tofs chew Winter 2010
Support for families of children born unable to swallow TOFS (Tracheo-Oesophageal Fistula Support), the charity that supports families of children with Tracheo-Oesophageal Fistula and associated conditions
Pictured: Shiver me timbers! Look at these bold pirates - seven year old TOF Toby Stephens and his brother, Nathan! Read more about Toby (and his recent appearance in the press!) over the page.
TOFS Awareness Week stories and photos Family Updates
...and much more!
News from our first TOF adult conference!
Want to make a difference? Fundraise for TOFS. Please call us on 0115 961 3092
From the editor... Welcome to your Winter issue of Chew! It’s been a busy few months – for us and for you! We were delighted by the success of our first TOF adult conference. You can read all about it inside. I was there and really enjoyed meeting lots of adult TOFs and seeing plenty of talking and networking going on! As well as photos from the event, you can also find write ups of all the talks. We were delighted by the support we received for TOFS Awareness week in October. See how our members embraced the chance to raise awareness of TOF/OA and TOFS in this issue. There’s plenty more to enjoy in your Winter Chew, if you can find a minute over the festive season! Chew is here to help you share your news so keep on sending us your updates and photos (in digital format please!). We wish you all a very merry Christmas and a great start to 2011!
Camilla Zajac, Chew Editor
A warm welcome to new members! G G
G G G G G
Jenny McInnes from Inverness - Riley born 07/12/09 Marietta and Alan Galbraith from Rochdale - David born 01/04/08 Debbie and Thomas Burnett from Gamston - Eva born 17/07/10 Grainne Ni Dhuill from Cardiff - Aoife born 05/12/07 Abby and Andy Ducksbury from St Albans - Lucy born 17/07/10 Martina Meincken and Thomas Niesler from South Africa Alexandra born 11/09/10 Avene Furness and Ian Monks from Warrington - Tilly born 17/05/10 Juliet Norris from Bingley - Adult born with OA Alex Doull from Milton Keynes - Adult born with TOF/OA Debbie Brewer from Plymouth - Adult born with TOF/OA Lesley Smith from Lancashire - Adult born with TOF/OA Josie Bamford from Nottingham - Grandparent
Government funded Short Breaks Grant Are YOU eligible? One of our members has let us know about the Short Breaks Grant Scheme which is currently funded in the Sheffield City Council area. This is a one-off payment of up to £370 per family for a holiday or short break activity. However time is running out; in Sheffield applications must be received by 30 December and money allocated must be used prior to 31 March 2011. The TOFS member who applied was successful even though the criteria had stated that you had to receive the high rate care component of DLA and this family no longer did. It’s a very straightforward form to fill in - just one A4 page with comments about how your child’s condition affects you as a family. We're unsure if this is something specific to Sheffield City Council. Several Councils had information on it so it may well be available in your area. Why not get in touch with your Children and Young People’s Service in your local Council Office to find out? The TOF mum who was successful is happy to help if you need more information. Just email your query to us at the office and we’ll pass it on. Good luck. Do let us know how you get on.
Chew cover story: Toby Stephens Pictured on the front cover is Toby Stephens at his seventh birthday party this summer with his brother, Nathan! Toby was recently featured in the Diss Express, as part of TOFS Awareness Week. The article covered Toby’s journey from being born with TOF/OA right up to now, when he enjoys going to school and spending time with his brother! We’re very grateful to Toby’s mum and dad, Claire and Michael Stephens for raising awareness of TOF/OA and TOFS with the article. They tell us, “We were really pleased with it and Toby is thrilled to be in the newspaper!”. You can read the article by visiting: www.dissexpress.co.uk/news/toby_s_a_born_fighter_1_1563114
Hey there, Spidey!
This eye catching superhero is young Jack Johnson pictured on his fourth birthday. Jack’s mum, Emma Johnson is also a bit of a hero. She was featured in the last issue of Chew for raising more than £1300 by taking part in the London 10k run!
Ben and Heidi on their special day last February!
TOFS Trustee Sue Lewis-Jones recently gave us an update about her son, Ben. She says:
Jack enjoys his new spidey powers!
“Ben, now age 27, did have the eating difficulties typical of many young TOF children, but at over six feet tall and to see him eating now, you'd never guess! At Durham University he was awarded a Masters degree in Mathematics and is now in his fourth year of teaching, whilst Heidi lectures in Chemistry. Both Ben and his wife are keen rowers. Heidi coxed for the winning team at Women's Henley two years ago which is where and when Ben proposed!”.
TOF mums unite! TOF mum power got into action in the Cambridge area recently when four local TOF mums (and one dad - Mark, Laurie's dad who took the picture!) met at a local country park for a chat and a cuppa! Rachel Hayes told us: “The weather wasn't as good as hoped, but a good time was had by all and included choccies for the kids and banana muffins for the adults!”
Pictured Rachel Hayes with Laurie (TOF - nearly 4) and baby Callum, Holly Masters with Olivia (TOF - 15mth), Amy Fox with Ethan (TOF - nearly 3) and Sue Pandey with Priya (TOF - 5yrs) and older sister Maya.
An update from OZ 4
Michelle and Janelle Kemp wrote to us all the way from Australia. They sent us these great photos illustrating the journey of their son, Marcus from a baby to a young man. They say: “We are very proud of how Marcus has grown into a lovely healthy young man. Marcus was born on 30 January 1992 at the Royal Women’s Hospital Ranwick/POW Sydney. Marcus was a TOF baby and had dextrocardia with an ASD (See glossary over the page for definition). He is now 18 and requires no further medical attention for his congenital condition.”
