongoing emails to support you through a difficult time, or with one-off enquiries or problem-solving.
Contact us at support@phauk.org at any time and we’ll get back to you within two working days.
“I'm so grateful to have this chance to email as I'm hearing impaired”
E-support service user
Members of our Feedback Forum and Research Forum panels play a key role in helping to guide PHA UK activities and contribute to important research work.
We contact all forum members with opportunities via email, and it is up to you whether you respond. We appreciate that PH comes with bad days so we understand if you need to give some of our invitations a miss. If it’s not for you at that time, simply ignore the email and look out for the next!
Whether it’s a booklet, poster, video, or web page, we need to be sure that it meets the needs of the PH community.
That’s why we’re building a mix of people affected by PH - patients, and their loved ones too - to give us feedback on new materials before they are launched. Your opinions are really important, and they help us make sure we’re getting things right.
As part of our Feedback Forum, we’ll ask you to spend a little time reviewing our materials before they are launched. This may be in the form of reading a leaflet, watching a short video, or having a look at a web page.
Get involved in your own time, from the comfort of your own home.
You will then be asked to fill in an online form with your thoughts. Sometimes, we may ask your thoughts on an idea for a campaign or project during the planning stages, to ensure we are taking things in the right direction.
Join at www.bit.ly/PHAUKfeedbackforum
There’s never been a more important time to lend your voice and experiences to PH research. As well as conducting our own studies to help people affected by PH live better lives, we are regularly approached by researchers from healthcare and academia, who recognise the importance of the patient voice.
Our Research Forum is for patients and loved ones or carers who would be willing to help with these studies when possible.
They often involve completing surveys or questionnaires, and sometimes they may involve online or telephone chats, or focus groups. Occasionally, you may receive an invitation to be involved in a different type of study, but it is unlikely you will be asked to take part in a clinical trial in this way. Join at www.bit.ly/ResearchForum_PHAUK
PHA UK Support Nurses Paul Sephton and Sian Richardson are former Clinical Nurse Specialists in PH, with long careers at the Sheffield Pulmonary Vascular Disease Unit. Still registered nurses, they now work part-time with our charity to offer support to our members.
“A friend has suggested I look into palliative care, but my specialist centre has never mentioned it to me. Does that mean I’m not eligible for it? What should I do?”
Palliative care, also known as supportive care, can make a huge difference to quality of life – so it’s really positive that your friend has brought this up with you.
We have both worked in palliative care and in hospices, as well as being clinical nurse specialists in PH during our nursing career.
Palliative care is all about living well, and that’s a really important thing for people to understand.
Please be reassured that just because your specialist centre hasn’t mentioned it to you, it doesn’t mean you are not eligible for this type of supportive care – or that it wouldn’t help you.
There are still lots of misconceptions about palliative care, with some patients believing it is only about dying. And research supported by the PHA UK has shown that for many healthcare professionals, the experience of receiving negative reactions from patients when discussing palliative care sometimes stopped them bringing up the topic earlier.
The 2019 report by Dr Sarah Woolcock, tiled Exploring the delivery of Palliative and End-of-Life Care in Patients with Pulmonary Arterial Hypertension: A Qualitative Interview Study summarised that ‘fear of such negative reactions was often a barrier to broaching the topic of palliative care with patients. Staff feared removing hope from the patients as well as they themselves being perceived as giving up on the patient. The implication of these experiences for clinical practice is that many staff delay introducing palliative care for fear of how the patients may react, thus reinforcing the negative perceptions of patients’.
However, the same research also showed that healthcare
professionals would welcome the patient bringing the topic of palliative care up themselves. So, our advice would be to bring this up with your Clinical Nurse Specialist (CNS), either at your next appointment, or by contacting them in the usual ways (you do not need to wait until you have an appointment).
Once you have spoken to your CNS, one of two things are likely to happen. Your nurse may liaise with a doctor within the PH service, who will write to your GP to request that you are referred to your local palliative care services. In some cases, the CNS will be able to make the referral themselves.
