Heartline Families Autumn 2013 No.1

Autumn 2013 No.1

Out with old... in with the new!

HeartLine gets a new name and a facelift!

Inside: Preparing your child for surgery, real life experiences Warfarin versus Aspririn, PGL adventure photos, and more

What’s inside 2

Farewell HeartLine, Hello Heartline

19-20 Against All Odds

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What you need to know about Heartline Families

21-22 Preparing Your Child For Surgery

4

Water Babies!

5-7

A Casual Stroll - Luke’s 100km Walk

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Help us make a difference

9-10 Time of Our Lives - PGL 11

Heart of Stars

12

What We Have Achieved

13

Summer Party! / Meet The Team

14

Coping With CHD Diagnosis

15-17 Warfarin Versus Aspirin Discussion 18

Supermum Does It For Thomas

23-24 Heart News 25

Sporty Arlo’s Special Award

26

Heartfelt Thanks

27-28 Jennifer’s Story 28

Meet The Team: Forum Moderator

29-30 Living in a Grown-Up Heart World

Heartline Families is edited by Natalie Persoglio Please send articles, photos and feature ideas by email to trustee1@heartline.org.uk TERMS USED: Some of our stories feature medical words. If you don’t understand the meaning of a medical term used, please see www.heartline.org.uk/termsused for a complete directory.

Farewell HeartLine ...

Hello Heartline Families!

We can hardly believe HeartLine has been in existence and helping to support heart families for more than 36 years! Hazel Greig-Midlane remembers.

Thirty years ago, I was pregnant and sitting in my very quaint local maternity hospital (The British Hospital for Mothers and Babies) when I saw a poster on the wall advertising HeartLine.

My middle child, Luke, was then two and a bit, and had very recently been found to have an endocardial cushion defect (AVSD to you!). A group to support families had just been started at Guy’s hospital – I had been to the first meeting and intended to stay in close touch with other parents to learn as much as I could, but this HeartLine had already been established for four years, so was worth a look.

We operated in those days mainly through post and landline – astonishingly different from the connections people make today.

Heart Children

Shortly afterwards I contacted Tricia Pope, a founding member and the then Chair, and within a few weeks was answering questions re benefits for families with disabled children for an updated version of Heart Children.

Since then I have attended many conferences, edited buckets-full of leaflets and magazines, been a communications adviser, representative, trustee, vice-chair and chair.

HeartLine Office Closes

The charity waxed and waned over the years – large conferences in London where families were bussed in from Birmingham were replaced with fallow times as the families running services ran into trouble and office space was closed to us.

We operated with Area Contacts many of whom are still members today, and did amazing work in raising funds, talking to new families, and running social events in their hospital, county and beyond. Heart Children was our main source of income for many years, and the main source of information for parents in the civilised world in the pre-Google age.

My main involvement with HeartLine started with Hugh Ballantine Dykes, then the Chair, setting up a staffed office in Camberley some 18 years ago.

The picture (right) shows the HeartLine area contacts, many of whom are members still today.

Although communications were slow, it matched to some extent the much longer average period we spent with our children in hospital or awaiting surgery. Quite straightforward operations required up to a week on ventilation, and weeks recovering on the ward, so parents had long periods of time as hospital residents.

And sadly there were far more bereaved parents among our members.

Amazing People

There are many amazing people who have been involved with HeartLine over the years. I must mention Anya Rowson who was treasurer and then chair, and was so important establishing support through social media – the Heartline Forums.

Source of Wisdom

Today these Forums have an enormous amount of information and wisdom. Although many, including Heartline, now use Facebook as first point of contact, the Forum stands as a place where you can return to earlier conversations, re-establish contact, and continue to support when your child becomes adult.

I personally have found it a great source of comfort when dealing with our own problems.

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Farewell HeartLine ...

Hello Heartline Families!

Continued

And we were blessed with Adelaide Tunstill – a founding member and Cardiac Liaison Nurse at Great Ormond Street, a friend and adviser, who for many years was available on the end of the telephone to answer general and specific questions posed by families throughout the UK.

What You Need To Know About Heartline Families

A big welcome to our new and old members you are now part of Heartline Families!

Although our name has changed and we’ve had a small facelift, we still offer the same support and benefits to you.

If you were already a HeartLine member you don’t need to do a thing as you are automatically added to our database.

Register now - it’s FREE

If you haven’t yet registered with Heartline Families, it really couldn’t be any more simple and it’s free, so join our other 2,300!

Just visit www.heartline.org.uk/joinus and complete the online form.

Joining Benefits

Once you have been accepted as a family, you will be given access to the ‘members only’ area of our website, where you will be able to get: - information about our caravan breaks - a free wetsuit for your child - stacks of information about dental hygiene, fundraising, hospital vists, the magazine and - access the forums, which carry a wealth of advice and discussion.

The charity HeartLine Association is no more, replaced by Heartline Families – a Charitable Incorporated Organisation, populated by those who joined HLA sometimes more than 30 years ago, sometimes last month - and new generations of families who we hope will benefit from the same high levels of support HeartLine has been renowned for.

You will also receive a copy of our magazine full of stories, news and innovations in the heart world.

More information at www.heartline.org.uk

Have you joined our community on Facebook and Twitter yet?

Come along and join us for up-to-the-minute information, news and discussion - all in your newsfeed. Facebook gives you the perfect opportunity to talk and become ‘friends’ with parents and families in similar situations with children who have similar conditions. Make sure you ‘like’ our page at www.facebook.com/heartlinefamilies and also on Twitter www.twitter.com/heartlineuk.

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!

S E I B A B WATER

FREE Wetsuits For Heartline Families’ Kids!

Children with heart conditions often can’t maintain their body temperature when swimming or playing in water, even in heated pools, but we don’t think this means they should have to miss out!

Heartline Families offers a free wetsuit for a child who is a registered as a member on the database.

All applicants will be checked by the Office before wetsuits are ordered and sent out.

Please send the following information via email to admin@heartline.org.uk (put ‘wetsuits’ in the subject line) or call 03300224466: - Name and address of parent - Name and age of child - Size (see information below) - Preferred colour choices (see below) Classic baby wetsuits come in sizes 0-6 months, 6-12, 12-18, 18-24 months, 2-3, and 3-4 years.

Please tell us your first and second preference for colour: pink, black, red, aqua, navy For older children (or larger 3-4 year olds!) you need to measure around the largest part of the child’s chest and from neck to crotch in inches. Colour choices for older children are: pink, blue, red or green.

We love to see our wetsuits in actions, so please send pics of your little ones in the water!

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a

casual STROLL

My 100km walk along the Thames for Heartline. By Luke Greig-Midlane. grab a quick cup of something which smelled eerily similar to coffee. I then filled out the form on the back of said registration number, just in case my bag fancied something to read.

ME: Morning *****. Everyone here seems so enthusiastic – like walking into a park full of exclamation marks.

Feel a bit out of place!* ME: (To Many) As if I’ve already lost the maps they gave me half an hour ago! Gonna have to do today the Luke way. Oh yeah, whilst filling in that form I lost my maps.

This is my account of the day I took the Thames Path Challenge 100km walk for Heartline, peppered with the occasional text update I sent to friends (text messages are in green italics).

I’d been thinking about doing something along these lines for a while (walking for charity, not texting my friends), and finally settled on a nice stroll along the Thames around late April, just about the time everyone had stopped banging on about the London Marathon... I would’ve done that, but I run for no man/woman/cause/bus.

Wave your hands in the air!

About 8:30am(ish) I arrived at the starting point, registered, collected my number (to be pinned onto my person or bag – I went with bag), and

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I made my way to the starting pen, where there was a wacky Scottish MC dressed up as a stick of Blackpool rock, telling everyone how great they were before handing over to superfun warm up exercise woman, for a quick session of pogoing whist waving your hands in the air like you just don’t care. When The Rock asked if anyone was doing the 100k challenge alone I raised my hand, before realising it was just me and one other poor lady.

I quickly withdrew my raised arm and left her to get singled out and shown on the big screen. Thankfully I also managed to grab a new map for the first 50km. Felt like the smartest man on the planet explaining to the steward that I’d managed to lose it already.

And they’re off!

Then, the countdown. 10, 9, 8, you get the picture, and we were off!

Well, until we got to the bridge steps about three minutes away, where it all got very congested. We’re not making good time, I thought to myself. It was already raining fairly heavily, which was a bit of a bummer

as I’d done all my walking training during the fantastic summer we’d just had. When I’d visualised the walk when I signed up, it was a vision of strolling by the river in blazing heat, not queuing in the rain to get onto a bridge in Fulham, or wherever we were. Another gripe I had (we’re about 2km in now by the way), was having to zigzag around giant puddles. I just couldn’t help but wonder how many extra kilometres this was going to add to my admittedly already reasonably long journey.

ME: (To ‘L’) I’ve just dropped my water bottle in a muddy puddle. L ‘L’: (To ME) The puddle was probably 90% water anyway. Don’t be such a baby.

Having barely got into my groove, I stumbled upon my first welcome distraction, that being my friend Karen, who had decided to meet me at Hammersmith Bridge and walk with me for a bit. I was pleased to see her until a jogger passed us and offered us encouragement and said well done. She didn’t correct him, at which time I realised she was clearly just there to try and steal my kudos. At 10KM we reached Kew Bridge and she departed, safe in the knowledge that I was a tenth of the way through.

