Emphasis Winter 2021

WINTER 2021

YOUR VOICES

Real-life experiences shared PH & the pandemic

Research findings revealed

Plus...

Pharmacy support, celebrating PH Awareness Week, expert interviews, fundraising news and much more

The magazine for supporters of the PHA UK

Hello

2022

What we’re all looking forward to next year

NG WISHIFAMILY H OUR P ERRY

A M MAS & T EW S I R H C YN P P A H YEAR

Hello Welcome to the final Emphasis issue of 2021.

The theme of this issue is #HearOurVoices, matching that of our recent PH Awareness Week. Each year we see more and more support for this campaign, and we are truly grateful to everyone who gets behind it.

PH

may be rare, but our community is united by the desire to raise awareness, and at the PHA UK it’s our privilege to help bring your voices together. You can find out what went on during the first week of November on page 8, and if you flip this magazine over, you’ll find a selection of your snaps.

You also made your voices heard in a very important way earlier this year, by taking part in our research into experiences of the pandemic. Turn to page 16 to see the headline findings and read the full report at www.phauk.org.

Healthcare professionals are a key part of our PH community too, and this issue brings you a fascinating interview with Mr Steven Tsui, Consultant Surgeon at Royal Papworth Hospital. On page 42 he tells us how operating is 'like conducting a symphony', and why kindness is the most important human attribute there is.

We also introduce you to Dr Sadia Quyam, the new(ish!) Consultant in Paediatric Cardiology and Pulmonary Hypertension at Great Ormond Street Children’s Hospital, on page 22.

There has been much in the news about winter pressures on the NHS, and the importance of keeping yourself well over the colder months.

On page 50, PH specialist Neil Hamilton explains how your community pharmacy can help - and you might be surprised by the services available.

I’d like to end by thanking everyone who has contributed to this magazine, and many other aspects of our work, throughout 2021. We are honoured to have such a supportive membership.

Merry Christmas!

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

IT’S OUR PRIVILEGE TO HELP BRING YOUR VOICES TOGETHER

# HEA

o vou r

R

ices

Look out for the orange badge on all of the real-life experiences in this issue WINTER 2021 emphasis 3

08

WHAT A WEEK!

How you got behind PH Awareness Week 2021

12

THE (IM)PATIENT AND THE CARER An honest account of married life with PH

ph + thec pandem

16

The headline findings of our research revealed

CONTENTS WINTER 2021 Emphasis magazine is produced by the PHA UK. This magazine is intended only to provide information and not medical advice on individual health matters. The PHA UK will not be responsible for readers’ actions taken as a result of their interpretation of this magazine. We encourage readers to always discuss their health with their doctors and medical team. Registered Charity Number: 1120756. © PHA UK.

This magazine is printed on paper from sustainably managed sources

Living with PH...

10 Poetry corner 11

Myra Morrison and Anita Jackson put their stories into prose

“I feel like I’ve been given a second chance”

Ros Knight on what having pulmonary endarterectomy surgery has meant to her

14 “I appreciate my health a lot more now” Jess Wescott’s personal experiences of PH

20 Words of wisdom

We asked seven PHA UK members what they would say to someone newly diagnosed with pulmonary hypertension

50 Your pharmacist is there for you this winter Neil Hamilton on how your local pharmacy can help you stay well

k on this issue, Have you got any feedbacon of Emphasis? or ideas for future editi s 01709 761450 Let us know by emailing media@

phauk.org or calling

36

GETTING SMART ABOUT PHYSICAL ACTIVITY How smartwatches can help you to move more with PH

42

THE INTERVIEW

Meet Consultant Surgeon Mr Stephen Tsui

39

“Connie’s illness has not stopped us living life”

Jane Price shares her family’s experiences of PH

More of your stories…

# HEA

R o u vo r

ices

24 Looking back on my PH diagnosis

Alex Porter in conversation with Dr Iain Armstrong

46 In sickness and in health

How Peter Beech supports his wife of 59 years

Regular features...

06 Emphasis Exchange

News, updates and support for loneliness this Christmas

28 Ask the experts

Your questions answered

52 Life after my transplant

31 Green Leaf Crew

56 Finding her brave

45 A window to our world

58 “I want to raise the profile of PH so that

54 Fundraising Focus

Julie Warner reflects on 23 years since her heart and double lung transplant Andrea Hampshire on helping her young daughter make the most of life with her health conditions

others get a quicker diagnosis”

Nerys Jones describes her difficult journey to diagnosis and how she is adjusting to the impact on her life.

Comic strip fun, and columns from Kaylee and Emily, in our special pull-out for children The new regular update from the PHA UK’s Shaun Clayton The wonderful ways you’ve been supporting us over the last few months

WINTER 2021 emphasis 5

Prestigious reco for EmPHAsis-10 gnition

PHA UK Chair Dr Iain Armstrong w as ‘highly commended’ in hi s category of a pr estigious national nursing awards in October.

He was recognised for his work in cr eating the clinical tool EmPHAsis-10 in the ‘Innovation s in your Specialt category of the Ro y’ yal College of Nur sing (RCN) Nursi Awards. ng The virtual cerem ony was hosted by TV favourite Kate Garraway, and sh e shared details of EmPHAsis-10, the quality-of-life m easure used in PH , with a national audience.

Designed as a sim ple questionnair e 11 years ago, it is now used arou nd the world to he lp doctors and nurses understand how people affect ed by pulmonary hypertension are responding to trea tment.

As well as co-foun ding the PHA UK , Ia in also works as a Nurse Consulta nt at the specialis t PH centre withi the Royal Hallam n shire Hospital in Sheffield. Chris Morley, Chief Nur se at Sheffield Te aching Hospitals Foundation Trus N HS t, said : “This com mendation is rich deserved by Iain, ly and is a fantastic recognition of his work and his dedi cation to improvin g the quality of lif for people with pu e lmonary hyperten sion.”

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Your Right to Re hab : An u

pdate on our cam paign

We’d like to say a big thank you to the hundreds of peop le who have pled ge d their support for our campaign to gi ve everyone with PH access to speciali st rehabilitation. At the time of going to print, we have re ceived almost 40 0 support pledges, which proves just how much this m eans to you too.

Having gathered this groundswell of support, we will now make contac t with commissioners an d roll out the next phase of our cam paign in the New Year.

Watch this space!

Summit success for Cindy

brought In the last issue of Emphasis, we dy you news of PHA UK member Cin s up a Regalado’s plans to hike 1001 metre ighted to peak in Snowdonia – and we’re del say she did it! in 2007, Cindy, who was diagnosed with PH r as part reached the summit of Glyder Faw limits of a personal challenge to test her ion. After and raise awareness of her condit and checking with her specialist team, , she ng following months of careful traini se who took on the trek as a tribute to tho have supported her over the years. that you speak to your PH

is important challenge. *speItcia list before taking on a physical

Last-minute stocking fille rs

If you’re look ing for meanin gful gif ts for you love this people Christmas, ch eck out the m in our online erchandise shop. Choose from insulate bottles, pin b d drinks adges, dog ba ndanas, mult and more – bu iw ra ps t hurr y, you’ll need to order delivery befo now for re the big day . Visit w w w.p and enjoy fre hauk.org e P& P on all o rders.

We recommen d ordering to co that you contact us before nfirm delivery before Christm is possible a s .

E m a il o ffice @phauk.o or call 01709 7 rg 61450.

WINTER 2021 emphasis 7

Making

your voices

heard We dedicated the first week of November to putting the spotlight on pulmonary hypertension - and your support was incredible.

With the theme this year of #HearOurVoices, together we made people sit up and listen.

Read on for a round-up of PH Awareness Week 2021…

21 # 21liln enge Cha

Ambitious Abbie Hampshire and her brothers took on 21 different challenges during PH Week, with activities ranging from 21 minutes of kitchen disco dancing, to listing 21 things that made them happy, and painting a picture with 21 fireworks in it. The siblings also made 21 cards for care home residents, lip synced to Disney songs for 21 minutes, and told 21 jokes - plus much more besides!

Terrific #21in21 challenges

Led by Clare Beeton, the St Neots Nordic Walking Group completed a soggy Saturday walk to raise £155.

We’re grateful to every single person that got nvolved; these are just a few examples of your imaginative fundraising:

Scott & Melanie Hatton each took on a challenge in support of their daughter Daisy.

Scott cycled 21km each day of PH Week, and Melanie walked 2.1km a day with Daisy and her brother Jaxon. The parents raised over £4,000 and documented their challenges daily on Facebook, raising an incredible amount of awareness too.

From baking to biking, your #21in21 challenges helped raised thousands of pounds alongside awareness.

Little PHighter Folakemi Ariwayo was joined by her sister to bake 21 cakes, which they sold to family and friends.

Katie Eaton, who has PH, cycled 21km with her husband and son to raise over £1000.

Pupils from Harleston Primary School in Norfolk were sponsored to complete 21 jobs at home during PH Week, supporting classmate Connie, who has PH.

WINTER 2021 emphasis 8

Lebo Mutsvairo used her Instagram account to generate £142 in sponsorship for walking 21 miles over 21 days.

Despite struggling to walk 1000 steps a day, determined Lea Atkinson completed 2021 steps around his local park, accompanied by family carrying his oxygen.

Mark Pincock chose a scenic route for his challenge, cycling 21km on each day of PH Week in the Spanish Alicante mountains.

60

CONDS E S

Express

yourself Getting creative We loved receiving your ‘Express Yourself’ submissions, including this cute needle-felting by Jane Barker-Benfield, and Tamsin Hutchings’ beautiful portrait of her pet bird.

Pausing for PH

To mark the start of PH Awareness Week, we came together to observe a 60-second silence to think about those fighting the condition, those we miss, those who inspire us, or those we are grateful to. The pause was led live on Facebook, and people tuned in from as far afield as Australia.

We played a special silent film during the reflection, made up of your photos of those you would be thinking about.

Making headlines

Your stories appeared in newspapers up and down the UK, helping thousands of people learn more about pulmonary hypertension. PH also reached the airwaves, with multiple radio interviews and even BBC television coverage.

It can be a big decision to share your personal experiences in this way, but it really does have a massive impact.

We are incredibly grateful to those who trusted us to tell their stories, and to those who contacted their local media themselves.

Making PH visible on film

A series of special short films brought your stories to life and helped people understand the reality of PH as invisible illness. As well as filming face-to-face, we also conducted interviews via Zoom - and the result was ten videos that generated hundreds of views, and that can be used to raise awareness for years to come.

A special thank you goes to everyone who spoke to us on camera, allowed us into their homes, and took the time to talk to us online.

You can view all of our PH Awareness Week videos on our YouTube channel.

As always, the support via social med ia was amazing too, with hundreds of p eople sharing exper iences, photos and fa cts, and spreading our content far and wide.

It was fantastic to se e so many of you making use of the photo card incl uded in the last issue of th is magazine - turn to the b ack cover and see if you can spot yourself!

earOurVoices We’ve continued the #H ue of iss s theme throughout thi stories the of Emphasis, with some and even , ek We PH you shared during t for ou ok Lo es. nc more real experie e! the orange badg

# HEA

R o vou r

ices

WINTER 2021 emphasis 9

Pcoorenterr y y e n r u o j H P E T C y M stor y in n craf ted her sing Myra Morriso eness Week, ri ar w A PH of t suppor creating chal lenge by to our #21in21 couplets. of 21 rhy ming a poem made

so slow the, I walked I couldn’t brea d to go.

I ha So to the doctor ead ill, I’ll ring ah You are quite they’ll give you a bed. ital, Go to the hosp long rong. night was oh so That very first leave, it was hard to stay st to d ha d My husban s and a test e bloods, scan We’ll take som d get lots of rest. d, an You stay in be h my hair? , perhaps was up t ge Can I ay there. t id, you mus st Not yet they sa re the tubes go he Groin to heart, t and gave me fear. e mos That scared m have got, H is what you EP likely a lot.

