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tofs chew Spring 2012

Support for families of children born unable to swallow TOFS (Tracheo-Oesophageal Fistula Support), the charity that supports families of children with Tracheo-Oesophageal Fistula and associated conditions

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Page 5 – Mutual support Page 8 – Interview with Miss Avoirt, Consultant Paediatric Surgeon Page 10 – TOFS Adult Conference, Saturday 15 September Page 13 – Long-term problems for adult TOFs

...and much more!

Pictured: Jenson Carter … then and now. Read his amazing story on page 3...

There’s only just time to book for the TOFS London party on Saturday 28 April, or book our

Huddersfield party in the North of England on Saturday 2nd June. Email us now with names of who’d like to come, and an up to date mobile number for the day. We’ll do the rest!


WELCOME 2

Welcome to your Spring 2012 edition of Chew! A warm welcome to our new members! G G

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Lucy Spaull from Middlesex – Freddie born 28 January 2011 Victoria and Andrew Connell from Lancashire – James born 4 June 2011 Anna Szubryt and David Noble from London – Shiloh born 17 October 2011 Abigail and Christopher Ashbey from Middlesex – Hannah born 11 March 2011 Penny and John Sharp from Stourbridge – Jayden born 8 November 2011 Sarah Miles and David Boyle from London – Eva born 5 August 2011 Melonie Bugler-Mitchell from Weston-Super-Mare – Riley born 6 October 2011 Hannah Jenkins and Jamal Choudhury from Malvern – Danniel born 5 March 2011 Gillian Gilkes and Michael Bellamy from Plymouth – Sam born 3 January 2011 Maria and Andrew Tsolakis from Cyprus – Artemios born 3 November 2005 Karoline Mayfield and Daryl Parnell from Mansfield – EbonyGrace born 1 March 2011 Amy and Jamie Welsh from London – John born 23 March 2011 Donal and O’Connor from Donnycarney – Sergio born 5 March 2011 Lindsay Govis from Kingsley, Australia – Andrew born 27 September 1989

Comment from TOFS Chairman,

Duncan Jackson:

‘You don’t have to be an athlete to have Olympic achievements’ Welcome to the Spring Edition of Chew. Before we know it cold dark nights will be over and summer will be here and (love them or hate them) the Olympics will be just around the corner. The Olympics are based upon 7 core values; respect, excellence, friendship, courage, determination, inspiration and equality. These values can be placed at the heart of any organisation, charity or family. G

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We all respect each other for the trials / troubles that we have been through in our lives either personally as a child, young adult, adult, parent or grandparent. We all want excellent care for all TOFS regardless of age or condition Everyone values the support that we receive and friendships that are made at parties and conferences Courage – when times are hard – we all need it We have to have Determination to work through all the problems and try to come out the other side Some TOFS / TOF parents get their inspiration from talking / seeing TOF adults or other older TOF children who are healthy and leading a normal life style

From the editor... Surely we’re not at the end of March? I know many of you will be glad to leave the winter, with all its chest infections, viruses, coughs and colds, behind you. But here at TOFS the year is passing all too quickly and we’ve still so much to do. The good news is there’s still time to create much needed publicity for our TOFS Awareness Week at the end of April. Many of you have already been in touch for publicity materials – best of luck with whatever you’ve got planned. Don’t forget to send us copies of any free publicity and we’ll do a round-up of them in our next edition of CHEW. Planning is well advanced for our next Adult Conference in Reading – Sat 15th September. The venue has been booked for some time and we are well down the track with getting the speakers. Is there anything you would particularly like to see addressed in the programme? Let us know and we’ll try to squeeze it in. At our last conference, despite our best efforts, we were unsuccessful in getting an Adult (TOF knowledgeable) dietician to come along. We’re on the hunt for one already this year. Do you know of someone who could help? Just because we’re trying to address adult-related TOF issues doesn’t mean we’re overlooking our young TOFs. Quite the opposite. We’ve just had a fantastic party in Scotland (more about that in our Summer edition); we’ve one in London at the end of April and we have a Northern England party planned for 2nd June. We’re keen to help support new parents better and we’ll tell you more about a pilot we’re running to do just that in a future edition of CHEW. This is the first Chew that I’ve had the pleasure of editing. We’ve made some changes – there’s more medical content for starters including a report from the literature on longterm oesophageal problems for adult TOFs. Do you want to see more articles like this? Remember it’s your newsletter and it’s important that the content reflects what you want to see, so do please give us some feedback. Don’t forget to keep sending those photos and stories for inclusion too. Email’s best, (info@tofs.org.uk) but feel free to post if it’s easier for you. Your next CHEW will be with you early July. Deadline for copy is end of May. Have a good Easter break.

Diane Stephens, Chew Editor G

Equality – We all want the best health care regardless of who we are or where we live

Therefore why not try to adopt some of these values? As the old saying goes ‘The important thing is not the winning but the taking part’, so why not hold your own Olympic fundraiser - a backwards race in your local park or an egg and spoon through the shopping centre just for TOFS? Have a Tea party, a get together like the Adult TOFS have done on the 31st March. If those sound like too much hard work and you prefer to watch the athletes on the television, why not make yourself feel better by supporting James Moore when he runs the London Marathon in April. Remember if you need support / help with anything then please contact the office and if all else fails check out www.conjunctivitis.com – it’s an eye sore!


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Cover story:

Jenson was born on 13 January 2007 at the Royal Victoria Infirmary, Newcastle. He arrived 7 weeks early weighing only 3lbs 7oz by emergency Caesarean and as if that wasn’t scary enough he was about to overcome some huge challenges. Mum Angela, tells us more below: “Jenson’s story actually started following our routine scan at 20 weeks when the sonographer couldn’t find Jenson’s stomach bubble….three scans later and we received the news no expectant parents want to hear – your baby is poorly. Following several tests Jenson was diagnosed with TOF (which was actually long gap OA) and a heart condition called pulmonary atresia. We spent a few weeks not knowing if our baby would survive the pregnancy as they thought he had a chromosome abnormality called Edward’s syndrome – thankfully for us it was not the case and in a way helped us to have a positive approach to all of Jenson’s problems.

Welcome to your second home Jenson (the RVI) We feel very lucky to have had Jenson’s problems diagnosed during our pregnancy, not only because we had time to prepare ourselves that our baby’s first few weeks would be spent in hospital, but also because he decided to join us 7 weeks early! Had we not been diagnosed prenatally, Jenson most certainly wouldn’t have survived.

His early arrival scuppered the best laid plans – as he was so small, and compromised by his heart defect, the medical team decided they would ‘grow’ him before the planned TOF repair and first stage heart operation. Jenson had a gastrostomy inserted at two days old and we literally lived on the PICU ward for the next 4 and a half months – it became our home and the medical team our adopted family! Jenson had a few blips in those first few weeks, but grew well and at 13 weeks old he had his OA repair. Again luck was on our side as despite all of the concerns we got the best news possible – an ‘easy’ repair! Jenson was so very poorly in the days following his operation, probably the scariest time we have ever had which was followed by the blow which we hadn’t been prepared for – our little baby had tracheomalacia which was so severe his airway would collapse, even while he was being ventilated. This was so hard for us to take in – we were devastated and it felt at the time like we had taken one step forward and been thrown three steps back – a feeling we are used to now!! Decisions were made to bring forward Jenson’s first stage heart repair and at the same time they did an aortopexy; once again lady luck was with us as three weeks later Jenson was back in the RVI, breathing on his own! At almost 5 months old Jenson had his first experience of being carried across the room (the nurses had to stop us running out the ward – ha!), having a ride in his pram in the fresh air – nothing was stopping us then and we were finally discharged from hospital – hurray!