Support in odd places

Made it to the 12.5k stop at Richmond Deer Park. Having checked in and eaten some kind of raisin based snack, I spotted a scraggy looking chap on a bench drinking a can of super strength lager who made eye contact. Prepared for either a

barrage of abuse or to part with some small change I was shocked to hear him very politely shout “Well done mate! It’s great what you’re doing!”. It seems you get a better calibre of drunk in Richmond.

Next significant moment was reaching 25km, and preparing for my pre-paid lunch. Problem was, the 25km rest stop was about 30km in, so walking that extra 50 minutes or so having already promised my stomach we were in for a treat was painful. To add insult to injury, I heard a man, having been asked by another man about what was going on, reply “They’re doing a 50k walk”.

50k? Pfffft.

Champagne thirst-quencher?

When I finally got to the 25km (30km) rest stop, I managed to feast on various barbecued meats, a lot of cold pasta, and a fair old whack of grated cheese, which to be honest I was surprised to see as an option.

Still, show me a man who can walk 25km and turn down cheese and I’ll show you a vegan.

Annoyingly, I had to share my lunch stop with people finishing the 25km walk, crossing a finish line, getting a glass of champagne etc... actually, in hindsight, well done to them... but at the time I viewed them as a big bunch of lazy charlatans. Also, they got to drink their champagne at a reasonable hour. How much use will a glass of champagne be to me at 8am? None, that’s how much.

ME: (To Many) Only 68km left to go.

Easy peasy!

‘L’ : (To ME) Shut up and walk x Karen: (To ME) Huh? You drunk?

So I walked on, dreaming of my morning glass of champers .

Walked a bit more. Saw some people pulling up and applying treatment for blisters.

Dad: (To ME) Watford 0-0 Charlton ME: (To many) Watford0-0 Charlton Hannah: (To ME) I DON’T CARE ABOUT THAT

ME: (To Hannah) That’s too bad, because you’re getting the full time score later.

Doing it for the jelly babies

I actually laid down at the first rest stop and stretched my legs in very, very mild agony. My spirits were lifted, however, when I realised that amongst the energy drinks, bananas, Nutrigrain bars etc, was a great big pick ‘n’ mix area.

ME: (To Many) Free pick and mix at rest stop! It’s a bad day to be a jelly baby!

ME: (To Hannah) Full time Watford 1-1 Charlton

Armed with jelly babies I made my way forward. By this point the crowds had thinned out a little, and keeping other walkers in my line of vision of utmost importance. I have a gigantic fear of getting lost, which is a shame, because I get lost all the time. All. The. Time.

I have great defence mechanisms though, so I turned my fear into cockiness. I noticed that I was about to overtake a walker who had a 7am start time strapped to his back.

ME: (To Many) Just overtook a man who started at 7am. I think I heard him softly weeping as he read the “9:30am” on my back before I vanished onto the horizon.

lose my balance every 10 seconds, right on cue.

Chris: (To ME) We believe in you Nick: (To ME) Keep going Luke. We believe in you.

Sam: (To ME)We believe in you.

So I carried on, trying to follow the odd person wearing reflective gear.

Sadly a lot of them were only doing the last 50km, meaning they’d just started and were leaving me for dust... or whatever you call bits of wet mud that fly through the air.

As I was walking through long stretches of nothingness in the dark, worrying about getting lost, I hit my first wall: the mental wall.

I wondered whether I was actually going to make it. I could barely see the reflective signs, couldn’t make sense of the map and could barely keep my balance while walking down slippery muddy paths the size of your thumbnail that were precariously close to the river – how I didn’t fall in at least once is anybody’s guess. The idea of another 10 or so hours of doing this on my own was frightening to me.

I’d started mumbling angrily to myself – it would have been funny if it wasn’t so sad.

More walking

50KM! Almost there, in a way. I left the 50km rest stop, feeling good (legs admittedly aching a bit) and pumped up to get the last measly 50km out the way, already tasting that morning champagne. But then, something happened. Something I wasn’t and could not possibly have been prepared for... it got DARK. Not dark like in London when God turns all the lampposts on. Dark dark. I turned my head torch on, which was great because that meant I could see a small bright circle 2 feet in front of me. Somehow I was still managing to stand in every puddle, trip over every rock or tree root, and almost

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Are you alright?

Just before the 61km rest stop, I saw a sign, attached to a wall, pointing straight forward, yet it didn’t specify whether I was supposed to walk round the left side of the wall or the right side of the wall. I leant against the wall (maybe that was what I was supposed to do?) until I heard those three magic words: “Are you alright?”

My saviour had arrived. I explained that I wasn’t alright and that I was losing my mind a little bit and I’d never done anything like this before etc, etc, etc. This man, thankfully, decided to take pity on me and walk me to the next rest stop where, at the time, I thought I might pack it all in.

I asked, let’s call him Gary, if he’d mind me tagging along with him for the foreseeable future so that I didn’t get lost or die or, even worse, fall in the river. He agreed.

What a guy.

Jon: (To ME) How we looking??)

ME: (To Jon) It’s dark and I hate it

I accepted my medal and glass of champagne and my silver foil cape (I’d always wanted one of those – apparently you have to exert yourself in some way to get one) at the finish, and fell in and out of consciousness until my mum and dad arrived to let me sleep in dads car.

‘L’: (To ME*asleep*) If you’re not done I want my money back To sum up: The first half was a walk in the park, the second half was HELL. If you decide to take up a similar challenge, for the love of God take it seriously!

But I’m glad I did it, and managed to raise over £1000 for Heartline. Thanks to all who have contributed. My Justgiving account is still running for a little while, so should you wish to donate, here’s the address: www.justgiving.com/casualstroll

Nothing left in the tank

On the way to the 75km stop I hit my second wall – the physical wall. All I could feel was the pain in my knees.

If it wasn’t for Gary walking hastily ahead, and my fear of admitting weakness, I may well have just collapsed.

There was nothing left in the tank, yet I kept walking.

People tried to tell me it was determination, but I think by that point it might just have been force of habit.

We walked across a couple of slippery bridges (less fun than they sound, trust me), suddenly my legs have sprung back into action. Must be the as yet unfulfilled dream of drinking champagne at 8am in the morning.

We step into the last rest point – by this stage I’m freezing at every stop, yet heating up every time I zip my coat up when walking. So by this time I’m stepping in big wet puddles with every second step, treading in cow pat every two minutes.

Sam: (To ME) How you doing soldier?

I’m sure I told everyone to leave me alone.

We weaved our way through what seemed to be several hundred sleeping cows, whilst every time my laces touched each other I was convinced that was the sound of a crazy bull on its way to come and get me.

Gary started to wane a little, at which point he very gracefully suggested that I go on without him.

1.This guy had saved my whole walk, I wasn’t going to leave him behind 2. I had NO faith in myself not to get lost without him. ...and so we went on together. Through some empty fields as the sun came up, ever some dodgy fences, and, eventually, to that bloody finish line. 21 hours and 20 minutes. Better than I expected, even before I knew how hard it would be!

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Luke Greig-Midlane has had a lot of heart surgery, including a mitral valve replacement when he was 5 (in 1987 – that’s lasted well!), and a supply of pacemakers ever since.

Help us make a difference

Heartline Families supports families with children with heart disorders, whatever the condition, wherever it is treated. The support we offer covers all aspects of being a heart parent or heart child. We reply solely on funds from our members and supporters.

We are grateful for any support you can give us. Log on to www.heartline.org.uk and click the Donate Now Button or send a cheque.

Direct Debit - Could you spare a little each month?

Make a regular donation direct to Heartline every month, and if you pay income tax, Gift Aid it so that Heartline receives an extra 25% on your gift.

Payment to Heartline Families, Lloyds Bank Sort Code 30-96-96 Account number: 59276960

I would like to help Heartline and will make a Direct Debit of £………..........

Please find enclosed a donation of £…………........…

I want Heartline to reclaim tax on the above donation and understand that I must pay an amount of income tax or capital gains at least equal to the tax HeartLine reclaims on any donation in the relevant year.

Signed: ............................................................

Date: ..............................

Return this form to: Heartline Families, 32 Little Heath, London. SE7 8HU Alternatively, email these details over to admin@heartline.org.uk and start helping another family just like you.

Fundraising?

You could also help by jumping out of a plane, holding a coffee morning, a sponsored silence, or getting your school or workplace to choose us as their charity. We have a wealth of ideas and lots of ways to help support you in your fundraising! From hooded bears and balloons to collection buckets and stickers - don’t delay, contact us today! Admin@heartline.org.uk

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Time Of Our Li

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ives!

Our annual PGL adventure weekends for heart children and siblings go with a swing and here are the photos to prove it!

My 4th time! By Kevin Ford

My Teenage thoughts by Joshua Ford

This was my 4th time at PGL this year and my 3rd at

This was my 3rd visit to PGL this year, and staying in the quadrangle away from the rest of the site the first time for myself. Even though I was the oldest in my room I didn’t get my way all the time as it should be!

Aged: Never you mind!

Boreatton. There were 12 Heart Children 4 boys and 8 girls, and two Female Leaders, Sharon and Annette, both seasoned Leaders.

