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Pioneer Autumn/winter 2013–14

Organ donation

The greatest gift of all

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The child first and always

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More than 50 per cent of our patients come from outside of London

Contents 04 Around the hospital

Latest news from the hospital

14 Feature: organ donation

06 The Pioneer interview: Professor Helen Cross

Welcome to the latest edition of Pioneer. This issue highlights Great Ormond Street Hospital’s role in not only delivering specialist care and treatment to some of the sickest children in the country, but also helping to deliver nationally on wider health issues.

More than 50 per cent of our patients come from outside of London, and the hospital’s UK Newborn Screening Programme Centre (UKNSPC) is the largest in the country. As part of this service, the hospital screens for a group of rare conditions. In this edition, you can read more about the UKNSPC and how the hospital is looking to extend the tests as a cost-effective way of diagnosing babies early in life, helping families to access treatment as soon as possible.

In the Pioneer interview, Professor Helen Cross talks about the benefits of the hospital being the UK’s national centre for epilepsy and her role in shaping government policy. Finally, we focus on organ donation and the hospital’s role as one of just two paediatric transplant centres in the UK. Dr Joe Brierley, Consultant in Paediatric and Neonatal Intensive Care at the hospital, who represents paediatrics on the National Organ Donation Committee, explains the complex issues around organ donation.

The Prince of Wales’s Chair of Childhood Epilepsy talks to Pioneer about meeting HRH The Prince of Wales and the future of epilepsy research

09 Me and my speech and language therapist

Three families introduce us to the therapists who help children with their communication skills

10 I have arthritis

Joe tells Pioneer how a new clinical trial has transformed his life

13 Why I support the charity: Jenny Halpern Prince

The founder of Halpern PR describes how she is inspired

by Great Ormond Street Hospital staff and raises money to support their vital work

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Pioneer takes an in-depth look at the complex issues surrounding paediatric organ donation and how Great Ormond Street Hospital is leading the way on a long-term alternative

18 Meet the team: Interventional Radiology

We meet eight members of the highly specialised team who utilise minimally-invasive image-guided technology

22 Newborn screening

Pioneer details the hospital’s role as the largest centre for newborn screening and its proposed expansion

25 Collaborating for better results

How Professor Martin Elliott’s friendship from his days as a junior doctor helped create an international database

26 A parent’s perspective: my precious Faith

Lisa, mum of Faith, recalls her shock at learning that some of her baby’s organs would be born outside the body and reveals her road to recovery

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I do hope you enjoy reading these stories and the rest of this edition of Pioneer.

Tim Johnson Chief Executive, Great Ormond Street Hospital Children’s Charity 02 Pioneer

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Around the hospital…

Life-changing operation for children with cerebral palsy Selective dorsal rhizotomy (SDR), a minimally-invasive procedure that can significantly reduce muscle stiffness in children with spastic diplegic cerebral palsy, is now being performed by a specialist team at Great Ormond Street Hospital.

Spastic diplegic cerebral palsy affects approximately 400 children in the UK each year. It is caused by injury to the developing brain and is often associated with premature birth.

Transplant milestone

The procedure helps children walk more easily and reduces painful muscle spasms.

The introduction of a SDR service at the hospital marks a major milestone for

Doctors at Great Ormond Street Hospital have performed their 500th transplant operation involving the heart and/or lungs. The hospital reached the milestone after a 25-year history of heart and lung transplants, which has been shaped along the way by new technologies and breakthroughs.

The 500 transplants figure includes a total of 351 heart transplants, 68 lung transplants and 81 heart and lung transplants.

You can read more about the hospital’s work in organ transplantation on page 14.

“The 500 transplants figure includes a total of 351 heart transplants, 68 lung transplants and 81 heart and lung transplants.”

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nerve roots arising from the lower spinal cord. Each nerve root is electrically stimulated and the responses from these roots identify those nerves contributing to the spasticity. This process informs the surgeon which nerves to cut. Eightyear-old Niamh from Dundee was the first patient to undergo the surgery at the hospital.

The SDR technique was brought to the UK

by Consultant Paediatric Neurosurgeon Mr Kristian Aquilina. It is less invasive than alternative techniques and is suitable for children as young as three years old. It involves the selective division of sensory

Mr Aquilina said: “The surgery has already proven to significantly reduce muscle stiffness and give children the ability to stand and walk independently for the first time. The impact on their quality of life and that of their families is therefore enormous.”

HRH Prince Harry hands out awards for patients and staff hospital admissions to stem violent bleeding.

Transplants involving the heart or lungs began at Great Ormond Street Hospital in April 1988 when Consultant Cardiothoracic Surgeon, Professor Marc de Leval, performed the first heart transplant. The hospital has since become one of the largest children’s heart transplant centres in the world.

Approximately 25 heart and/or lung transplants are now carried out each year at the hospital.

Dr Matthew Fenton, Consultant Paediatric Cardiologist at the hospital, said: “As we pass the 500 transplants mark we also need to remember that organ donation is a precious gift. I want to recognise the generosity and thoughtfulness of all the people and families who have donated organs over the years and made these transplants possible. At such an incredibly difficult time for their own family, organ donation can be a tough decision to make – but it’s also an extremely powerful one in granting a child a second chance at life.”

affected children and their families in the UK. SDR can give patients the ability to move without the use of a wheelchair or walking aids for the first time. In recent years, many families from the UK have had to travel to the US for surgery at the Centre for Cerebral Palsy in St Louis, where the minimally-invasive SDR technique was pioneered.

Amazingly, Nikki has managed to focus her attention on fundraising to help research into her incurable condition, for which she has already raised £70,000.

Premier Inn Clinical Building The next phase of the hospital’s longterm redevelopment programme is being kindly supported by Premier Inn, part of Whitbread Hotels and Restaurants.

The Premier Inn Clinical Building, the second part of the Mittal Children’s Medical Centre, will open in 2017. The corporate partner has pledged to raise £7.5 million towards the state-of-the-art building.

The Premier Inn Clinical Building will provide much-needed new inpatient wards, more operating theatres and a recovery unit. It will contain a new surgery centre, respiratory centre and specialist centre for children with severe forms of arthritis, skin conditions and infectious diseases.

Whitbread Hotels and Restaurants run Premier Inn, Beefeater Grill, Brewers Fayre,

Table Table and Taybarns restaurants. They announced Great Ormond Street Hospital Children’s Charity as their nominated charity in May 2012. Much of the fundraising is expected to take place in more than 650 Premier Inn hotels and 380 restaurants across the country and they’ve already raised over £1.7 million towards their total.

Simon Ewins, Business Development and Corporate Responsibility Director for Whitbread Hotels and Restaurants, said: “On behalf of thousands of our team members and millions of guests who have been a part of our fundraising since the start of our partnership I am extremely proud to say that to date we have raised over £1.7 million for Great Ormond Street Hospital Children’s Charity – a phenomenal achievement which will help some of the sickest children from all over the UK.”

Great Ormond Street Hospital staff, family members and patients (including Jonathan pictured above) were the recipients of WellChild Awards at the eighth annual ceremony.

HRH Prince Harry, patron of WellChild, the charity for sick children, recognised the patients, siblings and staff with awards that celebrate the courage of children coping with serious illnesses or complex conditions, as well as showing appreciation to those dedicated to going an extra mile to help these children and their families.