Home sweet home I can’t tell you how exciting and totally scary a time those first few weeks at home were!! Whilst they were joyful we had some scary moments as Jenson’s tracheomalacia caused problems when he was sick and he had ‘blue spells’ on two occasions requiring full resuscitation – not good…the result was we ended up back in the RVI and Jenson had a Nissen’s fundoplication – after that he just didn’t look back!!


4 Jenson’s biggest challenge yet Due to Jenson’s continuing problems with his tracheomalacia a decision was made to bring forward his full heart repair operation, with a hope that this would help. Jenson therefore had his biggest surgery on 14 Feb 2008, and was in theatre for 9 hours. Once again the operation was a total success but history was about to repeat itself as following the surgery we couldn’t get Jenson off the ventilator as his airway was actually worse – a bronchoscopy confirmed our worst fears and we were left with only one option – tracheostomy. I can honestly say that we were absolutely devastated at the time but actually, in hindsight it made such a positive step for Jenson – we didn’t realise how much he had struggled in that first year and the tracheostomy, although I never thought I would say this, was absolutely the best thing for him. Over the years the tracheomalacia has improved significantly, his tracheostomy tube was removed in March 2010 and although we still struggle with regular chest infections, we are able to keep Jenson at home as much as possible through giving IV’s (editor’s note - intravenous

antibiotics, we presume) and physiotherapy at home. A TOF story wouldn’t be right without discussing eating – and for Jenson that has been the slowest area of progress – he lost the suck and swallow reflex and had terrible oral aversion in his first year – this coupled with the tracheostomy has meant he remains to this day 100% gastrostomy fed. That said, as he is growing up, so is his interest in food and we have seen some big steps forward in the last 6 months or so – it’s amazing to think how proud I am when he asks for a snack and has two bites!!

We can’t say the eating issues have not been frustrating, and probably an area I have fits of stress about, but the support of TOFS and the various forums are so helpful - I know he’ll get there in his own space and time, we can’t ask for any more than that.

Look at me now! So, here we are now and Jenson has just started school this year. The last 5 years have been a roller-coaster of highs and lows. We have had more in-patient hospital stays then we care to think of, and Jenson has been in theatre a staggering 32 times (but who’s counting!). We owe so much to all those who have supported Jenson (and us!) during this time. Our motto has always been to keep things as ‘normal’ as possible and allow Jenson to develop in his own time and way – needless to say we are so proud to see our little Jenson, who remains a brave, happy and most gorgeous little boy, getting stronger every day.”

Editor’s note: I first met Angela and Steven Carter in 2010 when they bravely told Jenson’s story at a Nurse’s Study Day in Newcastle-upon-Tyne. Can you raise awareness in your local hospital? Is there a TOFS poster in the neo-natal unit? Have a look and let us know and we can arrange to have one displayed there.


Mutual Support

It’s so good to see how supportive TOF parents are of each other. If you’re a new parent (or not-so-new) and have a problem then do please call the office. Although we’re not a medical support group we will offer emotional support where we can, and we will always try and put you in touch with another TOF parent who may have dealt with a similar issue. Some of you use our TOFS forum for mutual support (www.tofs.org.uk), or the TOFS-enabled Facebook group. Occasionally we feature appeals for advice in CHEW and we are always grateful to those who take time to respond. We’ve summarised some of those responses below. Some of the online contacts suggest switching medications or that one form is better than another. Such suggestions are NOT medicallyapproved advice – merely someone reporting their own experience. TOFS cannot give medical advice, and we strongly recommend that you should talk to your doctor before changing any aspect of looking after yourself / your child. Maria Clifford sought help from parents of teenagers or adults who had to undergo a fundoplication for the SECOND time. She was grateful to hear from Lindsey Hay, mum to Megan (Shaw), who has not only had to get her fundoplication done twice, but a third and now a fourth time. Megan was born with TOF, only 1 functioning kidney, an extra thumb, and a slight abnormality on her spine. She had the operation for repair at one day old and had to have an aortopexy at six weeks to fix the tracheomalacia. The doctors couldn't take her off C-PAP and they had a look and found that she needed a tracheostomy. She subsequently had laser treatment to remove the tracheostomy but still has a laparotomy feeding tube in and will not eat willingly. However she has no physical difficulties apart from the TOF cough and feeding problems (so is still dependent on her tube). She is going to mainstream school. Frazer’s mum, Maggie Earl wrote to us telling us about Frazer’s lack of smell or taste, wondering particularly if it was TOFrelated. We had a number of responses: Lisa Gilbert, mum to 16 year old Olivia told us about meeting an adult TOF who had no sense of smell at the Adult TOF conference. She went on to say “However, my main reason for writing is to say that my husband, Paul, also has no sense of smell or taste and he has nothing to do with TOF at all - and he is not Olivia's father, so there is no genetic connection. He can tell whether something is salty, sweet, bitter or spicy but not what the actual taste is and decides whether or not he likes something largely by texture. He hasn't had any medical contact about his smell/taste since he was a teenager (he's in his late 40s now) when they said nothing could be done and he says he isn't interested now after all these years. I don't have any answer or suggestion as to what can be done, but thought I'd let you know that I had met both another TOF and a non-TOF with the "condition". Linda Howarth, mum to Adam, wrote: … I think it is probably TOF related. Our son Adam has CHARGE syndrome and it is very common in children with CHARGE to have no taste or smell because of the malformation of cranial nerves before 12 weeks gestation. In our Autumn 2010 edition, we featured little Rowan Flambard who was born with TOF/OA and Down’s Syndrome. Rowan’s mum Lauren wanted to make contact with other TOF members whose children were born with Down’s Syndrome. We’re pleased to report that Fiona Taylor, mum to Ryan responded and we’ve since featured little Toni Brown in CHEW. Editor’s note: Are you facing the same issues? If so, please get in touch.

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Here are some extracts from discussions on the TOFSenabled Facebook page…(editor’s note – names have been removed and some comments summarised) Hi everyone, my son was born with OA without fistula in November at 31 weeks gestation and we are still waiting for him to have his repair surgery. We're looking at surgery not being until March/April time. It's really hard having him in hospital for so long, knowing we can't get him home… he doesn't have a mic-key button, he has a gastrostomy and is currently having 60mls every three hours. And some responses: … We've only spent 11 weeks in hospital in total so far but I know how you feel. The days are long and your whole life is put on hold. Make sure you get to see a developmental physiotherapist who can help you find good positions for your son to develop and be stimulated. My son had a great chair that pushed his arms forward and encouraged him to play with his hands. …I know how you feel. Our daughter (long gap OA with fistula) spent 152 days (who's counting) in hospital before she could come home. Since being home we seem to be back every 2 weeks to a month for minor issues. People constantly say to me that she doesn't look sick. It is very hard but once she was home with the family you slowly start to forget it was so long. At 9 months she loves being home with her big brother and is doing all the things a normal baby would be doing despite being on continuous feeds. …they might change to a mic-key button as it’s cleaner and much easier for you to use, still fed via a machine / tube directly into belly. My son now takes 135ml every 3 hours via tube and keeps growing you would never know he’s ill by looking at him. My son is coughing off and on all night long at the moment. It doesn't sound like a chest infection this time. I think it could be reflux, his medication had been working really well. He is on Ranitidine & Domperidone, but is there a chance he could build up a tolerance to them? Last night we got about 2 hours sleep. 1/2 hour after getting him up his cough had eased off… I really do think it's reflux. And some responses: …try asking your Doctor to change the Ranitidine for Omeprazole as Ranitidine is weaker. My son was on Ranitidine and Domperidone and they changed his Ranitidine as it was not strong enough. He still has bad times with reflux but he’s a lot better. Ask for the liquid - they don't like giving the liquid out as it costs more and has to be kept in the fridge but is a lot better for them than the tablet form. …My daughter who is 9 had a night time cough on and off for years. I know how draining it is and believe me I tried everything but nothing worked. The good thing is she rarely has one now except when she has a cold. It definitely got better with age. … when my daughter was young we put builders’ bricks under her bed to raise it up and put a rolled up blanket under her feet to stop her slipping down. We also used a humidifier to moisten the air and this did help. At what age did you allow your TOF to go to nursery / playgroup? My son is 2 next week and generally ok with food but surprises us on occasions with his random choking, although better since been on medication. And some responses: …I sent my daughter from 14 months. Best thing I ever did. But I did stay for a few weeks.