Later on, Callan had some CAT scans to see exactly what was going on. Callan had some form of a pouch as a result of his surgery that was collecting secretions in his trachea and it was thought this is the cause of his halitosis. “Great” I thought, but the problem is there is little or nothing they can do to fix it as it would be a massive operation and with no guarantee and much risk. The doctor thought it might be an idea if Callan saw another respiratory doctor. Whilst talking to the doctor, I asked about Callan’s weight (he is 5’10 and only 8 stone). Even though he eats like a horse he just can’t put on any weight. She has done some tests and is getting him referred to see someone else. She has told me that perhaps he is not absorbing the nutrients from his food. It has been suggested that this could have something to do with the nerves being severed when he had his oesophagus removed as a baby. It would be great if someone knows anything that might help. If anyone knows of any answers or advice regarding Callan’s halitosis and weight it would be very much appreciated.”
Can you help John? If so, please contact him through the TOFS office.
to all our TOF-OA children. 6 years and over (Under 6 will recieve a card)
Can you help this TOF dad? Callan with his mum and dad at the adult TOFs conference.
If you were at the adult TOFs conference you might recognise these friendly faces! We enjoyed meeting Callan Chevin and his mum and dad, John and Tracey, from Staffordshire at the conference in October. They told us that the event was the first time they had got to meet other people with TOF/OA! Callan’s dad recently wrote to tell us about Callan, who after a very challenging start in life, is now 18 and studying History at university. John also wanted to ask for some advice: “Callan has been experiencing extreme halitosis episodes. He had a broncoscopy (See glossary over the page for definition) and took a sample to culture. Later he was sent some antibiotics and miraculously the smell vanished. At least for a couple of months then it came back again and again.
Joshua Banks 30/12/1996 Callum Smith 08/12/1997 Fergus McDonald 02/12/1998 Lucy Thompson 04/12/1998 Kristie Rolfe 05/12/1998 Seth Alun-Jones 12/12/1998 Tina Taglieri 17/12/1998 Jacob Eckstein 09/12/1999 Jae Dworzanski 22/12/1999 Alexander Bardini 30/12/1999 Jonathan Maze 08/12/2000 Sophie Wakeley 14/12/2000 Ben Cowling 30/12/2000 Elliot Bartrum 14/12/2001 Erin Nicoll 14/12/2002 Freddie Ellis-Batchelor 19/12/2002 John-Carlo Hanratty 12/12/2003 Joe Whitham 02/12/2004 Jasmine Rose 11/12/2004 Archie Muir 17/12/2004 Ela Sidhu 29/12/2004
January Joseph Ware 25/01/1995 Jordan Maycock 26/01/1995 Samiyah Jaman 15/01/1996 Ellen Fretter 04/01/1997 Abigail Gillespie 06/01/1997 Mason Webster 21/01/1997 James Morrison 23/01/1997 Colette Wylie 25/01/1998 Emily Bealing 07/01/1999 Amber Campbell 12/01/1999 Laura Strain 18/01/1999 Lydia Blackburn 09/01/2000 Molly Slack 15/01/2000 Ben Taylor 19/01/2000 Amy Norman 28/01/2000 Laura Jackson 04/01/2001 Ibrahim Meziane 16/01/2001 Jack Stewart 03/01/2002 Niamh Kettleborough 17/01/2003 Sean Keith 19/01/2004 Ryan Fido 28/01/2004 Sam Petherbridge 28/01/2004 Gagan Panesar 30/01/2004 Daniel Agar 30/01/2004 Daisy Marshall 03/01/2005 Grace Dorsey 07/01/2005 Charlie Lashmar 11/01/2005 Liam Buisson 17/01/2005 Olivia Medd 20/01/2005 Jessica Lindley 31/01/2005
February Jacob Mayo 08/02/1995 Luke Ashbery 12/02/1995 Rachel Couchman 14/02/1995 Tallulah Grant 14/02/1996 Benjamin Platten 02/02/1998 Adam Michael 03/02/1998 Tyler Willis 13/02/1998 Lydia Cooper 03/02/1999 Alexander Haynes 06/02/1999 Owain Roberts 07/02/1999 Liam Graham 12/02/1999 Oliver Hatton 18/02/2000 Rebbecca Slator 07/02/2001 Cameron Sheppard 06/02/2002 Alicia Godwin 25/02/2002 Holly West 27/02/2002 Sacha Kobovich 17/02/2003 Ned Melland 21/02/2003 Brooklyn Roberti 05/02/2004 Thomas Gluyas 10/02/2005 Nurah Maddar 10/02/2005 Kinsley Martinez 23/02/2005
Fundraising Donations and fundraising We’d like to thank:
5 Racing has been a great family concern and both Elliott and his brother Ben have been racing for many years. Elliott is always at the faster end of drivers and it amazes both me and my husband that he can manage this considering he has the radial defect in his right arm. In fact most folk are unaware of this defect and are amazed when they discover what he has had to overcome in his short life.”
Nicola and John Collins from Glasgow - £100 Sam Butler - £30.84 raised by wearing a TOFS badge
Elliott with his familyʼs favourite racing car!
at work Hazel James - £50 Belinda and Lee Simpson - £285 raised by holding a coffee and cake morning J Earl, Grandparent to Frazer - £50 Andrew and Cassie Iveson - £500 raised by doing a raffle. Gaynor Lloyd - £40 collection box donation
Group and corporate donations
The Catenians, Blackpool & The Fylde, Circle 25 - £511.26 Endurance GB - £25 West Byfleet Golf Club - £250 raised at their annual
Charity Day Big Lottery Fund £6835
Run in a million
TOF mum Sarah Tester braved the Bristol Half Marathon and raised £659 for TOFS! She also sent us this great photo of her and her son William. She tells us: “William was born with TOF/OA which was only diagnosed once he stopped breathing at home. Since the initial repair he’s had four dilatations, has twice been admitted to have food blockages removed and has just had a Ph study carried out to check his level of reflux. William has also had a hyperspadia repair (See glossary opposite for definitions) and is waiting to have a cyst removed from his nose. Despite all this, he manages extremely well with his food and is a very happy little boy who loves playing with his big brother Oliver.”