Nine-year-old Nikki won the ‘Most Inspirational Child’ award. Nikki has arteriovenous malformations, which leads to high-risk bleeding around her face. This has caused loss of sight in one eye and means that she has constant treatments and repeated emergency

Sixteen-year-old Hollie from Essex won the ‘Most Caring Child’ award following her nomination by nurse Chloe Dewar, who cared for Hollie’s brother Danny, on the hospital’s cancer ward. Danny was diagnosed with medulloblastoma, an aggressive form of brain cancer. Hollie would visit him every weekend as well as call him from

HRH Prince Harry, patron of WellChild, the charity for sick children, recognised the patients, siblings and staff with awards that celebrate the courage of children coping with serious illnesses or complex conditions

school every morning and lunchtime, while in the middle of sitting her GCSE exams. Sadly, Danny lost his fight with cancer in July 2012. However, this didn’t stop his sister. Last Christmas, Hollie returned to the ward to give out presents to the children and has set up a charity in Danny’s name to support research into children’s brain tumours.

Great Ormond Street Hospital patient Jonathan, six (pictured left), was recognised for his overwhelming courage and ability to see the positive in even the most difficult situations in the face of x-linked chronic granulomatous disease, a severe and life-threatening illness.

Clinical Nurse Specialist, Margaret Clapson, was awarded for her outstanding contribution to children and families affected by HIV/AIDS. Recognised in nursing as a passionate, committed and expert leader in this field, Margaret has also volunteered to share her expertise to help children in Africa.

The final award for the hospital went to Rachel Cooke, Bereavement Service Manager, who won the ‘Impact in Child Health’ award for her exceptional contribution to sick children. Rachel has led and developed many services to support children and families with complex health needs. She also works in leading, developing and expanding community children’s nursing services around London, ensuring children can be cared for at home whenever possible.

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Professor Helen Cross is the Prince of Wales’s Chair of Childhood Epilepsy based at University College London, Great Ormond Street Hospital and Young Epilepsy. She is Head of the Academic Neurosciences Unit at the UCL Institute of Child Health and was recently elected Secretary General of the International League Against Epilepsy, the first woman to be elected to the executive committee in over 100 years.

She lives in St Albans with her husband and three children.

When did you decide to be a paediatrician?

I was one of those irritating children who knew exactly what I wanted to do from an early age, which was to work in medicine and specifically with children. It was only at university that I realised I could combine the two! I was fortunate that I really enjoyed my paediatric attachments and had a special affinity with the people who taught and encouraged me to do my student elective in paediatric neurology in Sydney. When leaving my paediatric training in Birmingham I was very lucky as there were only two registrar posts for training in paediatric neurology and I got the one in London.

The Pioneer interview Professor Helen Cross, Prince of Wales’s Chair of Childhood Epilepsy, talks to Pioneer

What exactly is epilepsy?

Epilepsy is a symptom – the proneness of an individual to have recurrent epileptic seizures. An epileptic seizure is a change in movement, thought, behaviour or sensation – the direct result of a change in the electrical activity of the brain. It is an involuntary occurrence and there’s no prediction for when it is going to occur.

One in 20 individuals will have an epileptic seizure in their lifetime because it may be triggered by all sorts of things, like acute illness or alcohol withdrawal, but only one in 200 have a proneness to recurrent, unprovoked epileptic seizures.

When did you start specialising in epilepsy?

When I arrived at Great Ormond Street Hospital, Brian Neville had recently joined as the first Professor of Paediatric Neurology in the UK and he had a keen interest in

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epilepsy. He was very eager that an epilepsy programme be established and encouraged me to apply for a PhD that I started two years into my registrar training. It was linked into the fantastic Biophysics Unit that we have here.

Is the collaboration between clinical practice at the hospital and research at the UCL Institute of Child Health important?

It’s a fabulous advantage to have the Institute and Great Ormond Street Hospital so intertwined. You’re only able to do the clinical research if you have the patient base to do so, and as children come to Great Ormond Street Hospital from far and wide we have that sample size. The closeness between the two institutions also helps accelerate the pace of translating findings into clinical practice.

What does your role at the hospital involve?

Predominantly, my role is one of research into complex childhood epilepsy. My research started looking into new neuroimaging techniques that helped increase the number of children who could be suitable for epilepsy surgery. It has evolved into improving outcomes for children with complex epilepsy: 70 per cent of children with epilepsy can control it with medication, but 30 per cent continue to have seizures. So much of my research is trying to determine new causes, but also then trialling new treatments to improve outcomes in the long-term, particularly early onset epilepsy. Parallel to the research, the clinical epilepsy service has grown to be one of the largest in Europe.

I also have a policy and training role as an advocate for increasing the availability of epilepsy surgery across the country. A national children’s epilepsy surgery service was launched in November last year and the neurosurgeon William Harkness and myself are clinical advisors to the programme. It involves four centres across the country being able to evaluate children with

complex epilepsy for possible surgery, with an aim of quadrupling numbers that are undergoing surgery in the UK. Is surgery now a viable option for children with epilepsy?

Surgery is available for children when we feel the seizures are arising from one area of the brain and they’re resistant to drugs.

By undergoing a very detailed evaluation including imaging, electroencephalography (EEG) brainwave testing and psychological assessments, we can determine whether the seizures are coming from one specific area and whether that can be removed without causing further damage to the child. We’re obviously not in the business of removing part of the brain if it’s going to halt a child’s speech or change their personality.

Our main goal is to try and complete this evaluation as early as possible. By stopping their seizures early we aim to improve their long-term learning potential and neurodevelopmental outcome.

What does being the HRH Prince of Wales’s Chair of Childhood Epilepsy entail?

The position was set up in 2004 and I’ve been in the role since 2008. I’ve met HRH The Prince of Wales several times over the past decade and he has always shown a keen interest in individuals with epilepsy.

When he visited the hospital recently he was incredibly chatty and spent a long time with the patients. He’s interested in the different treatments available, in particular the ketogenic diet, which is a high fat diet used when a child is resistant to epilepsy drugs. This is an area I have a keen interest in and I recently completed the first randomised controlled trial demonstrating the effectiveness of the diet where drugs have failed. The diet has been used for a long time but my trial was the first to really prove that it worked. We’ve now got funding to do a European study for which I’ll be the chief investigator of 15 centres around the continent. I have also recently obtained National Institute for Health Research funding for a trial of the diet in younger children.

“70 per cent of children with epilepsy can control it with medication, but 30 per cent continue to have seizures. So much of my research is trying to determine new causes but also then trialling new treatments to improve outcomes in the long-term, particularly early onset epilepsy.”

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“I’ve met HRH The Prince of Wales several times over the past decade and he has always shown a keen interest in individuals with epilepsy.”

Are you involved with government health policy?

I was involved in the development of the NICE guidelines in 2004. I was on the Clinical Guideline Group and then when they did the most recent pharmaceutical update I was Clinical Advisor to that.

What have been the main changes related to epilepsy over your time at the hospital?