6 …My son was 3yrs old when he started nursery but he had a one-to-one who watched him like a hawk. He is now nearly 6 yrs old, no longer has a one-to-one but the school know all about him & his needs. He always goes equipped with a packed lunch & does have his own "special" seat at the dinner table (next to a teacher). …My son has been at private nursery since he was 6 months old for four full days a week. He is now 3.5 yrs old and loves it. The staff have dealt with all his TOF issues brilliantly, they try and clear stickies before calling us. They blend his food if they think he is struggling, and always keep homemade soup in the freezer for "smooth" days. …My son goes 20 hours a week since he was 20 months. It’s quite a daunting experience letting someone else watch over your little one but he absolutely loves preschool and enjoys being around children his own age. …My daughter was 12 months and it brought her on in leaps and bounds, they (the nursery) also have been really good with her, braver than me sometimes with new foods! Editor’s note: One of the TOFS leaflets “About TOF, OA and VACTERL” can be very useful to give to new playgroups or nurseries or schools as a way of “officially” explaining the TOF condition to the staff there. My son is having a stomach uplift in GOSH in Feb after his long gap OA was unsuccessful. He was in neonatal intensive care in for 110 days from birth where they tried to fix his long gap, but unfortunately it didn’t work so he’s now got a gastrostomy which we feed via a pump every 3 hours and a right oesphagostomy and copes fine with it all. I have read that after his stomach uplift he will then have a J tube and fed 24 hours continuously. And some responses: … my daughter had a gastric transposition done about 7 years ago at GOSH by Prof Spitz. She was 7 when she had it done and hadn’t eaten up until that point. She now eats anything and everything and we have never looked back. She never had any problems with dilatations etc after and has been very healthy since, although quite small for her age. She is today celebrating her 15th birthday which is something at times we would never have thought we would have seen. She had a J tube in for a few months after the operation but as she began to eat quite soon after coming home, allbeit soft food to start with, this was only there to top up her feed if we felt she hadn’t eaten enough during the day. Then after a couple of months she was managing to thrive on the food she was taking in. Her tube was removed once we hadn’t needed to use it for about 6 weeks from what I can remember. She can eat anything now, the only thing she is not allowed is fizzy drinks as they would bloat her stomach which is now situated behind her lungs, so not a good idea! She is a bit of a grazer rather than 3 large meals a day but we don’t mind as it is just lovely to have her eating. We still keep her bed slightly tilted and try not to let her eat before going to bed to avoid anything coming back up, and fingers crossed to this day she has never had a problem with that and is not on any medication at all and hasn’t been since the operation. I have 7 month old twins, born 12 weeks early. My son has TOF/OA and is due his third dilatation, due to the OA repair done at the end of September shrunk to less than 1mm (Ed- we assume this means the oesophagus internal diameter), meaning no oral feeding, with a mini button which is leaking constantly. …Is his skin sore from it leaking? If so we use Maxitrol eye cream......yes strange,I know, but its the only thing that works and keeps it clean and sore free. The neonatal unit used it, everything else has been rubbish.

Our son was in intensive care for 110 days from birth so he is behind in some things compared to his twin.....such as he won’t roll, he doesn’t shout a lot, he hates the bottle but will sham feed ok etc but he is advanced in grabbing and holding things, hand to mouth action and has a much stronger head. I think all of these things are from hospital as he had a play specialist and had to know how to keep his dummy in his mouth. He also just sits and watches a lot, takes everything in, only smiles if you smile at him where my other one is constantly laughing and smiling. I’m not so bothered as its expected from being in hospital for so long. He will come on once his operations are over, I’m sure. I am looking for some advice from you all on 'stucks'...today I was feeding my son (8 months old) some small pieces of grape. At one point when he was sucking on the grape a chunk came off. Not long after he had saliva drooling out of his mouth and his nostrils. It was a bit scary as the little lad was clearly in distress. Thankfully my hubby was on hand to give him some first aid for choking... And some responses… …My son is now 3. He salivates when he has sticky. Thick stringy saliva. Blows bubbles in it and all sorts and it is not nice to see. The body produces the saliva to break the food down and if they have a total blockage they can't swallow the saliva. I wouldn't worry unless it happens all the time. I found that a stricture in our case caused him to put back small amounts of food as he couldn't get them down fast enough. He didn't salivate the same with the stricture as it was not a total blockage and the liquid could still get through albeit a bit slower. …A sign for us was a rasping sound and our son drawing his head back. Saliva is definitely an indication too as the obstruction, whatever it may be is stopping our normal "spit" going down and so it pools and goes back up as it builds. This in itself can be useful as it can cause a gag reaction that can help an obstruction to come up. …Yoghurt was useful for us. If that did not work we would use full fat coca cola. Or pineapple juice as it helps to break down food that’s stuck. It takes time to start to work though, so slaps on the back time. If still no joy that was the time for us to ring the hospital. All this is on his care plan for school too. With the proviso that if ever he has trouble breathing or is in distress they ring for an ambulance. …My son gets raspy and if the stuck food totally blocks the oesophagus so that even liquid can't pass then we get stringy saliva when he vomits it up. When he was a smaller baby, he coped less well and it can come out of nose and mouth however you have to be careful as when it comes out of the nose there is more chance it could get to the airways. It's amazing how much saliva can pool in the pouch above the join before they bring up the offending food. I would be careful with things that won't easily fall apart at 8 months, things like fruit and vegetables as when they get stuck the sticky tends to be worse than with biscuit and such like. …My son’s culprits are parsnip, bread and mango. but when he is due a dilatation many types of food becomes stuck, not just one. My surgeon suggested keeping a food diary and just noting what gets stuck and what doesn't. As the number of things gettings stuck increases, a dilatation is needed.


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EAT: What’s in it for TOFS?

