tofs Spring 2012

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

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

Inside� Page 5 – Mutual support� Page 8 – Interview with Miss

Avoirt, Consultant PaediatricSurgeon

� Page 10 – TOFS AdultConference, Saturday 15September

� Page 13 – Long-term problemsfor adult TOFs

...and much more!

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

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A warm welcome to our new members!� 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 – Ebony-

Grace 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

From the editor...Surely we’re not at the end of March? I know many of youwill be glad to leave the winter, with all its chest infections,viruses, coughs and colds, behind you. But here at TOFSthe year is passing all too quickly and we’ve still so muchto do. The good news is there’s still time to create muchneeded publicity for our TOFS Awareness Week at the endof April. Many of you have already been in touch forpublicity materials – best of luck with whatever you’ve gotplanned. Don’t forget to send us copies of any freepublicity and we’ll do a round-up of them in our nextedition of CHEW.

Planning is well advanced for our next Adult Conference inReading – Sat 15th September. The venue has beenbooked for some time and we are well down the track withgetting the speakers. Is there anything you wouldparticularly like to see addressed in the programme? Let usknow and we’ll try to squeeze it in. At our last conference,despite our best efforts, we were unsuccessful ingetting an Adult (TOF knowledgeable) dietician to comealong. We’re on the hunt for one already this year. Do youknow of someone who could help?

Just because we’re trying to address adult-related TOFissues doesn’t mean we’re overlooking our young TOFs.Quite the opposite. We’ve just had a fantastic party inScotland (more about that in our Summer edition); we’veone in London at the end of April and we have a NorthernEngland party planned for 2nd June. We’re keen to helpsupport new parents better and we’ll tell you more abouta pilot we’re running to do just that in a future edition ofCHEW.

This is the first Chew that I’ve had the pleasure of editing.We’ve made some changes – there’s more medical contentfor starters including a report from the literature on long-term oesophageal problems for adult TOFs. Do you wantto see more articles like this? Remember it’s yournewsletter and it’s important that the content reflects whatyou want to see, so do please give us some feedback.Don’t forget to keep sending those photos and stories forinclusion too. Email’s best, (info@tofs.org.uk) but feel freeto post if it’s easier for you. Your next CHEW will be withyou early July. Deadline for copy is end of May. Have agood Easter break.

Diane Stephens, Chew Editor

Comment from TOFS Chairman,Duncan Jackson:

‘You don’t have to be an athlete to haveOlympic achievements’

Welcome to the Spring Edition of Chew. Before we know itcold dark nights will be over and summer will be here and (lovethem or hate them) the Olympics will be just around the corner. TheOlympics are based upon 7 core values; respect, excellence,friendship, courage, determination, inspiration and equality. Thesevalues can be placed at the heart of any organisation, charity orfamily.

� We all respect each other for the trials / troubles that wehave 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 orcondition

� Everyone values the support that we receive and friendshipsthat 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 arehealthy and leading a normal life style

WELCOMEWelcome to your Spring 2012 edition of Chew!

� Equality – We all want the best health care regardlessof who we are or where we live

Therefore why not try to adopt some of these values? Asthe old saying goes ‘The important thing is not thewinning but the taking part’, so why not hold your ownOlympic fundraiser - a backwards race in your local parkor an egg and spoon through the shopping centre just forTOFS? Have a Tea party, a get together like the Adult TOFShave done on the 31st March. If those sound like too muchhard work and you prefer to watch the athletes on thetelevision, why not make yourself feel better by supportingJames Moore when he runs the London Marathon in April.Remember if you need support / help with anything thenplease contact the office and if all else fails check outwww.conjunctivitis.com – it’s an eye sore!

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Cover story:Jenson was born on 13 January 2007 at theRoyal Victoria Infirmary, Newcastle. He arrived7 weeks early weighing only 3lbs 7oz byemergency Caesarean and as if that wasn’tscary enough he was about to overcome somehuge challenges. Mum Angela, tells us morebelow:

“Jenson’s story actually started following our routine scan at 20weeks when the sonographer couldn’t find Jenson’s stomachbubble….three scans later and we received the news noexpectant parents want to hear – your baby is poorly.Following several tests Jenson was diagnosed with TOF (whichwas actually long gap OA) and a heart condition calledpulmonary atresia.

We spent a few weeks not knowing if our baby would survivethe pregnancy as they thought he had a chromosomeabnormality called Edward’s syndrome – thankfully for us itwas not the case and in a way helped us to have a positiveapproach to all of Jenson’s problems.

Welcome to your second homeJenson (the RVI)

We feel very lucky to have had Jenson’s problems diagnosedduring our pregnancy, not only because we had time to pre-pare ourselves that our baby’s first few weeks would be spentin hospital, but also because he decided to join us 7 weeksearly! Had we not been diagnosed prenatally, Jenson mostcertainly wouldn’t have survived.

His early arrival scuppered the best laid plans – as he was sosmall, and compromised by his heart defect, the medical teamdecided they would ‘grow’ him before the planned TOF repairand first stage heart operation. Jenson had a gastrostomyinserted at two days old and we literally lived on the PICU wardfor the next 4 and a half months – it became our home and themedical team our adopted family! Jenson had a few blips inthose first few weeks, but grew well and at 13 weeks old hehad his OA repair. Again luck was on our side as despite all ofthe 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 wasfollowed by the blow which we hadn’t been prepared for – ourlittle baby had tracheomalacia which was so severe his airwaywould collapse, even while he was being ventilated. This wasso hard for us to take in – we were devastated and it felt at thetime like we had taken one step forward and been thrownthree steps back – a feeling we are used to now!!

Decisions were made to bring forward Jenson’s first stage heartrepair and at the same time they did an aortopexy; once againlady luck was with us as three weeks later Jenson was back inthe RVI, breathing on his own! At almost 5 months old Jensonhad his first experience of being carried across the room (thenurses had to stop us running out the ward – ha!), having aride in his pram in the fresh air – nothing was stopping us thenand we were finally discharged from hospital – hurray!

Home sweet home

I can’t tell you how exciting and totally scary a time thosefirst few weeks at home were!! Whilst they were joyful wehad some scary moments as Jenson’s tracheomalacia causedproblems when he was sick and he had ‘blue spells’ on twooccasions requiring full resuscitation – not good…the resultwas we ended up back in the RVI and Jenson had a Nissen’sfundoplication – after that he just didn’t look back!!

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Jenson’s biggest challenge yet

Due to Jenson’s continuing problems with histracheomalacia a decision was made to bring forward hisfull heart repair operation, with a hope that this wouldhelp. Jenson therefore had his biggest surgery on 14 Feb2008, and was in theatre for 9 hours. Once again theoperation was a total success but history was about torepeat itself as following the surgery we couldn’t getJenson off the ventilator as his airway was actually worse –a bronchoscopy confirmed our worst fears and we were leftwith only one option – tracheostomy.