I think there are three areas in which epilepsy has moved forward. Firstly, we’ve improved our ability to diagnose epilepsy, specifically the type of epilepsy and its underlying cause. This improvement stems from major advances in genetics and neuroimaging, which has led to increasingly targeted treatment. We’ve also moved forward in terms of providing families with more accurate genetic information so they can be reassured about the risk to further children. Finally, imaging has really moved forward, so we are able to more accurately pinpoint underlying abnormalities of the brain’s structure, including malformations of the brain that may be causing some epilepsies.

What are the benefits of Great Ormond Street Hospital being the UK’s national centre for epilepsy?

The main advantage for the children is that we see patients from around the UK, which helps build our expertise. The sheer mass of patients we see can only improve the care we’re able to deliver. 40 per cent of my patients are referred from other paediatric neurologists from around the UK. By coming to the national centre, we’ve got our finger on the pulse, knowing all the research going on and whether there are any clinical trials on the horizon. As the largest academic neurosciences unit in the UK, we have a wide 08 Pioneer

spectrum of research, so have more options to integrate patients into our research.

Do you collaborate internationally?

I have links with Australia, Italy, the United States and worldwide. I was recently elected as the Secretary General of the International League Against Epilepsy, which is a great honour, especially as I am the first woman to have been elected to the executive committee in over 100 years of its history.

The great advantage of international collaboration is not only bringing expertise together but also having a much larger patient base. By completing international studies and bringing centres around the world together you can do a robust analysis. For example, I was the chair of a task force for paediatric epilepsy surgery that included 46 centres around the world, which gave us the professional base necessary to put together published with recommendations.

“40 per cent of my patients are referred from other paediatric neurologists from around the UK. By coming to the national centre, we’ve got our finger on the pulse, knowing all the research going on and whether there are any clinical trials on the horizon.”

The new Cupcake Telemetry Unit in the Morgan Stanley Clinical Building opened last year. What difference has it made?

The Unit is part of the new Koala Ward, which is absolutely fabulous and brings neurosciences and neurology together for the benefit of patients. We’re so thankful to all the supporters whose generosity allowed us to open the new ward and building.

The Cupcake Telemetry Unit is a four-bed Unit based in the ward. It has modern EEG equipment with video facilities so children can have brainwave monitoring while being observed by expert staff just across the hall.

We record continually, so if the child has a seizure, we can go back over the recording and analyse the footage with the brainwave results. This is vital as seizures occur randomly and are therefore difficult for a clinician to witness. The technology removes this problem for us.

What are your hopes for the future of epilepsy research?

I hope that we are able to further improve our longer-term outcomes for children by performing surgery earlier on suitable patients. If we’re able to operate on children after a couple of years of epilepsy rather than after 10 to 15 years it’ll make a real difference. We can get to this situation through genetics and recognising abnormalities. When we have this information we’ll be able to look at shortand long-term outcomes for patients and refer children appropriately.

You have a very busy and stressful job.

How do you relax outside of work?

I have little time for hobbies but I have a husband and three teenage daughters who keep me busy. I find just being a normal mum outside of work and doing things with my daughters keeps me entertained!

Me and my…

Great Ormond Street Hospital has a specialised team of children’s speech and language therapists involved in the assessment, diagnosis and treatment for children with speech, language, communication and swallowing difficulties. They are a vital part of the holistic clinical care that the hospital provides. Here, three families introduce themselves and their speech and language therapist:

Mum Daiva and four-year- old Jonas

Therapist name: Ele anor Favourite thing abou t coming to hospital : Jonas likes to drive his car aroun d Lion Ward Reason Jonas comes to the hospital: As we ll as having chemotherapy, Jonas, who has Down’s syn drome, is learning Makaton sign languag e which uses signs an d symbols to help people communicate Biggest achievemen t: The more Jonas do es signing, the more he talks, which helps both him and those around him Enjoyable sessions: Yes, we learn togeth er Favourite hobbies: Anything active – inc luding trampolining!

Rebecca, 13 -years-old Therapist name: Nivi ety of specialists ry three months to see a vari Visits to the hospital: Eve s me ech so everyone understand Hopes: To improve my spe I’m also learning . ces ten sen Ks and Gs in full Therapy: We’re working on eating strategies to help with my to play loads of games Enjoyable sessions: We get oter and swimming riding, cycling, riding my sco Favourite hobbies: Horse

Mum Sarah and 10-month-old Stanley Therapist name: Vicky Interesting fact: Stanley has a twin sister called Isabel

Reason Stanley comes to the hospital: He has a tracheostomy, an artificial opening into the windpipe, and he can’t make any noises or feed without a tube Making a difference: Vicky offers great advice and new ideas to support Stanley Enjoyable sessions: Yes, very positive and there is always progress ion Next goal: To give Stanley a speech valve so that he can say his first word Pioneer 09

I have arthritis Joe from Sittingbourne was diagnosed with juvenile idiopathic arthritis when he was six-years-old. He was so ill he couldn’t get out of bed and missed weeks of school. Now aged 12, Joe’s life has been transformed by a clinical trial at Great Ormond Street Hospital. He tells Pioneer his story

What is juvenile idiopathic arthritis?

Arthritis is a painful condition that affects the joints and bones. The tissue lining the joints, like elbows and knuckles, becomes inflamed, making them stiff and swollen.

Juvenile idiopathic arthritis (JIA) is the most common form of arthritis that affects young people. It is similar to, but not the same as, rheumatoid arthritis that affects some adults. JIA occurs when the immune system, instead of just attacking infections, attacks the tissues of the body as well.

This makes the lining between the joints stiff and swollen.

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Joe enjoys playing Warhammer with his dad

“My first memory of Great Ormond Street Hospital was travelling up in the taxi from Sittingbourne where we live. I was with my dad and I was very nervous as we neared the hospital. Even though I was only six at the time, I do remember my mum crying when I was diagnosed – I think it was a shock to everyone. I thought arthritis was just something that old people get. When the doctors explained to my mum that this was something without a cure, I think that’s what really upset her.

“Although it wasn’t nice being diagnosed, it was good we at least knew what was wrong with me. At the time I couldn’t walk, play football or go to school. At one point I couldn’t even get out of bed.

The bit that upset me the most was missing out on seeing my friends and doing the things that other boys my age were doing. I was off school for about six weeks in total.

“After being diagnosed, I was put on steroids and methotrexate (a drug that decreases the pain and swelling caused by arthritis, and can reduce damage to joints and long-term disability). Although it helped, there were some bad side effects, for example I used to get out of breath easily playing football. It was during a trip to the hospital that I was made aware of a new clinical trial, a drug that was still at the testing stage. The new drug was called canakinumab and wasn’t licensed for children in the UK. It was a bit daunting at first, taking part in this new trial, but the alternative involved having an injection every day, so I was eager to try this new option!

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“Eight months after being diagnosed, I started the new trial of canakinumab at the Somers Clinical Research Facility at the hospital. Being on the trial means I visit Great Ormond Street Hospital once a month for my injection as well as having a series of other tests done. Even though we’re there for most of the day, the time passes quite quickly; they have play specialists to entertain us and TVs to stop us getting bored. Everyone is so nice to me there. Once, the staff surprised me with a birthday cake and singing. I was so embarrassed I didn’t even look up from my drawing, but it was a lovely thing for them to do.

