Autumn 2012

TOFS (Tracheo-Oesophageal Fistula Support), the charity that supports families of children with Tracheo-Oesophageal Fistula and associated conditions.

Pictured: Superhero Thys Bell, 20 months, son of Emma and Michael Bell from Sutton-in-Ashfield. Mum Emma,

tells us their story on Page 3.

InsidePage 4 Interview with Joe Curry, Consultant Paediatric Surgeon

Page 6 41 years without TOFS

Page 11 Becoming a mum

Page 14 Adult Conference report

TOFS was s t ar ted by TOF parents and cont inues to be run by TOF parents , g rand -parents and adu l t TOFs .

“Born at just 28 weeks’ gestation, weighing 985g (2lb 2oz), Eva was twin sister to Charlotte, precious daughter to Sarah and David and cherished granddaughter to Maureen, John, Sophia and Hubert. Eva was diagnosed with TOF/OA a few hours after birth and underwent emergency surgery on 6 August 2011 at Chelsea and Westminster Hospital. Her low birth weight and unstable condition meant that only her fistula could be ligated and her family had to wait until she was bigger and stronger before an attempt to repair her atresia in October 2011. Unfortunately it was discovered that she had a long gap and would need a gastric pull-up, so once again there was a wait - this time until she weighed over 5kg. Slowly but surely Eva began to thrive: she grew well, came off her medications and breathed independently. She loved watching her jungle friends on her mobile, sitting in

her musical chair and kicking her arms and legs to Eddie the Elephant’s tunes. Tragically Eva never woke up from the operation on 1 February 2012 to move her stomach into her chest, having suffered catastrophic brain damage. She died, at home in her Mummy’s and Daddy’s arms, on 23 February 2012. Unlucky in her short life but loved by everyone who met her, Eva’s smile and spirit will always be remembered. Sarah and David are grateful to TOFS as a valuable source of information and to all the nurses (Eva’s aunties) at Chelsea & Westminster NICU who cared for her so wonderfully.”

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WelcomeWelcomeFrom the EditorWelcome to your Autumn 2012 issue of Chew.

Hello to all parents, grandparents, siblings, aunties, uncles, TOF adults, professionals, patrons and friends… and not forgetting

our TOF children too! Quite a list, and I hope there’s something for everyone.

Good news - the long-awaited Adult TOF leaflet aimed specifically for health professionals is here! Not in Chew of course, but if you’re an Adult TOF and would like a copy for your GP then please email us and we’ll post one out to you. Right, who’s going to win our return trip for two to the Big Apple with the TOFS Fantastic Flyaway Raffle? What a prize! And the tickets only cost £1 each – every penny of which goes direct to TOFS. See our ad on page 15 and get your shout in for the chance to win.

Did you know that TOFS was started by TOF parents some 30 years ago and is run by TOF parents to this day – along with some grandparents and TOF adults? More about that from TOFS Chair Duncan Jackson, in the next edition of Chew.

Plans are already afoot for the 2013 TOFS conference, which will focus on childhood TOF/OA issues. Is there anything in particular you’d like to see included? Now’s the time to let us know. Just email the office and we’ll make sure your suggestions are put to COM.

We’re also budgeting to continue our children’s parties across the UK in 2013. Where and when is yet to be decided. Not surprisingly, London always seems well supported but some of the regional areas less so. With a limited spend we need to make sure we’re benefiting as many families as we can. If you have any ideas, please get in touch. As we’re putting this edition to bed our Durham party’s in full swing. Full report to follow in the next Chew.

We held our second successful TOF Adult Conference in September. Read all about it on page 12. Before our next, we need to engage with more TOF Adults. Can you point one in our direction? It’s very good for TOFS and for other Adult TOFs to have a large number of adults signed up in their own right. By the way, in relation to TOF Adults, an issue that keeps cropping up is the importance of getting a copy of your medical records after discharge from paediatric to adult care.

Our treasurer’s been squeezing every last penny out of the budget and you’re rising to our fundraising challenges so thank you for that. I look forward to writing about your successes in Chew, space permitting, or on our website.

Bye for now,

Diane Stephens, Chew Editor

A warm welcome to our new members!• Ros and Brendan Fox from London –Isobelborn16May2012• Kylie and Matthew Hawthorne from Australia –Benjaminborn25July2011• Elizabeth Amos and Ian Milburn from Millom –Maxborn26January2011 • Trace and Paul Loffman from Warwickshire –Bethanborn27May2012• Simona Pankova from London –Tinoborn26September2011 • Rosalind and Wayne Cross from Weston-Super-Mare –Albertborn20March2012• Isla and James Cripps from London –Roryborn01March2012• Jo-ann Barrett and Michael Carter from Crawley –Jayborn28March2012• Vicky Punchard and Mike Dearman from Moreton in Marsh –Ameliaborn11June2011• Stephanie and Carl Redman from Preston –Hollyborn02March2011• Lucy and Rob Hazlem from High Wycombe –Albertborn02May2012• Jeremy Goldkorn from Beijing, China –Violaborn22June2012• Katie Bailey from New Zealand –Charlieborn05March2011• Rachael Haukvik from Hull –AdultbornwithVACTERL• Louise Clarke from Middlesex –AdultbornwithTOF/OA• Heather Robinson from Australia –AdultbornwithOA

IN MEMORYEva Sophia Boyle 05.08.11to23.02.12

“Loved right up to the moon – and back”

In an age when we rely on our brilliant Doctors, nurses and NHS at times of need, we can so easily forget that things sometimes can and do go wrong, often for no particular reason. Once upon a time all of our TOF babies would have died but

now increasingly, few do so. We would like to take the time to remember one such little girl, Eva, whose parents write so movingly about her:

Family updates & newsWelcome Family updates & news

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‘Ratbag’Mum Emma, tells us Thys’s story...

I guess our story starts in May 2010 when I found out I was pregnant. We were overjoyed as we had been trying for over a year; finally I had passed the test!

The twelve week scan came and went with nothing unusual to report. Fast forward to the twenty week scan, I had started to show a little (which I was really excited about as I no longer looked like a weirdo shopping in Mothercare). We found out he was a boy (we had no choice it was the first thing the radiographer showed us). Fluid was normal, he was a normal size, all appeared as it should, except they couldn’t find his kidneys. It took two scans to establish that he probably didn’t have any (I can remember thinking ‘typical boy, he will show us his willy but not his kidneys’). We were referred to the Queens Medical Centre in Nottingham but strangely enough we weren’t overly worried as Thys had been wriggling on the first scans, so we hoped they had just been missed, plus it all felt a little surreal. On the up side, we got to see our little man again.

The consultant couldn’t find his kidneys either , so off we pottered to another consultant. We were left devastated as she also could not see his kidneys and so were told the chances of survival for him were slim. Two weeks passed and another scan came and went, he grew and so did I. MRI scans were done and thankfully kidneys (or rather a mass of kidney tissue) was found but in the wrong place (typical male again, not looking at the manual). This was a huge relief, there was a kidney after all. However, the list of things that were wrong with him just kept growing, TOF/OA being one of the many possible issues that could potentially occur but none were confirmed and so we didn’t give it too much thought.

Just before Christmas I looked like I was about to pop. Knowing what shoes I had on or even if they matched was a thing of the past. The doctors gave me steroids as it was suspected that I would go into labour early due to the excess water I was carrying which they put down to his kidney function rather than anything else. I got to 35 weeks and was fed up; huge and fed up. Getting out of bed was a monumental mission, nothing fitted me and I was simply uncomfortable. I was given my induction date, the 21st January 2011, I had to wait four more weeks which was hard as I had reached whale status!Finally four weeks were up, it was the 20th of January and the plan was to get the last bits and bobs for him and just simply relax as we knew the chance of sitting down again to watch a film would be slim to none. This was not to be however, as at ten past nine in the morning my waters broke. We went through all of the formalities that labour brings. The labour progressed and at 2:30am Thys Sebastian Bell was born, the most perfect little boy I had ever seen.

After a few minutes it was clear that something wasn’t right (I had not properly met him at this stage) and he was rushed off to the NICU. Five hours later I finally got to see the consultants, surgeons, anaesthetists, nurses, nurse practitioner, Doctors and (I feel like I have forgotten someone) oh yes, my little man! He was perfect, so small and perfect. They confirmed he had TOF/OA but just how bad was unknown. They wheeled him off to theatre, one of the worst feelings I have ever experienced. Around five o’clock he returned to the NICU. He had a short gap TOF/OA, a stoma had been fitted and it was confirmed that he was VACTERL. Most importantly though, the operation was a success. Recovery was pretty smooth running and two weeks and three days later we got to take him home, what an amazing day! We thought we could put all this behind us and finally be a regular family but that wasn’t to be. Ten days later we were back in hospital. Thys had stopped breathing (on the M1), we were rushed back to QMC, they gave him antibiotics and a week later we returned home.