I can honestly say that we were absolutely devastated atthe time but actually, in hindsight it made such a positivestep for Jenson – we didn’t realise how much he hadstruggled in that first year and the tracheostomy, althoughI never thought I would say this, was absolutely the bestthing for him.

Over the years the tracheomalacia has improvedsignificantly, his tracheostomy tube was removed in March2010 and although we still struggle with regular chestinfections, we are able to keep Jenson at home as much aspossible through giving IV’s (editor’s note - intravenousantibiotics, 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 oralaversion in his first year – this coupled with thetracheostomy has meant he remains to this day 100%gastrostomy fed. That said, as he is growing up, so is hisinterest in food and we have seen some big steps forwardin the last 6 months or so – it’s amazing to think how proudI 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 thesupport of TOFS and the various forums are so helpful - Iknow he’ll get there in his own space and time, we can’t askfor any more than that.

Look at me now!

So, here we are now and Jenson has just started school thisyear. The last 5 years have been a roller-coaster of highs andlows. We have had more in-patient hospital stays then wecare to think of, and Jenson has been in theatre astaggering 32 times (but who’s counting!). We owe somuch to all those who have supported Jenson (and us!)during this time.

Our motto has always been to keep things as ‘normal’ aspossible and allow Jenson to develop in his own time andway – needless to say we are so proud to see our littleJenson, who remains a brave, happy and most gorgeouslittle boy, getting stronger every day.”

Editor’s note: I first met Angela and Steven Carter in2010 when they bravely told Jenson’s story at a Nurse’sStudy Day in Newcastle-upon-Tyne. Can you raiseawareness in your local hospital? Is there a TOFS posterin the neo-natal unit? Have a look and let us know andwe can arrange to have one displayed there.

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Here are some extracts from discussions on the TOFS-enabled Facebook page…(editor’s note – names have beenremoved and some comments summarised)

Hi everyone, my son was born with OA without fistula inNovember at 31 weeks gestation and we are still waiting for himto have his repair surgery. We're looking at surgery not beinguntil March/April time. It's really hard having him in hospital forso long, knowing we can't get him home… he doesn't have amic-key button, he has a gastrostomy and is currently having60mls every three hours. And some responses:

… We've only spent 11 weeks in hospital in total so far but Iknow how you feel. The days are long and your whole life is puton hold. Make sure you get to see a developmentalphysiotherapist who can help you find good positions for yourson to develop and be stimulated. My son had a great chair thatpushed his arms forward and encouraged him to play with hishands.

…I know how you feel. Our daughter (long gap OA with fistula)spent 152 days (who's counting) in hospital before she couldcome home. Since being home we seem to be back every 2weeks to a month for minor issues. People constantly say to methat she doesn't look sick. It is very hard but once she was homewith the family you slowly start to forget it was so long. At 9months she loves being home with her big brother and is doingall the things a normal baby would be doing despite being oncontinuous feeds.

…they might change to a mic-key button as it’s cleaner andmuch easier for you to use, still fed via a machine / tube directlyinto belly. My son now takes 135ml every 3 hours via tube andkeeps 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. Itdoesn't sound like a chest infection this time. I think it could bereflux, his medication had been working really well. He is onRanitidine & Domperidone, but is there a chance he could buildup 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 forOmeprazole as Ranitidine is weaker. My son was on Ranitidineand Domperidone and they changed his Ranitidine as it was notstrong enough. He still has bad times with reflux but he’s a lotbetter. Ask for the liquid - they don't like giving the liquid outas it costs more and has to be kept in the fridge but is a lotbetter for them than the tablet form.

…My daughter who is 9 had a night time cough on and off foryears. I know how draining it is and believe me I tried everythingbut nothing worked. The good thing is she rarely has one nowexcept when she has a cold. It definitely got better with age.

… when my daughter was young we put builders’ bricks underher bed to raise it up and put a rolled up blanket under her feetto stop her slipping down. We also used a humidifier to moistenthe 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 foodbut surprises us on occasions with his random choking, althoughbetter 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.

Mutual SupportIt’s so good to see how supportive TOF parents are of eachother. If you’re a new parent (or not-so-new) and have aproblem then do please call the office. Although we’re nota medical support group we will offer emotional supportwhere we can, and we will always try and put you in touchwith another TOF parent who may have dealt with a similarissue.

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 arealways grateful to those who take time to respond. We’vesummarised some of those responses below. Some of theonline contacts suggest switching medications or that one formis better than another. Such suggestions are NOT medically-approved advice – merely someone reporting their ownexperience. TOFS cannot give medical advice, and westrongly recommend that you should talk to your doctorbefore changing any aspect of looking after yourself / yourchild.

Maria Clifford sought help from parents of teenagers or adultswho had to undergo a fundoplication for the SECOND time. Shewas grateful to hear from Lindsey Hay, mum to Megan (Shaw),who has not only had to get her fundoplication done twice, buta third and now a fourth time. Megan was born with TOF, only1 functioning kidney, an extra thumb, and a slight abnormalityon her spine. She had the operation for repair at one day oldand had to have an aortopexy at six weeks to fix thetracheomalacia. The doctors couldn't take her off C-PAP and theyhad a look and found that she needed a tracheostomy.

She subsequently had laser treatment to remove thetracheostomy but still has a laparotomy feeding tube in and willnot eat willingly. However she has no physical difficulties apartfrom the TOF cough and feeding problems (so is still dependenton her tube). She is going to mainstream school.

Frazer’s mum, Maggie Earl wrote to us telling us about Frazer’slack of smell or taste, wondering particularly if it was TOF-related. We had a number of responses:

Lisa Gilbert, mum to 16 year old Olivia told us about meeting anadult TOF who had no sense of smell at the Adult TOFconference. She went on to say “However, my main reason forwriting is to say that my husband, Paul, also has no sense ofsmell or taste and he has nothing to do with TOF at all - and heis not Olivia's father, so there is no genetic connection. He can tellwhether something is salty, sweet, bitter or spicy but not whatthe actual taste is and decides whether or not he likes some-thing largely by texture. He hasn't had any medical contact abouthis 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'tinterested now after all these years. I don't have any answer orsuggestion as to what can be done, but thought I'd let you knowthat 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 CHARGEsyndrome and it is very common in children with CHARGE tohave no taste or smell because of the malformation of cranialnerves before 12 weeks gestation.

In our Autumn 2010 edition, we featured little Rowan Flambardwho was born with TOF/OA and Down’s Syndrome. Rowan’smum Lauren wanted to make contact with other TOF memberswhose children were born with Down’s Syndrome. We’repleased to report that Fiona Taylor, mum to Ryan responded andwe’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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…My son was 3yrs old when he started nursery but he had aone-to-one who watched him like a hawk. He is now nearly 6yrs old, no longer has a one-to-one but the school know allabout him & his needs. He always goes equipped with a packedlunch & does have his own "special" seat at the dinner table (nextto a teacher).