Once it has been confirmed that you have been referred to your local palliative care services, there may be a wait until you then hear directly from them.
This is because they will be considering the referral, and which palliative care services are most appropriate, to ensure you receive the best possible care.
It is important to understand that palliative care encompasses many different things and there are lots of different ways of accessing it. For example, you might be surprised to know that district nurses deliver generalist palliative care (visiting patients in their homes) and they are really good at it.
Hospice care is one part of this type of supportive care. In-patient hospice care is part of what’s known as ‘acute’ care, and this can be for things like symptom management.
Hospices also offer holistic care, often through what’s commonly known as ‘living well’ services, and this can include things like counselling support, complementary therapies
such as reflexology, and activity-based group sessions. These services often involve attending hospices for the day, as part of a six-or-eight-week programme.
If palliative care hasn’t already been brought up with you, then it is likely that this type of holistic care is what will be most appropriate for you at this time.
Regionally there are other palliative care services available, but they vary around the UK so it is something you will need to speak to your team about. If you would like to discuss palliative care, or you have questions about what it entails, or how to access it, here at the PHA UK we would be very happy to talk to you. Please email us at support@phauk.org or call 0300 373 5367 (if there is no answer, please leave a message and we will call you back as soon as we can. This may be few days later but please be assured we will respond! )
In some cases, because we are both still registered nurses, we are able to refer people who contact the PHA UK to palliative care services. However, this depends on circumstances, and for data protection reasons we are unable to access clinical information from your centre, so please contact us for an initial discussion. We also need the consent from your own GP to process any palliative care referral we complete.
To summarise, please do not be afraid to bring up palliative care with your specialist team, or with us if you would prefer. This type of supportive care can make such a difference to people living with pulmonary hypertension.
With advice from experts and real experiences from people with PH, Palliative Care & PH aims to challenge misconceptions and help you understand more about this type of supportive care. The guide also includes a separate Anticipatory Care Plan booklet, tucked inside the back cover, to help you plan for the future. Scan this code to order now for free
Find your local hospice at www.hospiceuk.org/hospice-care-finder
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…and much more to help with all aspects of living with PH
We’ve invested in technology that allows people to access our online content at www.phauk.org in a way that works for them. Assistive toolbars will read text aloud, translate into 99 languages, magnify text size, convert content to MP3 files, and much more.
Simply tap on this button at the bottom of each web page to access all of the assistive features.
A PHA UK collaboration has enabled the launch of a new book to help children with pulmonary hypertension understand more about their condition.
Exploring PH with KiKi the Koala has been written and designed by 22-year-old Londoner Maryam Hussain, who was inspired by her little sister Fatima’s experiences (both pictured together below).
The PHA UK has covered the print and production costs to allow as many families as possible to benefit from this unique publication.
By following KiKi’s journey, the book aims to help children aged three to nine learn more about PH and realise how special they are – just like KiKi!
Scan left to find out more about Maryam and Fatima, and the inspiration behind the book.
Did you know...?
Great Ormond Street Children’s Hospital had just 10 beds when it
Our new regular space for children with PH
Daisy Hatton is 5 years old and lives with her mum, dad, brother Jaxon and pet dog Coco. She was diagnosed with PH when she was 1.
My favourite colour is...
My favourite food is...
Pink Strawberry Mini Milk lolly
My favourite Disney Princess is...
Mirabel
My favourite place to visit is...
The beach. Running on the sand, paddling in the sea and collecting shells
My favourite school subject is...
Swimming
My favourite TV show is...
Peter Rabbit. I love Lily Bobtail
My favourite book is...
The Gruffalo
Did you know...? Lungs are the only organ in your body that will float in water!
Would you like to tell us about your favourite things? Ask an adult to email media@phauk.org
Did you know...? Humans blink around 21,600 times each day. Can you count how many times you blink in a minute?