Driven to support TOFS Thank you to all the members of the Mother’s Union from St Mary’s Church on the Rock in Ellon, Aberdeen. TOF Mum Angela Mason wrote to tell us that they raised £75 for TOFS at a meeting held at the home of her Mother-in-Law, Beryl. The event was held as part of their summer programme, which included a garden walk, a meeting and then tea and coffee supplied by Beryl. Angela explains: “Obviously, having a grandson, Elliott, who was born with VACTERL in 1988, this was Beryl’s first choice and was agreed by all the ladies. Elliott is now 22 and has grown into a mature and sensible young man. He is now four years into his degree in Architectural Technology with an Aberdeen-based company who are sponsoring him. He has never let anything get in his way and is playing golf off a handicap of 11 and has achieved great respect in his other hobby of motor racing, having gone from karts to cars.
Glossary Definitions of technical and medical terms mentioned in some of our articles and family updates
ACE procedure (Antegrade Colonic Enema): this is a procedure in which an enema is given through an opening on the stomach. ASD (Atrial Septal Defect): a heart problem in which there is a hole between the top chambers.
Aortopexy: a surgical procedure used to treat severe tracheomalacia in which the aorta (a major artery which lies in front of the trachea) is anchored to the sternum (breast bone). This opens up the trachea.
Broncoscopy: an endoscopic examination of the airways. Dextrocardia: a condition in which the position of the heart is reversed and is on the right side of the chest, instead of on the left.
Dilatation: a procedure undertaken to widen a narrowing of the oesophagus (stricture).
Gastro-Oesophageal Reflux (GOR): the acidic stomach contents pass back into the lower oesophagus. This can cause pain, a reluctance to eat and possible problems later in life. Hyperspadia: a condition where the urethral opening is on the side of the penis, which can cause problems with urination.
Imperforate anus: this refers to the absence of a normal anal opening, one of the anomalies that can occur in babies diagnosed with VACTERL Association. An imperforate Anus can occur alone and/or with other conditions too.
Nissen Fundoplication: this procedure is commonly used to reduce Gastro-Oesophageal Reflux (GOR). The lower oesophagus is freed up and the top part of the stomach (known as the ‘fundus’) is wrapped around the lower oesophagus to make a valve at the junction of the oesophagus and stomach. The hole in the diaphragm through which the oesophagus passes may also be tightened. Patent foramen ovale: a relatively common condition in which the opening between the top two chambers of the heart remains open after birth.
Tracheomalacia: floppiness of the trachea (windpipe). Scoliosis: a lateral curve of the spine. VACTERL Association: the term ‘VACTERL’ describes a group of anomalies which can occur together in newborn babies. It is an acronym for: Vertebral (spinal) defects, Anorectal atresia (failure of the anus and lower end of the gut to form), Cardiac (heart) defects, Trachea-oesophageal fistula with or without Esophageal atresia (American spelling of ‘oesophageal’), Renal (kidney) anomalies and Limb defects.
A great way to start the day 6
Getting the kids to school on time can be a challenge, but getting them to school early is an even bigger achievement! Rachel Blake, mum to four year old TOF Oliver Rickards, wrote to tell us about a charity breakfast she organised at Bishop Carpenter School to raise money for TOFS. She says: “We raised £101 by asking parents, staff and children to come to school early, contributing a minimum of a £1 to have breakfast at school. The Friends of Bishop Carpenter School PTA organized the event and attendance was fantastic.”
200 Club Winners
Surprise, surprise! Turning 40 is an important milestone. One of our supporters decided that a surprise 40th birthday party was the ideal occasion to help raise funds for TOFS! An impressive £365 was raised at a party organised for Brian Nicoll, father of Erin Nicoll (now aged seven).
August: September: October:
Keith Gardner Eleanor Tham Deborah Williams
We hear it was also a special night because Brian’s brother and sister in law, Peter and Rebecca, flew over specially from Dallas, Texas to attend the party! Entertainment for the evening was provided by Brian’s close friend, singer-songwriter, Phil Campbell. Phil (www.philcampbellmusic.com) who has appeared twice on The Jools Holland show and has 11 CDs to his name, played two entire sets of old and new songs.
Erin, who was born with a long gap OA, is now doing well and a picture of health, according to her dad! Brian’s partner Libby organised the surprise party which was attended by around 60 friends and family members. Everyone attending generously responded to the request that any gifts should come in the form of TOFS donations.
On the night – L-R Brian Nicoll, Phil Campbell, Brianʼs partner Libby and seven year old Erin (in front). Erin (right) enjoying some of her own home cooking with her sister Brennah!
TOFS Awareness Week: A big success!
Myles of smiles
Thank you to all of you who worked so hard to make TOFS Awareness Week such a huge success! From 11 to 18 October, our members and their friends, and work family colleagues helped to raise funds and awareness of TOF/OA and TOFS. You can read about their efforts here!
Thank you to TOF dad, Myles Thorne and his wife Suzanne who made raising funds for TOFS Awareness Week a big part of a special cycling challenge. Myles explains: Shortly after his birth in December 2006 at the Norfolk and Norwich Hospital, our son Kyle was diagnosed with TOF. After a very difficult first ten months, Kyle started having ‘dying spells’. He was diagnosed with severe tracheomalacia and an aortopexy was conducted. Kyle also suffers from scoliosis (See glossary on p5 for definitions).
Thank you to those who continue to send in donations raised during the TOFS Awareness Week. If you’re not mentioned in this issue, we’ll make sure that your donations are acknowledged in the next Chew. Our thanks also go to Michelle Russell who looked after the PR for the week.
We were thinking about what we could do to raise awareness of TOFS during the awareness week and also to raise money for the three charities closest to our hearts. We decided to take on the challenge of cycling from where Kyle was born, in the Norfolk and Norwich Hospital, to his current home at RAF Brize Norton 162 miles away. Our chosen charities were TOFS, Great Ormond Street Hospital and the Norfolk and Norwich Neonatal Intensive Care Unit.
Bonkers for conkers!