EAT (The Federation of Esophageal Atresia and Tracheo-esophageal fistula support groups) is the recently founded international federation of OA/TOF support groups. In this article TOFS Trustee Graham Slater, the first Chairman of this new organisation, talks about what EAT hopes to achieve. TOFS has had valuable informal contact with other support groups for a number of years; indeed one of the founder members of KEKS, the German support group, was for a time a trustee of TOFS. Our conferences have frequently been attended by representatives of other groups, and a degree of information exchange was achieved. A number of support groups (including TOFS) were invited to the first-ever International Workshop on OA which was held in Lille, France in 2010, as an international group to present to an audience of eminent paediatricians from across the world, many of whom were unaware of the work of groups like TOFS. It became apparent at that time that we needed a more formal platform to build our relationships upon, both with one another and with the medical profession. And so the concept of EAT was born. One of EAT’s primary goals is to raise the profile of the OA/TOF condition at an international level. It’s early days yet but in particular EAT hopes to do this through the European Commission’s Health Programme, by gaining the sponsorship of some major European/global corporations, and by participating in international medical forums. These are things which TOFS or any one of the other member organisations would find difficult to do alone.

Secondly, whilst EAT won’t replicate, take over or supervise the work of national self-help groups, it is there to ensure an effective exchange of information, publications, ideas and initiatives so that each member organisation of EAT has the opportunity to benefit from what is being done in other countries and what has been learnt by all the other member organisations. A lot of the most interesting medical work relating to the TOF condition appears to be going on abroad, so participating in EAT should be really useful to TOFS in this regard. EAT also intends to develop its own relationships with other institutions such as universities and medical schools to initiate research and studies which would benefit from being conducted over a wider population than just one country. One particular area EAT wants to look at is the whole area of adult ‘TOFs’, their ongoing health, social and employment issues and the question of transition to adult care. This is a vexed question which each of the founder members of EAT has expressed – about the problematic transition from ongoing child care managed ‘horizontally’ by paediatricians to the world of adult healthcare with its ‘vertical stovepipes’ of specialist care whereby adult ‘TOFS’ find themselves dealing with not one specialist but different specialists in gastro-enterology, cardio-thoracics etc. Another of EAT’s objectives is to encourage parents in European countries without a national support/self-help group to establish one. EAT is also already active in this regard, in particular in Italy where we are cautiously optimistic that a support group will be formed in 2012. It is actually in all our interests for such groups to be formed especially in large countries such as Italy as this adds to the pan-European credibility of EAT and will benefit the approaches we make to the EU and to other potential corporate sponsors. At a practical level EAT also plans to introduce an ‘emergency card’ which each ‘TOF’ can have and which summarises their specific conditions and problems. Together with a dictionary of common OA/TOF related terminology, in languages such as German, French and Dutch, Italian and Spanish, this should provide parents (and adult ‘TOFs’ themselves) with a useful aid should they find themselves in the unfortunate position of requiring medical support whilst travelling abroad. EAT is still a relatively new organisation and its real success will probably be better measured over several years rather than over the next 12 months. Nonetheless the Board of EAT is determined to have some ‘quick wins’ (such as the emergency card) as well as putting its efforts into longer-term objectives. Whatever it does, it will provide TOFS with opportunities both to influence and to get benefits which would be either impossible or very difficult to achieve alone. In the words of one of our European colleagues, ‘together we are stronger’ and that is why this new organisation has the potential to bring additional benefits to TOFS and to all ‘TOFs’ and their families.


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TOFS interview… Ms Avoirt Consultant Paediatric Surgeon

Tell me about your experience as a TOF surgeon I started off in Belgium as a general surgeon but I always had a particular interest in neo-natal and paediatric surgery. I travelled to get experience and develop my skills in this area, firstly to Holland, then to South Africa and I came to the UK initially for a post of Senior Registrar with Southampton General Hospital. I was subsequently appointed as consultant in The Royal Alexandra Children’s Hospital in Brighton and I’ve been here ever since. At one point, there were only two paediatric surgeons here in Brighton and I did most of the TOF/OA repairs, so was probably operating on around about 6 a year. Now we see about 8 or 9 children born with the condition, but we have a larger team and last year I repaired 4 babies. I’m a surgeon and I like operating on TOFS – it’s become one of my favourite operations.

Have many of these parents had the diagnosis antenatally? It’s hard to say. The absence of baby’s stomach combined with evident polyhydramnios (excess fluid in the womb) may point to a likely TOF/OA but often this is just a suspicion. If the child has pure oesophageal atresia it

may be more likely to be diagnosed antenatally but again, often it can’t be confirmed until baby’s born. Once TOF/OA is diagnosed after birth we routinely make parents aware of the potential associated abnormalities (VACTERL - see glossary) and check baby thoroughly for these.

Is there something the parents could have done to prevent TOF? Absolutely not. We are still unsure exactly what causes TOF/OA but it is certainly not the fault of the parents. I think of it as an accident of nature.

If a baby has a 'long-gap' - why does he / she have to wait for a repair? We prefer to do a primary anastomosis (join) but in some cases we are unable to do this and have to wait for the child to grow in the hope that the distal pouch becomes longer and the gap shorter. It is always better to have your own oesophagus than an alternative. We hope to do the delayed primary anastomosis within 3 months although if the gap hasn’t decreased enough during that stage we may have to consider an oesophageal substitution (an operation in which another part of the


baby’s intestines is used to bridge the gap between the ends of the oesophagus). There are various options for this type of surgery, depending on where you live in the UK; personally I favour the gastric interposition as I believe it is safer to put the stomach with the oesophagus, but of course it’s a major operation so still not without risk. These patients will need careful monitoring throughout their lives.

What advice would you give a new parent leaving hospital with their newly repaired TOF/OA baby? We give lots of advice. We tell them honestly that their children may have complications after the initial repair. We make parents aware children can develop strictures at the site of the anastomosis. Reflux is a common complication because of the distortion of anatomy. I routinely prescribe children an anti-acid until one year of age because it protects the site of the join. If they subsequently display a lot of reflux symptoms that aren’t responding to medication we may have to look at doing a fundoplication (antireflux operation).

At what point should parents make contact with their hospital if they suspect there is a problem? If a parent feels their child has aspirated or swallowed something which has stuck they should contact the hospital as soon as possible. Generally we have open access for those children who have had TOF/OA.

What tests do you do to establish if a child needs a dilatation? If the child has had to have regular dilatations we are guided by the advice of parents as they usually know. If the child has reached say a year old with no strictures and the parents now think he has one, we’ll assess things by initially doing a contrast study. If the stricture is caused by reflux we do further investigation to assess how bad the reflux is.

Are there any foods which parents of TOF children should avoid? I tell parents about the importance of chewing well as their child gets older and moves on to solids, and that soft and sloppy food is good, and the importance of drinking a lot during meals to make food softer to swallow. Some children cope better than others when things get stuck. Sausages seem to be common culprits or dry foods like bread.

