Breathless not voiceless | What it means to live with pulmonary hypertension today

LIVING WITH PH TODAY

BREATHLESS, NOT VOICELESS

2023 Report published November

What it means to live with pulmonary hypertension today

LIVING WITH PH TODAY Welcome to the results of the UK’s largest ever study into the lived experiences of pulmonary hypertension.

This is the PHA UK’s fourth major survey into the impact of this rare disease, and we’d like to thank everyone who took the time to complete it.

The findings in this report reflect the experiences of 859 people with pulmonary hypertension, representing a 35% increase in the number of responses received in 2017.

It is clear that the PH community wants to be heard, and this research provided a platform for their voices.

Please read on and listen.

Dr Iain Armstrong Chair, PHA UK

LIVING WITH PH TODAY

CONTENTS ABOUT THE RESEARCH WHAT DOES A PH PATIENT LOOK LIKE TODAY? KEY FINDINGS DIAGNOSIS LIVING WITH PH TREATMENT AND SPECIALIST CENTRES DISCUSSION: WHAT’S CHANGED, AND WHAT’S NEXT?

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ABOUT PH Pulmonary hypertension (PH for short) causes high blood pressure in the blood vessels connecting the heart and lungs (the pulmonary arteries).

In a healthy cardiovascular system, the right side of the heart pumps blood into the lungs to pick up oxygen and this oxygen-rich blood is then pumped around the body by the left side of the heart.

When a person develops PH, the walls of the pulmonary arteries become stiff and thickened, or blocked by blood clots. This makes it difficult for them to expand; and trying to pump blood through these tightened, narrowed, scarred or blocked arteries puts increasing strain on the right side of the heart as it tries to do its job.

The essential task of pumping blood into the lungs to pick up oxygen which can then be circulated to every cell in the body becomes much harder.

Pulmonary hypertension is rare.

Around 8,000 people are diagnosed with PH in the UK. It can affect anyone, regardless of age or ethnic background.

It is more common in women than men.

Watch this short animation to understand the symptoms of PH

ABOUT THE PHA UK The Pulmonary Hypertension Association (PHA UK) is the only charity in the UK dedicated to supporting people affected by PH.

Its support network helps patients and those around them too – including family, friends, and carers. Set up in 2000, the PHA UK gives a voice to the PH community.

Find out more about the charity at www.phauk.org

859

ABOUT THE RESEARCH The 2023 Living with PH survey was launched in December 2022 and responses were gathered * between then and June 2023 .

responses were received:

*Data only. The quotes and videos included in this report were gathered by other means during 2022 and 2023.

This represents around 10% of all those living with PH in the UK. Similar surveys have been conducted in 2007, 2010, and 2017.

ABOUT THE RESEARCH Who completed the survey and how?

The survey asked for the experiences of all adults with PH living in the United Kingdom.

It was available to complete online, and hard copies were sent to all PHA UK members.

It was also made available to patients on PH-specific targeted therapy through a network of homecare delivery

services. This means that it wasn’t only PHA UK members who completed it.

The results showed patient characteristics very similar to those found in the UK National Audit of Pulmonary Hypertension, representing patients normally seen in a clinical setting.

34%

completed it online

66%

returned hard copies

What does a UK pulmonary hypertension patient look like today?

73%

identify as female (27% identify as male)

identify as White

(4% Asian, 2% Black, 1% Mixed race)

(average age at diagnosis)

56 64 93%

DEMOGRAPHICS

(average age at the time of completing the survey)

KEY FINDINGS /DIAGNOSIS PEOPLE ARE STILL WAITING JUST AS LONG TO BE DIAGNOSED WITH PH AS THEY WERE 15 YEARS AGO

For 51% of people, it took over a year to be diagnosed with PH after first noticing symptoms. For 24% of people, it took over three years.

32 %

of people with PH had to see four or more doctors before receiving their diagnosis.

KEY FINDINGS /LIVING WITH PH PH IMPACTS MANY AREAS OF PEOPLE’S LIVES

96 84 79 78 60 %

of people with PH say the condition has an impact on their overall quality of life

%

say it has an impact on their mental or emotional wellbeing

%

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

%

say it has an impact on their relationships or family

%

say it has an impact on their financial situation

KEY FINDINGS /TREATMENT AND SPECIALIST CENTRES SATISFACTION LEVELS AROUND PH CARE ARE VERY HIGH

91 92 %

of patients describe their PH care as excellent or good

%

say it’s better to travel to a specialist PH centre than to be under the care of a non-PH specialist at a more local hospital

52 %

of patients said that when it comes to PH treatment, improvement in overall quality of life is what matters to them most

LIVING WITH PH TODAY

DIAGNOSIS /DELAYS PEOPLE ARE STILL WAITING JUST AS LONG TO BE DIAGNOSED WITH PH AS THEY WERE 15 YEARS AGO.