…My son has been at private nursery since he was 6 months oldfor four full days a week. He is now 3.5 yrs old and loves it. Thestaff have dealt with all his TOF issues brilliantly, they try andclear stickies before calling us. They blend his food if they thinkhe is struggling, and always keep homemade soup in the freezerfor "smooth" days.

…My son goes 20 hours a week since he was 20 months. It’squite a daunting experience letting someone else watch overyour little one but he absolutely loves preschool and enjoysbeing around children his own age.

…My daughter was 12 months and it brought her on in leapsand bounds, they (the nursery) also have been really good withher, braver than me sometimes with new foods!

Editor’s note: One of the TOFS leaflets “About TOF, OA andVACTERL” can be very useful to give to new playgroups ornurseries or schools as a way of “officially” explaining the TOFcondition to the staff there.

My son is having a stomach uplift in GOSH in Feb after his longgap OA was unsuccessful. He was in neonatal intensive care infor 110 days from birth where they tried to fix his long gap, butunfortunately it didn’t work so he’s now got a gastrostomywhich we feed via a pump every 3 hours and a rightoesphagostomy and copes fine with it all. I have read that afterhis stomach uplift he will then have a J tube and fed 24 hourscontinuously. And some responses:

… my daughter had a gastric transposition done about 7 yearsago at GOSH by Prof Spitz. She was 7 when she had it doneand hadn’t eaten up until that point. She now eats anything andeverything and we have never looked back. She never had anyproblems with dilatations etc after and has been very healthysince, although quite small for her age. She is today celebratingher 15th birthday which is something at times we would neverhave thought we would have seen. She had a J tube in for afew months after the operation but as she began to eat quitesoon after coming home, allbeit soft food to start with, this wasonly there to top up her feed if we felt she hadn’t eaten enoughduring the day. Then after a couple of months she wasmanaging to thrive on the food she was taking in. Her tube wasremoved once we hadn’t needed to use it for about 6 weeksfrom what I can remember. She can eat anything now, the onlything she is not allowed is fizzy drinks as they would bloat herstomach which is now situated behind her lungs, so not a goodidea! She is a bit of a grazer rather than 3 large meals a day butwe don’t mind as it is just lovely to have her eating. We stillkeep her bed slightly tilted and try not to let her eat before goingto bed to avoid anything coming back up, and fingers crossedto this day she has never had a problem with that and is not onany medication at all and hasn’t been since the operation.

I have 7 month old twins, born 12 weeks early. My son hasTOF/OA and is due his third dilatation, due to the OA repair doneat the end of September shrunk to less than 1mm (Ed- weassume this means the oesophagus internal diameter), meaningno oral feeding, with a mini button which is leaking constantly.

…Is his skin sore from it leaking? If so we use Maxitrol eyecream......yes strange,I know, but its the only thing that worksand 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 isbehind in some things compared to his twin.....such as he won’troll, he doesn’t shout a lot, he hates the bottle but will shamfeed ok etc but he is advanced in grabbing and holding things,hand to mouth action and has a much stronger head. I think allof these things are from hospital as he had a play specialist andhad to know how to keep his dummy in his mouth. He also justsits and watches a lot, takes everything in, only smiles if yousmile at him where my other one is constantly laughing andsmiling. I’m not so bothered as its expected from being inhospital for so long. He will come on once his operations areover, I’m sure.

I am looking for some advice from you all on 'stucks'...today Iwas feeding my son (8 months old) some small pieces of grape.At one point when he was sucking on the grape a chunk cameoff. Not long after he had saliva drooling out of his mouth andhis nostrils. It was a bit scary as the little lad was clearly indistress. Thankfully my hubby was on hand to give him somefirst aid for choking... And some responses…

…My son is now 3. He salivates when he has sticky. Thick stringysaliva. 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 theyhave a total blockage they can't swallow the saliva. I wouldn'tworry unless it happens all the time. I found that a stricture inour case caused him to put back small amounts of food as hecouldn't get them down fast enough. He didn't salivate the samewith the stricture as it was not a total blockage and the liquidcould still get through albeit a bit slower.

…A sign for us was a rasping sound and our son drawing hishead back. Saliva is definitely an indication too as theobstruction, whatever it may be is stopping our normal "spit"going down and so it pools and goes back up as it builds. Thisin itself can be useful as it can cause a gag reaction that can helpan obstruction to come up.

…Yoghurt was useful for us. If that did not work we would usefull fat coca cola. Or pineapple juice as it helps to break downfood that’s stuck. It takes time to start to work though, so slapson the back time. If still no joy that was the time for us to ringthe hospital. All this is on his care plan for school too. With theproviso that if ever he has trouble breathing or is in distress theyring for an ambulance.

…My son gets raspy and if the stuck food totally blocks theoesophagus so that even liquid can't pass then we get stringysaliva when he vomits it up. When he was a smaller baby, hecoped less well and it can come out of nose and mouth howeveryou have to be careful as when it comes out of the nose thereis more chance it could get to the airways. It's amazing howmuch saliva can pool in the pouch above the join before theybring up the offending food. I would be careful with things thatwon't easily fall apart at 8 months, things like fruit andvegetables as when they get stuck the sticky tends to be worsethan with biscuit and such like.

…My son’s culprits are parsnip, bread and mango. but when heis due a dilatation many types of food becomes stuck, not justone. My surgeon suggested keeping a food diary and justnoting what gets stuck and what doesn't. As the number ofthings gettings stuck increases, a dilatation is needed.

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EAT: What’s in it forTOFS?

EAT (The Federation of Esophageal Atresia andTracheo-esophageal fistula support groups) isthe recently founded international federationof OA/TOF support groups. In this article TOFSTrustee Graham Slater, the first Chairman of thisnew organisation, talks about what EAT hopesto achieve.

TOFS has had valuable informal contact with othersupport groups for a number of years; indeed one ofthe founder members of KEKS, the German supportgroup, was for a time a trustee of TOFS. Our conferenceshave frequently been attended by representatives ofother groups, and a degree of information exchange wasachieved.

A number of support groups (including TOFS) wereinvited to the first-ever International Workshop on OAwhich was held in Lille, France in 2010, as aninternational group to present to an audience ofeminent paediatricians from across the world, many ofwhom were unaware of the work of groups like TOFS. Itbecame apparent at that time that we needed a moreformal platform to build our relationships upon, bothwith one another and with the medical profession. Andso the concept of EAT was born.

One of EAT’s primary goals is to raise the profile of theOA/TOF condition at an international level. It’s early daysyet but in particular EAT hopes to do this through theEuropean Commission’s Health Programme, by gainingthe sponsorship of some major European/globalcorporations, and by participating in internationalmedical forums. These are things which TOFS or any oneof the other member organisations would find difficultto do alone.