Okay, so CT estions, most qu e m ed so ve ha You’ll is what you ne and books, this s et afl le n, ri Warfa ad. e and have a re So now go hom , er s into a draw I put the book sand and tried to ignore. the Put my head in oks came out, s’ time, the bo th on In a few m t. and had a shou I read, I cried, and a chat, st te d oo k, bl Six-minute wal I’m okay with that. ths, Every few mon ey thought heart again th I ought. to n oi gr do Let’s that do it, but knew Didn’t want to u can go, yo w ay, so no You’re doing ok we really don’t know. ng, You may live lo ways, in oh so many d ge Life chan days. d ol uff like in the Couldn’t do st , I found my way stay. d Little by little ul co I so ust fight, ‘Cos I knew I m stay home, to d ha e COVID, so And then cam nt in, and no longer roam. ng se d, Get the shoppi ade me feel ba depression, m d an d. sa Anxiety ly al time and felt re I cried all the e so much, and helped m My lovely husb en, and he is my crutch. a burd But I still feel e some art, bread, and mad was a start e m so d ke ba I d it feel better, an That made me s oh so much, id dk and gran I missed kids and such. t n hug and mee But now we ca es sad, ed and sometim me glad.

I still feel scar es ak m re, and that But I’m still he

WINTER 2021 emphasis 10

Here at the PHA UK, we’ve long been advocates for writing as a form of therapy and self-expression - and we love it when you send us your poems.

All bets are off

and lives w ith PH Anita Jackson star ted d Sy ndrome an Eisenmenger ing y whilst shield w riting poetr last year.

At 3 they said operation’ ‘needs a heart ation Before the oper they said ce 50 /50’ ‘Survival chan ble e operating ta 12 hours on th they said ere’ ‘Got a fighter h Teenage years they said see 40’ ‘Don’t expect to

Mid 30s they said ecade’ rvive to fifth d su to y el k li n ‘U Mid 40s they said 60’ ‘Should make Mid 50s They stopped saying.

ll stop near?

Is the final fu g, n Or, tired of losi aking bets? m they’ve ceased

Want to see your work in Em phasis? send your p oems to media @ pha uk and we’ll do .org ou best to share r them.

# HEA

R o u vo r

ices

Ros Knight

“I feel like I’ve been given a second chance”

Ros Knight lives in Hertfordshire and was diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) in 2016. This Christmas marks five years since her pulmonary endarterectomy surgery.

M

y diagnosis of CTEPH took about three years. I started going downhill following what was thought to be pneumonia and I couldn’t get anybody to listen to me.

I was going to my GP surgery at least once a week, but I was getting nowhere.

Doctors wouldn’t run any blood tests, and one simply told me not to eat so much.

That time, as I left the room, I burst into tears and had a complete meltdown.

Another doctor saw this, and it was him who ordered the blood tests for me - finally.

I received a phone call the next morning asking me to go to hospital for further tests as they suspected I had ovarian cancer.

The scan [of my uterus] caught the bottom of my lung and showed something there, which they suspected was a tumour, and this led to investigations of my lungs and heart.

Whilst I was in hospital, I started coughing up blood and scans revealed that the mass they had seen was down to clots, not a tumour. I was referred to Royal Papworth Hospital where I was officially diagnosed with CTEPH. Although I was told that a pulmonary endarterectomy operation [to remove the clots] can help some people with CTEPH, I was too poorly for it to be an option.

I was told I had a few weeks left to live

and probably wouldn’t leave hospital.

I was just 45.

This was at the beginning of December, and I started making arrangements.

I started buying everyone’s Christmas presents and paid all the bills, so my husband Richard wouldn’t have to worry.

We discussed my funeral, and what I would like.

“My husband and I have adapted our lives around my illness” And then a surgeon decided to take a chance with the operation. He said that he was willing to give it a go if I was, and I knew I had nothing to lose.

I underwent the surgery on 12th December 2016 and although I’m not cured, I am a lot better.

LIFE WITH PH TODAY

Five years down the line from someone who only had a few weeks left to live, I’m doing really well.

I still get quite short of breath, and I can’t walk long distances without taking rest stops. I have adaptations at home, like a stairlift, and I do have days where I am completely exhausted. But the main thing is that I can still do things.

Top left: The night before the operation, December 2016. Top right: With her grandchildren in hospital. Middle: With son Callum in hospital. Bottom: With husband Rich in hospital.

My husband and I have adapted our lives around my illness, and I still get to see my family and I can still go for walks.

I definitely feel like I have been given a second chance.

I’ve had a lot of help from my local hospice. To be told I needed hospice care at 45 years old felt like the end of the world for me, but it turned out to be the best thing. It gave me back something to live for.

Through their day services, I’ve been able to access singing and crafting groups, coffee mornings, complementary therapy and even a gym - which helped me to build up my strength again.

Both my husband and I were able to benefit from counselling, and all through the pandemic lockdowns I was still able to access their services via Zoom.

As well as the hospice, I can’t thank the surgeons at Royal Papworth enough for giving me a second chance. My family and I always celebrate the anniversary of the surgery as it’s like a second birthday.

It’s the day I got my life back.

WINTER 2021 emphasis 11

# HEA

R o u vo r

ices

Diane Kaylor

The (im)patient

and the carer

Diane Kaylor lives with pulmonary arterial hypertension, along with multiple autoimmune diseases, and finds writing helps her to cope with the challenges of her health conditions. In the first of a new series of musings for Emphasis, she takes an honest look at what it all means for her relationship.

*Karl’s name has been changed by Diane to protect the innocent.

WINTER 2021 emphasis 12

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

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

I

just want to make it clear that we didn’t sign up for this. I’m pretty sure you didn’t either. Whether you are husband and wife, mother and son, sisters, or friends - the dynamic of the patient/carer relationship brings about some fairly seismic shifts which I’m hoping to document here.

Why? Well to get it off my chest for a start. And controversially, I can get a bit fed up with hearing all about these perfect caregivers (usually sainted husbands) who anticipate every need and the effusive gratitude they elicit from their appreciative recipients.

I tell you it isn’t like that in our household. And maybe it’s not in yours.

Gratitude’s all well and good (and I assure you I experience it every day) but isn’t there also a place for seething resentment on both sides?

“Old fools are babes again.”

Shakespeare (King Lear)

The thing is, I’m not old. Isn’t 60 meant to be the new 50 now, or even 40? And friends of my age and older are cycling and wild swimming, whereas I’m sitting on a sweaty rubber cushion trying to stave off pressure sores.

Believe me, I don’t begrudge them re-enacting their own private version of a multi-vitamin advert, and apologies if

I sound self-pitying, but sometimes I do feel sorry for myself and I don’t see that as a heinous crime.

When Karl* and I met nearly 20 years ago, I might have been more of a sex tabby than a sex kitten, but I certainly had some libido and a life which involved going out of the house, socialising and even staying up beyond 8pm. How the mighty have fallen!

Ten years into our relationship, my health began to deteriorate rapidly and now, a further ten years on and Karl is my long-suffering carer and I can often feel like I’ve short-changed him.

I lured him in as an attractive, lively 40-year-old (me not him) who over a decade literally decayed. I’ve said already that we didn’t sign up for this, but the thing is, I haven’t got a choice.

It’s happening to me and of course I can try to live the best life I can within the constraints of my disease. But actually Karl has got a choice and he (so far) chooses to support and look after me rather than clear off. And guess what, I get my knickers in a twist because he doesn’t do it perfectly! And I know there’s no point in spinning off down that particular rabbit hole of asking whether he stays out of loyalty, duty, pity or love.

There’s no mileage in it and it’s probably a mixture of all of the above and more.

We’re probably the worst possible combination of patient (there’s a misnomer) and caregiver.

Karl has the temerity to do things in his own way rather than use my methods, which of course are infinitely superior (who on earth pegs out wet trousers by the waistband rather than the ankles?) Such matters of course are of paramount importance.

So yes, we drive each other mad in myriad ways. But having said that, when Karl manages to escape for a couple of days (call it ‘time off for good behaviour’) and I’m fending for myself, the gratitude factor ramps up quite considerably.

Come back Karl, all is forgiven. I’d like to think he misses me too and no doubt he does - just not the fetch me/carry me/ waiting-on-me-hand-and-foot aspect of our relationship.

WINTER 2021 emphasis 13

# HEA

o u vo r

R

ices

jess wescott

I appreciate my health a lot more now

WINTER 2021 emphasis 14

Litigation executive Jess Wescott is 29 and lives near Brighton with her husband Liam. She was diagnosed with pulmonary hypertension at the age of 20, and wanted to share her story to show that everyone has different experiences of the disease.

W

hen I got diagnosed around nine years ago the thought of being 30 seemed really far away, and I’m happy that my health has stayed the same and I’m so stable.

I was born with a hole in my heart and had surgery at the age of six to repair it, followed by check-ups every three or four years.

At one of these check-ups, I was with my mum, and she mentioned that she had noticed me getting out of breath when going up the stairs.

They ran some tests and that’s how the PH was identified quite quickly. I was told it was from the damage that was caused by the hole in my heart before it was repaired, and that it had just taken a long time to show.

I had no idea what PH was, and I didn’t understand the impact it could potentially have on my life.

When I was given my diagnosis, the doctor drew a diagram and explained that I shouldn’t have children. It was a shock.

I don’t remember much from those first few days, but I do know that everything I read online about life expectancy was worrying me. I didn’t feel like the people in the stories I was reading about, so how could it be true?

I was in a bit of denial, because I’d always lived a really ‘normal’ life - going on girl’s holidays, travelling, hiking, swimming, and playing rounders at school. I never noticed there was anything wrong.

Once I started speaking to specialists, they explained everything properly and helped me understand that everyone is different when it comes to PH. They said they were hopeful my symptoms could be managed by medication, and I know I was lucky that it was picked up so quickly.

I take two types of medication and my symptoms are minimal; I just need a bit more time to rest and recover than other people. I’m okay when I’m actually doing things, but it can hit me afterwards.

I’ve been married to my husband for two years now.

We got together about 12 months before I was diagnosed, and I remember having an awkward and uncomfortable chat about the doctor suggesting to me that I don’t have children.

I haven’t ever been one of those girls who’s always said they can’t wait to be a mum, or that they can’t wait to be pregnant, but it was still tough to hear that I shouldn’t have a baby.

To have that decision taken away from me when I was so young was a massive shock.

I have since been referred to a specialist in London who deals with pregnancies in people with conditions like pulmonary hypertension, and they have told me that because I am so stable, it could still be an option.

It’s amazing to have the choice back, but I’m very aware that it wouldn’t be a straightforward pregnancy and Liam and I had already discussed the route of adoption, so that could still be a possibility.

I don’t think too much about the future. I’ve got quite a positive outlook on everything, and since being diagnosed with PH I definitely don’t take anything for granted. I just appreciate the smaller things in life, like being able to go for a walk. I appreciate my health a lot more, and what I’m able to do.”

"I’ve got quite a positive outlook on everything...I definitely don’t take anything for granted" WINTER 2021 emphasis 15

ph + thec pandem

Your experiences of living through the pandemic with PH THE EXPERIENCES OF PEOPLE WITH PH

Respondents had the following types of PH:

Thank you to everyone who took part in this major piece of research over the summer.

We asked for the experiences of people with PH, their loved ones and those they live with, and parents / guardians of children with PH – and over 1000 responses were received!

In the last issue of Emphasis, we brought you a snapshot of some the hundred of comments shared, and this time, we’re reporting on the headline findings. You can view further findings in our online report at www.phauk.org.

We will be digging deeper into these statistics next year. With in-depth interviews and further analysis planned, we will ensure that lessons are learned, and the PH community is heard, as we rebuild after COVID-19.

WINTER 2021 emphasis 16

30% 20% 11% 16% IPAH

24% CTEPH

PH in association with congenital heart disease

58%

PAH in Other / association unsure with systemic sclerosis

said their physical health worsened as a result of the pandemic.

11%

had received their PH diagnosis during the pandemic

(after March 2020)

66% said their general health worsened.

77% said their fitness / physical activity levels worsened.

46% said they gained weight.

88%

76%

68%

‘fully followed’ shielding advice during the 1st lockdown.

‘fully followed’ shielding advice during the 2nd lockdown.

‘fully followed’ shielding advice during the 3rd lockdown. felt they 91% personally needed to shield.

said they found it difficult or very difficult to access support from their GP services.

In early summer 2021, at the time of completing the survey, 98% of people with PH had received both parts of their COVID-19 vaccination.

said they found it easy or very easy to access support from their specialist PH centre.

25%

said they had missed medical appointment/s since the start of the pandemic. Of these people, 17% said they had missed 3 or more.

22% 43% 10%

of people with PH said they had experienced new symptoms of depression since the start of the pandemic.

said they had experienced new symptoms of anxiety.

said they had started experiencing panic attacks.

