Emphasis Spring 2021

SPRING 2021

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

SLEEP & PH

How to have a restful night

CTEPH

procedures

and us

Real experiences of pulmonary endarterectomy and balloon angioplasty

Plus...

Fundraising news, research updates, Green Leaf Crew, interviews and more

Listening

LINE The new support service from the PHA UK

Ready,

set…shop!

Products to help you spread the word about pulmonary hypertension

www.phocusonlifestyle.org

With you on

your PH journey ✓ Real people, real experiences ✓ Exercise and nutrition ✓ Travelling with PH ✓ Claiming benefits ✓ Emotional wellbeing ✓ Blogs …and much, much more!

Accessible for all!

Content can be read aloud in 99 different languages

NEW AREAS !

Contraceptio n and PH Pregnancy an d PH Intimacy and PH

Hello Welcome to the spring issue of Emphasis.

The world has changed in multiple ways over the last 12 months and despite some ongoing challenges, at the PHA UK we are confident this year is going to be a better one.

T

he rollout of the COVID-19 vaccine has been a huge early success story for 2021 and I’ve felt personally privileged to play a part in administering the vaccine at locations in Sheffield. We should be truly thankful for science and our amazing NHS. On the subject of science, on p17 we bring you the real experiences of people who have undergone pioneering medical procedures for chronic thromboembolic pulmonary hypertension (CTEPH). And on p48 Dr Mark Toshner, a researcher who played an important role in the development of the Oxford / AstraZeneca vaccine, talks about the future of PH drug trials and the concept of a ‘cure’.

Looking after our general wellbeing is just as important as targeted treatment for PH. We know that with or without a pandemic, sleep is often an issue for our community, so we hope our feature on p11 helps you develop some strategies for proper rest.

Our emotional wellbeing has also never been more crucial, which is

why we’ve launched a new support service enabling you to talk through any troubles with an experienced PH specialist and PHA UK team member.

Find out more about our new Listening Line on p16.

By the time the next issue of this magazines comes around, I’m sure the world will look very different again, with more freedom and some of life’s simple pleasures restored. Until then, do continue to look after yourselves and each other.

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

PS. We’d really love to see as many of you as possible get involved in our tea & talk campaign to coincide with National Tea Day on 21st April. Grab a cuppa and find out more on p9.

Looking after our general wellbeing is just as important as targeted treatment for PH

SPRING 2021 emphasis 3

09

tea& ta lk

TEA & TALK

TO G ET

HER

Get #TogetherForPH on National Tea Day in April

14

READY, SET… SHOP! The PHA UK branded goodies available to order now

CONTENTS SPRING 2021 YOUR

DOUBL

E

GOLRDD AWANING

WIN INE !

M AGAZ

CIPR PRide Awards

‘Gold’ for Best Publication 2017 & 2019

This magazine is printed on paper from sustainably managed sources

11

WELCOME TO THE LAND OF NOD How to have better sleep with PH

Living with PH...

10 Taking control of remote healthcare appointments

17

Prepare and get organised with our bespoke planning form

CTEPH procedures and us

Three members share their experiences of pulmonary endarterectomy and balloon angioplasty

31 A life in all its fullness

Andrea Hampshire reflects on a milestone moment for her daughter Abbie

34 Remembering Alero

A personal story of navigating grief

36 Giving back and finding myself again

How volunteering for a special housing project helped Shana reconnect with her old career

41

THE LONGEST WALK

How Sarah overcame her challenges to raise almost £17,000 with her husband

27

GREEN LEAF CREW Our regular pull-out for children with PH

16

LISTENING LINE

The new support service to help you through difficult times

Latest News...

Regular Features...

24 What causes pulmonary arterial

06 Emphasis Exchange

hypertension?

The study trying to find the answers

32 New publication to help you prepare for benefits assessments

All you need to know in our brand new toolkit

News, updates and what you’ve been saying on social media

22 Fundraising Focus

The wonderful ways you’ve been raising money over the last few months

38 Understanding palliative care

44 Sleep medication & PH

46 The National Audit of PH

48 The Interview

53 Monthly e-updates

56 Win big with the PHA UK lottery

The results of our member survey and what happens next What the audit has revealed in its 11th year Keep up-to-date by signing up for our monthly e-newsletter

Our regular column from consultant pharmacist Hamilton tackles this important subject Meet PH consultant and researcher Dr Mark Toshner A great way to contribute a little money on a regular basis to support our work

SPRING 2021 emphasis 5

ok o b e c a F e t a v i r P !

New rs e r a c d i a p n u r o f group eryone being there for ev on lf se it es id uding those Our charity pr pertension, incl hy y ar on lm pu affected by tion. e with the condi on d ve lo a r fo who care p for patients ate Facebook grou iv pr ng vi ri th a We have st for unpaid a second group ju up t se ve e’ w w advice and no to others, share at ch to e ac sp without carers. It’s a safe understanding d an t or pp su om and benefit fr e journey. those on the sam judgment - from oup official carers gr Search PHA UK in. on Facebook to jo

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Prestigious pa for the PHA UKnel appointment

We're delighted to have been acce pted onto the Eu Lung Foundation ropean (ELF) Patient Adv isory Panel.

This means we w ill be representi ng the voices of pulmonary hype the UK rtension commun ity on a European – supporting the level ELF in their missi on to improve lu and advance diag ng health nosis, treatment and care.

You can find abou t more about the ELF by visiting www .europeanlung. org

SPRING 2021 emphasis 6

We take your ously privacy very seri ate so both our priv have Facebook groups g in a strict screen process to ensure to the those admitted ine nu group have ge .

PH connections to

Post-vaccinecial smiles on so

y see so many happ It’s been great to shared on social post-vaccine pics ths. st couple of mon media over the la w of your smiley Here are just a fe n following your ke ta y dl ou pr s, snap enfolk – we know COVID-19 jabs. M re urs too, but you’ you have had yo to y when it comes obviously more sh the selfies ;-)

follow u Remember to LHAUK Facebook @ PU uk Twitter @pha_ ha_uk_insta Instagram @p A ORG UK YouTube @ PH

SPRING 2021 emphasis 7

s...

Emphasis exchange

Organ donation law changes in Scotland Scotland has The law around organ donation in , following in now changed to an ‘opt-out’ system d. the footsteps of Wales and Englan

21 in

2021

lebrating The PHA UK is ce is year a big milestone th !

– our 21st birthday s changed A huge amount ha n of our since the inceptio e year 2000 charity back in th king our and we’ll be mar e next few milestone over th s ways. There months in variou opportunities will be plenty of lved too. for you to get invo ldn’t be here After all, we wou ort of our without the supp 0 of you ! members - all 4,50 rward to We’re looking fo coming of celebrating our age with you.

aged 16 years Under the new system, if all adults er they want and over have not confirmed wheth red to be willing to be a donor, they will be conside when they die, to donate their organs and tissue changes came unless they choose to opt out. The into effect on 26th March. you want to You can still choose whether or not istering your be an organ and tissue donor by reg r faith, beliefs decision and telling your family. You ed. and culture will always be respect

at Find out more about the changes g www.organdonationscotland.or

The law on organ and tissue is donation changing

Scotland rch 2021, From 26 Ma t out system. op an ve ha ll wi tion tissue dona Organ and cision personal de remains a e. oic ch a ve and you ha oklet read this bo So please explains: carefully. It ging law is chan • How the you aw affects • How the l ve oices you ha • What ch ord your • How to rec cision de donation ppens when nor • What ha comes a do someone be

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From 26 th organ an of March the law arou d tissu e dona nd in Scot tion is land. changin g If you’r e aged 16 or ov conside er, you red as will be a possibl you die e dono , unless r when you op you decid t ou e, pleas e let fam t. Whatever know yo ily and frie ur decis ion, so ensure nds they ca it’s hono n ured.

For mo re abou t the law and your change choices organd , visit on or call 03 at ionscotland .org 00 303 20 94.

ow n put a bit nch plans. In the last issue of a dow ner on of this magazi new venture our podcast but not being ne, we told you able to get tog a ll episodes to en about our ether stopped able us to lau us recording n nch EmPH Asi ew s on air proper Rest assured th ly. e ones w

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SPRING 2021 emphasis 8

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#TogetherForPH

lk tea&tta h er toge

s National rk a m t 1s 2 il r p A et together Tea Day so let’s g a and chat. for a virtual cupp

The last year has been lonely for many, and we’ve all missed being with friends and family. A cup of tea has always been a good old British way of bringing people together and with technology making it easier to meet up at a distance, let’s make the most of the opportunity to connect.

Maybe you haven’t spoken to a friend or family member for a while? Schedule a chat with a cuppa on 21st April and let’s get talking.

Fancy turning it into a fundraiser?

A tea & talk party is a brilliant way of raising some money for our charity with friends.

Simply set up a chat using your favourite virtual platform, grab a drink, and ask your guests for an entry fee – whatever you and they can afford.

Donations can be made via text, telephone, bank transfer, or online (see www.phauk.org for the full details.)

Tea not your thing? Go continental with coffee or get glamorous with cocktails!

Perhaps you could even hold a COVID-secure tea and bake sale at work or school – depending on restrictions at the time, of course. Please contact us for PHA UK branded bunting, cake flags and price cards.

tea& ta lk TO G ET

HER

Show off your favourite mug !

Take a selfie on National Tea Day 21st April and share it on social media with #TogetherForPH Could you donate the cost of a cuppa to help support our charity at the same time?

Text Text TEATALK 2 TEATALK 5 to donate £2 to donate £5

Send your text to 70085.

Once you’ve posted your selfie and made your donation, why not tag a friend and ask them to do the same?

We’ve also got a Facebook frame or you to add your profile pic for the day. Search for it on Facebook or find the link at www.phauk.org

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SPRING 2021 emphasis 9

Making the most of your

FREE !