DIAGNOSIS /DELAYS FIRST NOTICING SYMPTOMS

For 51% of people, it took over a year to be diagnosed with PH after first

noticing symptoms.

For 24% of people, it took over three years.

DIAGNOSIS/DELAYS

35 EMERGENCY ADMISSIONS

%

were admitted to hospital in an emergency because of their symptoms, which led to them being diagnosed with pulmonary hypertension.

Of those not admitted to hospital as an emergency due to their symptoms, 40% waited over a year for a referral to a PH specialist from their GP.

When I saw my GP about my symptoms I was referred to an asthma nurse. I knew I didn’t have asthma, but because of the pandemic, having a spirometry wasn’t possible.

I was then given a long covid referral…

It was all very frustrating because I felt like the doctors I saw thought I was making it up.

DIAGNOSIS/DELAYS SEEING A DOCTOR FOR THE FIRST TIME

33% waited over a year

from first noticing symptoms

before seeing a doctor.

(15% waited over three years)

SPECIALIST REFERRALS

Of those who were referred to a local (non-specialist) hospital,

31% waited over a year

for a referral for specialist pulmonary hypertension care.

DIAGNOSIS /DELAYS I had symptoms for a good couple of years.

Doctors thought % it was asthma and we went through a long process of changing different inhalers, using steroids, and nothing really improving.

Kate*

*Names have been changed

I was looking up the symptoms on Google, wondering is it lung cancer?

Are there other things that are seriously wrong that doctors aren’t picking up on?

32 %

Kate’s partner Paul*

of people with PH had to see four or more doctors before receiving their diagnosis.

DIAGNOSIS /DELAYS In their own words: Watch nine people with PH reflect on their diagnostic delays

LIVING WITH PH TODAY

DIAGNOSIS /EXPERIENCES PEOPLE WERE SATISFIED WITH HOW THEY WERE GIVEN THEIR DIAGNOSIS

DIAGNOSIS /EXPERIENCES We all sat around the bed to hear the results of the final test. We were told that [my adult daughter] had a very rare illness called PH and that it was life threatening and there was no cure.

To be honest, being diagnosed was a relief to me, because I finally knew what was wrong.

65% of people were given their PH diagnosis by a specialist PH consultant. 33% were given it by a consultant at a local hospital.

79% agreed that overall, they were satisfied with the explanation of their diagnosis.

(40% ‘strongly agreed’) 77% agreed they had the time and opportunity to ask questions around their diagnosis.

(41% ‘strongly agreed’) 60% felt the person/ people with them (such as a family member or friend) were involved in the conversations around their diagnosis and were given the chance to ask questions too.

(30% ‘strongly agreed’)

DIAGNOSIS /EXPERIENCES

56 32 18 INFORMATION RECEIVED AT THE TIME OF DIAGNOSIS

%

of patients were given information about the PHA UK

%

of patients were given information about the effect of PH on work and education

%

of patients were given information about financial support

DIAGNOSIS /EXPERIENCES The diagnostic odyssey: Watch PHA UK Chair Dr Iain Armstrong discuss the impact of these findings

LIVING WITH PH TODAY

LIVING WITH PH

PH IMPACTS MANY AREAS OF PEOPLE’S LIVES

LIVING WITH PH Andy describes his diagnosis as a ‘life changer’.

Watch him talk about the impact of PH in this video.

LIVING WITH PH

96

EVERYDAY LIFE %

of people with PH say the condition has an impact on their everyday life. 54% state it has a ‘major impact’.

96

QUALITY OF LIFE %

of people with PH say the condition has an impact on their overall quality of life.

50% state it has a ‘major impact’.

87

CONCERNS ABOUT LIFE EXPECTANCY %

of people with PH say the condition has an impact on their concerns about life expectancy. 44% state it has a ‘major impact’.

A lot of people don’t understand what it means to have PH and how it can affect your life.

I have feelings of sadness that life expectancy is shortened and I’ll not have chance to see the future.

LIVING WITH PH I have social anxiety, so I didn’t go out much anyway, but PH has made the anxiety worse. It’s also the low mood. Thinking about the future is difficult.

I have been struggling with severe depression since my 20s, and I am now 50. Everything is a struggle.

Since being diagnosed with PH all of these feelings have increased ten-fold. The struggle and lack of motivation is now as much a physical problem as a mental one.