As usual, the staff were well organised on arrival and we were to stay at in the quadrangle at the bottom of the site, which isn’t as bad as it seems, as it connects to most of the routes across site (and we had a lounge to ourselves mostly) and the Children can make as much or as little noise as they liked without Brown Owl telling us off.

We had a busy schedule of activities as usual this year including kayaking, boat building, archery, air rifle shooting, abseiling and the giant swing. The whole weekends schedule was reorganised on the Friday afternoon, once we had met the children and had been able to determine limits. This happens every year and it is a credit to PGL to the way they help us refine the weekend to maximise our fun, without tiring everyone out at the same time.

The activity leaders and our ‘Groupie’ Ryan were all great, taking the mixed abilities of the group into account very well and this year had a big introduction of new faces, with everyone doing well all weekend and India being the biggest and brightest star of all for me, having tried every activity, including the abseiling she was so nervous about and beating them all with her huge grin.

The weather held out for us (and the V-Festival, so have moved the dates to avoid it for both weekends next year), and overall, I’d like to think everyone went away happy, if not tired and looking forward to coming back next year.

Remember to apply for a place for 2014 when they are advertised and young adults (18+), Heartline Families needs to introduce new blood into the long line of Leaders. If you’ve been as a child, you know how much fun it could be as a Leader and you get to do every activity, well, except for the quad bikes :(

Aged 15 ¾, going on 16

There were a lot of activities planned and I enjoyed the quad bikes most and then the archery and air rifle shooting (I cheated by poking some holes in the target!). I especially liked the Sunday activities where we soaked Ryan (our PGL Groupie) and my dad (a Leader). Other activities were raft building, kayaking (thankfully I only got a bit wet) ,abseiling, giant swing and high ropes, which I wasn’t so keen on as I like to keep my dry feet firmly on the ground at all times!

I liked the Gift Shop, though I was disappointed that the sweets were closed at the time. I was pleased that I found vanilla flavoured coke and an Oreo Kit-Kat. The food was good, especially the sausage and the bacon, but there should have been less fruit.

Personally, I think the weekend could only have be improved by having no water sports and the zip wire being a little closer to the ground and not so far away from everything else on site because I have short legs. (Did my dad say I was a teenager?!).

Would I like to go again? YES!!

Book your place on the PGL weekend for August 2014!

We’re planning to do it all over again next year. So make sure you book your child’s place early to avoid disappointment!

A form will be available on the Heartline website shortly for you to register your interest for the weekends. Alternatively, you can send an email to admin@heartline.org.uk with ‘PGL’ in the subject.

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Heart of Stars

Where our children are remembered, At the darkest times the stars are brightest Some years ago HeartLine Association sought the help of an astronomer from the Greenwich Observatory.

We wanted an area of the sky where we could have our own constellation of stars, to dedicate to the memory of our children.

Dr John Griffiths found us an area that could readily be identified – with the heel of your hand over Polaris, the North Star, and your fingers spread, you are covering a heart shaped area, identifiable on a clear night from anywhere in the UK.

The dedication

When the child’s name is typed into the box on the Heart of Stars page on the Heartline Families website, their particular star shines. Clicking on the star will produce a dedication to the child, where the family has written one.

We send a card with their child’s name, and an image of the Heart of Stars showing the bright dedicated star and its coordinates.

We would want to offer a place in the Heart of Stars to families who lose children at a very young age and may not have had a chance to contact support organisations like Heartline. There may also be families who have not been in touch since they were bereaved.

Please let any parent who has lost a heart child know that there is a star for them, awaiting a dedication.

There was then a long period of time while Dr Griffiths identified the magnitude (brightness) of the stars, so that we could list those that can easily be seen using binoculars or a small telescope. The Heart of Stars took several years to become a reality. In 2010 the original record of the stars was lost when Heartline moved from the Camberley Office.

We tried unsuccessfully to reach Dr Griffiths again, but it was only later we learnt that sadly he had died.

Recently the records were found, and once again we are able to offer bereaved families a star to dedicate to a loved child.

Caravan Holidays

Time to think about this next year’s holidays, and if you’ve got your eye on one of our caravans, as a Heartline Family, you can book now.

Priority will be given to families who haven’t used the caravans before. To arrange a booking, please contact Melissa by email at melissa.needham@sky.com, with ‘Caravan Booking’ as the title. Melissa will discuss the deposit and payment terms and arrange to be released. Find out more about the caravan parks:

Mablethorpe Golden Sands at: www.haven.com/parks/lincolnshire/golden-sands

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Oakdene at: www.shorefield.co.uk/OurParks/Oakdene-Forest-Park

What we have achieved 2011-2012 Report

HeartLine’s financial year runs from 1 November to 31 October Despite a substantial drop in funding and little administrative input we have fulfilled our objectives in supporting families: • • • • • • • • • • •

Ran a very successful summer party Ran two activity breaks, for heart children and siblings Printed and distributed 1050 copies of Heart Children Distributed a review magazines to all members Published 3 newsletters to members to maintain knowledge and interest in HeartLine activities Recruited 198 new members Distributed 87 wetsuits to encourage children who find it difficult to maintain their body temperature to take to the water Ran two caravans for the season, implemented improved caravan booking systems.

Maintained a valued message board with forums for many of the areas of interest Covered the HeartLine help and information telephone line 24/7, and dealt with all email requests for support and information Provided a conduit for parents’ views and experience for national issues

Cash flow summary year ended 31 October 2012 Donations £51,299 Activities £14,070 Investment income £9 Total income: £65,378

Payments Fundraising activities £539 Direct expenditure £28,114 Governance £8,124 Total expenditure £36,777 General fund balance £57,994

Finances as of September 2013

There is a positive balance of £3,800 after costs from an income of £19,670 so far this year. The balance of funds in the bank (General Fund Balance) is over £60,000, similar to last year.

Costs are under control and kept as low as possible through substantial volunteer work replacing the former office facilities. The main projects of Caravan Holidays, Children and Sibling Holidays, the Heart Book, Wetsuits, the 100 Club, Heart of Stars and the Summer Party have taken place successfully or are continuing to be provided.

The business plan and budget will be available at the end of the financial year together with the completed accounts for the Association for 2012-2013.

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Summer

Party

A fantastic time was had by all at our summer party with heart friends and families from Exeter Heart Families.

The party took place at Dartmoor Zoo and saw lots of refreshments, entertainment and activities and a beautiful sunny day!

Thanks to everyone who helped to make it an extra special party!

the

t e e M team

Just WHO is behind the famous

Heartline email address?

Meet Kevin Ford...

Name: Kevin Ford From: Medway, Kent Role: Volunteer email administrator (and odd-jobber) - the man behind admin@heartline. org.uk What do you do at HL? I manage the admin@ heartline email account, directing emails accordingly and trying to keep the spammers at bay.

Background: I work in Construction Logistics, not that that helps(!), but I have been with Heartline for nearly 15 years and on supporting the Committee for circa 12 years.

Other life: Oldest son is our heart child with a rare collection summarised as Variation of Tetrallogy of Fallots, also proud owner of one Titanium spinal rod. Youngest son is 8 and well.  13

Coping with CHD diagnosis Hearing the news that your child has a congenital heart defect is a huge shock for any parent, and brings new emotions and challenges. At whatever stage you receive a diagnosis you are suddenly faced with new emotions and challenges, from grief and confusion to concern about the future. We asked our families, who have been exactly where you are now and survived, what advice they would give you and here are their answers.

NEVER give up hope. Lorretta Hlhs Mum Hammond Let them live as normal a life as is possible for their defect/condition.

I grew up knowing I had a heart condition but I was never limited in what I could do. If that's possible for your child let them have that normal life, just leave it for the doctor’s appointments for the time to remind them of their condition. Nicola Chisholme Don't ever Google your child's condition. Look for local support groups and Heartline is a fantastic resource. Tara Reynard Listen to other parents and take all the advice you can from those who have been through it, it really does help. Jane Cowen Join Heartline and find lots of lovely people who understand your questions and concerns as a parent and make sure you have a phone number for your CLN. Most people who don't have heart children don't understand so build up your own support network of people who do! Nicola Hotchen Keep a diary so you can read back.

Hospital days all seem to be a bit of a blur if you don't write it down plus it's a good keepsake for your child when they're older if they want to know what happened.

Marije Kootstra Never blame yourself!

Shelley Baker

Don't look at centile charts. My little girl lived under bottom of those charts and my health visitor insisted on weighing and plotting each week which added to my stress - any weight gain should be celebrated. Health visitors are not cardiac specialists and you are lucky if you get a good HV. Lucy Taylor

same, their journey and yours will most likely be very different.

Vivienne McQuade

I was just so relieved when I found Heartline and found children who had survived similar heart conditions to my unborn baby. It gave me hope, encouragement, a listening ear and quite honestly my sanity back. List questions as they come into your head and DO NOT be afraid of asking them. Above all be kind to yourself, you cannot change anything and it’s NOT your fault. Give yourself time to grieve for the pregnancy/child you thought you would have and learn to move forward and love what you have been blessed with.

It's a tough road but one that is amazingly rewarding. Clare Burgess

Take all the support/help you are offered from friends and family - it really is a stressful time and just having someone help with the housework or cooking is a godsend.

I shut people out and wish I hadn't.