Two days later we were rushed back to QMC. He had stopped breathing AGAIN. This time more investigations were done, his reflux meds were changed and home we went with a list of instructions on how he needed to sleep, drink his milk, a suitcase full of medication and an overwhelming feeling of being out of our depth. We watched his every move, if his breathing went quiet my thumb was twitching over the famous three numbers. To say we were on our toes is an understatement. As the incidents were always induced by crying, Thys got whatever he wanted just to ensure he continued

to breathe. We found ourselves wishing his life away, to when he was able to hold his head up which would mean his trachea would be stronger and hopefully these ‘blue spells’ would stop. Luckily they did. Hospital stays in his first year were frequent for numerous reasons, mostly the usual TOF/OA suspects of chest infections and many various viruses he picked up.

Four months on and Thys was ready for food, however his ability to swallow that food was not. On eight occasions Thys choked, stopping breathing on all of them. Many people would ask, “Why continue weaning?” but it was always on food he had eaten on many occasions before the choking occurred and a stage behind on what he was generally on. There was no specific food that caused a problem, so meal times were an anxious occasion for us. Oesophageal dilations made no difference either. Knowing that you could lose your child on at least three occasions during a day was something I could not face and so haven’t. *Touch wood, Thys’s eating issues have calmed down significantly, he still gets his usual ‘stickies’ from time to time, but on a whole Thys eats like any other nineteen month little boy.

Thys has had three oesophageal dilations in his life and six operations with one more to go. At times it feels as if we live in hospital but he is getting stronger and so hospital stays are becoming further and further apart. He is a typical little boy who now eats most things, he loves to ‘help’ Daddy fix things and is the cheekiest ‘ratbag’ I know.

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TOFS Northern party 2012 – Huddersfield

On Saturday 2nd June the TOFS Northern Party took place in Huddersfield, West Yorkshire, co-ordinated by TOFS and organised locally by Martyn and Claire Cooper-Jones. Martyn

and Claire have six year old twins, Harrison (TOF/OA) and Oliver. We were joined by eight families who had travelled from all areas along the M62 corridor.

‘Megan’s Rainbow fun-time’ provided the children’s entertainment, which included puppets, magic and games. After we had all tucked into our picnics, there was time to share stories and experiences of our TOF journey.

Fun was had by all children and grown-ups alike and once again I can say that I have made new friends, learnt new things and shared in great conversation. Many thanks to all of those families that attended and helped to make a truly enjoyable day.

(Editor’snote:thankyoufromTOFStoMartynandClaireforalltheirhelp.Duetotheirhelpthecommunitycentrechargedusonlyhalfprice for theroomhire so thanks to them too!.Seeall thephotoson theEventspage(picturegallery)oftheTOFSwebsite:www.tofs.org.uk)

Martyn Cooper-Jones

Many of you will have watched the BBC’s Great Ormond Street documentary series earlier this year, which followed some of our most experienced paediatric surgeons dealing with complex medical cases.

Diane Stephens talks to Mr Joe Curry MBBS, FRCS (Paed Surg), who was featured in the series. Mr Curry is the Specialty Lead for Specialist Neonatal and Paediatric Surgery at GOSH.

Tell us about you and your experience as surgeon.I've been a paediatric consultant surgeon at Great Ormond Street since February 2003. I knew when I first started studying medicine that I'd end up in paediatrics; I really enjoy being around children. I first experienced a TOF/OA baby when I was working as a trainee in Bristol. The first repair I carried out was when I worked at Southampton. As a surgeon, there's something special about this particular operation - it's immensely satisfying knowing that, with the technical competence, the repair can make such a difference to a child's life. I'm sure all children’s surgeons would agree.

How many repairs do you do annually? What is the success rate? GOSH will see on average 12-15 children annually and I’m responsible for about 2 to 4 of those repairs. For those children who have a straightforward TOF/OA, the success rate is very high. TOFS patron Professor Lewis Spitz did a study in the eighties and I was involved in the comparative studies in the nineties and the naughties and it is interesting to note the increase in success over these decades. That’s not to say that there were fundamental differences in the surgery over that period, just improvements in some of the things that go hand-in-hand with it such as improved antibiotics, better stitch materials, improvements in neonatal intensive care etc.

One of the main changes in the management of children with TOF/OA has been increased use of minimally invasive techniques and I've been championing keyhole surgery here at GOSH for some time now. Of course, its success depends on the individual surgeon's ability and training. The primary aim is to repair the oesophagus just as well as using the open procedure, but using keyhole surgery offers faster recovery for baby and of course less scarring. It’s been pioneered in the UK by Gordon MacKinlay at Edinburgh amongst others, and here at GOSH we were one of the first centres to trial it, but it’s only in the last 2 or 3 years that more and more of it is being done. About half of the repairs here are currently done using this

Mr Joe Curry MBBS, FRCS (Paed Surg)type of surgery and we are taking part in an on-going study on the effects of keyhole surgery on newborn infants in general.

What's the likelihood of babies getting infection post-surgery? It depends what you mean by infection; if you’re referring to infection in the scar area then the likelihood is low, just 2-5%. In general, the outcome for parents whose child faces surgery for repair is usually a happy one. What sort of problems do you see post-repair? Swallowing is probably the biggest issue. After the initial repair, the most common surgery we have to do are dilatations (see glossary) – on up to 30% of babies.

As a matter of routine, I immediately put my TOF/OA babies on anti-reflux medication as a precautionary measure, in the hope of reducing strictures. I believe that’s why, in the past ten or so years, in the 20/25 patients I’ve repaired, I’ve only had to perform 2 Nissens (see glossary). Before this, we know that up to 30 per cent of these patients were having anti-reflux operations. The percentage needing surgery for reflux is so much lower because they've had medication from the start.

What sort of follow-up do you do for your TOF/OA babies?I like to see my patients every 3 months for their first year; every 6 months for the next 2 years and annually after that until they're a teenager. I tend to see them all until they get into puberty. I spend a lot of time talking to parents about feeding, in particular about the likelihood of their child’s ability to swallow never being quite ‘normal’. I think they need to know from the outset that they may need to control the consistency of food, (but not necessarily the variety). I speak to parents about lung problems and signs of tracheomalacia and what to look out for, and they get resuscitation training from the nurses.

I like to talk with them about how to get through weaning although it may not be until they get on to stage 3 solids that children can experience problems. The oesophagus can't stretch at the site of the join so this is a potential area where food can get stuck.

We generally keep an ‘open-door’ policy so that parents can easily approach us if their child is having problems.

Send e-cards this Christmas and help TOFS!

We all like to receive Christmas cards from friends and loved ones. But in recent years, some people

have been sending really nice electronic cards and donating what they would otherwise have spent to

their favourite Charity. What a great idea!

In 2012, please think about sending your Christmas Cards electronically, and donate the savings to TOFS.

To help you do this, we can email you with details of suitable free or nearly free e-card services that you

could consider using.

Remember, we get no government funding, and every penny counts! Thank you.

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Family updateMayada Wazaify, mum to TOF Mohammad, 3, from

Jordan writes: “Mohammad is a very happy and active child. He faces some troubles in feeding every now and then and has had 6 oesophageal dilatations so far. I would really love to meet other parents of TOF children when I am in the UK in the future and of course I would like Mohammad to meet other children too. I tried to form a support group here in Jordan for TOF parents in collaboration with TOFS UK but I did not succeed in having doctors co-operating with me in terms of numbers and statistics. etc. Still I will be very happy to help and share with you my experience.”

Mr Joe Curry MBBS, FRCS (Paed Surg)Are you aware of difficulties experienced by your former patients as they need to get care in adult/adolescent hospitals?This is an issue for some patients. More and more, paediatric surgeons are thinking very carefully about what plans they put in place in relation to transition. Certainly, when I have a TOF adolescent who is continuing to have problems, I endeavour to refer them to someone with relevant experience.

What surgical techniques do you use when repairing long-gap OA?We always try and preserve the native oesophagus where we can, but not at the expense of a child being condemned to losing their childhood from being stuck in hospital. We’ve looked at other techniques, including the Foker technique, but with little success. In the absence of being unable to use the oesophagus we replace it by bringing the stomach into the chest. I’m really excited about the developments in stem-cell technology - the technology is racing ahead. I’d like to think we may be able to use this technology for oesophageal growth or replacement in the next 5 to 10 years.

When you can't do immediate repair do you do an oesophagostomy and sham feed?We don’t do an oesophagostomy immediately. We establish feeding through a gastrostomy and control secretions and swallowed saliva by suctioning through a special tube called a replogle tube. When babies are between 6 and 8 weeks of age we attempt to join the ends of the oesophagus. If this is unsuccessful then we may consider an oesophagostomy. I can’t over-emphasise the need for sham-feeding when a baby can’t have an immediate repair and has undergone oesophagostomy. It’s so important to maintain the normal swallowing skills in the baby particularly at a time when they have a full stomach. These skills can be lost in a matter of weeks but sometimes take years to restore. It can also remove the need for jejunal feeding tubes if the stomach is used to replace the oesophagus.

When we met at BAPS in Belfast last year you talked about specialist centres for TOF? Is this a good idea? Why?There are five surgeons here in GOSH who can do the TOF/OA repair. In the UK as a whole there are about 140 surgeons and bearing in mind that there are some 120 children to be repaired annually, my opinion is that we need to maintain institutional expertise in a smaller number of surgeons who would ‘specialise’ in doing this operation. This is indeed the case at many UK centres now. There are no moves as yet to establish specialist centres in the UK for more complicated cases and surgeons currently make appropriate decisions about treatment based on their own experience.