Happy Birthday

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

March Jordanne France 07/03/1996 March Hollingbery 08/03/1997 Pippa Hynes 08/03/1997 Jack Mullens 12/03/1997 Amy Howes 23/03/1997 Georgia Jenkin 23/03/1998 Megan Knox 07/03/1999 David Malcolm 11/03/1999 Daniel Sandford 28/03/2000 Lauren Hulligan 07/03/2001 Olympios Gougoulias 06/03/2002 Mia Bradbury 13/03/2002 Grace Walsh 06/03/2003 Lukas Weber 09/03/2003 Lauren Naylor 21/03/2003 James Rogers 26/03/2003 Ben Smith 03/03/2004 Lewie Herbert 05/03/2004 Ryan Lowe 09/03/200 James Flynn 24/03/2004 Grace Nolan 06/03/2005 Justin M Albrechtsen 24/03/2005 Isabelle Maude 31/03/2005 Arwin Bool 22/03/2006 Tobias Jones 25/03/2006

April Jack Berry 09/04/1996 Megan Evans 18/04/1996 Thomas Smith 19/04/1996 Daniel Heath 19/04/1996 Charles Shotton 25/04/1997 Lucy Robson 26/04/1997 Phoebe Smith 29/04/1997 Robert Doward 17/04/1998 Laura Thompson 18/04/1998 James Soper 25/04/1998 Andrew Day 12/04/1999 David Fenton-Smith 26/04/1999 Bradley Newman 11/04/2000 Samuel Mullins 18/04/2000 Miriam Holland 03/04/2001 Kimberley Taylor 25/04/2001 Samuel Holleron 30/04/2001 Phoebe Cannetti 10/04/2002 Zijian Guo 10/04/2003 Max Swarbrick 24/04/2003 Lily Rolfe 08/04/2004 Cormac Carolan 11/04/2004 Gemma Hewitt 01/04/2005 Edie Witton 05/04/2005 Ellis Elson 12/04/2005 Peter Riis 13/04/2005 Ewan Brocklesby 19/04/2005 Robbie Edwards 20/04/2005 Thomas Flowers 23/04/2005 Emma Morrison 26/04/2005 Riley Clifford 09/04/2006 Gabriella Fox 12/04/2006 Victoria Romero.F 21/04/2006 Savannah Taylor 23/04/2006 Erin Davis 29/04/2006

May Connor Middlehurst 15/05/1996 Matthew Flynn 19/05/1996 Jordan Clifford 24/05/1996 Eilidh Latimer 15/05/1997 Stephen-Thomas Gray 17/05/1997 Victoria Mince 01/05/1998 Anna Rose Greco 05/05/1998 Charlie White 10/05/1998 Charlie Gough 11/05/1999 Nicole Morley 31/05/1999 Matilda Green 10/05/2000 Isobel Shorrock 15/05/2002 Jac Griffiths 03/05/2003 Robert Corbett 19/05/2003 Leonardo Capodacqua 06/05/2004 Thomas Smith 07/05/2004 Katy Rainforth 11/05/2004 Luke Burgess 13/05/2004 Cyrus Dezfulli 19/05/2004 Adam Gowler 05/05/2005 Connor Hardy 16/05/2005 Priya Pandey 17/05/2005 Henry Keating 17/05/2005 Joe Turner 30/05/2005 Kathryn Lloyd 02/05/2006 Abbie Stubbs 12/05/2006 Eve Stevenson 12/05/2006 Tahish Shrivastava 18/05/2006 Thomas Fryer 21/05/2006 Jack Johnson 22/05/2006 Shay Murphy 24/05/2006


Teen party!

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By TOFS trustee, Gill Jackson Would you and your teen TOF (aged between 10 and 20) like a get together with others? Have you been to one of our TOF parties in the past when your TOF child was a baby or toddler? Did you like the contact with other parents? Value the opportunity for your TOF to meet others? Enjoy the benefits of being part of the TOFs organisation? BUT, is your child too old now to really enjoy the children’s parties? I am mum to TOF Laura; you may have met her if you’ve been to one of the parties or conferences. She loves getting together with other TOFs – it gives her a sense of identity and helps her to understand how TOF/OA has been a part of her life-story. She also loves being active, being challenged and making new friends. I love meeting new people too. Comparing our stories – the ups and the downs! Even though our problems are far less now than they were in the first 5 years – I would like to meet other parents of older TOFs; ones who are approaching transition to adult hospital care. So, I wondered if you might be interested in attending a get-together? I’m open to suggestions as to where to go or what to do. Some ideas so far have been activity ropes courses, go-carting, or swimming. An area of the country suggested was The Cotswolds – but I would really like to hear your thoughts. The time of this event is also open to suggestion. If you think you would be interested, have ideas as to when, where to go or what to do please let us know by ringing or emailing the TOFS office, or just send me or Diane a message on Facebook. Hope to hear from you.

Gill x

The TOFS Adult conference this September is part of our initiative in supporting those Adult TOFs who continue to suffer from respiratory and / or gastroenterological problems. We chose Reading for its good transport links and the hotel is reasonably-priced should you want to make a weekend of it. (Please book your accommodation directly with the hotel.)

Would YOU like to take part? Are you an adult TOF who has recently made the transition from paediatric to adult care? If so, please get in touch with the office. You may be able to make a valuable contribution

Raise a tenner for TOFS during TOFS Awareness Week 22 - 29 April Are you ready for the challenge? Awareness Week takes place every second year to highlight TOF/OA and the plight and achievements of our youngsters all over the UK. You may remember our last official Awareness week in 2010: we managed to get TOFS and TOF people mentioned in many newspaper and magazine articles. What’s more, amazingly you raised over £10,000 – every penny of which is vital as we get no government funding. This year, as well as raising awareness, we’re challenging all our members to ‘Raise a Tenner for TOFS’. Can you do it? Can we beat 2010? Here are a few ideas how to raise that tenner and maybe much more:

Have a coffee morning for TOFS – we can supply invitations, collecting box for people to pop in cash or a cheque – in fact a full fundraising pack. Non-uniform days, supermarket bag pack, sponsored swim, walk, run, silence haircut or shave (we’ve even had a sponsored scoff!), raffles, pub quizzes, Collections (just ask us for a collection box), cake sales, car boot sales, car washing,… Visit our website for fundraising tips or call the office for a leaflet. Whatever you are planning keep it SAFE and LEGAL (more information available from our office) to help protect yourself and others at your event. And remember, every penny counts. Don’t forget to give the office a call to tell them what you are doing and for more help and advice but most importantly – have fun! If you are having an event, please try to get TOFS and your TOF mentioned in your local press – all awareness is helpful. Let the office know what’s going on and we can send you some outline press releases?

Editor’s note – Don’t worry if the date for your event doesn’t fall within with Awareness Week – just have it at a time to suit you and we can feature it in another edition of CHEW. We’re aware that many of you raise awareness and funds for TOFS all year around and we are very grateful, especially in the current economic climate.

TOFS Adult Conference Saturday 15 September 2012 (full day) Holiday Inn, Reading South, Basingstoke Road Reading RG2 0SL Excellent transport links (only half mile from M4 Junction 11 with A33), on-site free parking, only 3 miles from Reading train station (very good train services from many parts of the UK) Are you an adult with TOF/OA, a Relative, Health Care Professional, or will your TOF teenager soon be making the transition to Adult Care? If so, this conference is for YOU.

BOOK IT TODAY – spaces are limited – call us NOW on 0115 9613092 or email: info@tofs.org.uk


Fundraising

TOFS and the Virgin London Marathon We had a difficult choice in selecting the successful entrant to run the Virgin London Marathon for TOFS. It will probably be another five years before we are awarded another official TOFS place so it was an important decision, and a really tricky one as all of the four candidates seemed very committed and worthy of a place, and it was regrettable we could only choose one. We are pleased to announce that the coveted place has gone to James (Jezz) Moore, uncle to little TOF/OA toddler Joseph Wood.

