Winter 2012/2013

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

Pictured: Jack Johnson from Pyrford near Woking, Surrey fundraises for TOFS!

Read his story on pages 8 & 9 and all about his fundraising on page 12

Inside

Pages 6 & 7 Interview with Shalinder Singh, Consultant Paediatric Surgeon

Pages 4 & 5 Managing Mealtimes, Cathy Birchmore SaLT

Pages 3 & 4 Little Louis Witham’s story

Page 13 Lili keeps up to date with Chew

Did you know…TOFS was s t ar ted by parents and cont inues to be run by parents , g randparents and Adul t TOFs .

2

WelcomeWelcomeFrom the EditorWelcome to your Winter 2012/13 issue of Chew.

Our cover story features the inspirational Jack Johnson. Jack’s long gap was repaired using the controversial Foker technique, which

worked thankfully, albeit with some complications. As TOF parents you’ll identify with his “energy, life and love, amazing laugh and very loud TOF cough!” Read all about it in his dad’s words on page 6.

Was your little one born with a long gap atresia? Do you have experience of the Foker technique? Your story may be useful for other parents. Please do get in touch.

We know how important it is for new parents to find out that TOFS exists straight after their baby has been born and this is a real challenge. But more and more we read on our membership forms that parents have found out about us in their neo-natal unit (even in Australia!), either from their consultant, nurse or our poster, so we know we must be doing something right. It brings home how much, as a parent-led organisation, we work in partnership with our members - thank you for spreading the word.

We debate how to best support our TOF/OA families. Are we wasting time and money sending Chew to folk who never feel inclined to read it? Are you weary of receiving too many emails from TOFS? Or do you feel you don’t hear from us enough? Are you fed up fundraising? Do you feel you’re getting enough in return? What should we be doing that we’re not? What are we doing that’s not needed? Please do tell us. We’re reviewing all of this when we next meet at the end of January and all your suggestions or comments are welcome. Email diane@tofs.org.uk One thing we are doing that we know is certainly of value is organising the next TOFS family conference. Some of you are already making plans to come. More information on page 9.

I’m very pleased to announce we have a new medical patron on board. Anthony Lander, from Birmingham Children’s Hospital. We are grateful for his support to TOFS over the years, and we’re delighted to welcome him on board. We hope to carry a full interview in the next edition of Chew.

On a very sad note, we know that, although thankfully rare, tragedy can and does strike in our TOF community. Our very deepest sympathies to anyone grieving at this time. TOFS is here if you need us, whenever that may be.

Diane Stephens

Chew editor

A warm welcome to our new members!• Lucy and Duncan Laidlaw from Surrey – Hamish born 05 February 2011• Barbara Szomler and Kamil Siekanski from London – Adam born 30 August 2011• Dawn and Paul Knox from Tyne & Wear – Ayla born 03 May 2012• Emma and Graham Webster-Newman from Hertfordshire – George born 09 September 2012• Camilla and Ed Harvey from London – Harry born 05 August 2012 • Ildiko and Simon Evans from Surrey – Indigo born 02 August 2012 • Shannon Stevens from Australia born with TOF/OA• Laura and Colin McCartney from Falkirk – Tom born 04 November 2012• Erin and Matthew Carver from Australia – Abbey born 07 November 2011• Caroline Tims and Michael Maher from Hampshire – Rowan born 31 October 2012• Katy and Scott Moylan from Australia – Conor born 25 March 2008• Kathryn and Simon Turner from Buckinghamshire – Christopher born 15 May 2012• Hannah and Peter Eddowes from Nottingham – Lola born 12 November 2012• Linda and Neil Derbyshire from Derbyshire – Thomas born 08 August 2012• Nicola and Callum Sutherland from New Zealand – Abby born 21 April 2012

From the Tofs Chair, Duncan Jackson

Happy New Year!

Resolutions: we all make them and most of us generally break them within the first few months. Having a very busy day job means that my resolutions often centre around finding more time to be at home, as being with my family is most important to me. This set me thinking: TOFS and the people linked

to the organisation are like an extended family. But, who are TOFS? We were started way back in 1982 by a small group of parents who had similar problems with their children and who wanted to support each other.

TOFS today is still run entirely by parents and grandparents of TOFs and by Adult TOFs. We are a small charity supporting about 8000 TOFs in the UK and respected by leading paediatric surgeons. Mr Kiely from GOSH said “he was humbled to chair our conference”. Every trustee has been touched by TOF/OA so we know what it’s like. That is why we are all prepared to give so much time to helping others.

TOFS tries hard to help its members and the wider community. Make your 2013 resolution to support TOFS, organising get-togethers, fund-raising or letting us know if you think we can do better in some way or another. As Christmas is over, one last cracker joke. Q: Why is it so hard to take a blood sample? A: Because you're always searching in vein.

Duncan Jackson

Family updates & newsWelcome Family updates & news

3

Louis’ Story

Louis was born on 17 May 2010 to the sound of Van Morrison’s ‘Brown Eyed Girl’. The 20 week scan didn’t give us any clue as to what to expect when our bundle of preciousness made it into the world. And

although he sounded a little chesty at birth, we were told by the theatre staff that this was a natural consequence of the Caesarean birth. His nail beds and lips were tinged with a blueness which remained all day and when he fed it came back through his nose each time. It wasn’t until later that same evening when, following much investigation, we were asked to come up as the doctors needed to speak with us in the Neonatal ICU. We were ushered into a room and asked to sit down; they told us that Louis had what was called a TOF and that an emergency operation was needed. We signed the consent forms and went back downstairs to our room with the fresh Polaroid photo they took of him before he went to theatre. We did not speak for 4 hours such was the shock.

Louis had what seemed like a dozen pipes coming out of him as he lay in his Perspex cot in the NICU dept of Hull Royal Infirmary. We were told he was doing fine and that the operation had gone well as the gap was short so the repair was fairly straightforward. We so wanted to hold and cuddle him but we could not. This was to be the case for the next 4 weeks; it was heartbreaking for us and we never left his side. But despite developing Horner’s Syndrome (a condition relating to damage to the nerves controlling the eyelids during the operation) and suffering a collapsed lung due to a less than careful nurse accidentally dislodging his chest drain, he managed to go from strength to strength. Following another couple of weeks on the main ward of the Neonatal unit we bought our sunshine boy home with us.

Louis has had four dilatations which all went very well and all were performed within the first six months. Since then he has been checked but deemed ok for the time being. Louis’s doctors say that it is likely that he will need further dilatations but certainly not right now. Incidentally, the surgeon who performed Louis’ repair was Sanja Besarovic: of course we knew nothing of this lady before 17 May 2010 but since then we have come to know her, not only for her quite astonishing skill as a surgeon, but also for her personal story of fleeing war-torn Sarajevo smuggled inside the trunk of a car, but then going on to becoming one of this country’s most accomplished and respected paediatric surgeons. It seems that, in a bizarre way, we were lucky to be living in the same city where she practised. Louis does see his surgeon regularly at his TOF Clinic sessions.

Development IssuesWhen Louis was over 1 year old we noticed that he sometimes seemed a little withdrawn and this seemed to get worse. Sometimes we wondered if Louis even knew we were in the house or indeed the same room. Other things gave us cause for concern about Louis’s development; he never pointed or gestured that he was hungry or thirsty like other children of his age, all the time he seemed to be withdrawing more and more into himself. He never responded to his name and would not give us any eye contact. We decided

to seek professional help and now, following months of appointments with specialists and tests etc, Louis is getting the help he needs to develop social and communications. Louis regularly receives Portage sessions at home and at the local children’s centre and is doing well. He also started preschool in September and is funded for one-to-one care at the preschool. Louis’ one-to-one is a wonderful lady that genuinely cares for him and has definitely fallen for the Louis charm!! We think that, on reflection, our decision for Louis to start preschool a year early is perhaps one of the best things we could have done for him to aid his development issues. Now we get all the eye contact we ask for and he sometimes points to what he wants, like food, and responds fully to his name albeit when he wants to!