Also, you can breastfeed a heart baby with the right support.

Alix Harley

Don't overfeed your tubefed baby/ child because the dietician has a set volume they want them to have. If they are being sick after every feed, reduce the volume. And join the Heartline forum. And it DOES get easier. Jane Van Wyk Your child's diagnosis does not determine the kind of life that they will have, nor is it as bleak an outlook as you may initially think!

Do not compare your child to any other, even if the diagnosis is the

You are not alone, heart family best family I have. Cassie Symms Being honest to friends/family and discussing all your worries really helps. Julie Mcquade

Try and live in the now as much as possible - so don't fret about the future because at the end of the day none of us know what is around the corner. Enjoy them and seek support. Let them be them and don't be over anxious - most heart children are such strong little fighters with personalities to match. Let them develop in their own unique ways Rachel Buckle Each day that passes in hospital brings you a step closer to going home. Charlotte Cooper Although you will go through lots of things you never thought you would and sometimes you will feel lost and alone always treat your child as normal because they are so much more than their heart condition.

Helen Preece

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Warfarin versus

Dr Robert Johnson, Consultant Paediatric Cardiologist at Alder Hey Children’s Hospi

Families faced with the diagnosis of a baby who has a univentricular heart have to learn to trust the team looking after their child. Hopefully thisDr is aRobert process ofJohnson growing By confidence as hurdle after hurdle is surmounted successfully. But one of the difficulties with a long term relationship with a medical team, involving a rare diagnosis in which there are differences in management strategy across different teams, is that parents may become aware of different strategies and question the choices made by their team.

A difficult debate The Warfarin versus aspirin debate remains one of the knottiest in paediatric cardiology. There is undisputed evidence that a clot or thrombus tends to form in the Fontan circuit. This is not particularly surprising since the circuit has factors in each of the elements that lead to a pro-thrombotic state – sluggish flow; damage to the lining of blood vessels (at least soon after surgery) and deranged clotting properties due to liver congestion and increased red cell concentrations.

Some studies have looked for and found either a clot detected by echocardiography or serious events relating to clot formation such as stroke (clots blocking blood flow to the brain) and pulmonary embolus (clots flying off and blocking the blood flow to the lungs).

15

Minimising risk Medical teams have sought to minimise the risk of these serious complications and used standard medical treatments to reduce the chance of blood clotting. The most common two drugs used for this are aspirin and Warfarin. Aspirin is a drug derived from the bark of the willow and reduces the ‘stickiness’ of the platelets (small cells which provide the bricks in clots) in the blood. Warfarin is a drug which reduces the amount of clotting factors (the chemicals in the blood which alter to form the glue or mortar, if you like, which sticks the platelets together to form the clot).

Fewer clotting factors mean the body takes longer to build up clots to seal cuts and breaks in blood vessels.

Often the term ‘thinning the blood’ is used for these treatments but really the blood is not thinner – just less sticky. “So I need to have my blood thinned to reduce the chance of clot forming – just give me the best medicine and I'll get on with my life.” Except, of course, it's not as simple as that.

Rare events, rare conditions In understanding why there is such debate about the approach to protecting patients against these events, it’s important to understand the problem of studying rare events in rare conditions. Basically it's a numbers thing. Collecting enough individuals with characteristics similar enough to compare and look

for patterns of events is impossible in any individual centre whilst organising multi-centre studies is fraught with difficulties of resource and confidentiality. Even in the whole of the UK the number of patients may be inadequate (there were 151 survivors of Fontan surgery from 2008 to 2009 in the United Kingdom). If I may make a list of characteristics, which might affect an individuals tendency to form clot - age, sex, weight, single right or single left ventricle, heart function, type of Fontan (classical, lateral tunnel, extra-cardiac) - one can see the difficulty in collecting enough patients who are comparable, maybe 30-100 all similar would be required in order to see the impact of just medical treatments. Then there are all the factors around surgeon, intensive care team, etc.

What is best?

As doctors, we understand that parents want to trust us and I am confident that the doctors who are caring for children with congenital heart disease of all varieties want to do the best for their patients. But how do we know what is best? The traditional Gold Standard of randomly offering two similar groups of patients two alternative treatments and seeing which group does best is almost a non-starter (but I will come back to that). Other approaches - such as looking back over a number of patients over a period of time - are fraught with bias

Aspirin in Fontan patients

ital

(I will come back to that too). A lot of the time we are left with trying to learn from studies which are well structured but are done in patient groups who are different from ours and who have different problems.

Many of these studies come out of research into adults with relatively common problems for which it is relatively easy to collect 100s and even 1000s of participants to study effects in great detail. This lack of clearly applicable evidence allows personal interpretation of ‘what is best for the patient’ to come in to play.

Important influences An important influence on the choice of management strategy are studies which one thinks are relevant in a different condition. There are numerous studies in adults with a condition called atrial fibrillation (AF) in which the normal rhythm of the heart becomes very irregular. These show the superiority of Warfarin over aspirin for preventing clot formation in this situation. Furthermore Warfarin continues to be superior to almost any other medication in prevention of clot in patients who have prosthetic valves. The AF analogy would appear to be very similar to the Fontan situation with sluggish flow though generally less surgery/ prosthetic tissue. Finally Warfarin has been shown to be an effective treatment where clot has already formed. So why would it not be more effective than aspirin for preventing clot formation?

Stronger evidence - a better choice On the other hand many very sensible clinicians feel that, without much stronger evidence for superiority of Warfarin over aspirin in the Fontan patient, there is a downside of Warfarin therapy; higher risk of bleeding, limitations to activities because of the risk of severe bruising/internal bleeding and, of course, the continual monitoring requiring blood tests, mean that aspirin is the better choice.

Some centres, mine included, have shifted backwards and forwards over time weighing the latest evidence and taking into account the department’s experience at that time.

So what is the latest thinking?

Over the last few years, a few studies have started to sway the evidence in favour of aspirin. I have mentioned the difficulty of performing randomised controlled trials but an international team from Melbourne, Toronto and Hamilton (and these are big players in congenital heart disease world) managed to perform one (21798429). For two years, following Fontan surgery, patients treated with either Warfarin or aspirin had much the same risk of thrombosis or bleeding (thrombosis based on both medical events and careful looking by echocardiography including transoesophageal echo).

“Great!” I hear you say, “– can I stop my Warfarin now?” Well this was a great study but it has its limitations.

Firstly it only followed patients for two years following surgery. One would have to allow for the possibility that thrombosis may become more common later on. It wasn't ‘blinded’ (very difficult to achieve with two very different drug regimens) by which I mean that the teams involved could tell what treatment the patients were on.

Practically the outcomes used are unlikely to be biased by the team involved knowing the status of the patients but this is always a subtle possibility (checks and balances were put in place to avoid this). The rates of drop-out were different, a not altogether surprising finding, and there were practical exclusions such as inability to be followed up, which meant that bias of patient risk may creep - though it is difficult to imagine what they might be. The numbers remain relatively small and the rates of events seem relatively high but this is about the best we can do without a much bigger and longer study which would be tremendously difficult.

What other evidence do we have?

Well there are many papers looking back at groups of patients who have had the Fontan procedure and searching to see if they have had some form of clot and comparing groups with no anticoagulation, aspirin and Warfarin. The problem for the busy clinician is that these papers come up with conflicting results.

Some will favour Warfarin over aspirin; some find no difference.

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Warfarin versus Aspirin in Fontan patients continued...

Again this type of study is fraught with potential bias.

Maybe events have been missed or overlooked the people performing the study have a subtle bias for finding in favour or against a medication and look harder for events in one group compared with another.

Making it add-up Every so often someone comes along and does you a big favour. One of the problems of multiple studies apparently giving different results is seeing how they add up together. The last two decades has seen a lot of research brought together using a technique called meta-analysis. This technique aggregates the results of multiple studies, weighting the aggregate according to the strength of the study (mostly this is a numbers thing – the bigger the number the stronger the study; the technical term is the power of the study). In 2011, a meta-analysis of all the studies published on the topic of aspirin and Warfarin in the Fontan circulation were synthesised in a single review. The bottom line – there did not appear to be any benefit of Warfarin over aspirin (21798429). This despite the fact that some studies did suggest a benefit – overall these positive studies were cancelled out by studies demonstrating no benefit of Warfarin over aspirin.

A 2012 paper published after the meta-analysis discussed above suggests Warfarin significantly better than aspirin (22513267) but another study soon to be published found the opposite (23870650)!

Aspirin as good as Warfarin?

So in the absence of a more definitive study we are left with a mixture of studies which, on balance, suggest that aspirin is as good as Warfarin in preventing clot in patients with a Fontan circulation. Let me be clear about this – the studies do not say aspirin is perfect, there is still a risk of thrombus formation, but aspirin appears to be as good a drug as Warfarin for minimising that risk compared with nothing at all.

New drugs on the market Looking to the future, a number of new drugs have come on to the market which appear to be effective in preventing clot formation. They need only be taken regularly by mouth and do not need repeated level monitoring. As yet none of these drugs has been licensed for use in children. I suspect they are not available in child friendly forms (liquid) or doses. Nor or their long term side-effects well known (there is a big difference between being on a drug for the last decade of your life compared with hopefully 40 or 50 years ahead of you).