As of 31st July 2021, 57% of the whole of the English population had received two doses of the vaccine*

51% 45% said they felt good or very good about normal life resuming.

said they felt worried or very worried.

44%

said the restrictions on everyday life imposed by the pandemic did not make much difference to their lives.

WINTER 2021 emphasis 17

*Source:https://www.england.nhs.uk/statistics/statistical-work-areas/covid-19-vaccinations/covid-19-vaccinations-archive/

47% 87% 98%

ph + thec pandem

THE EXPERIENCES OF LOVED ONES

said they felt good or very good about normal life resuming.

WINTER 2021 emphasis 18

said they felt worried or very worried.

Different type of relative or ‘other’

We asked respondents how they thought the person they live with (with PH) has felt about the restrictions on everyday life. This is how the responses compare: "The restrictions have not made a difference to their life"

43 %

7 %

"They have found the restrictions difficult to live with"

10%

of respondents are classed as clinically extremely vulnerable themselves

61 %

10 % Patient

32 %

"The restrictions have made their life easier"

44 % Patient

46% 48%

Sibling

Loved one

of respondents said they have felt ‘extremely concerned’ about the person they live with (with PH) contracting COVID-19.

Son or daughter

Loved one

62%

Spouse or partner

Patient

would describe themselves as an ‘unpaid carer’

81% 5% 3% 11%

Loved one

62%

Respondents had the following connections to someone with PH:

THE EXPERIENCES OF PARENTS AND GUARDIANS OF CHILDREN WITH PH

14% 78% 92% of respondents said their child received their PH diagnosis after the pandemic began (after March 2020)

43%

said their child has found the pandemic difficult or very difficult.

said the support from their child’s specialist PH team has been outstanding or excellent.

32% said it has been good or very good.

Fear of their child contracting COVID-19 caused the greatest distress to parents / guardians.

said they have it found it difficult or very difficult themselves.

42%

said the support from their child’s school has been outstanding or excellent.

27% said it has been good or very good.

gs online at in d n fi re o m w ie V d . We can also sen www.phauk.org via email – just rt you the full repo rg office @ phauk.o drop us a line at WINTER 2021 emphasis 19

WORDS

OF WISDOM

e c n e i r e p x e y b s t r e p x e FROM THE

Medical professionals play a vital role in helping us managing our health conditions, but advice from those with lived experiences can make a big difference too. We asked seven PHA UK members what they would say to someone newly diagnosed with PH.

Julie Royle, diagnosed with PH in 2012

“Have an open dialogue with your specialist centre and your specialist nurses. The best thing you can do is talk about it. I didn’t, and when I look back, I realise I would overthink things but sometimes I wouldn’t talk about them. Half of the time it was because I didn’t want to accept what could be happening and what the future could be. I was trying to deny what was going to be the rest of my life. But talk to your family, and don’t hide anything.”

Kevin Rowland, Julie’s partner

“My advice for a partner or loved one is to learn as much as you can about what PH is, and not just what appears on Wikipedia. Learn from the professionals and know exactly what you are talking about. Then just take each day as it comes - and keep a sense of humour.”

WINTER 2021 emphasis 20

Tess Jewson, diagnosed with PH at the age of 17

“There is help and support out there. Live life to the full.

Pulmonary hypertension will change your life, but as I always say, PH lives with me - not the other way around.

If I could speak to myself 13 years ago, before I was about to receive my diagnosis, the message I would give myself is that it’s going to be okay.”

Lynsey Childs, diagnosed with PH in 2015 “Don’t Google it! That’s the biggest mistake anyone can make. Read Emphasis, and anything that comes through from the PHA UK. Anything you can get your hands on that’s not fabricated, and anything to help. Ask your hospital if they know anyone around you [who has PH]. To begin with I didn’t know anybody with PH. I’m quite lucky that I’ve now met a few people, not too far from where I live, and they are like family. I can probably talk to them a lot more easily, and more openly, than I can to my own family - because they understand.”

Pam Fenton, diagnosed with PH in 2018

“Listen to your doctors and do what they say. Know your limitations, and don’t let anyone tell you what you can or can’t do.

Put yourself first; you’ve got to be selfish. If you get up in the morning, pat yourself on the back - and if you go and make yourself a cup of tea, give yourself another one.

Embrace what you can do, and don’t worry about what you can’t.”

“Join the PHA UK private Facebook group.

It’s a lonely illness - I haven’t met anyone in my area with it - and it’s very scary to be told these things [when you are first diagnosed]. If you go into that group, there are people [with PH] there to help you, day or night. So, if you can’t sleep, there is always someone there who will answer any question. And I find it reassuring that there are people on there who were diagnosed 20 years ago, and they are still alive, and still living with their own heart and lungs, and on oral medication like I am. That is such an uplifting thing to read and listen to.”

e would What advic someone YOU give to with PH? nosed newly diag auk.org

@ ph ail to media , Send an em subject line e th vice’ in d A the y in ‘M n h ca it e w ny as w a m s a e d u cl zine. and we’ll in f this maga nex t issue o

Find details of our Facebook groups on page 35.

Andrew Sweeney, diagnosed with PH in 2014

Lou Chadburn, diagnosed with CTEPH in 2012

“Take each day at a time and take in all of the information that is given to you. Yes, it is scary, but don’t let your fears overcome you. There are things that people can do for you. Communication is a big thing. Talk to someone you love. I used to bottle things up and start to cry for no reason, but my husband encouraged me to talk, and I started to get things off my chest. The experience has changed both of us, as we now speak our minds more.”

WINTER 2021 emphasis 21

Meet Dr Quyam Dr Sadia Quyam is now eight months into her role as Consultant in Paediatric Cardiology and Pulmonary Hypertension at Great Ormond Street Children’s Hospital (GOSH). We caught up with her to find out more about her motivations, inspirations, and what the future might look like for the treatment of PH in children.

I believe strongly in what the service is for and what we have the potential to achieve, so it’s my dream job really.

WINTER 2021 emphasis 22

Welcome back to the world of PH, Sadia! Could you tell us what brought you to your current role at GOSH? "I trained in London; at GOSH and the Royal Brompton Hospital, with a year as a Magnetic Resonance Imaging (MRI) research fellow at the University College London Institute of Cardiovascular Imaging.

I also spent a short time in San Francisco with the PH team there, and my previous role was as a Peadiatric Cardiologist at the Evelina London Children’s Hospital.

My sub-speciality training was in peadiatric PH here at GOSH, so it’s really nice to be back doing what I enjoy the most and seeing some familiar faces.

I first became interested in PH really because of Dr Shahin Moledina, who is the lead for the peadiatric service here at GOSH. I was quite a junior doctor at GOSH, early in my training, when he took the time to explain some of the basic principles of PH to me. This definitely sparked my interest and

made me want to learn more!

Once I had the opportunity to work in the service I was so impressed by the whole team, it very quickly became something I wanted to be a part of.

I believe strongly in what the service is for and what we have the potential to achieve, so it’s my dream job really.

What does a typical day (if there is such a thing!) look like in your job?

"I start and finish my day generally in the same way; by cycling over the river on Waterloo bridge. The view is amazing at both times of the day and really puts me in the right frame of mind.

After that (and most doctors probably say this) one of the great things about my job is the variety.

My work is mostly direct clinical care, sometimes with in-patients, in clinics, or seeing patients and families who come to see us for a full day assessment.

I also have a role in education and training with the junior doctors and when I can, I also try to spend time working to develop the service and keeping myself up-to-date."

What are you enjoying most about it all so far?

"I am enjoying pretty much everything about being back! We are a small team and I feel very lucky to work with such inspiring, hardworking, and kind people.

At the same time, I am enjoying

...the inspiration to be better is everywhere; from friends and family, and colleagues in other specialties and in research, to the patients and families we treat. seeing some of the patients I met very early in their journey and hearing their stories.

For me, there is something about this role that feels like coming home, and I am very much enjoying that feeling" What do you think the next 5-10 years might look like in terms of the management of PH in children?

"Things have definitely changed in the time I’ve been away from the PH service. I think looking forward, our understanding of genetic causes will move us towards personalising medicine.

If we can better understand the differences in the types and causes of PH, then our approach will change from titrating medication to current measures of severity, towards a more complex approach that looks at a certain type of PH, in a certain age of patient with a certain genotype etc.

At the same time, we will find more targets for drugs so this new understanding will inform our approach to their use. I think this will also be informed by better design of the systems we have for the approval of these medications, particularly for children. Then my hope is that we will see improved access to high quality care, particularly in middle and low-income countries. "

When I trained there were not many women in peadiatric cardiology. Dr Helen Spencer [Respiratory Paediatrician and Clinical Lead for the Lung Transplant service at GOSH] was definitely someone I looked up to and saw as a role model [at that time], and I still do.

I think it would embarrass him very much if he read this, but Dr Shahin Moledina has certainly had a huge (positive!) impact on the kind of doctor I am as well.

The PH team in San Francisco are also very inspiring in the way they work as a team and their dedication to their patients. But really, the inspiration to be better is everywhere; from friends and family, and colleagues in other specialties and in research, to the patients and families we treat."

Finally, how do you like to relax away from work?

"I take a weekly yoga class and am also part of a local running club. I won’t pretend to excel at either but it’s fair to say I’m better at stretching than at running.

I grew up in Yorkshire and I miss having so much natural beauty on my doorstep, so I try to get back up there as much as I can - which I hope is also preserving my accent."

For me, there is something about this role that feels like coming home

Who, or what, inspires you in your career?

"I probably wouldn’t say a single person, but as you go through your career you meet many inspiring people.

Often, I will see the way someone approaches a problem or communicates something difficult, and it inspires me to develop those skills in myself.

WINTER 2021 emphasis 23

In conversation...

y m n o k c a b g n i k Loo

s i s o n g a PH d i Alex Porter was told she had idiopathic pulmonary hypertension in 2011, after experiencing symptoms following the birth of her son. To mark a decade since her diagnosis, the 37-year-old from Leicester looks back on those early days with PHA UK Chair Dr Iain Armstrong…

I A

Could you tell me what the path to your diagnosis was like - the good, the bad and the ugly?

My son Finnley was born in 2006 and it was the start of 2007 when I experienced my first blackout. From then on, it gradually got worse in terms of my breathing.

I was struggling because getting up the stairs was difficult, but I put everything down to being exhausted from having a newborn.

I couldn’t keep my eyes open because I was so tired all the time and I was starting to swell up as well. At the time, my doctor put my swelling down

WINTER 2021 emphasis 24

to my contraception pill, and my exhaustion and breathlessness down to panic attacks and anxiety.

I A

So, what prompted the diagnosis in the end? What was the crucial stage in all of this?

My symptoms, and going back and forth to the doctors, continued until 2011. My whole body was swollen, and I couldn’t even walk, even from one room to another. One day, my son was supposed to be going to a birthday party and I was trying my hardest to get him ready when my fiancée (now husband) Ben insisted on taking me to A&E to finally get things sorted.

Off we went, and I didn’t come home then for six weeks.

I

I

A

A

It seems a lot of people have their early symptoms labelled as panic attacks or anxiety, particularly young females and young mums. How did you handle that?

It made sense to me at the time because my partner had just broken his back, and I had an 18-month-old, so I thought who wouldn’t be anxious with everything I had on my plate back then? To be honest I think I just accepted it.

It was a long build-up to that point wasn’t it. If you had that time again, would you have done anything sooner?

Yes, definitely. We look back now, and everyone says, why did we think ‘that’s just Alex’? It sounds awful to say but I think everyone thought that I was lazy, and I didn’t want to do anything. I think I am one of those people who tries to get on with things

Alex with husband Ben and son Finnley no matter what. But I should have pushed for more tests or asked to be referred. Obviously, what was going on at home was not normal. Every time I walked up the stairs I passed out, and I couldn’t bend down to put clothes in the washing machine without thinking that I was going to collapse. When I went into hospital, they drained 12 litres of fluid from me so that’s how swollen I was. It’s ridiculous really.

I A

Can you remember the time when you and Ben were told that you had PH? What was it like?

It’s all a bit of a blur to be honest.

I think part of me was relieved that I knew what was finally wrong with me and that there was a plan in place to, hopefully, relieve my symptoms.

Obviously, there’s the other part of you

"Take the time to absorb everything you've been told... everyone deals with things differently"

that’s petrified because Finnley was just turning five. When you’re having all of that information given to you, it’s hard to absorb it when all you are thinking of is your son and everything else.