Louis has recently been subject to the rather gruelling Griffiths Assessment which is designed to give a clearer, more defined, picture of any developmental delay issues, and will illuminate the paths available for us to ensure that Louis receives the very best of care and help in order for him to achieve the goals for a normal 3 - 4 year old when the time comes. As we write this, we

Continued on page 4

Mum and Dad Kelly Neylon & Mostyn Witham from Hull share Louis’ story

4

I don’t think I’ve ever met a parent who hasn’t battled with their child at mealtimes at some point - myself included. After all, we all want our kids to eat and it’s our job to get them to eat, right? Well, no actually. Counter-

intuitive it may sound, but it is not your job to get your child to eat. Let’s come back to that later.

Children who have TOF/OA and their parents each have differing journeys through their child’s infancy, and childhood. Some children wean relatively easily, others have more complex medical interventions and may encounter significant difficulties extending into early childhood. It’s rarely completely plain sailing. In general, the more complex the intervention your child has received, the more likely that weaning will encompass some problems.

There are some feeding issues which are quite specific to TOF/OA children. These are related to the oesophageal incoordination, stricture or reflux and are well described in the TOFS leaflet ‘Feeding Your TOF/OAChild.’ Another valuable resource, particularly for nursery or school transition times is the TOFS Short Guide for Parents and Teachers. (Editor’s note, both these leaflets are available from TOF office/website.) This will help teachers or carers understand some of the issues and react appropriately when they arise.

For those children who have had a lot of medically necessary but invasive procedures, sensory issues can result. These can be difficult to understand - if surgery has been successful and there’s no longer any major anatomical reason why your child is struggling, why is she so reluctant to eat? The answer may be rooted in the abnormal and often unpleasant and uncomfortable experiences in your child’s early life - tubes, reflux, and vomiting, gagging and, illness. Unfortunately, the effects of these early experiences can be long-lasting; they interrupt sensory development so that the gag reflex is overly sensitive and the child can learn that feeding is not a pleasant experience.

But how do we manage mealtimes at home? If mealtimes are a battlefield at home, with your child reluctant to try new foods, or to eat a sufficient quantity, how do you deal with it? Well, aside from the specific TOF/OA difficulties referred to above, your child is, of course, no different from other children; whatever the level of medical intervention and degree of feeding difficulty, fundamentally the principles you can use to help your child at home are the same as for any other child. I should add, however, that this article is not designed to supplant advice from your local therapeutic team - they should be your first port of call for your child’s specific feeding difficulties and overall nutritional intake.

So, what are these principles of mealtime management? I’ve never encountered a better way to explain these than the framework used by American Dietician and researcher, Ellyn Satter. She uses a Division of Responsibility to describe the parents’ and child’s jobs at mealtimes. Thus, her golden rule for mealtimes is:

‘Parents are responsible for the what, when and where of food.

Children are responsible for how much and even whether they eat.’

You’ll see that this premise requires a huge amount of trust in your child - we all worry that our children aren’t eating the right quantity or variety of foods. But in fact, research shows that infants and young children have a strong innate ability to eat the right amount and variety of foods their body needs to grow. It’s our job to foster this in-built appetite self-regulation by letting your child get on with it. The fact is that the more we interfere, the more likely we are to impede their ability to self-regulate.

Another way of putting it is: pressure doesn’t work. The more we pressurise children to eat, the more likely they are to refuse, and the more frustrated the parent becomes; the scene is set for those mealtime battles. And there are a myriad of ways that we put pressure on our children to eat - all those little habits and tricks we use: ‘Here comes the aeroplane’, ‘Just three more mouthfuls’, ‘Eat some more carrot and you can have pudding’. These may work in the short term, but in the long term you are undermining your child’s control over the ‘whetherwhat and how much’ of food; you are not sticking to your side of the Division of Responsibility.

Managing TOF Mealtimes - Manic or Mellow?are awaiting the results of that assessment. Paediatricians are not yet sure of the reasons that Louis is suffering developmental delay in some areas. It is possible that this delay could have been caused by early life trauma; I mean, heaven knows how the operation must have seemed for him at the time, as if being born isn’t traumatic enough.

In the early days we read about the TOF condition and what to expect of a TOF child in terms of feeding and eating. But, for the most part, Louis seems to buck the trend on the food issue. Yes, he does get the odd thing stuck and, yes, he is often sick but it does not seem to diminish his love of food. Now he has all of his teeth we think that he must see all types of food as a challenge! Give him anything and he’ll eat it, or at least have a very good go at it. It was this summer in a burger joint on holiday in Turkey that we discovered his penchant for cheeseburgers (with fries and a coke). Of course we love that he loves food and hope that it continues all his life.

Louis does seem inclined to chest infections and we read that this is not out of the norm for TOF kids, even our GP knows the drill and now is used to discerning the difference between a normal Louis chest sound and one with an infection.

Encouraging outlookHopefully, Louis is on the road to a full recovery TOF-wise. As for his other development issues, well, we are doing our best with him at this time and can do no more, we just hope that will sort itself out as he grows up more. We found out about the TOF Charity very quickly and registered on the website, and we find the CHEW magazine to be a wealth of information. It has been a constant source of hope and inspiration to both of us over the years. The TOF Facebook forum page has also been of particular help and interest for us also. We intend to become even more involved with events in the future.

Continued from page 3

own Dietician and SALT team in relation to your child’s specific feeding difficulties.We’d like to talk more about feeding issues in our next edition of Chew; please email us (by mid-Feb at latest) if there’s anything you’d like to discuss or share with other parents.

ReferencesPowell, F., Farrow, C., Haycroft, E., Meyer, C. (2011). Appetite regulation in children: the impact of parenting behaviours and child temperament. In S. Mitchell (Ed.), Appetite, Regulation, Role in Disease and Control (pp. 1-29). New York: Nova Science Publishers.Satter, E. (1995). Feeding dynamics: helping children to eat well. Journal of Pediatric Health Care, 9, 178-184.

Satter, E. (2000). Child of Mine, Feeding with Love and Good Sense: Colorado: Bull Publishing. www.ellynsatter.com www.

infantandtoddlerforum.org

5

Managing TOF Mealtimes - Manic or Mellow?By Cathy Birchmore, Specialist Speech and Language Therapist

It’s helpful to consider the Division of Responsibility in more detail. The child’s responsibilities are self-explanatory: they have control over how much and whether to eat – even if that means nothing or very little at some meals. The tricky bit is learning about your own responsibilities and setting limits so that you and your child stick to your own side of the division. Here are some more tips on the parent’s responsibilities:

• You choose appropriate balanced meals, cook and present the food to your child.

• You provide as much help as your child needs (spoon-feeding, cutting food up - whatever is developmentally and TOF/OA appropriate) and no more.• You choose the when of meal times; for a toddler this means offering regular snacks as well as meals, but not allowing free access to food outside mealtimes. It also means that you’re responsible for setting rules such as coming to the table and only leaving when they have permission. For older children this means setting reasonable limits (‘Get out of the cupboard, dinner’s in half an hour’), but also gradually allowing them to make some food choices themselves, again trusting that they will self-regulate their appetite.

• You provide a good role model for healthy eating within the family. This is hugely important. Your child learns so much from you, including food habits. As Satter explains: if your child sees you eating and enjoying green beans, then it must be the thing to do. You don’t need to do or say anything else; watching you is lesson enough. It doesn’t mean that your child will instantly devour green beans, but your attitude allows for the possibility in future; ‘you don’t want to eat green beans now, but you’ll probably like them when you’re a bit older’. And a word about what it doesn’t mean: it doesn’t mean a puritanical diet excluding ‘naughty’ foods. All food is good food, and we all enjoy sweets, snacks and junk food sometimes. It’s about enjoying food and providing a balanced diet including these foods in moderation too.

• You provide a pleasant and relaxed mealtime. If possible - and I appreciate that lifestyles don’t always allow for it every day - eat with your child at the table with the TV off. This has the advantage of letting you concentrate on enjoying your food, providing a good role model, and taking the focus of attention off the child (who ever did enjoy being watched when eating?). It also allows you to enjoy the time with your child and family; you know the saying: the family that eats together, stays together.

• You need to be clear about your family’s rules for mealtimes. This doesn’t mean you have to run a boot camp, just be clear about expectations for behaviour and stick to them. One family I know had their 8 year old write up their agreed rules (pictured). Do they rigidly stick to them? Of course not, but in general they do, and when necessary the family return to them and clarify the Division of Responsibility.