I'm sure with time the risks and benefits of these new drugs will become clearer and maybe they will come to play a part in preventing a serious risk in the ever growing population of children and young adults living their lives with only one pumping chamber in their circulation.

About the author Dr Johnson has special interest in cross-esectional imaging and particularly cardiac MRI, also in evidenced and physiology based practice.

Outside of work, Dr Johnson likes to dabble in distance running and triathlon.

Definitions of terms used

Thrombus or clot - the lump of jellified blood formed by the triggering of coagulation within a blood vessel.

Coagulation - the natural process by which the platelets (cells = bricks) and clotting factors (chemicals = mortar) are

triggered to change from soluble into solid to block holes in blood vessels and stop bleeding.

Anti-platelet - a drug which prevents platelets functioning normally.

Anti-coagulant - a drug which blocks the chemical changes which cause the soluble clotting factors to become solid and sticky forming a clot.

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Supermum Does It for Thomas

(and Heartline!)

great family holiday. The Heartline forums were also my first port of call for help and advice. I became friends with many families in the same situation and who helped us a lot, so I thought it only right that I picked Heartline for my charity.

“I was only hoping to raise £100, but using Facebook to raise awareness, getting family and friends to take sponsor sheets into their workplaces, and even getting the ladies at Weightwatchers to donate made my total over £300!

Believe in yourself “On the week before the run I was very apprehensive – would I come last or not finish at all! Everyone told me to just believe in myself. The day of the race was fabulous - a few waves from supporters, a short warm up, the gun went off and we started running. The crowd surrounding the gates were amazing – they really kept me going.

Emma Dargie, supermum to Thomas who was born with Transposition of the Great Arteries, Double Outlet Right Ventricle, ASD, VSD, Hypoplastic Left Ventricle and Pulmonary Stenosis. pulled on her trainers and hit the ground running when she decided to raise money for Heartline by taking part in the Bupa Great Yorkshire.

She talks about what she’d let herself in for and why she did it!

“I had to stop a few times and stretch - I walked and ran, walked and ran. I didn't run the whole thing - I just couldn't! I had planned on doing a heart-shape with my hands as I went through the finish line, but my hands were too shaky! My partner Matthew and Thomas were waiting for me – it was great to see them and it felt brilliant knowing that I’d raised money to help families just like ours.

Not the fittest!

“I decided to do take part in the 10k Bupa run after another heart mum said she was thinking of taking part. I entered straight away, not really thinking about what I’d let myself in for! I’m not the fittest person and I couldn't even manage to run for 60 seconds at the start of my training, but I was determined. In between looking after Thomas, I managed to get to the gym a few times a week to build up some fitness. It was a double-whammy of a plan for me, as I wanted to lose some weight and raise money for a charity, so I had the perfect goal to keep me motivated.

Proud!

When I checked my running time a few hours later, I even amazed myself! I went from not being able to run for 60 seconds in July, to running 10K in 1hr 15 mins and 53 seconds!”

First glimpse into the heart world “Heartline were our very first glimpse into the heart world. We were handed the Heart Child book all about congenital heart conditions, while on the ward at Alder Hey Children’s Hospital with Thomas. I still have that book today. Heartline also gave Thomas his very first wetsuit (and his second!) and made swimming possible for him. We also recently stayed at the Heartline caravan in Mablethorpe, for his heart day, which was a

18

Against All

The Odds

Tyler was born with a very rare condition that saw him face a multitude of challenges.

His mum, Katie Steele-Arnett, tells his story.

After four rounds of IVF treatment Tyler was born at 29wks. There were a few issues arising from him being a premature baby – he had a ventricular septal defect (VSD) and a small PDA. Tyler started life in at the Trevor Mann Baby Unit, in Brighton.

Tyler got to 6 months old and he still hadn’t been able to come home. He was being cared for at Royal Alexandra Children's Hospital, in Brighton. He began to have blue spells and we had the feeling, like so many others, that something just wasn’t right. He crashed, stopped breathing for 30 seconds and we were told that it was related to a virus.

Something very rare It was following CT and MRI scans that we began to get a better picture of why Tyler was so very poorly. The scans showed something very rare. Tyler's pulmonary veins were stenosing. They were collapsing and stopping the blood getting to where it needed to go. The team at Evelina spent hours and hours trawling through imagery to double-check that pulmonary vein stenosis was the main issue.

Couldn’t believe he was alive Normal stenosis starts within either the heart or the lung end of pulmonary veins, Tyler's was smack bang in middle of three veins, and the lower right one was barely there. The specialists at Evelina had even contacted Boston Children's Hospital’s Pulmonary Vein Stenosis team and, after hearing Tyler’s diagnosis, they couldn’t believe he was still alive. It really hit home how lucky Tyler was! Nobody had seen a case like his in over 10 years. The amazing cardiology team decided that they were going to take Tyler into the catheter lab and insert 6/7mm cutting balloons in the middle of 3 pulmonary veins to see how that went. It was either try or lose Tyler.

Breathing for himself A gruelling eight and half hours later, Tyler’s dad and myself finally got to see him in PICU. Apart from few hiccups he was ok. He was off the ventilator and breathing for himself within a week, and on nasal oxygen. He was moved to the cardiac ward and plans were to discharge him back to our local hospital in Brighton after three and half months in London.

Blue lights

We received a call in the early hours of the morning telling us to get back to the hospital as he had taken a turn for the worse. By the time I reached the hospital, they were already bundling him into an ambulance to blue light him to Evelina London Children's Hospital. The doctor had to manually ‘bag’ Tyler for most the journey.

He was taken to PICU to recover and get stronger.

Eventually, they were happy enough with his recovery of the numerous numerous colds and viruses he’d suffered from and he was moved to the cardiac ward. His pulmonary hypertension had been affected by the colds, the hypertensive fluid had reached critical levels. Tyler had got worse again and was again rushed downstairs to PICU and ventilated again, with teams of professionals trying to figure out what was going on. Needless to say, I couldn’t sleep for worry and unanswered questions that were going around my head.

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Blue spells As Christmas, 2011, closed in it was looking more and more unlikely that we’d make it home to celebrate, as his blue spells returned as did the symptoms from before.

We headed straight back to Evelina, had an MRI scan and straight into another catheter lab, this time to fit 8mm cutting balloons. Surgery went well but we knew, from the post-surgery echo, that the balloons weren't going to hold for long as they were already starting to collapse immediately after surgery.

Evelina threw Tyler a little farewell party with cakes and presents! And the fabulous team at the RACH did the same to welcome us back.

A first birthday It’s weird to think we’d never heard of pulmonary vein stenosis and, yet, there we all were celebrating my son’s 1st birthday and the life he was still living – thanks to the wonderful people around him. He was finally discharged for the first time (since birth!) when he was 15 months and 10 days old!

For a couple of months, he was an inpatient again, due to his lungs, adenoids and a hernia, but now he has movement in right side. He is still on oxygen and NG feeds, he needs physio and occupational therapy support, as he isn’t walking or crawling but he so wants it!

His pulmonary hypertension is resolved, the pulmonary vein stenosis is a silent CHD it can creep up on anyone.

A difficult decision Tyler was discharged and we were back at the Royal Alexandra Children's Hospital after just a couple of weeks, before the same issues started again. He was transferred to Evelina in mid February 2012. Another MRI scan showed cutting balloons failed. They had no choice but to stent the pulmonary veins. They used 7 and 8mm adult size stents. The fact that they used these size of stents was not an easy choice, as once you stent the middle pulmonary veins they can't be replaced, which would reault in making Tyler's pulmonary vein stenosis life limiting. Once these stents fail (and they can fail at any time) they can't be replaced and this made Tyler's prognosis look awful. He wouldn’t have reached the end of the week if the stents hadn’t have been fitted and so there was no choice for anyone to make – it had to be done.

Update: Tyler has been well but his right stent is starting to collapse on his right pulmonary vein. The blood is flowing around but not as much as it should. He will be having a transoesophageal echocardiography after Christmas to help assess him properly.

A turn for the worse After another gruelling wait Tyler was in PICU. He was taken off the ventilator the next morning and back on nasal cannula. He was really upset the morning back on cardiac ward and even sedation failed to work. A repeat echo was ordered to check the stents and all was fine.

A moment later and Tyler had gone limp on the right side of his body. With his SATS reading being normal, he was rushed for an urgent CT scan where they realised he had suffered a massive stroke. A small clot had formed behind his left stent, which had travelled and caused a clot in the left side of his brain.

Rehabilitation So it was back to PICU for a night’s observation before he was moved to rehabilitation for the right side of his body. Occupational Therapy’s Play and Physio team did their best to keep Tyler's lungs clear and remind him that he had right side! The week of Tyler's 1st birthday, we were transferred back to RACH. The lovely team at

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Preparing Your Child For Surgery

There are many challenges in the day-today life of a heart parent and child, not least of all when the time comes for surgery.

For a parent not only are there concerns around the practicalities of surgery and the worry of associated risks, but also how to break the news to the child and the best approach to take depending on their age.

We spoke to the Children’s Heart Federation to get their advice and also asked members about their experiences in the run up to surgery.

Preparing your child for surgery will help them to understand their illness and treatment better. Often, introducing a doll or figure toy can help your child ‘play out’ any fears or worries they have about the surgery.