I think mine was a mixture of depression and PTSD [Post-Traumatic Stress Disorder] following diagnosis of PH.

MENTAL & EMOTIONAL WELLBEING 84% of people with PH say the condition has an impact on their mental or emotional wellbeing.

34% state it has a ‘major impact’.

LIVING WITH PH Lou has had her husband by her side since she was diagnosed with Chronic Thromboembolic Pulmonary Hypertension (CTEPH).

Watch their story.

RELATIONSHIPS OR FAMILY

78% of people with PH say the condition has an impact on their relationships or family.

28% state it has a ‘major impact’.

LIVING WITH PH I can’t pick my little girl up or play with her properly. I used to love having baths with her but I can’t anymore [because of the Hickman line] and I cant go swimming with her either.

I look at couples walking along the street holding hands, and I feel bad that my husband and I can’t do that because I’m in a wheelchair. I feel bad that we can’t do fun things as a family.

Over the years, my wife has lost contact with most of her friends due to the last-minute cancelling of planned events because of her fatigue.

I try and play with my kids as much as I can. I don’t want them to go through life without a proper father there.

LIVING WITH PH WORK AND EDUCATION

79% of people with PH say the condition has an

impact on their ability to work or attend education.

41% state it has a ‘major impact’.

My wife used to work full-time and gradually had to reduce her hours to a point where she had to give up work completely.

LIVING WITH PH I had always planned to % work until state pension age, but since being diagnosed with CTEPH, every year it got harder. After being on sick leave for six months, I officially left work on ill health retirement..

Steve*

Ill health retirement has left Steve with less than half of what he was bringing home each month when he was working. I was made redundant the year before and because I had my redundancy pay, we didn’t feel as financially exposed.

But without that, goodness knows what we would have done.

Karen*, Steve’s wife

*Names have been changed

LIVING WITH PH

FINANCES

60% of people with PH say the condition has an

impact on their financial situation.

25% state it has a ‘major impact’.

I had to give up my job and the next big hurdle was the benefits system.

The process went on for months, but eventually, after going to a tribunal, I got what I was entitled to. It was exhausting.

LIVING WITH PH TODAY

TREATMENT AND SPECIALIST CENTRES SATISFACTION LEVELS AROUND PH CARE ARE VERY HIGH

TREATMENT AND SPECIALIST CENTRES

My care has been excellent.

I’ve definitely seen the best of the NHS.

TREATMENT AND SPECIALIST CENTRES Jo is under the care of three different healthcare trusts, due to her PH and other conditions.

Watch her discuss her treatment, and experiences with different providers during the COVID-19 pandemic, in this video

TREATMENT AND SPECIALIST CENTRES

I have felt involved in treatment decisions

I know who to ask if I have questions or concerns about things like side effects and dosing

37% Strongly agree, 38% Agree, 16% Somewhat agree, 4% Somewhat disagree, 4% Disagree, 2% Strongly disagree

48% Strongly agree, 37% Agree, 9% Somewhat agree, 2% Somewhat disagree, 2% Disagree, 2% Strongly disagree

75% agree

The choice of treatments were explained to me

78% agree 40% Strongly agree, 38% Agree, 13% Somewhat agree, 3% Somewhat disagree, 4% Disagree, 2% Strongly disagree

85% agree

My family / carer / friend / those who support me know who to ask if they have questions about my treatment

68% agree

31% Strongly agree, 37% Agree, 16% Somewhat agree, 7% Somewhat disagree, 7% Disagree, 3% Strongly disagree

TREATMENT AND SPECIALIST CENTRES WHAT MATTERS MOST

MEDICTION DELIVERY

OXYGEN PROVIDERS

52% of patients said that when it comes to PH treatment, improvement in overall

86% of patients are

92% of patients on long-term oxygen are

Life expectancy = 33% Control of symptoms = 14% Lack of side effects = 3%

(The company that delivers PH medication to their home)

quality of life is what matters to them most

‘satisfied’ or ‘very satisfied’ with their homecare delivery company

‘satisfied’ or ‘very satisfied’ with their oxygen provider

TREATMENT AND SPECIALIST CENTRES/IMPACT CENTRES 48%

24%

38%

34%

30%

23%

of patients say their PH treatment and management has had a major impact on their overall quality of life (45% ‘some impact’, 8% ‘no impact’).

say their PH treatment and management has had a major impact on their concerns about life expectancy (47% ‘some impact’, 15% ‘no impact’).

say their PH treatment and management has had a major impact on general mental / emotional wellbeing (51% ‘some impact’, 18% ‘no impact’).

say their PH treatment and management has had a major impact on their relationships / family (51% ‘some impact’, 26% ‘no impact’). say their PH treatment and management has had a major impact on their ability to work or attend education (41% ‘some impact’, 25% ‘no impact’).

say their PH treatment and management has had a major impact on their financial situation (35% ‘some impact’, 43% ‘no impact’).