So relax: even as a TOF parent, once you’ve chosen and prepared appropriate foods and can present it in a supportive and positive way, your job is done. It really isn’t your job to get food into your child. That’s her job.I would recommend Ellyn Satter’s books and website to all families regardless of whether their children have TOF/OA or any other special need. And I hope you and your family have many happy, tasty mealtimes together.

Editor’s note: Special thanks to Cathy for writing this article and for her contribution at our Durham children’s party in October. Sadly, we recognise the challenges in maintaining clear boundaries in this Division of Responsibility! We know that weaning in particular can be a major cause of concern for many TOF parents and that some of our TOF/OA children continue to be gastrostomy-fed. It’s impossible to do a ‘one size fits all’ article; please do contact your

6

Mr Singh is a Consultant Paediatric Surgeon and Service Lead for Children’s Surgery and Critical Care at the University Hospital, Nottingham.

Please tell me about your experience with TOFs, beginning with your first TOF operationMy experience of Tracheo-Oesophageal Fistula and Oesophageal Atresia (TOF/OA) goes back to India. Prior to 1994, I worked in the Post Graduate Institute of Medical Education and Research (PGIMER) in Chandigarh (Punjab) India. This institute cared for a population almost as great as the entirety of Great Britain! In Nottingham we see 6 to 8 children born each year with TOF/OA whilst at PGIMER I would often repair 2 or 3 in one night. In 1993, I performed my first OA/TOF repair in PGIMER as a senior trainee. In those days the post operative care was left to nurses who were not fully trained in neonatology. The survival was dependent on postoperative care which was up to the operative surgeon to provide or organise. I remember sitting at the bedside of this baby for over a week performing routine suctioning and other related cares. When the child was discharged on the tenth postoperative day the watch keeper outside the neonatal unit congratulated me thinking that I had been looking after my own child. He was amazed when I told him that the child was my patient. Peri-operative nursing care is vital to outcome and I was amazed when I came to the UK at the level of training and excellence of the Neonatal Nursing Teams.

Are there any Parent Support Groups in India?Unfortunately, there are no support groups in India.

When did you come to work in England?I came to England as part of the Overseas Doctors Training Scheme in 1994. Prior to this I had reached the role of Assistant Professor in PGIMER which is equivalent to the status of a UK Consultant. I worked in London, Kuwait and Sydney before being appointed as a Consultant in Nottingham and taking over the role of Miss Leela Kapila (past president of the British Association of Paediatric Surgeons) on her retirement. During the 10 years that I have been a Consultant I have taken a one year sabbatical to work as a Consultant (Attending) in Washington DC in 2009-2010.

Do you see a difference in the operation in other countries?I have seen the TOF/OA operation in 5 countries, over 4 continents.In India, the technique is the same but the mortality is higher: Approximately 75% used to die, and 25% would survive (I am talking about the figures in 1990s). The chances of survival were related to the length of time the child was operated on; if this took more than one and a half hours, the mortality rate increased because the chest remained open in less than ideal operating theatre conditions. The second factor is that post-operative care was not as good as it is in the West. If there were any post-operative problems such as a leak, there was no opportunity to take the child back to surgery.

Interview With Mr Shalinder Jit Singh Ms Dnb Mch Frcs (I) Frcs (Eng) Frcs (Paed) Dm MbaWhat would you say about the mortality rate nowadays in England?These days, due to the improvements in neonatal care and operative equipment, the TOF/OA repair has less bearing on the overall mortality compared to other factors such as prematurity, associated heart defects and syndromes or associations such as Edward’s or CHARGE which accounts for the wide variation in published survival figures. In my experience, almost every baby born with TOF/OA without any of the above mentioned associations survives.

Do you know how many TOF/OA operations you have carried out?In England, I only carry out 2 or 3 a year, because there are other Paediatric Surgeons in my institution who carry out the remainder. The majority of my experience I have brought from India where I probably carried out 60 or 70 in 3 years and Australia where I performed approximately 20. Overall, I have probably repaired slightly more than 100.

How do you approach parents once a diagnosis has been made, and following the operation?It is important to first confirm the diagnosis, through physical examination or an X-ray. This is particularly important if an ante-natal diagnosis has been made. Once that is done, I meet with the parents and explain the anatomical abnormality to them, using layman’s language and simple line drawings. At this stage, I don’t talk to them too much about the medium and long-term period because it is too much for the parents to take in, so I only talk about the technique and any difficulties which may arise during the operation and in the immediate postoperative period. My discussion with the parents includes the nurses who will be looking after the baby as I need to ensure that they also understand so that they can support the parents. Because the nurses will have dealt with previous TOF/OA babies, they will be in a position to relate stories of success to the parents. Following the operation I sit with the parents again and discuss the operative findings and plan for the postoperative period. I go through the operation with line diagrams again and make them aware of common postoperative complications. I must say I am a half glass full type of person.

Have you noticed an increase/decrease in the number of children born with TOF/OA since you first started operating? I have not observed a large change in the number of cases presenting to Nottingham.

Do you see an increase in the number of ante-natal diagnoses?Since the introduction of routine ultrasound scanning in pregnancy in the 1990’s we have seen an increase in antenatal diagnosis for which we run an Ante-Natal Counselling Clinic where parents can meet with a Paediatric Surgeon to discuss the results. However, there is no specific test for TOF/OA but it is suggested in 50% of cases with polyhydramnios (excess fluid) and absent stomach bubble on ultrasound scan due to a blocked oesophagus.

Antenatal diagnosis is useful. It highlights babies that may potentially have problems and allows parents to become mentally prepared.

Thanks to those who joined us at the TOFS North East meet in Durham in early October. Although we were a small group, we had a

great afternoon. The children were entertained by a magician while we parents shared stories and experiences. It was great as there were families there with a range of experiences – some with young babies while others with older TOFs, Some eating well, some still plodding on….it’s always good to hear others’ struggles and successes….makes your own experiences feel so much more normal! A big thank-you also to Caroline Love (Adult TOF and TOFS trustee) and Cathy Birchmore, Speech and Language Therapist who we had the chance to chat to and quiz with all of our queries and questions!

All in all, it was a fantastic afternoon. Hopefully we can run a similar event next year where more TOF

families will be able to join us – thanks TOFS as always for your support!

Angela Angela Carter TOFS NE TLC

Editor’s note: Would you like a TOFS event in your area in 2013? With a limited budget we’re currently debating where best to run our parties so the optimum number of TOF families can benefit.

We’ve already secured funding for a party in Nottinghamshire – could you do the same for your area? If you can target a potential funder, between us we can put a proposal together to make that party happen – near you! Drop me an email or give us a buzz at 0115 961 3092.

TOFS Nor th -East par ty

7

Interview With Mr Shalinder Jit Singh Ms Dnb Mch Frcs (I) Frcs (Eng) Frcs (Paed) Dm MbaDo you see many TOF children with additional anomalies, and what are these?In my experience, I would say 2/3rds of children have predominantly uncomplicated Tracheo-Oesophageal Fistula and Oesophageal Atresia (ie they do not have any significant associated malformations), and the remaining third have other significant abnormalities which could be renal, other atresias, or there may be a syndrome, or the VACTERL spectrum.

What advice do you provide for parents when the child is discharged from hospital?When a baby leaves hospital with its parents, we arrange for an appointment in 3-4 weeks’ time and then if all is well, they are brought back every few months after which review intervals increase with age. We aim to follow children until they are 18 years old when they will be discharged with long term advice or, if there are persistent problems their care transitioned to our adult colleagues.

At what point should parents make contact with their hospital if they suspect there is a problem?We tell them frankly that, if there is a problem with breathing or swallowing then they should contact us immediately especially in the first 6 months to one year. Parents contact our team then dependent on need we can see them immediately through the Emergency Department, the following day as a ward review or we can bring forward an outpatient appointment.

Are children discharged back to their own local hospitals?The TOF/OA operation is only carried out at large specialised Paediatric Surgery Centres like Nottingham. Once recovered from surgery it may be possible to transfer children closer to home for ongoing care of other issues.

For the past 10 years, we have worked on a “hub and spoke” model, where our Paediatric Surgeons hold outreach clinics at peripheral hospitals to save parents and children the long commute to Nottingham for reviews.

Do you run a multi-disciplinary clinic for children who have other anomalies?Due to the relatively small number of children presenting with this condition it is not possible to run a clinic solely for TOF/OA. However, those with associated complex spine, bowel and bladder problems are seen in a combined clinic for these problems and we aim for all children born with TOF/OA to be seen by a Respiratory Consultant as we feel it is important that the children are examined thoroughly for any respiratory diseases.

Communication amongst different teams and families is paramount and to this effect we copy correspondence letters to parents/guardians as well as other clinicians.

Do you advise that, once children reach 18, they are followed up by Adult Consultants?We suggest that children will need life long review to monitor for problems with the oesophagus but may not need further intervention.

This poses a problem as most General Surgeons / Gasteroenterologists have limited or no experience with rare Paediatric congenital conditions. A better system for transitioning care from the paediatric to adult services is something that we need to work towards. Ideally this transition period would run from approximately 16 to 25 years. However, this would be untenable in every Centre, but could be achieved in a smaller number of supra-regional centres throughout the UK in future.

Overall, the UK holds a privileged position in the world as it provides first-class Primary and Secondary care services which are free at the point of entry.

Christine Shepherd

8

Cover StoryCover Story Call it female intuition or divine intervention, but if Emma had not been so

insistent in returning that morning, we’d have been looking a completely different and more tragic scenario. Emma’s specialist took one look at the screen, seeing that the placenta had completely detached itself from the womb and that the baby was lying motionless he exclaimed; “I’m very sorry, but we need to get him out right now!”

Emma was prepped immediately for an emergency caesarean. It went well and Jack Giles Johnson arrived at 35 weeks weighing just 1.8Kg. After he received the customary first cuddles he was whisked off to the neo-natal care unit in his incubator.

The next morning we began to meet all the amazing doctors and nurses in the neo-natal unit and to familiarize ourselves with the unit and their procedures and practices. Fortunately we had received a tour a couple of weeks before but it still felt very strange to be there for real. Next we met our surgeon, Mr Keith Holmes, who would be operating on Jack. He explained his plans and the next day a tube was fitted directly into Jack’s stomach for feeding and another into the pouch of his incomplete oesophagus.

At this point Mr Holmes realized that the gap between Jack’s oesophagus and his stomach was a medium size and not a small gap, as he had hoped. Therefore Jack would need to remain in the neonatal unit for some time until he was big enough for further surgery. This would also allow him to grow and become stronger. During this time he was nil by mouth and was fed

using a Gastrostomy tube directly into his tummy. This made the practical things like changing, washing and bathing much more difficult. Jack also delighted in repeatedly pulling his replogle tube out at every possible opportunity. This then needed to be reinserted by the duty registrar or Mr Holmes himself.

Looking back, the biggest psychological impact on us as parents was probably the fact that on the day of Emma’s discharge, our baby remained in hospital. Nobody prepares you for that and it’s a very difficult experience to get to grips with. Being born unable to swallow also meant that Emma was unable to bond with Jack through traditional breast feeding and to do all the other things you learn about in NCT classes, see or hear about from others. However you don’t really have time to think about it at the time, as you exist in a state of limbo harbouring

high levels of dread and uncertainty of just what might lie ahead.

We made our daily trips to the hospital in shifts, trying to spread the care load. Luckily Tooting was a relatively short hour’s drive but we still needed to plan well ahead to avoid peak hours and traffic. Our families were especially amazing and enormously supportive.

Finally, the day come for the surgical team to repair his oesophagus, which unfortunately wasn’t as straight forward as we had hoped. It turned out that Jack had two fistulae that Mr Holmes repaired, but he was unable to repair the oesophagus as the gap was still too large, due to a previously withered part of the oesophagus that they had not seen before. Mr Holmes chose to do the Foker technique, pioneered in the USA. At the time it was not a typical procedure in UK hospitals and had not been successfully completed in the UK before. However it was felt that it offered the best option for Jack, as it is designed to encourage growth at the two ends of the oesophagus through stretching, thus making a better join possible.

The Foker technique meant that Jack was then placed in coma with the two ends of his oesophagus under tension. This tension then needed to be maintained for a week, gradually being tightened daily by the surgeon using

In our own words… Jack’s story

We were delighted when Emma fell pregnant shortly after we were married. The pregnancy was going well. We had our 20 week scan at our local hospital in Kingston. Everything looked great and

we went away incredibly excited. Shortly after the scan Emma felt a little concerned and she wasn’t feeling the baby moving very much and Emma went into hospital to be checked over. The midwife remarked that she looked quite big for her dates and suggested a scan the next day.

It was during this scan that they told us that Emma had Polyhydramnios and it was highly probable that our baby would be born with something called Oesophageal Atresia as there was no obvious stomach bubble to be seen. We were immediately referred to St George’s in Tooting, where their specialists confirmed that our baby was unable to swallow and confirmed he had Oesophageal Atresia.

Even now we still shudder remembering how we were ushered into ‘that room’ where you are always taken to discuss the fate of your unborn child. Though the content of the meeting is a blur we can recall how we met

with a surgeon who explained that when our baby was born it would face an uncertain future and certain lifesaving surgery. He went onto explain that there was no way of knowing the severity of the condition or eventual prognosis. He also warned that there might also be other problems, drawing a series of diagrams and using acronyms like TOF, OA and VACTERL and was very matter-of-fact. We tried to take it all in, but it was a huge shock and we were obviously devastated. One of his assistants gave us a TOFS flyer with the details of the website and we returned home that night in a daze but determined to understand what to expect.

From that point on, we used the TOFS site and started reading forum posts. We also ordered the TOF book and tried to understand what might or might not come to be. It was a very worrying time, but we tried to remain positive and focus on practical tasks while hoping for the best possible scenario.

During this time we moved from our flat to a new family home. On the weekend we picked up the keys Emma was concerned that she could not feel very much movement at all and was taken to St George’s to be monitored. The weekend staff confirmed there was a heartbeat but Emma was still worried, so she returned the following morning determined to get the opinion of a specialist.

9

Cover Storytiny anchors on the outside of Jack’s body. Initially all went to plan, however it was heart-breaking to see our tiny son in such a terrible way as he remained ventilated and motionless covered in tubes and wires.

Then suddenly one morning, there was a problem. The tension had released and Jack was in real trouble. He was immediately taken to theatre. Amazingly after another nine hour operation, the team were finally able to join his oesophagus and stomach together.

Mr Holmes came to see us immediately after theatre. He was exhausted, relived and elated that it had worked but very unsure that it would stay joined. Again Jack remained in an induced coma for 10 days to give his tiny body the best opportunity to heal itself and the precious oesophagus to join.

During these days it was extremely difficult for us all and we relied very heavily on our families, friends and the amazing doctors and nurses of the intensive care unit. The other parents on the ward were also extremely supportive, providing practical support and the feeling of reassurance you get from the sense of a shared experience.

Finally, Jack was allowed to wake but remained poorly for some time as various infections were first identified then fought off with medication. Jack was obviously a fighter and he soon started to make a remarkable recovery. Mr Holmes still finds it hard to believe that Jack made such a great recovery as the join was so tight. However, Jack went from strength to strength and he finally left hospital for the first time at the age of four months, drinking milk.

Those first few months at home and the following early years were extremely difficult. Like all babies born with his condition, once repaired, Jack suffered extreme reflux. Feeding was a challenge with many feeds apparently lost with reflux after only a short period of digestion. It was often frustrating and extremely exhausting.

There followed a number of inspections and stretches of the repair area but no significant narrowing was ever seen. Though peristalsis was obviously absent in the repair area during tests, Jack quickly moved onto solids with only a limited number of ‘stickies’ or problems swallowing. His reflux medication was and remains relatively low doses of Domperidone and Ranitidine.

Jack’s biggest health issues in the early years actually related to his right lung, as it was damaged when it was collapsed for surgery. He had physiotherapy but it did not ever recover and his mid-right lung is permanently collapsed. It was only detected later on and together with reflux this weakness in his chest made him especially susceptible to infections. As a toddler he seemed to get every cold and virus going which often resulted in him ending up in the local hospital with a course of super strong antibiotics and a nebulizer.

We tried all the tricks and tips shared by other parents on the TOFS forums and took every opportunity to attend the dedicated TOFS conferences where we were able to meet other parents and families like us.

In the end, we found that we could best manage the habitual winter coughs and resulting reflux by ensuring Jack always had hot steamy showers, slept elevated and ate early enough in the evening to limit the effect of reflux. He was also diagnosed with sinusitis and therefore still has a nasal spray last thing at night, has his teddy bears frozen regularly to kill dust mites and we do our best to limit dust in his room. What’s more, he is now able to blow his own nose so that limits the amount of liquid that can find its way down his throat and cause him to cough.

The great thing was, when he reached five years old, he seemed to become a lot stronger and more able to fight off the effects of common viruses. And by his sixth birthday his chest specialist (Mr Ian Balfour-Lynn at The Royal Brompton) was so happy with his progress that he ‘officially’ discharged him.

Jack continues to make enormous progress; he is able to eat almost as well as anyone else. He is incredibly small for his age and very slim and it’s often difficult to find clothes that fit him. Although he still faces some challenges you’d never know it to look at him and he continues to amaze and enchant everyone who meets him.

David Johnson, Pyrford near Woking, Surrey

Editor’s note: Many thanks to David and Emma for sharing Jack’s story with us. Read more about this remarkable little boy’s fundraising efforts on page12.

AdvertisementShare experiences and hear from the experts

At ourTOFS Family Conference 2013

OnSaturday 12 October 2013 (full day)

At:Holiday Inn, Coventry,

M6 Jct 2, CV2 2HP(Good transport links)

Speakers include: Mr Joe Curry, GOSH, Mr Shalinder Singh, QMC Nottingham, Mr Charles Shaw-Smith, Royal Devon and Exeter

Informal workshops*covering Feeding issues; Learning Difficulties; Genetics

*full list to be confirmed Ofsted-approved Crèche available

(£25 per child)

Number of children are limited so please book early.

Make a weekend of it with other TOF families - book your accommodation

directly with the hotel.

TOF-friendly lunchEarly-bird rate includes all

refreshments and lunch Only £25 per person

Once full details are confirmed a webpage will be available to take

bookings. We will email you whenit’s ready. In the meantime register

your interest by email:info@tofs.org.uk

10

FundraisingJanuaryEllen Fretter 04/01/1997Abigail Gillespie 06/01/1997Colette Wylie 25/01/1998Samantha Berk 31/01/2006Jamie Rimmer 03/01/2006Jessica Lindley 31/01/2005Ariana Fischer 06/01/2007Laura Strain 18/01/1999Amber Campbell 12/01/1999Mason Webster 21/01/1997Molly Slack 15/01/2000Lydia Blackburn 09/01/2000Ben Taylor 19/01/2000Laura Jackson 04/01/2001Ibrahim Meziane 16/01/2001Niamh Kettleborough 17/01/2003Jack Stewart 03/01/2002Daniel Agar 30/01/2004Gagan Panesar 30/01/2004Ryan Fido 28/01/2004Sean Keith 19/01/2004Daisy Marshall 03/01/2005Olivia Medd 20/01/2005Liam Buisson 17/01/2005Charlie Lashmar 11/01/2005Grace Dorsey 07/01/2005Jack Dillon 30/01/2006Terry Stuart 22/01/2007Laurie Shepherd 03/01/2007Jenson Carter 13/01/2007Chloe Dale 07/01/2003 FebruaryBenjamin Evans 06/02/2001Tyler Willis 13/02/1998Adam Michael 03/02/1998Oliver Rickards 08/02/2006Suraiya Mirza 21/02/2006Freddie Stevenson 17/02/2006Benjamin Platten 02/02/1998Lydia Cooper 03/02/1999

Oliver Hatton 18/02/2000Rebbecca Slator 07/02/2001Holly West 27/02/2002Alicia Godwin 25/02/2002Owain Roberts 07/02/1999

Ned Melland 21/02/2003Sacha Kobovich 17/02/2003Kinsley Martinez 23/02/2005Thomas Gluyas 10/02/2005Gabriella Greenwood 22/02/2007Eilidh Lowe 14/02/2007Joshua Smith 03/02/2006Edward Bridges-Pugh 24/02/2007

MarchPippa Hynes 08/03/1997Amy Howes 23/03/1997March Hollingbery 08/03/1997Jack Mullens 12/03/1997Ben Smith 03/03/2004Tobias Jones 25/03/2006Lukas Weber 09/03/2003David Malcolm 11/03/1999Daniel Sandford 28/03/2000Olympios Gougoulias 06/03/2002Georgia Jenkin 23/03/1998Grace Walsh 06/03/2003James Rogers 26/03/2003James Flynn 24/03/2004Ryan Lowe 09/03/2004Lewie Herbert 05/03/2004Grace Nolan 06/03/2005Isabelle Maude 31/03/2005Lauren Hulligan 07/03/2001Lauren Naylor 21/03/2003Tiago Gudge 14/03/2007Amy Vallis-Brown 15/03/2007Francesca Watkins 13/03/2007Katie-Belle Calladine 02/03/2007Efa Mai Davies 02/03/2007

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

Thank you to:• Republic (Grimsby) – £35.00 Collection box donation.

• Claire Burnside, mum to Olivia born with OA, - £100 donation.

• Shire Oak News & Off Licence customers - £50 shaker box donation. Our thanks also go to Sylvia Docker (grandmother to Jayden Sharp born with VACTERL) for putting our name forward.

• Barry Waite - £10 donation.

• ‘Country Baskets’ in Stourbridge - £20.00 shaker box donation.

• The Explorer Scouts in Kent very kindly made 30 beautiful birthday cards and donated them to TOFS. The group is run by Nicola Mallett, mum to Christopher born with TOF. (We have sent these cards to TOFS volunteer Craig Burley who sends greeting cards to all our youngsters under the age of six.)

• Stockwood Free Church in Bristol - £150.00 donation. Thanks to Renate Nickolay-Lilley, mum to Peter born with OA, for putting our name forward.

• High Wycombe Lions Club in High Wycombe - £100 donation.

• Autologic in Somerset held a ‘Dress Down’ day and donated £159.00 to TOFS.

• John Pearce - £50.00 CAF donation.

• Judith Bland - £20.00 donation in lieu of sending Christmas cards.

• Margaret Hayton, mum to John born with TOF - £20.00 donation.

• Joy Howell, grandparent to Colin Ryan born with TOF - £20.00 donation in lieu of sending Christmas cards.

• Wally & Sheila Slater – Parents of Adult TOF trustee Graham - £50 donation in lieu of sending Christmas cards.

Read about some of our other fundraisers in separate articles in this Chew!In memory of:• The late Mrs Elsie Ayling who left a legacy of £100 to TOFS.

• The late Stan Perry, Great uncle to Isobel Cattle born with VACTERL - £115.00 donation from work colleagues at ‘Kerry Foods’.200 Club Winners:• October - Katrina Tyler from Shropshire (Katrina very kindly donated her winnings back to TOFS).

• November – Hazel Button from Kent.

• December – Rebekah January from County Antrim.

Thanks to members of the TOFS Council of Management who kindly donated some or all of their expenses back to TOFS

Mum and dad Robert and Janine Meredith

donated £105 on behalf of the Fraud Response Team at First Direct in Leeds. Their daughter, Isabella (TOF/OA), was born in August 2011. They wrote: ‘We are members of TOFS as our daughter Isabella was born with TOFS last year.

At First Direct we will continue to fund raise for TOFS. The money we have donated this time was raised through a bake sale we had at work. Last year we raised money for a different cause by walking the Yorkshire 3 peaks and raised over £1500 and we hope to complete

something for TOFS towards the beginning of next year. Until then we will continue to hold raffles and bake sales and similar smaller events.’

Editor’s note: Does the company you work for make charitable donations? Please ask, and find out if it is possible to nominate TOFS. Or if you work at a racecourse or other major sporting venue like a golf club or big shopping centre perhaps you could nominate TOFS for a collection? We’d be very happy to approach them on your behalf, or help with the nomination, just get in touch with the office and we could work together on it.

F i r s t D i r e c t t e a m d i r e c t t h e i r f u n d r a i s i n g t o T O F S

Fundraising

11

FundraisingSimon skydives for TOFS!Simon Mann, from South Shields, Tyne

& Wear, bravely took to the skies recently to raise £430 for TOFS.

Simon’s sister is TOFS TLC Angela Carter.

He says, “I decided to do a sponsored Skydive for TOFS because my nephew Jenson Carter was born in January 2007 with a long gap OA and a serious heart abnormality. I have never done anything for charity before and thought I should contribute something to TOFS for the help and support they have given to my sister and her family through their difficult times. Jenson is 5 years old now and still has eating problems but is growing stronger every day.”

Fundraising

Jane Cannetti, mum to 10 year old Phoebe (OA) from

Sheffield, raised £130 for TOFS by completing the Great Yorkshire Run. She writes, “My nephew Adam and I completed the 10k run early last September. It was held in Sheffield on a very hot day. I was hoping to complete it in under 1 hour but only managed 1 hour, 1min and 13 seconds. Adam managed it in 56 minutes. It was hard work but knowing I was doing it for a good cause helped me along the way.”

Well done Adam and Jane!

Fundraising for TOFS isn’t just limited to the immediate family in the Johnson

household. As well as cover-boy Jack’s Scootathon, his uncle Andrew Johnson, from London, raised £1197.50 for TOFS on a fantastic 35-mile charity walk over one weekend on the Avon Valley Path from Wiltshire to Dorset.

Andrew played down the difficulty of the challenge before-hand but it turned out the ‘Path’ is a bit of a red herring – mud, stinging nettles, bogs, busy roads and barbed wire fences all played their part in making the ‘walk’ one to remember! He and five fellow-walkers set off from Salisbury and spent the first

night at Ringwood in the New Forest, before walking the final 17 miles to Christchurch. However Andrew got huge blisters and was in absolute agony, and ended up having to visit the doctors for a course of antibiotics!

Apparently, it’s par for the course if you do very little training and buy a new pair of shoes a few days before! Of the six walkers who started, only five made it to the end. One of them sank up to her waist on the River Avon floodplain and had to abandon the walk! A very big thank you to Andrew for this amazing effort and to everyone who helped by sponsoring him.

Andrew, (far right) with his fellow walkers at Christchurch Priory

And there’s more…Michael Craig from Nailsea,

Bristol, writes to tell us of his intrepid 50-mile

walk in the Mendips (over 2 days) to fundraise for TOFS. Michael is married to a healthy Adult TOF and arranged the walk to coincide with his 50th, raising £320. He writes:

‘The photo is of the intrepid three, boldly going where my boots have feared to tread, through mud, hedges and steep hills and

changeable weather conditions. 50 miles of charted territory over 2 days, for a man of my experience in walking a sometimes impossible task, but, thanks to my 2 keen walkers I managed to accomplish this feat with my feet. I think I might do something a little different for my 60th.’

Belated birthday wishes Michael, thanks for thinking of TOFS!

50 miles for a fit 50 year old

12

Didn’t they do wellDidn’t they do well

Jack Johnson, aged six, recently participated in the HSBC Scootathlon at the Eton Dorney Olympic venue. Thanks to wonderful family

and friends, Jack (whose story is featured on page3) has raised an amazing £2258.50 for TOFS.

A Scootathlon is a fun mini-triathlon for children 4-8 years old. The event features a one lap scoot of the 390m track, before a transition, where the children all put down their scooters and pick up a bike to cycle the same 390m lap. Then they go back into transition for the last time, leaving the bike next to the scooter and set out for the final 250m run to the finish.

Jack’s first priority was to learn to ride his new bike so he even joined a local Guildford cycling club, to help prepare better for the event. He also spent the summer improving his scooting and being able to switch from scooter to bike. Mum Emma tells us about the day of the event:

“On the day of the Scootathlon we all got up early and headed out to Eton Dorney, near Windsor, which had been the scene of the Olympic Rowing and Canoeing events a few weeks earlier. Jack was very excited and taking it all very seriously.

The older children went first, so it was a chance to see how it should be done. Some of them

performed like little professionals, especially in the transitions, when swapping from scooter to bike. There were also a few accidents as it was quite chaotic with bikes and scooters flying left and right. Undaunted, though looking a little apprehensive, Jack lined up for his race on his scooter. However once the race starter sounded the horn everyone forgot any concerns as the adrenalin took over.

Jack made a great start and held his own in the one lap scoot, before arriving at the transition area and switching over to his bike. Cycling is his strongest discipline and after avoiding the other cyclists he left the transition area and started what was a great ride. He even managed a few overtakes!

Then it was the time for the run. It’s fair to say Jack ran his little heart out trying so very hard and managed to cross the line still smiling but totally exhausted. Like all the other competitors, he received an Olympic Style Gold medal and left the event a great little champion!

Money is still coming in but Jack has so far raised over two thousand pounds for TOFS. We have been completely overwhelmed by the response of all our friends, neighbours and family and cannot thank them enough for all their messages of support and generosity. We hope that Jack’s

efforts have gone some way to help highlight this very rare condition and the work of the TOFS charity.”

Indeed they have! Jack’s an inspiration. A huge thank from TOFS.

Cover-boy Jack raises thousands for TOFS

Jack crosses the finish line.

Monday 17th December was the scene of much excitement in the TOFS

office as we were to draw the winner of TOFS Fantastic Flyaway raffle. The Mayor of Gedling kindly agreed to draw the winner, witnessed by trustees Sue Lewis-Jones, Wendy Bell and Graham Slater. We were delighted also to welcome TOFS president Christine Shepherd. We’d borrowed a huge drum especially for the occasion as so many tickets had been sold and we wanted to make sure they’d had a really good mix-up. The winner was contacted and announced on our website that same day.

41 of you (plus several trustees) took up the challenge of selling tickets on our behalf. The list’s too long to include here but the names are on the website. We know what a task this can be so special thanks to you all. Top sellers were Brian Ayre, Redcar, and Shona Sneddon, Dundee. They were closely followed by Katie Burrell, Julie Logan, Christine Shepherd, Howard Sharman, Kay Leech, Andrew Cowling, Jo Oakley, Claire Stephens and Claire Stewart. Not forgetting those of you who bought directly from our webpage, and spreading

the word, forwarding the link to friends and family. Finally, those of you who bought tickets at our Adult Conference.

Special thanks must go to trustee (and chief ticket folder!) Wendy Bell for getting the prize in the first instance and selling furiously thereafter!

So how do we follow this in 2013? Could you help us source the main prize? We managed to get this prize in the first instance because trustee Wendy Bell works for Virgin Atlantic and nominated TOFS as a charity in their monthly draw which is part of their community investment project. Does the company you work for run similar charity draws? If so, can you nominate TOFS to win? As well as the main prize this year we’d like to offer smaller prizes. Vouchers would be great as they’re easy to post.

Or would you be able to help us by getting involved with the administrative tasks of running the raffle. We’d like to set up a small committee to oversee it in 2013? You needn’t be close geographically to the TOFS office as we could do everything remotely. Please email if you’d like to know more.

thank you for your support.Congratulations to Adult TOF Judith Rennie from Marlborough in Wiltshire, winner of our Fantastic Flyaway Raffle!

TOFS Fantastic Flyaway Raffle Raises over £3,000

13

In October 2010, the first ever International medical conference on TOF and OA was held in Lille, France. We at TOFS saw this as a great

chance to engage with the medical community, and took part in that event, even giving a presentation to the whole conference. We learned some useful things from it as well as an article in Winter 2011-12 Chew showed.

We have subsequently joined with Europe’s 3 other leading TOF/OA support groups to form EAT, mainly as a vehicle for better engagement with leading medical authorities.

The second International Oesophageal Atresia symposium took place in Montreal in October 2012, and the TOFS COM again felt that we/EAT really should be there. TOFS Trustee Graham Slater represented both TOFS and EAT: he is EAT’s Chairman. EAT Vice-Chair JoAnne Fruithof (from The Netherlands), Philipp Schwabl (from Austria) and representatives from the French and Italian support groups went along too. Graham and JoAnne made a presentation to the whole conference on behalf of EAT; A Canadian parent of a TOF child and a Canadian Adult TOF who has run several marathons also gave presentations.

More than 200 specialists from around the world and from different areas of medicine, participated over two days to exchange and present the results of the latest research in developmental biology, paediatric surgery, the management of reflux, stenosis and other problems associated with OA. These included presentations on the psychological impact of OA on parents and patients.

Besides the expert audience, several TOF people as well as parents and representatives of support groups attended the conference.

The conference showed that over the last few years considerable progress has been made, especially in areas such as keyhole surgery and the diagnosis and treatment of reflux, but no international consensus so far yet exists in a number of other key areas. For example: what is the definition of ‘long-gap’ OA?; what is the most appropriate surgical technique? (e.g.: thoracoscopy vs. thoracotomy); what is ‘best-practice’ in post-surgical care and longer-

term after-care? A number of these topics were vigorously debated during the ‘Questions & Discussion’ sections which followed each presentation.

The medics very much agreed that support groups like TOFS are a key element of the treatment and care of patients with OA.

It also emerged that a number of issues which have been worrying the OA Support Groups are being addressed in certain parts of the world: for example the allocation of psychologists into paediatric after-care clinics, and the establishment of Adult TOF clinics at a number of centres including Montreal itself.

In Montreal, EAT was able to raise its profile and demonstrate its strength in developing Europe-wide, indeed world-wide, networking amongst its member support groups and also in its engagement with the medical community.Recently, a new support group has been formed in Italy, and there are hopes that one will soon be founded in Poland, supported by the enthusiasm of two leading Polish paediatricians who were present in Montreal. In addition, there were representatives of AQAO, the support group based in the Quebec province of Canada, and OARA from Australia. Both these groups are now associated with EAT.

At Montreal, EAT had its own exhibition stand with information and publications available in the different languages of its various member associations. This reinforced EAT’s presence and demonstrated the importance of the support groups and their collaboration.

Despite a crowded agenda, there was still time for exciting discussions and animated exchange between those present. The atmosphere was very positive and many important contacts were established.

Most importantly, EAT is becoming far more effective as a means to get the healthcare professionals to take real notice of support groups like TOFS and our partners.During the presentation given by Graham and JoAnne, EAT had the special honour of announcing that there will be a 3rd International OA symposium

to be held in Rotterdam, The Netherlands, on the 2nd and 3rd of October 2014. (provisional dates). More importantly, EAT has also been asked to be a member of the organizing committee of this symposium. This role will be carried out by Vice-Chair, JoAnne Fruithof, who lives in the Netherlands. So TOFS can now influence (via EAT) what the doctors talk about at the World’s major conference on TOF/OA. That’s quite a result!

What’s more, the medical community announced that it has now decided to form its own international OA working group which will address five areas: Genetics & Basic Science; Surgery; Gastro-enterology; Pulmonary; and Psychology & Quality of Life. This working group intends to make a significant contribution to the 3rd International OA symposium in October 2014. This group, of course, also has the potential to provide another opportunity for EAT to collaborate with the medical profession.

Meanwhile EAT also intends to quickly develop its web-site ( www.we-are-eat.eu ), to help new support groups to get established, and, if possible, commission a study into the lives of Adult TOFs - a subject in which, as yet, there is world-wide relatively little information that healthcare professionals can use.

In summary, the congress gave a clear signal that many well-motivated people are pulling together in the same direction, in order to improve things for TOF people. EAT, TOFS and the other individual support groups are being increasingly seen as partners of the medical community, which is really starting to take our views, and most importantly the needs of TOF people , seriously.

Note: This article is based on a report written by adult TOFs Graham Slater and Philipp Schwabl, with translation help from Paula Grolig and Rebecca Supper. Philipp will qualify as a medical doctor in early 2013. The synopses of all presentations made at the symposium in Montreal are available on-line at: http://onlinelibrary.wiley.com/doi/10.1111/j.1442-2050.2012.01430.x/abstractThe actual presentation slides are available on-line at:http://www.eamontreal2012.com/152444864164631674814163526321563.php

Meeting with the Medics in MontrealDidn’t they do well

Norbert Zsiros and Dr Anna Sós from Békéscsaba in Hungary sent us these pics

of their gorgeous daughter Lili. We were pleased to have been able to put a Hungarian family living in the UK in touch with the Zsiros family.

Lovely Lili was born with OA in August 2011. Seems she’s always keen to find out what her contemporaries have been up to in Chew!

Editor’s note: If, like Lili, you live outside the UK and would like to receive a hard copy of Chew, instead of an emailed version, please let us know and we’ll arrange for it to be posted to you.

F a m i l y u p d a t eAdvertisement

Training in basic life support available

MoonrakerContact:

Marijke van Eerd Qualified A&E children’s nurse

2hr session £15 per person

Tel: 07980 615092e-mail: marijkevaneerd@hotmail.com

14

Adult Tofs

Phil Radford was born in 1965 at the Women’s Hospital, Peel Street, Nottingham. His

condition was described to his parents in simple terms and the term “Deflated Oesophagus” was used as an explanation for the condition. But all the time he was growing up, and through most of his adult life, Phil himself never really had a name to use to describe his various issues!

This changed when he read an article in the Nottingham Evening Post last summer written by Sue Lewis-Jones to promote TOFS and to advertise her Coffee Morning. The article prompted him to call Sue to see if he was indeed born with TOF/OA.

At Sue’s coffee morning, Phil remembered that his father had once spoken to a member of TOFS, but apart from that one contact, Phil really had no idea that he was born with TOF/OA as this was never discussed with him.

Phil says, “I do sound very hoarse all the time; some of my friends actually refer to me as Mutley, the dog of the cartoon, since I laugh in exactly the same way (or so they say). I am constantly being asked

if I have a cold or bad chest. As for the gastro-oesophageal reflux, if that means do I make a weird sound like an overly exaggerated hiccup, then, yes I do … and this seems to have become more prevalent as I have got older. As for the extra thumb, I’m not really sure if it’s related or not because I’m not really sure what I’ve had!!”

Life was difficult for the family. Parents were not encouraged to stay at the hospital in the 1960s and, as they had no telephone, Phil’s mother had to walk to the telephone box each day to ask about his progress. Life was tough for his brother as all the focus was now on Phil. His parents were told on a number of occasions that Phil would not survive the night and arrived at hospital once to be told that Phil had actually died. This was a mistake. The staff had the wrong child!!!

As a child, Phil had bronchitis on a number of occasions. As an adult Phil is always, or nearly always, careful what he eats especially if he is going out for a meal or on holiday abroad.

Phil now has 2 children of his own, both boys, aged 21 and 17, who are both absolutely fine.

The TOF who didn’t know …… that he’s a TOF!

201 3 eventsPlanning for 2013 is well underway at TOFS. Please make a note in your diaries!

Sat 27 April Nottinghamshire Children’s Party

Advance booking needed

Sat 27 April Annual General Meeting

Sat 27 April – Sat 4 May

Tea for TOFS week

Sat 29 June Summer Children’s Party Advance booking needed

Sat 12 Oct TOFS Conference - Coventry Advance booking needed

We have not had a Children’s party in the Midlands for some time and we are fortunate enough to have a grant to run one in Nottinghamshire in 2013. So that’s where the first one will be!

The official TOFS AGM will happen in another room at the same location on the same day.

We will run another two parties in 2013 – maybe more if there is clear demand and we can fairly anticipate a good attendance. We will soon fix the location for the summer party. The location will most likely be near to or in a large city: London, Manchester, or maybe Bristol. Contact the office if you have preferences. The final party will be in November – Date yet to be fixed.

We need your help in the Tea for TOFS week. The idea is that we ask each member to invite their friends and neighbours to a Coffee Morning or Tea afternoon during this week, to raise money for TOFS. Several people did this in 2012 – some raising tens of pounds, some raising hundreds. Events like this are easy to run and there is some back-up from the TOFS office. Please let the office know by email (info@tofs.org.uk) if you are thinking you could take part.

Calling Adult TOFs Do you know an Adult TOF? Do tell that

person about TOFS.

Our organisation is trying really hard to help Adult TOFs. Over the past couple of years,

our conferences for Adult TOFs and our recent Adult TOF leaflet are the most obvious concrete examples of something happening. We think we are starting to get somewhere

with the medical establishment, too.

We are very keen to be in contact with more Adult TOFs, as many are blissfully unaware that they are at a substantially

higher-than-normal risk of developing some complications in later life if unchecked. And of course we would really like to have more

Adult TOFs as members!

15

Adult Conference 2012

The Workshop was led by TOFS trustee Dr Caroline Love. She is a practicing Dermatologist, and an expert on scarring, whether caused by medical interventions or by injury, etc. Caroline illustrated her

opening remarks with photographs of many scars – many of which were not particularly pretty to look at!