Your child may like to play with the doll and use it as a ‘patient’ to play hospital at home.

When should I prepare my child?

You know your child best. Some things to consider are: • How old is your child?

• What developmental stage are they at?

• Do they have any previous experience of hospitals?

• How well or unwell do they feel?

These will affect how and when you prepare your child.

Toddlers (18 months to two and a half years) Children of this age will only understand that they are in a strange place, with strange people who sometimes do things to them that are painful. They will need lots of cuddles and reassurance. Distracting them at the time of a painful procedure is the best way to help and support them.

Pre-school (three to five years old)

Children use play as a way to communicate and this can help them to prepare for their hospital stay. Allowing children to see, touch and play out their worries with toy medical equipment and dolls before they come into hospital, provides an opportunity to ask questions and familiarise themselves with what is going to happen to them. It can also help them to play out their experiences afterwards and come to terms with what has happened.

They may also wish to bring the doll with them, when they go into hospital.

Pre-school (three to five years old): may have no understanding of time, so preparing them a few days before they come into hospital should be enough.

Why should I prepare my child?

Older children/teenagers (eleven years old plus): may need much more detailed information and time to absorb it. They may need more time to adjust to the idea of the procedure they are going to have.

• It helps them to understand their illness and treatment.

• Preparing your child psychologically for surgery can help them to recover more quickly afterwards. It lowers the risk of long-term distress following their experience in hospital.

• It helps to correct any misunderstandings your child may have about what happens in a hospital, or during their particular surgery.

• It gives your child a chance to express their worries and feelings (for example, will it hurt? What will my scar look like?).

• It helps your child to find ways of coping with the treatment (for example, watching my favourite cartoon will help to take my mind off things).

• It speeds up recovery. Children tend to be more co-operative when they understand what is happening to them.

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School aged (five to eleven years old) School aged children (five to eleven years old): one to two weeks before is usually about right.

Older children / teens (eleven upwards)

Your Say! We asked our heart families for their advice in helping to prepare a little one for surgery.

Contact the play therapists in advance, they are so useful in making things less scary for the children. Jeanette Jennings

From a young age, children are often keen to discuss what they know about hospitals. This could be right or wrong information. You may think that it is best not to tell your child about the operation, but this usually causes more distress in both the short and long-term.

What do I say?

• Use words that your child is familiar with (scar, special mark, zipper and line).

• Give truthful, factual information (it may hurt a bit, but mummy will be there with you and you will have medicine to help).

• Explain why they need to come into hospital (it will make you feel better, feel less breathless).

• Describe the people your child may meet (doctors, nurses).

• Explain the procedures he/she may have, for example a chest x-ray, ECG (electrocardiogram), Echo (echocardiograph) or blood test.

School aged children often talk about operations with vivid and sometimes frightening descriptions, such as cutting and pulling things out. As a parent, you may find this distressing to hear. Make sure you look after yourself and prepare yourself as much as possible before talking to your child. If your child asks a question that you do not know how to answer, or are too shocked or distressed to answer, give yourself some opt out time.

Say ‘I do not know the answer, but I will find out for you.’ This gives you the time to have a break, contact someone for support or have time to find a suitable answer.

What if my child won’t talk to me?

Parents often say ‘if only he/she would tell me what is wrong’.

Children use play and behaviour as a way of communication. Role play (acting out), ‘naughty’ behaviour or being extra quiet (withdrawn) may be a way of letting you know what they are afraid to say or do not know how to say.

Hitting the doll or giving repeated painful injections can indicate anger towards the illness or impending operation. Every child will express themselves differently.

Who can I contact for support?

Do not forget, you are not expected to know everything. If you would like more information on what to do, contact your cardiac centre and talk to the play specialist and cardiac liaison nurses. For children who are particularly anxious about admission to hospital you can request a play schedule, to help guide you along the path to surgery, through your play specialist.

Molly’s Dolly

CHF produces a doll for the purposes of preparing a child for surgery, called Molly’s Dolly. The dolls have scars that match your child’s (if needed) and can help explain procedures before they’re carried out at hospital. Apply for a Molly’s Dolly via the Children’s Heart Federation on 0808 808 5000.

I wish I had taken more photo including her chest before it gained a scar. Jenny Payne Take lots of photos, be honest but only tell what they need to hear. we told our 3.5 year old he had a poorly heart and the doctors were going to fix it. (We said) The bloodtests would hurt but only for a second and he would have more energy to have swimming lessons and to play football once we would be able to go home. Marije Kootstra Keep them involved if you can, I’ve never had a conversation with the doctor without my child in the room, I believe whatever age they are they will pick up info as they go, making it easier for them and for you as they get older. If you try to hide things they pick up on it and worry. If i’ve been told anything scary then I’ll put it in unscary/simple terms for my child. I have to say too that kids are amazing, they can cope with a lot more than we give them credit for and normally cope better than us!

Ruth Stevens We found a few books to help but none that were as relevant as the Heartline story about baby bear.

Victoria Richardson When Ellen had her fontan aged 5, the best advice I got came from Lynnne Carney. She advised to tell Ellen she would wake up after the op with a ventilator in (breathing tube), to say it was completely normal. I said it was helping her to breath so her body could rest. Jane Van Wyk

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HeartNEWS

A round-up of what’s going on in the world of hearts.

Building parts to repair the heart Scientists are on the brink of being able to grow replacement heart valves in a lab from human tissue, which could help 100,000 people a year.

The first heart

generations to enjoy high survival rates

New figures released reveal a huge 83 per cent drop in the number of children dying from congenital heart disease – birth defects of the heart – over the last three decades.

Progress in diagnosis, intervention and post-op care has created a generation, now in their twenties and thirties, who are the first to have enjoyed high survival rates for major heart defects.

There are so many survivors that a new speciality – adult congenital heart monitoring – has emerged to treat them in later life.

An American research team stands at the brink of a medical breakthrough - engineering replacement parts for the human circulatory system - and a life’s work coming together in an assembly line of arteries and heart valves manufactured from human tissue.

Their novel manufacturing process could create promising new alternatives for the tens of thousands of adults and children, across the world, who need replacement heart valves every year. For the children it may lead to valves, which are able to grow along with their young bodies.

Around eight in every 1,000 babies is born with a heart defect – 12 every day in the UK. The latest figures illustrate children’s vastly improved chances. Between 1979 and 1983, more than 5,200 children lost their lives to congenital heart disease. Between 2004 and 2008, that figure had plummeted to 893 and experts say it’s still falling.

“It was once the case that patients with complex congenital heart disease would not be expected to survive,” said Professor Andrew Taylor, a leading cardiologist at Great Ormond Street Hospital (GOSH). “Nowadays it’s completely the reverse. In our own institution we’re looking at two to three per cent surgical mortality rates.”

Artificial Cow Heart in Trials A company based in Paris, has designed an artificial heart fashioned in part from cow tissue. The device, soon to be tested in patients with heart failure, is regulated by sensors, software and microelectronics. And its power will come from two external, wearable lithium-ion batteries.

Fifteen years in development, the heart has been approved for clinical trials at cardiac surgery centres in Belgium, Poland, Saudi Arabia and Slovenia, where staff members are receiving training and patients are being screened.

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Heart Brothers & Sisters Study Results

Some of our families recently took part in Bangor University’s study, which investigated the emotional effects on the brothers and sisters of children with life-limiting conditions, such as CHDs. The results have now been released and highlights of the findings are below.

Siblings of children with life-limiting conditions showed: • More ‘prosocial’ skills’ compared to other children of the same age without a brother or sister with a life-limiting condition (prosocial skills means being considerate of other people’s feelings, helping out someone in need, or is kind to others).

• More emotional difficulties (like low mood or anxiety).

• More behavioural difficulties (like ‘acting out’).

• Lower quality of life.

• Difficult relationships with their brothers or sisters.

Parents of children with life-limiting conditions reported experiencing: • More financial strain compared with other parents without a child with a life-limiting condition.

• More personal strain (such as extra activities and duties they have to perform).

• More difficult family and social relationships (such as having less time available to spend with other family members).

Not all siblings and parents of children with life-limiting conditions will experience these difficulties.

Why is this important? And what does it mean?

These results are important for helping us to understand the experiences and needs of siblings of children with life-limiting conditions. By being more aware of the potential issues, we could think of ways to help them. When looking at other studies, it would seem that there are some ways in which we can help siblings: • Involving them in their brother or sister’s care might help reduce worries wellbeing.

• Talking openly about the condition and treatment, in an age-appropriate way.

• Talking about or drawing pictures of their worries, • finding some time to do enjoyable activities as a family, or talking to other siblings about their experiences.

If you would like more information or a complete copy of the results please email: j.fullerton@bangor.ac.uk

Heart Rhythm Specialists’ Directory Searching for a heart rhythm specialist?

The Heart Rhythm Specialists directory highlights heart rhythm disorder specialists near you.

The database houses a list of healthcare professionals, including doctors, GPs, nurses and cardiac physiologists, who have an interest in heart rhythm conditions disorders like atrial fibrillation, supraventricular tachycardia and sudden cardiac arrest http://www.heartrhythmspecialists.org/

Help Save Lives In Under a Minute

The Pulse Oximetry test measures oxygen levels and is a simple, pain-free way to potentially diagnose heart problems in early life.