“The difference the drug has made to my life is amazing. Beforehand I could barely walk, now I hardly notice I’ve got arthritis. I’m pretty close to 100 per cent health wise – I get the odd cold more than most people, as my immune system is supressed, but I can do everything someone my age normally does. From being jealous of my friends playing football when I was diagnosed, I now play for Doddington Village who won the league last year, which was great. I also like playing Warhammer in the garden shed with my dad, I normally beat him!

“I’ve been coming to Great Ormond Street Hospital for half of my life and the doctors and nurses there have made such a difference. Every time I visit I see other children who are really sick. Without the staff working hard to develop these new drugs and treatments, my life would be very different to how it is now. When I’m older I want to be a footballer or cricketer.

Anything is possible now thanks to Great Ormond Street Hospital.”

In 2012, the world’s first research centre dedicated to understanding how and why arthritis affects teenagers opened in the UK. The £2.5 million centre was a collaboration between University College Hospital and Great Ormond Street Hospital. Professor Lucy Wedderburn, Head of Rheumatology at the UCL Institute of Child Health and a consultant at Great Ormond Street Hospital, said; “This is the world’s first centre dedicated to understanding the very specific needs of young people who are growing up with arthritis. By focusing our attention on understanding why and how arthritis is different in adolescence, and what happens as they enter adult life, we hope to dramatically improve treatment and care for young people living with this painful disease.”

“The difference the drug has made to my life is amazing.

Beforehand I could barely walk, now I hardly notice I’ve got arthritis. I’m pretty close to 100 per cent health wise”

Why I support the charity

By Jenny Halpern Prince

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was introduced to Great Ormond Street Hospital Children’s Charity by Charles Denton, former Chief Executive, who was running Molton Brown at the time and was one of my clients. He asked me to sit on the charity’s Corporate Partnerships Board and use my contacts for the benefit of the charity. I remember Paul Pindar, the chair of the board at the time, interviewing me for the role and I got quite nervous as I realised just how much I wanted to be on the board! While my introduction to the charity was through a personal connection, I wouldn’t have accepted if it hadn’t been a cause close to my heart. At the time, my husband and I were trying for children, so I grew to appreciate even more what a precious gift children are. Since we’ve had the twins this feeling has only grown and many of my visits to the hospital leave me teary-eyed.

The Corporate Partnerships Board is quite a simple idea that just works so well.

By getting together a group of senior executives we expand the charity’s network. I’ve been involved in bringing a few new corporate partnerships to the table, such as Center Parcs and The White Company. It’s important when dealing with these potential partnerships that you understand their business needs and how this relationship could help with their corporate objectives. For example, with The White Company we were able to create a product that helped raise money for the hospital as well as generating profit for the company. The staff also got heavily

“As with all great institutions, there will always be something else that needs improving and something else that we need to fundraise for.”

involved with their own fundraising. It had a feel good factor on all sides and has raised over £280,000.

“The staff who work at the hospital really are lifesavers and if I can play a part in helping them with their goal of delivering world-class care then I’ll always do what I can.”

As well as sitting on the Corporate Partnerships Board, I’ve set up a campaign with a friend called Save the Day, which raises money for vital hospital equipment.

The idea arose when I realised I was only really speaking to my work contacts about the charity. Now I engage my friends with the cause as well. We host breakfast meetings or afternoon teas, which are based around tangible pieces of equipment that everyone can contribute to. The first event raised about £90,000 and the campaign has gone from strength to strength. Everyone appreciates the tangibility of the ask – you know exactly what you’re funding and how important it is. We also invest time in saying thank you.

Only recently we showed someone around the hospital who’d invested a great sum of money and were able to show him exactly what he’d funded. It was very rewarding to show him how he’d made a difference.

As with all great institutions, there will always be something else that needs improving and something else that we need to fundraise for. At the moment I’m talking to a mobile app company so there are new and exciting avenues to be explored. While it can be busy juggling my company, children and supporting the charity, it’s about being organised and managing your time correctly. I’m sure everyone on the board who supports the charity is busy, but it’s important work so it needs to be prioritised. I also encourage

Jenny Halpern Prince founded Halpern PR, a consumer brands PR agency, over 20 years ago. Based on the Kings Road in London and employing over 40 staff, Halpern PR has worked for many large corporate brands including Unilever, Avon, Marks & Spencer and Burberry. Jenny lives in north west London with her husband and twin daughters.

all my staff to give a few hours to charity where possible.

A part of my relationship with the charity I really enjoy is meeting the amazing doctors and nurses. I always feel privileged when they take time out of their day to show a potential donor or corporate partner around the ward. They’re always so eloquent and unassuming given just how fantastic they are at their jobs. I remember the surgeon David Dunaway talking through an operation he worked on to separate conjoined twins and just being in awe of the man. The staff who work at the hospital really are life-savers and if I can play a part in helping them with their goal of delivering world-class care then I’ll always do what I can.

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The greatest gift of all

Dr Matthew Fenton, Consultant Paediatric Cardiologist

Pioneer takes an in-depth look at the complex issues surrounding paediatric organ donation and how Great Ormond Street Hospital is leading the way on a long-term alternative

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here’s a famous Mark Twain quote that runs: “Facts are stubborn things, but statistics are pliable.” Yet there’s no manipulating the numbers that matter when it comes to organ donation and transplants. One in three children on the transplant list will die waiting for an organ.

Few facts are more stubborn.

But at Great Ormond Street Hospital, the donation and transplant team doesn’t talk of doom and gloom. Rather, they discuss the bravery of both the parents who allow their children’s organs to be donated, and the gratitude of the recipients. They describe new ways to increase the numbers of organs donated, and to keep more children alive until that happens. They reflect on the many questions that this life-saving – and relatively recent – treatment throws up. And they point out another statistic: the organs from one child can save up to nine lives.

“It’s difficult to talk about death, particularly the death of a loved one,” says Angie Scales, Specialist Nurse in Organ Donation. “I find it hard myself, and I’m very used to it. But 14 Pioneer

we need to take away that taboo.

We need to increase awareness of organ donation.”

It’s been 25 years since Paediatric Cardiologists Dr Philip Rees, Dr Bruce Whitehead and Paediatric Cardiothoracic Surgeon Professor Marc de Leval set up the UK’s cardiothoracic transplant programme. Now, Great Ormond Street Hospital is one of just two paediatric transplant centres in the UK, covering the south of England, while Freeman Hospital in Newcastle covers the north. The hospital conducts more paediatric kidney transplants than any other centre in the UK and is one of the largest centres for children’s heart transplants in the world.

Organ transplants are usually portrayed as fast-moving events, and certainly once a suitable organ is found for a recipient, teams snap into action. But the process starts long before a patient is even put on the list. The first question, says Consultant Paediatric Cardiologist Dr Matthew Fenton, is how his team can best help a sick child.

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“A heart transplant can offer a fantastic quality of life for the sickest children with heart problems.” everybody is the shortage of suitable donors. A heart transplant can offer a fantastic quality of life for the sickest children with heart problems. It is disappointing when we can’t help, either because a donor doesn’t come along or a child is so sick that they are not likely to get through the transplant operation. If more donors were available, it is likely that more children would be given the chance at this life-enhancing treatment.

g n lu “There are two broad groups of children,” he explains. “First, those who have heart muscle problems, where the heart doesn’t pump very well. We don’t often know why for sure but sometimes there is a genetic or familial reason. The second group have structural heart disease, congenital problems which are usually present at birth. The majority of babies who we see with structural problems have had conventional surgical procedures, and often they don’t need a transplant. Some of the

children with structural heart problems will be referred for a heart transplant because they have no surgical repair option open to them and others will have had surgery which hasn’t solved the problem.”