Secondly, whilst EAT won’t replicate, take over orsupervise the work of national self-help groups, it isthere to ensure an effective exchange of information,publications, ideas and initiatives so that each memberorganisation of EAT has the opportunity to benefit fromwhat is being done in other countries and what has beenlearnt by all the other member organisations. A lot ofthe most interesting medical work relating to the TOFcondition appears to be going on abroad, soparticipating in EAT should be really useful to TOFS inthis regard.

EAT also intends to develop its own relationships withother institutions such as universities and medicalschools to initiate research and studies which wouldbenefit from being conducted over a wider populationthan just one country. One particular area EAT wants tolook at is the whole area of adult ‘TOFs’, their ongoinghealth, social and employment issues and the questionof transition to adult care. This is a vexed question whicheach of the founder members of EAT has expressed –about the problematic transition from ongoing childcare managed ‘horizontally’ by paediatricians to theworld of adult healthcare with its ‘vertical stovepipes’ ofspecialist care whereby adult ‘TOFS’ find themselvesdealing with not one specialist but different specialists ingastro-enterology, cardio-thoracics etc.

Another of EAT’s objectives is to encourage parents inEuropean countries without a national support/self-helpgroup to establish one. EAT is also already active in thisregard, in particular in Italy where we are cautiouslyoptimistic that a support group will be formed in 2012.It is actually in all our interests for such groups to beformed especially in large countries such as Italy as thisadds to the pan-European credibility of EAT and willbenefit the approaches we make to the EU and to otherpotential corporate sponsors.

At a practical level EAT also plans to introduce an‘emergency card’ which each ‘TOF’ can have and whichsummarises their specific conditions and problems.Together with a dictionary of common OA/TOF relatedterminology, in languages such as German, French andDutch, Italian and Spanish, this should provide parents(and adult ‘TOFs’ themselves) with a useful aid shouldthey find themselves in the unfortunate position ofrequiring medical support whilst travelling abroad.

EAT is still a relatively new organisation and its realsuccess will probably be better measured over severalyears rather than over the next 12 months. Nonethelessthe Board of EAT is determined to have some ‘quickwins’ (such as the emergency card) as well as putting itsefforts into longer-term objectives. Whatever it does, itwill provide TOFS with opportunities both to influenceand to get benefits which would be either impossible orvery difficult to achieve alone. In the words of one ofour European colleagues, ‘together we are stronger’ andthat is why this new organisation has the potential tobring additional benefits to TOFS and to all ‘TOFs’ andtheir families.

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

Is there something the parents could have doneto prevent TOF?Absolutely not. We are still unsure exactly what causesTOF/OA but it is certainly not the fault of the parents. Ithink of it as an accident of nature.

If a baby has a 'long-gap' - why does he / shehave to wait for a repair?We prefer to do a primary anastomosis (join) but in somecases we are unable to do this and have to wait for thechild to grow in the hope that the distal pouch becomeslonger and the gap shorter. It is always better to haveyour own oesophagus than an alternative. We hope todo the delayed primary anastomosis within 3 monthsalthough if the gap hasn’t decreased enough during thatstage we may have to consider an oesophagealsubstitution (an operation in which another part of the

TOFS interview…Ms Avoirt Consultant Paediatric Surgeon

Tell me about your experience as a TOF surgeonI started off in Belgium as a general surgeon but I alwayshad a particular interest in neo-natal and paediatricsurgery. I travelled to get experience and develop myskills in this area, firstly to Holland, then to South Africaand I came to the UK initially for a post of SeniorRegistrar with Southampton General Hospital. I wassubsequently appointed as consultant in The RoyalAlexandra Children’s Hospital in Brighton and I’ve beenhere ever since. At one point, there were only twopaediatric surgeons here in Brighton and I did most ofthe TOF/OA repairs, so was probably operating onaround about 6 a year. Now we see about 8 or 9children born with the condition, but we have a largerteam and last year I repaired 4 babies. I’m a surgeonand I like operating on TOFS – it’s become one of myfavourite operations.

Have many of these parents had the diagnosisantenatally?It’s hard to say. The absence of baby’s stomachcombined with evident polyhydramnios (excess fluid inthe womb) may point to a likely TOF/OA but often this isjust a suspicion. If the child has pure oesophagealatresia it

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baby’s intestines is used to bridge the gap between theends of the oesophagus). There are various options forthis type of surgery, depending on where you live in theUK; personally I favour the gastric interposition as Ibelieve it is safer to put the stomach with theoesophagus, but of course it’s a major operation so stillnot without risk. These patients will need carefulmonitoring throughout their lives.

What advice would you give a new parentleaving hospital with their newly repairedTOF/OA baby?

We give lots of advice. We tell them honestly that theirchildren may have complications after the initial repair.We make parents aware children can develop stricturesat the site of the anastomosis.

Reflux is a common complication because of thedistortion of anatomy. I routinely prescribe children ananti-acid until one year of age because it protects thesite of the join. If they subsequently display a lot ofreflux symptoms that aren’t responding to medicationwe may have to look at doing a fundoplication (anti-reflux operation).

At what point should parents make contact withtheir hospital if they suspect there is a problem?

If a parent feels their child has aspirated or swallowedsomething which has stuck they should contact thehospital as soon as possible. Generally we have openaccess for those children who have had TOF/OA.

What tests do you do to establish if a childneeds a dilatation?

If the child has had to have regular dilatations we areguided by the advice of parents as they usually know. Ifthe child has reached say a year old with no stricturesand the parents now think he has one, we’ll assessthings by initially doing a contrast study. If the strictureis caused by reflux we do further investigation to assesshow bad the reflux is.

Are there any foods which parents of TOFchildren should avoid?

I tell parents about the importance of chewing well astheir child gets older and moves on to solids, and thatsoft and sloppy food is good, and the importance ofdrinking a lot during meals to make food softer toswallow. Some children cope better than others whenthings get stuck. Sausages seem to be common culpritsor dry foods like bread.

