Before she was born, doctors diagnosed baby Vanellope Hope with a rare condition and put her chances of survival at ‘next to zero’. But Vanellope Hope whose beating heart grew on the outside of her body, has defied eight-in-a-million odds and is three weeks’ old. For her delivery, a team of 50 doctors, midwives and nurses had to come together to safely deliver her in an operation rarely successfully performed in Britain. Her overjoyed and ecstatic parents who had dismissed doctors’ advice to terminate the pregnancy, showed off the miracle daughter they never thought they would have.
Vanellope is recovering from three extraordinary operations to relocate her heart from outside her chest to inside her body. Her condition, ectopia cordis, was discovered during a scan at nine weeks into pregnancy. Vanellope’s mom Naomi Findlay, recalled, “I burst into tears. The condition came with so many problems.” Her partner Dean Wilkins said, “We were told our best bet was to terminate and my whole world just fell to bits.
The parents were warned their baby might have chromosomal abnormalities, potential damage to her heart and circulation, and was unlikely to even survive until birth. Miss Findlay further explained, “All the way through it, it was ‘the chances of survival are next to none, the only option is to terminate, we can offer counselling’, and things like that. In the end, I just said that termination is not an option for me. If [death] was to happen naturally, then so be it.”
However, hope began to return when consultant paediatric cardiologist at Glenfield Hospital, Frances Bu’Lock , conducted further scans at 13 and 16 weeks and discovered that – apart from her heart being in the wrong place, Vanellope ‘appeared essentially normal’. Mr Wilkinssaid, “When she starts moving her arms, you feel like, ‘That’s a life in there, she is there.”’
Determined to give their child a fighting chance, the couple paid for a special blood test to check for chromosomal problems. Mr Wilkins said, “When the results of that test came back as low risk of any abnormalities, we jumped up and down in the living room and cried. At that point we decided to fight to give our daughter the best chance of surviving.”
Doctors began drawing up a hugely complex plan, conducting ultrasound and MRI scans on the baby’s chest wall, lungs and brain. Dr Bu’Lock said, “We came together as a team of fetal medicine doctors, obstetricians, anaesthetists, cardiac and abdominal surgeons and cardiologists to review all of the available information and discuss how best to plan for a delivery, surgery and subsequent care. It was decided that delivery by caesarean section would be best to reduce the risks of infection, risks of trauma or squashing of the heart during delivery, and that surgery to provide some sort of covering to the heart would be needed immediately after baby was delivered.”
At thirty five and half weeks into the pregnancy, On November 22 at 9am, Miss Findlay was wheeled into a cardiac theatre at Glenfield Hospital. She met the 50 doctors, midwives and nurses who were split into four teams to deliver the baby, keep the mother safe and carry out the complex heart surgery.
Shortly after, baby Vanellope was born. Miss Findlay recalled, “I started to panic. I actually felt physically sick, because I thought there was a big possibility I wouldn’t be able to see her, or hear her, or anything really. But when she came out and she came out crying that was it – the relief fell out of me.” Mr Wilkins said, “When she cried, we cried. I felt hopeless and just held onto Naomi and was staring into her eyes praying that it was all going to be ok.”
Vanellope and her exposed beating red heart was immediately wrapped in a sterile plastic bag and was whisked to the next team in an adjoining theatre. Consultant Neonatologist Jonathan Cusack said, “The bag keeps the organs sterile but also keeps the tissues moist. We inserted a breathing tube into her mouth and gave medications to sedate her and stop her moving. Vanellope was born in good condition. She cried at birth and coped well with the early stabilization and her heart continued to beat effectively.”
Almost an hour after birth, Vanellope was stable enough to be transferred back to the main theatre – where surgical teams began the task of putting her entire heart back inside her chest. They carefully stretched apart a tuppence-sized chest hole to create more space for the heart, and installed a protective membrane over the beating organ. Over the next nine days, lying on her back in intensive care, Vanellope’s heart gradually sank into the hole in her chest. Surgeons replaced the temporary membrane with a permanent one, and created a special mesh to join loose ribs together and form a cage within her chest. Finally, they made cuts in her skin and stretched it to cover over the hole.
As her organs fight for space inside her chest, Vanellope is still attached to a ventilation machine but her chances of long-term survival are improving by the day. Dr Moore said, “She has a long way to go but so far at least she now has a chance at a future.” No records exist as to how many times the operation has been done before, but it is believed to be one of the first to have taken place in Britain. Mr Wilkins said, “The moment she was born, I realised that we had made the right decision. We know this is going to be a rollercoaster and have started to prepare ourselves for the difficult times ahead, but we needed to give her a chance, and the team here have done that. I can’t begin to thank them for what they have done for my girls.”