We set out on a tandem with our brother in law, Jamie Bowie, on his racer, on 13 October at 3.25am. My brother Andrew provided invaluable support by following us in his van which was packed full of food and spare bikes and navigating for us en-route. Our journey took us through rural Norfolk by night which was an amazing experience.
Joanne Winspear and her family found a very seasonal way to mark TOFS Awareness Week! Joanne, along with her son Dylan and daughter Isobel (born with TOF/OA) asked family and friends to guess how many conkers they’d collected! They raised a total of £35 for TOFS and their lucky Auntie Susan won a great big box of chocolates with a correct guess of 238 conkers! What a great fundraising idea, Dylan!
The last 50 miles were the most difficult, but pure determination and hard work saw us overcome all this. We completed the challenge in a fantastic 17.5 hours, arriving to a fantastic reception and welcome party at RAF Brize Norton at 20.50hrs. Not content with the 162 mile challenge, we then cycled the additional two miles home where we were met by a further group of supporters brandishing banners, balloons, poppers and champagne. We have raised in excess of £3000 for our chosen charities and would like to thank everyone who has supported us! The brave cyclists with nurses from the Norfolk and Norwich NICU, just before they set off!
Isobel and Dylan hoping their Auntie Susan will share her prize with them!
Celebrating at the welcome at RAF Brize Norton!
TOFS Awareness Week! 8
A special thank you
We’re always delighted to hear of all the clever ways our friends and supporters find to help us keep on doing our work. The family of young TOF, Harriet Leech, managed to find all kinds of smart ideas to raise funds for TOFS Awareness Week! Harriet’s mum, Kay, told us all about it: Harriet enjoying all the excitement of fundraising for TOFS Awareness Week! After having Harriet on 22 May 2008, Gary and I have had a tough time. This culminated in the doctors finding that Harriet had another three fistulas and that silent aspirating had left her with chronic lung disease. When we heard about TOFS Awareness Week, we wanted to get involved to say thank you to the charity which has been so supportive. I am pleased to advise that we have to date raised a total of £2,235.53 for TOFS, excluding Gift Aid. This has been through various methods. We decided to hold a charity event to raise money, but also to get our friends and family together. Harriet loved the charity event and was still singing Twinkle Twinkle at 1.30am when we wanted to go to bed! We also raised £110 through an article in BT Today (I work for BT and this is their online magazine). These donations were from colleagues whom I have never met and just shows how spreading the word can raise awareness, and also funding for the charity. We also raised funds through promotion on Facebook from friends who could not attend the charity event and £305.03 from Harriet’s nursery, St Mary’s college in Blackburn, who had a Pyjama party and raffle, plus £50 from a friend, Mel Booth, who has agreed to run the Accrington 10k.
Council of Management
report: September 2010
The Council of Management held its final meeting of 2010 on Sunday 19 September. Our busy agenda included discussion of the following:
The arrangements for the adults’ conference (just held in October) were discussed and approved. Progress against our commitments in the 2010-12 Strategic Plan was also reviewed. One activity which we intend to increase is to hold more Children’s Parties which have proved an excellent vehicle for entertaining our TOF children whilst giving parents and grandparents a chance to meet with others. Please keep an eye on the TOFS website for further information, as at least three dates for various events have already been earmarked. We also committed to increase our efforts in hospital liaison. We also discussed our attendance at the annual British Association of Paediatric Surgeons (BAPS) conference. We agreed to hold our 2011 TOFS conference on Saturday October 15th.
A taste for fundraising Sarah Procter, mum to Max Swarbricks, wrote to tell us about a sizzling event she organized for TOFS Awareness Week! What better way to raise awareness for children born unable to swallow than to hold a fundraising cooking show! On 15 October, Pampered Chef consultant Amy Johnson arrived at our house with a huge bag of cooking equipment to tantalise the tastebuds of the party guests. After an hour of chopping, mincing, stirring, frying and audience participation, Amy served up a fantastic feast of Chilli Lime Pork Meatballs, after which the guests were able to purchase the cooking products used throughout the evening. A percentage of the sales went directly to TOFS and coupled with the raffle (which saw me trailing the streets selling tickets in all weathers!) we raised a fairly decent £110. TOFS is a charity close to our hearts as our son, Max, was born with VACTERL Association. Along with his TOF OA, Max was also diagnosed with an imperforate anus, patent foramen ovale and spinal problems (See glossary on p5 for definitions). Over the years, TOFS has been an invaluable support for our large family (Max has three siblings!) and we feel we want to give something back.
Sarah says “Max’s diet is very limited, but what he does eat he enjoys!”
Throughout his life, Max has undergone numerous procedures including a Nissen Fundoplication, Bowel Pull Through surgery, approximately eight oesophageal dilatations, and his recent surgery for an ACE procedure (Antegrade Colonic Enema) (See glossary on p5 for definitions) so we can wash out his bowel from the top rather than the bottom! Max is doing fantastically well, always seeming to bounce back with more energy than ever! 2010 even saw us being able to take him on his first holiday abroad! Thank you TOFS, and keep up the good work!
Max having fun at school!
Something smells good! The cooking show in full swing!
Adult Conference: 16 October 2010
Saturday 16 October marked an important first for TOFS. It was the date of our first conference dedicated to the needs, concerns and interests of adults with TOF/OA. The conference grew directly out of feedback from adult TOF, TOFS Trustee Sophie Smallwood. The conference was attended by more than 50 adult TOFs and their parents, from places as varied as Swizerland, Suffolk and Staffordshire! We were delighted to be joined by Bridget Barson, who was the first TOF to have a successful operation (in 1947)! We were also very pleased to be joined by a number of medical specialists and others, including Mr John Black, president of the Royal College of Surgeons.
We would like to say a big thank you to Miss Kapila for being MC on the day and to all our speakers, especially adult TOFs Colette Lemon and Lindsay Bull who shared their personal experiences of growing up with TOF/OA. Our thanks also go to organisers Sandra Hawkins, Sophie Smallwood, Diane Stephens and Jayne Allitt. They also go to Graham Slater for looking after our European colleagues and for presenting on the day and also to Duncan Jackson and John Pearce for presenting on the day. Our thanks also go to the Grimstock Hotel for hosting the conference and to Michelle Russell for organising PR for the event. We would also like to thank delegates who kindly donated their original conference fee back to TOFS. Finally, we would like to extend a big â€˜Thank Lottery Fund for their generous grant.