On a soggy Saturday afternoon, nearly 80 people, including 26 TOFs, turned out for the TOFS

London party. This was held very close to Gt. Ormond Street Hospital, indoors at Coram’s Fields play park. We carried some of the photos of the fun in Summer Chew, but had to hold this article over. (Editor’snote–lotsofphotosontheEventspage(picturegallery)oftheTOFSwebsite.www.tofs.org.uk)

The main point of these parties is to enable parents of young TOFs to meet up and talk, whilst there is something for the children to do. However 5 adult TOFs came along, and we were pleased to welcome the UK’s oldest TOF, Bridget Barson. Entertainment for the afternoon was provided by the larger-(and louder)-than-life Hillary of Heels n Toes, and many of the children had their faces painted.Trustees Duncan Jackson, Sophie Smallwood, and John Pearce came along to the party to chat to everyone – or at least to try to do that!

In parallel with the children’s entertainment in an adjacent hall, Dr Sandhia Naik gave a very interesting talk about the TOF condition to many of the parents and adults. Dr Naik is

a consultant paediatric gastroenterologist at the Royal London Hospital, where there is a lot of interest in living with the TOF condition.

She looks after a number of TOF children, including Megan Bryne, daughter of TOFS TLC and the party’s local organiser Jenny Bryne. (Thank you Jenny.) Dr Naik not only spoke at length and in a very understandable way, but she answered many of the audiences’ questions for quite a while.

We are very grateful to Dr Naik for coming along and helping TOFS to help TOFs in this way. We raised over £100 at the party from a raffle (thanks to Laura Jackson for masterminding the ticket sales) and continuing sales of the book “Noddy and the Farmyard Muddle” , which was written by Sophie Smallwood, who is the granddaughter of Enid Blyton!

The late April weather was a disappointment. Though there was plenty of space for everyone indoors at the two halls we had hired, it was not possible to really enjoy the extensive play equipment areas and mini-zoo at Coram’s. Oh well. Next time?

TOFS London Party 2012

Carole Shipley's first baby was born in 1971 and like many of us mums

in TOFS she was told that her baby

was ‘likely to be big'. Three weeks before her due date she was in hospital when her waters broke and, in the early hours on a Sunday morning, Adam was born. He was not a big baby at all. Carole remembers he didn't cry at first and needed a puff of oxygen. When staff tried to pass a tube, they announced that “something wasn't quite right” and whisked him away to the Children's Hospital.

Carole hadn't even held him.She was asked if she wanted him christened and was asked to sign a consent form 'just in case'. Hours later at 8am, her husband Pete returned with the Ward sister to say that Adam had oesophageal atresia and trachea-oesophageal fistula and was being operated on. Four hours later they were told that the operation had been successful … long disbelieving faces from Carole's mum and mother-in-law did nothing to dispel her anxiety.

In those days, new mums 'laid in' for ten days and for Carole this meant being sent to a convalescence home some miles away from the hospital where her son was being looked after. She expressed milk for her baby but was told she couldn't go home and she couldn't visit him.

Her GP visited her and said she was sorry to hear about her baby but “No”, she couldn't go home and “No”, she was not to go down and make a nuisance of herself! As a new mum aged 24, and at a time when most women hesitated to be assertive, she didn't dare to discharge herself. To add to her anxiety, relatives came to visit her having seen baby Adam, saying how lovely he looked and adding thoughtlessly, "...what a shame if he were to die..."!

When Carole was finally allowed to go to her son, she didn't even know which one was hers and on her first opportunity to hold him - to try to bond with him - she was only allowed to stay with him

for the duration of visiting time!

Thirteen stitches ran from his scapula right under his armpit and, on taking him home, no real advice was given. There was no support at home from midwives or from Health Visitors as the time period for visits was over and so Carole and Pete muddled through as best they could.

Carole was breast-feeding baby Adam but found he was frequently sick afterwards. She then established formula feeding and while he was still sick afterwards at least she was able to replenish it more easily. Later, they found he was allergic to dairy products.

He had a dreadful sounding (TOF) cough which had the usual effect on other mums in the vicinity who thought he had something infectious! Chest infections were routine but he coped better once diagnosed with asthma and given an inhaler.

Weaning took a long time and mealtimes literally took hours. Carole used the opportunity to talk to him and to read him stories which paid off in other ways as, not only was he an early talker, but he was able to read before he went to school. It helped that he was able to communicate about his difficulty with eating - he was able to tell Carole when another mouthful of food might make him sick or when he needed to go and be sick.

Following a barium swallow he was discharged by his hospital consultant around the age of three or four but mealtimes continued to be a difficult time. Going to school presented another hurdle but, as it was only around the corner, Adam came home for lunch.

Initially, Carole wasn't sure if Adam's difficulties were more hers as an inexperienced mum but her subsequent children were so easy to rear in comparison that she realised that the problems were his.

As an adult, Adam has always been very fit as he runs and cycles and he sports a good six pack! He has a Business Studies degree and a successful

career, spending some time out in Australia. He still uses an inhaler but doesn't seem to have any problems with eating and no problem with reflux that Carole knows about.

Fast forward to 2011 - a plane flight to Tenerife and for Carole, the need to tell a stranger in the next seat about her son having major surgery when he was born...as you will have read in the last Chew, the stranger was TOFS Trustee Sue Lewis-Jones, mum of a TOF child herself and, for Carole, the first time she had ever spoken to anyone else who'd had that experience.

Carole can't explain why she wanted to share that information. Was it something unresolved? Was it questions still unanswered that made Carole talk of her experience so long after?

Carole has since read TOFS leaflets, back copies of Chew and 'The TOF Child' book, soaking up hitherto unknown information like a sponge! She has met other TOF parents and other TOF adults and was looking forward to going to the Adult TOFS Conference in September to learn more.

Nothing can change the anger and guilt she still feels about being unable to hold and bond with her first baby in his first weeks but in finding TOFS she has learned so much about Adam's condition and realises the extent to which that affected his early years. She has read of the dreadfully complex problems that some TOF babies have. She is also now very aware of the need for Adam as a TOF adult to take good care of himself, to take his health seriously and to get any signs of reflux checked out thoroughly.

Carole would have loved to have had the support that TOFS can offer forty one years ago.Meeting another TOF Mum on a plane to Tenerife was an amazing coincidence, one that has brought answers to questions she didn't realise needed answering, and something that Carole will never regret.

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Forty one years without TOFS! Carole’sstory

Look out Jezz Moore – we have another Marathon contender in the shape of little 4 year

old Issac Jones, (VACTERL) son of Philippa and Owen, from Yorkshire. Philippa writes:

“My son Isaac completed the Mini Great North Run in September and proceeds are to be split between TOFS and the Leeds Teaching Hospital. Issac was amazing – he ran the whole course without stopping or walking! I had to keep telling him to slow down as I couldn’t keep up with him! It was a truly fantastic and inspirational day. I felt like the proudest mummy in the world as we

crossed the finish line. When we had finished the run the first thing that Isaac said was, “Can we run it again now mummy?”

We were delighted when Josef Craig the Paralympic Gold medallist and the Lord Mayor both gave him a ‘high 5’. We also got the opportunity to watch Mo Farah in the City Games.

I haven't collected all of the sponsor money yet but so far we have raised £1229.20! We never expected to raise such a huge amount.” Editor’snote – Wow! If you’re impressedwith Issac’s achievement you cancontinue to support him with hisfundraising page: charitygiving.co.uk/isaacjones

Go Issac Go!

TOFS trustee and adult TOF, Wendy Bell raised an amazing £740 with a

coffee-morning. Read on and find out how she managed to raise such an amazing amount…

‘As a trustee of TOFS I felt it was time to roll up my sleeves and do some good old fashioned fundraising!

I decided on a coffee morning, chose a date and decided to pre-sell tickets at £2 each to include a cup of tea or coffee and a biscuit. It was a great way to get a donation from people even if they then didn't come.

Mum and I baked like mad and some friends donated cakes for our cake stall. We had a tombola for which I got prizes donated by local shops and friends. People had been so generous with prizes that I was able to hold a raffle as well. I got gym passes from the Nottingham Belfry where I am a member. I got two beauty treatments donated, a beautiful bouquet of flowers from a florist and a meal for two at a Thai restaurant.

All I did was ask for donations! If you don't ask you don't get! I explained what the charity was and that I was holding a coffee morning and hey presto I got some amazing prizes.

I had a book stall, bring and buy, and a beautician doing manicures. I also invited two ‘friends of friends’ to sell

cards and jewellery for a donation to TOFS out of their takings. There was plenty for people to spend their money on on the day. We set out tables and chairs so people could stay and socialise and eat their cakes, and buy a second cup of tea!

I was amazed when after just 3 hours on a lovely Saturday morning we’d raised £740 for TOFS! Obviously I couldn’t have done this without the support of my family and friends who helped out and spent

their money. The best money raisers were the cake stall which made £145 and the raffle that took £120. Yes it took hard work but it was well worth it to have achieved such a fantastic result.