It’s the job of Dr Fenton and his team to assess these patients. The decision to offer a transplant is always based on a balance between risk and the improvement in quality of life – it is never made unilaterally and is always part of a team approach

which involves the patient’s family. And it poses some very hard questions. Having a transplant can transform a child’s quality of life, but the procedure itself might be very risky. This makes it difficult for the family to decide what to do and the team helps with that decision.

“The decision to transplant is based on whether a child is sick enough to warrant taking on the risks,” says Dr Fenton. “The major limitation to helping

The other side of the transplant process is just as delicate. There can be fewer harder questions than the one Angie Scales has to ask. It is her role to approach parents to see if they would consider donating their child’s organs.

“An important part of my job is to enable every family where donation is a possibility to have that choice,” she says. “I work with families and clinicians right from the point when treatment is deemed to be futile – when the child is going to die and there is no other outcome. I speak to the family about options around donation, and if they wish to proceed then I guide them Pioneer 15

Angie Scales, Specialist Nurse for Organ Donation

through that whole process, right through from consent to offering the organs to the transplant centre, through to theatre and then supporting the family afterwards.”

National Organ Donation Committee. A key factor, he says, is to ensure that every parent with a child likely to die is asked if they want to donate their child’s organs.

Specialist nurses like Angie are a key part of increasing donations of childrens’ organs.

The consent rate among the families of children is 44 per cent, while the adult rate is significantly higher at 59 per cent. Dr Joe Brierley, Consultant in Paediatric and Neonatal Intensive Care at the hospital, is a Clinical Lead for Organ Donation and National Lead for Paediatric Organ Donation. He represents paediatrics on the

“It’s always a balance,” he says. “The very skilled senior nurses never just ask: ‘Would you like to donate your child’s organs?’

But there’s a slow way of asking. We have many conversations with the families before we even bring up end of life. We introduce difficult situations, the fact that things aren’t going well and that a child’s death is a possibility. For many years, people used to say: ‘don’t stress the family any more, it’s already a bad time, it’s not appropriate.’ And all those things people did for good reasons. But it prevented parents from being given the chance to donate organs.”

“The most important part of my job is to enable every family where donation is a possibility to have that choice”

Regenerative medicine – the future

The Berlin Heart and other breakthroughs One of the greatest developments of the hospital’s 500 heart and lung transplants has undoubtedly been the advent of the Berlin Heart in 2004, which keeps children alive while they wait for a donor heart to become available. The machine effectively takes over from the heart, helping to pump blood around the child’s body, and allows the child to move around without being confined to a bed.

In 2012, Bellah-Rae (pictured right) broke records by being kept alive for 273 days by a Berlin Heart. Bellah-Rae, who was diagnosed with dilated cardiomyopathy, later underwent a successful heart transplant at Great Ormond Street Hospital.

In the same year, four-year-old Mason also entered the record books by becoming the smallest person ever to undergo a double lung transplant in the UK. The youngster from Warwickshire was just three-feet tall when he received the new lungs measuring 11cm in height and weighing 140g each.

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There’s a growing pressure for more to be done on a national scale to help increase donation rates, such as the soft opt-out system recently passed by the Welsh Assembly, meaning that consent for donation is assumed unless a person has

“Transplantation is fantastic, but often we don’t have enough organs and when we do, they may not be the size that we need for these little patients,” says Dr Paolo De Coppi, Consultant Paediatric Surgeon at Great Ormond Street Hospital and a world leader in this field.

In 2009, premature baby Sarah also broke new ground after having a heart transplant at the age of just 23 days – making her the youngest surviving heart transplant patient in the UK at the time (by age at transplant).

The pioneering breakthroughs continue at the hospital with Dr Graham Davies and his work in thymus transplants being just one example that has helped to save the lives of children.

“Regenerative medicine is a new science that combines well-established scientific fields like cell biology and polymer science, the study of cell structures. You take the cells from the body of the patient who needs them. You grow them in the laboratory and try to engineer them to make the organ that you need. Then you transplant the organ into the patient’s body.”

Three years ago, Dr De Coppi and his team at Great Ormond Street Hospital successfully transplanted a replacement trachea into 11-year-old Ciaran, who was born with a fatal narrowing of the windpipe. The trachea was from a donor

stated a wish to opt out. But the future of organ donation is not all about organs.

It’s increasingly about technology.

Artificial hearts (known as Berlin Hearts) can be used to support a child’s weak or damaged heart while they wait for a transplant. Stem cells and their ability to regenerate could be another way forward (see below). But one thing’s for sure: those who are involved in organ donation – whether it’s helping parents of donors, keeping future recipients alive, or finding new ways to make it more efficient – will not let that one-in-three statistic stand.

“We have been proactive in improving the chances of receiving a suitable donor organ for the sickest children, either by supporting them with mechanical hearts or performing a heart transplant across blood groups,” says Dr Fenton. “This was previously thought to be impossible but has now become routine. More than 60 children have been bridged to a heart transplant with a Berlin Heart, giving them the chance to stay healthy whilst waiting for a transplant. We need to be

Dr Joe Brierley, Consultant in Paediatric and Neonatal Intensive Care

at the forefront of that kind of work. We need the ability to push things forward.

It’s going to be hard and expensive. But there is masses to be done.”

“You grow them in the laboratory and try to engineer them to make the organ that you need.

Then you transplant the organ into the patient’s body.” but was then ‘cleaned’ of its own stem cells and combined with Ciaran’s stem cells. This ground-breaking procedure made headlines around the world – and saved Ciaran’s life. The operation was a complete success.

Now, Dr De Coppi and his team are hoping to progress onto other organs.

They are currently working on growing an oesophagus, which could be transplanted into children missing parts of this organ.

We use our oesophagus – our ‘food pipe’ – to swallow. So missing it means a very poor quality of life.

“For many years people used to say: ‘don’t stress the family any more, it’s already a bad time, it’s not appropriate.’ And all those things people did for good reasons.

But it prevented parents from being given the chance to donate organs.”

Dr Paolo De Coppi, Consultant Paediatric Surgeon

“It’s still a tube but it needs to move,” explains Dr De Coppi. “A baby needs to swallow saliva and food, so we are working on creating the muscle and nerve environment to make this food pipe mobile. We have been quite successful in the preliminary results and we hope that soon we will be able to help a child at the hospital.”

The team is also looking at engineering intestines for transplantation. It’s a natural next step, says Dr De Coppi,

because again, it’s still a tube. But it’s a particularly difficult task, given the complexity of the organ.

“The windpipe is like a tube – a relatively simple organ,” says Dr De Coppi. “It would be much more difficult to engineer a liver, or a lung, or a heart.

“But eventually, following the same pattern that we have used for the windpipe, it may be possible in the future to engineer more complex organs.”