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

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

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

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Sleep u PH

Welcome

Nod to the land of

Sleep is vital for wellbeing, but we know that if you have pulmonary hypertension, it can sometimes be a problem. The Sleep Council’s Lisa Artis told Emphasis: “A good night’s rest is essential to a healthy lifestyle - protecting you physically and mentally as well as boosting your quality of living. Just one night of interrupted sleep negatively affects mood, attention span and cognitive ability.” The consequences of not getting enough, she said, are serious.

“Each hour of sleep lost per night is associated with a temporary loss of one IQ point and chronic sleep debt can have a seriously damaging effect on our mental and physical health.”

If sleep is a struggle, try not to despair. Turn the page for tips, advice and myth busters to help you nod off and stay there >>> SPRING 2021 emphasis 11

our advice for a good night's

sleep

Turn your bedroom into a sanctuary.

A messy room means a messy mind.

Putting clothes away, tidying your bedside table, removing dirty cups and keeping cosmetics in the bathroom instead will create space, and in turn, a feeling of calm.

Blackout blinds or curtains can be a life-saver during the summer months.

And the temperature of your room is really important too. The Sleep Council recommends a cool temperature of around 16-18 C (60-65 F).

Decorate your room in soft, muted tones and add special touches such as family photographs or plants to make the space welcoming and personal to you.

MYTH: You can ‘catch up’ on sleep by napping

FACT: Daytime napping isn’t a substitute for proper night-time sleep. Firstly, you can’t get the same quality in short bursts, and secondly, it then makes it much harder to fall asleep at night.

If fatigue means you need to nap, keep it to less than 30 minutes and take it before early afternoon.

SPRING 2021 emphasis 12

Experiment with ear plugs.

If you frequently wake in the night, it may be due to noises that you can’t do much about. Creaking pipes, a snoring partner, heavy rain or pets padding around the room can all make you stir, especially if you’re not in a deep sleep.

There are all sorts of ear plugs available now, from cheap disposable ones to premium reusable silicone ones. Ask your pharmacist if you’re not sure which to go for.

Keep technology away from the bedroom.

That means TVs, laptops, tablets and games consoles - and ideally, your mobile phone too!

If you must use your phone or tablet, use the blue light filter option (usually available via ‘settings’). This decreases the amount of sleep-hormone-zapping blue light displayed on the screen of the device.

If you normally use your phone as an alarm, use a traditional alarm clock instead, or place your phone outside of the room (this has the added benefit of ensuring you get up as soon as your alarm goes off!)

Empty your head.

If stress or worry is making your mind race at bedtime, writing things

MYTH: Alcohol will help me sleep

FACT: It’s sedative effects may help you fall asleep, but it makes it harder to stay there, as the liver works so hard to metabolise the alcohol. Alcohol is a diuretic, meaning you’ll probably wake up in the night needing the loo. Plus, excessive alcohol consumption means you spend more time in deep sleep and less time in Rapid Eye Movement (REM) sleep - which is why you can feel so exhausted after drinking, even if you’ve been in bed a long time. down is a simple but highly effective tactic. Keep a pen and paper by your bed and make a list of what’s troubling you, or what you need to do the next day, and free up that space in your head.

Reading a book can also help you switch off by providing escapism without a screen.

Be careful with caffeine.

Caffeine is a stimulant, which means it promotes alertness - the opposite of sleep!

Avoiding coffee, energy drinks, tea and coca cola (all of which contain

What works for you?

MYTH: Everyone needs eight hours a night

FACT: Most guidelines state that adults need seven to nine hours of sleep each night, but the truth is there is no magic number. Everyone is different; you may feel fine on seven, while someone else needs ten to feel functional. However, less than six hours a night, on a regular basis, is thought to be unhealthy. caffeine) completely is the best idea, but realistically, limiting them to before noon will still make a difference.

Hot drinks can still be enjoyed in the evening but stick to herbal / fruit teas or hot chocolate if you can. Caffeinefree coffee (really!) and teabags are becoming more widely available, so you can get the taste without the stimulation.

Move more to sleep more.

Regular exercise (within your limitations) has been proven to make a difference to sleep patterns.

It helps release any pent-up tension, making you more relaxed, and lowers your body temperature too - which promotes better sleep.

The jury’s still out on the best time to exercise, so it’s worth experimenting to see what works for you.

We asked our social media community for their top sleep tips...

Linda M: "I find washing my hands and face with mint liquid soap helps me sleep."

Sarah L: "An audio book helps me get to sleep and my room has to be as pitch black as possible.

Even lights on my TV box can annoy me.

When I get up in the night, for a while I would tap my phone to check the time, but I try not to do that anymore."

Robert O: "A bottle of water by the bed helps so I don’t have to get out of bed for a drink."

Joanna K: "A bath before bed helps me relax.

And clean sheets and fresh pyjamas always make bedtime more inviting!"

Ian B: "My wife uses an app called ‘Insight Timer’ which has various meditations and calming music. She prefers the stories on it to get to sleep."

Gillian H: "Plenty of fresh air helps me sleep.'

Elizabeth F: "Counting backwards from 1000 helps me sleep as it takes concentration so distracts me from racing thoughts. I don’t think I’ve ever got down to below 500!"

MYTH: Your body gets used to sleep deprivation

FACT: You might feel like your daytime drowsiness stabilises after a few nights of reduced sleep, but the reality is that your sleep deprivation will continue to affect your metabolism, immune system, hormone production, cardiovascular system and mental health.

Turn to p44 for consultant pharmacist Neil Hamilton’s advice on over-the-counter sleep medication.

SPRING 2021 emphasis 13

, y d a e R

Free P& P on all it e m s !

! p o h s … t se

Our PHA UK branded merchandise d is practical as well as stylish, an r shopping with us helps support ou too. charity and raise awareness of PH to order online All of these products are available If you’d prefer at www.phauk.org with free P&P. l 01709 761450. to order them via phone, please cal

s Face covering fe with these sa rs e th o p e e K randed face high-quality b ilable coverings, ava o. in packs of tw • 100% cotton d • Triple-layere

d • Option to ad r your own filte r • Nose frame fo comfort and to prevent steaming of glasses

SPRING 2021 emphasis 14

£ 10

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Car window stickers

These self-clinging stick ers are designed to raise awar eness of PH as an invisible illness an d help make things a little ea sier when parking in disabled ba ys.

Don’t have a car? These eyecatching stickers will lo ok just as good in a house win dow!

(These stickers are self-clinging, so there’s no need to peel the back off )

£5

each

Thermal mugs insulated hot drinks

l mugs keep These cheerfu are cold – so they s k n ri d ld co hot and seasons! perfect for all asher • 350ml, dishw e safe and microwav • BPA free UK • Made in the -on lid • Handy screw p collar and rubber-gri

l a m r e h T bottles

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NEW! £ 16.99

ld hot or co s k n i r d Keep le, se reusab e h t h t i w tainless s d e l l a w doubletles. steel bot -free ium leak the m e r p a ’t spill in n With o w y e deal lid, th hey are i t d screw-top n a – your bag t out and s l i h car or in w d e t ng hydra p. for keepi warms u r e h t a e the w about as ua, royal q a , k c a l sh b e rom styli f e s ach bottl o E o . h d C e r r o ky pink, ives blue, dus d and arr i u q i l f o ml too. holds 500 vely gift o l a t i g a kin boxed, m

each

Portable electric fan

Using an electric fan ca n help relieve breathless ness, particularly in warm w eather, and this compact produc t fits easily in pockets or hand bags.

Batteries are included.

£ 3.9 each 9 SPRING 2021 emphasis 15

Our new Listening service to help LINE you through difficult times

PHA UK

We all need a listening ear, and our new 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.

This free, confidential* support service is available to all PHA UK members and their family and friends.

This is not a counselling or therapy service, but a chance to offload your worries, or talk through any concerns that are troubling you. Perhaps you want to discuss something without worrying your family? Or you feel you have no-one to talk to? Sometimes simply talking things through with someone completely impartial can take a weight off your shoulders or help you work things out in your mind.

Weekly appointments last for 45 minutes and are available in blocks of six. You can use the time to talk through anything that is troubling you and we can also signpost you to further support, for example via your local NHS mental health services, if required.

Please note this service is not intended to replace clinical advice from your specialist centre team. *Nothing discussed during the calls will be shared without the express permission of the service user. The only exception is where we may have concerns about someone’s safety.

SPRING 2021 emphasis 16

WE'RE HERE

TO HELP

Your listening ear...

The Listening Line is manned by Paul Sephton, a PHA UK team member and former specialist PH nurse. Paul has worked in the field of PH for 15 years and is a skilled and patient listener, with many years of experience of helping people with their worries. Paul is not a trained counsellor, so these sessions do not replace specialist mental health support.

However, Paul has already helped many members in this way and the positive feedback has prompted us to formalise the support service and promote it to all.

Find out more or request an appointment by emailing listeningline@phauk.org or calling 01709 761450

Image courtesy of Royal Papworth Hospital

CTEPH procedures and us

Pulmonary endarterectomy and balloon pulmonary angioplasty help hundreds of patients with chronic thromboembolic pulmonary hypertension (CTEPH) every year.

Here, three PHA UK members share their very different experiences of the procedures, and the journeys that carried them there. >>> SPRING 2021 emphasis 17

“I was much more relaxed about everything once the decision to have surgery had been made” David Whittington is 77 and lives in Loughborough. He underwent pulmonary endarterectomy surgery in November last year.

My breathlessness began in 2018 and it was thought that it was a side effect of my blood pressure medication. But in November 2019 I experienced a near collapse due to low oxygen levels and my wife took me immediately to my GP, who arranged an emergency A&E admission to my local hospital in Leicester.

Various tests identified a pulmonary embolism close to the right side of my heart, causing it to dilate to three times its expected size due to the strain that it was under to circulate oxygenated blood.