So come on all!!!Get fundraising!! If I can do it so can you!’

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Past trustee Pat has completed her Coast to Coast expedition and

raised an amazing £734 for her two nominated charities, TOFS and Fylde Sharks (swimming for disabled children).

Pat has long been involved with both TOFS and her local branch of the women’s group Soroptimist International. The donations were made through Soroptimist International of The Fylde and we are very grateful that current president, Linda Beddows, persuaded her to follow up sponsorship when Pat thought she had exhausted the generosity of friends and family far too often. Pat writes“Attached are a couple of photos: the first of Kidsty Pike which is the highest point of the walk and the other is the ritual dipping of my toes in the North Sea. The intention at this point was for a suitably tee-shirted photo call but the weather had been so wet all day. I am sure it would have been easier to

swim the last 20 miles rather than walk it, as we did. The triumphal march along the cliff-top to Robin Hood's Bay gave absolutely nil visibility and I had been wondering all day just how long you can have wet feet before your nails drop off.

But that being said, it was the most wonderful experience and every moment of it was fantastic in its own way, not only the route but the people, the odd places and situations we found ourselves in and the terrific camaraderie of the people Chris, John and I met along the way. We managed to walk 196 miles instead of the prescribed 190, proving it wasn't all a stroll along signposted ways. It was fascinating to plot the route over mountains and moorlands, through dales and rivers from the Irish Sea at St Bees to the North Sea of Robin Hood's Bay. My long allegiance to TOFS continues. I do hope that funds and publicity continue to grow for both TOFS and Fylde Sharks.”

Pat Fergusson, continues to fly the flag for TOFS!

A n A m a z i n g C o f f e e - M o r n i n g

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SeptemberCarly Campbell 09/09/1996Frazer Earl 13/09/1996Kate Robbins 15/09/1996Daniel Green 15/09/1996Christopher Benjafield 04/09/1997Samuel Riley 09/09/1997Martin McDonnell 16/09/1997Amy Lawton 26/09/1997Bethany Gardner 09/09/1998Jamie Gorman 25/09/1998Phoebe Crabtree 01/09/1999Daniel Turner 03/09/1999Lewis Espley 04/09/1999Megan Byrne 20/09/2000Thomas Spencer 18/09/2001Harry Knighton 19/09/2001Richard Weedon 1/09/2002Joshua Earl 24/09/2003Daniel Jeffreys 09/09/2004Amy Fox 18/09/2004Daniel Hall 20/09/2005Lolah Thompson 01/09/2006Charlotte Royston 05/09/2006Ruby Moran 06/09/2006Sam Michele Neeson 09/09/2006Samuel Harrington 09/09/2006Serena Browne 18/09/2006Niamh McGlinchey 28/09/2006

OctoberPhillip Mainwaring 21/10/1996Annie Mae Doherty 12/10/1997Francis Harrison 22/10/1997Caitlan McCoy 26/10/1999Bernadette Quinlivan 05/10/2000Corin Astles 09/10/2000Bailey Stark 13/10/2000Dylan Baker 22/10/2003Lucas Nixon 27/10/2003Adam Howarth 31/10/2003Louisa Shadlen 21/10/2004Bradley Rowlinson 01/10/2005Nadia Esseku-Otoo 01/10/2005Skye Barker 11/10/2005Daisy Stacey 16/10/2005Isla Norrie Botto 23/10/2005Evie Hardwick 03/10/2006Ashley Tipler 09/10/2006Benjamin Evans 13/10/2006Kayden Dearman 15/10/2006Connor Maskrey 16/10/2006

NovemberCiara Myton 09/11/1996Eleanore Derham 18/11/1997Max Williams 23/11/1997Kate Remmington 26/11/1997

Jodie Kreft 28/11/1997Miles Caven 19/11/1998Adam C Williamson 17/11/1999Maddison Tyrer 21/11/2000Dylan Robinson 07/11/2001Sinead Forde 08/11/2001Elliot Smith 20/11/2001Saffron Corbett 27/11/2001Luke Warburton 03/11/2002Milly Dimelow 07/11/2002Nathan Ayling 11/11/2002George Plapoutas 14/11/2002Benjamin Sims 27/11/2002Matthew Dolan 28/11/2002Isobel Cattle 19/11/2003Elizabeth Mole 21/11/2003Benjamin Bailey 08/11/2004Amy Ansbro 20/11/2004Honey Chapman 21/11/2004Artemios Tsolakis 03/11/2005Megan Fletcher 04/11/2005Freddie Hughes 18/11/2005Isaac Blagden 22/11/2006Isobel Winspear 30/11/2006Olivia Burnside 30/11/2006

December Carrissa Page-Best 02/12/1996Joshua James 14/12/1996Emily Boyce 25/12/1996Alexandra Dickinson-Lomas 26/12/1996Joshua Banks 30/12/1996Lucy Thompson 04/12/1998Kristie Rolfe 05/12/1998Jacob Eckstein 09/12/1999Jae Dworzanski 22/12/1999Alexander Bardini 30/12/1999Jonathan Maze 08/12/2000Sophie Wakeley 14/12/2000Ben Cowling 30/12/2000Elliot Bartrum 14/12/2001Ioli Heskes 06/12/2002Felicity Brown 11/12/2002Erin Nicoll 14/12/2002Freddie Ellis-Batchelor 19/12/2002John-Carlo Hanratty 12/12/2003Joe Whitham 02/12/2004Jasmine Rose 11/12/2004Archie Muir 17/12/2004Harriett Cole 05/12/2005Ethan Reynolds 08/12/2005Daisy Fuller 14/12/2005Leo Allen 25/12/2005Kyle Thorne 03/12/2006Maisie Wharton 05/12/2006Jack Brockley 10/12/2006Olivia Lancaster 15/12/2006Alexander Barker 27/12/2006Cheyna Fusco 29/12/2006

Happy Birthday to all our TOF-OA childrenChildren six years and above are listed below, under sixes will receive a birthday card.

Happy belated birthday to Sasha White who was 9 years old on the 30th August.Fundraising

Thank you to:• Rivendell Europe Ltd - £10 donation from Mr Graham Flynn for retaining his old Company mobile phone• £20 donation from Billy Salterthwaite (uncle to Luke Scott born TOF/OA) and £10 from Dorothy Tuddenham who is a family friend• Chepstow School held an annual fundraising week and raised £91.98. Our thanks go to Tyler Morgan-Huggett, sixth form student and twin to Tudor born with TOF, for putting our name forward to be their nominated charity• St Peter’s Church, Ipsley donated £32.84 following a recent service• Gedling Borough Labour Councillor Barbara Miller donated £50.00• £20 donation from Jean Bradley – In lieu of gifts celebrating the wedding of Charlotte Shaw and Michael Scott, parents to Zachary born with TOF/OA• Ysgol Gynradd Cwrt Henri Primary School - raised £150 by doing a sponsored walk• Keri Patton from County Down and mum to Blake born with TOF/OA, raised £200 by holding a Coffee Morning in our April ‘Awareness Week’• Sarah Bowmer raised £70 by having a Table Top Sale in their village• Andrew and Rose Anderson recently celebrated a Wedding Anniversary and donated £70 to TOFS in lieu of receiving gifts• Karen Pollock - £50 donation• Bluebell Barn Nursery donated £40 in support of James Moore’s Marathon run• Julie Francis (mum to Marley born with TOF) and mother-in-law Teri Francis who raised £260.00 by holding a candle party• Provincial Grand Lodge of Surrey - £750 donation• Easyfundraising.org.uk - £36.63 donation• Barry Waite - £10 donation• Carole Shipley - £30 donation• Frances Unsworth who was born with TOF/OA, and her friends at the Bowburn Toddler Group raised £82.50 by doing a sponsored walk • Dave and Lesley Butler, neighbours to Iris, Keith and Christopher Benjafield, donated £200• Mrs Beryl Mason, grandmother to 24 year-old Elliott (VACTERL) and the Mother’s Union, attached to the St Mary’s on the Rock Church, Ellon, Aberdeenshire. Beryl made tea and cakes and hosted a garden walk around the grounds of her bungalow and raised £110.81. (And congratulations to Elliott, who has now successfully graduated with an Honours Degree in Architectural Technology – whilst working at the same time! Well done and good luck in your chosen career)• Vikki Hibberd who raised £148 through Justgiving• John & Alison Pearce - £35 CAF donation Read about some of our other fundraisers in separate articles in this Chew!

In memory:£145 collected at the funeral of Edith Mary Sayer, Great-Grandmother of Joseph Styles born with TOF Noreen Ramsay-Dowling - £20 donation from Zoe Powers, mum to Colin born with TOF/OA

200 Club winnersJuly – Carolyn Seeley from LiverpoolAugust – Joanne Winspear from LeedsSeptember – Tracy Robinson from Kent

N e w b u r y Night Races

We were delighted to be invited, yet again, to

make the charity collection at Newbury Racecourse (this year, at the Pump Technology Race Evening).