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The team:

My fondest memory is when we had a patient who was completely cured by coming to IR. I think there is nothing better than seeing a patient being discharged completely from the hospital

Interventional Radiology

Amy Clark Maxwell Staff Nurse since 2010 • As a staff nurse, my role includes scrubbing in for cases, assisting with procedures, setting up trollies and helping patients to recover.

The Interventional Radiology (IR) Service offers a comprehensive range of procedures for children from newborns to 16 years of age.

The team utilises minimally-invasive image-guided procedures to diagnose and treat diseases in nearly every organ system. The concept behind interventional radiology is to diagnose and treat patients using the least invasive techniques currently available in order to minimise risk to the patient and improve health outcomes. The Interventional Radiology Service performs around 3,100 procedures each year for patients with hundreds of different conditions

Alex Barnacle Consultant Radiologist since 2003 • I originally came to Great Ormond Street Hospital as part of my training on rotation and decided to stay. My role as a consultant radiologist involves regular all-day operating lists in IR, managing the clinics, seeing patients with vascular anomalies and much more! We’re a very busy team.

• The IR team plays a vital role at Great Ormond Street Hospital. We are involved in the care of nearly every patient who comes through the hospital, whether it’s to do with imaging, helping to make a diagnosis through biopsies, or supporting the treatment of patients from other teams, for example by putting in a Hickman line or feeding tube. We treat a lot of patients referred directly to us as well as patients on behalf of other teams.

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We offer an emergency service so the IR team need to be highly skilled, have great scientific knowledge and be very flexible • It can be challenging because we see a lot of really sick children – it’s a bit like A&E at times! We offer an emergency service so the IR team need to be highly skilled, have great scientific knowledge and be very flexible.

• The patients at Great Ormond Street Hospital really make my job, they’re just fantastic.

• I’d previously visited Great Ormond Street Hospital before I started working here, when a friend of mine was diagnosed with cancer. Sadly, she passed away before her 16th birthday but I remember watching the nurses and thinking they were amazing. I wanted to be like them and have a job helping people.

• My fondest memory is when we had a patient who was completely cured by coming to IR. I think there is nothing better than seeing a patient being discharged completely from the hospital.

• I was voted Student Nurse of the Year in 2010, which was a great honour for me.

I didn’t plan to work for Great Ormond Street Hospital but once I got a job here, I stayed because I loved it - that was 15 years ago!

Clare McLaren Clinical Specialist Radiographer since 1998 • A key aspect of my role is running the X-ray equipment. I am involved with taking the pictures needed for a range of different tests, but I also do some procedures myself and work closely with other teams around the hospital, including the Tracheal team and Renovascular Hypertension team.

• In my role you need to be flexible.

In order to get the right image for the radiologist you need to know which picture they need, exactly how the equipment works and you need to be able to adapt quickly to changing

situations. All the children here are different so you can’t just go to a textbook and look up a procedure – you need to be flexible and know what to do in an emergency.

• I didn’t actually plan to work for Great Ormond Street Hospital, but once I got a job here I stayed because I loved it – that was 15 years ago!

• Something people might not know about me is my absolute love for cats – despite both my husband and I being allergic!

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Jakki Brandon

Craig Gibson Consultant Interventional Radiologist since 2013 • I came halfway around the world to Great Ormond Street Hospital from Australia, because this is a centre of excellence and really is my dream job.

There aren’t many hospitals around the world which practice cutting-edge interventional radiology like Great Ormond Street Hospital.

• The hospital sees a lot of chronically unwell children, many of whom are not well enough for open operations.

We offer an alternative to some procedures which is minimally-invasive, and hopefully aids in a speedy recovery.

• The large proportion of children with rare illnesses and syndromes presents a unique challenge for the team.

Sometimes it is difficult to know the extent of the problem until we’re already into the procedure. We need to be prepared for any eventuality and have plans B, C and D ready to go.

Gillian Cheyne Superintendent of Interventional Radiology and Cardiac Angiography since 2007 • My role involves running the IR department and cardiac catheterisation laboratory, from a radiographer’s perspective. I make sure we have the best team in place and that everybody is properly trained and happy within their positions – it’s quite a diverse role.

• Currently we see between 16 and 20 patients a day, but this could be increased and recovery time reduced if we had more space to work. Next year we’re moving to a new facility with brand new equipment, a larger clinic and more space so we can give patients an even better experience.

• I think the key to our team is the diversity. Everyone is from a different background with different personalities, but we have one aim in common, to give the patient the best possible experience, which allows us to work really well together.

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• The team is brilliant. Even though we are all interventional radiologists, my colleagues have different sub-specialty interests and other consultants will often come into the hospital outside their working hours to assist in procedures and offer their expertise.

• Something people may not know about me is that I was born with a defective heart valve, so I’ve had open-heart surgery a couple of times myself. I think this helps me to empathise with what the patients and families have to go through.

Department Sister since 2009 • In our department we run two operating lists each day and the nurses have a very varied role, scrubbing in, circulating patients and recovery from anaesthetic.

In most cases we use general anaesthetic, but some procedures are done under local, so we get to interact with the patients and families.

• We’re a relatively small team but we do a large number of procedures, many of which are very specialised. I think we might be a mystery to some departments around the hospital, but we actually support every department and every specialty.

There’s a huge variation in what we do, which requires a thorough understanding of how the hospital works and the needs of different patient groups.

• My fondest memory of working at Great Ormond Street Hospital was my very first day. I remember being excited at all the new challenges and the new people I would be working with. I also remember helping one particular patient who was nervous about using Entonox gas instead of going under general anaesthetic. I went through everything with him, chatted to his mum and although he was nervous at first, it went really well and he was chuffed. It was a great moment.

• Outside of work I like to walk my Labrador and take her swimming. I even like to go pond swimming with her, which my colleagues may not know!

Nicole Grossman Everyone is from a different background with different personalities, but we have one aim in common, to give the patient the best possible experience

Healthcare Assistant since 2011 • My role involves everything and anything from making up sterile trolleys for procedures, unpacking deliveries, assisting in recovery, taking parents back up to the ward, stocking up, circulating in the laboratory, assisting if it’s a non-sterile procedure, taking somebody to the pharmacy and playing with the siblings if the child has been put to sleep inside the laboratories.

• Outside of work I’m involved in the Scouts and I play a lot of netball.

• It’s my dream to become a nurse so it was an easy decision to come to Great Ormond Street Hospital. I have got a four-year-old myself so my heart skips a beat with children. It’s so great to see the work that gets done here.

• The IR team is important because the kind of care that we offer here is non-invasive.

It means that the recovery time for patients is better, their stay in the hospital is shorter, it’s better for them and less costly for the hospital.

• Patients may not know that before working here I was a magician’s assistant. He was called Mr Giant, he was almost seven feet tall and I did little tricks and made balloon animals for kids.

The key to our work as a successful team is good communication between the members

Shareen Dadla Senior Administrator since 2013 • My role involves arranging slots for patients coming into IR. As soon as we get a request form we work it into the diary.

• I chose to come to Great Ormond Street Hospital because it’s a good environment.

It’s a children’s hospital and the experience for patients and staff is so positive.

• The key to our work as a successful team is good communication between the members. Everybody needs to be kept informed of the different patients coming into the Unit each day and any special requirements we need to make for them.