My future was all that I was bothered about at the time because Finnley was so young. We had planned to have a bigger family, but we were then told that wasn’t really the path we could go down. I knew then that I just wanted to know exactly how my future would be for Finnley’s sake.

I A

How did you approach those conversations with Ben and your family?

I think Ben is a very positive person anyway and he will always look for the positive in things.

To be honest, our conversations were quite easy because we knew that we would get through it together.

I knew he wasn’t going anywhere.

My parents spent a lot of time going backwards and forwards [between hospitals]. When we did have ‘the big chat’ about the diagnosis, they were there. We are a very open family, and my son knows that he can talk to us about anything. He’s never known any different, this is just how I’ve always been.

I A

What were the main questions you had back then?

It sounds quite morbid, but it really was all about my future. I went into panic mode about how long

I thought I was going to be here, but no-one can tell you answers for that because every person is different.

I got the diagnosis and within a couple of weeks I went back to work.

I think I was almost in denial. I got told all of this information, started my medication which made me feel so much better, and almost felt back to my old self. I think I just threw myself into everything. It’s only now, ten years on, that I think to myself that I was a bit nuts for being the way I was back then.

I should have slowed myself down.

I

Based on your experiences at the start of your journey, do you have any nuggets of advice for someone newly diagnosed with PH? I think the main thing is to take the time to absorb everything you’ve been told. There is no rush to get your head around it. Everybody deals with things differently.

The PHA UK Facebook group is really helpful. I think it is important to speak to patients so that you have got real life experiences rather than going to Google. You can put anything into Google, and it will tell you the worst news, but there is always a positive in any situation

A

.

WINTER 2021 emphasis 25

Do you need someone to

talk to?

PHA UK Listening

LINE

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

• Available to all PHA UK members and those close to them • One-off or weekly telephone appointments available • An impartial listening ear

Find out more or request an appointment by emailing listeningline@phauk.org or calling 01709 761450 Appointments can also be booked via our website at www.phauk.org WINTER 2021 emphasis 26

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

g n i k o o L . . . d a e h a ar VID-19 turmoil, a new ye Af ter more months of CO PH for us all. We asked our represents a fresh start what they are looking community on Facebook for most in 2022… forward to and hoping e hoping to be able to go Melanie Hatton We ar gether and make many on lots of family trips to love to take precious memories! We’d Peppa Pig World Daisy (our PHighter) to ay on a proper family holid fully 2022 Fiona Ballantyne Hope nsplant will bring me a lung tra to have our kitchen Gail Lanigan We hope k ar....and what we so loo extension done next ye ore quality time with forward to is spending m is outside our loved ones, even if it g forward to getting Gaynor Oates I’m lookin one last year due married! We had to postp ding my 6-year-old to restrictions and shiel she will get chance PHighter.... so hopefully aid! next year to be a bridesm have my belated 50th Steph Dodge I'd like to a, and finally get to birthday holiday in Florid Fender in London see the Killers and Sam g forward to seeing Sybille Wood I’m lookin y again after two years. all my family in German ce my diagnosis It will be the first time sin next year so Mark Kingston I am 60 ble to have I’m hoping it will be possi celebrate a great family party to ar my son is getting Joanna Young Next ye r October married so I can’t wait fo pe to Kevin McAllister I ho get insured to fly abroad

2 02 2 Here at the PH A UK, we’re lo oking forward to ne xt year too…

“I’m excited ab out the birth o f my second child in May. It will be a busy year as a dad-o ftw o !”

Shaun C

layto of Membersh n, Director ip Support

“I’m planning to get a dog, st art doing more exercise, and spend mo re time with my grow n-up kids.”

John Smith , Associate D

esigner

“I’m looking fo rward to spen ding time with my new p uppy Benji and creating more downtim e to read and en Paul Sephton joy walks.” , Research Su pp

Developmen

t Coordinato

r

ort and

“I can’t wait fo r th spring and sum e better weather of mer to enjoy m y horse.

I’d also really lo ve to get abroad Mary Ferguso in 2022.” n, Associate

Communicat io

ns Manager

A R E Y W E N Y S ALL! P P MU A H RO F WINTER 2021 emphasis 27

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

Stuart Craig Clinical Nurse Specialist in PH, Royal Brompton Hospital

Professor Nick Morrell

Research Director, National Pulmonary Hypertension Service, Royal Papworth Hospital

“I’ve been told that my PAH is idiopathic, but I’ve heard it can be hereditary too and I’m worried for my children.

Is there a chance it could have been passed on to them?”

The short answer is yes, but let’s dig into this a bit deeper. I am assuming that you do not have a family history of PAH. If you did have other family members with PAH it makes the likelihood of a genetic cause of PAH very high indeed. If you have idiopathic PAH, which occurs ‘out of the blue’, it is still possible that there is a genetic (hereditary) cause for the condition. About one in four people with idiopathic PAH carry a genetic fault (a mutation) in one of the PAH genes. If you carry a faulty PAH gene, there is a 50% chance that the faulty gene will be passed on to a child. The most common

WINTER 2021 emphasis 28

Dr John Cannon

Director of the National Pulmonary Hypertension Service, Royal Papworth Hospital

genetic cause of PAH are mutations in the BMPR2 gene. If a child receives a faulty BMPR2 from a parent, then they have an increased risk of developing PAH. The risk of developing PAH if they carry a BMPR2 mutation is higher in females than males, but overall, the chances of a child with a BMPR2 mutation actually developing PAH in their lifetime is around 20-30%. For these reasons, recent international guidelines recommend that genetic counselling and testing is offered to all people with idiopathic PAH. If, after testing, there is no identifiable genetic cause it is extremely unlikely that a child will be at risk of PAH. If on the other hand, a genetic cause is found in a parent with PAH then the family can decide whether they want the children tested for the faulty gene also. If a faulty gene is found in a child, then screening can be offered every year to make sure there are no signs of PAH developing, and to detect it as early as possible.

If the child does not carry the faulty gene, then there is no concern about developing PAH. Pulmonary hypertension centres are increasingly aware of the genetic issues in PAH and will be able to provide further guidance and referral to local clinical genetic counsellors and services.

Professor Nick Morrell

cruiting more PH is currently re of y ilit ab rit he e into th cohort.com or A major UK study e visit www.ipah as Ple rs. be em m ily r further details patients and fam schl.cam.ac.uk fo ed m n@ io at in rd email cohortcoo

“Winter weather, particularly cold and rain, makes me more breathless - but I like to get out and about as much as I can. What can I do to limit the impact on my breathing?”

The cold weather causes trouble for a lot of people living with pulmonary hypertension. The cold irritates the airways which make can make you more likely to cough and find your breathing a bit more difficult. Fortunately though, there are a number of things that can be done to lessen the impact of the cold weather. Breathing in through your nose and out through your mouth will help as the air gets warmed as it passes in through your nose. Try, if possible, to wear

“My husband has been diagnosed with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and balloon pulmonary angioplasty has been mentioned as a possibility.

How does it work and what are the risks and benefits?”

Balloon Pulmonary Angioplasty (BPA) is a fairly new procedure in the UK, with the first procedure undertaken at Royal Papworth Hospital in November 2015. Subsequently it has been formally commissioned by NHS England for patients who are unsuitable for pulmonary endarterectomy surgery since 2018. The procedure involves a very fine wire being inserted into blood vessels in the lungs, which guide a tiny balloon into position under x-ray guidance. The balloon

a scarf that covers your nose and mouth. Use a smooth material that will be more comfortable than an itchy one which may feel irritating. Keeping well hydrated will help too. Make sure you have some water to drink, or even a boiled sweet, to stop your mouth getting too dry.

Making sure you stay warm when out and about will make a difference too. Hats, gloves and warm socks to keep your hands and feet warm are helpful additions for outings. Finally, try and keep an eye on the weather forecasts and plan your trips to avoid the coldest days if possible. It is really important to keep getting out and about for your physical health and your mental wellbeing. Hopefully these tips will help.

Stuart Craig

is inflated, to around the size of a pea, for a few seconds to push the blockage aside and restore blood flow to the lung tissue. The balloon is then deflated and removed. This is repeated several times in different parts of the lung during a single treatment session that lasts about 60 minutes in total. Light sedation and a local anaesthetic are given during a session, so your husband would feel sleepy, but he would still be able to hear and understand instructions. The response to BPA and total number of BPA sessions needed is dependent on the severity of CTEPH. The expected outcomes and risks will be discussed with you and your husband in a BPA clinic if your husband is suitable. BPA has been shown to significantly improve breathlessness symptoms and quality of life, lower lung blood vessel pressures, and relieve heart failure - but it is unlikely to fully cure his CTEPH. The effects appear to be long-lasting, although because it’s a fairly new treatment, further work is required to confirm these results in the longer term.

Dr John Cannon

Got a question for the next issue?

Email media@phauk.org

WINTER 2021 emphasis 29

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4 PAGE

t s e g g i b y m s i r e h t ” o d n e i r f t “My br s e b y m d n a supporter

n up with me ild, he had just grow ch a as d PH y m d understoo e, with oxygen, an eelchair all the tim wh . or y ily gg m fa bu a a as in as g bein ot h al to us all ist Kaylee Myn ed as a to him. It was norm n al m rm no lu st co ju en s wh wa ew s uggled with wa Green Leaf Cr PH, having been diagnos other it ing we really all str ax br h I think the only th um wouldn’t see M m d grown up wit tells us about her younger ey. an hospital. Me in ys e m e sta rn ho ve u sh at ha jo e, be to d er d er I ha he woul baby. H ade to h dn’t get to see us as rence he has m every day and he di course it made that of , m and the diffe hi th wi se clo y all re e g re in th with Dad. Be lled Max. He’s ger brother ca ve n e’ u w yo p a u very difficult. g everything, ve in ha ow "I r understanding of s me and gr tte in be an a tw d th ed ha r ll ax ge ca M n t u to ge r’. He became years yo As we grew up close. We used ike and were e role of ‘big brothe ly th al on re ok t! to en ly be ick s so al alway and very qu inks he’s the oldes use we looked d everyone still th ca an be e, e m m an ti th r e lle th ta all much hile. er as we have height for a w much closer togeth ber so n em ow m gr re st t ju n’ ve do also the same ha ble. Looking back born so I Max and I thought was possi as when Max was er w e ev I re up th g an th in ly him ! e or on ow m gr , as Iw grown up h all this without However, d have got throug unger sibling. ul yo co I a k in ng th vi n’t ha do t no now I . d. He’s always for me and Max r and my best frien he ter ot or at pp th su t ng es very different hi gg He’s my bi d there to pick me do everyt ing my best life an urse I couldn’t difficult liv e co or of I’m , m re e PH su er e to w ak e ts m Du there to s or spor ng, ter-school club . tempt everythi at children did. Af to up on my bad days st be y m y. d having PH has gh I tried th very quickl ea br g up with Max an of in t for me. Althou ow ou t gr k go in new things, or th d I re ce ti o ni ay to ld back and to try ho d it was so t an no , w to e ne I was always w m ng ed hi ag encour s trying somet d my PH Max was alway I can’t succeed an he enjoyed. if gs en in ev th e th do h led wit l, Max is to just see him activities I sett stops me. After al e erent clubs and ff ax di M th ! of t do ith lo w to e a g r er Afte ite thin always th was my favour ng and ch ts hi hi ke w us an br ng bl e d di th ri an h e n it hors popcor g and help w on al e m co s would alway for a very chilled horses. d di s ay w al feeding of the ax movie day! ” e and M dn’t do a lot, m ever fully ax M k in th Although I coul t . I don’ joyed together things that I en

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WINTER 2021 emphasis 32

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MEET THE

During P H Aware ne Novemb er, we as ss Week in k ed for sh outs for li outtt hyperten le ones with pulm sio o na r y PHighter n. Here are the li s we cele ttle brated o what the nline, an ir parent d s said ab o u t t he m …

Little PHighters

Abbie Hampshire, 5

“Abbie is our courage and our hope”

n, 5

n and u f e h t s e “Ivy se verything. We joy in eproud of her.” are so

a Ivy Ry

att, 6 Izzy Pl life

s “Izzy give

her all”

WINTER 2021 emphasis 34

Hat Daisy

ton, 2

ur he o s i y s i “ Da

ro”

Jessica Hollis, 16

Folakemi Ariwayo, 7

“The joy and love Fola mi brings us is our everythike ng”

“Jess always has a smile”

Lilly Ra 2 monthes Rumbles,

“ We feel bless have Lilly in ed to our lives”

Sarah Sebti, 10

“Sarah is so courageous”

,4 ockton t S n a Morg spires me

Morgan ibnetter person to be a ay” every d

Safe spaces to talk to others affected by PH

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

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

PHA UK Official Facebook group

This is our original forum, which now has almost 2000 members.

