A patient's view: Heart transplant helps Caroline look to the future
Caroline Earnshaw, 27, was born with Danon disease, a rare genetic disorder that is currently known to affect fewer than 100 people across the world. The illness causes muscle weakness and potentially lethal heart disease.
Her mother died in 1988 waiting for a transplant when Caroline was just three. She too had Danon disease.
“I was so young and didn’t really understand what was happening,” recalls Caroline. She and her two brothers were tested for the genetic illness and both Caroline and her brother Sean tested positive. Her other brother, Paul, had escaped it.
The illness affects men and women differently and in men, the symptoms begin to appear much earlier. When Caroline was 12 and Sean 14, he was admitted to Harefield Hospital in the hope of a life-saving heart transplant. However, like his mother, Sean died before a donor could be found.
Caroline says: “I was distraught. I’d lost my brother and I was also scared for myself. I couldn’t help thinking ‘When will it be my turn?’ It really hit home that I was a genetic timebomb, just waiting to explode.”
Aged 19, the point where the illness typically worsens in women, Caroline’s symptoms grew severe. She felt tired and lethargic and had palpitations.
The disease’s sudden onset also coincided with Caroline finding out that she was pregnant.
Caroline explains: “I was in a state of shock. The doctor warned me that the strain of the pregnancy on my heart could kill me.”
But baby Keira was born safely in May 2005. After blood tests and an ECG, Caroline received the news she had been dreading – Keira had Danon disease too. “I was devastated. Words can’t describe how I felt,” Says Caroline. “But I knew I had to pull myself together and get on with bringing up my little girl.”
When Keira was three Caroline’s health deteriorated and even the simplest tasks left her exhausted: “Some days I could barely move. I wanted to be out and about with my little girl but instead, I was laid up at home in bed and missing it all.”
In March this year, Caroline was referred for an emergency heart transplant at Harefield: “It was the moment I’d dreaded all my life. I’d lost both my mum and brother as they waited for transplants, But what other option did I have? Without the operation, I faced certain death,” she explains.
Mr André Simon, consultant cardiac surgeon and director of transplantation, explained: “When Caroline was referred to us for a heart transplant she was really very ill and had a very poor quality of life. She was also very distressed that she would die while waiting for a donor heart – just like her mother and brother had. It was up to the team at Harefield to keep her well both emotionally and physically so that she was well enough for the transplant.”
A suitable heart was found for her just over a month later. When Caroline was told the operation would go ahead she felt: “nervous and excited at the same time. This was a chance for a long and happy life.”
She explains “I couldn’t speak to Keira. It was too difficult. At the back of my mind I knew I might not pull through. There was the possibility I’d never see my daughter again.”
The 10-hour operation was a success and Caroline was discharged from Harefield just a month later.
Mr Simon said: “The operation was extremely successful and we are all delighted that she can look forward to a brighter future with her daughter.”
Caroline has also been busy fundraising since her transplant and has raised over £4,000 for the hospital. She said: “I am just so grateful for the care I received at Harefield. They saved my life. The skills and expertise there are amazing and everyone from the physiotherapists, to the nurses and surgeons were fantastic.”
It’s all in the teamwork
The team that looked after Caroline included:
- Transplant surgeons
- Cardiology consultants
- Theatre staff
- Clinical nurse specialists
- Nursing staff – intensive care and ward
- Healthcare assistants
- Transplant coordinators
- Catering staff
- Ward manager
- Medical secretaries
- Ward clerks
- Transport staff