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E

very child born in the UK is offered screening for a group of rare but serious conditions.

Most babies screened will not have any of the conditions, but for the small number that do, the benefits of screening are enormous.

Great Ormond Street Hospital houses the largest of these Newborn Screening Centres in the UK, testing samples from 130,000 babies every year. As part of its service it screens babies in the area of north London, Bedfordshire, Hertfordshire and Essex, the largest newborn bloodspot screening laboratory catchment area in the UK.

“We run a service that identifies children at risk of five rare conditions early on in life,” explains Professor Simon Heales, Director of the Newborn Screening Centre at Great Ormond Street Hospital. “These newborns look seemingly healthy and so detecting these conditions early through a simple heel prick test ensures children are given the best possible treatment.

For some of these conditions, early treatment makes all the difference to the health of the child. In some cases treatment can protect vital organs such as the brain, while in other cases it can save lives.”

The importance of the service in identifying children born with these conditions is highlighted by Adeboye Ifederu, Head of Newborn Screening at the hospital. “Early intervention in some of these rare conditions can transform the lives of children and their families.

That is why our team of highly-dedicated and skilled staff is committed to providing an extremely reliable, accurate and efficient service. Samples in our laboratory are processed quickly and accurately, following the highest clinical guidelines. Our service ensures children and their families are provided with a definitive diagnosis and delays to future treatment are minimised.”

Although pockets of screening programmes existed in the 1970s and 1980s, in 2002 a national Newborn Screening Programme was launched.

Celebrating its 10th year last year, this public health programme has improved the health and well-being of screened babies in the UK. The programme has also vastly improved the information families are given about these conditions and their treatment options should their child be diagnosed.

A skin prick that saves lives Pioneer details the hospital’s role as the largest centre for newborn screening and its proposed expansion

22 Pioneer

The success of the programme means it now screens 99 per cent of babies born each year for five rare conditions: phenylketonuria (PKU), congenital hypothyroidism (CHT), sickle cell disease, cystic fibrosis and medium-chain acylCoA dehydrogenase deficiency (MCADD).

Adeboye Ifederu, Head of Newborn Screening

For example, early diagnosis of MCADD means children go on to lead normal, healthy lives. Children born with the condition MCADD are unable to break down fat to make energy for the body.

This can cause problems when a baby misses food, or is ill and urgently requires energy. Newborn screening means babies with MCADD are identified early on in life. Treatment can involve simple measures such as teaching parents to pay special attention to their child’s diet, including making sure they eat regularly.

Adeboye is also quick to emphasise how his team is integrated as part of a wider group at the hospital, making the screening centre at the hospital

“Early intervention in some of these rare conditions can transform the lives of children and their families. That is why our team of highly dedicated and skilled staff is committed to providing an extremely reliable, accurate and efficient service.”

Professor Simon Heales, Director of the Newborn Screening Centre

“We run a service that identifies children at risk of five rare conditions early on in life [...]. For some of these conditions early treatment makes all the difference to the health of the child. In some cases treatment can protect vital organs such as the brain, while in other cases it can save lives.” unique. “The key to ensuring that these conditions are identified promptly and treated appropriately is good communication. At Great Ormond Street Hospital we have immediate access to a range of clinical experts who have an excellent understanding of how to manage these conditions, despite their rarity among the childhood population.

We have had instances where our laboratory has identified a child at risk on one day and they have been seen by the appropriate specialist the next day.”

Thanks to the generosity of supporters, the laboratory has been able to keep pace with advances in newborn screening technology. “We are extremely grateful for the funding of vital equipment for newborn screening,” says Professor Heales.

“Purchasing an additional new tandem mass spectrometer means we can now increase our capacity to test for several metabolic conditions using a single sample. This allows us to identify children at risk even earlier because we are able to process more samples in a shorter period of time. These machines Pioneer 23

are also very reliable, which is critical to a service that handles over 500 samples a day.”

Moving forward, his vision for the laboratory at the hospital goes beyond screening for the five current conditions.

“We want to lead on studies that look to introduce other rare conditions onto the national screening programme. If there are existing treatments for other rare conditions we should carefully consider whether screening can be expanded beyond the current five conditions” says Professor Heales.

Last year the laboratory was chosen as one of six leading centres in the UK to pilot studies on five additional conditions.

The group successfully demonstrated that the laboratory possessed the right mix of technical and scientific skills and the infrastructure to play a leading role in these pilot studies. Importantly, they proved they had the capacity to contribute to these studies without compromising their ability to screen

for the existing five conditions.

Professor Heales is excited to be part of these pilot studies. He adds, “Great Ormond Street Hospital is ideally placed to determine whether screening can be expanded to other rare conditions. The rare conditions being tested for as part of the pilot are maple syrup urine disease, homocystinuria, glutaric

Moving forward, his vision for the laboratory at the hospital goes beyond screening for the five current conditions. “We want to lead on studies that look to introduce other rare conditions onto the national screening programme.”

acidaemia type 1, isovaleric acidaemia and long chain fatty acidaemia. It is imperative that tests for newborn screening are capable of identifying every child at risk. The infrastructure of our laboratories means we have the capability to develop these high quality tests, which provide accurate diagnoses.”

He also reiterates why the laboratory at the hospital is unique. “We have the in-house clinical expertise on how to manage and treat these five additional rare conditions. Expanding the programme will ensure more children affected have immediate access to vital treatment.

“When you work with patient focus groups for these conditions, you recognise how tremendously important and vital this service is to children and their families. Over the coming years we hope to add new conditions to the screening programme, which will give other children affected a chance to lead normal and healthy lives.”

Five days after birth, the midwife will collect a small sample of blood from the baby’s heel using a simple heel prick test. Samples are taken from five days as this offers the earliest point at which these conditions can be reliably detected. The sample is collected on a special type of card, which is then sent to the screening laboratory.

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When samples are received, the child’s information is logged into the computer to provide a record that the sample has arrived in the laboatory. Samples are ‘punched’ out of the cards and tested for five conditions on a tandem mass spectrometer. Samples are processed within 24 hours of being received.

If no condition is identified the negative result is fed back through the child health records system to the appropriate health professional. The health professional will then inform the parents of the result.

If a positive screening result is identified the team will perform additional tests to confirm the d

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The clinical team will inform the child’s GP and parents of the positive result. The child is then immediately referred to a specialist and given the appropriate treatment. Without delays, the process of taking a heel prick test to parents being informed of a positive result can take as little as three days.

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How Professor Martin Elliott’s friendship from his days as a junior doctor has helped create an international cardiac database for the benefit of patients across the world Warsaw Professor Martin Elliott, Co-Medical Director and Professor Bohdan Maruszewski

London

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result. This will be performed within 24–48 hours of a positive identification to ensure any subsequent access to treatment is not delayed. Following confirmation of the positive result the team will immediately notify the relevant clinical specialist at Great Ormond Street Hospital.

firm

at the heart of our work

ver 30 years ago, Professor Martin Elliott, Co-Medical Director at Great Ormond Street Hospital, met Professor Bohdan Maruszewski in Liverpool. Back then they were junior doctors, yet to establish careers as distinguished paediatric cardiac surgeons. Their friendship, built up over three decades, has changed the face of paediatric cardiac surgery, with both men playing an instrumental role in the establishment of a global database used to benchmark the success of operations.