11

BIG thanks to Cath Cobden from Worthing who held a raffle for TOFS at a Zumba event and raised £200. And she’s not finished fundraising – she’s got plans for 2012 - here’s her most recent fundraising appeal: “Hello to all TOFS subscribers! I am a TOF mummy to Oliver now 2 and a half!! I have recently been asked if I would like a stall at our local fire station open day in July to raise funds for TOFS! I immediately said yes and went along to a meeting to discuss the dos and don’ts! They (like many others) had never heard of the charity or the condition and were stunned to see my pictures of Oliver as a baby and

James is 23 and lives in Redditch, just outside Birmingham in the West Midlands. He works as an account manager for City Electrical Factors - an electrical suppliers. His nephew Joseph Dylan Wood (parents Lis & Gary Wood) was born with TOF/OA in 2009. He also has a friend, now 23, who was born with TOF. James told me: “I’m a very sporty person. I love all types of sport, especially football, golf and cycling. It is a dream come true to have the opportunity to run the London Marathon 2012. Thank you for all of your support and I hope I can do everyone at TOFS, and everyone involved proud.” James has already been training and some of you have sponsored him already. You can continue to sponsor him at www.charitygiving.co.uk/jamesmoore We mustn’t forget those of you who have been successful in getting a Marathon place in the ballot and intend to run it for TOFS. We’ve heard a whisper that Nicklos Tyrer, from Dorset (TOF/OA daughter Maddison is now 11) is also running for us – best of luck to you both with your training…. Are you running a Marathon (or a half marathon) for TOFS next year? Let us know NOW if you are. Email the office: info@tofs.org.uk. We can send you a TOFS sponsorship form and a TOFS T-shirt, if you’d like one. Indeed if you are thinking about doing any other sponsored event for TOFSlike one of those long bike rides, perhaps, please let us know.

Our thanks to Elaine Willis from Chippenham, mum to TOF son Tyler who is now 13. Elaine ran the Paris Marathon last April in a staggering 26ºc raising £307 in the process. Elaine completed her run in 4 hours and 37 minutes. She tells us that her employer’s son who is now 5, was also born with TOF.

Cath’s son Oliver is pictured above comparing them to the real life toddler being a mischief around the office! So....they decided there and then that not only could I have my stall but the Fire Station would take on TOFS as their charity this year! So with this in mind I’m asking for help... The Fire Station would like to hold a raffle with 6 "BIG" prizes like perhaps a short break or helicopter ride? I will be holding a tombola and maybe a small silent auction on the day. So if anyone knows of any companies or individuals who would like to help out by donating to the raffle or tombola then please please contact Diane at the TOFS office. Vouchers etc can be sent to her for the attention of Cath Cobden. Also on the day I hope to be able to give out around 500 goody bags with TOFs information inside as well as pens, sweets etc etc so if anyone would like to advertise in these or give something towards the cost of raffle request letters or stamps etc then please again contact Diane.

Thank you so much in advance, TOF mummy, Cath Cobden


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Thank you to: 

      

David Paton grandparent to Lewis Carney donated £25.00 after giving a talk on the history of his collection of toast racks. Wolverton & Stony Stratford Rotary donated £190.00 raised at a golf day charity event. Kids Planet Day Nurseries donated £105.90 raised through fun days at nursery. Notts Fire & Rescue Retired Members Associated £125.00 raised at an AGM fundraiser.

obstruction of the pulmonary orifice .

Edward’s Syndrome: a very serious abnormality that leads to severe mental and growth retardation, abnormalities in the heart and other organs and malformed features.

Joy Howell (Colin Ryan’s grandmother) - £20.00 donation in lieu of sending Christmas cards

severe tracheomalacia, in which the aorta (a major artery which lies to the front of the trachea) is anchored to the sternum (breast bone). This has the effect of opening up the trachea.

Tiger Developments - £250.00 in lieu of sending Christmas cards.

Nissen Fundoplication: this procedure is commonly used

Felicity Anderton, grandmother to Joel Anderton, donated £100 in lieu of sending Christmas cards

Terry Collins and Graham Parkin - £50.00 donation



Pulmonary Atresia: congenital severe narrowing or

Aortopexy: surgical procedure used in the management of





Long-gap OA: when the gap between the two ends of the oesophagus is too large to bridge immediately.

Tracheomalacia: ‘floppiness’ of the trachea.

Leela Kapila - £150 fee for lecture given for Medlink



Definitions of technical and medical terms mentioned in some of our articles and family updates:

Chris & John Layne (Ben Bailey’s grandparents) – donation £100.00





Glossary

Julie Molyneux - £60.00 collection from Webb Ivory Sales Staff at The Smallberry Green Primary School raised £100 instead of sending Christmas cards to one another.

to reduce Gastro-Oesophageal Reflux (GOR). The lower oesophagus is freed up and the top part of the stomach (known as the ‘fundus’) is wrapped around it

Tracheostomy: the surgical creation of a connection between the trachea and the skin at the neck, and the insertion of a tube into the trachea to preserve a clear airway. Used in the management of severe tracheomalacia.

C-PAP: Continual Positive Airway Pressure – the application of a continuous positive pressure to the airways, used (for example) in the management of tracheomalacia to keep the airways open

Laparotomy: Operation involving the opening of the Margaret Lemon, mother of Colette Stones born with TOF donated £50.00

abdominal cavity, usually as an investigative procedure.

Mic-key button: enteral feeding tube Kirsty and John Robb - £50.00 donation

In Memory:

Oesophagostomy: surgical creation of a connection between the oesophagus and the body wall (skin) usually so that a feeding tube can be inserted directly to the oesophagus. Gastric transposition: method of oesophageal replacement (also known as stomach pull-up or uplift)

£160.00 donated in memory of the late Mr Roy Marten, grandfather to Jack Morten, including - £5 donated from Angela & Brian, £25 donated from Mrs K Morten, £10 donated from Doris Procter. Collection made at the funeral of the late Mrs Joyce Mary Sharpe – £394.79

Dilatation: A procedure undertaken to widen a narrowing of the oesophagus (stricture).

Barrett’s Oesophagus: condition in which the cells of the lower oesophagus become damaged, usually from repeated exposure to stomach acid. The damage causes changes to the colour and composition of the oesophageal cells.

Reflux: also known as Gastro-Oesophageal Reflux, the

200 Club Winners

involuntary return of gastric (stomach) contents to the lower end of the oesophagus

Fundoplication: surgical procedure to treat reflux, see Nissen’s above

November – Sarah Thomas December – Mary Hartnett January – Ernest Towner

Dysplasia: Abnormal development of cells. Endoscopy: procedure where the oesophagus, stomach and duodenum is examined internally using an endoscope (a tiny swallowable camera on a long thin flexible mount)


13

Long-term oesophageal problems for adult TOFs

Continued

doing is about following up outcomes. It is not all about medical or surgical techniques, as a layman might have thought. Secondly, most of the published follow-up work is being done outside the UK: it is just as well that TOFS has connected itself up via EAT to what is going on abroad.