Sophie Smallwood and Mr Drake with the new Noddy book!
you!â€™ to the Big
At the conference, TOFS Trustee Sophie Smallwood signed copies of her new Noddy book, with donations for copies going to TOFS!
Conference Welcome: Duncan Jackson, TOFS Chairman
With young people moving away from home, there is a need to keep track of continuing problems with a database of specialists who can provide care for adult TOFs in all the specialities that may be required.
TOFS Chairman Duncan Jackson launched the conference by welcoming delegates and commenting on the importance of adult TOFs sharing their experiences. Duncan explained that at TOFS we’re trying to see how we can move forward, branch out and offer help to adult TOFs. Duncan also reminded delegates that we need adult TOFs to help us by sharing their experiences with us. This input is potentially helpful to parents who need to know about potential issues later on in life. There is also the possibility of including these experiences in the next edition of the TOFS book, The TOF Child. Duncan also gave a special mention to delegate and adult TOF, Bridget Barson, who was operated on in 1947! Miss Kapila speaking at the conference
Transition to Adult TOF: Leela Kapila Miss Kapila was Consultant Paediatric Surgeon in Nottingham from 1974 to 2002. She went on to become Senior Vice President of the Royal College of Surgeons of England in 2003. She served as President of BAPS from 1999 to 2000. She is currently Patron of TOFS, Trustee of the Drexler Foundation, Trustee of Rainbows Hospice for Children and Young Adults and Council Member of the Medical Protection Society. Miss Kapila started her talk by explaining how the idea for the adult conference came about. It all began when a patient (and now a TOFS Trustee) Sophie Smallwood got in touch with her about two years ago, seeking advice. Miss Kapila explained that she found it was very difficult to pinpoint people who look after adult TOFs. This eventually led to the conference with the aim of creating an opportunity to share experiences. Miss Kapila explained that while there is still a lot of work to be done, there is good news. She covered a brief history of TOF, from the first case of TOF to be described – in 1670 by William Durston, through the first successful primary repair – 3 March 1941, right up to current times and keyhole surgery. Miss Kapila outlined the different types of TOF, OA and corrective surgery. She said that in her view, the unsung heroes of TOF are adult TOFs. They are now independent young people and they need to be supported. She went on to share the story of Tom Shepherd.
My Experience: Tom Shepherd, Adult TOF Tom spoke about his experience of TOF and VACTERL with his usual dry wit! Anyone who saw him speak at the last TOFS conference will know what we mean! Tom was born with TOF and VACTERL. In his talk, Tom shared the challenges of his transition from child to adult. He explained the differences in having ‘stretches’ done now, compared to when he was a child. Now he goes to an endoscopy unit where he is sedated. He no longer has to stay overnight, as he did as a child. Tom explained that he has six of the seven symptoms of VACTERL, with swallowing difficulties and back problems being his main issue now. Tom has had 40 stretches with the culprits being a variety of food (mainly Chinese food and sausages!). Tom’s many achievements include winning a place with the charity Flying Scholarships for the Disabled, following a very competitive selection process. He is now close to gaining his pilot’s licence. He lives independently and works as a store supervisor. Read more about Tom in an article by his mum, Christine Shepherd in the next issue of Chew.
There are very few long-term studies of adult TOFs. The first one was done in Australia and was written in 1988. A lot has changed. A new study needs to be done. Miss Kapila went on to cover the history of TOFS, from its start in the 80s, to the current stage and to its next stage, which Miss Kapila says should be to address the interests of adult TOFs.
At the conference, Tom waits with other adult TOFs for the paparazzi to gather!
International Collaboration –
Post-traumatic stress –
relevance to adult TOFs: Graham Slater
Somatic Experiencing: Anna Colgan
Graham has been a member of the TOFS Council of Management since 2008. Graham is the second adult TOF to become a TOFS Trustee and is one of the oldest TOF survivors.
Anna is a counsellor and a psychotherapist who works in Exeter and Taunton. Anna incorporates the Somatic Experiencing (SE) approach into her work. In her talk Anna applied the principles of SE to issues of trauma around growing up with TOF.
Graham started his talk by sharing his own story as an adult TOF. He underwent the first successful surgery in North West of England in Manchester. He became involved with TOFS when he challenged himself to run the marathon for the charity. Family support groups can make an essential contribution to follow up care. Graham covered the events at the international TOF/OA conference at which TOFS was recently invited to speak. Graham went on to unveil the new logo for the Esophageal Atresia Trachea Support group which combines TOFS, KEKS (German speaking part of Europe), VOKS (from the Netherlands) and AFAO (France).
EAT’s specific objectives are to:
Provide communications between families affected by TOF/OA Enable the sharing of experiences Provide help and guidance Raise awareness of TOF/OA condition Raise awareness of its existence Encourage and promote research Promote the establishment of similar support organisations in other countries.
International collaboration is as focused on adult TOFs as it is on younger TOFs because each sister organisation faces similar issues. The aim of the organisation is to play a part in helping adult TOFs to tackle those problems – whether of a medical, nutritional or emotional nature – which are specific to them because they are adults.
Members of the newly established EAT group at the adult conference.
Two very special adult TOFs! Graham Slater with Bridget Barson at the conference.