Indeed most of the TOF families present had attended one of our Newbury events before, so

thanks are very much due to the Baker, Thompson, Ellis-Dale and Poole families. Trustee Sophie Smallwood was there as well: Vice-Chairman John Pearce also attended along with his adult TOF son Rob and a couple of his family members to make up the numbers.

We all had the chance to see several of the evening’s seven races, and everyone seemed to enjoy themselves. Better still, the collection from our 16

buckets added up to £1070 – a great outcome for TOFS. Thanks are particularly due to: Newbury Racecourse (for inviting us), and Ian Thompson, the top collector with just over £200.

Editor’s note: We couldfundraise at a racecourse (orany major sporting venue) inyour area. Just let us knowif you’re willing to approachthem on our behalf and we’llworkwithyoutoputaproposaltogether.

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TOF dad, John O’Connor and friends, Dave Morgan, Gareth Simpson and Jon Joy from Colerne, Wiltshire raised an amazing £717 for TOFS. Thanks very much indeed!

John writes: “Mountain Mayhem is an annual 24 hour mountain bike event centred around the woodlands and hills of Eastnor Country Park at the southern end of the Malvern Hills.

My three willing accomplices and I decided we would enter a 4 man team and use the opportunity to raise some money for TOFS. Dave and I had been part of a 5 person mixed team the previous year so had a reasonable idea what was involved and agreed the added challenge of competing with a smaller team would be fun … if only we had known!

It was announced Saturday morning that conditions were the worst ever experienced in 15 years of Mayhem.

The race started at midday Saturday with a Le Mans style start; this meant a 1.5km run before collecting bikes and heading off on the course. Dave was our designated runner and completed the run in good time, collected his bike and set off on the 10 mile loop, returning 2 ½ hours later. With a quick shout of ‘Man, that was muddy’, he handed over the baton and I set off on the second lap.

He wasn’t wrong, much of the course was so deep in mud, it was virtually unrideable and frequent stops were required to remove the build-up of mud on the bike that was preventing the wheels from turning! I eventually made it back to the arena and handed over to Jon who completed his lap in a little over 3 hours due to mechanical failure and emergency repairs needed at the trackside before he could continue.

Last to go was Gareth, a last minute replacement for one of our original team members. Gareth had only returned from holiday in Egypt 3 days earlier and was still suffering from the effects of the Egyptian equivalent of Delhi belly! Despite this he set an excellent time of 2 hours 15 minutes.

By now we were riding in the dark, with Dave setting off for his second lap at around 11 pm. And to make things worse, the rain had started again. Dave made it back a little after 1 am reporting worsening conditions; more mud, more puddles!

I set off but soon experienced mechanical problems. At around 3 am, I found myself pushing my bike up the longest climb, in the dark, in horizontal rain, wondering what on earth I was doing there! I persevered and finally made it back in to camp at 4 am.

Having had mechanical failure and being unable to buy a replacement part, Jon was unable to go out again and Dave and I had both declared we were done so this left Gareth but, given his gastro problems, he also felt unable to do anymore.

So our final tally was 6 laps which turned out to be quite respectable, placing us 81st from 125 teams in the Open male category. There were a large number of teams that completed 1 lap each and called it a day (I don’t blame them).

Despite the conditions we had a great time and to make it especially worthwhile, we raised £717 for TOFS. We chose to support TOFS as my son Ronan, was born with TOF & OA in June 1997. He was successfully operated on 24 hours after his birth at St George’s Hospital in Tooting. Since then he has required a dilatation on a number of occasions but has otherwise been well. During the early stages, we made use of the services provided by TOFS and are extremely grateful for their support.

We hope that raising this money will help enable TOFS to continue providing support to families in need.”

Mountain Mayhem

Annual fundraisers, the Offord/Wilsmore family from Essex have been at it again, completing a very WET and COLD 7 mile Thurrock Cycle Ride in

early June. Alice (TOF/OA) has a weekend job so couldn't make it, but mum Ann-Marie, dad Ian Wilsmore, Uncle Andrew Offord and 14 year old sister Laura Wilsmore finished it in around an hour which was amazing considering the awful weather!! They raised £150, £68 of which was raised by Laura asking her generous school friends to sponsor them! No wonder mum and dad are so very proud of her!

Thank you, from TOFS.

Thurrock Cycle RideWhen James (Jezz) Moore from

Redditch in Worcs was awarded the much-coveted TOFS place in

the London Marathon, he didn’t let us down, completing the marathon in 4hrs 30mins despite the heavy rain, and raising an

amazing £3004.80 for TOFS. James is uncle to 3-year old TOF/OA Joseph Wood from Eastbourne, East Sussex. James is currently on his travels. Please send him a big

thank you from TOFS!

London Marathon

l-r Gareth, John, Dave and Jon

l-r Ian, Andrew, Laura and Ann-Marie

Joseph Wood Jezz Moore

Fundraising

This edition, our ‘Didn’t they do well’ series features Torchbearer TOF Teenager, Ben Fox

So many of us have taken part in one aspect or other of the Olympic or Paralympic games this Summer – mostly as spectators, no doubt. VACTERL teenager Ben Fox did better than most of us – he was selected as an Olympic torchbearer, and carried

it through Royal Wootton Bassett – close to where he lives in Swindon, Wiltshire. For someone born with quite a catalogue of anomalies, including only one leg, this was a very impressive achievement. Having completed the journey, Ben described it as “incredible. An amazing experience”, as well he might. There is a video clip of him carrying the Torch on the BBC News website and he stirred up quite a lot of comments on Twitter at the time.

Ben was born in Swindon to mum Carol Hearne. It was immediately apparent that he had no right leg; but it also turned out that he also had 2 extra vertebrae, a hole in the heart, imperforate anus, TOF and OA. He was whisked off to Oxford’s John Radcliffe Hospital, now the Oxford Childrens’ Hospital. He has been a very frequent visitor there, having had some 32 operations over the years. As you might expect, he has got to know the staff there really quite well, and surgeon Rowena Hitchcock showed up to support him when he carried the Olympic Torch. She told the local newspaper at the time: “I met him the day he was born. Ben’s condition was very complex, he is an extraordinary boy. When he was born we were struggling to keep him alive.” Ben’s family were supporting him as well of course – he has a brother and sister who don’t have TOF, OA or any of the other VACTERL problems.

Since taking part in the Torch relay, Ben has visited Oxford Childrens’ Hospital, and showed off his Olympic Torch to his old nurses and doctors, and some of the current patients too.

Ben’s care has now been handed over from the Childrens’ hospital largely to expert consultants at Oxford, quite a lot of them: like many teenage TOFs he is now having to get used to many consultants rather than the one trusted person (Rowena Hitchcock) who has looked after him throughout his childhood. Further surgery is expected when he reaches his early 20s.

Ben took his GCSEs in Summer 2011, and is now studying BTEC Sport at New College in Swindon. He passed his first year, has just started year 2 and has added Photography to his studies in order to achieve enough points to be able to go to university, either Bath or Loughborough. He currently hopes to become a Sports Coach, and .already has a part time job with his local council in Swindon, working with children – both able bodied and disabled - introducing them to all kinds of sport.

Ben is a keen sportsman. He began playing amputee football, playing for Chelsea - quite hard to do when you are an Arsenal Fan. He has skied in

Colorado and Italy, on one leg with outriggers. Following a chance meeting in a car park, Ben took up wheelchair basketball. He is a member of the South West Scorpions club based in Bristol, and was Captain of one of the teams taking part recently when they set a new World record for the longest ever wheelchair basketball match, playing for 27 hours and 32 minutes. Unsurprisingly, he has watched the current GB wheelchair basketball teams in action in London in the Paralympics. As a member of the GB wheelchair basketball development squad, Ben has been receiving support from the Talented Athlete Scholarship Scheme, a government-backed development programme. Others who have been supported in the past include Rebecca Adlington, Amy Williams, Ellie Simmonds, and Tom Daley. Ben has already played for Great Britain once (in 2010). He has hopes to make the grade as part of the GB wheelchair basketball team for the Rio 2016 Paralympics! We wish him the very best of luck, and hope to see him on TV then!

Didn’t they do well

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Didn’t they do wellB e n Fox

The 75th Bolton (St Peters) Brownie Pack is led by Charlotte Underwood, who is an adult TOF. The pack has been very busy

raising a lot of money for TOFS this year. It adds up to a fantastic £700 – thanks very much to the young ladies and their many helpers, of course.

The pack have been holding Coffee Evenings, doing sponsored Good Turns, running stalls at the local Belmont Fell Race Day and collecting donations.

TOFS local contact Kay Leech went along an event (as requested by Charlotte’s mum, Fiona) and was delighted to be presented with a cheque for the £700 for TOFS.

Kay took her husband Gary and TOF/OA Harriet (aged 4) along with her, and had a lovely time.

B r o w n i e Fundraiser

Gary and Kay Leech with Harriet and Charlotte Underwood

Ben Fox

Do you know a member of Rotary, Lions, Masons or Soroptimists? Please ask them to nominate TOFS in their fundraising. Remember, we get no government funding. Please let the TOFS office know you have done so. Does the company you work for (or someone you know) make charitable donations? Please ask, and if it’s possible to nominate TOFS, we can help wilth filling in forms etc. Just get in touch with the TOFS office on 0115 961 3092 or email info@tofs.org.uk

Or perhaps you’d like to help with Chew? If you think you’d make a good proof-reader and have a few hours to spare (once every three months) then get in touch with Diane at the office.