Meet our new Kids Corner columnist, 10-year-old Coco Praest. Coco started being treated for PH when she was 5 months old, and she lives in London with her mum, dad, and twin sister Aika. At school, she loves art, science, and computing. When she’s at home she enjoys playing Marion Kart8 and DragonVale with Aika, watching TV, reading, and drawing. In her very first column, Coco reviews Exploring PH with Kiki the Koala, the new book to help children understand PH…
I really liked reading the book Exploring PH with Kiki the Koala, because Kiki is cute and friendly.
This book is about life with PH for kids. It would be good for families who have children with PH. The book has pretty illustrations of Great Ormond Street Hospital, which is my hospital as well! My nurses and doctors are kind and supportive too. Children with PH can do fun things like everyone else. When I was little, I liked dressing up and I still enjoy baking now, especially chocolate cake! Just like Kiki, I have ways to help myself with PH. At school, when I feel tired I have a rest as well.
Kiki talks about her multiple medications. I have medications too, including an inhaler and tablets to keep me healthy. I think that people with PH are very brave because they have many challenges, especially children because they're little!
The page with the diagram of the heart is helpful to understand PH and how it affects the arteries. I agree about doing things in my own way because sometimes I need to sit down and take a break. In my school, I have a beanbag and a blanket in the playground, for cold and damp days, so I can read and rest outside near my friends! However, most days I ride on the roundabout, build on the Lego wall and play with my friends.
Soon my family and I will be moving to a new house. It will have lots of stairs so the breathing tips from Kiki might be useful as I don’t have many stairs in my old home.
The colouring pages, at the back of the book, are a good idea for kids who like colouring. That’s why I think Exploring PH with Kiki the Koala is an amazing book! You should read it!
You can ask an adult to order you a copy (it’s free!) at www.bit.ly/KiKiTheKoala
Dr Joe Newman is a Clinical Research Fellow at Royal Papworth Hospital NHS Foundation Trust.
A study involving PHA UK members has shown that a one-minute walk test might be just as effective as a six-minute walk test when it comes to assessing pulmonary hypertension. But how soon could we see a change in clinical practice? The study’s lead researcher Dr Joe Newman explains more…
InMarch 2022, along with the PHA UK, my Royal Papworth Hospital and University of Cambridge colleagues and I conducted a large online survey to ask about proposed research methods. You may remember completing this survey, which was published as a report called 'Clinical trials: What matters to you?'
We learned an awful lot from the 112 patients who completed it. They told us what's important to them when it comes to clinical trial design, what's difficult in terms of taking part, and what we can do to help improve or facilitate them taking part and getting over some of those burdens.
that it’s been around for.
This exercise test’s strong points are that it’s really useful because we can both use it in clinic, and we can use it in trials to measure patients' progress over time. So, for example, when patients get better with drugs, we see the walking distance increase. But likewise, if we see the walking distance getting shorter, then that might be a sign that a patient is starting to deteriorate.
Some of the feedback was around the six-minute walk test, which I'm sure many patients across the UK are familiar with (patients at the Royal Hallamshire Hospital in Sheffield do the shuttle walk test instead, which is a similar concept).
It’s a great test, but it's not perfect, and it’s not changed in the 40 years
So, it's really useful, but we know that it can be a bit of a challenge for some patients because walking for six minutes is a relatively long time.
We know that often it involves travelling into a specialist centre to conduct the test, which can be exhausting in itself. So, we started to wonder whether we could make the test shorter, and whether we could make it digital.
In terms of making the test shorter, when we looked back at the original research papers from back in the early 1980s, we found that the six-minute time frame was a bit arbitrary – a bit of a random choice. It could have been one minute, or it could have been 12 minutes, but it came out as six
minutes as a bit of a compromise.
We studied about 200 patient records from Royal Papworth Hospital and compared the distance walked in one minute versus six minutes. And we found that the one-minute walk test is actually just as useful as the six-minute walk test. The other advantage of doing the one-minute walk test is that it’s much more practical for patients to complete in the community, rather than travelling to a specialist centre.