Unbelievably, this test is not a standard at birth and, as a result, many heart conditions, which may have been picked up, are missed.

The petition to introduce the Pulse Oximetry screening for all newborn babies in the UK needs your support.

Together, we can help bring about a change that could affect diagnosis and early treatment for babies who may not have otherwise had their conditions picked up.

Evidence shows it is an effective test in detecting three quarters of congenital heart conditions. Please support this campaign and push for its inclusion in the national screening programme of all newborns by signing the peition here http://bit.ly/1c9UBdS.

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! rd a w A l ia c e p S ’s o rl A y rt o Sp Arlo was diagnosed before birth with pulmonary atresia, VSD, ASD and MAPCAs. Without a pulmonary valve, oxygenated and deoxygenated blood mixed via the holes in his heart and flowed to his lungs via collateral arteries connected to his aorta. We were strangely proud that he had worked out a Plan B for his circulation!

Destined to be sporty

Arlo clearly had other plans (maybe because his birth was induced on the night of the World Cup final...).

Before his second operation at the age of 14 months, when he couldn’t yet walk on his own, he played football in the hospital corridor while we were waiting to be called for his anaesthetic. Now, aged seven, he attends football clubs twice a week, takes part in rugby, was highly motivated during the school’s Olympic topic and cleared out his dad’s loose change at the School Summer Fair’s Beat The Goalie stall.

A remarkable boy

Two open heart surgeries

He had two open heart surgeries as a baby, one aged six months to connect his right ventricle to the pulmonary arteries and close the main collateral artery, and another one nine months later to insert a homograft pulmonary valve and close the holes in his heart. He also had a cardiac catheter procedure to close the remaining collateral arteries.

He will need more surgery to replace his pulmonary valve when he outgrows it, and he also has issues with his aortic valve.

Not as active as other kids

Naturally we were told when Arlo’s condition was diagnosed that he might not be as active as other kids - the cardiologist who did the scan said our baby was unlikely to be a footballer, but we assured him that since neither of his parents are exactly sporty we didn’t think that would be a problem.

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In July, Arlo received a Special Achievement Award from his local School Sports Partnership.

Arlo’s school teachers put him forward for a Special Award because while he may not be the fastest or strongest player, his determination and his enthusiasm for sport are remarkable.

Not going to stop him

Of course, Arlo’s condition does hold him back a little. He doesn’t have the stamina of his school mates and when it comes to travelling to school he far prefers his scooter to walking.

But it’s very clear he’s not going to let his heart health stop him from getting the most from any and every sporting activity he can muster: whether that’s cricket during the summer or ping-pong across our front room coffee table in the winter.

Walking with giants

Being recognised at the School Sport Partnership ceremony and receiving his award from Jason Gardener – a real-life Olympic sprinter – was a fantastic way to celebrate his achievements.

Facing challenges

We are extremely grateful to Arlo’s team at Great Ormond Street and at the Bristol Children’s Hospital who continue to give him excellent care.

We’re also immensely grateful to Arlo’s teachers who have seen his enthusiasm for sport, built on that and given him the confidence to get the most out of what he can do.

As he grows up, his sporting activities will give him a level of fitness that should stand him in good stead whatever challenges he has to face.

Heartfelt Thanks Heartline is solely reliant on the generosity of its members and supporters to enable us to keep supporting the countless heart families out there who are in need of a friendly shoulder, advice, and so many benefits to help make life a little easier in adverse times. Without these people, who spend their valuable time fundraising, holding events, donating their hard-earned money to our association – Heartline Families just wouldn’t be possible.

From the bottom of our hearts, and on behalf of the families and children who benefit from your kindness, we would like to express our gratitude.

Donations of £2,000 and more have been made recently by: Theo Walker Fund Basepoint Camberley Edith Spencer Memorial Fund The Springs Golf Club St Aldhem Lodge Other donations have been made by Kathleen Laurence Trust Gordon Leadbeater Ysgol Gymunedol Peniel (School) Anne McKain St Lawrence Players The Miss Caldwells Debbie Green, Community Life Champion ASDA Miss Verna Cunningham Mrs E Walker Mark Fields James Denny Emma Dargie Sutton-upon-Derwent Parochial Church Council Stanstead Abbotts Lodge Wycombe Abbey School And the many more who were good enough to send donations, sometimes at a time of sadness in remembrance of loved ones.

Thanks to all those behind the scenes who help to make Heartline possible for the families and members who need us most.

post surgery, publish the Heart Children book, a source of much-needed factual advice to heart families.

It all adds up!

A little each month

The incredible support we receive helps provide children with wetsuits, encourages nervous parents to involve their child in sports from an early age, helps to provide reduced-cost holidays for families who wouldn’t otherwise have the funds to take a much needed break with their loved ones, gives the gift of parent-to-parent support in times of need and helps us to provide information sheets and advice to families pre and

By just giving a little each month, you could be helping to continue the circle of support that HeartLine offers. Please look out for opportunities to donate regularly, or contact us for our details to set up a Direct Debit; whatever the amount.

Contact the office at: admin@heartline.org.uk.

See fundraising information at: www.heartline.org.uk

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Jennifer's Story

By Amanda Kitely

We were told, antenatally, that our baby had a Congenital Heart Defect.

I also had only one artery in my umbilical cord. This being so, we were expecting a small, blue baby to be born who would have to be resuscitated.

She was born six days late at 8lb 8oz and was pink and screaming - an excellent start! She went straight down to Great Ormond Street Hospital (GOSH) - I literally held her for 30 seconds and she was taken. They gave her a drug to stop the valve closing that usually closes at birth to give them some time to decide what to do.

A diagnosis Ebstein Anomaly (EA) with pulmonary stenosis (narrowing of the vein to the lung) was diagnosed at GOSH and it was decided, after much discussion, not to operate straight away but to wait and see, mainly because her condition is so rare (1 in 214,000 are diagnosed with EA).

She had a few bouts of tachycardia (racing heart) when she was a couple of months old and was put on medicine. She wasn't able to suck very well and lost weight, so was on a sippy cup very early, I spoon fed her quite quickly too and she had to have a high fat food diet to increase her weight.

Glenn Procedure When she was two and a half years old she was very poorly which set her tachycardia off. At this point, it was decided then that they would do the Glenn procedure, the first part of the bypass, which they did just before she was three years old. She recovered well and it was as thought they’d given her

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Duracell batteries, as she was so full of bouncing energy.

She has pink cheeks and was very sensitive to the sun due to her medication for her tachycardia.

Following an ablation, where they snipped the extra circuit in her heart, she’s no longer on any medicine.

They also did an MRI scan to see if they might be able to do something with her valve and chambers rather than doing a complete bypass. The result of this was that her valve was too bad and too displaced to be able to do anything. We were told she would need a Fontan operation, which would completely bypass the right hand side of her heart.

Trust in medical science After doing further scans they decided against the Fontan, as there was too much blood going from the right to the left side through the hole in her heart. This wouldn’t have been such an issue but it was also regurgitating back again. As the operation entailed the right side of her heart being cut off they couldn’t close this hole, it would have meant blood would have collected in the bottom right chamber and as the blood whooshed passed it would have caused a whirlpool effect and the blood would have clotted. We were told we would have to ‘wait and see’ and hope that medical science would come up with a solution in the meantime.

I couldn’t let it lie Well me being me, I couldn’t let that lie! I had gone on many online message forums for heart conditions, including ones for Ebstein’s Anomaly (EA). On one particular forum a surgeon from the USA answered questions from worried parents and patients. I wrote to him with all the information we had been given and asked if he would review Jennifer’s case. He was more than happy to do so and requested scans etc, that had been done. Luckily our consultant at Great Ormond Street Hospital was happy to send the information over

and they also sent it to two other consultants that they work closely with.

Worst possible severity We met with Jennifer’s consultant at GOSH and he sat with us for over an hour explaining the complexity of Jennifer’s condition. We were told EA ha four grades of severity and that Jennifer had ‘D’ - the worst severity of a rare condition. The online American doctor and our doctors at GOSH agreed that Jennifer should not have the Fontan.

We were so very lucky that Dr Del Nido, from Boston’s Children’s Hospital, offered to come over and assist Dr Tsang in doing a relatively new operation called the Cone Technique.

Wires coming out of everywhere In November 2011, Jennifer had the cone technique. It was a huge operation and we were shocked when we first saw her despite having seen her following heart surgery previously.

She seemed to have wires coming out everywhere and I think the biggest shock was that we were told they hadn’t closed her chest up due to swelling.

She didn’t have any major issues except her kidneys stopped working for a short while but after some medication they started again – phew! The amazing thing is, whilst you’re in the midst of it, you just accept what you’re being told. They said they may have to use a machine to start her kidneys working and I was “oh ok” and afterwards I realised they meant kidney dialysis.

Fortunately she didn’t need that. he recovered well from the operation and was discharged 11 days later just in time for her to be able to go and watch her class assembly.

A real fighter

When we first found out she had EA it was devastating but we have been so lucky that she has coped with it well and is a real fighter. She still can’t keep up with her peers as there’s still not the ‘normal’ amount of oxygen getting to her lungs but she did manage to run the whole way around the school’s track last sports day instead of just helping or watching from the sidelines!