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

Join here: www.facebook.com/groups/125342770826215

PHA UK Official Carers Group

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

Join here: www.facebook.com/groups/402808577486104

PHighting On: Life after loss to PH

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

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

Join here: www.facebook.com/groups/215307796620821

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

Getting smart about daily activity

Technology is helping more of us than ever to get active and organised, with smartwatches tracking steps, reminding us to take medication, and much more besides. Here, two PHA UK members tell us how their wrist gadgets have made a difference to them.

WINTER 2021 emphasis 36

Pauline Barrett lives in Nottinghamshire and was diagnosed with PH three years ago. She uses an Apple Watch, with an app called MyHeart Counts, to keep as healthy as she can.

“My family knows I love gadgets, but this little Apple Watch has certainly made me smile, and I am just getting used to using it to its full versatility. I didn’t realise just how much you could do with a small band around your wrist.

The watch has motivated me daily both physically and mentally to keep busy, and to do as much as I can to reach my goals. I have been using the MyHeart Counts App to perform my six-minute walking distance test at home.

I still have a few hiccups, but all in all, it is proving to be an exercise I will continue for as long as possible. It has given me a new stance on life and how exercise hopefully can improve my quality of daily living.

I recently completed a steps challenge and both my watch and the app were extremely handy to register the steps.

The reminders to breathe and stand, and the ability to receive your messages and alarms to remind you it is time to take your medication, are all proving to be a godsend. It is extremely handy when your phone is in another room and your wrist starts to vibrate and you can answer it wherever you are.

I don’t know who comes up with such wonderful inventions, but I am definitely hooked!”

Julie Royle lives in Manchester and has had PH since 2012. She has been using a smartwatch for the last year.

“I’d seen the Apple Watch, so I was happy to be offered it as part of a study relating to PH and fitness.

After having it ten months now, I wouldn’t be without it. I follow and monitor the alerts reminding me to move, stand and do some activity. It’s been amazing as I’ve not been aware of just how active I can be, even on a poor day, especially when it comes to moving and standing.

I’m just starting to use it for sleep monitoring too. It’s made such a difference to my wellbeing and I’m more aware of my movements and activities.

It tells me my heart rate and records all these things to my phone. I can even access the weather. And because it’s linked to my phone, I can receive calls and messages on it, meaning I don’t need to rush to get my mobile.

The exercise is becoming more of a goal to me as I’m constantly looking for ways to fulfil the 30 minutes per day target which I’ve set for myself.

I also have reminders and alarms set for medications.

I’m smashing the target when I’m cleaning, hoovering, ironing, changing the bed and even decorating. As a PH patient all these activities are a challenge and work your heart and lungs and some muscles, so they matter.

Our homes are our gyms!

I’ve found the watch to be a great motivator for keeping me more active than I used to be. I feel better for it, and most importantly, I feel safe when I read the results and monitor my heart rate.

Plus, because it’s on your wrist 24/7, if you need emergency assistance you can call from your watch without having to reach for your phone.”

Wearable technology to improve outcomes fro m treatment

Pauline and Julie received and use their watches as part of a pioneering study for people with pu lmonary arterial hyperte nsion. The study, based at the Sheffield Pu lmonary Vascular Diseas e Unit, is being led by Professor Allan Lawrie of the University of Sheffield . He is working with colleagues at Stanford Un iversity in California to tes t the effectiveness of collecting physical act ivity data, and at-home six -minute walk test data, alongside in-hospi tal exercise assessment such as the shuttle walk test. Using the Sta nford University-develop ed MyHeart Counts app to track heart rate da ta, the study is also lookin g at the impact of COVID-19 on people with PAH. The watches were provided as part of Apple’s ‘Investigator Ini tiated Research Programm e’, and they are also able to collect heart rate data and other measures to generate a fuller picture of an individual’s activity and cardiovascul ar health. It is hoped that by linking this data to each patient’s hospital record, signals can be found that correlate with response to treatm ent, and / or changes in disease. The team behin d the research are now ho ping to expand these studies across all the PH centres participating in the National Cohort Study of Idiopath ic and Heritable PH. If yo u would be interested in taking part in this study when the tim e comes, please speak to your spe cialist centre.

atch tw r a m s a in g n ti Inves sic makes £70 for the most ba

star t from around . Second-hand New smar twatches yone’s price points er ev to in fit t no may lower prices and models, which be found at much n te of n ca els od ed m or community watches or refurbish resellers like eBay, via or es sit eb w r’s around and on manufacture some time to shop g kin ta th or w s It’ ar t watch is Facebook groups. e. If a shiny new sm er th t ou ns tio op the ved ive deals across all research the pre-lo as, look out for fest tm ris Ch is th t lis s. le top of your evitable January sa hang on for the in ps major retailers, or of excellent free ap ere are a number th ch at ck tw ar tra sm to a u Even without ts, which allow yo ding MyHeart Coun clu in e, on ph ur yo for e walk tests. perform six-minut your activity and WINTER 2021 emphasis 37

Wondering what financial support you might be entitled to?

r o t a l u c l a C s t fi e n Be ch and Grants Sear le b tools now availa rg o at www.phauk.

ould access c u o y if t u o d in F , charitable welfare benefits pport that u s r e th o d n a ts gran ing out on. s is m e b t h ig m you

WINTER 2021 emphasis 38

In association with Turn2Us rg.uk www.turn2us.o

# HEA

R o u vo r

Jane Price

ices

Family Matters

“CONNIE’S ILLNESS HAS NOT STOPPED US LIVING LIFE” Connie Price was given a devastating diagnosis of pulmonary hypertension as a toddler - but four years later, the future is starting to look brighter. Here, her mother Jane remembers the route to diagnosis and reflects on family life in the shadow of this rare disease.

I

t was a bit of journey to get Connie’s diagnosis. When she was around 18 months old, I started to notice things that were different to her older sister Lucy [now ten].

She wouldn’t play with her friends, always wanted to be in the buggy, and was thirsty all the time. You could hear her breathing and she would get blue lips very easily.

I had no idea what it was, but I knew something wasn’t right. I went back to the doctors on a few occasions, and I kept getting told that everything was fine. Then one day, during Lucy’s birthday party, Connie came in from the garden and passed out in my arms. As quickly as she went down, she recovered, so we put it down to the heat outside.

But the next day, I took her to the doctors...

Continued overleaf >>

WINTER 2018 2021 emphasis 39

Family Matters ...with a list of everything I had noticed, and insisted they figure out what was going on.

A heart scan was arranged, but there would be an eight-month wait because it wasn’t deemed to be serious.

In the meantime, Connie caught a sickness bug which caused her to be hospitalised because her heart rate went through the roof, but still we had to wait.

We had a lot of support, but we “weren't ready to hear the diagnosis.

”

The scan date came around and I remember telling my husband not to come because I’d only be ten minutes at the hospital down the road. After 45 minutes of scanning her heart, it was obvious they had found something.

Suspecting a hole in the heart, another scan was carried out by someone from the Evelina [Children’s Hospital in London].

My husband was with me this time and with Connie out of the room, we were told it was either something mechanical which is easily fixable, some kind of cardiomyopathy, or pulmonary hypertension.

They followed it up with the words ‘we don’t want it to be pulmonary hypertension, and don’t Google it’. Obviously, we Googled it, and what we read was horrendous.

Dealing with the diagnosis Connie was admitted to hospital and that was first time we realised how ill she was.

When the PH was confirmed, we were transferred to Great Ormond Street Children’s Hospital and we entered the world of trying to get the medication right for her. I felt floored [by the diagnosis]. At that point it just felt so dark to be told that your three-year-old child has such a serious illness. We had a lot of support, but we weren’t ready to hear it.

We were living at Great Ormond Street at the time and our older daughter was living with my parents because we wanted her to have some stability.

Jon and I didn’t see each “otherMyforhusband more than an hour a day for three months, because one of us was always with Connie and the other was with Lucy. ” Reflecting back on when Lucy received her diagnosis of cerebral palsy, we were there together and that made us really strong. But when Connie was diagnosed with PH, we were passing ships in the night. It was really difficult because we wanted to deal with it together, but we needed to do what was right for both children at that point.

WINTER 2018 emphasis 40

Getting the treatment right Within three weeks of arriving at GOSH Connie went onto epoprostenol because her PH was so severe.

We were told that she needed to go on a Hickman line and at the time, I don’t think we registered that meant 24 hours a day, 365 days a year.

The line went in through the veins in her chest and into her heart and was connected to a very heavy pump.

We had this little three-year-old carrying this heavy pump on her back permanently, and Jon and I were in this world of making up her drug that had to be made and replaced at the same time every day.

That is a really hard deal because you’re then very closed off in terms of what you can and can’t do as a family.

We had to be home for a certain time, we couldn’t stay anywhere, and we couldn’t leave Connie with anyone who wasn’t trained to be able to trouble-shoot the pump.

We would literally be putting Connie’s life in their hands.

Connie’s never really responded to any of the medication in the way [the doctors] would have liked her to; she has always had a delayed reaction to everything.

Connie with mum Jane and sisters Lucy and Annabelle

Today, she is no longer on the Hickman line because she ended up having surgery.

The drug combination, which was the gold standard, wasn’t working - and instead of transplant, we were given the option to have a pioneering surgery called the ‘Potts shunt’ [a sideto-side connection from the left pulmonary artery to the descending aorta].

It had only been carried out once before on a child in the UK, and it carried a 15% risk of her not making it through the operation.

It felt like an impossible decision, but it was made for us when we were told there would probably be an 18-month wait for lungs and Connie probably didn’t have that long.

She sailed through the surgery but in true Connie fashion, it didn’t make a difference for a long time - meaning she remained on the Hickman line and the pump for another 18 months.

Today, Connie is on oral medications for her PH, as well as

# HEA

o u vo r

R

ices

Connie 2 weeks before diagnosis

In hospital

other tablets to help manage their side effects.

She has recently had one of her medications doubled and her last MRI scan showed a 10% improvement in her heart function, which was amazing and somewhere we probably didn’t think we would be.

Living with PH These days, Connie can just about make it up two flights of stairs, but she is very breathy when she gets to the top. She doesn’t really keep up with her friends and she finds that tough, especially if they are playing run-around games on the playground. She gets tired a lot and you can tell when she is struggling because her face will go quite sallow. But she is quite good at regulating herself and knows when to stop and take a breath.

We also have a two-year-old, Annabelle, and she has a specially-designed buggy that has a seat on it for Connie.

We try very hard not to limit Connie. We enjoy going on bike rides, to theme parks, exploring, and spending time with family and friends. She missed nearly four years of swimming because of being on the Hickman line, so we have a holiday with a pool booked for next year.

We have to be very careful with the language we use with the girls. Connie understands that her lungs don’t work, and that it makes her have a 'naughty' heart. She knows, to some extent, that the doctors can’t fix it but that they are doing everything they can to make sure it doesn’t get any worse.

We have worked quite closely with the psychology team at Great Ormond Street because one of my biggest worries is that the girls will Google it before we have a chance to put it in the right way for them.

With big sister Lucy

With her backpack

There isn’t a cure right now, but you don’t know what’s coming as things are advancing all the time. There is no deadline that we have to play to.

“

Connie understands that her lungs don’t work, and that it makes her have a 'naughty' heart".

”

Learning from our experiences My advice to other parents [faced with a diagnosis of PH] is to give yourself time to grieve. I think that was what was missing in my understanding back then.

You are grieving the loss of a future for your child in the way you expected it to be, but you will get through those first few weeks.

They will be scary, tough and frightening but you just need to allow yourself to feel it because if you start shutting yourself away at that point, it makes it harder to come out of.

If you want to cry, then cry. If you want to shout, then shout.

There is a world of help on Facebook groups, support groups and from the PHA UK. You probably won’t be ready for any of that in the first few weeks but it’s good to know it’s there in case you do need it. At the time, we didn’t know it was there and we felt very alone.

Having a child with PH is devastating and it does make me feel angry. But Connie is such a happy, giggly, little girl who absolutely loves to go on adventures, and her illness has not stopped us living life.