On the back of these early findings, we partnered again with the PHA UK and a medical technology company called Aparito to develop an app, linked into a wearable Garmin watch, that would enable patients to complete walk tests in the community. For example, this could be on a pavement outside of their homes, or in a local park. Here we could compare the new digital one-minute walk test against the six-minute walk
In the 6-minute walk test, you walk as far as you can up and down a corridor in six minutes. During walk tests your heart rate and oxygen levels are also measured. Walk tests act as a measure of heart and lung health, they can show how able you are to do everyday tasks and can provide clues to the cause of some symptoms of pulmonary hypertension. Walk tests are also a good way to measure how effective your PH treatment is and if any changes to treatment are needed. Understand more about walk tests in our specially-created video at www.bit.ly/6MWT_PH
test and see how it performs when done more frequently and away from the hospital setting.
As well as being scientifically accurate, feedback from patients showed that they generally preferred the shorter timeframe of the digital one-minute walk test and the possibility that it offers for home testing. They were very happy to do it in a digital format from home, to avoid travelling into specialist centres. We do acknowledge though that digital technology is not necessarily for everyone but we try to make it as simple to use as possible.
These findings have now reached a global audience, having been published in the respected 'American Journal of Respiratory and Critical Care Medicine' in January 2024 and presented at medical conferences.
Getting these results published is really just the starting point. We've got lots more work to do on this, for example validating the test with more patients, improving the usability of the tech and studying how the test results change in response to treatments.
So, we've got ongoing studies in Royal Papworth Hospital in Cambridge and
Royal Hallamshire Hospital in Sheffield, and hopefully in other specialist centres across the UK too, to start to work out how we can use this new test in clinical trials and eventually in clinical practice.
One challenge will be taking a test with a 40-year history and trying to convince clinical colleagues, as well as patients, that doing something new is also potentially better.
Change like this can take time, but we're hopeful that by taking this really patient-centred approach to understanding not only what's good for the trials and the clinic, but what is good for patients too, it will help to make the difference.
We’re really excited with the progress that we've made with this project so far and we can trace its roots right back to the survey that we did with patients and the PHA UK in March 2022.
So we've come quite a long way already in two years but we're still at the early stages of developing this new digital test for pulmonary hypertension – and we’ve got a long way to go.
We need to do more studies, enrol more patients, and pass it through the regulatory authorities as well. We need to look at the data and hopefully eventually rubber stamp it.
But these things take a long time, so I wouldn't expect any dramatic change over the next few months, or even years. But gradually over time we might start to see changes in clinic, and in the way we do clinical trials using this walk test.
Watch this space!
A study into why people impacted by PAH take part in genetic research, co-funded by the PHA UK, will influence future activity in this increasingly-important field. Dr Emilia Swietlik of the University of Cambridge and Addenbrooke’s Hospital led the study, and here, she explains more about the findings.
Genetics plays a crucial role in various diseases and recently, genetic research and testing has become vital in uncovering the underlying mechanisms of pulmonary arterial hypertension (PAH) and developing effective treatments. Two significant studies, the National Institute for Health Research BioResource Rare Diseases (NBR) study and the Cohort study of idiopathic and heritable PAH (the PAH Cohort study) , have contributed to this.
Understanding patients’ knowledge and attitudes towards genetic research is crucial for its success and integration into clinical practice. Limited studies have directly explored the reasons individuals participate in genetic research, emphasising the need to address patients' concerns.
Co-funded by the PHA UK, the RAPID-PAH study was a comprehensive investigation of the attitudes to genetic research among PAH patients who participated in the NBR and PAH Cohort studies. It set out to understand the motivations and barriers to participating in research.
Our collaborative effort saw a total of 63 UK patients, caregivers, relatives, clinical teams, and researchers take part in interviews and focus groups. This allowed us to gather their perspectives and experiences of research and the impact of genetic findings on patients and their families.
Our findings showed many different motivations, obstacles, and expectations associated with engaging in genetic research.