I was treated with blood thinners and advised to isolate until I visited a respiratory specialist again. Whilst waiting, at the beginning of 2020 I became aware that my weight had increased by 16kg (due to water retention) and I ended up in hospital again, where I received an intravenous medicinal diarrhetic. At this point I was referred to Royal Papworth Hospital and just a couple of weeks later, the country went into its first lockdown. I was already isolating, and now I was shielding too.

A few months later, in September 2020, I visited Royal Papworth for a right heart catheterisation, which resulted in my diagnosis of CTEPH. I began medication and made a subsequent visit for a coronary angiogram in October, which showed no problems with cholesterol.

I had already been made aware that pulmonary endarterectomy surgery may be required, and on 6th October BBC2 broadcast an episode of ‘Surgeons: At the Edge of Life’ which showed this very operation being carried out. This contained a lot of detail and I found it very reassuring that the team had the experience and commitment.

Watching the programme reassured me that it would be the best course of action; therefore, I was prepared to proceed with the operation if it was to be suggested.

SPRING 2021 emphasis 18

Shortly afterwards, it was. The operation was discussed in detail with me, and I was made aware of associated risks and how long it may be (nine hours). I was happy to complete the form giving my agreement for the treatment.

I am very fortunate to be supported by my family and friends, as well as a very caring church family who have remained in contact throughout.

My own strength of faith also contributed to convince me that I had to trust the advice and co-operate with the doctors and medical staff with whom I had consultations.

Plus, I felt I had two choices – stay as I was and continue experiencing breathlessness, or accept the operation

with at least a 93 per cent chance of success. I felt I had little option and I was much more relaxed about everything once the decision had been made.

I had the surgery on 20th November 2020 and it was a success. Unfortunately, due to COVID-19 lockdown restrictions and living 100 miles from Cambridge, I was unable to have any visitors.

Now, seven weeks after the operation, my breathing has markedly improved and I am able to walk up and down stairs again - whereas previously I was using a stair lift that had been installed when my mother was living with us.

I am feeling very positive that I will be able to resume swimming and jogging, as well as driving my 53-year-old MGC

David (second from right) with family at his granddaughter's graduation

GT in the not-so-distant future.

I am also looking forward to attending church and resuming my governor duties at my local primary school. And - when COVID-19 restrictions allow - I’m looking forward to meeting up with the many people I haven’t seen for over a year.

I hope that anyone reading this might be inspired by the journey I have been on, most of which has been beyond my personal control!

Despite all the burdens placed upon NHS staff, the attention and treatment by every member of staff - from receptionists through to ward orderlies, consultants and surgeons - has been exceptional. I wanted or needed for nothing, and I cannot praise them highly enough.

Given all that has happened, I did my best to co-operate with all that was asked of me and I am sure that maintaining this attitude with the staff made the whole experience easier to tolerate.

About pulmonary endarterectomy surgery A pulmonary endarterectomy (also known as PEA or PTE) is a complex surgical operation in which the blood vessels of the lungs are cleared of clot and scar material.

Royal Papworth is the only centre in the UK that offers the surgery, and it has performed over 2000 operations since 1996.

Not everyone with CTEPH will be suitable for this operation, and a number of additional investigations must be performed in order to assess the appropriateness of surgery and the level of risk involved.

Shelly with husband Phil and their dog Rambo

“Watching my operation felt overwhelming” Shelly Rouney had her pulmonary endarterectomy filmed by the BBC for ‘Surgeons: At The Edge of Life’ and it was shown on primetime television in October last year. In an interview conducted in January, she reflects on the unusual experience and the health challenges she has faced with her husband.

My operation was in December 2019 so it was almost a year until it was shown on TV, and it was only then that my family realised how serious it was.

When I first got the call to ask if I would be happy with the filming, I said yes straight away, but I then started to wonder what I had done! It was daunting at first, but the film crew were absolutely brilliant, I couldn’t have asked for a better bunch. They were lovely.

They filmed in our home a few times before the operation and they were so understanding if I got upset or anything.

They followed my journey all the way through, filming me in hospital two or three times in the run-up to the surgery. They did want to film me again afterwards, to show my recovery, but COVID-19 put a stop to it. We had a video chat instead and I sent them photos.

I was happy with how it all turned

out when I watched it on TV, but my husband Phil and I still cried all the way through. It was overwhelming, and to this day, I don’t think my mum has watched it.

Phil was interviewed for the programme too, and we had people recognise us out shopping, asking ‘was it you two on the telly?’

My operation was brought forward because I was diagnosed with breast cancer a few months after I was diagnosed with CTEPH. I was told I wouldn’t survive the mastectomy operation unless I had the pulmonary endarterectomy first, which is why I ended up having the surgery over Christmas. Two months later, I had my right breast removed.

I was in hospital for about two weeks for the endarterectomy and left on New Year’s Eve. >>>

SPRING 2021 emphasis 19

Being in hospital on Christmas Day was odd, but the staff were so good. They even got us all presents.

Phil stayed with his nephew while I was in hospital as it was closer, but it was still 100 miles each way, so he only saw me in hospital a couple of times. It was hard being away from him so much.

Unbelievably, he was taken poorly

himself whilst I was in hospital and the symptoms turned out to be cancer of the oesophagus. I don’t think a lot of people can believe what can happen in the space of a few months. But we have got each other through it all.

My recovery from the PTE went well.

All the clots were cleared, but I do have right side heart failure. And I’m having

a lot of problems as a result of the breast cancer.

But me and Phil stay positive together, knowing that I am going to live longer, whereas before it was very different.

And Phil’s cancer is now in remission, so we know there are better times ahead.

Pictured here and on p17: Shelly's operation was shown on BBC2

Shelly’s pulmonary endarterectomy was carried out

by Dr David Jenkins, Clinical Director of Surgery at Royal Papworth Hospital. He told Emphasis: “The filming did not interfere with the operation.

We are used to visitors in theatre and I have been filmed before. In addition, the documentary makers were very skilled and many of the cameras were remotely operated so the clinical team were not interrupted.

Dr David Jenkins

SPRING 2021 emphasis 20

I think the programme has helped [to raise awareness of pulmonary hypertension and the pulmonary endarterectomy surgery]. Many of our patients have seen it, and others have contacted us about the surgery subsequent to the programme being aired. The more awareness the better with a rare disease. It may also help raise awareness with other health professionals too.”

“I was really happy when angioplasty was first mentioned” Former nurse Mel Lee is 63 and lives near Peterborough. She had a balloon pulmonary angioplasty procedure in 2019, following years of complex health problems culminating in a CTEPH diagnosis.

I had breast cancer in 2002 and uterine cancer in 2005 and then in 2007, I had a massive pulmonary embolism following a 50th birthday trip to South Africa.

Then in 2009 I had a further breast cancer diagnosis which resulted in mastectomy and implant. This was followed by a reconstruction in 2011.

Unfortunately, that didn’t work, so I was having liposuction to replace the fat lost when I had a recurrence of the multiple bilateral pulmonary embolisms in September 2012. The doctors increased my warfarin target, but no other treatment was considered or advised at that time.

I began to get more breathless in 2015.

I help with a lunch club and I started to find setting the tables difficult. I was getting dizzy, had palpitations and I was sweating in a circle around my head.

My ankles were swelling too, and I kept seeing my GP until finally I was referred to a respiratory consultant. By this point it was 2018 and I couldn’t walk further than ten yards without becoming breathless and dizzy. My extremities were very cold and when I came downstairs in the morning I had to rest before going to the kitchen for breakfast.

I was volunteering as a driver and befriending coordinator for a local charity at the time, and I had to stop driving which I loved as it gave me a reason to get out of the house.

After various tests I was finally diagnosed with CTEPH and given an urgent referral to a specialist centre.

As a nurse, that felt really scary. I had never heard of CTEPH, although I had trained at a hospital where we did cardiothoracic surgery.

I had lots of further tests at Royal Papworth Hospital, and the diagnosis was explained properly to me. They also

explained it to my sons, which was lovely.

But after the first trip to Papworth I came home, wrote Mel Lee my Will and organised my funeral as I couldn’t see there was a way out. So, when the angioplasty was first mentioned, quite early on, I was really happy that there was something that would be able to make me breathe much better.

I started medication and a heart Balloon pulmonary angioplasty catheter was repeated every three months until the pressures were low (BPA) has been available for five enough for me to have the procedure. years and is a minimally invasive I remember when I was having the procedure that can be carried out angioplasty my blood pressure suddenly when pulmonary endarterectomy went back to what it was before all of this. They managed to get the large web is not an option. It is only offered in my pulmonary artery and the two big at Royal Papworth Hospital. ones in my lungs, but unfortunately the small vessels were unable to be fixed.

During the procedure, an I went home the day after the interventional cardiologist angioplasty and felt better quite inserts a very fine wire into blood quickly. It was about three months vessels in the lungs and guides afterwards that I realised I wasn’t having any palpitations or chest pain a tiny balloon into position. The anymore. But I still can’t walk very far balloon is inflated, to around the before becoming breathless. Having size of a pea, for a few seconds lived alone for many years I’ve got used to push the blockage aside and to doing things and then having to sit down afterwards. restore blood flow to the lung If anyone else is in the same position tissue. The balloon is then as I was a few years ago, struggling for a deflated and removed. This can diagnosis, I would say keep going back to be repeated several times in the doctor and insist on being referred to a specialist. My daughter will be doing a different parts of the lung during PhD in making diagnosis easier for CTEPH a single treatment session. patients next year and I feel hugely proud that she might help others in my position.

About balloon pulmonary angioplasty

Read more experiences of CTEPH procedures at www.phocusonlifestyle.org SPRING 2021 emphasis 21

s u c o f CELENG G N I S I A Family Matters R D N U F

Virtual fundraiser!

Swear jar pots pounds

£ 3I0SE0D RA

Imaginative Sheffield charge nurse Ian Todd raised hundreds by setting up a virtual swear jar with his friends.

