Meet Thomas, recipient of the 500th heart transplant at GOSH

Thomas has Carvajal Syndrome, which in his case meant his heart muscle was too thick, and he was at serious risk of abnormal heart rhythms.

The condition has affected two of Thomas’s three siblings, and, as far as they know, they are one of the only families in the UK to have this rare syndrome.

Parents Khristine and Lee found out they were carriers of 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,” Khristine says. “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.”

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 heart donation from a donor whose heart had stopped beating. This type of heart donation is called Donation after Circulatory Death (DCD), and it has helped to increase the donor pool.

There were multiple complications, and Lucy spent two months on the cardiac intensive care unit at GOSH before returning home and fully recovering.

Thomas then became unwell. His heart function dropped, and his family were incredibly worried about a transplant.

“The team did everything possible to help us through such a difficult time,” Khristine says.

When Thomas did have a heart transplant, it lasted five hours. He was discharged from the hospital within two weeks with no complications.

“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’s 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.”

A year after his operation, Thomas is now back at school, and Lucy is sitting her GCSEs. She would like to train to be a paediatric nurse or a police officer.

The GOSH transplant unit, 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 annually for children and young people 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, so 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 the 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 children waiting on the transplant list in the UK with a new heart. (There are currently more than 50 children on this list.) Over the past decade, there has been a consistent increase in adult consent rates, whereas child donation rates have remained low and practically static.

For Jacob Simmonds, Heart transplant lead at GOSH, the 500th transplant milestone has been bittersweet.

“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,” he says.

“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.”

John Richardson, who oversees the organ donation hub at NHS Blood and Transplant, describes how each transplant is a “huge feat”.

“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 saves lives.”

You can learn more about organ donation on the NHS Blood and Transplant website.

From buildings and equipment to groundbreaking research, we’re proud to support cardiac care at GOSH in many ways.

Below are a few examples of how – thanks to our supporters – we’re helping the hospital hit more milestones like this one.

• Equipment-wise, over the last five years, we’ve funded over £420,000 into specialist cardiac equipment called ECMO Machines.
  These machines are highly specialised pieces of equipment that can do the job of a child’s heart and lungs when their body can’t. They are vital during heart and lung transplants and for complex heart surgery, as well for children who are critically ill with heart conditions.
• The Zayed Centre for Research into Rare Disease in Children (ZCR), a partnership between GOSH, University College London and GOSH Charity, houses the GOSH Cardiac team and expert cardiac academics from UCL. More than 70% of visits to the Centre’s Falcon outpatients unit are from children with cardiac conditions.
• Some cardiac research takes place in the Noé Heart Centre Laboratories, located inside the ZCR and supported by the Rachel Charitable Trust via GOSH Charity. The laboratories include a cardiac morphology lab, a confocal lab, a 3D printing lab, a bench testing lab and a Nitinol Room. The work taking place in these labs supports research into new cardiac treatments and underpins cardiac clinical care.
• The Love Hearts appeal, set up by a GOSH family, funds groundbreaking research to help save the lives of children undergoing heart transplants. The appeal also funded a new cubicle in the Cardiac Intensive Care Unit.

Want to know more about how the impact we make? Head over to our What we do section.