Oesophageal Cancer: a concern, but few TOFs yet known to be sufferers

by TOFS Vice-chair John Pearce

Why write this review article? I remember sitting in a TOFS conference several years ago, and being startled by something said by one of the paediatric surgeons who was presenting. This was that TOF people are at higher-than-normal risk of a condition called Barrett’s Oesophagus and eventually even oesophageal cancer. For anyone related to a TOF, this is hardly the happiest of thoughts, especially since this form of cancer is a relatively unpleasant one. I made a mental note to find out a bit more about this one day. More recently, I took part in the 2011 TOFS conference: it was obvious in the adult TOF session that some clear commentary is wanted on the extent to which adult TOFs need ongoing care of the oesophagus. This can only become more of an issue as the numbers of adult TOFs and their average age goes up: so far there are very few adult TOFs older than 55. What I have found is that groups of doctors (almost all outside Britain) have been publishing their findings, having followed up groups of adult TOFs, to check up on the severity and extent of long-term outcomes. I have reviewed a lot of what the doctors have published relating to long-term oesophageal problems. Positively, there are as yet very few reports of TOFs suffering from oesophageal cancer. However the doctor’s studies make it clear that Barrett’s Oesophagus (which is treatable) is far more common amongst TOFs than amongst the population as a whole

What the Doctors are publishing The subject of an unusual complication associated with the rare TOF/OA condition does not come up all that frequently in the medical journals. But it certainly was mentioned at the late 2010 First International Workshop on Esophageal Atresia. [Note – spelt the American way] The papers from this conference were published in mid 2011, and I’ve been reading… Incidentally, TOFS attended this workshop. One of the papers was given by our own Graham Slater, who is Chairman of EAT, the pan-European co-operation body for TOF support groups – which TOFS has set up with KEKS, VOKS and AFAO over the past 3 years or so. Two things struck me when I looked in some detail at what the doctors are publishing. Firstly, quite a lot of the work they are

The question of potential oesophageal cancer is principally a concern for Adult TOFs rather than for children: it takes some time for cancers to develop. One 2011 paper (1) said “ To date, Worldwide, six cases of esophageal cancer have been reported in young adults treated for EA”. Six is not a huge number at all. Moreover, very few adult TOFs appear to have developed oesophageal cancer so far. One study (2) checked up on some 270 known adult TOFs in Finland, and found 3 cases of cancer, none of which were oesophageal cancer. The authors of this Finnish study (3) concluded that “the overall cancer risk of adults with repaired oesophageal atresia is similar to that of the general population”, though they did not estimate how high is the oesophageal cancer risk for adult TOFs. A similarly large study of adult TOFs in Australia (4) saw 130 adult TOFs assessed, and only one was found to have a form of oesophageal cancer. So, encouragingly, it seems that very small numbers of TOFs have so far developed oesophageal cancers – we are not yet aware of even a single one reported in the UK. According to the NHS, oesophageal cancer is the seventh most common type of cancer in the UK, with an estimated 7,000 new cases diagnosed here each year (5). [For comparison, Breast cancer is the most common cancer, with 46,000 new cases annually] Men are more likely to suffer from oesophageal cancer than women, and the risk of contracting this cancer is significantly increased by smoking, and drinking alcohol,(5). Obesity and drinking very hot drinks are also thought to increase the risk of this cancer. The average age at diagnosis is 72: since there are as yet few adult TOFs over the age of 55, no-one can yet really say how much higher (than normal) is the risk of oesophageal cancer for TOFs.

Barrett’s Oesophagus could be a big deal for older TOFs For TOFs, oesophageal cancer may not yet obviously be a major issue, but a precursor condition, Barrett’s Oesophagus, might well be a major issue for adult TOFs. You hardly need us to tell you that reflux is one of the most common problems experienced by TOF people. Significant numbers of TOFs have fundoplication surgery to tackle ongoing excessive levels of reflux. Ongoing significant reflux means that the lower parts of the oesophagus are exposed to stomach acid and bile, which should not normally happen much at all, and which irritate the lining of the oesophagus, leading to inflammation, sometimes referred to as reflux oesophagitis or GORD: Gastro-Oesophageal Reflux Disease (5,6). Americans call this GERD for spelling reasons, by the way.


14

Over several or many years of GORD, the cells lining the lower oesophagus can start to change to become more like those that line the intestine. There is some debate amongst experts as to exactly which types of cells mean that a patient has Barrett’s (7). In order to diagnose Barrett’s, doctors would typically do an endoscopy (have a look with a camera scope which the patient swallows through the mouth) and take biopsies (tiny samples) of the cells, looking for evidence of something called intestinal metaplasia. The UK Barrett’s Oesophagus Campaign (8) estimates that 375,000 people (or about half of one percent of the population) in the UK have Barrett’s. Some Barrett’s sufferers will go on to develop further cell abnormalities (dysplasia), which can lead in fortunately few cases to cancer: up to 10% of those with Barrett’s according to the Campaign and also to some other experts (9). The Campaign seeks to draw attention to Barrett’s Oesophagus – and to encourage people to take it seriously. The main message here is that frequent or persistent reflux (often observed as “heartburn”) should not be ignored. The medical literature also gives some insight into the extent of Barrett’s Oesophagus among TOFs. The Finnish study (3), found that 34% of their adult TOF patients had symptoms of reflux (vs 8% in a normal-population group). Barrett’s was confirmed in 6% of these TOFs. The Australian study (4) reported that 63% of their adult TOFs had reflux symptoms. Barrett’s Oesophagus was found in 11% of those TOFs whose symptoms were thought significant enough to warrant investigation (or 5.3% of the whole study group). A small Canadian study (10) found reflux symptoms in 61% of adult TOF respondents: as many as 18% of those who were examined in detail (or 12% of the study group) were found to have Barrett’s. Two small studies of adult TOFs in the Netherlands found Barrett’s in 2.6% (11) and 5.8% (12) of their study groups. These percentages vary quite a lot, but on the strength of the various papers, I think it is reasonable to expect that something in the region of 3% to 10% of current-day adult TOFs might be suffering from Barrett’s. If so, then Barrett’s is far (perhaps 1020 times ) more common amongst adult TOFs than amongst the rest of the population. And bear in mind that the average

age for diagnosing Barrett’s in the population as a whole is 62 (6). The oldest TOF in the UK is not yet 70 and there are few TOFs over 55.

Standard guidance on Endoscopies seems inadequate for TOFs So what’s the point of this? If you have ongoing reflux and it’s not getting any better, you certainly should think about asking your doctor to consider sending you off for an endoscopy. The older you are, the more important this could be. Standard UK guidance from NICE to Doctors says that endoscopies are not normally worth doing in people under age 55 (13) . One assumes that this is because there is a cost to the NHS, a small risk to the patient, yet a very small chance of finding any problems in the normal population below age 55. But in this regard TOFs are not the normal population – the papers I have reviewed suggest that the risk of Barrett’s is much higher in TOFs than the normal population. Moreover, the strong consensus among the experts whose papers I have reviewed is that TOF patients should have endoscopies, whereas the normal population might not warrant such examination. However there is debate about under what circumstances or how frequently endoscopic examinations should be done. The Finnish (3) investigators, for instance,(3) say that studies are needed to “define guidelines for long-term endoscopic surveillance of adult TOFs”. One paper suggests screening all EA patients at the age of 10, and that such “symptomatic patients should undergo investigation regardless of age”.(7) One group of researchers (11) are less than convinced: “it is still very disputable if all EA patients should be screened at an adult age”. But another paper recommends “clinical assessment as adults and upper endoscopy for reflux symptoms or dysphagia” (4), whilst other experts (10) say “Special monitoring including endoscopic screening may be beneficial” to adult TOFs. Another (14) suggests “systematic screening of endoscopic lesions in all patients with EA, even in the absence of upper GI symptoms” with regular endoscopies every 5 years.

References 1)

2)

3)

4)

5)

6)

7)

Long-term results of esophageal atresia: Helsinki experience and review of literature. Sistonen SJ, Pakarinen MP, Rintala RJ. Pediatric Surgery International 2011 Nov Cancer after repair of esophageal atresia: population-based long-term follow-up. Sistonen SJ, Koivusalo A, Lindahl H, Pukkala E, Rintala RJ, .Pakarinen MP, Journal of Pediatric Surgery 2008 Apr Outcome of Oesophageal Atresia beyond Childhood. Rintala RJ. Sistonen SJ, Pakarinen MP, Journal of Pediatric Gastroenterology and Nutrition 2011 May Supplement 1. Gastroesophageal reflux and related pathology in adults who were born with esophageal atresia: a long term follow-up study. Taylor AC, Breen KJ, Auldist A, Catto-Smith A, Clamette T, et al Clinical Gastroenterology & Hepatology 2007 June Oesophageal cancer From website: NHS: Your health, your choices 2010 April http://www.nhs.uk/conditions/Cancer-of-the-oesophagus/Pages/Introduction.aspx Heartburn and gastro-oesophageal reflux disease - Complications From website NHS: Your health, your choices 2010 June http://www.nhs.uk/Conditions/Gastroesophageal-reflux-disease/Pages/ Complications.aspx Esophagitis and Barrett Esophagus: Unifying the Definitions and Diagnostic Approaches, with Special Reference to Esophageal Atresia. Hassall E Journal of Pediatric Gastroenterology and Nutrition 2011 May Supplement 1.

8) 9)

10)

11)

12)

13) 14)

What is Barrett’s Oesophagus? Leaflet from Barrett’s Oesophagus Campaign (www.barrettscampaign.org.uk) Treatment of a pre-cancerous condition of the oesophagus Australian Radio National interview on “Health report “(transcript) with Associate Professor N Shaheen 2009 June Prevalence of gastroesophageal reflux disease (gerd) and associated complications in adults born with esophageal atresia Maynard S, Pomerleau M, Faure C, Bouin M Poster presented at First International Workshop on Esophageal Atresia, Lille, France 2010 Oct Gastroesophageal reflux: prevalence in adults older than 28 years after correction of esophageal atresia. Deurloo JA, EkkelKamp S, Bartelsman JF, Ten Kate FJ, et al Annals of Surgery 2003 Nov Gastroesophageal reflux and Barrett's esophagus in adults born with esophageal atresia. Krug E, Bergmeijer JH, Dees J, de Krijger R et al American Journal of Gastroenterology 1999 Oct Dyspepsia: Managing dyspepsia in adults in primary care NHS: NICE Clinical guidelines, CG17 - Issued: August 2004 Endoscopic Features in Esophageal atresia: From Birth to Adulthood Faure C Journal of Pediatric Gastroenterology and Nutrition 2011 May Supplement 1


TOFS Children’s Parties

15

A Bright new TOF event in Brighton On a cool afternoon late in 2011, some 17 TOF families (32 adults and 24 children!) met in Brighton for the first recent TOFS social event in the deep south of England. After some excellent organisation by TOFS volunteer Cath Cobden, the Royal Sussex County Hospital had very kindly agreed to let TOFS use their on-site conference suite, the Audrey Emerton building, free of charge for the afternoon. We had plenty of space for the children to be entertained (by Robby the Magician), because we had the building to ourselves! To make a really worthwhile day for TOF parents, as well as for the children, the surgical team at Brighton were good enough to give up their Saturday afternoons to make a short presentation and hold an informal question-and-answer session. Consultant surgeons Miss Avoirt and Miss Hallows came along with Specialist Registrar Mr Adams and Sister Marchant from NICU. Their presence was generally thought to have been very worthwhile, and much appreciated. TOFS vice-Chairman John Pearce commented that “it was great to be told – by someone who clearly knows – that TOFS is one of the very best UK support groups for complex neonatal conditions”. Later in the afternoon, everyone had a chance to chat over a tea-time light meal, where we all enjoyed the sea view in the roof-level café. TOFS trustee Sophie Smallwood, was at the party as well, offering signed copies of her recent Noddy book, “Noddy and the Farmyard Muddle”. Thanks are certainly due to TOFS member Cath Cobden, who lives close to Brighton and who set up the event for TOFS. We are also very grateful to Miss Avoirt and her colleagues. Their participation added a lot to this event and we hope to be able to get the local paediatric specialists along to future children’s parties.

John Pearce TOFS Children’s Party in LONDON - Saturday 28 April We’ve booked Coram’s Fields again (near GOSH) for our London-based family fun afternoon of 28 April 2012. The entertainment’s going to be fantastic and we’ve a Consultant Paediatric Gastroenterologist coming along to talk to parents. Numbers are limited. Make your booking today. Email info@tofs.org.uk

TOFS Northern Party - Saturday 2 June TOFS Northern England party in Huddersfield, West Yorkshire – Saturday 2 June, 2-4pm. Meet and share experiences with other TOF mums and dads. Entertainment for the children, bring your own picnic. The venue (St Philip's Community Centre, in Birchencliffe, Huddersfield HD3 3NL) is just off the M52, junctions 23 or 24. Ample car parking, disabled facilities etc. Book NOW as spaces are limited. Do you live in Northern Ireland ? Would you like to get together with others there? We have a couple of members who are interested. BUT WE NEED MORE. If you’re interested email us now at info@tofs.org.uk


support for families

of children born unable to swallow

TOFS Address TOFS St George’s Centre 91 Victoria Road, Netherfield, Nottingham NG4 2NN Telephone 0115 961 3092

Sunday 13 May

22 to 29 April 2012

TOFS AGM

TOFS Awareness Week

Everyone welcome Venue: TOFS Office, Nottingham

Saturday 28 April

Saturday 2 June

TOFS Children’s

TOFS

LONDON party –

Northern England Party

Coram’s Fields

Huddersfield HD3 3NL

Sunday 13 May

Sunday 8 July

TOFS

TOFS

COM meeting 10.30am

COM meeting 9.30am

Sunday 9 September

Sunday 25 November

TOFS

TOFS

COM meeting 9.30am

COM meeting 10.30am

Fax 0115 961 3097 Email office@tofs.org.uk Website www.tofs.org.uk Registered charity number 327735 Company number 2202260 Medical Patrons Raymond Buick MB Bch FRCS James Dickson FRCS FRCSE FRCPCH John Duffy FRCS, MS, SF(CTh) Bruce Jaffray BMedBiol ChM FRCS(Glas) FRCS (Paed) Leela Kapila OBE FRCS Edward Kiely FRCS FRCS(1) FRCPCH Paul Losty MD FRCS (Paed) Gordon A MacKinlay FRCSEd FRCS Sean Marven FRCS (Paed) Agostino Pierro MD FRCS(Eng) FRCS(Ed) FAAP Charles Shaw-Smith BM BCh MRCP PhD Lewis Spitz PhD FRCS Non-Medical Patrons Richard Briers OBE CBE Dennis Foxon BA David Griffiths MA (Oxon)

Saturday 15 September

Adult Conference full day, Reading

see our website for full details

To book your place at any of our events, please call us now on 0115 9613092 or email info@tofs.org.uk. More details of events at:

TOFS Presidents Gren and Christine Shepherd

http://www.tofs.org.uk/index.php/tofs_events/dates_for_your_diary

Council of Management Hon. Chair: Duncan Jackson Hon. Vice chair: John Pearce Hon. Treasurer: Sandra Hawkins Hon. Secretary: Graham Slater Sue Lewis-Jones Dennis Harvey Gill Jackson Sophie Smallwood

TOFS office staffed:

Office personnel Office manager: Diane Stephens Office administrator: Jayne Allitt Administrative assistant: Debra Marks The views expressed in Chew are not necessarily those of the editor or the Council of Management.

Mon - Thurs: 8.30am - 3pm Fri: 8.30am - 12.30pm (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. Chew editorial board: John Pearce Christine Shepherd Sue Lewis-Jones Diane Stephens

design: www.instaprint.co.uk

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