Playing at the park

Playing together in hospital

WINTER 2021 emphasis 41

theinterview

“Being kind to people is one of the highest things you can do as a human being” CONSULTANT SURGEON MR STEVEN TSUI in conversation with

Mary Ferguson

WINTER 2021 emphasis 42

Mr Tsui is a Cardiothoracic and Transplant Surgeon at Royal Papworth Hospital. After graduating from the University of Cambridge, he underwent cardiothoracic surgical training in Cambridge, Oxford, and at the prestigious Duke University Medical School in America.

Mr Tsui specialises in pulmonary endarterectomy surgery for people with chronic thromboembolic pulmonary hypertension (CTEPH) and has been part of the PH team at Royal Papworth since 1998.

Q. Did you always know that a career in medicine was for you?

A: As a child, I was very attracted to machines and engineering. But one day at school, I happened to walk past the television room where the BBC was sharing news of the UK’s first successful heart transplant. They were interviewing the surgeon, Sir Terence English, and I was mesmerised. How could you possibly take a heart from a dead person and give it to someone else, and keep them alive? I thought it was incredible, and straight away I told my teacher this is what I wanted to do. When it came to applying for university, I initially applied for engineering but then changed my mind and decided to challenge myself with medicine. I had an interview for Cambridge University, and this was the turning point in my career. It was only whilst studying and working at Papworth that I realised it was the same hospital I had seen performing the heart transplant on the BBC all those years ago, and that very same surgeon became my mentor. I was hooked.

Q. How did your career path lead you to where you are today?

A: After finishing my medical degree, I was fortunate

enough to get a training post at Papworth and completed my training in heart and lung surgery.

I started working as a consultant there in 1998 and was then fortunate enough to take over and run the transplant programme. To me, it was a fairytale that came true.

I know that to pursue a career like this it takes a lot of hard work and commitment, but I have to say that along the way a lot of my friends and colleagues have had as much, if not more, talent and commitment than I have.

I think I was just lucky, as well being in the right place at the right time, when a job came up.

Q. Can you tell me about the different surgical aspects of your role?

A: The difference between pulmonary endarterectomy (PEA) and transplantation is almost two extremes.

PEA is 99% of the time a planned operation, so those with CTEPH have plenty of time to consider all the treatment guidelines, and they then go onto a waiting list, so we can plan these things ahead of time.

I carry out one PEA every week and we have four surgeons here [at Royal Papworth] who are part of the programme.

We average four a week [between us], so 200 a year.

WINTER 2021 emphasis 43

“During a PEA operation, I feel almost like the conductor of an orchestra, conducting a very complicated symphony”

Heart and / or lung transplantation is something which only happens when we are offered a suitable donor organ.

We can’t predict or plan for these things.

Sometimes we have gone three or four weeks without any suitable donor organs and then suddenly we may get three in 24 hours - so for that programme, we have a team of staff who are on duty 24/7.

The PEA side is very predictable [in comparison] and, in a way, even though it is a massive and complex operation, it is something I find immensely enjoyable and relaxing because I am in full control. I feel almost like the conductor of an orchestra, conducting a very complicated symphony.

It takes a whole day and I’m in charge of the whole team, directing everybody, and it is well rehearsed. It is very rewarding when you know you’ve done a good job.

Here at Royal Papworth Hospital, we are the only PEA centre in the UK. We’ve got surgeons from all over the world coming to learn from us, and [the service] is very much one of the jewels in the crown of the NHS.

Q. Who has inspired you most in your life and career?

A: There are two people who have really inspired me.

When I look back and wonder why I considered becoming a doctor when I originally wanted to be an engineer, I think of a very good family friend, David Tse. When I was 13, my father was diagnosed with cancer and given three months to live, and although family associates were courteous and tried to be supportive, I felt they distanced themselves a bit.

But David, who also happened to be a doctor, supported us and held our hands through the difficult times. It wasn’t his medical skills, but his very humane approach and kindness to us as a family in need that touched me.

It doesn’t matter how clever you are; I think being kind to people, particularly when they are in need, is probably one of the highest things you can do as a human being.

The other person who has inspired me is of course Sir Terence English. I’ll always be very grateful to him because once I

WINTER 2021 emphasis 44

started my training at Royal Papworth, he very much took me under his wing, and it was he who arranged my research fellowship in the US. He was genuinely very interested in my career and really helped me.

I didn’t tell him that it was watching him on TV that inspired me to become a surgeon until he was long retired, and I was running the transplant programme at Royal Papworth. I’m sure I’m one of many thousands of youngsters who were inspired by him.

Q. How do you feel when you look back on your career so far?

A: People have different pictures of how they see surgeons, and I think a lot have the impression that we must be really stuck up, difficult people. But we’re here to serve and it’s a real privilege to be able to.

It is tough and a lot of responsibility, and it’s not a job for everyone - but it’s my passion.

I’m very lucky that my journey has taken me so far and at this point in my life, I can’t ask for more. It’s a miracle for the schoolboy who saw that news report all those years ago.

w o d n i A w world to our

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By Shaun UK A H P e h t t a t r o p Membership Sup

es, from the PHA UK offic te da up r la gu re s hi In this new podcast - bringing r ou es uc od tr in n au Sh es… magazine to the airwav

"This time around I wanted to talk about a project that we've been wanting to work e on for some time, but for on reason or another we just couldn't... until now!

EmPHAsis on Air is a podcast launched by us here at the PHA UK. The aim was to create something that was fun and light-hearted but that would also bring you in formation, advice, stories y. and interviews along the wa is In fact, it’s very similar to th g very mag azine you're readinto - which is why we decided give it the same name.

New episodes are available every fortnight and they

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The easiest way to make sure Like with anything we do you never miss an episode is here at the PHA UK, we're to like and subscribe to the always keen for input from podcast, but we'll definitely our members. If there's be posting links to new a something you' d like to hear episodes on our social medi us discuss on our podcast, . pages too. please feel free to let us know 've we k ac db fee e th So far, m You can email us at had has been great - my mu office@phauk.org or give loves it ;-) us a ring on 01709 761450. by ed st ho is ast dc po e Th Happy listening!" mysel f, and PHA UK team , members Mary and John e with special guests along th o way. If you're wondering wh Shaun, Mary and John are,

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including r streaming platforms, ajo m e th l al on le lab ai s episodes on these EmPHAsis on Air is av n listen to all previou ca u Yo . ify ot Sp d an ts you hit the Apple Podcas the latest, make sure iss m to nt wa n’t do u platforms, and if yo at www.phauk.org find the latest episode so al l u’l Yo n. tto bu ’ ‘subscribe

WINTER 2021 emphasis 45

# HEA

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Peter Beech

In sickness and in health

Peter Beech has been married to wife Betty for 59 years, and his support has taken on new importance since she was diagnosed with pulmonary hypertension. Here, the 80-year-old from Nottingham explains his role in their changed life together.

I

t took a couple of years for Betty to get her diagnosis and it was quite stressful watching her struggle with her symptoms when we didn’t know what the problem was.

When we were told that it was pulmonary hypertension, I think I was probably in shock more than anything else. All I could think about was that it was such a serious condition, and I didn’t really understand what it was, or whether anything could be done.

After speaking to a nurse consultant, the situation was made a lot clearer.

I don’t think you realise the impact that a diagnosis like this has.

On reflection I would say that it made me more concerned about Betty and what her capabilities were; what she couldn’t do and what assistance she required.

I found that initially quite stressful.

We’ve always shared our duties, but Betty couldn’t do anything herself. She found walking and breathing difficult and I felt I couldn’t help her to relieve those symptoms.

I have to manage everything now.

On a number of occasions, she has tried, but it’s been too much for her.

It’s been very difficult to find out

WINTER 2021 emphasis 46

what is available in terms of social care.

Betty’s got district nurses who attend twice a week for a leg ulcer, and they put me in touch with a charity for carers, who sent me some literature about what helps would be available if we needed it. It made me realise that as much as I could get on by my own to begin with, there are places and people who are prepared to help you.

The hardest thing for Betty is trying to maintain some form of dignity. She can’t shower herself, or dress herself, as she can’t stand for long. She needs practical help, which I provide, but she gets frustrated that that is unable to do the things she is accustomed to doing.

I can leave her at home alone to go shopping but I’m very conscious that I’ve only got 90 minutes because her medication means that she needs to go to the toilet after that time, and I have to help her with that.

Despite all of this, I don’t think of myself as a ‘carer’ for Betty.

We’ve been married for 59 years, and this sort of situation puts you into a different light. But I don’t use the terminology of ‘carer’ unless I need to explain why I need to accompany her somewhere.

Betty

We have family who are extremely good, and I can ring them whenever I need to. I also have an old work colleague who phones me every week and always offers his help.

I don’t have time to pursue my own hobbies or interests because of the need to help Betty with the toilet so often.

I used to go and watch cricket, but I can’t do that now, so I watch it on the TV instead. I do walk the dog once a day, which gives me a little bit of a break.

We used to go out once or twice a week for a meal, but we can no longer do this due to Betty’s deteriorating health.

It’s very difficult to try and give advice to others in our situation as they may feel differently. But I would certainly say it’s helpful to have a good understanding of exactly what the condition is, the medical terminology around it, and what effect it can have on a person.

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Shelley Higham

“As long as I have got my family and friends, that is all I need” Shelley Higham was diagnosed with PH at the end of 2019. Here, the mother-of-two from Lancashire reflects on her diagnosis and explains how it has altered her outlook on life.

The family to

gether

I

was working as a retail assistant when I started noticing something wasn’t right. I was getting breathless, but I tried to ignore it, and I put off going to the doctors.

But then I started getting chest pains and would be gasping for air when I went to bed at night.

At first, I was told it was pneumonia, but my legs started swelling and when the chest pains got bad at work, my employers called an ambulance. Tests showed my legs were full of fluid, which was rising to my chest.

CT scans then revealed pulmonary hypertension and heart failure. It was December 2019, close to Christmas, and by this time I had been experiencing symptoms for a year.

It was heart-breaking to be told I had an incurable disease, and one that I had never heard of.

I take two types of oral medication and have to use oxygen for 16 hours a day. I’m also on a BiPAP (ventilator) machine overnight.

I had to leave my job, which was a hard thing to do, but I just couldn’t be walking up and down stairs or operating the tills.

PH does affect me quite a lot because I feel like I am restricted when I go out. I have two children, Callum (21) and Jack (12), and when they go out with my husband Gareth I often have to stay at home because the oxygen makes it so difficult. I can’t walk far, because I know I will get out of breath.

I’m lucky to have a lot of support from my family.

My parents only live around the corner and my husband is fantastic. He does everything around the house, and I couldn’t be without him.

Having PH has changed my outlook on life, and I never take anything for granted now. I appreciate what I have got, and although I don’t know if I will ever work again, money is nothing. As long as I have got love and my family and friends, that is all I need.

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# HEA

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Pam & Graham Fenton

g n i t r a st e k i l s a w “It

” p i h s n o i t a l e r w a ne

Pam Fenton was diagnosed with idiopathic pulmonary arterial hypertension in 2018, and her husband Graham gave up his job to help care for her.

The great-grandparents, who live in East Yorkshire, shared their story for PH Awareness Week to encourage more understanding and earlier diagnosis.

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Pam is 65 and lives with a Hickman line delivering medication to her heart. She is on oxygen therapy for 16 hours a day and also takes multiple tablets and inhalers.

“I had never been ill in my life until 2013. I couldn’t walk up the stairs, I was blacking out, and I put on four stone of water weight. I used to cry; a newborn baby can breathe, so why couldn’t I? I didn’t get my diagnosis for five years, and in some ways, it was a relief to know there was a reason for it all.

I was broken during those years before diagnosis. They were the worst years of my life. I couldn’t go out, so people stopped inviting us, and they didn’t believe I was as poorly as I said I was.

People don’t understand how nasty and horrible they’re being.

Pam and Graham with family on the day they renewed their wedding vows Before I was diagnosed, doctors were telling me I had asthma or angina. Eventually, after I started leaking water from my legs, I was blue-lighted to hospital, where I blacked out. I was referred to the specialist centre in Sheffield where I was told I had PH and started on the Hickman line shortly afterwards.

Now, I have good days and bad days. But the medication has made a big difference and thinking about ‘before’ and ‘after’ helps me realise that.