Amongst many other things, Caroline talked about Keloid scars, in which the scar tissue expands above the surrounding skin and wider than the original wound. She mentioned that treatment for these can include steroid injections and applications via tapes.

Caroline addressed abnormal healing of skin surrounding a scar, discussing blackheads and Milia spots. Rashes can sometimes be associated with the areas surrounding a scar. Caroline also spoke about Psoriasis, a red flaky skin condition which can develop on top of old scars.

Some questions were asked about looking after new scars. Caroline spoke about silicone sheets and tape which can be used all day to put a little pressure on scars and can have an anti-inflammatory effect. She mentioned Dermatix, Mepilex, and Haelan tape. Some of these are available without prescription.

Several questions were asked about adhesions. These are internal scars which occur normally as a result of surgery. They may limit the normal movement of internal organs, and can cause difficulties including pain, and can only be tackled by more surgery. Mr Kiely, who is a leading and extremely experienced surgeon, commented that in his experience adhesions are typically found in 5% of people after a first operation, 10% after a second operation, etc: the risk increases with the number of operations in the same area. He also added that if adhesion problems are to emerge, this will normally happen within a year of an operation. Sadly, he said, none of the anti-adhesion techniques that have been tried have yet been shown to work.

In response to some questions, Caroline commented that some scars can be painful for a very long time, or maybe be particularly problematic when cold. Shingles and cold sore scarring are both known for this. A prescription-only cream called XEPIN can help.

Several people mentioned problem scars where former gastronomy or chest drain tubes had been removed. Caroline agreed that these sometimes take the form of deep indentations (a special case of indented scars) which indicate the surface layers have become firmly attached to the underlying tissue. Sometimes further surgery might be necessary to tackle such scars and plastic surgeons might be the most relevant experts in terms of re-doing scars. At least one of the Adult TOFs at the workshop said that they had had a large and unsightly surgical scar re-done by a plastic surgeon, with good results.

John Pearce

James Taylor trained as a specialist instructor with the British Lung Foundation. He is currently working for the Health Improvement Team for South Gloucestershire Council to promote physical activity

through group and individual exercise programmes for people affected with respiratory problems. One stark fact is that physical inactivity is now regarded as being as bad for you as smoking, three times worse for you than obesity, and five times worse for you than being stressed!

As they age, Adult TOFs run the same risks of getting diabetes, osteoporosis etc. as anyone else if they do not adopt a healthy lifestyle. Whilst most adult TOFs are healthy, for some, their respiratory problems are on a par with those who have respiratory disease.

There is a cycle of inactivity which is common in respiratory patients: they feel breathless so they don't exercise; the body gets deconditioned and so exercise is harder the next time and breathlessness is increased. An exercise programme is vital to break this cycle of breathlessness and James told us about exercise referral schemes, whereby health care professionals (eg at your GP surgery) can refer patients who would benefit from regular exercise.

Inactivity over time causes muscle wastage in the legs. As your legs are your engine whilst walking, oxygen demands increase with their use and breathing (or breathlessness) increases accordingly. Conditioning exercises for the lower limbs should be undertaken regularly. Weight bearing exercises are good for improving leg muscle function, as is cycling, which also improves aerobic fitness. Cardiovascular or aerobic exercise is most beneficial for reducing breathlessness, helping you to become fitter and healthier.

It is important to maintain a healthy weight and to aim for three to five lots of 30 minute sessions of moderate exercise each week.

Warm ups are very important before starting exercise, as they dilate the blood vessels and increase oxygen transport to working muscles which should help to reduce breathlessness. Warm up should last roughly fifteen minutes, starting light and increasing gradually.

Interval training may improve fitness more quickly: exercise to a level where one is mildly breathless, for say, 30 seconds, and then recover for a short period of 30 seconds, then repeat.

It must be assumed that physical activity is of benefit to Adult TOFs , especially as it has been discovered that long periods of sedentary activity are very bad for your health. Those present at the session shared thoughts on the types of exercise they enjoyed – from gym classes, cycling, walking, Nordic walking, running and swimming, to canoeing, rowing, zumba and belly dancing.

If you are active, stay active but if you are inactive, start to take small steps to increase your activity!

Sue Lewis-Jones

Scarring Workshop

Vicky Amery and mum Pat, Merseyside. Glenda Hope from Essex and Wilkinson family, West Sussex.

Health and Fitness WorkshopMore From TOFS 2012 Adult Conference…

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

Telephone0115 961 3092

Fax0115 961 3097

Emailoffice@tofs.org.uk

Websitewww.tofs.org.uk

Registered charity number327735

Company number2202260

Medical patronsRaymond Buick MB Bch FRCSJames Dickson FRCS FRCSE FRCPCHJohn Duffy FRCS, MS, SF(CTh)Bruce Jaffray BMedBiol ChM FRCS(Glas) FRCS (Paed)Leela Kapila OBE FRCSEdward Kiely FRCS FRCS(I) FRCPCHAnthony Lander Phd FRCS (Paed) DCHPaul 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: John PearceHon. Secretary: Graham SlaterWendy BellDennis HarveySandra HawkinsGill JacksonSue Lewis-JonesDr Caroline LoveSophie Smallwood

Office PersonnelOffice Manager: Diane StephensOffice Administrator: Jayne Allitt

Chew Editorial BoardEditor: Diane StephensSue Lewis-JonesJohn PearceChristine Shepherd

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

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

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

Glossary CHARGE Syndrome – association including Colobama (defects affecting the pupil of the eye), heart disease, Atresia of the choanae (the passages at the back of the nose, Retarded growth, Genital hypoplasia (underdevelopment of the genital organs) and Ear anomalies/deafness.FOKER technique – surgical technique pioneered by John Foker in the USA, to treat long gap oesophageal atresia - sutres are placed in the disconnected or damaged ends of the oesophagus and small amounts of tension are added with the aim of stimulating the ends of the oesophagus to grow closer together, prior to surgery.Nebulizer/Nebuliser - a device used to administer medication in the form of a mist inhaled into the lungs. Peristalsis - contraction and movement of the oesophagus, which normally forces the contents into the stomach.Polyhydramnios - excess amniotic fluid - the protective ‘waters’ surrounding the baby in the womb.Reflux - flow of stomach acid (or acidic gas) back into the oesophagus.Sinusitis - inflammation of an air sinus.Stricture - tightness or restriction.

Judith Bland

Make contact with your TLC

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

Erratum “The article in our previous edition related to the Adult Conference held in Reading last September, and in particular the report on the counselling workshop run by Beverley Slater, should have been headed ‘It’s ok NOT to be ok!’ We apologise to our readers and, of course, to Beverley for this error. “

You may have read in the Summer Chew about our TOFS Local Contact (TLC )

pilot. Our TLCs are all experienced TOF parents, and one grandparent, who know all too well the stresses and strains (and the joys) of TOF/OA. They would be delighted to hear from you, even if you are not ‘brand new’ to the TOF world. They are listed below in their geographical areas along with a little information about how they have been personally touched by TOF. Just give us a call and we’ll happily put you in touch.

Scotland Bruce Rennie, son Christopher (VACTERL) born 2008, TOF/OA, large VSD

EnglandNorth-West: Kay Leech, daughter Harriet, born 2008, TOF, long-gap OA, VSD – Nissen’s Fundoplication, bronchiectasis, tube-feedingNorth-East: Angela Carter, son Jenson, born 2007, born premature with long-gap OA and serious heart abnormality – tracheomalacia,

tracheostomy, gastrostomy feedingMidlands: Kate Clayton, grandmother to Joe Styles, born 2000, TOF/OALondon: Jenny Byrne, daughter Megan (twin to Hazel), TOF/OA, born 2000 - gastric transposition, reflux and chest infections.

Northern IrelandJulie Logan, son Lewis, born2001, TOF/OA, feeding difficulties, reflux, chest infections.

Southern IrelandCatherine Downey, son Aaron, born 2005, TOF.

Note that we are not represented in the South-East or Wales. Would you like to get involved? TLCs may take posters and leaflets to hospitals, act as a point of contact for new families and help organise meetings in their area. If you are interested, please contact us and we’ll put you in touch with the trustee responsible for this pilot, Sue Lewis-Jones.