My specialist team have seen my highs, my lows, and everything in-between.

They have saved my life.

TREATMENT AND SPECIALIST CENTRES /SATISFACTION The National Audit of Pulmonary Hypertension (a care quality measure carried out by NHS Digital) states that people with PH should be seen at least once a year by a PH specialist. think this is appropriate for their needs

72%

78% SPECIALIST CENTRE CONTACT

think they have enough contact with their specialist PH doctor or nurse

I believe the specialist PH service is one that the NHS could learn a lot from. As a patient I have felt fully supported and comforted by all those I have had contact with.

Everything is geared towards supporting me as an individual.

TREATMENT AND SPECIALIST CENTRES /SATISFACTION

48% DISTANCE

of patients travel over 50 miles to visit their specialist PH centre

21%

of PH patients use hospital-provided transport to attend their specialist centre appointments.

Despite this, 92% say it’s better to travel to a specialist PH centre than to be under the care of a non-PH specialist at a more local hospital.

However, 42% say they have difficulty travelling to appointments because of physical health.

27% say they have difficulty travelling to their appointments due to cost.

TREATMENT AND SPECIALIST CENTRES /SATISFACTION Since the COVID-19 pandemic, appointments with PH specialists now take place either face-to-face or over telephone or video call.

We asked people when they feel a face-to-face appointment would be more appropriate, in order of importance, and this is what they said:

1. When I feel my condition is changing 2. When I’m struggling with symptoms or my condition 3. To seek reassurance my condition is stable 4. To discuss new treatments

90%

of patients describe their PH care as excellent or good 62% Excellent, 29% Good, 8% Fair, 2% Poor

My specialist centre has really helped me, which has made a big difference, and I have a lot of trust in them.

TREATMENT AND SPECIALIST CENTRES /RESEARCH I get involved in trials whenever possible… I do what I can to give back for the help I’ve had from the NHS. I remember medical research opportunities being mentioned to me pretty early on, and I saw lots of advantages. When you are diagnosed with a condition like PH, I suppose you either lay down and accept the cards you’ve been dealt with, or you try and fight it a little bit – and getting involved in research is my way of doing that.

Less than half of PH patients (42%) say they have been asked to take part in medical research by their PH team.

However, 76% say they would welcome being asked.

LIVING WITH PH TODAY

DISCUSSION

WHAT’S CHANGED?

This survey shows that people with PH are still significantly impacted by their condition, and that they place a high value on specialist treatment and care.

The key findings that are significantly different compared to 2016 all relate to people’s experiences of being diagnosed with PH.

The responses to other questions asked in both surveys are largely the same.

DISCUSSION /WAITS FOR DIAGNOSIS

In 2016, 10% of patients told us they waited over three years to be diagnosed with PH.

This has trebled to 32% in 2023.

DISCUSSION /REFERRALS FOR DIAGNOSIS In 2016, 21% of patients waited over a year to be referred to a hospital by their GP after reporting symptoms.

This has doubled to 40% in 2023.

In 2016, 16% of patients waited over a year to be referred by their local hospital to a specialist hospital.

This has doubled to 31% in 2023.

DISCUSSION /INFORMATION GIVEN AT THE TIME OF DIAGNOSIS

At the time of diagnosis in 2016

69%

of patients were given information about support groups

59%

DOWN

13%

DOWN

of patients were given 27% information about the effect of PH on work and education

40%

of patients were given information about financial support

DOWN

22%

At the time of diagnosis in 2023

56%

of patients were given information about the PHA UK

32%

of patients were given information about the effect of PH on work and education

18%

of patients were given information about financial support

DISCUSSION In 2023, our survey /PARTICIPATION was completed by UP In 2016, our survey was completed by 563 people.

10%

completed it online

859 people.

% 5 3

34%

completed it online UP

24%

WHAT’S NEXT?

The results of this research will help us set our strategic priorities for the next five years, underpinning our charitable activity and determining the direction of the PHA UK. By sharing their voices in this survey, patients have helped us to help them.

The findings will also be used as an evidence base to campaign for access to new treatments or policy changes, and to protect specialist service provision.

We will be conducting sub-analysis of these findings to dig even deeper, and add to the significant value of this data set.

Thank you to everyone who responded to this survey.

People with PH may be breathless, but they’re certainly not voiceless.

QUESTIONS? If you have any questions about this report, please contact office@phauk.org