Joe underwent a successful heart transplant at Great Ormond Street Hospital (GOSH) last year after being diagnosed with dilated cardiomyopathy (DCM) soon after birth.
The condition, which causes the left and also sometimes the right hand side of the heart to stretch, left Joe’s heart muscle weak and unable to pump blood efficiently around his body.
Joe spent nine months in hospital attached to a device called the ‘Berlin heart’, a 15 kilogram machine the size of a small chest freezer, which supports the work of the heart and acts as a life-saving bridging device for children awaiting a transplant.
His parents, Mark and Rachel, have spoken for the first time to urge families to discuss organ donation and to join the NHS organ donor register.
Rachel said: “We are eternally grateful to the donor family. We cannot imagine what they went through. Their generosity of thought at such a horrendous time is completely selfless and amazing.
“Organ donation is a topic everyone should discuss. It is not just those with conditions present from birth who end up needing transplants. Some children, like Joe, will become very sick, very quickly.
“We would also like to thank all of the staff at Great Ormond Street Hospital for what they have done for Joe, but especially his consultant, Dr Alessandro Giardini, and the nurses on Ladybird Ward.”
Dr Giardini, consultant pediatric cardiologist at GOSH said: “We are really delighted to see Joe doing so well and thriving at home. He spent a long time with us in hospital.
“Joe was very lucky to be able to have a heart transplant. There is a chronic shortage of donor organs for children in the UK and at any one time we have several children in the hospital awaiting a transplant. Lots of children wait years for an organ to become available and can very sadly die while they are doing so.”
GOSH is the largest centre for cardiothoracic transplantation in the UK, and one of the largest in Europe. Later this year, the hospital will open the Wolfson Heart and Lung Centre, part of the Morgan Stanley Clinical Building. The new centre will contain state of the art inpatient, daycare and operating facilities.
When Joe was three weeks old Rachel woke one morning to find him ‘grey’. He was rushed to the Evelina Children’s Hospital where an x-ray showed his heart was severely enlarged. Doctors diagnosed him with dilated cardiomyopathy (DCM).
He returned home, his DCM initially managed through a combination of drugs and a strict diet. However, Joe’s health deteriorated rapidly in December 2009 and he spent time on intensive care. Fearing the worst, Rachel and Mark had him christened.
Joe rallied, and was later diagnosed with Barth syndrome, a condition linked to the x gene which causes muscle weakness, short stature and feeding problems. The syndrome had also caused his DCM. Despite this, he spent the next year in generally good health with Rachel describing him as a ‘happy little monster’.
However, by December 2010 Joe began showing signs of heart failure. Doctors confirmed his heart valves were leaking. Joe was short of breath, puffy in the face and had lost his usually healthy appetite.
An ECHO scan showed that Joe’s heart had become further enlarged and was now taking up too much space in his chest cavity. Rachel and Mark were told he would need a transplant and he was placed on the waiting list.
A few days later, Joe had a rapid decline in health and had two cardiac arrests in hospital. Rachel was told the best chance of stabilising Joe would be to transfer him to GOSH and put him onto an Extra-Corporeal Membrane Oxygenation (ECMO) machine, which could temporarily do the work of his heart and lungs to give them a rest.
Within half an hour Joe arrived at GOSH, where he suffered another cardiac arrest. Doctors were able to resuscitate him for a third time and he was placed onto the ECMO machine. 36 hours later, Joe was stable enough to be attached to a Berlin heart.
Rachel explains: “Joe went through so much in such a short space of time, but once he was on the Berlin heart we started to see his character coming back.
“We took things one day at a time, while we waited for a heart to become available. We tried to plan as many ‘everyday’ activities for Joe as we could, Dr Giardini nicknamed him ‘The Swiss Watch’ because he likes to do everything with precision timing!
“When Joe passed the 200 day mark on the Berlin heart we started to really question what else could be done if a heart wasn’t found, but we knew deep down there wouldn’t be much.
“On day 251 the call we had been waiting for came. We were offered a heart for Joe. The call came early in the morning and time seemed to stand still before he was taken down to theatre.
“Thankfully, Joe’s operation was successful. After transplant he spent four days on cardiac intensive care before he could have his chest closed safely. Seeing his new heart – a normal size and thumping away in his chest, was incredible. None of it would have been possible without the selfless generosity of the donor family.
“In total we spent 322 days in hospital before returning home. Our first night back involved a celebratory pizza for dinner, which was Joe’s favourite of course.”
To join the NHS Organ Donor Register, call the Organ Donor Register line on 0300 123 23 23, or log onto the website: www.organdonation.nhs.uk, or text the word 'SAVE' to 84118.
For further information please contact Hayley Dodman, Great Ormond Street Hospital press office on 0207 239 3126 or email firstname.lastname@example.orgFor genuine and urgent out-of-hours queries call switchboard on 020 7405 9200.
Notes to Editors
2. All media enquiries are to come through the Great Ormond Street Hospital press office
3. Great Ormond Street Hospital is the largest heart and lung transplant unit for children in the UK
4. Great Ormond Street Hospital is the only paediatric hospital in the UK to offer the full range of National Commissioning Group (NCG) cardiac services. These are ECMO, bridge to transplant, heart transplant, tracheal services and pulmonary hypertension