In December 2017, five months before I was diagnosed, Graham and I took two of our great-grandchildren to a Santa’s grotto and when we got there, I couldn’t walk far enough to watch them with him. I was heartbroken.

A year later, after I’d started on medication, we went back and I was able to watch them with Father Christmas, plus a reindeer and a donkey. I had to take my oxygen with me, and it took me about three days to recover, but I did it.

There’s still a lot I can’t do, but I try.

It’s hard to explain how much the breathlessness affects me.

Sometimes I can’t get a sentence out and sometimes I can’t get out of bed. It’s very difficult to walk from one room to another without having to rest.

Sometimes the more I know about this disease the more it frightens me, and other times the more I know, the less it frightens me.

Having PH makes me feel physically and mentally tired, but also grateful to be alive. I am determined that I am going to do as I’m told [by my medical team] and do the best I can.”

Pam with her granddaughter (above) and greatgranddaughter (right)

Graham has been married to Pam for 25 years.

He gave up his job to support her when her symptoms worsened in 2015.

“PH has turned our lives upside down. In some ways we’re closer, yet sometimes we can be distant as well. We’d been so active together, and suddenly it was like hitting a brick wall.

It was like starting a new relationship, and we had to become more honest with each other.

I didn’t understand the disease at first and I was looking at her and thinking ‘how am I going to cope with this?’

I had a very good job with a good bunch of people but as time went on, I began to realise that I was going to have to give up work to look after Pam. There was a sudden shock of being unemployed and not getting up at six in the morning to go to work, and then trying to establish a routine that suited Pam. The first few days we did nothing but sleep. I was a bit lost.

I was left in the dark a lot [by our local hospital] but once we got referred to the specialist centre, I was included in the conversations and some of the lights started to come on.

I help with the medication side of things, but it can be very daunting if you don’t know what you’re doing. I do the pump [for the Hickman line] every so often, and I sometimes wonder if I’m doing it correctly. My confidence can take a beating if I do it wrong, but I know that our specialist centre is always there to help.

Now I can just look at Pam and see that she needs a rest or a drink. I do the shopping - even though I dislike it - and sometimes you feel like you’re just fetching and carrying.

It’s the realisation that I’m now a carer… what a word.

It can make me feel tired, sometimes even lonely, as I’m doing things I wouldn’t have been doing if I’d been at work.

It’s difficult to carry on with it and understand I have to do it for my wife’s health and not for myself.

She was getting her confidence back and then COVID-19 came along and that took the wind out of her again. But now that things are improving, we are getting out a bit more.

We can’t go far because of the oxygen, and it is so tiring for Pam that we have to justify what we do because of the impact on her. It’s difficult, but the treatment is helping.”

You can watch a video of Pam and Graham talking about their lives with PH at www.phocusonlifestyle.org WINTER 2021 emphasis 49

YOUR PHARMACIST is there for you this winter Local pharmacies can help with more than you may think, as Neil Hamilton explains…

I

was fortunate enough to have a week off over half-term which allowed me to have a week on the beautiful Gower Peninsula near Swansea. Having done my pharmacy degree in Cardiff, I always relish the opportunity to go back to South Wales.

For us, The Gower was a different bit of coast for my family to explore and enjoy and I would definitely recommend it. It was a beautiful time of year for colourful countryside or blowy coastal walks, and it was a time to make the most of the outdoors before winter came.

My first week back at work was our PH Awareness Week, where the PHA UK focuses their on-going efforts even more sharply to raise the profile of this rare condition. I’m sure that many of

Did you know?...

you will have been busy supporting this important week in the calendar.

For my profession, the same week was also our national ‘ask your pharmacist’ week. The theme of this year’s campaign was ‘your local pharmacy can help’, raising awareness of the variety of problems to which community pharmacy has solutions.

People’s positive experiences of community pharmacy during the coronavirus pandemic has raised expectations of what their local pharmacy can offer them.

Pharmacy teams work with other professionals, such as doctors and nurses, to give you the best possible care as part of the local healthcare team. The range of clinical services provided by community pharmacies

Pharmacists train for five years in the use of medicines, and many pharmacies have a private consultation room where you can discuss issues without being overheard.

WINTER 2021 emphasis 50

has expanded significantly in recent years, not least with cholesterol checking, blood pressure monitoring and of course vaccination.

This year, more than ever before, you should give yourself the best protection against both COVID-19 and flu, by ensuring that you are vaccinated against both conditions.

Colder weather and darker nights means increased social contact indoors, which favours transmission of respiratory viruses. Both COVID-19 and flu have the potential to cause serious illness and hospitalisation, especially for those who are clinically vulnerable.

NHS England has recently undertaken some survey work, which showed that some people are underestimating the combined threat of COVID-19 and flu this winter. Nearly a third of those surveyed were unaware that both can circulate at the same time, and over a quarter did not realise that flu can be fatal.

PH specialist Neil Hamilton is a Consultant Pharmacist within the Sheffield Pulmonary Vascular Disease Unit at the Royal Hallamshire Hospital.

Not many people caught flu last winter, so there isn’t so much natural immunity in the population this time around. In addition, we can all socialise with less restrictions this winter, so vaccines will be vital in protecting our loved ones and ourselves.

Making sure everyone who is eligible has the flu vaccine as well as the COVID-19 booster has become a priority for the NHS and there are national advertising campaigns reminding everyone, with very good reason.

Flu, like COVID-19, can be an extremely debilitating and unpleasant viral illness for the fittest in society and unfortunately those people with other conditions will fare even worse.

Having pulmonary hypertension may not only put you at greater risk of catching flu or COVID-19, but it will have a far greater impact on you.

Indeed, your body may really struggle to fight it off and this will almost inevitably have an impact on

flu jab programme is the largest in history, with it being offered to 35 million eligible patients. Once again this year, there is an egg-free flu vaccine, which is great news for those with allergies or who are vegan.

The flu jab is available free on the NHS to people who: • are 50 and over (including those who'll be 50 by 31 March 2022) • have certain health conditions • are pregnant • are in long-stay residential care • receive a carer's allowance, or are the main carer for an older or disabled person who may be at risk if you get sick • live with someone who is more likely to get infections (such as someone who has HIV, has had a transplant or is having certain treatments for cancer, lupus or rheumatoid arthritis) • frontline health or social care workers

“For anyone with PH unfortunate enough to come down with a nasty cold, or even flu, the local pharmacy should be your first stop for helpful advice” your breathing. I cannot emphasise enough how sensible it would be for PH patients to ask their GP, nurse, pharmacist or specialist centre about both jabs.

Much of what we have heard so many times regarding reactions to COVID-19 vaccines apply also to the flu jab. Not many people have serious reactions or side effects, but some people feel a bit ‘fluey’ for a day or two. This can often be easily controlled with a few doses of paracetamol if necessary. This year’s

Even if you don’t fit any of this NHS criteria, I know my local pharmacy is selling private flu jabs for only £10, which is hopefully not too expensive for most to afford.

For anyone with PH unfortunate enough to come down with a nasty cold, or even flu, the local pharmacy should be your first stop for helpful advice.

Not only is your pharmacist available without an appointment, but pharmacies are usually open longer hours than a GP surgery, so should be much more accessible.

The pharmacist will be able to advise you which cold/flu remedies will be best for you.

In terms of PH-specific advice, I would advise against patients with PH taking decongestants such as pseudoephedrine (found in Sudafed and other products) as this constricts blood vessels. I also advise to avoid anti-inflammatories such as ibuprofen. Whilst one-off doses may be harmless, these can cause problems with your kidneys and possibly worsen breathlessness.

Whenever you seek advice over the pharmacy counter, it is important that you take along an up-to-date list of current prescribed medicines so that the pharmacist can avoid any potential interactions.

Although I have mostly talked about the upcoming winter, pharmacy teams work with other professionals, such as doctors and nurses, to give you the best possible care as part of the local healthcare team all year round.

So, remember to ask your pharmacist for advice across a whole range of healthcare themes.

.

Your local pharmacy can help with many issues, including:

✓ Advice and treatment for minor illnesses such as coughs, colds and earache

✓ Advice on staying well and preventing disease ✓ Support to maintain good sexual health ✓ Help to quit smoking

✓ Personalised support to get the most from your medicines

WINTER 2021 emphasis 51

Li fe a fter m y tra nspl ant 23 years of memories Julie Warner’s heart and double lung transplant saved her life, and she will always be grateful to her donor. This is her extraordinary story.

I

was 37 years old when I was diagnosed with idiopathic pulmonary hypertension and I was listed for transplant on my 38th birthday, in July 1995.

I was on the waiting list for two years and nine months. I was given a pager to carry 24/7 and advised to have a bag packed ready for admission. I continued to work full time as a District Nurse Team Leader and my employers were very supportive at that time.

The first 15 months of waiting was a period of adapting both physically and emotionally.

I was put in touch with a monthly support group for pre- and post-transplant patients at my hospital and I attended as often as I could.

The information and advice I got from these sessions certainly helped in preparing for surgery and my postoperative recovery. The experiences of the post-transplant patients were invaluable for me, and I still keep in touch with one friend that I met there.

support of my parents and good friends I was able to remain mostly positive, but inevitably I had down days the longer I was on the waiting list.

On my 40th birthday, in July 1997, my parents organised a garden party at their home for family and close friends. It was a beautiful sunny day and they worked hard to make it so special. I will never forget it.

Starting to struggle

By January 1998 I was really suffering with my symptoms, both physically and emotionally. I was reassessed but nothing could be offered.

By the time I had been on the list for 17 months I was struggling, and I was admitted to hospital for two weeks with heart failure and low oxygen levels.

Intravenous medication eased my symptoms and once home I needed a minimum of 18 hours of oxygen therapy each day, so I had to take continuous sick leave from my job whilst I waited for the transplant.

This was a particularly difficult time to adjust to emotionally as I thought it was going to be the end of my life. With the

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He explained my heart would recover in time, once relieved of the PH, so it could help someone else in need. I didn’t hesitate in consenting if it meant someone’s life could be saved too. Plus, it felt like I was giving something back.

The recipient of my heart was a man who had suffered many cardiac arrests.

I received a letter from him afterwards, expressing his gratitude, and we continued to exchange occasional letters and transplant anniversary cards until he sadly died ten years later.

Undergoing surgery

Julie with her parents, at her 40th birthday party whilst on the transplant list

Becoming a ‘domino donor’

I met with [renowned cardiothoracic surgeon] Professor Magdi Yacoub at this time and expressed how my quality of life was suffering. He spoke to me about offering to be a ‘domino donor’ of my heart – which meant donating my own heart for transplant when I received another.

On 2nd May 1998, I had an alert from my pager. I rang the transplant co-ordinator and was told there was a match available, and I had two hours to get to the hospital.

I was accompanied by parents and my best friend, Barbara, and went into theatre at midnight.

The surgery went smoothly, but, as in a lot of cases, when the donor organs are handled it can cause them to swell a little - but once transplanted and allowed to rest, the swelling subsides.

This happened to me, so I was on life support for longer and my chest wasn’t fully closed for three days. I was ventilated for 11 days in total, but for others it’s quicker.

I spent six weeks gradually recovering and getting stronger. I initially noticed the shortness of breath had subsided and as I recovered, the dreadful fatigue had gone, and my energy levels increased by the day. I was able to manage longer walks, climb stairs with ease, and almost run up inclined paths! I restarted swimming which helped build up my strength further.

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Julie Warner

Julie (in the blue dress) dancing with friends at her 60th birthday party Getting back to ‘normal’ life

I couldn’t return to work until 1999 as I had to wait for my driving licence to be reinstated, but it gave me extra time to rebuild my strength and enjoy some of the social life I’d missed out on.

I continued my full-time role as a District Nurse Team Leader for ten years, until tremors in my hands (a side-effect of the anti-rejection drugs) forced me to take early retirement. The tremors haven’t stopped me living life independently; I just adjust my routine to manage.

The life-reliant medication I have been taking since my transplant isn’t without potential side effects, but I undergo regular screening including blood tests, heart scans every five years, and an annual meeting with a pharmacist. I also have access to the wider multidisciplinary teams at my hospital if required.

Making memories

Since my transplant I’ve enjoyed a healthy life and I’ve not had any rejection problems in the last 23 years, so I know I am extremely lucky.

I have had many wonderful holidays abroad with friends, had fun times at pop concerts and festivals, and enjoyed theatre trips and memorable social gatherings with family and friends.