Ian decided to ‘fine’ any friend who used a swear word during their group chats on WhatsApp, raising £300 during November.

He chose our charity in support of his sister Lily, who has pulmonary hypertension, saying: “My sister is very poorly, and I thought that donating the money to the PHA UK, who have supported our family so much, would be a nice thing to do.”

Ian is pictured with friend Joshua Evers, who was the WhatsApp group member fined the most for swearing. The pair delivered the money in person to the Sheffield Pulmonary Vascular Disease Unit (SPVDU), where PHA UK chair Iain Armstrong works as a nurse consultant.

£ 54E5D RAIS

Thank you so much to everyone who continue s to support our charity wi th their fundraising effo rts, despite more lockdow ns and coronavirus restriction s.

We are so grateful!

Ian Todd with sister Lily and mum Lynda last year In early M a Ian teame rch, d with frien up d Caddick to Josh take on a 48-hour running challenge to even more raise money for our ch arity.

We’ll brin g more on th you is the next is in sue !

Ian Todd and Joshua Evers after delivering the money to the SPVDU

Virtual fundraiser!

Spreading love, not germs

Lockdown didn’t stop Emsworth Sailing Club team brightening up a cold January – they streamed a virtual disco into people’s homes and got them busting moves for money as well as smiles.

The club’s resident DJ Mark Covell (pictured bottom left during the event) provided the tunes via Zoom, with music for the whole family to enjoy. A pre-ordered ‘boogie box’ of buffet goodies was available too, with food designed to fuel the fun.

Mark provided his services for free and donations were invited for both the boogie and the buffet. A total of £1,090 was raised and divided between our charity and Cancer Research UK. The event was held in memory of Will Acres (pictured top left), who had PH and was passionate about music. His mother Lea Acres told us: “The virtual disco was so good and it wonderful to boogie together, even if it was apart in our own homes. Mark played some of Will's tracks too, keeping his spirit alive - which was wonderful to hear.”

SPRING 2019 2021 emphasis 22

*Source: www.charitychoice.co.uk

Thank you to Armagh Homing Pigeon Society in Northern Ireland, who raised £1045 in memory of Ally Whan

Socially distanced support

In 2020, Facebook Fund raisers raised an incredible £16 537 !

Facebook fundraisers are an amazing way to mark your birthday and support our charity at the same time - especially when we can’t get together for celebrations.

You could ask family and friends to donate instead of sending cards, or instead of shouting you a celebratory drink? You can create a fundraiser at any time and for any reason, but you’ll be prompted by the platform when your birthday is approaching. A special shout out goes to Jamie Sinclair who marked his 40th birthday by collecting an amazing £1230 via his Facebook fundraiser recently. He said he wanted to smash the £235 he raised for his 39th – and he certainly did!

The Next plc warehouse at Tong, Bradford, packaged up £500 for us by fundraising throughout 2020 Friends and family of John Locke donated £340, generated via a quiz at The Letter B pub in Peterborough and sponsorship from a planned annual fundraising walk (which was unfortunately cancelled due to lockdown restrictions).

DID YOU KNOW?

93p from every £1 donated to the PHA UK goes directly to support people affected by PH*

©www.theletterb.co.uk

93

NEW

FUNDRAISING PACKS COMING SOON ! £

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We’ll help you get started with posters, sponsor forms, bunting, balloons, donation boxes and lots of information to help you raise awareness of PH at the same time.

In the meantime, we still have plenty of bits and pieces we can send you to help you in your fundraising ventures. Drop us an email at office@phauk.org or call 01709 761450 and we’ll get something in the post.

Turn to p9 to find out how to get involved in our Tea & Talk fundraiser!

lanning. p e ’r u o y t a h w w t us kno Don’t forget to le 01709 761450 n o s u ll ca , rg .o k au Email office @ph r Instagram cebook, Twitter o or tag us on Fa

SPRING 2021 emphasis 23

What causes pulmonary arterial hypertension A major study is trying to find the answers, and you can help them get there.

The National Cohort Study of Idiopathic and Heritable PAH has been running since 2014 and with two years to go, it’s full steam ahead to collect more data to understand what might trigger the disease.

Research study coordinator Carmen Treacy explains…

Over the past 20 years, treatment for PAH has improved but we still don’t have a cure - and there are still many unanswered questions. The study is looking at understanding more about the triggering factors that lead to PAH, including environmental factors, and factors that cause disease progression.

There is a strong genetic susceptibility associated with PAH and the study also aims to observe the impact of having a PAH mutation on the course

of the disease over time. In the future it is hoped that we will understand the disease better and be able to identify patients who will respond well to specific treatments (personalised medicine) and ultimately improve survival times.

The study aims to recruit patients diagnosed with idiopathic and heritable pulmonary arterial hypertension (PAH) in the UK, along with some of their relatives.

This is the largest national longitudinal (multiple visits over time) PAH study and therefore it is very important. We still don’t fully

by people volunteering to take part in research studies that we can learn more about this disease.

The study has been hugely successful in recruiting participants so far and we now have a cohort of over 900 participants with longitudinal samples and data.

Our biobank holds over 60,000 samples which are stored for PAH research as and when required. We have a governance committee who meet quarterly and review applications from researchers for the use of study data and samples.

As a part of the study patients were asked to complete an epidemiology

“It is only by patients volunteering to take part in research studies that we can learn more about this disease” understand the disease and only by collaborating with our colleagues nationally will we be able to learn more. PAH is a rare disease so we need as many people as possible to participate so that we may collate the data and samples we require to carry out our research. It is only

questionnaire to capture exposure to any toxic gases or pollutants. This has been published and it is the first time that an environmental factor such as the proximity to a high traffic area has been shown to be related to

Carmen Treacy SPRING 2021 emphasis 24

Further

research

disease severity at diagnosis. You can read the published paper here: www.pubmed.ncbi.nlm.nih.gov/30923185/ The cohort study has been running since 2014 but it is constantly evolving as we identify factors that may affect PAH. We are now looking at remote follow-up of our relatives due to the COVID-19 pandemic but also to assess ‘real life’ data of our patients over a period of time, rather than at a one-off clinic visit. In the future, remote exercise monitoring may replace tests such as the six minute walk test and give us more reliable data.

We have two more years to run with our grant from the British Heart Foundation. Our current goal is to recruit as many newly diagnosed patients and relatives as possible and those who have heritable PAH. Recruitment of relatives of patients is particularly important so that we can learn more about the mutations associated with PAH and their effect on disease manifestation and progression.

Patients who volunteer for the study will be seen at their specialist centre and invited to sign a consent form. We will then take some study bloods along with their clinic bloods and they will be asked to complete an epidemiology questionnaire with the nurse. They will be followed up by the study team after approximately one year and then three years, during a routine clinical appointment. There are no extra visits required, and the visits and follow-ups may be remote depending on the COVID-19 pandemic.

Relatives who volunteer for the study will be invited to attend their closest specialist centre and travel costs will be reimbursed. They will have some blood taken and some medical tests will be performed. They will then be followed up annually until the end of the study.

If your specialist centre is unable to see you due to the COVID-19 pandemic, recruitment and follow-ups will be conducted remotely by telephone. You may be asked to provide a saliva sample for DNA analysis, which can be posted back to us.

As the study progresses you may be asked to visit the centre to have some medical tests such as an echocardiogram (an ultrasound of your heart).

It is really important that people take part in research like this as without people volunteering, we will never be able to learn more about PAH and improve outcomes for our patients and future patients.

To find out more or volunteer for the study, visit www.ipahcohort.com or speak to your specialist PH team. You can also email cohortcoordination@medschl.cam.ac.uk with any questions.

Pulmonary Arterial Hypertension (PAH): A review of genetic counselling and testing practices in the UK

Over the last year, Carmen has also worked on a master’s project in genomics, part of which involved researching the clinician genetic referral patterns of PAH patients in the UK. This is her overview of this work.

The study found that the majority of clinicians refer patients for both genetic counselling and testing at the same time.

Patients with heritable PAH (where there is a family history of PAH) were referred for genetic counselling much more than idiopathic cases. The main reason for not referring patients for genetic services was due to the patient not wanting it. Factors suggested to increase genetic referrals included access to a genetic counsellor at their centre, and clarification of results and costs involved.

Current european respiratory guidelines from 2015 state that all patients with idiopathic pulmonary arterial hypertension (IPAH) and heritable pulmonary hypertension (HPAH) should be advised about the availability of genetic testing and counselling due to the major genetic contribution of PAH. Clinicians were asked if their genetic referral patterns had changed since the 2015 guidelines were introduced and the study showed that referrals for both IPAH and HPAH increased over the five-year period, but more so for patients with IPAH. The study also showed that genetic referral practices between specialist centres was not uniform in the UK. In August 2020, the National Genomic Test Directory criteria was released, detailing which diseases are eligible for genetic sequencing (determining the order of the four chemical building blocks called ‘bases’ - that make up the DNA molecule).

As this national service becomes integrated it is hoped that PAH genetic referral practices will become more consistent.

In 2020, the PHA UK conducted an online survey which showed people affected by PAH are keen to learn more about genetics and be referred for genetic testing (visit www.phauk.org and search ‘genetics’ to see the results).

It is hoped that as the genetic sequencing service becomes integrated into the NHS, more patients will be offered genetic testing. Genetic testing in PAH is an important part of a patient’s diagnosis. Further knowledge of the genetic background of PAH will enable earlier diagnosis and precision treatment of patients, resulting in better outcomes.

SPRING 2021 emphasis 25

PH &

CHILDREN Published with the help of experts in paediatric PH and families living with the condition, this guide aims to help you understand more about pulmonary hypertension and the care and support available.

FREE GUIDEs Also available...

medikidz Explaining PAH to little ones Last few comic books left!