MarchJordanne France 07/03/1996 March Hollingbery08/03/1997 Pippa Hynes 08/03/1997 Jack Mullens12/03/1997 Amy Howes 23/03/1997 Georgia Jenkin23/03/1998 Megan Knox 07/03/1999 David Malcolm11/03/1999 Daniel Sandford 28/03/2000 LaurenHulligan 07/03/2001 Olympios Gougoulias 06/03/2002Mia Bradbury 13/03/2002 Grace Walsh 06/03/2003Lukas Weber 09/03/2003 Lauren Naylor 21/03/2003James Rogers 26/03/2003 Ben Smith 03/03/2004Lewie Herbert 05/03/2004 Ryan Lowe 09/03/200James Flynn 24/03/2004 Grace Nolan 06/03/2005Justin M Albrechtsen 24/03/2005 Isabelle Maude31/03/2005 Arwin Bool 22/03/2006 Tobias Jones25/03/2006

AprilJack Berry 09/04/1996 Megan Evans 18/04/1996Thomas Smith 19/04/1996 Daniel Heath 19/04/1996Charles Shotton 25/04/1997 Lucy Robson 26/04/1997Phoebe Smith 29/04/1997 Robert Doward 17/04/1998Laura Thompson 18/04/1998 James Soper 25/04/1998Andrew Day 12/04/1999 David Fenton-Smith26/04/1999 Bradley Newman 11/04/2000 SamuelMullins 18/04/2000 Miriam Holland 03/04/2001Kimberley Taylor 25/04/2001 Samuel Holleron30/04/2001 Phoebe Cannetti 10/04/2002 Zijian Guo10/04/2003 Max Swarbrick 24/04/2003 Lily Rolfe08/04/2004 Cormac Carolan 11/04/2004 GemmaHewitt 01/04/2005 Edie Witton 05/04/2005 Ellis Elson12/04/2005 Peter Riis 13/04/2005 Ewan Brocklesby19/04/2005 Robbie Edwards 20/04/2005 ThomasFlowers 23/04/2005 Emma Morrison 26/04/2005 RileyClifford 09/04/2006 Gabriella Fox 12/04/2006 VictoriaRomero.F 21/04/2006 Savannah Taylor 23/04/2006Erin Davis 29/04/2006

MayConnor Middlehurst 15/05/1996 Matthew Flynn 19/05/1996Jordan Clifford 24/05/1996 Eilidh Latimer 15/05/1997Stephen-Thomas Gray 17/05/1997 Victoria Mince01/05/1998 Anna Rose Greco 05/05/1998 Charlie White10/05/1998 Charlie Gough 11/05/1999 Nicole Morley31/05/1999 Matilda Green 10/05/2000 Isobel Shorrock15/05/2002 Jac Griffiths 03/05/2003 Robert Corbett19/05/2003 Leonardo Capodacqua 06/05/2004 ThomasSmith 07/05/2004 Katy Rainforth 11/05/2004 Luke Burgess13/05/2004 Cyrus Dezfulli 19/05/2004 Adam Gowler05/05/2005 Connor Hardy 16/05/2005 Priya Pandey17/05/2005 Henry Keating 17/05/2005 Joe Turner 30/05/2005Kathryn Lloyd 02/05/2006 Abbie Stubbs 12/05/2006 EveStevenson 12/05/2006 Tahish Shrivastava 18/05/2006Thomas Fryer 21/05/2006 Jack Johnson 22/05/2006 ShayMurphy 24/05/2006

Happy Birthdayto all our TOF-OA Children.Children six years and above are listed below, under sixes willreceive a birthday card.

10

Teen party!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 pastwhen 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 TOFsorganisation? BUT, is your child too old now to reallyenjoy the children’s parties?

I am mum to TOF Laura; you may have met her if you’vebeen to one of the parties or conferences. She lovesgetting together with other TOFs – it gives her a sense ofidentity and helps her to understand how TOF/OA hasbeen 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 arefar less now than they were in the first 5 years – I wouldlike to meet other parents of older TOFs; ones who areapproaching transition to adult hospital care.

So, I wondered if you might be interested in attending aget-together? I’m open to suggestions as to where to goor what to do. Some ideas so far have been activityropes courses, go-carting, or swimming. An area of thecountry suggested was The Cotswolds – but I wouldreally like to hear your thoughts. The time of this eventis also open to suggestion.

If you think you would be interested, have ideas as towhen, where to go or what to do please let us know byringing or emailing the TOFS office, or just send me orDiane a message on Facebook.

Hope to hear from you.

Gill x

The TOFS Adult conference this September is part of ourinitiative in supporting those Adult TOFs who continue tosuffer from respiratory and / or gastroenterologicalproblems. We chose Reading for its good transport linksand the hotel is reasonably-priced should you want tomake a weekend of it. (Please book your accommodationdirectly with the hotel.)

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

TOFS Adult ConferenceSaturday 15 September 2012 (full day)

Holiday Inn, Reading South, Basingstoke RoadReading RG2 0SLExcellent transport links (only half mile from M4Junction 11 with A33), on-site free parking, only3 miles from Reading train station (very goodtrain services from many parts of the UK)Are you an adult with TOF/OA, a Relative,Health Care Professional, or will your TOFteenager soon be making the transition toAdult 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

Raise a tenner for TOFS during TOFSAwareness Week 22 - 29 April

Are you ready for the challenge?Awareness Week takes place every second year to highlightTOF/OA and the plight and achievements of our youngstersall over the UK. You may remember our last official Awarenessweek in 2010: we managed to get TOFS and TOF peoplementioned in many newspaper and magazine articles. What’smore, amazingly you raised over £10,000 – every penny ofwhich is vital as we get no government funding.

This year, as well as raising awareness, we’re challenging allour members to ‘Raise a Tenner for TOFS’. Can you do it? Canwe beat 2010? Here are a few ideas how to raise that tennerand 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 facta full fundraising pack. Non-uniform days, supermarket bagpack, 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, carboot sales, car washing,…

Visit our website for fundraising tips or call the office for aleaflet. Whatever you are planning keep it SAFE and LEGAL(more information available from our office) to help protectyourself and others at your event. And remember, every pennycounts. Don’t forget to give the office a call to tell them whatyou are doing and for more help and advice but mostimportantly – have fun!

If you are having an event, please try to get TOFS and yourTOF mentioned in your local press – all awareness is helpful.Let the office know what’s going on and we can send yousome outline press releases?

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

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FundraisingBIG thanks to Cath

Cobden from Worthingwho held a raffle for TOFS at a Zumba event and raised£200. And she’s not finished fundraising – she’s gotplans for 2012 - here’s her most recent fundraisingappeal:

“Hello to all TOFS subscribers! I am a TOF mummy to Olivernow 2 and a half!! I have recently been asked if I wouldlike a stall at our local fire station open day in July to raisefunds for TOFS! I immediately said yes and went along to ameeting to discuss the dos and don’ts! They (like manyothers) had never heard of the charity or the condition andwere stunned to see my pictures of Oliver as a baby and

comparing them to the real life toddler being a mischiefaround the office! So....they decided there and then thatnot only could I have my stall but the Fire Station wouldtake on TOFS as their charity this year!

So with this in mind I’m asking for help... The Fire Stationwould like to hold a raffle with 6 "BIG" prizes like perhapsa short break or helicopter ride?