I’ve been able to do all of this with energy and enthusiasm instead of feeling fatigue, shortness of breath and needing oxygen therapy.

In the year 2000 my friend nominated me for a local ‘achievement over adversity’ award, which I won. I was interviewed by the local media and presented with my award by the hostage survivor Terry Waite. It was my few moments of fame!

Julie with her close friend's grandchildren, who call her 'Nana Juju' pandemic and times of shielding. I am now looking forward to making more happy memories in the future.

Receiving a 'triumph over adversity' award from Terry Waite I have passed a few milestones, including my 50th and 60th birthdays – which was an excuse for a party on both occasions. My 60th party was a celebration I’ll never forget, and we all danced the night away in my garden.

Looking to the future

I have been lucky to have some good friends of many years who have given their love and support when I needed it and I thank them all for helping me stay positive.

My close friend Barbara has been with me from the start of my PH journey, and I shared the joys of her children growing up. I am a close part of their lives now and her daughter Milly’s children affectionately call me ‘Nana Juju’. I love them dearly and I’m lucky to have them in my life. They are all like family to me and helped support me through the

Julie on her 60th birthday

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g n i s i a r d n Fu

S U C O F

Walking for John Sue Locke and friends from the Letter B pub in Peterborough have completed another successful sponsored walk in memory of Sue’s husband John. The committed group have been striding out together every year since 2017, raising thousands along the way. Sue said: “I have some amazing friends and supporters, who look forward to doing it every year. It is definitely more of a social event now.”

£1150D

RAISE

The sky’s the limit for Lucy Fearless fundraiser Lucy Draper from Rugby leapt out of a plane to help us in support of her mum, who was diagnosed with PH after years of suffering with blood clots.

£ 34SE5D RAI

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Some of th amazing w e you’ve beeays supportin n over the l g us few montast hs

Harrison finally gets his medal Harrison Bradford finally got his IRONMAN medal, following multiple cancellations due to the coronavirus pandemic. Harry was originally due to complete an IRONMAN event in France in July 2020 and transferred from race to race as the crisis raged on. It was worth the wait, as in October this year he crossed the finish line at the epic Mallorca IRONMAN challenge - tackling a 2.4 mile swim, 112 mile bike, and 26.2 mile run to raise an amazing amount of money in memory of Andrew Kitson.

£15IS0E0 D RA

Trekking the Thames Bridges Helen Richards and friend Tracy Gigov completed the 26km Thames Bridges Trek in support of Helen’s niece Alex (pictured), smashing their original target of £200.

The route took them across London, zig-zagging across 16 historic bridges.

£13IS6E8D RA

Coast-to-coast couple Colin and Vivien Nutter trekked 190 miles from St Bees to Robin Hood’s Bay to raise over £700 in support of their future son-in-law. David Stott was diagnosed with PH out of the blue in 2013 and the couple walked across the country, from the Irish Sea to the North Sea, to raise awareness of his condition and generate funds.

£71E2D

RAIS

£15IS0E0 D RA

Birthday challenge exceeds targets Scottish supporter Roy Dewar set himself a very special challenge for his 77th birthday, and thanks to the generosity of family and friends, he almost doubled his fundraising target along the way.

Roy, who was diagnosed with PH in January, walked non-stop for 25 minutes every morning in the lead-up to his big day, raising awareness as well as funds.

£93SE0D RAI

Half marathon success for Nic Nicola Englefield ticked off a personal goal, as well as raising money, when she completed the Weston Super Half in October. Nic signed up to the 13.1 mile challenge after taking up running during lockdown, and she chose to take on the challenge in memory of her mother-in-law Sue.

Scaling Snowdon for Amlyn Sean Hickey trekked up Snowdon in memory of his daughter Amlyn Sultan, who would have turned 26 just before Christmas. Sean was joined by her boyfriend and friends for the challenge, and together the group raised over £900 in her name.

£19SE5D RAI

r #21in21 s Week u o y w o h Find out for PH Awarenes age 8 s p challenge raising funds on n have bee

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

WINTER 2021 emphasis 55

Finding her

brave

Abbie Hampshire has lived with PH since 2018.

Now five, she is making the most of life with her health conditions, as mum Andrea explains…

Abbie at hospital

Relaxing at home

L

iving with pulmonary hypertension is hard and we know that supporting a child to live their best life with PH is even harder. Every normal routine is affected and trying to keep to time is near impossible.

Ask any of us; it feels like we are living in organised chaos every day and we wish we had the arms of an octopus.

But we never stop trying, fighting, supporting and encouraging to make sure our children live a near-to-normal, happy life. We have been on our PH

WINTER 2021 emphasis 56

journey since 2018 with our five-year-old daughter Abbie, and it has been a bit of a rollercoaster since her diagnosis. She also lives with severe scoliosis, lung disease and delayed physical development - to name a few.

We still don’t know why she has the degree of PH that she does and since 2018 she has undertaken countless tests and scans to find a cause, but to no avail. This means it could be a genetic cause and it also means that her PH will progress, get trickier to manage as she gets older, and could be life-limiting.

We have locked that devastation in a little box in the back of our minds and now focus all our attention on hope for the future and making sure Abbie and her two older brothers are happy in their present lives - that they feel supported, and that Abbie has the emotional resilience to tackle what challenges life will bring her.

We value our home life and the stability and safety net it gives to her, and we encourage the boys to join in with activities and clubs for their own wellbeing. School, church and community youth and sports groups are all good influences in their lives.

So, she goes to mainstream school every day. This is important to her and something we have valued as a great achievement.

Our morning routine is unlike many doing the school run as she needs to be supported to get dressed, refasten her spinal body brace, toileted, medication given and encouraged to eat breakfast.

It all takes longer than normal as she needs to do everything at her pace.

She also arrives at school with what

feels like everything but the kitchen sink. She brings with her school bags, a medical bag, q walking frame and a powered wheelchair. She is greeted by a teacher from her class when we arrive who gives her a welcoming ‘hello’ and she is helped into school, barely waving goodbye to me as she goes in.

In school she also has a supportive chair, oxygen concentrator and access to an inclusive nurture room for when she gets a bit fatigued. They also know to give me a call when she is struggling to manage so she never ends her day feeling like it was too much. Her mental

“We take the little wins as big celebrations and enjoy them” health is good because she has positive relationships with adults and children around her, she is encouraged to talk about how she feels, and takes part in school life as much as possible.

Attending many hospital appointments has just become another part of life.

Abbie does find them upsetting and her fight mode will kick in despite the wonderful work of the doctors, nurses and play-therapists who try to make the experience easier for her. She is well-known in many hospitals in North Yorkshire and of course at Great Ormond Street.

We often look for our ‘brave’ before we step through the doors. We try to talk about being brave as something

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Andrea Hampshire

With her dad normal, something that we all have but sometimes we forget where we put it, so we have to go looking for it, like hide and seek. She finds it funny when it was in her pocket the whole time!

On weekends and during holidays we try to keep our family experiences as normal as possible and are creative in how we figure out access for her. We like to be out in the fresh air, so we go to places that are outdoors or that are accessible activities; places that we know she feels able to enjoy because it is familiar.

We take things with us, so she is able to rest and watch her brother Joey play football or cricket with Daddy, or enjoy the natural world around her. Family time is important for us all, and we all do our share in helping Abbie to thrive, so we take the little wins as big celebrations and enjoy them.

Making sure her brothers get quality time is important too, so they often do things with Daddy like adventure walks, fishing or garden camping. Looking after our wellbeing as a family is essential; although it can be difficult at times, we remember to reflect in her strength and keep going.

Evenings are much the same as the mornings; time consuming and a little like military precision. She takes nine different medications between teatime and bedtime, and she takes overnight oxygen to give her a boost for the next day.

She needs help to get into the house, put her school things away, and read her book. She is helped to rest, encouraged to eat at teatime, carried up the stairs, supported in the bath and helped to get ready for bed.

Her big brother Freddie has taken on helping her brush her teeth for a school ‘toothbrush challenge’ and it has been a joy to watch them together. Evenings can often get swept away with the routines, so when Abbie is finally in bed, we like to simply get comfy on the sofas together

With her brothers Joey and Freddie

With her mum Family selfie

Outside school for a chat, a bedtime read or a sneaky bit of TV. Friday nights are a more relaxed time, so we often play family charades which is one of Abbie’s favourite games.

She can do a great chicken impression!

We try to balance everything in our daily life, keeping up with appointments and managing her health needs; attending school and doing homework;

prioritising family time and not being hard on ourselves if something doesn’t work out. We are together on this journey; the up and the downs, for Abbie, because she is our little PHighter.

When she smiles because she has managed to do something she didn’t think she could do, the world just seems to light up. She is finding her brave.

WINTER 2021 emphasis 57

# HEA

R o u vo r

ices

Nerys Jones

Former counsellor Nerys Jones was told she had pulmonary hypertension in April 2021, a decade after her symptoms began.

Here, the 68-year-old describes her difficult journey to diagnosis and how she is adjusting to the impact on her life.

WINTER 2021 emphasis 58

O

ver ten years ago, I started coughing and having difficulty breathing when climbing stairs and inclines or hills. I was diagnosed with asthma, and two chest x-rays showed lung scarring and a slightly enlarged heart, but I was told there was nothing to worry about. I was too frightened to ask further questions.

I was given inhalers which made no difference and my cough, and my breathing difficulties, became worse. Eventually, with a swollen foot, I was checked for deep vein thrombosis – but even after several blood tests I was told that everything was fine.

In early 2020, I pleaded with my GP for an endoscopy (a procedure that uses a camera to look inside the body via the throat) because I thought my cough was to do with reflux, and I had lost weight.

The endoscopy, which was looking for cancer, was clear but a CT scan showed pulmonary fibrosis. The planned referral to a respiratory consultant never happened because of covid.

At great expense, I saw a private functional doctor who treated me for small intestinal bacterial overgrowth and reflux, but my breathing became much worse over the months.

In desperation, after my legs, ankles and feet became swollen with water retention, I spoke to a new GP in March 2021. This is when things finally started moving.

Nerys and her husband Phil groups and read many positive stories. The staff at Sheffield were all amazing and so helpful, and it was a turning point in my PH. Although my medication is starting to make breathing easier, I still panic when I cough or get out of breath from walking too far, and it takes me a while to get my breathing back on track.

I’m supported totally by my lovely husband Phil, who does so much for me every day. He takes on all on the tasks I’d have done, such as washing and ironing, and I could not survive without him.

Our lives are much more restricted, with Phil having to do so much over the last few years.

Being on oxygen 24/7 affects everything, although we are

“I want to raise the profile of PH so that others get a

quicker diagnosis I was admitted to the acute ward at my local hospital and after lots of tests, I was officially diagnosed with scleroderma, pulmonary fibrosis and pulmonary hypertension.

Finally, the seriousness of my symptoms had been recognised, and I was eventually transferred to a specialist PH centre in Sheffield.

I was frightened by the diagnosis and what I read via Google, but I joined the PHA UK and various Facebook

With three of her grandsons

working out how to manage it at home and when we go out.

I’m determined to keep doing some of the activities we enjoy such as gardening, looking around other gardens that have limited inclines, and going out for coffee and cake - although we do have to check disabled toilet facilities first because of my water tablets.

Phil and I have two lovely daughters and five grandsons, but it’s difficult to visit them as it’s a four-hour journey and I’d need to organise oxygen supply.

I’ve now got a blue badge for my car which is useful, although I haven’t driven for months. I’m hoping to soon though, as I do feel I’ve lost my independence.

Other people can’t really understand my PH and the effect it has on both my psychological and physical well-being. I’ve always been the one to help others and now I feel I’m a burden, especially to my husband.

We’re having to get used to people looking at me with my oxygen. If I have the opportunity, I tell them why I’m on it as I really want to raise the profile of PH so that others get a diagnosis much quicker than I did.

WINTER 2021 emphasis 59

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In the next issue of Emphasis... Spring

the We’ll be bringing you March 2022, issue of Emphasis in n look and here’s what you ca forward to :

Menopause and PH pport Specialist advice and su Hospital chaplains healthcare Their important role in s Yoga for mindfulnes ith PH Helping you live well w Plus, lots more articles, interviews and news…

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Emphasis Review sor seen a film you read a book, used an app th interested in? Tell ink other readers may be us about it!

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otos Thanks to everyone who shared ph 21 20 in support of PH Awareness Week