How does newborn screening work?

Newborn screening is a tightly controlled process. The group adheres to strict laboratory standards and timelines set out by the UK Newborn Screening Programme Centre.

This ensures results are delivered to the highest degree of accuracy and children are given access to treatment as early as possible.

Collaboration

“I’ve been interested in using data for comparative outcomes for as long as I can remember,” Professor Elliott explains. “After some initial work in the UK, I transferred the whole database to Bogdan in Warsaw when it was still in its infancy. They had much more flexibility in Poland when it came to semi-commercial ventures and it cost a lot less to set up.

It’s grown exponentially and now collects information on every piece of surgery done for congenital heart disease in over 100 countries across five continents.

“There are various benefits of having this amount of data. It enables you to benchmark your results so surgeons know how they’re performing. As well as striving

to do their best for the children, people are naturally competitive, so this drags results upwards. Secondly, by putting this information out in the public domain it allows patients and their families to make informed decisions. Finally, it enables governments and hospitals to decide where investments should be made and which cohorts of patients the research should be concentrated on.”

safety and the airway work that they do. It’s the only other tracheal service in the world apart from the one I run here.

I also work with teams in St Petersburg, Florida and Chicago among others.

Sometimes that work can be offering secondary advice via teleconferencing.

Only recently I was advising on a patient based in New Zealand.

An international database isn’t the only fruitful development to spawn from the relationship between Professors Elliott and Maruszewski. The latter has been able to establish a charity attached to his hospital in Warsaw, taking learnings from Great Ormond Street Hospital Children’s Charity. Professor Maruszewski, working with fundraising colleagues, established a television telethon translated as ‘Great Orchestra of the Christmas Age’. The event was a fantastic success and raised so much money that funds could be diverted across Poland to ten cardiac centres. The two professors also regularly visit each other’s’ countries to give lectures and pass on learnings.

“International collaboration isn’t always just about improving results either, although that is the heart of our work.

For example, we have an interesting relationship with India, where I sent one of my senior registrars for a years’ training in a place that did more than 4,000 paediatric open heart procedures in one hospital – 50 per cent more than all UK hospitals combined. He went there to learn how they run operations in such a systematic way but still get good outcomes in a difficult environment and how they do it for $1,400 a case.

There are clearly economic lessons we could learn from India and these cost reductions are very relevant to us as we enter the next decade.

Staff at the hospital have links with countries around the world and Professor Elliott is no exception. “In addition to my work with Bogdan and Poland, I work very closely with Cincinnati Children’s Hospital Medical Center on quality and

“Across the world, paediatricians want to do better for children. It’s a moral duty to learn from others wherever they’re based but also, as one of the world’s leading hospitals, pass on our knowledge where we can.”

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A parent’s perspective: my precious Faith

W

hen I was told by the midwife at my 12-week scan that my baby had gastroschisis I was in a state of shock. I was immediately booked for another scan the next day and I didn’t sleep at all that night for worry. Although I’d never heard of gastroschisis I looked it up on the internet when I returned home, despite the midwife warning me not to. Gastroschisis is where the child’s organs are born outside the body so the pictures I saw on the internet really scared me.

After my local hospital confirmed that Faith had gastroschisis, I was referred to Great Ormond Street Hospital and University College London Hospital (UCLH). Initially being referred to the hospital only made me more nervous as I thought only really serious cases were referred there. However, the monthly meetings with surgeon Mr Joe Curry really put my mind at ease. Joe was the first person who was able to talk me through the condition in depth, explain what would happen at birth and the plans that were being put in place.

I was induced early, as is standard practice with gastroschisis, and gave birth to Faith in UCLH at 37 weeks. She was born with her bowel and half of her bladder outside of her body, although when I came to hold her she’d been wrapped up in cling film and a towel so I couldn’t see most of that. I was allowed to hold her for a precious minute before she was rushed over to Great Ormond Street Hospital in an incubator. I was told I had to wait in the hospital for six hours before I was able to follow her to the hospital, which felt like an eternity. My husband accompanied her but has never really spoken about the whole process. As Faith was his first child he found it really tough and blamed himself.

By the time I arrived at Great Ormond Street Hospital, Faith had already had her first major operation. They’d been able to 26 Pioneer

oscillator were probably the lowest point of Faith’s whole journey, from the happiness of having her body closed to watching her fighting for her life. I didn’t leave her side for a minute over those 48 hours.

put most of the bowel and bladder back in her body but not all of it. When I’d held her for that one minute after birth she was so well wrapped up that she looked like a normal baby. Now to see her on the intensive care unit with wires and machines everywhere was quite a shock. At one stage she had cannulas in both hands, both feet and in the side of her head. We were assured she wasn’t in pain, but it just looked really horrendous.

Over the coming days, the doctors and nurses slowly but surely started tucking the rest of her organs back into her body.

It was a very stressful time, especially when Faith dislodged her silo that was holding her external organs so had to go down for further surgery. I stayed at the hospital the whole time and was fortunate enough to have a room at the patient hotel, which was a great help. My husband would come and stay but he also had to look after my two other children so it was really hard for the family.

After 12 days, Faith was taken off the ventilator and moved to an inpatient ward. After a week on the ward we were informed she was being taken to theatre with the hope that they’d be able to completely move all her organs into her body. If it was successful she’d be taken to intensive care, if not then we’d be back to the ward. While I was delighted when I was told to meet her in intensive care, I was shocked when I saw her. She was now on an oscillator as well as an incubator as her body couldn’t handle the arrival of all her organs. The next 48 hours on the

Thankfully, Faith showed what a little fighter she is and made it through this critical time. When we were moved back to the ward and she was able to feed for the first time I knew she had a good chance.

This was the first time her dad was able to hold her and we were able to give her a bath as well. After a brief stay at our local hospital after leaving Great Ormond Street Hospital, we were able to bring Faith home.

The first year was a bit up and down, she had her Hickman line removed, which was a significant milestone but spent her first Christmas in the local hospital as she was susceptible to chest infections. However, she now just has to go back for regular check-ups at Great Ormond Street Hospital with the wonderful surgeon Kate Cross. All she’s left with is a small scar which she’s happy to show off to anyone!

Faith and Lisa at home in Frinton-on-Sea

Faith is now four years old and loves ballet, painting, stickers and is adored by her older brother and sister. Faith’s sister initially gave me the silent treatment when I returned home as I’d been away for a month. However, the whole family is now doing really well and Faith has just started at her local primary school. She’s very independent and tough, she never cries, and I think that comes from having to fight just to stay alive when she was born.

The staff at Great Ormond Street Hospital have done so much for Faith and our family – I wouldn’t want her to go to any other hospital in the world. The staff are just amazing. Joe Curry was able to allay my fears when I was panicking before I gave birth and Kate Cross visited Faith one Sunday when she was off work. This mix of expertise and commitment is what makes the hospital so special. Great Ormond Street Hospital gave my daughter Faith a chance at life and I simply can’t thank them enough.

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Great Ormond Street Hospital Children’s Charity 40 Bernard Street London WC1N 1LE 020 7239 3000 www.gosh.org Great Ormond Street Hospital Children’s Charity.

Registered charity no. 235825.