Produced by the PHA UK

CHILDREN Information

Advice

Support Help Contacts

A comprehensive guide to understanding pulmonary hypertension and children

SPRING 2021 2021 emphasis emphasis 26 28 SPRING

Order your FREE copy of both publications... www.phauk.org office@phauk.org 01709 761450

4

PULL - OUT

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SPRING 2021 emphasis 27

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SPRING 2021 emphasis 28

g n i c u d o Intr t s e w e n r ou t s i n m u l GLC co You’ve probably seen her words an d photos in this mag the past and from the next issue of Emphasis, young Emily Graven stede will be joining Kaylee Mynot as an offi cial Green Leaf Crew columnist. Emily will be giving us a glimpse into her world and sharin g some of her experiences since being diagnose d with PAH at the age of three. There will be som e advice too, and top tips for other youngsters living with PH. To help you get to know her bet ter, here is a factfile she wanted to share

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o g n i b e Springtim All of these pieces of nature are a sign that springtime has arrived! Can you spot any on your next walk, or even in your garden? Tick them off as you go!

■ Daffodils

These bright yellow flowers are always a sign that the weather is getting warmer

■ Frogspawn

Peer into some ponds and see what you can spot

■ A bumblebee

Queen bumblebees hibernate over winter, emerging when the weather warms up

Cut out and take with you !

■ A lamb

This is the Green Leaf Crew’s favourite sign of spring !

■ Crocuses

Look out for varying shades of purple, yellow and white

■ A bird nest You might have to look up into the trees for this one!

SPRING 2021 emphasis 29

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SPRING 2021 emphasis 30

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‘A life in all

its fullness'

Last September, four-year-old Abbie Hampshire finally achieved her dream of starting school.

Her mother Andrea shared her reflections soon afterwards, celebrating a milestone amongst her daughter’s health problems.

Abbie is

four years old and she has pulmonary hypertension, most likely caused by abnormal lung development due to her severe scoliosis. She also has a genetic abnormality, one kidney, ulna dysplasia, leg length difference and physical developmental delay.

She is described by most doctors as ‘complex’. She wears a spinal brace, has a walking frame and a powered wheelchair, takes various medications throughout the day and she has oxygen treatment at night and throughout the day when she is breathless or fatigued.

Life with a child who has both physical and invisible disabilities is hard and feels like a constant fight to help her experience a childhood just the same as her two older brothers, Freddie and Joey.

After her heart catheterisation in 2018, which confirmed she had stage four PH and was in heart failure, her consultant told us to throw everything we could into helping her heart and ultimately her quality of life. This is what we did, with lots of help from all her many consultants and the great PH team at Great Ormond Street.

As her parents, we decided to focus on her being well enough to be able to start school. An exciting and milestone moment for all children and a place we consider a sanctuary away from the difficult scans, blood tests

and procedures that seemed to fill her very young life. There are no masks or nameless people at school and Abbie would be able to thrive in the positive, encouraging and inclusive environment that school could offer her.

However, this involved lots of effort from so many professionals to allow everything to be in place for her. The nursery owner who applied for an Education, Health and Care Plan; all her therapists and consultants who wrote supporting letters; the school Special Educational Needs Coordinator who made sure the staffing, classroom, toilet facilities and equipment was organised, ordered and ready; and the whole class team who attended every meeting and carefully listened, taking on board all the medical training required to meet her needs at school.

As Abbie’s parents, we felt very fortunate to have such great support and felt we had everyone on board with our wish for her to start school. Then COVID-19 happened and everything had to be put on hold whilst we were in shielding for four months. At this point we thought all hope was gone and that we would need to rethink our options for her.

However, we made it through the storm and our wish for her to start school came true! She is the happiest girl in the world to be able to go to school alongside her brothers, who are equally as proud of her. She attends school part

time and we regularly talk with school regarding how she is coping.

We feel fortunate that we had the determination to keep going and the support from a wide range of professionals, especially at a time of such uncertainty, to make starting school for her possible. She has been accepted into a school that is all about the positives and the great abilities that she does possess. It honestly is refreshing! She loves to sing number songs and takes part in her class Zumba.

She draws me little pictures and she is even ‘reading’ stories to us at bedtime, rather than the other way round.

We know being at school posing some risks to her health during this difficult time, but we also know that for Abbie, the experiences and education she will miss by not being at school outweigh the risks that can be controlled by a detailed risk assessment in place at school.

It really does take a village to raise a child and we will never forget the effort and support, but most importantly the joyful look on Abbie’s face when we pick her up from school.

She truly is living life in all its fullness and we are so happy for her.

Andrea wrote this in October 2020, when children were allowed back to school following the summer holidays.

SPRING 2021 emphasis 31

HELP TO PREPARE FOR YOUR

benefits assessment

FREE! NEW RESOURCE aims to give you the tools, information and understanding to be totally ready when it counts. ✓ Unpicking the assessment process – who, what, why and when ✓ Evidence, key phrases and what you should draw attention to ✓ The role of carers / loved ones in the process ✓ Checklists and prep forms ✓ Space to record your own notes, prompts and reminders

TOTALLY

FREE !

Order our new Benefits Assessments Toolkit at

www.phauk.org or call 01709 761450

Unsure what financial support you might be entitled to?

Check out our benefits calculator and online grants search tool, in partnership with Turn2us, at www.phauk.org Here, you’ll also find our series of online guides to help you through the process of applying for benefits.

the PHA UK we strongly believe everyone should be given the support they are entitled to.

“AtHaving PH is not your fault, and it can make life challenging, so the process of accessing the benefits you are entitled to shouldn’t compound that situation. It’s important to remember that you’re not on your own; we’re here to help you fight your corner.

Shaun Clayton, Director of Membership Support, PHA UK SPRING 2021 emphasis 32

”

“ We knew everything about each other.

Apart from this.”

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

Talk to your loved ones about organ donation.

Visit organdonation.nhs.uk

Family Matters

REMEMBERING ALERO Clive Smith lost his wife to PH at the end of last year and wrote this piece just a few weeks later. Keen to help others know they are not alone in grief, here he shares his experiences of Alero’s illness and how he said goodbye.

A

lero and I met online in 2009 and married six years later. I loved her for so many reasons.

Yes, she was stunningly beautiful on the outside, but she was beautiful inside as well. She was loyal, hardworking, funny, interesting, kind, and selfless - but she did not pander to whims. She was tough but understanding. She was the most introverted/ extroverted person I have ever known. One moment so gregarious, the next so shy. Alero could be demure and sensitive but rarely showed the latter part to anyone other than me.

I will miss her terribly for the rest of my life, but I have the fondest memories to cling to and will try my best to mirror her outlook and well-meaning actions to all.

Alero was diagnosed with PH in August 2018 but her symptoms were evident a few months before that. At first, I was relieved it wasn’t lung cancer (as that was initially suspected) but then we were both in denial for a while.

“

The hospital team did their best to support me

”

I tried hard to support Alero but as she was so selfless, she didn’t want negativity (I am naturally pessimistic, and she was optimistic – so we were sort of the wrong way around).

However, I did everything I felt that I could for Alero. She felt she was often ‘putting me out’ but regardless of the things you should do for your partner if they are ill, I did them out of my total and undying love - rather than an honourable duty.

Alero died on 9th November 2020 and the hospital team did their best to support me. I had some help from my local mental

SPRING 2021 emphasis 34 SPRING WINTER2021 2019emphasis emphasis34 22

health team and was prescribed tablets for anxiety, depression and sleep deprivation, but I stopped taking them as I felt they were suppressing my grief and leaving me numb.

“

I will try my best to mirror her outlook and well-meaning actions to all

”

We held a two-hour Celebration of Life for Alero, that only 15 people were allowed to attend due to COVID-19 restrictions.

As a surprise, her niece played Bob Dylan’s ‘Make You Feel My Love’ for me on the saxophone, which was simply beautiful.

The funeral was held later, with only 30 people allowed to attend, and no wake was able to take place afterwards.

If I could speak to Alero now, I would say ‘I love you’, which is what I said every day. We were both middle-aged, but we would always be sending the pulsating red heart emoji on WhatsApp to each other.

I am thoroughly heartbroken, and I’ll do everything I can to find a cure for pulmonary hypertension - whatever that may be.

A tribute fund set up in memory of Alero raised over £2,300 for the PHA UK. We are very grateful for this kind support from her family and friends, and to Clive for sharing his story so honestly.

Clive is keen to connect with others who have experienced loss due to pulmonary hypertension. If you would like to contact him, please email media@phauk.org and we will pass your message on.

SPRING 2021 emphasis 35

Giving back and

f l e s y m g fi nd i n

When Shana Hindle was diagnosed with PH, she had no choice but to take medical retirement from the career she loved. Then a chance Facebook post led to her volunteering her skills to open a supported housing complex for homeless veterans in Lancashire – a role which has helped her in unexpected ways. Here, Shana takes up the tale.

My

illness came completely out of the blue. I was diagnosed with PAH in 2015 and at the time I was head of supportive housing for a housing association.

I struggled on for 18 months, but it was a demanding job, and it was making me worse, so I took medical retirement.

I had been there for 17 years, working my way up, so it was a hard decision.

I always say I’m sick in body, but not in mind. Going from a career focused on problem-solving to doing very little was hard, and I missed helping people.

I felt a bit useless really.

So, I started volunteering my time to help people in need with their benefits applications and that kept me busy for a while. Former colleagues and work contacts would pass people my way who needed help, and I was happy to do it for free.

Then in June last year, shortly after shielding restrictions were eased,

SPRING 2021 emphasis 36

I spotted a post on Facebook advertising a new support service for homeless veterans in Burnley - a supported housing complex, managed by a community interest company called Healthier Heroes. At the bottom it gave the address - Elizabeth Street - and I realised it was being opened in the same building I managed in my former life, which felt like fate.

“I always say I’m sick in body, but not in mind” I decided to give them a ring and see if they needed any help with referrals, signposting, and partnerships, and it all went from there. Initial conversations evolved into me taking on a volunteer director role and I now do about three

days a week for them.

The work involves looking after ‘behind-the-scenes’ things like funding, partnerships, policies and procedures, staffing and financial administration support.

I helped bring in new volunteers, and pulled favours from previous colleagues, to help the team turn an abandoned building into a 22-bed housing complex, Bancroft House, which opened in December.

The role was office-based until shielding returned but luckily all the work can be done from home.

Being back in an office environment was a bit overwhelming at first as it was so similar to what I did before. But the passion that Andy and Rio Powell, the directors of Healthier Heroes, showed for the service made me sure I was doing the right thing. It’s been a real partnership with them, whose idea it all was.

The beds at Bancroft House are always full, which shows how much the need

was there. We allow the veterans to bring pets with them too, so as well as people we have a chihuahua, a west highland terrier, a cat and an albino hedgehog.

The complex was officially opened at a small event in December where Andy gave a speech thanking me for my help which made me cry. It was so nice to be acknowledged that way.

It’s been a two-way process; we’ve both benefitted greatly from the partnership and I think I’ve made new friends for life through this.

“I’m more than just my illness” The project has also helped me reconnect with old colleagues and friends from work, and even though we can’t meet up because of coronavirus restrictions, it’s great to be able to interact in a professional capacity.

Realising I still have something to give has really helped my self-worth. If I felt I was well enough to take on a full-time job I’d go back to doing that, with full-time earnings, but I like the flexibility of volunteering. If I was employed, I’d feel guilty for taking time off sick and letting people down. This takes the pressure off.

Through my volunteering, I’ve learned I still have limits.

If I do too much on the bounce, I do get really tired. But I’ve still got it. I’ve still got that thought process, problem solving, crisis management – it might be at a slower pace, but it’s still there.

Despite the negativity of PH, there is still something positive I can give. I’m more than just my illness.

“Realising I still have something to give has really helped my self-worth”

Burnley-based Bancrof t House is ope rated by Healthier Heroes, a community inte rest company (CIC) dedicated to suppor ting mili tary veterans and NHS staf f in need.

Find out more at www.healthierher oes.co.uk @HealthierHeroesCIC @HeroesCIC

Rio and Andy, the founders of Hea lthier Heroes

Seeing that post on Facebook was fate, which I’m a big believer in, along with positive thinking. I used to say I had so much bad luck that if I dropped £1 I’d lose a tenner picking it up, but I keep going.

I hope that sharing this story helps just one person realise what you can still achieve, both with the pandemic going on and with pulmonary hypertension.

Never write yourself off, never think you can’t work towards life goals, and never think you no longer have anything to offer. Never, ever give up.

At Shana's suggestion, Bancrof t Hou se was named after local man Jordan Bancrof t, who was killed in Afghanistan

SPRING 2021 emphasis 37

Total responses

SURVEY 97 RESULTS 18

82

Have PH

have a loved one with PH

Helping people live well with PH:

e r a c e iv t ia ll a p f o The role At the PHA UK, supporting people to live well with pulmonary hypertension is one of our main aims – so we would like to help people access palliative care if they would benefit from it.

At the end of last year, we conducted an online survey to find out what PHA UK members already understand about this type of care, so we can ensure we produce the right resources and support.

There were 97 responses - a good number considering the subject - and they proved there is a real need for work in this area. These findings will inform the development of resources and support systems that improve quality of life for people with PH and their loved ones too.

Thank you to everyone who shared their voice in this survey.

SPRING 2021 emphasis 38

12

%

TALKING ABOUT PALLIATIVE CARE Just 12% have had palliative care discussed with them by their medical team.

Those discussions have been with a mixture of GPs, consultants and nurses.

9

%

ACCESSING PALLIATIVE CARE 9% of respondents said they had accessed palliative care, with 6% of that number accessing it more than a year ago.

This support included assessments, counselling, complementary therapies such as massage, physiotherapy, medication for pain management, home visits and an in-patient stay.

PERCEPTIONS OF PALLIATIVE CARE

We asked which of the following statements respondents thought were true.

These results show there are misconceptions about palliative care which may be preventing some people accessing vital help and support.

60 1 55 9 17

% think palliative care is for anyone with a life-limiting illness.

% think palliative care is for people with cancer only.

✓

✗ ✗ ✗ ✗

% think palliative care is only for those at the end of their lives. % think palliative care means going into a hospice.

% think palliative care is only for the person with the diagnosis.

99

%

INFORMATION NEEDS

99% of respondents said if the PHA UK produced information and resources about palliative care and its role in supporting people with PH they would find this useful or very useful.

WHAT NEXT

?

We will be using the results of this survey to create resources that meet the palliative care information needs of people affected by PH. We will also work to challenge misconceptions about this type of supportive care, and liaise with specialist PH teams to encourage more conversations and support routes – with the ultimate aim of helping patients and loved ones benefit from the care that is available.

Do you have something you’d like to say about palliative care and PH? We would love to hear your thoughts and experiences to help us with our work. Please contact paul@phauk.org or call 01709 761450.

SPRING 2021 emphasis 39

FREE

PUBLICATIONS

Support and advice for every stage of PH

Order online at www.phauk.org

You can also order by calling 01709 761450 or by emailing office@phauk.org

The longest walk Amongst the pain of the pandemic, Sarah Marshall will remember 2020 for something much more positive – completing a walking challenge she never imagined she could do, in a combined effort with her husband that raised almost £17,000 for our charity.

In an extract from her blog, Sarah reflects on seven days she will never forget.

It was the final day of the hardest week of my life. And I was exhausted. My heart was a-twitching, my lungs a-panting, and I felt sick. After seven days of pushing myself to the extreme, my body was screaming for rest.

But I barely noticed. Instead, I had the biggest smile on my face. Pride and astonishment and happiness and relief. Because through the mist I could see my husband Phil cheering me on from the virtual finish line. Amazingly, wonderfully, thankfully, I was about to achieve my impossible.”

Read Sarah's inspiring story overleaf >>> SPRING 2021 emphasis 41

Celebrating completing the challenge

I

n my life before pulmonary hypertension, I loved walking. But all that changed overnight in 2013 - when I awoke to find myself blue, struggling to breath, and barely able to stumble two metres. My lungs were at quarter capacity, my heart in failure, and I was left bedridden almost 24/7 from extreme fatigue. Every time I tried to walk, my body struggled.

It zapped my entire daily energy allowance and left me poorly for days.

Over the last seven years, despite being on powerful medication, doing daily strengthening and cardio exercises, and keeping myself as fit as possible, I’ve never regained enough health to easily walk. But I have improved immensely.

I remember the excitement of walking two metres around my hospital bed, the achievement when I first trudged from lounge to kitchen. Each extra few metres that I am now able to walk, took months of medicine and training, and many poorly recovery days. Consequently, despite trying and trying and trying, I’m reliant upon my mobility scooter whenever I leave the house. Walking is my nemesis.

In September last year, storm clouds rolled into my world. After months of shielding and isolation and missing friends and constant fear, and with no end to the pandemic in sight, life began to look dark and grey. And with many of my usual coping mechanisms off-limits due to the virus, I struggled to prevent the downwards pull. For a while, I was like a lone tree flailing in the winds and the rain and the thunder. At the mercy of the gale. I desperately needed new umbrellas. I needed a routine and exercise and sunshine and something to look forward to. So, I entered a 5km walking event.

It felt like a mad challenge.

An impossible challenge. Walking exhausts me, steals my energy, and makes me ill. My legs go wobbly, my heart works too hard, and my lungs struggle to breathe. In seven years, I’ve never managed further than 490m.

But a little voice inside me was excited to try. If ever there was a time, this was it.

I could take the negatives of the pandemic and turn them into positives. My free diary meant, for once, I had time and energy coins to devote to walking and its repercussions.

SPRING 2021 emphasis 42

With events banned, the race was being run as a virtual one, so I could walk locally, whenever I felt well enough, and spread it over seven days. And thanks to shielding and a summer devoted to gardening, I was stable and well and the healthiest I’d been since becoming ill. Now was the time to try. All the stars were aligned.

But even so, the mountain seemed enormous. 720m for seven days straight was still too much. Too dangerous. I’d never walked that far, nor exercised on consecutive days.

So, for the next month or so, I prioritised training above everything else. A few times a week, I’d get off my scooter in the middle of a dog walk, and slowly saunter across a field. Mud and grass, boggy and dry, hilly and flat. First 400m, then 500m, then 600m. Every time I upped the distance, or walked too fast, or I was already fatigued, my body would go on strike, and I’d end up bed-ridden.

Eventually, a fortnight before the big event, I managed 720m for the first time.

And then a week before, I managed to walk for two consecutive days.

I knew there was a high chance I’d not manage the 5km, but for me it wasn’t the outcome, it was the trying. And what a journey it was.

Race week was the physically hardest seven days of my life. Although we strategically picked routes that were flat and solid underfoot, went out at my optimum time and increased my oxygen level, the walking was bloomin’ tough.

Although I laid down for 23 hours a

Please speak to your PH specialist before taking on a physical challenge like this

day, did absolutely nothing else, got looked after by Phil (on top of full time work and running 100 miles himself!) my wonderful body struggled to recover.

As the days passed, I was just getting increasingly poorly. Every day I’d feel worse than the last. By the second half of the week, even when resting I was breathless and fatigued and almostconstantly nauseous. I couldn’t sleep on my back or side, I had the squits, and even night sweats. And scarily, my heart developed new worrying symptoms.

It was psychologically hard to go out each day, knowing I was making myself ill, possibly causing damage. It turned into a battle between head and heart; health anxiety versus determination.

However, I loved it. I’d seriously missed walking in the countryside.

The mud underfoot, the ridges to step over, the long grass tangled in your boots. The silence, the bird song, the peace. Seeing over hedgerows, surprising wildlife, stepping over branches. Walking hand in hand with Phil, holding Lottie on her lead, looking ‘normal’.

I loved having a challenge, I loved pushing myself, I loved having something to aim for and focus on.

I loved being forced to go out each day, I loved experiencing the mist and the frost, I loved being ‘in’ winter. And I was forever in awe that my own lungs and heart were moving me forward. Each walk may have lasted a mere ten or fifteen minutes, but whether in training or doing the real deal, those moments were always the highlight of my day.

And I was overwhelmed by the showering of love and support. I’d initially been reluctant to share the challenge - I felt embarrassed and vulnerable and exposed. But I’m so glad I did.

The comments and messages I received after each daily update cheered me, gave me confidence, and encouraged me to keep trying.

They were the medicine that made the poorly hours easier; the sunshine after the big bad storm. And best of all, thanks to everyone’s incredible generosity, an unbelievable amount of money was raised for the wonderful PHA UK! This is an extract from Sarah’s blog, which she uses to chronicle her life with PH. Read more at

Sarah's husband Phil joined the challenge by running 100 miles over seven days

Sarah is the most determined person I know

Most of my previous challenges have involved stepping away from everyday life and being entirely single-minded for the time it takes to complete. This time, normal life was carrying on and it was very strange to be constantly switching attention from a daunting running challenge to a business meeting over Zoom.

The logistics of fitting in my running alongside everything else that week was the hardest part. We had to plan a strict schedule for runs, walks and meals and be very disciplined throughout no hitting the snooze button when the alarm went off at 6am every day! If I wasn't running, working, eating or helping Sarah, I was sleeping.

Sarah is the most determined person and has the strongest selfdiscipline of anyone I know, so I knew she would do everything to complete the challenge. I was worried that might mean she pushed herself too far. But we always say that every PH patient is the world-leading expert on their own PH, so I knew she would be able to monitor how she was.

I am enormously proud of Sarah. The determination, strength of character and iron will that she showed to get out each day and push herself a far as she did was incredible.

No matter how tough the situation she faces, Sarah will sit down and plan a way through. She will work out how to find happiness, joy and satisfaction, and then go for it. She is a true inspiration.

Phil’s employer, Barclay’s, kindly matched the couple’s sponsorship – resulting in an incredible total of £16,912.

SPRING 2021 emphasis 43

“Sleeping tablets are a sticking plaster, not a cure"

Medication has its place, but tablets shouldn’t be the first thing you turn to when you’re suffering with Insomnia says consultant pharmacist Neil Hamilton…

The pandemic and poor sleep

As I write this in late February, I am buoyed by the news that over a third of adults in the UK have now received a COVID-19 vaccine. In spite of everything we have all been through during the pandemic, the vaccination programme here has been a fantastic success story to celebrate. The comingtogether of the NHS and an army of volunteers has delivered massvaccination on a scale unmatched anywhere in the world. Providing this rate of progress continues in the

AUTUMN SPRING 2021 2020 emphasis emphasis 44 46

coming months, we can all see light at the end of the tunnel and the gradual return to normal (whatever that will look like!).

The pandemic has affected all of us differently, and the PHA UK’s recent research report ‘Shielded Voices’ gave me a detailed insight into how the PH community in the UK has been impacted. I was involved in national work this time last year to ensure that PH was given due recognition as a serious condition and as such, patients with PH were classed as clinically

extremely vulnerable and advised to shield. Whilst shielding may have prevented some loss of life, there have been inevitable consequences, all of which are described by the findings in this report (which you can view at www.phauk.org).

It came as no surprise to me that shielding and isolation have had a detrimental effect on mental wellbeing and with that, insomnia.

If either goes unmanaged, insomnia affects mood and vice-versa.

Tackling insomnia

According to the NHS Choices website, insomnia is defined as regularly having problems sleeping.

Luckily, although sleep difficulty is problematic if it goes on for a long time, it usually gets better by changing your habits. It is also really common, with most people experiencing it at some point. Our bodies need good quality sleep, but the quantity of sleep a person needs can vary.

Stress, anxiety and depression are the most common causes for insomnia, but other causes include noise, room temperature (maybe too hot or cold), your bed, shift-work, jet-lag, alcohol, caffeine, nicotine or recreational drugs.

Identifying the most likely cause in

When to explore sleep medication

In the unfortunate event that these lifestyle suggestions don’t work, and your insomnia has been going on for several weeks, help is available via your local pharmacy in the form of sleeping tablets. These will not directly address the cause, but lack of sleep will affect your ability to function and cope. As such they may give shortterm relief, but you must be aware of increased drowsiness the next day.

Dependence is a major concern, so I must also be clear that all pharmacists should advise anyone buying sleeping tablets that they are only sold in small packs as a temporary solution.

If you do find yourself needing to seek advice from your local pharmacist, you

“It is important to bear in mind that many herbal remedies will not have undergone clinical trials” your particular case will be the first step to treating the insomnia.

Indeed, insomnia usually does improve if you successfully change your sleeping ‘habits’- and you can read about what to try on page 12 of this magazine. I would strongly advise giving all these a good try before seeking advice from your GP or local pharmacy about other options.

In addition to lifestyle changes, there are a wide variety of ‘nonpharmacological’ options available.

These range from earplugs, noisecancelling headphones and sleep therapy pillow speakers, to devices like Dodow© which uses light to help you fall asleep faster, or air-humidifiers and aromatherapy. With these working in all sorts of ways, it may be a case of trial and error until you find the one that suits you best.

should be asked what other medications you take, so go prepared with your repeat prescription list. Options will comprise of herbal/natural remedies or medications such as Nytol© which cause drowsiness. In general, agents such as Nytol© are likely to be more effective, but I know that some people would prefer to try herbal tablets.

It is important to bear in mind that many herbal remedies will not have undergone clinical trials and will definitely not have been researched in the context of PH or in patients taking other medications. They can, however, still interact with some prescribed medicines and alcohol, causing additional drowsiness. This may be problematic if you plan to work or drive the next day.

The most commonly sold sleep aids such as Nytol© contain an antihistamine medicine called diphenhydramine. It may be cheaper

to ask for this by name rather than the brand name (as with paracetamol vs Panadol©). Older antihistamines, including diphenhydramine and chlorphenamine (also called Piriton©) were developed and found to be very effective for relieving allergic reactions and skin itch. However, they also cause drowsiness.

For insomnia, we use the drowsiness as a ‘beneficial side effect’. A similar example of beneficial side effects familiar to PH patients is sildenafil.

Originally developed as a treatment for high blood pressure and angina, the ‘beneficial side effect’ for men with erectile dysfunction was discovered in early clinical trials.

In the event that you are still struggling with insomnia despite all of these strategies, your GP may refer you to a therapist. GPs now only rarely prescribe sleeping tablets (such as zopiclone or zolpidem, not available at the pharmacy counter) for reasons of dependence.

These two medicines are cleaner and more preferable compared to older tablets such as temazepam. This means they are just as quick to take effect but are cleared from the body more quickly, giving less risk of a ‘hangover’ or drowsiness the next day.

The fact that insomnia is common will not be any consolation if you’re affected. However, I hope this has given some more background, together with up-to-date NHS advice as to how it can be tackled. There is undoubtedly much work being done by large companies into products that will help tackle insomnia. It may seem ironic that this is instead of the pharmacist article almost steers you away from medicinal solutions, but that is the best advice! If all else fails, sleeping tablets are a last resort which may help you put in place some healthier sleep habits. But they are a sticking plaster, not a cure.

.

SUMMER SPRING2020 2021 emphasis 45 47

AL A NNU T REPOR

11 years of the National Audit of Pulmonary Hypertension The latest data shows specialist PH centres are continuing to meet or exceed key targets The UK National Audit of Pulmonary Hypertension (NAPH) is the largest audit of pulmonary hypertension in the world.

The audit was created in 2009 to collect and report on data across all specialist PH centres in the UK, with the aim of improving patient care and informing future service planning.

In previous years, performance against audit standards has been evaluated based on individual centres.

However, due to the challenges of the coronavirus pandemic, this time standards have been evaluated at a national level instead.

At the PHA UK we played an important part in the creation of the audit by funding its first year. Since then, we have continued to provide support and advice.

Every year we bring you the key findings in a clear and understandable way. And this time, we were asked to provide anonymous

SPRING 2021 emphasis 46

Professor David Kiely is lead clinician for the National Audit of PH. He said: The national audit has demonstrated that the UK network of specialist pulmonary hypertension centres continues to provide high quality care. This is despite the impact that the nationwide lockdown in March 2020 had on elective hospital services. The data in this report should provide reassurance to patients and their families.

The continued success of the audit reflects the hard work of many individuals. I would like to thank everyone for submitting data despite many competing pressures, the team at NHS Digital for their flexibility, and our on-going partnership with the UK Patients’ Association (PHA UK), which has ensured that the audit remains relevant at time of increasing change in healthcare provision.

It is reassuring that during a national emergency data quality has remained high and excellent standards of care have been maintained.

patient quotes about some of the areas being audited for the NHS’s own report - another way we ensure your voices are heard.

It’s very encouraging to see that, despite the challenges of the last 12 months, the specialist centres have continued to provide excellent care.

The audit is delivered by NHS Digital and commissioned by NHS England.

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

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

Please be assured that this will not affect your treatment and care in any way.

THE FINDINGS All eight specialist centres in England and Scotland submitted data, with the audit covering the period between 1st April 2019 and 31st March 2020.

The four findings below were... were selected by people with PH as the most important to them and the percentages shown are an average across the centres. The percentage circled is the professionally agreed standard that the centres are expected to meet.

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

96%

90 %

TARG E

T

The full National Audit of Pulmonary Hypertension, including all the findings, is available by visiting www.digital. nhs.uk/pubs/naph11

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

90 %

94%

TARGET

(98 % of patients has an EmPHasis-10 score recorded)

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

9

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

99% 9 4% 5%

TARGE T

95%

TARG E

T

SPRING 2021 emphasis 47

theinterview

“My career has been driven by the slings and arrows of outrageous fortune” DR MARK TOSHNER in conversation with

Mary Ferguson

SPRING 2021 emphasis 48

Dr Mark Toshner is a busy man. With official titles of Director of Translational Biomedical Research and Honorary Consultant at Royal Papworth Hospital NHS Trust, his professional life is dedicated to research and clinical practice.