Trauma is the result of undischarged energy in the nervous system. The mind and body are closely connected. The key issue is energy which has not been discharged. These are flight or flight energies that have not been released. The body has an innate capacity to heal itself. Crying is a really good form of release/discharge. Another good sign is trembling. What happens with someone who is operated on? Whilst the operation may be vital, it may still be experienced by the body as an invasion. Even if a person is asleep, their body knows something is going on. These energies will be cycling around the body. These kinds of difficulties are resolveable and can be healed in the body. Somatic Experiencing is a bodywork therapy that addresses how trauma is held in the body and healed in the body. Going through life with unattended issues will tend to manifest in some form. SE allows you to experience things in the body more consciously. Start by paying attention to sensations on the body. Tingling can be a sign of discharge so encourage it. Operations can be sources of trauma, as can very early separation from mother. Pay attention to yourself and allow yourself to know that what you experienced was a hard thing to go through. Use your awareness to heal yourself. As you pay attention, some of that tension releases: “Paying attention is inherently healing.”
Questions from delegates brought up issues such as alternative therapies, the impact of separation on relationships and the importance of allowing TOF children to be allowed to be in touch with their frustrations.
Respiratory – medical: Professor Alyn Morice
Respiratory – surgical: Mr Duffy
Professor Morice is Professor of Respiratory Medicine and Head of Cardiovascular and Respiratory Studies at the Hull York Medical School, University of Hull. The Hull Cough Clinic is now the largest centre in Europe with an international pattern of referral.
Mr Duffy is a Consultant Thoracic Surgeon at City Hospital in Nottingham. His interests include general thoracic surgery, minimally invasive oesophageal surgery (antireflux surgery; thorascopically assisted oesophagectomy), open antireflux surgery including redo surgery and chest wall surgery. Mr Duffy gave a very thorough presentation from a surgical perspective on the long-term outcomes of those born with TOF/OA.
It was heartening to hear that the outcome for patients is generally good and most patients achieve a good quality of life in the long term. Problems in adulthood may depend upon the initial disorder, its treatment and subsequent complications. Surgical methods of repairing TOF/OA include: Primary anastomosis and division of fistula Delayed reconstruction Colon interposition Gastric interposition (stomach pull-up) Reversed gastric tube
Liquid from the acid is important when you’re talking about coughing, but it also comes up as a gas or mist which can get anywhere. It can bang on your voicebox and make you cough or go up your nose and cause sneezing and rhinitis. 80% of people have no allergic response, but 20% of people do. This sort of reflux is not the same as the reflux which causes heartburn. Most of the people who have this cough don’t have heartburn. It makes the nerves extremely sensitive because of the irritation. Professor Morice has developed a cough questionnaire. You can find it at: www.issc.info/HullCoughHypersensitivityQuestionnaire.html
Signs can include:
Coughing when you lie down – this is an allergic reaction to the mist. Coughing with eating – immediate, but you may also often get a cough after meals. Getting a cough after meals is linked with the bowel opening because we eat air as we eat food. (Ten minutes after eating food is peak time for opening the bowel.) Strange taste in mouth – metallic. This is because when the gas comes up, it lands on the metal detecting nerve which is at the back of the tongue. Sneezing – not just strange taste in mouth. Comes up into your nose and you sneeze it out and people may complain about the smell! Drug treatments for this are usually something like an antacid, but this only blocks the acid. Two common antisickness drugs are used – Metaclopramide and Domperidome. Other drugs used are: - Baclofen – tightens up the bowel. - Azithromycin – an antibiotic that improves the movement of your gullet. Gets stuff going in the right way. - Disofrol – an old fashioned drug, but it works. Gets rid of irritation. The oesophagus is the most neglected tube in the body. It has a very difficult job to do. It’s a very complicated organ. Damage means it is much more likely to behave abnormally. The gases cause halitosis and can burrow into the tooth enamel. This chemical irritation can be the cause of constant throat infections and laryngitis. The stomach is full of bugs so when you cough it up, you cough up the bugs that were there anyway. Swollen glands and throat can be connected to this too. There is a new test to prove it – the Pharyngeal PH probe. Questions from delegates covered issues such as the link between reflux and feeling hungover, the mechanism that causes the TOF cough and the connection between the vagus nerve and the bowel.
Mr Duffy explained that the majority of patients will have a primary repair. The problems that may arise after this are different from those that occur after stomach or colon interposition. They may include:
Issues with oesophageal motility (Difficulty swallowing because the oesophagus itself does not propel food downwards. Antireflux surgery can make the problem worse.) Reflux oesophagitis and stricture. If reflux is severe the options for treatment include medication, oesophagectomy or replacement of conduit Problems with conduit (usually colon). This would only apply if a colon transplant had been effected. - anastomotic stricture - redundancy of colon or stomach (as above)
Secondary problems from a colon interposition may be caused by a lack of squeezing function and a tendency for redundant loop formation.
Mr Duffy speaking at the adult conference
Secondary problems from a stomach interposition may include: • Reflux • Stricture at anastomosis (join) • Regurgitation • Poor emptying of stomach – empties by gravity • Reduced capacity stomach – ‘little and often’.
Conference Respiratory problems in adults post TOF/OA treatment were listed as including cough, wheezing, recurrent aspiration, and bronchietasis. Dysphagia can also be a problem. The measurement of oesophageal function can be done by contrast study, manometry or PH study. Mr Duffy went on to discuss Barrett’s Oesophagus. This is where the lining of the gullet changes as a result of reflux. This happens in a small percentage of patients, who will then have changes in the lining that, if left alone, in an even smaller percentage of patients, could become a cancer. The overall risk
is very, very small. In a study of 502 patients followed up over 50 years, there were no cancers. (Sistonen et al. 2009). The risk of cancer developing in Barrett’s is 1 in 200 patient years. The treatment for this can be adapting your lifestyle, lifelong treatment with acid reducing drugs such as omeprazole, dilatation of strictures or revisional surgery (on rare occasions, this could be a resection with a colon replacement). Oesophageal surgery in adults may be difficult if the TOF/OA patient has had multiple previous operations or complications. Should adult TOFs undergo regular assessments for the risk of Barrett’s Oesophagus? Although the overall risk of Barrett’s Oesophagus is very very slim, Mr Duffy said that ideally everyone with an uncommon condition would see a specialist once a year though obviously common sense should prevail and that this depends upon the individual's needs.
