Family with rare condition are recipients of 500th Heart Transplant at GOSH

24 Jul 2023, 9 a.m.

Siblings Lucy and Thomas stood outside of the Great Ormond Street Hospital entrance. The entrance doors are colourfully decorated and the siblings are stood in front of floor displays.

A team of more than 50 specialists from across the country pulled together to help carry out the 500th heart transplant at Great Ormond Street Hospital (GOSH).

The team had less than four hours to bring the heart to GOSH and operate on Thomas, then aged 11, to ensure he had the best chance of a successful transplant.

Thomas has Carvajal Syndrome, which in his case meant his heart muscle was too thick and so he was at serious risk of abnormal heart rhythms. It has affected two of Thomas’ three siblings and, as far as they know, they are one of the the only families in the UK to have this rare syndrome.

This morning, Thomas, Lucy, their mum, Khristine and oldest sister Bethany shared their story on the BBC Breakfast sofa.

'We cannot thank them enough for the family they have given us’

Parents Khristine and Lee found out they were carriers for the gene for Carvajal Syndrome, when their second eldest daughter, Aliesha, was diagnosed with it, aged five.

At the time, they were expecting their third child, Lucy, and were told there was a one in four chance their children would inherit the condition. Lucy, and later Thomas, were diagnosed with Carvajal Syndrome when they were born. Sadly, at eight years old Aliesha passed away from sudden cardiac arrest.

Aliesha was an inspiration, and we will always carry her with us. She is a big part of our family, and her legacy has helped her sister and brother become the incredible young people they are today.

Khristine

When Lucy was 14 her health began to deteriorate. She was on the waiting list for just a few days because she was able to receive a donation after circulatory death (DCD) heart, a new technique which increased the donor pool. There were multiple complications and Lucy spent two months on the cardiac intensive care unit at GOSH, before returning home and making a full recovery.

When Thomas then became unwell, as his heart function had dropped to less than a third of normal values, and his family were incredibly worried about a transplant.

The team did everything possible to help us through such a difficult time. It was during one of his follow up appointments we were told Thomas was the 500th heart transplant at GOSH. It was wonderful to know what an amazing milestone Thomas was a part of. Of course, it isn’t just Thomas’ heart, but Lucy’s, and the other children who have received a heart transplant at GOSH, and the incredible transplant team, that have made this moment possible. Both Lucy and Thomas are now living healthy ‘normal’ lives, and it is all thanks to the people that made the decision to donate their organs that this is possible. We cannot thank them enough for the family they have given us.

Khristine

Thomas’ operation lasted five hours and he was discharged from hospital within two weeks, with no complications.

A year after his operation, he is now back at school and Lucy is sitting her GCSE’s and would like to train to be a paediatric nurse or a police officer.

Some members of the transplant team stood in a line wearing face masks. Some are wearing uniform.

‘I have had the privilege to help co-ordinate 100 of the 500 heart transplants at GOSH’

The GOSH transplant unit, which was set up by Professor Marc de Leval in 1988, is one of the largest specialist centres in the world. It carries out an average of 25 heart transplants each year for children and young people from across the UK.

Finding a donor match for children is incredibly difficult as the heart must have the compatible blood and antibody type and be the right size, which means that they often need a young donor to save their life.

To help children on the wait list, the team use treatments such as the Berlin Heart, which pumps blood around the child’s body instead of their heart. They also carry out research to try and find ways to match more hearts. For example, a team at GOSH funded by British Heart Foundation and the UCL Institute of Cardiovascular Science developed a technique that helps children receive a mismatched heart with a different blood group, more than doubling the number of children able to receive a heart transplant.

Even with these techniques, there are not enough donors to provide the 53 children waiting on the transplant list in the UK with a new heart. Over the past decade there has been a consistent increase in adult consent rates, child donation rates have remained low, and practically static.

We support children and young people, and their families from the moment they go on the transplant waiting list until a suitable donor organ is found - this can be a matter of days, months or years. I have had the privilege to help co-ordinate 100 of the 500 heart transplants at GOSH, including Thomas and Lucy. They are all incredibly resilient and inspiring.

Sarah Mead-Regan, Transplant Co-ordinator at GOSH

While we’re incredibly proud to have reached this milestone, the sad fact is we wish it were higher, but there are simply not enough donor hearts to save the lives of all the children on the transplant waiting list. Many children waiting for a heart transplant are relying on a young donor to save their life because the donor heart needs to be the right size for a child to receive it. This means that although donor hearts are an incredible gift for the recipient, they are from children who have sadly passed away, and their family has made the incredible decision to donate. For this, the team and families are so grateful.

Jacob Simmonds, Heart transplant lead at GOSH

It is a huge team effort when someone donates their organs, to make sure everything that needs to happen goes smoothly and lives can be saved. It is a race against time, with the donor, their family and the transplant patients at the heart of the process, which involves dozens of people working together, from specialist nurses in organ donation, intensive care staff, NHSBT’s organ donation hub, retrieval teams, transport drivers, transplant surgeons, recipient co-ordinators and more. Each transplant is a huge feat and thousands of people’s lives are saved in the UK every year by the incredible generosity of those selfless donors and their families who agree to support donation at such a tragic time for themselves. Please join the NHS Organ Donor Register, organ donation save lives.

John Richardson, who oversees the organ donation hub at NHS Blood and Transplant

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