Just WHO is t e e M Forum e h t Manager m a e t Kerry MS? Name: Kerry Morgon-Shaw Role: Forum administrator From: Nr Lichfield, South Staffordshire What do you do at HL? I deal with an message board issues as I become aware of them with the help of my dedicated moderators, approve new members and try and prevent spammers from getting through the net.

Background: I am currently a self employed wedding and baby photographer after giving up my job over nine years ago when I was became a heart mum. I took so many photos of him when we knew there was something wrong, but not what was wrong, my skills improved, so I've put them to good use! I've been a member of Heartline since 2005 (crikey, has it really been that long!)

We also still have to plaster her in sun cream as her skin is still sensitive from the medication she used to have to take. But now the only medication she’s on is aspirin. Every time we go for a check up she has lots of visitors as she’s renowned at GOSH as she was the first patient to have the Cone Technique.

I hope that this story will encourage anyone who’s experiencing the stress of having a CHD baby. We’ve had our ups and downs and she will always have a heart condition and plenty of check ups but Jennifer is doing amazingly well and in all honesty you’d look at her and not believe she had such a severe heart condition.

Other life: My middle son, Jamie is my heart child, he's nine now! He had OHS for Coarctation and hypoplastic aortic arch repair when he was nine months old. He's currently nice and stable, on yearly appointments and is an absolute joy despite the complicated little life he has had so far. Thankfully he has outgrown his wheat and dairy allergies but unfortunately suffers from regular migraines.

He likes to keep us on our toes!

Terms Used:

Ebstein’s Anomaly (EA): is a rare heart defect in which parts of the tricuspid valve are abnormal.

Pumonary stenosis: is a narrowing of the valve between the right pumping chamber (right ventricle) and the large blood vessel that carries blood to the lungs to pick up oxygen (pulmonary artery).

Tachycardia: an abnormally fast resting heart rate.

Ablation: a treatment that aims to control or correct an abnormal heart rhythm.

Fontan procedure - aims to separate the blue (deoxygenated) blood supply and the red (oxygenated) blood supply.

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Living in a Grown-up Heart World _______________ Rachel Adam-Smith was born in 1977, in Bradford, and diagnosed, like many babies at that time many days after birth, with Transposition of the Great Arteries.

The doctors did not notice that anything was wrong with my heart when I was born. It was when my mum was being discharged four days later that a registrar on duty noticed a murmur and from then as my mum would say 'her world collapsed' - she thought she had a healthy baby girl. She went home with me, my grandparents were waiting and my brothers. My mum burst into tears, she had started out the morning thinking everything went so well and by the end of the day she thought I was going to die.

Balloon Catheter

It was a few days later when I was taken to Dr Olive Scott at Killingbeck for initial tests. She told my parents exactly what was wrong and what needed to be done. The first step was to bring me in the following month for a balloon catheter to make a hole in my heart, so the blood could circulate better until I was 16Ibs in weight when I could then have the Mustard procedure. Once my mum had seen Olive Scott she felt calmer, she was very good at reassuring my parents, years later I always enjoyed seeing her and dressing up proudly to show her how well I was.

Reassuring sounds of the hospital

My childhood was very good. I was placed on heart drugs from birth and had yearly check ups. I loved the reassuring sounds of the hospital and can still remember the smell of the corridors. Mum would often ensure that I had extra clothes on to protect my chest from colds. I often got coughs and colds, sometimes developing into bronchitis and I would end up back in hospital needing a bit of extra help.

An outdoor, healthy child

I loved the outdoors and horses. I was very lucky to be able to go horse riding and have my own horse, I think this was a huge benefit to my health. I spent every hour after school and all weekend outdoors and was very active, I would compete at show jumping, lift bales of hay and bags of feed around the yard and never felt that I could not do anything. I loved horses and the life I had outside, it made me a stronger, healthier person.

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I played squash at school, although I did stop sometimes to catch my breath, I would always be last around the track at running, I was advised not to do cross country but insisted on going and walking instead, otherwise some children would say that it was not fair that I did not do it.

Something not quite right

When I was 19 I became cabin crew for Monarch airlines.

The training was extremely difficult but I succeeded and worked as cabin crew for five years. Towards the end of my career as cabin crew, I could feel that something was not quite right. I was beginning to notice that my heart was going very fast and I would have trouble with palpitations. By this stage I was working at cabin crew at Gatwick. So I went to the Royal Brompton Hospital for my check up. They discovered that I had atrial fibrillation and needed a cardioversion. So at 23 years old I was having my first cardioversion, it was successful and I was placed on Amiodarone.

Trying for a baby

Unfortunately I had to give up flying. I lived with my husband and after taking advice from specialists at The Royal Brompton Hospital we decided to try for a baby.

I was told the risk to my life was increased by 1% and I had a chance of going into atrial flutter.

As I had worked as cabin crew for five years with a heart rate of 130 bpm and coped, I felt that I would be ok. I had scans every two weeks and was doing ok until I got up one morning, at 32 weeks pregnant, and went into atrial flutter at a rate of 220 bpm. We then had a two hour drive from Gatwick to London in rush hour. I held ice on my artery to try and calm my heart down.

The pain was unbearable from the speed my heart was going at. I insisted on not using a wheelchair on getting to hospital because I felt I was giving up by doing this and I wanted to keep some form of control.

Holding my baby bump while going under anaesthetic I had to be cardioverted whilst pregnant and I can remember holding my baby bump as I went to sleep, it was a very strange feeling. Unfortunately, although successful, the next day I went into heart failure and had to be cardioverted again. The doctors decided to keep me in hospital until Francesca was born at 36 weeks by caesarean under a general anaesthetic.

She was a safe weight at 5Ibs and my blood pressure was very low. Francesca was placed into special care and I could not see her for the first day. We went home two weeks later.

32bpm – 270bpm heart rate

As I had to be placed on so many drugs whilst pregnant, I had bradycardia and my heart rate went down to 32 bpm. I had to have a pacemaker fitted a year after my daughter's birth. The pacemaker would not control the Atrial Flutter and I decided - after much pressure from the hospital - to have an ablation. On waking up I realized something was very wrong, my heart was still going incredibly fast, I had extreme discomfort in my groin and my blood pressure crashed shortly afterwards.

My artery had become perforated when they removed the catheter and I was losing a huge amount of blood.

I was rushed to intensive care and a surgeon had to be called from the other side of London to try and 'fix me'. The pain was unbearable and my heart rate went up to 270 bpm. I had a laporotamy and cardioversion and spent time in intensive care on a ventilator. I was very lucky to survive, my third near death experience and am thankful to the wonderful surgeon who got to the hospital in time and saved my life.

The strength that your child needs

My daughter is now 10 years old and suffers from a rare genetic disorder to Chromosome 16. She is unable to speak, we communicate using Makaton sign language.

Francesca has developmental delay and is physically very small. She needs help eating, dressing and feeding.

Francesca has been a patient at Great Ormond Street Hospital suffering with renal failure, pneumonia, septicaemia, she has needed hip surgery, stomach, ear, nose and throat surgery. I have managed to keep calm and have strength through all of this despite my own health problems.

Nurses would often worry that I was about to pass out when Francesca was critically ill and when I had to stay up all night by the side of her bed, but it is quite amazing that in the most stressful situation you get the strength you need to be there and to be the strength your child needs.

You look healthy, so you must be healthy!

My heart has been extremely strong and I am thankful to all the wonderful staff that have helped me over the years. You cannot see heart disease, I will often get comments that I look too healthy to have heart disease and people cannot believe that I have a pacemaker. Sometimes it is difficult for people to understand why you might be more tired and need to rest more often or cannot join in certain activities, you don't necessarily get the help you need because of a lack of understanding, you look healthy, so you must be healthy!

Heart disease is the hidden killer of many and we need to offer more support to those that might just need extra support, my disease is lifelong, it will never go away.

My scars are a story of the life that I have had and I am proud of them.

Terms Used:

Ablation is a treatment that aims to control or correct an abnormal heart rhythm. Amiodarone is used to treat heart arrhythmias Atrial flutter (AFL) is an abnormal heart rhythm that occurs in the atria of the heart.

Atrial fibrillation is a heart condition that causes an irregular and often abnormally fast heart rate.

Cardioverted – shocking a heart into a normal rhythm.

Murmur – noise produced by blood flow in the heart and vessels.

Laparotomy - an incision made in the abdomen (the tummy).

Mustard procedure - this procedure restores the circulation but reverses the direction of the blood flow in the heart.

Pacemaker - a small device that monitors the heart and restores it to a normal heart rate.

Share Your Story Do you have a story or experience that you’d like to share with our families? Or perhaps an idea for something you’d like to see featured in the next edition of Heartline Families Magazine? Please do get in contact and let us know - we’d love to hear from you!

Email trustee1@heartline.org.uk

In the next edition: A Week In The Life of a Cardiac Liaison Nurse Grown-Up Congenital Heart Stories A Guide To Talking To Your Doctor More Real Life Stories and Experiences And much, much more.

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Heartline Families 32 Little Heath London, SE7 8HU www.heartline.org.uk Telephone: 033 00 22 44 66 Email: admin@heartline.org.uk Facebook: www.facebook.com/heartlineuk / www.twitter.com/heartlineuk Heartline Families is a Charitable Incorporated Organisation registered with the Charity Commission No: 1153442