Progress in the treatment of TOF and associated conditions: Mr David Drake Mr David Drake recently retired as President of the British Association of Paediatric Surgeons, following a career as Consultant Paediatric Surgeon at Great Ormond Street Hospital. Based on the survival curve at Great Ormond Street Hospital, in 1946 none of the babies born with TOF/OA survived. By 1962 survival rate was approaching 70%. The survival rate now is 95%. There have been a lot of improvements in anaesthesia in the last 70 years and also in intensive care and pain relief. Not all the questions have straightforward answers e.g. the cause of TOF/OA. There are no simple answers to why a baby is born with OA. A lot of work has been done on pre natal diagnoses. While a detailed scan might show a dilated stomach, it is very difficult to get a detailed enough picture to show for sure. So usually the diagnosis is not made until after the baby is born. Pain relief has changed a great deal. Pain relief and analgesic weren’t given after surgery to newborn babies. Since about 1980 we now give this routinely. This means babies have to be in intensive care. There has also been an increase in breast feeding of babies born with TOF/OA. We do a lot of Ph studies. Impedance studies measure fluid in the oesophagus. It doesn’t matter whether it’s acid or alkali, but it will tell you whether fluid is being swallowed and it will tell you whether there is refluxing.
We treat GOR (Gastro Oesophageal Reflux) with medicine and if it fails, we consider an operation. This is a major operation even though it is done laparoscopically. About one in four of patients with OA will have a fundoplication before they are teenagers. There isn’t always a stricture. Sometimes motility is an issue. There is a contrast study of the oesophagus showing a very tight stricture. Used to dilate these with ‘dyno rods’. We now usually dilate them with a balloon. The balloon pushes outwards which causes less swelling than the old method. Before 1970 babies got better as they got older or suffered mortality. VACTERL – great progress has been made in treating ano-rectal anomalies. Long gap OA – lots of work has been done on this. For the last eleven years surgeons have been using keyhole surgery for repairing oesophageal atresia so that instead of a scar, there are just three small incisions. Keyhole surgery has a number of advantages over conventional surgery such as enormous magnification and a bright light. The aesthetic advantage is very obvious. Gordon Mackinlay of Edinburgh popularised this. To date he has done 22 operations with excellent results.
What does the future hold? Paulo de Coppi at GOSH is doing research with tissue engineering to produce a new oesophagus. It’s also being done to make a new trachea. Since the first operation, there have been much better survival figures and a much better quality of life. Medical professionals are also beginning to understand some of the adult issues, thanks to feedback. Medical professionals also understand the symptoms such as reflux, coughing etc, much better than in the past. This is also true for anaesthetics, pain relief and intensive care, too. There is also better interhospital transfer, better materials and better communications. Since 1997 there has been a multi disciplinary clinic at Alder Hey Hospital – patients can see a surgeon, a respiratory paediatrician and a dietician in one visit in one day. This is a model that improves transition for adolescents and adults. As professionals we have to learn from adult TOFs. Keyhole surgery is here to stay and younger surgeons will master the skills.
The challenges are:
The Nissen Fundoplication is not a perfect operation. In five or ten years reflux often comes back. Transition into adults care generally needs to improve. We still need to understand the scientific causes of oesophageal atresia. In terms of global medicine, a big part of world is still back in 1947 where chances of survival were very small. There is still a huge amount of work to do in continents like Africa and certain parts of Asia.
Mr Drake at the conference
What TOFS offers adult TOFs:
Adult TOF forum:
featuring the day’s speakers and chaired by Miss Kapila
John explained his role as Vice Chairman of TOFS, and some of his personal experiences as a parent of a now-adult TOF. John reminded everyone that the work of TOFS has focussed very much on children in the past, reflecting the received wisdom that people tend to "grow out of it". However as numbers of adult TOFS have increased, it has become increasingly clear that not all TOFs "grow out of it", and that there is a valuable role for TOFS to play in supporting the adult TOF community. John remarked that TOFS estimates that there about 5000 adult TOFs and about 2800 child TOFs in the UK at present. In addition to organising conferences for adult TOFs, the Council of Management has identified several specifics that it can offer to adult TOFs, John explained. These include an adult version of the leaflet for GPs, having an adult TOF available on the telephone support service, expanding Chew with some content of greater interest to grown-ups (as seen in this Chew!) and organising occasional adult TOF meet-ups. John wound up by appealing for adult TOFs to make sure that they do become members of TOFS. Though the Council of Management believes there is a need to support adult TOFs, we need to see a material number of adult TOF members for this to make sense.
Points raised included: Should adult TOFs be having check ups and what should we be looking out for? The challenges of receiving the required treatment from GPs. Stomach swelling after eating. Dumping, bloating and food allergies such as coeliac disease. What is known of the cause and prevention of bronchietasis? What is the link between TOF and scoliosis? (There was a discussion of whether there is a connection between scoliosis and thoracotomies in childhood.) Any chance of organising specialist clinics so we can have all our appointments in one day? How do you apply for Disability Living Allowance? There was a discussion about the value of getting a copy of your own medical notes. This is your right as patient and is invaluable for taking to different appointments with medical experts. Delegates and the forum also discussed the value of linking information provided through TOFS to build awareness amongst GPs.
Adult TOFs Questionnaire: Sophie Smallwood At the conference, TOFS Trustee Sophie Smallwood introduced the TOFS Adults Questionnaire which is designed to identify the interests and concerns of TOF adults. The questionnaire will help TOFS to gain a full picture of all the different issues – mobility, respiratory, financial etc. This will help us gather information that we can share with all adult TOFs. Professionals need to hear from adult TOFs about what the issues are. The information will also help us develop a community for when young people become adults.
Thank you to all of you who have completed your questionnaire! If you’ve not done so yet, there’s still time. Please email it to email@example.com as soon as you can. TOFS Trustee, John Pearce
Nutritional issues: Duncan Jackson, TOFS Chair
“Katie and I would like to congratulate the TOFS team on a very informative and well organised conference. It was good for Katie to speak to fellow TOFs (she has received an email from one of them already) and for me to speak to mothers sharing good and bad times.”