Participants told us they were driven by a desire to "contribute to science and improve health outcomes for others," recognising the potential benefits for future individuals, including family members. Despite limited personal benefit, participants acknowledged the "potential to help others" through their contributions, emphasising the long-term impact of research.
Altruism (selfless concern for the wellbeing of others) emerged as a common thread, with participants recognising their role in advancing science for the greater good. Many were motivated by a desire to find a cure and improve existing treatments.
Findings also showed that personal experiences, including long and difficult diagnostic journeys, created gratitude towards PH teams, inspiring participants to ‘give back’ by taking part in research. And family considerations are motivators too, with many of those who took part in the study telling us they want to improve outcomes for their children and relatives.
Participants told us that by taking part in research, they often felt a sense of belonging to a wider community, finding support through social media or building connections with others facing similar challenges.
linked to their pulmonary hypertension centre.
In the studies we asked about, researchers shared important genetic findings with local clinical teams, who then communicated them to willing patients. These patients were later directed to Clinical Genetics services for additional NHS testing and counselling.
Participants' understanding of this process varied, influenced partly by whether they have genetic mutations. Those with mutations and NHS counselling generally had a better understanding, while those testing negative and not receiving results or counselling had lower awareness.
Challenges and concerns related to research participation included time and financial considerations, burdensome study procedures, and invasive tests such as right heart catheterisation.
Interest in research was also influenced by disease severity, with levels of engagement fluctuating depending on symptoms.
Invasive procedures, particularly right heart catheterisation, were cited as the biggest deterrent for some people. Plus, some were worried about having to change their medication in case their health deteriorated.
Some participants in the study themselves acted as barriers to recruiting their relatives due to concerns about the impact of genetic results on family dynamics. They took on various roles, including being information gatekeepers, emotional managers, and observers of symptoms.
Watching children for the onset of telltale symptoms was a common theme in the patient interviews. Several participants reflected on the strain of constant vigilance, combined with the hope that spotting signs early would be the best way to prevent the onset of disease. Many interviewees discussed whether their children should be tested for their genetic status.
The impact of the COVID-19 pandemic introduced additional barriers, including health concerns, delays in results delivery and testing disruptions.
The level of trust patients have in their healthcare professionals, as well as the perceived relevance of the research they offer, plays a significant role in their decision to take part in studies.
Many people in the interviews mentioned that they didn't feel the need to read the fine print in the consent forms as they believed being helpful and trusting their clinical team was enough assurance for them to participate.
Patients often trust their clinical teams and understand the rigorous ethical review processes involved in studies. Some people hesitated to join a study that wasn't backed by a trusted institution or
Patients, on the whole, expressed satisfaction with the process and showed a good understanding of genetic diagnoses, however, confusion arose in some cases. For example, a few patients were puzzled by the different technologies used in research.
Some patients mentioned being disappointed with long waiting periods and those without detected mutations expressed frustration at not receiving negative results.
Several participants were frustrated about not receiving feedback on the study's findings, and they wished to know if their contribution had any impact. This aspect should be considered in the design of future studies.
Like patients, relatives expressed satisfaction with how they were informed about positive results and felt well-supported by family members and healthcare professionals. Those with some background knowledge in biology found the topic fascinating and unanimously agreed that knowing the risk is preferable to living in uncertainty.
Participants showed a diverse range of perspectives, reflecting the dynamic nature of disease experiences and showing the importance of addressing specific concerns to enhance participant engagement.
This study has provided insights into the reasons why patients and their family members engage in research. We've identified common motivations and barriers yet recognised the uniqueness of each patient, emphasising the need for a personalised approach. The study underscores the importance of PH teams in delivering research, as they have a deep understanding of the patients, enabling honest, clear, and compassionate communication.
Recognising the need to bridge the gap between research and clinical care, research needs to be integrated within the NHS framework. Support from healthcare providers, policymakers, and researchers is crucial for the seamless translation of research findings into clinical practice. .
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