Scaling a mountain at 22 ignited a passion for pulmonary hypertension research and a case of ‘right place, right time’ saw Mark work on the Oxford/AstraZeneca trial that led to a COVID-19 vaccine.

He chats about his inspirations, what a cure for PH might look like, and the defining trial of his career.

Q. Did you always know that the world of medicine was for you?

A: I knew I wanted to work with people and do something worthwhile, so my decision to go into medicine was informed by my desire to do something that might help. But it was a decision made by a 16-year-old Glasgow schoolboy who didn’t really understand the implications of any of the stuff he was doing! It turned out to be lucky that I picked something I would grow to really love.

Q. Did you find PH, or did PH find you?

A: There is a real story here. I went to university in Aberdeen, where I got into rock climbing and hill walking, and that actually ended up introducing me to pulmonary hypertension.

Because of my interest in mountains, as a junior doctor 20 years ago I got involved in high-altitude research and went off to do some high-altitude physiology research in Bolivia with some friends and colleagues. Whilst up a mountain, we were measuring our own pulmonary artery pressures as part of a Sildenafil trial, and it turned out that with pressures of just under 70 I actually had pulmonary hypertension. At that point, we didn’t quite know what to

do with me, so we ended up giving me Nifedipine (a blood pressure drug) and sending me back down the mountain until I had adjusted to the altitude more.

When I went back up, I still had a very high artery pressure, but this time I didn’t develop high altitude pulmonary edema (HAPE). Possibly, to date, that’s the highest recorded pulmonary artery pressure in a participant at altitude who didn’t have HAPE. So physiologically, I was an outlier.

That sparked my interest in PH, as I was up at altitude, experiencing its symptoms of breathlessness, racing heart and very low oxygen saturations.

When I returned from Bolivia, I became really interested in the cell and molecular biology about why this might be – why I was slightly different than the average person.

I started looking around to see if there was an expert in the UK on the cell physiology of PH, and I found Professor Nick Morrell [now Research Director within the pulmonary hypertension service at Royal Papworth Hospital]. In 2007 I decided to move to Cambridge to do a PhD under his supervision.

So, my career has been driven by the slings and arrows of outrageous fortune. I could never have predicted that climbing sea cliffs in Aberdeen would lead to researching pulmonary hypertension in Cambridge. >>>

SPRING 2021 emphasis 49

“It feels like we are seeing the first trials of the ‘new era’ in PH research and that’s enormously encouraging.”

Q. Given the timings, it sounds like your interest in PH coincided with the establishment of the specialist centre network?

A: Yes, when I went up the mountain in Bolivia, the person I was collaborating with all those years ago was Andy Peacock, who set up the Scottish Pulmonary Vascular Unit (SPVU) at the Golden Jubilee National Hospital.

It gets more interesting still. When I came to Cambridge, I was initially more interested in high-altitude physiology, because I hadn’t really had any exposure to PH as a disease, but because Royal Papworth Hospital had its PH unit, I started realising that this was a really devastating disease that affected people indiscriminately. That’s when my high-altitude interest waned a little, and I started researching PH instead.

So, the journey to PH was twisty - and randomly influenced by my love of mountains! The irony is that I now live in the flattest bit of the country.

Q. What does your job look like now?

A: There are three different parts to it. There’s my clinical job, which is mostly in the PH unit (although I do still do general respiratory clinics).

Then there’s my teaching; I run a master’s course in Translational Biomedical Research at the University of Cambridge, so that’s quite a big time commitment for me.

And of course, my research. My research is mixed in the space between the lab and the patients and covers everything from molecular biology all the way up to clinical trials, with a focus on the latter.

Q. How do you have time to sleep?

A: I have a very understanding wife! There is a lot of work there but I’m lucky because I love all of those parts of my job. And I have an incredibly short attention span, so it suits me very well. The job never gets boring because every day is slightly different.

Q. Aside from the COVID-19 vaccine trial, are there any studies you are particularly proud of?

A: I would have to point to research that I have been lucky to be part of. Right at the start of my career I lucked out and was involved in two very positive studies - including the Sildenafil study in CTEPH. And before Covid, the work that I’ve done with Nick Morrell in the lab has directly led to a new PAH therapy

SPRING 2021 emphasis 50

- Sotatercept - that is about to go into phase three studies (the phase before it gets licensed).

Research often goes in fits and bursts and it can be difficult to judge at any one point whether the stuff you’re doing is important or working, and it can take a decade before you really know. But when it does work, it’s incredibly satisfying because you know you might have helped to improve the lives of your patients.

Q. How do you think drug trials in PH will evolve over the next few years, and what will that mean for patients?

A: The first thing we really need to do is change how we approach trials. We have a lot of therapies now, but we don’t know how combinations affect patients, and we haven’t really started to look at mixing and matching and getting the best combination - until now.

Trials where we try to individualise therapy are going to have to be run differently and be a lot more patient-centred.

The old model of bringing patients in for a catheter six months apart isn’t nice for them, and probably isn’t appropriate for the next round of trials we need to run. So, I’m envisaging we will be trying to embed trials in everyday practice that are much easier for patients to sign up to, and much less onerous for them. We now know we have a set of drugs that are safe, so we don’t need to do extensive safety checks, we just have to figure out what the best way of checking the drugs are working is, without lots of interventions that are not necessarily well set up from the patient’s viewpoint.

It feels like we are seeing the first trials of the ‘new era’ in PH research and that’s enormously encouraging.

“I could never have predicted that climbing sea cliffs in Aberdeen would lead to researching pulmonary hypertension in Cambridge.”

Q. The million-dollar question then; how close are we to finding a cure for PH?

A: The word ‘cure’ is a loaded term. We are aiming for a ‘chronic managed condition’ where we return patients to a normal envelope of activity with drugs that don’t give severe side effects.

It could be comparable to something like HIV, where although there isn’t necessarily a ‘cure’, the outlook for patients has been transformed. We’ve already seen that to some extent in Chronic Thromboembolic Pulmonary Hypertension (CTEPH), where pulmonary endarterectomy (surgery) has unequivocally transformed lives, and balloon angioplasty is now doing the same thing.

But actually, when you look at the hearts and lungs of patients who have undergone these procedures, they are not ‘cured’. What we’ve done is taken the disease down to a level where people can function and have a normal life, and where we prevent the disease progressing.

We’re aiming for a situation in PH where the disease is ‘chronically managed’ and patients can just get on with their lives. It’s unlikely we will find that magic bullet where we give one tablet or one injection and PH goes away, and it’s important to be realistic about that.

Q. Are you saying the aim is for PH to no longer be classed as a ‘life-shortening’ disease?

A: Exactly. The idea would be to get to the point where it doesn’t have an impact on life expectancy, and the symptoms are manageable, and you can have a normal life. Whether you call that a ‘cure’, or whether you call it ‘managing a chronic disease’, is an open question for debate.

Q. Back to you, what has made you proud in your career?

A: It may be too fresh for the moment but having contributed in a small way to the vaccine studies is almost certainly going to be something I look back on. [Mark was Local Investigator in the Oxford/AstraZeneca COVID-19 vaccine study] I feel lucky to have been in the right place at the right time to be able to help, but also pride that I had the skills to be able to do that. On a global scale, it’s hard to compete with that study because it’s so important. It led to many sleepless nights for me and although it’s too early to sit back and reflect on it - we’re still in the midst of a global pandemic - I can see that further down the line I will find it amazing to say I got to help.

“I still see some patients who I got to know in my first studies 15 years ago. It’s incredibly rewarding to have shared that journey with people who know and trust me.” and absolute inability to take no for an answer, she set a PH unit up, pioneered a national service, and has been a driving force behind the pulmonary endarterectomy service.

Nick has shaped and influenced my career. He has been a wonderful person to work with all these years and it has been an honour to have learned from these two world class researchers. I love pulmonary hypertension as a community because with it being so small, we get to know our patients incredibly well. The rewards of that are quite profound and the fact we are set up to look after our patients properly is what motivates me to continue to practice clinical medicine.

I still see some patients who I got to know in my first studies 15 years ago. It’s incredibly rewarding to have shared that journey with people who know and trust me.

Q. Finally, how do you spend the limited time you have away from work?

A: At the moment, I sleep! And feel guilty that I’m not spending enough time with my kids. I have two, aged two and seven. Life outside of work is essentially small children and chaos. I still retain a love of mountains and climbing, although I haven’t had much time for either over the last few years. I’m very family orientated. My wife is from France, so we split our time between her family over there and mine who are spread all around the UK.

Q. What, or who, inspires you in your work?

A: I’m going to be slightly sycophantic - and you can quote me on that – but I’m going to say I’ve been lucky to work with some world class and fabulous mentors. The two most prominent ones are, without a shadow of a doubt, Joanna Pepke-Zaba [Director of the National Pulmonary Vascular Diseases Unit at Royal Papworth Hospital] and Nick Morrell.

Jo is a legend. Through the force of her magnetic personality

SPRING 2021 emphasis 51

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In your summer issue of Emphasis...

The next issue of Emphasis is due out in June and planned content includes: The PHA UK story

Where we came from and why

Post-Traumatic Stress Disorder (PTSD)

When medical episodes have a lasting impact

Cooking with fatigue

Advice for preparing meals with PH Plus, lots more articles, interviews and news…

You can get involved in Emphasis too: Emphasis Reviews - read a book, used an app or seen a film you think other readers may be interested in? Tell us about it!

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We always love to hear from you – contact media@phauk.org with any feedback or ideas.

SPRING 2021 emphasis 53

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