Can you help TOFS?

Didn’t they do well

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All I ever wanted to be was a Mummy. I remember as a little girl when I wasn't sorting out maggots on fishing trips with my Dad, I was upstairs putting my doll in her cot, closing my bedroom curtains and letting her have her afternoon nap! I was lucky

enough to find Mr Right and so Nick and I, a year after being married went to Guys Hospital to see a team of Drs that specialised in ladies who had long standing health problems. I was the first TOF/VATER Syndrome patient who had come to see them. They had read my numerous medical files before seeing me and all they could really say was that I would have to be watched like a hawk and have extra monitoring as, put simply, they had never had anyone like me before so we were all going into it blind. What they didn't say was, "No".

I had my oesophagus and hole in the heart repaired at birth in 1976 but when I was sixteen my oesophagus packed up. The motility failed to work and I became a very unwell teenager. I was transferred to Guys and St Thomas' after becoming too old for GOSH. My transition to St Thomas' was nothing but positive (apart from the mixed wards at the time). I had a myotomy which didn't work which then resulted in a total oesophagectomy at eighteen by a wonderful man, Mr Dussek who has now retired. Mr Dussek then had to do further surgeries due to my stomach not emptying, removing adhesions, and performing a pyloroplasty.

Under the care of my current surgeon, Prof Bob Mason, I have had several more operations, from sorting out two diaphragmatic hernias where my bowel had entered my chest, more adhesions and having feeding tubes fitted. I also have a horse shoe reflux kidney with reduced function on the left side, a stomach that still doesn't empty, reflux, a leaking valve and a bi-cuspid valve and an aneurysm in my ascending aorta in my heart (I didn't find out about my aneurysm until after I was pregnant). I had an ASD repair in my heart in 2006. I get bad chest infections from aspirating and lots of diarrhoea.

Upon finding out I was pregnant there were tears of both joy and fear. I was taken under the care of Miss Rao at my local hospital in Chelmsford and by the super team at St Thomas' made up of Prof Catherine Nelson-Piercy and Kate Hardy. I fell pregnant in September 2007 and by January 2008 I was in hospital where I remained until our little Beatrice was born six weeks early on April 30th at 8pm. My main problems when I was pregnant were pain, chest infections and vomiting. The bigger Bea got, the more she pushed up on my hernia and stomach in my chest which caused pain and severe sickness. Even when I was rock bottom I always knew everything was going to

be ok. Bea always kicked loads in the evening so Nick would stay late at my local hospital so we could sit and get comfort from seeing my belly move! The staff would tell everyone to go apart from Nick. I became part of the furniture, as did all my family. Every day I would have the doppler put on my tummy and there it was, that strong beating heart telling me everything was going to be fine.

The day I went into labour was very strange. I had been moved back down on the labour ward yet again due to another chest infection. I remember lying there with the CTG monitor on and seeing a few peaks on the print out. I buzzed for a midwife and very calmly said, "Am I in labour?" I guess the look

on Barbara's face and tears was a "Yes!" Word soon got about that their long serving pregnant Mummy was in labour and my room was soon full of Drs and the midwives from upstairs on the pregnancy ward that I had been on. I tried calling Nick but no answer. I rang Mum who was on a bus. "Mum, where are you?" "I'm on a bus coming into Chelmsford". "Oh, it's just that I am in labour!" Mum was soon in my room being given a sweet cup of tea. The paramedics and ambulance arrived and soon enough I was on my way to St Thomas' with sirens. Even after getting to St Thomas' Hospital at rush hour from Chelmsford within 30 minutes, I felt so calm. I loved my bump more than ever and I remember just telling Bump and myself that this was the beginning of something amazing, something that miracles are made of. My Mum as ever, was just brilliant, calm on the outside but probably worried sick on the inside but she never showed it. Waiting at St Thomas' was Nick and my Dad soon to be followed by my big sister, Rachel and my now brother in law, Matt. I was given an epidural straight away and waited....and waited.....at about 7.30 in the evening Bump’s heart started showing signs of distress so I was whisked into theatre. I was eventually given a general anaesthetic to get Bea out as quickly as possible. Beatrice was born. She needed some help for a little while and was then taken to ITU as she was early. My family all saw Bea before I did and then Nick went to ITU with her. I was taken to high dependency on the birthing ward and slowly came round with lots of tubes in me. I remember Nick coming up to see me and showing me photos of Bea and told me I would see her in the morning. My heart sank. I slept on and off but at 11.15pm the doors opened and in came a nurse with a baby in a cot saying, "Here she is!" I always knew Bump was a fighter, She had clearly got bored of ITU and was able to come and see me after only four hours. She was tiny. She was perfect. She was 100% healthy!!!!! The next day was so special seeing my parents and my sister and Matt with Bea.

Over the next few days I got back on my feet and had close family and friends visit. Bea and I came home on May 9th and we have never looked back. My pregnancy was far from a text-book pregnancy, it was hard and I was very unwell at times. I guess there will be people who will say that when you full well know you have something wrong with you, should you go on and have children? The jury seems to still be out on this when it comes to TOF but I only have to look at Bea and my answer is there. She was worth every injection, every pain, every tear, everything. She makes my poorly heart sing the loudest it’s ever sung.

Chew hears from Adult TOF Katie Spall-Burrell on her quest to be a mum….

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Our second TOF adult conference was held in Reading, at a smart conference suite

in a Holiday Inn. TOFS Chairman Duncan Jackson welcomed everyone to Reading, and introduced our chairman for the day, TOFS patron Mr Ed Kiely. Mr Kiely is a very senior paediatric surgeon at GOSH, who has operated on and subsequently cared for hundreds of TOFs.

We were particularly pleased to be joined by TOFS patron Professor Lewis Spitz, who pioneered many neonatal surgical approaches and techniques at Great Ormond Street before he retired. We welcomed as our guests Pam Dowling and Tammie Sims (mother and carer to the late Noreen) and some representatives from our fellow support groups overseas.

Our first major session in the morning was from Dr Richard Russell, who is an expert on long-term respiratory health and who practices at Windsor, amongst other places. Dr Russell answered many questions about lung health after his talk.

Mr Nick Maynard, a senior surgeon and expert on oesophageal disease from Oxford, led our second major session. The feedback forms suggest that this was one of the conference’s highlights, perhaps indicating the prevalence of reflux and its relevance to our TOF adults. (See separate articles on both sessions in this Chew).

John Pearce (TOFS Trustee: Vice chairman and Treasurer) spoke

briefly about the various things TOFS has done to help support adult TOFs. He mentioned particularly the increase in stories and articles relating to adult TOFs in Chew. He also launched the new TOFS leaflet for GPs about adult TOFs. Most people took a copy or two of this away with them!

Graham Slater (TOFS Trustee: Secretary, and also Chairman of EAT) explained about EAT, the federation of support groups which TOFS has founded with the German, French and Dutch TOF/OA support groups. Graham pointed out that there are probably 60,000 TOF people across the EU – it does not seem like quite such a rare condition.

The conference included a major session on the transition from childhood to adult care including some scene-setting from TOFS Trustee Dr Caroline Love and personal stories from an adult TOF, Lauren Leaver and parent Mary Parkes, mum to Adult TOF Nick.

We ran three interactive “workshop” sessions (in parallel) at the end of the afternoon, on Scarring, Counselling and Health and Exercise. We will report on the workshops in the next edition of Chew.

These conferences have a lively social element and a small group took over the hotel’s bar on Friday night. On the day of the conference itself we had probably the largest gathering of adult TOFs in Europe this year. Many were pleased to meet Bridget Barson, who is Europe’s oldest TOFs. There was a great deal of chatting going on!

TOFS 2012 Adult ConferenceAdult Conference 2012

Judy Riley (Beds), Hazel Walsh-Atkins (Birmingham), Christine Shepherd (Nottingham)

Karen Maycock (Cambs) Gill Jackson (Derbys)

Our group of Adult TOFs

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Adult Conference 2012

Making the Transition: listening and learning from each otherDr Russell, Respiratory/Chest SpecialistHonorary Clinical Lecturer, Imperial College, London and Consultant Chest Physician

Dr Russell is the Consultant Chest Physician, King Edward VII Hospital, Windsor. His presentation centred on chest infections with particular

emphasis on communication with adolescents.

Dr Russell began his presentation with a cartoon sketch to indicate that not everyone is the same and should be treated on an individual basis. He likes to treat his adolescent patients as adults and believes that it is important to explain the origins of a respiratory disease in a simplistic way with the use of pictures, stories and colour, so that patients can understand the reason for their treatment. In order to alleviate the wheezing, coughing, shortness of breath that is associated with chest conditions, there is often a need for very high doses of medication. Dr Russell explains this to his patients so that they understand the reasons for the treatment. In this way, he hopes they will not cease taking their medication. When lungs are inflamed, they produce unpleasant symptoms and the purpose of the proposed treatment is to limit damage to the airways.

Professor David Price, of Aberdeen University, carried out a survey of the wishes of patients. He found that patients would like no “bad asthma” days and no restrictions. They were not particularly interested in peak flow monitoring. A further study indicated that there was a significant percentage of asthma sufferers who reported problems with sleep, missing work or school, and having to receive urgent medical care as a result of asthma attacks, causing disruption to normal activities. The purpose of medication is to relieve and prevent attacks. Inhaled corticosteroids are the anti-inflammatory treatment of choice. Correct usage results in rapid improvement of symptoms and lung function even at low doses of steroids and their use is associated with a decreased risk of death from asthma. In Great Britain last year, 1330 people died as a result of not taking their medication correctly. In Finland there were 7. People must be educated to manage their symptoms.

Few patients regularly use steroids correctly. Why? One of the reasons is that they are afraid of developing enormous muscles! Using appropriate language and education in the use of steroids is important in getting “buy-in” from patients. Everyone should remain positive – there is no room for negativity. It is very important to take the prescribed medication to prevent attacks.

The pattern of referral for a patient is typically:• Admission either to an Emergency Department, or via a GP or nurse

• Consultation with a Specialist Nurse or Consultant

Each patient is treated individually ensuring that they are prescribed the correct drug, dose and device. A self-management plan is discussed. Patients require someone to listen to them, and thoughtfulness when being dealt with.

In response to questions from the audience, Dr Russell explained the link between chronic reflux and bronchiectasis. Bronchiectasis does not cause asthma. Reflux produces changes in the oesophagus over a period of time. This can lead to Barretts Oesophagus. Acid from the stomach may get into the lungs where it sinks to be bottom of the lungs causing the lungs to respond by producing excess mucus. This may lead to the formation of ulcers, inflamed cells which thicken and reduce the lungs’ capacity making breathing difficult. The mucus allows bugs to enter the lungs where they remain, causing infection. The nose filters some of these bugs, but not all of them. We each produce about 1 pint of mucus each day. If bacteria enter the lungs, then a chronic attack may occur some 14 – 16 hours later. Individual inflammatory responses require different drugs. Constant attacks will eventually damage the airways.

Christine Shepherd

Grandparents Sue and Mike Bartlett, and Sandra Pickett (Thatcham, Berkshire) French guests Adult TOF Aurèlie Villecourt, Frèdèric Armand, and son Jules (OA)

Jay-Anne Vogan with Adult TOFs Stephanie Vogan (Berkshire), Faye Barnes (Plymouth), and Mark Burgess (Southampton) Trustee and Adult TOF Sophie Smallwood (Midhurst) and TOFS Patron Lewis Spitz

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It has become apparent that finding specialist care for adult TOFs experiencing TOF related problems is very difficult as few adult surgeons and physicians have experience of the issues we encounter. This makes the transition from paediatric to adult care challenging; as yet there is

no clear pathway and many young people are referred back to their GP who may have little understanding of the condition. This issue of transition from paediatric to adult care was addressed from three perspectives. Dr Caroline Love discussed transition from a medical angle; Lauren Leaver from Australia described her experience of moving from paediatric to adult care and Mary Parkes, mother of 21 year old Nick, spoke from a parent’s perspective of her son’s experience of transition.

Caroline began by defining transition: “Transition is defined as a purposeful planned process that addresses the medical, psychosocial and educational needs of young adults with chronic physical and medical needs as they move from child-centric to adult centric health care”. Judging by the experience of Lauren, the Parkes’ and many others, this is not yet happening for young people with TOF. Lauren in Australia and the Parkes in England finally found the right doctor but they had to do the research themselves and the care Lauren and Nick received in the interim was poor.

Caroline pointed out that when a person moves from paediatric to adult services the family centred holistic outlook is left behind and one is confronted by busy and often impersonal departments where there is little knowledge of the condition and time for the medics to understand the surrounding issues. At 18 Lauren was discharged from paediatric care and told to find her own doctor. Lack of knowledge of the condition meant that the doctors she then saw did not believe she needed any treatment. The first dilatation she had as an adult was done without a general anaesthetic. The experience was so unpleasant that she understandably refused further much needed dilatations until she found a more careful/caring surgeon. Nick was also transferred out of paediatric care at 18. He was seen as an outpatient in an adult department and saw a different doctor at each appointment, none of whom had knowledge of his history or particularly of TOF. Mary spoke of the concern and confusion she felt when Nick was called for tests that did not seem to relate to his medical problems.

Mary and Nick asked to have his care monitored locally. They waited 18 months for an appointment. They had waited so long that when the day came,

he actually missed the appointment. As Caroline points out this is a common problem amongst young adults, particularly those managing their own health care for the first time. Sometime after that Nick developed symptoms of reflux despite having had a Fundoplication. He was admitted to A&E vomiting old blood and was seen by a registrar who had little understanding of TOF and disbelieved what Nick told him. The doctor diagnosed constipation and he was discharged. In desperation to get real help Mary was put in touch with another parent through the TOFS office who told her about the gastroenterologist Dr Levitt at University College Hospital in London (UCHL). For funding reasons the local hospital would not refer Nick directly, and he had to ask the GP to do it. It took many months, phone calls and a special request to the Primary Care Trust (PCT) before he got to see Dr Levitt.

Lauren and Mary’s talks illustrate the points Caroline made regarding the lack of understanding and, in some cases, lack of interest in TOF problems in adult services. As Lauren discovered, there is a huge difference in the type of care given once you enter adult departments. She went from feeling very special in paediatrics to being frankly disbelieved by the adult department. Caroline emphasised how important it is for people with TOF to learn about their condition and what issues may arise. Lauren indicated that she had no mechanisms for coping on her own and felt it would have helped if she had known what to expect.

Because there is no pathway for transition to adult care for TOFs as yet, Caroline recommended that young people should hold a copy of their medical notes, or at least a summary so they have access to their medical history when needed. This is particularly important if they are away from home or travelling. Towards the end of her talk, Caroline outlined what a successful transfer could achieve. 1) The transfer to a doctor who is knowledgeable about TOF with whom the young person is confident. 2) A transfer of the full medical notes. 3) The young person should understand and be involved. An important part of the transition would involve the young person being made aware of potential long term health problems and having the recommended 5 yearly endoscopy. They would learn about lifestyle behaviours that reduced reflux and the importance of avoiding smoking and excessive alcohol intake as it carries greater risk to TOFs. They should be aware of what their medication is and when and how to take it and, very importantly, who to approach if they have a medical crisis.

There are embryonic plans for a specialist adult TOF clinic. Discussions are underway with the Department of Health. A crucial aspect of moving towards having the clinic is to make clinicians aware of the specific needs that TOF/OA adults have.

Sophie Smallwood

Transition to Adult Care: A young TOF Adult’s perspective: Lauren LeaverA parent’s perspective: Mary ParkesThe issues: Dr Caroline Love

Adult Conference 2012

That’s the maxim which counsellors like our workshop presenter Beverley Slater operate by. Beverley Slater, (MBACP) works in both the NHS and private practice in Worcestershire.

During her workshop sessions, Beverley de-bunked some of the myths about counselling and emphasised that the need for counselling is in no way a sign of weakness – but more a sign of strength. The truth is that the recognition and acceptance that you need help is of itself the first step in deriving benefit from the counselling process.

But - Beverley pointed out – there are some other key principles which determine whether counselling will be successful. The first of these is that the patient must accept the ‘givens’ in their life and about their circumstances and/or condition. Self-evidently, a counsellor cannot alter the fact that, for instance, a TOF is a TOF! But what a counsellor can do is help a patient work through the issues, and will do this is in a manner and an environment which is safe, confidential and non- judgemental. Secondly, the patient must accept that counselling does not offer ‘an instant fix’ but helps them to accept their situation in their life (which could include a health condition, past experiences and so on), help identify what needs to change in how they respond to the challenges they face, and help them to adapt to those changes.

Beverley explained that her practice is based on the principles of ‘Person-Centred Counselling’, using what is known as an ‘eclectic approach’, and that this means that the patient is encouraged to lead the process with interventions from the counsellor that are appropriate for each set of issues. She also explained that some counsellors use another approach known as Cognitive Behavioural Therapy (CBT).

Beverley went to explain that every counselling situation will ultimately go back to ‘loss’; loss of a relationship, or job, self-esteem, confidence, health and so on.

Beverley advised that counselling services can be available through the NHS following referral by a GP. However the extent to which these services are available varies not only area by area, but even from one GP practice to another within the same area.

Alternatively, counsellors can be engaged privately, and she strongly advised that anyone considering this should ensure that any counsellor they consider using is fully qualified and a Member of the British Association for Counselling & Psychotherapy. In this way you will get a counsellor who not only operates within a strict ethical code and framework, but one who will have gained the necessary qualifications and the level of experience required, thereby offering the greatest likelihood of a positive result.

Graham Slater

It’s ok to be ok!

15

Mr Nick Maynard gave a very interesting and informative talk on reflux and its impact on adult TOFs. Mr Maynard has a major interest in

the surgical management of oesophageal motility disorders and gastro oesophageal reflux disease and sees an increasing number of patients with on-going oesophageal problems following TOF/OA repair in childhood.

Strictures, oesophageal dysmotility and reflux are all common in adults born with OA and there is increasing concern for future health and well-being when these symptoms go untreated.

There are several reasons why these symptoms should occur: the original length of the oesophagus, the type of anastomosis (join), the type of oesophageal replacement used (in some), no peristalsis in the area of the join, strictures at the join, weak peristalsis, (which may or may not be caused by vagus nerve damage) and an ineffective sphincter into the stomach allowing reflux to occur, which can worsen strictures and cause further narrowing and scarring. None of these occur in isolation and each will have an effect on the other, depending on the individual.

60% of adult TOFs have a significant degree of dysphagia - difficulty with swallowing food or drink.

Dilatations, even in adulthood usually sort out a stricture – it is very rare these days that a stricture has to be removed but it is possible, using the stomach, a section of colon or small intestine as a replacement.

Any TOF adult experiencing difficulty with swallowing or reflux should be seen by a

specialist. It is very important that symptoms are investigated thoroughly with tests which might include endoscopy, 24 hour pH study, barium swallow, manometry, impedance studies, CT scan, or nuclear medicine emptying studies.

GORD (Gastro Oesophageal Reflux Disease) is a hidden disease which has increased fivefold since 1977 in the general population, the majority of whom will buy over the counter medicines to control it. Reflux can cause a lot of problems and affect quality of life in many ways. Self-treatment can include life style changes such as avoiding triggers for instance spicy foods, not eating late at night, avoiding smoking, limiting alcohol, losing weight, tilting the bed, and using antacids when necessary. A GP may prescribe medications which suppress acid production and those such as prokinetics, which enhance gastric motility and speed up the emptying of the stomach.

Reflux should never be treated lightly and Mr Maynard suggests that adult TOFs should have an endoscopy to check out the health of their oesophagus, as mild symptoms might not mean mild disease.

Typical symptoms of reflux will be heartburn, regurgitation, pain and indigestion.

Atypical symptoms might include chest pain, dysphagia, wheeze, asthma, hoarseness of voice, sore throat, tooth decay, halitosis, and globus (a sensation of having a lump in the throat). The sphincter at the top of the oesophagus can go into spasm.

In TOF adults up to 75% will get some reflux with 50% experiencing it significantly enough to affect their lives. 7% will suffer long term damage to

the oesophageal mucosa as a result. Compared to similar aged people, TOF adults will have experienced reflux over a far longer period of time with corresponding risks to their health.

Complications can affect life style, cause stricture, respiratory problems, bleeding, or changes in the oesophageal cells known as ‘Barrett’s Oesophagus’.

Barrett’s Oesophagus is caused by reflux over a period of time, the incidence of which increases with age. It is a pre-cancerous condition but Mr Maynard stressed that for the vast majority it will not develop into cancer. It is very important however, that anyone diagnosed with Barrett’s should see a specialist. Caught early, it can be treated and any progress halted. Barrett’s cells can now be destroyed but the condition must be managed properly, with regular surveillance endoscopies and by eliminating reflux as far as is possible, through anti-reflux surgery where necessary and anti-reflux drugs. Proton pump inhibitors, a range of drugs which include omeprazole, lansoprazole and esomeprazole are highly effective at inhibiting gastric acid production and Mr Maynard was extremely positive about their use in his patients.

The message for adult TOFs is simple. Do take your reflux seriously; do ask your GP for a referral to a consultant with a special interest in oesophageal disease; do have an endoscopy when advised (and any other tests necessary to assess and define the individual anatomy and physiology). A lot of symptoms that TOFs take as normal for them, can and should, be treated.

Sue Lewis-Jones

Swal lowing and Ref lux Problems in Adults following TOF/OA repair

Nick Maynard, Lead Consultant Surgeon and Clinical Director for Surgery, Oxford University Hospitals Trust.

Adult Conference 2012

S u p p o r t T O F S i n o u r F A N TA S T I C

F LYAW AY R A F F L E a n d W I N

2 F L I G H T S T O N E W Y O R K

Have you bought your tickets yet?This could be your chance to win two return flights to the Big Apple and support TOFS. The lucky ticket holder will receive a voucher for two airline tickets flying with Virgin Atlantic from London to New York on a date of his or her choosing (subject to some date restrictions and payment of Air Passenger Tax). More details and buy your tickets on-line NOW from: www.charitygiving.co.uk/tofsflyawayraffle

You could help us even further by selling just a few draw tickets to your family and friends and neighbours. The tickets cost £1 each and are available in books of five. If you are able to sell some tickets for us please e-mail the office and we will send you the number of books you request: info@tofs.org.uk, or give us a call at 0115 961 3092. The draw will take place on 17th of December 2012 at the TOFs office. Please return any monies, stubbs or unsold tickets by Friday 13 December at 5pm.

TOFSAddressTOFSSt George’s Centre91 Victoria Road,Netherfield,Nottingham NG4 2NN

Telephone0115 961 3092

Fax0115 961 3097

Emailoffice@tofs.org.uk

Websitewww.tofs.org.uk

Registered charity number327735

Company number2202260

Medical patronsRaymond Buick MB Bch FRCSJames Dickson FRCS FRCSE FRCPCHJohn Duffy FRCS, MS, SF(CTh)Bruce Jaffray BMedBiol ChM FRCS(Glas) FRCS (Paed)Leela Kapila OBE FRCSEdward Kiely FRCS FRCS(I) FRCPCHPaul Losty MD FRCS (Paed)Gordon A MacKinlay FRCSEd FRCSSean Marven FRCS (Paed)Agostino Pierro MD FRCS(Eng) FRCS(Ed) FAAPCharles Shaw-Smith BM BCh MRCPPhDLewis Spitz PhD FRCS

Non-Medical PatronsRichard Briers OBE CBEDennis Foxon BADavid Griffiths MA (Oxon)

TOFS PresidentsGren and Christine Shepherd

Council of ManagementHon. Chair: Duncan JacksonHon. Vice Chair: John PearceHon. Treasurer: John PearceHon. Secretary: Graham SlaterDennis HarveySandra HawkinsGill JacksonSue Lewis-JonesSophie Smallwood

Office PersonnelOffice Manager: Diane StephensOffice Administrator: Jayne Allitt

Chew Editorial BoardEditor: Diane StephensSue Lewis-JonesJohn PearceChristine Shepherd

TheviewsexpressedinChewarenotnecessarilythoseoftheeditorortheCouncilofManagement.

TOFS office staffed:Mon and Tues 8.30 - 3pmWed, Thurs and Fri 8.30 - 1pm(Shorter opening hours in school holidays.)

Out of hours emergency telephone support:Please call our answerphone on 0115 961 3092 for the number of the volunteer currently providing this service.

Glossary Adhesions-bandsoffibroustissueAnastomosis -joinBarretts Oesophagus -changesinoesophagealcellsandliningBronchiectasis -dilationofthebronchiDilation -stretchingEndoscopy -inspectionbycameraofthegullet,stomachanduppersmallintestineGastric pull-up-movingthestomachintochestareaGastrostomy -insertionthroughtheskinofafoodpipedirectlyintothestomachJeujnal -partoftheuppersmallbowelLigated -sewnupMyotomy-cuttingthroughamuscleNissens fundoplication -surgicalproceduretoreducerefluxandhiatushernia,whentheupperstomachisusedtowrapcompletelyaroundtheloweroesophagusOesophageal dismotility -poorperistalsisandinco-ordinationintheoesophagusOesophagectomy -removalofallorpartoftheoesophagusPeristalsis -synchronisedcontractionsofthealimentarytracttomovethecontentsoninaco-ordinatedmannerPyloroplasty -operationtowidenthestomachvalvethatformsthejunctionofstomachandduodenum(uppermostsmallbowel)Tracheomalacia - flaccid cartilage in the trachea causing reduced air entry throughtrachealcollapse,particularlywhenincreasedairflowisdemandedsuchasinexercise

Judith Bland

We’re delighted that Kate Clayton has joined the TOFS TLC team in the West Midlands.

“Hi, I'm `Nonna` to Joe Styles, who is now a lovely, lively young man aged 12. I was present with my daughter and son-in-law when he was born at Birmingham Women's Hospital. Following a swift diagnosis, he was transferred the next day to Birmingham Children's Hospital for his TOF/OA surgery. We were lucky to find TOFS at this point after an Internet search.

The support from TOFS has been invaluable through his difficult early years. Our GP, nursery school and primary school have all been on a learning curve and of course all the family. Joe is becoming a good resource from his first-hand experience, it’s so much easier to talk to him now about eating and breathing!

I was a teacher for 25 years, but am now semi-retired from my second career as a relationship therapist. I hope, in this TLC role, that I will be able to offer a listening ear,

signpost to helpful resources and generally enable people to navigate through the complex TOF journey. Having a local contact can, hopefully, lessen the isolation many families feel during those difficult times. Perhaps also it is important to be able to share the joys.”

T O F S T L C s

Just give the office a call to find out if there’s a TOFS TLC in your area. Tel: 0115 961 3092