I will be holding a tombola and maybe a small silent auctionon the day. So if anyone knows of any companies orindividuals who would like to help out by donating to theraffle or tombola then please please contact Diane at theTOFS office. Vouchers etc can be sent to her for theattention of Cath Cobden. Also on the day I hope to be ableto give out around 500 goody bags with TOFs informationinside as well as pens, sweets etc etc so if anyone wouldlike to advertise in these or give something towards the costof raffle request letters or stamps etc then please againcontact Diane.

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

Cath’s son Oliver is pictured above

TOFS and the VirginLondon MarathonWe had a difficult choice in selecting the successfulentrant to run the Virgin London Marathon for TOFS. Itwill probably be another five years before we areawarded another official TOFS place so it was animportant decision, and a really tricky one as all of thefour candidates seemed very committed and worthy ofa place, and it was regrettable we could only chooseone.

We are pleased to announce that the coveted place hasgone to James (Jezz) Moore, uncle to little TOF/OA toddlerJoseph Wood.

James is 23 and lives in Redditch, just outsideBirmingham in the West Midlands. He works as anaccount manager for City Electrical Factors - anelectrical suppliers. His nephew Joseph Dylan Wood(parents Lis & Gary Wood) was born with TOF/OA in2009. He also has a friend, now 23, who was born withTOF. James told me: “I’m a very sporty person. I love alltypes of sport, especially football, golf and cycling. It isa dream come true to have the opportunity to run theLondon Marathon 2012. Thank you for all of yoursupport and I hope I can do everyone at TOFS, andeveryone involved proud.”

James has already been training and some of you havesponsored him already. You can continue to sponsorhim at www.charitygiving.co.uk/jamesmoore

We mustn’t forget those of you who have beensuccessful in getting a Marathon place in the ballot andintend to run it for TOFS. We’ve heard a whisper thatNicklos Tyrer, from Dorset (TOF/OA daughter Maddison isnow 11) is also running for us – best of luck to you bothwith your training….

Are you running a Marathon (or a half marathon) for TOFSnext year? Let us know NOW if you are. Email the office:info@tofs.org.uk. We can send you a TOFS sponsorshipform and a TOFS T-shirt, if you’d like one. Indeed if you arethinking about doing any other sponsored event for TOFS-like one of those long bike rides, perhaps, please let usknow.

Our thanks to Elaine Willisfrom Chippenham,mum to TOF son Tyler who is now 13. Elaine ran the ParisMarathon last April in a staggering 26ºc raising £307 inthe process. Elaine completed her run in 4 hours and 37minutes. She tells us that her employer’s son who is now5, was also born with TOF.

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In Memory:

£160.00 donated in memory of the late Mr Roy Marten,grandfather to Jack Morten, including - £5 donated fromAngela & Brian, £25 donated from Mrs K Morten, £10 donatedfrom Doris Procter.

Collection made at the funeral of the late Mrs Joyce MarySharpe – £394.79

200 Club WinnersNovember – Sarah ThomasDecember – Mary HartnettJanuary – Ernest Towner

GlossaryDefinitions of technical and medical termsmentioned in some of our articles and familyupdates:

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

Pulmonary Atresia: congenital severe narrowing orobstruction of the pulmonary orifice .

Edward’s Syndrome: a very serious abnormality that leadsto severe mental and growth retardation, abnormalities in theheart and other organs and malformed features.

Tracheomalacia: ‘floppiness’ of the trachea.

Aortopexy: surgical procedure used in the management ofsevere tracheomalacia, in which the aorta (a major arterywhich lies to the front of the trachea) is anchored to thesternum (breast bone). This has the effect of opening up thetrachea.

Nissen Fundoplication: this procedure is commonly usedto reduce Gastro-Oesophageal Reflux (GOR). The loweroesophagus is freed up and the top part of the stomach(known as the ‘fundus’) is wrapped around it

Tracheostomy: the surgical creation of a connectionbetween the trachea and the skin at the neck, and the insertionof a tube into the trachea to preserve a clear airway. Used inthe management of severe tracheomalacia.

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

Laparotomy: Operation involving the opening of theabdominal cavity, usually as an investigative procedure.

Mic-key button: enteral feeding tube

Oesophagostomy: surgical creation of a connectionbetween the oesophagus and the body wall (skin) usually sothat a feeding tube can be inserted directly to the oesophagus.

Gastric transposition:method of oesophageal replacement(also known as stomach pull-up or uplift)

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

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

Reflux: also known as Gastro-Oesophageal Reflux, theinvoluntary return of gastric (stomach) contents to the lowerend of the oesophagus

Fundoplication: surgical procedure to treat reflux, seeNissen’s above

Dysplasia: Abnormal development of cells.

Endoscopy: procedure where the oesophagus, stomach andduodenum is examined internally using an endoscope (a tinyswallowable camera on a long thin flexible mount)

Thank you to:� David Paton grandparent to Lewis Carney donated

£25.00 after giving a talk on the history of his collectionof toast racks.

� Wolverton & Stony Stratford Rotary donated £190.00raised at a golf day charity event.

� Kids Planet Day Nurseries donated £105.90 raisedthrough fun days at nursery.

� Notts Fire & Rescue Retired Members Associated -£125.00 raised at an AGM fundraiser.

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

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

� Tiger Developments - £250.00 in lieu of sendingChristmas cards.

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

� Leela Kapila - £150 fee for lecture given for Medlink

� Terry Collins and Graham Parkin - £50.00 donation

� Julie Molyneux - £60.00 collection from Webb IvorySales

� Staff at The Smallberry Green Primary School raised£100 instead of sending Christmas cards to one another.

� Margaret Lemon, mother of Colette Stones born withTOF donated £50.00

� Kirsty and John Robb - £50.00 donation

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Long-term oesophageal problems for adult TOFs

Why write this review article?I remember sitting in a TOFS conference several years ago, andbeing startled by something said by one of the paediatricsurgeons who was presenting. This was that TOF people are athigher-than-normal risk of a condition called Barrett’sOesophagus and eventually even oesophageal cancer. Foranyone related to a TOF, this is hardly the happiest of thoughts,especially since this form of cancer is a relatively unpleasantone. I made a mental note to find out a bit more about this oneday. More recently, I took part in the 2011 TOFS conference: itwas obvious in the adult TOF session that some clearcommentary is wanted on the extent to which adult TOFs needongoing care of the oesophagus. This can only become moreof an issue as the numbers of adult TOFs and their average agegoes up: so far there are very few adult TOFs older than 55.

What I have found is that groups of doctors (almost alloutside Britain) have been publishing their findings, havingfollowed up groups of adult TOFs, to check up on the severityand extent of long-term outcomes. I have reviewed a lot ofwhat the doctors have published relating to long-termoesophageal problems. Positively, there are as yet very fewreports of TOFs suffering from oesophageal cancer. Howeverthe doctor’s studies make it clear that Barrett’s Oesophagus(which is treatable) is far more common amongst TOFs thanamongst the population as a whole

What the Doctors are publishingThe subject of an unusual complication associated with therare TOF/OA condition does not come up all that frequently inthe medical journals. But it certainly was mentioned at the late2010 First International Workshop on Esophageal Atresia.[Note – spelt the American way] The papers from thisconference were published in mid 2011, and I’ve beenreading…

Incidentally, TOFS attended this workshop. One of the paperswas 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 thepast 3 years or so.