Ben Radford chats with Phillipp Schwabl from Austria.
Katie Spall-Burrell and Jeanette Burrell
Duncan decided to make this an interactive session! He asked a group of willing volunteers to stand up at the front and move from one side of the room to the other to show whether they were a ‘yes’ or ‘no’ for questions such as:
Mr Duffy chats with Paula Sandford from Cambridgeshire
Do you have a stricture? How many of you consider that you have actual nutritional needs? How many people have asked for dietary assistance from professionals? How many of you suffer from a lack of taste or smell?
“I really enjoyed the day and found the patients a very interesting and inspiring group. I would be very happy to be involved in the future.”
These questions and their responses were a great way to inspire discussion.
Two volunteers, Colette Lemon and Lindsay Bull, then shared their experiences of growing up with TOF/OA. Duncan asked delegates to write down their tips for controlling reflux. Over the day, the ideas shared were:
Reflux: your views
Eat at the table Red cross pillow raise Triangular pillow at night Don’t eat too late at night Milk Alkaline powder or PPI when I feel heartburn Bed head up on 6 inch blocks – slight incline and PPIs.
In addition to the tips listed above, nutritionist Rita Carmichael kindly provided us with tips on healthy eating for TOFs which we included in each conference pack. If you would like a copy, please request one from the TOFS office.
Bridget Barson pictured with David Kirby from the Oesophageal Patients Association and Mr Duffy, one of the speakers for the day.
“Just to say a huge well done for a good day on Saturday. Great organisation, excellent speakers both the medical professionals and the TOFs themselves. I would be very surprised if anyone went away NOT having learnt something new.”
Gathering all the adult TOFs in one place at the conference took a little time, but it was worth it!
Melinda and Ben Laws
“Thank you for making me so welcome yesterday and all your help with the arrangements. The day was most enjoyable and a great opportunity to meet some adults with TOF and catch up with some folk I had not met for many years. The organisation and location could not have been better. Very best wishes to you and TOFS.” David Drake
We were fortunate to hear from adult TOFs, Colette Lemon and Lindsay Bull at the adult conference.
Mum and daughter, Margaret and Colette Lemon at the conference. Colette spoke about her experience as a TOF as part of the conference schedule.
support for families
Register your interest for any of the events below NOW. Numbers may be limited. To secure your place, please email firstname.lastname@example.org
of children born unable to swallow
TOFS Address TOFS St George’s Centre 91 Victoria Road, Netherfield, Nottingham NG4 2NN
TOFS Family Day in London - Coram’s Fields, near the British Museum and Great Ormond Street Hospital Saturday 30 April 2011. Easily accessible by public transport. Contact the office now to book your place at this event. TOFS Family Day in West Yorkshire - Kirklees Light Railway, Yorkshire’s Great Little Steam Train, Saturday 4 June 2011. Call us NOW to book your place.
Telephone 0115 961 3092
TOFS Family Day, Newbury Race Course - Sunday 31 July 2011. Come and help us fundraise and have a flutter at the same time! Call us now to register interest.
Fax 0115 961 3097
TOFS Children’s Conference 2011 West Midlands, Saturday 15 October 2011, venue to be confirmed.
Email email@example.com Website www.tofs.org.uk Registered charity number 327735 Company number 2202260 Medical Patrons Raymond Buick MB Bch FRCS James Dickson FRCS FRCSE FRCPCH Bruce Jaffray BMedBiol ChM FRCS (Glas) FRCS (Paed) Leela Kapila OBE FRCS Paul Losty MD FRCS (Paed) Gordon A MacKinlay FRCSEd FRCS Sean Marven FRCS (Paed) Agostino Pierro MD FRCS(Eng) FRCS(Ed) FAAP Charles Shaw-Smith BM BCh MRCP PhD Lewis Spitz PhD FRCS Edward Kiely FRCS FRCS(1) FRCPCH Non-Medical Patrons Richard Briers OBE CBE Dennis Foxon BA David Griffiths MA (Oxon) TOFS Presidents Gren and Christine Shepherd Council of Management Hon. Chair: Duncan Jackson Hon. Vice chair: John Pearce Hon. Treasurer: Sandra Hawkins Hon. Secretary: Graham Slater Sue Lewis-Jones Dennis Harvey Gill Jackson Sophie Smallwood Office personnel Office manager: Diane Stephens Administrative assistant: Jayne Allitt The views expressed in Chew are not necessarily those of the editor or the Council of Management.
Report from Nurse’s Study Day Tuesday 5 October Centre for Life, Newcastle upon Tyne Diane Stephens
The event was organized by the National Neonatal Surgical Benchmarking Group (NNSBG) and attended by around 45 nursing staff from various TOF units around the country, including Bristol, Edinburgh, Leeds and Newcastle. I took 60 brochures and copies of Chew and they’d all been snaffled by the end of the first coffee break! I made contact with a number of staff interested in joining TOFS – NICU nurses/educators, outreach sisters and one registrar. Parents Angela and Stephen Carter from Durham did a fantastic presentation on the journey of their son, Jensen. It was very beneficial for TOFS to be represented at the event. As well as learning more about the condition, it gave me an insight into how things happen in different centres. It was heartening to see that the medical people there recognised the emotional implications for a family whose child had swallowing difficulties. I was struck with how well they rate TOFS in terms of our support to patients. There was a real partnership approach. The NNSBG’s presentation was very informative: They had surveyed surgical units and found there were no standard guidelines for the management of TOF. They’ve now written a series of guidelines covering medical and nursing management of TOF/OA and Replogle tube management. I’ve requested a copy for the office. Our thanks go to the NNSBG for inviting us along to this excellent event.
TOFS office staffed: Monday to Wednesday: 8.30am - 2.30pm Thursday: 8.30am - 1pm Friday 8.30am - 12.30pm
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TOFS 2011 programme of events: