PAH & Pupils

Produced by the PHA UK with input from Great Ormond Street Children’s Hospital

PAH& PUPILS INFORMATION FOR SCHOOLS

A resource to help you understand more about the condition your pupil lives with.

Important contact details Your pupil’s name:

The specialist nurses at Great Ormond Street Children’s Hospital can be contacted on 02074059200 (ext 1007) or at phnurses@gosh.nhs.uk Page 2

Pulmonary arterial hypertension (PAH) is a rare condition which affects people of all ages, including children. If a pupil at your school has recently been diagnosed, or is new to the school, you probably have lots of questions – and possibly some concerns too.

This resource aims to help you understand more about the disease, how it affects children, and how you can help support your pupil.

It has been produced by the Pulmonary Hypertension Association (PHA UK) in collaboration with Great Ormond Street Children’s Hospital (GOSH).

PAH at-a-glance

Fast facts for staff • PAH restricts oxygen flow around the body and puts pressure on the heart • The most common symptoms are breathlessness, fatigue, and dizziness • Treatment varies from tablets to intravenous therapy • There is no cure for PAH • Children with PAH may feel self-conscious or isolated

IMPORTANT Children can be affected differently by pulmonary arterial hypertension, so your pupil will have individual needs.

The information in this resource is intended as a general guide, and we have left space to make notes specific to your pupil.

• Frequent medical appointments are required • You can’t tell a child has PAH just by looking at them • Around 400 children have PAH in the UK • PAH is often referred to as PH (short for pulmonary hypertension). There are different types of PH, but PAH is the most common in children.

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s i t a h W al pulmonary arteri

H) hypertension (PA

It’s a serious condition that causes high pressures in the blood vessels connecting the heart and lungs (the pulmonary arteries). This pressure restricts the flow of oxygen around the body and puts increased pressure on the heart.

Some children develop PAH out of nowhere and this is known as ‘idiopathic’ PAH. For others,

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There are other types of pulmonary hypertension (known as PH) but PAH is the most serious.

When a person develops PAH, the walls of the pulmonary arteries become stiff and thickened.

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.

The pulmonary artery in a healthy person

The pulmonary artery in a person with PH

The artery stretches in response to an increased heart rate

The thickened artery cannot stretch enough in response to an increased heart rate

*Number of children receiving treatment for PAH, according to The National Audit of Pulmonary Hypertension 2024.

PAH is a rare disease, affecting around 8,000 people in the UK. Around 400 of these are children*.

it may be linked to congenital heart disease or some other health conditions.

The symptoms of PAH In most cases, you can’t tell that a child has PAH just by looking at them. Some children may be pale, with a blue-ish tinge to their lips, but it is generally an ‘invisible illness’.

Symptoms can occur at rest, but more often during periods of movement, such as walking around, climbing stairs, or taking part in PE.

Dizziness

Shortness of brea th

• Breathlessness • Feeling tired or fatigued • Dizziness

The nature of PAH means symptoms tend to vary day-by-day. It is possible that your pupil will experience these symptoms every day, but it’s important to speak to them and their parent / guardian to ascertain what is ‘normal’ for them as an individual. That way, you can look out for changes that may indicate that help is needed.

e r fatigu o s s e n Tired

The most common symptoms are:

Some children with PAH may also experience chest pain, or swelling in the ankles, arms or stomach area. Fainting (blackouts) are also experienced by some.

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In their own words...

What it’s like going to school with PAH “I can’t go out when it’s raining heavily because of my line and dressing. Sometimes people don’t include me or are mean.

I have to miss school a lot for appointments but my teaching assistant helps me catch up.

I have to be careful when playing outside and sometimes I get tired. I can’t go to swimming lessons because of my line and I can’t always do the same things in PE.

Teachers and others who work at school can make it better for pupils by knowing what PAH means, understanding it’s hard when I miss out, being sensitive to the fact that sometimes I am tired and being more patient in helping me catch up with work.”

Ivy, 9

“It’s hard not being able to do as much as everyone else in PE. My teachers make sure I’m alrig ht if I look like I’m not doing what I normally would do. They let my parents go on trips to change my medication so I can go away with my year group.

They let me leave lessons earlier if I need it to get to the next lesson in time, and I have a lift pass if I want to use it.”

Charlie, 15 Page 6

“The hard parts of having PAH at school are the questions I get asked from others about my line and condition and I struggle to answer. When having trouble with my symptoms my teachers allow me time to rest and help with the alarms on my pump.”

Amelia, 9

“Not being able to keep up with my friends at break and lunch times can be hard. PE lessons can be really hard and I have to stop a lot. If it’s too cold or too hot outside, I need to be inside to help me breath more easily. I worry I won’t be able to go on trips if I am not thought about and planned for carefully.

My teachers know me really well and can see if I am working too hard. They remind me to take rests or they work out another way for me to do something so I can join in .

They make sure someone is with me if we are being really active so that I am safe.

School staff can help by thinking carefully about where my classes are so I don’t have to walk really far or climb lots of stairs. Get to

know me. You can tell how well I am by looking at my face or hearing how breathy I am - but you won’t see or understand it if you don’t know me. Get to know what’s normal for me.

Realise that just because I look like all the other kids, I am not. My lungs and heart don’t work the same and I need to be able to go at my own pace.

I might be late to class, I might not be able to complete everything, I might be really tired a lot of the time but I promise I am trying really hard - it’s just I also have to work really hard just to breathe.”

Connie, 11

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What are the signs that your pupil is struggling with their symptoms or that something may be wrong?

Things to look out for include an increase in breathlessness beyond what’s ‘normal’ for the child, complaints of feeling light-headed, or experiencing fast heart rate (palpitations).

Blacking out is also an obvious sign that something is wrong.

If you think the child is struggling, make contact with their parent / guardian and if at all concerned, ring 999. In the event of a blackout, place the child in the recovery position and if it’s not something that happens regularly, call 999.

Specific notes about your pupil...

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The following school-day situations may cause symptoms to flare and so may need to be avoided:

t utside a t Going o ery ho me in v i t k a e r b ather cold we y r e v r o nificant (and sig ges ure chan t a r e p m te inside)

Taking p art in some PE c lasses (this will vary betw een individua ls)

g Climbin ng ki l a w stairs or es to n up incli ooms or access r es faciliti

Unexpe cted stressfu l situatio ns Page 8

How children are treated for PAH All children with this condition are treated by Great Ormond Street Children’s Hospital, which is based in London.

Outreach clinics take place around the UK but there are times when a visit to London is required, and this can result in absence from school.

Treatment can involve one or a mixture of the following:

• Oral medication (tablets) • Nebulised medication (where medication is inhaled through a mouthpiece or mask).

If your pupil is on this type of therapy, you will receive additional specific information.

• Intravenous medication (where medication is delivered into the body 24/7 via a line into the chest). If your pupil is on this type of therapy, you will receive additional specific information.

Treatments slow disease progression and ease symptoms but there is no cure for PAH.

Some children take water tablets (diuretics) to counteract fluid build-up in the body. This means they will need to be excused from class to use the toilet more often. Additionally, some PAH medications can cause children to need to defecate more frequently.

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Absences from school The emotional impact of PAH Having PAH can affect children emotionally. They may feel left out or isolated because they can’t take part in PE or run around with their friends at breaktimes. They may also feel self-conscious or frustrated about being ‘different’.

Children with PAH must attend regular medical appointments, and sometimes this will include stays in hospital. This may result in frequent or prolonged absence from school.

It’s important to remember that children with PAH are still children, with the same social and emotional needs as their peers.

It’s really helpful to encourage other children and young people at your school to be understanding of PAH and how it affects their fellow pupil. If you would you like any resources to help with this, please contact the PHA UK by emailing hello@phauk.org.

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Get to know the PHA UK Influence, Hope, Integrity.

We are the only charity in the UK dedicated to supporting people (of all ages) who are affected by pulmonary hypertension. Our support network helps patients and those around them too – including family, friends, and carers.

Scan the code below right to find out more about us.

How your school can help our cause

Our charity receives no government funding, so we rely on donations and fundraising to help us continue our work. Could you consider holding a non-uniform day or bake sale to support us?

PH Day UK takes place on the first Friday in November. Our annual initiative is designed to raise the profile of PH – and it’s the perfect time for you to get involved with your own activities.

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Hear from parents about some other children with PAH… Archie, 9, was diagnosed with pulmonary arterial hypertension when he was 4.

These words are from his mum, Sarah.

“Archie’s main symptoms are breathlessness and fatigue.

He falls asleep in school sometimes, and he sits down while the other children enjoy playtime. He can’t really do much at all, compared to other children, and that’s hard to see.

But despite everything, Archie just gets on with things.

He’s so inspiring, and such a character. One of the hardest things about Archie having PH is that no-one has heard of it, and he doesn’t look like he’s ill.

People don’t understand why he can’t do what other kids do, because he looks like there’s nothing wrong.”

Sarah Page 12

Fatima, 11, was diagnosed with pulmonary arterial hypertension when she was 5.

These words are from her sister, Maryam.

“Fatima has definitely adapted over time, but it has stopped her from doing a lot of things.

She’s at the age now where she is coming to terms and understanding what PH really is, and she can see how it affects her body more now because she is getting older.

You can’t really expect children to understand fully thoug h because even as an adult I sometimes can’t get my head around it.”

Maryam

Abbie, 5, lives with pulmonary arterial hypertension alongside other conditions including scoliosis, lung disease and delayed physical development. These words are from her mum, Andrea.

“Abbie goes to mainstream school every day. This is important to her and something we have valued as a great achievement…

Her mental 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.”

Andrea

Connie, 7, was diagnosed with pulmonary arterial hypertension at the age of 3.

These words are from her mum, Jane.

“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.

It is really difficult to get across what’s wrong with Connie, because for all intents and purposes, she looks just like any other little girl.”

Jane Page 13

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Independently developed by the PHA UK with input from Great Ormond Street Children’s Hospital, and independently reviewed by primary school staff.

Registered Charity No. 1120756. Copyright © 2025 PHA UK. Produced July 2025. Next review date July 2028. Version 1.0

Further copies of this publication are available by emailing hello@phauk.org.

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