Two things struck me when I looked in some detail at what thedoctors are publishing. Firstly, quite a lot of the work they are

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

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

The question of potential oesophageal cancer is principally aconcern for Adult TOFs rather than for children: it takes sometime for cancers to develop. One 2011 paper (1) said “ To date,Worldwide, six cases of esophageal cancer have been reportedin young adults treated for EA”. Six is not a huge number atall.

Moreover, very few adult TOFs appear to have developedoesophageal cancer so far.

One study (2) checked up on some 270 known adult TOFs inFinland, and found 3 cases of cancer, none of which wereoesophageal cancer. The authors of this Finnish study (3)

concluded that “the overall cancer risk of adults with repairedoesophageal atresia is similar to that of the generalpopulation”, though they did not estimate how high is theoesophageal cancer risk for adult TOFs. A similarly large studyof adult TOFs in Australia (4) saw 130 adult TOFs assessed, andonly one was found to have a form of oesophageal cancer.

So, encouragingly, it seems that very small numbers of TOFshave so far developed oesophageal cancers – we are not yetaware of even a single one reported in the UK.

According to the NHS, oesophageal cancer is the seventh mostcommon type of cancer in the UK, with an estimated 7,000new cases diagnosed here each year (5). [For comparison, Breastcancer is the most common cancer, with 46,000 new casesannually] Men are more likely to suffer from oesophagealcancer than women, and the risk of contracting this cancer issignificantly increased by smoking, and drinking alcohol,(5).Obesity and drinking very hot drinks are also thought toincrease the risk of this cancer. The average age at diagnosis is72: since there are as yet few adult TOFs over the age of 55,no-one can yet really say how much higher (than normal) isthe risk of oesophageal cancer for TOFs.

Barrett’s Oesophagus could be abig deal for older TOFsFor TOFs, oesophageal cancer may not yet obviously be a majorissue, but a precursor condition, Barrett’s Oesophagus, mightwell be a major issue for adult TOFs.

You hardly need us to tell you that reflux is one of the mostcommon problems experienced by TOF people. Significantnumbers of TOFs have fundoplication surgery to tackleongoing excessive levels of reflux. Ongoing significant refluxmeans that the lower parts of the oesophagus are exposed tostomach acid and bile, which should not normally happenmuch at all, and which irritate the lining of the oesophagus,leading to inflammation, sometimes referred to as refluxoesophagitis or GORD: Gastro-Oesophageal Reflux Disease (5,6).Americans call this GERD for spelling reasons, by the way.

Continued�

by TOFS Vice-chair John Pearce

14

Over several or many years of GORD, the cells lining the loweroesophagus can start to change to become more like thosethat line the intestine. There is some debate amongst expertsas to exactly which types of cells mean that a patient hasBarrett’s (7). In order to diagnose Barrett’s, doctors wouldtypically do an endoscopy (have a look with a camera scopewhich the patient swallows through the mouth) and takebiopsies (tiny samples) of the cells, looking for evidence ofsomething called intestinal metaplasia.

The UK Barrett’s Oesophagus Campaign (8) estimates that375,000 people (or about half of one percent of thepopulation) in the UK have Barrett’s. Some Barrett’s suffererswill 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 tosome other experts (9). The Campaign seeks to draw attentionto Barrett’s Oesophagus – and to encourage people to take itseriously. The main message here is that frequent or persistentreflux (often observed as “heartburn”) should not be ignored.

The medical literature also gives some insight into the extent ofBarrett’s Oesophagus among TOFs. The Finnish study (3), foundthat 34% of their adult TOF patients had symptoms of reflux (vs8% in a normal-population group). Barrett’s was confirmed in6% of these TOFs. The Australian study (4) reported that 63%of their adult TOFs had reflux symptoms. Barrett’sOesophagus was found in 11% of those TOFs whose symptomswere thought significant enough to warrant investigation (or5.3% of the whole study group).

A small Canadian study (10) found reflux symptoms in 61% ofadult TOF respondents: as many as 18% of those who wereexamined in detail (or 12% of the study group) were found tohave Barrett’s. Two small studies of adult TOFs in theNetherlands found Barrett’s in 2.6% (11) and 5.8% (12) of theirstudy groups.

These percentages vary quite a lot, but on the strength of thevarious papers, I think it is reasonable to expect that somethingin the region of 3% to 10% of current-day adult TOFs might besuffering from Barrett’s. If so, then Barrett’s is far (perhaps 10-20 times ) more common amongst adult TOFs than amongstthe rest of the population. And bear in mind that the average

age for diagnosing Barrett’s in the population as a whole is62 (6). The oldest TOF in the UK is not yet 70 and there are fewTOFs over 55.

Standard guidance onEndoscopies seems inadequatefor TOFsSo what’s the point of this? If you have ongoing reflux and it’snot getting any better, you certainly should think about askingyour doctor to consider sending you off for an endoscopy. Theolder you are, the more important this could be.

Standard UK guidance from NICE to Doctors says thatendoscopies are not normally worth doing in people under age55 (13) . One assumes that this is because there is a cost to theNHS, a small risk to the patient, yet a very small chance offinding any problems in the normal population below age 55.But in this regard TOFs are not the normal population – thepapers I have reviewed suggest that the risk of Barrett’s ismuch higher in TOFs than the normal population.

Moreover, the strong consensus among the experts whosepapers I have reviewed is that TOF patients should haveendoscopies, whereas the normal population might notwarrant such examination. However there is debate aboutunder what circumstances or how frequently endoscopicexaminations should be done. The Finnish (3) investigators, forinstance,(3) say that studies are needed to “define guidelinesfor 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 undergoinvestigation regardless of age”.(7) One group of researchers (11)

are less than convinced: “it is still very disputable if all EApatients should be screened at an adult age”. But anotherpaper recommends “clinical assessment as adults and upperendoscopy for reflux symptoms or dysphagia” (4), whilst otherexperts (10) say “Special monitoring including endoscopicscreening may be beneficial” to adult TOFs. Another (14)

suggests “systematic screening of endoscopic lesions in allpatients with EA, even in the absence of upper GI symptoms”with regular endoscopies every 5 years.

References1) Long-term results of esophageal atresia: Helsinki experience and review

of literature.Sistonen SJ, Pakarinen MP, Rintala RJ.Pediatric Surgery International 2011 Nov

2) Cancer after repair of esophageal atresia: population-based long-termfollow-up.Sistonen SJ, Koivusalo A, Lindahl H, Pukkala E, Rintala RJ, .Pakarinen MP,Journal of Pediatric Surgery 2008 Apr

3) Outcome of Oesophageal Atresia beyond Childhood.Rintala RJ. Sistonen SJ, Pakarinen MP,Journal of Pediatric Gastroenterology and Nutrition 2011 May Supplement 1.

4) Gastroesophageal reflux and related pathology in adults who were bornwith esophageal atresia: a long term follow-up study.Taylor AC, Breen KJ, Auldist A, Catto-Smith A, Clamette T, et al ClinicalGastroenterology & Hepatology 2007 June

5) Oesophageal cancerFrom website: NHS: Your health, your choices 2010 Aprilhttp://www.nhs.uk/conditions/Cancer-of-the-oesophagus/Pages/Introduction.aspx

6) Heartburn and gastro-oesophageal reflux disease - Complications From websiteNHS: Your health, your choices 2010 Junehttp://www.nhs.uk/Conditions/Gastroesophageal-reflux-disease/Pages/Complications.aspx

7) Esophagitis and Barrett Esophagus: Unifying the Definitions and DiagnosticApproaches, with Special Reference to Esophageal Atresia.Hassall EJournal of Pediatric Gastroenterology and Nutrition 2011 May Supplement 1.

8) What is Barrett’s Oesophagus? Leaflet from Barrett’s Oesophagus Campaign(www.barrettscampaign.org.uk)

9) Treatment of a pre-cancerous condition of the oesophagusAustralian Radio National interview on “Health report “(transcript) with AssociateProfessor N Shaheen 2009 June

10) Prevalence of gastroesophageal reflux disease (gerd) and associated complicationsin adults born with esophageal atresiaMaynard S, Pomerleau M, Faure C, Bouin MPoster presented at First International Workshop on Esophageal Atresia, Lille,France 2010 Oct

11) Gastroesophageal reflux: prevalence in adults older than 28 years after correctionof esophageal atresia.Deurloo JA, EkkelKamp S, Bartelsman JF, Ten Kate FJ, et alAnnals of Surgery 2003 Nov

12) Gastroesophageal reflux and Barrett's esophagus in adults born with esophagealatresia.Krug E, Bergmeijer JH, Dees J, de Krijger R et alAmerican Journal of Gastroenterology 1999 Oct

13) Dyspepsia: Managing dyspepsia in adults in primary care NHS: NICE Clinicalguidelines, CG17 - Issued: August 2004

14) Endoscopic Features in Esophageal atresia: From Birth to AdulthoodFaure CJournal of Pediatric Gastroenterology and Nutrition 2011 May Supplement 1

15

TOFS Children’s Party inLONDON - Saturday 28 AprilWe’ve booked Coram’s Fields again (near GOSH) for ourLondon-based family fun afternoon of 28 April 2012. Theentertainment’s going to be fantastic and we’ve a ConsultantPaediatric Gastroenterologist coming along to talk to parents.Numbers are limited. Make your booking today.Email info@tofs.org.uk

TOFS Northern Party- Saturday 2 JuneTOFS Northern England party in Huddersfield, West Yorkshire– Saturday 2 June, 2-4pm. Meet and share experiences withother TOF mums and dads. Entertainment for the children,bring your own picnic. The venue (St Philip's CommunityCentre, in Birchencliffe, Huddersfield HD3 3NL) is just off theM52, junctions 23 or 24. Ample car parking, disabledfacilities etc. Book NOW as spaces are limited.

Do you live in Northern Ireland ? Would you like to gettogether with others there? We have a couple of memberswho are interested. BUT WE NEED MORE. If you’re interestedemail us now at info@tofs.org.uk

TOFS Children’s PartiesA Bright new TOFevent in BrightonOn a cool afternoon late in 2011, some 17 TOFfamilies (32 adults and 24 children!) met in Brightonfor the first recent TOFS social event in the deep southof England.

After some excellent organisation by TOFS volunteer CathCobden, the Royal Sussex County Hospital had very kindlyagreed to let TOFS use their on-site conference suite, theAudrey Emerton building, free of charge for theafternoon. We had plenty of space for the children to beentertained (by Robby the Magician), because we hadthe building to ourselves!

To make a really worthwhile day for TOF parents, as wellas for the children, the surgical team at Brighton weregood enough to give up their Saturday afternoons tomake a short presentation and hold an informalquestion-and-answer session. Consultant surgeons MissAvoirt and Miss Hallows came along with SpecialistRegistrar Mr Adams and Sister Marchant from NICU.Their presence was generally thought to have been veryworthwhile, and much appreciated. TOFS vice-ChairmanJohn Pearce commented that “it was great to be told – bysomeone who clearly knows – that TOFS is one of thevery best UK support groups for complex neonatalconditions”.

Later in the afternoon, everyone had a chance to chatover a tea-time light meal, where we all enjoyed the seaview in the roof-level café. TOFS trustee SophieSmallwood, was at the party as well, offering signedcopies of her recent Noddy book, “Noddy and theFarmyard Muddle”.

Thanks are certainly due to TOFS member Cath Cobden,who lives close to Brighton and who set up the event forTOFS. We are also very grateful to Miss Avoirt and hercolleagues. Their participation added a lot to this eventand we hope to be able to get the local paediatricspecialists along to future children’s parties.

John Pearce

TOFS office staffed:Mon - Thurs: 8.30am - 3pmFri: 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 thenumber of the volunteer currently providing this service.

Chew editorial board: John PearceChristine ShepherdSue Lewis-JonesDiane Stephens

support for familiesof children born unable to swallow

tofsTOFSAddressTOFSSt 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(1) 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 MRCP PhDLewis 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: Sandra HawkinsHon. Secretary: Graham SlaterSue Lewis-JonesDennis HarveyGill JacksonSophie Smallwood

Office personnelOffice manager: Diane StephensOffice administrator: Jayne AllittAdministrative assistant: Debra Marks

The views expressed in Chew are not necessarily those of theeditor or the Council of Management.

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Sunday 13 May

TOFS AGMEveryone welcome

Venue:TOFS Office, Nottingham

22 to 29 April 2012

TOFSAwareness Week

Saturday 28 April

TOFS Children’sLONDON party –Coram’s Fields

Saturday 2 June

TOFSNorthern England Party

Huddersfield HD3 3NL

Sunday 13 May

TOFSCOM meeting10.30am

Sunday 8 July

TOFSCOM meeting

9.30am

Sunday 9 September

TOFSCOM meeting

9.30am

Sunday 25 November

TOFSCOM meeting10.30am

Saturday 15 September

Adult Conferencefull day, Reading

TOFS 2012 Calendar

see our websitefor full details

To book your place at any of our events, please call us now on0115 9613092 or email info@tofs.org.uk.More details of events at:http://www.tofs.org.uk/index.php/tofs_events/dates_for_your_diary