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friedreichs ataxia
Struggling for years with balance and coordination, Sam Rudland was diagnosed with Friedreich's Ataxia – causing thickening of the heart muscle – at age 18, after a blood test. Subsequently told his low life expectancy ruled out a heart transplant, the Bristol systems engineer was overjoyed when he met psychologist Amy Watts through a dating app in 2012 and fell in love – having a daughter, Esmee, together in December 2013.
“We spoke for about three weeks then had our first date at an Italian restaurant. We had the same sense of humor, outlook on life, and values," Sam recalled. “We both really wanted a family and wouldn't let my condition stop us. I explained that I might not always be there but remained optimistic about the future. I think that rubbed off on Amy."
Over the years, Sam's health, which meant numerous hospital trips for his heart to be restarted, became their focus – leaving them blindsided when Amy was diagnosed with breast cancer in February 2016.
Astonishingly, Sam's condition improved and he was finally accepted for a transplant, which he had in May 2017, but Amy's cancer spread to her liver and she died in April 2019, aged 42, two days before her wedding – going on to be cremated in her bridal gown.
“Amy was an incredible mum to Esmee, who is the most precious, amazing little girl," Sam said. Sam also has a son, Tobias, 11, from a previous relationship,
“It is a very cruel twist of fate that she so badly wanted to be a mum, then had it snatched away. We probably had two good years together which weren't plagued by illness. They were as close to perfect as you could expect. Esmee has wonderful memories of Amy, but I worry they'll fade. She's come so close to losing both parents, it doesn't bear thinking about."
At times desperately ill with Friedrich's Ataxia – which the NHS describes as a genetic condition affecting one in every 50,000 people in the UK – Sam refused to give in. While symptoms can include balance and co-ordination difficulties, slurred speech, increased weakness in the legs, vision loss and diabetes, his real problems only began in his late 20s, after starting medication for inflammation of the heart muscle.
Abnormal heart rhythms followed in his early 30s, leading to a harrowing prediction that he would not live past 35, making him ineligible for a heart transplant.
“I knew what came with having the condition – but never thought it would happen to me," Sam said. “Despite my heart problems, we were really happy."
Sam proposed over a candlelit dinner at their home at the end of 2014, but his Ataxia worsened, making them nervous about setting any firm wedding plans.
“My heart rate would get to 140bpm, rather than the normal 60 to 100bpm, so I'd regularly end up in hospital," he said. “I had five cardiofversions, where an electric shock delivered from a defibrillator is used to restore the heart's rhythm to normal."
“I also had two cardiac ablations – procedures where surgeons burn away areas of the heart to destroy the tissue allowing incorrect electrical signals to cause the abnormal heart rhythms. Sadly, they didn't work," he recalled. “In November 2015, I was fitted with an implantable cardioverter defibrillator (ICD), which uses an electrical signal to regulate my heartbeat. Amy often drove me to hospital, then paced the corridors while the doctors fixed me up."
“She did this all while trying to keep things as normal as possible for Esmee," he said.
With everyone focussed on Sam's health, Amy was shocked to discover a small lump in her right breast, while showering towards the end of 2016.
Sent for tests, in October 2016, aged 39 – with Esmee then just two – she was diagnosed with breast cancer, which had spread to her lymph nodes.
“We were totally shocked – but convinced the treatment would work. We even discussed having another child when she was better," he said.
In November that year, Amy had her right breast removed, followed by a three-week round of chemotherapy, then radiotherapy a month later.
Then, in March 2017, Sam was hospitalized with intense swelling of his feet, fluid retention and dangerously low blood pressure – all linked to his heart.
“I kept fearing I'd die, Amy's cancer wouldn't get sorted out and Esmee would lose both her parents," he said.
Constantly in and out of hospital, Sam, then 39, asked doctors if they would reconsider a transplant.
Fortunately, his results from a five-week assessment at Cambridge's Royal Papworth Hospital, combined with the fact he had exceeded his life expectancy, made him eligible – leading to a successful six-hour heart transplant operation in May 2017.
“Afterwards my neurologist told me that no-one with Freidreich's Ataxia has had a heart transplant in this country before. They actually took my old heart off to do some tests on it," he continued.
But in November 2017, rushed to hospital after suffering a cardiac arrest, it took 10 minutes of CPR to stabilize Sam and, fearing his body was rejecting the donor organ, a balloon was fitted in his heart to prevent valve blockages.
He was also hooked up to an extra corporeal membrane oxygenation (ECMO) machine, to deliver oxygen to his blood.
“The ECMO saved my life, but it damaged the femoral artery in my right leg, which started bleeding internally, causing necrosis – or the death of body tissue – in my skin," he said.
Hospitalized over Christmas, he was left with a hole in his leg, which he could still lose if a blood clot develops.
“I spent Christmas attached to machines keeping me alive, but despite all she was going through, Amy was always there in person – or on the phone," Sam said.
“Things were feeling bleak for me at the time, and she really lifted my spirits and gave me hope," Sam explained. “It was the worst time of year to be in hospital – Christmas Day. Amy came all that way so I could we with Esmee. She was so small at the time we got the nurses to put wrapping around the cannulas and scary things like that."
On doctors' advice Amy continued having chemo every three weeks for a year. But, in January 2019, they halted treatment, as her white blood cell count was dangerously low, putting her at risk of infection.
Further tests showed the cancer had spread to her liver, where she had a rapidly growing tumor the “size of a small child's head."
In February, Amy's cancer became terminal. Preferring not to know how long she had left, the couple hoped for at least six months and planned their wedding for the end of April.
“It was devastating," said Sam. “Amy had been given a death sentence, while I had been given a chance with my new heart. She was so upset thinking she wouldn't see Esmee grow up, graduate and get married. That was all she wanted. She wanted to have the same last name as Esmee, so we planned our wedding for the end of April. Amy sat down and explained Mummy was poorly and while Esmee knew something was wrong, we didn't push the fact – we wanted their time together to be unspoiled," he explained.
They were determined to make their wedding day extra special – particularly for Esmee, who was to be their bridesmaid.
“Amy bought her wedding dress and we booked a boutique hotel near Bristol for the ceremony."
Towards the end of March 2019, in considerable pain, Amy asked to be taken to St. Peter's Hospice in Bristol.
“She was crying, so afraid that she would never see the house again," Sam recalled.
“I thought Amy would spend a few days in the hospice, then come home. But her liver was pressing against her stomach and diaphragm, causing pain and breathing problems. Luckily St. Peter's is an amazing place and Esmee was able to see her mum every day before being looked after by her grandparents."
Terrified he would lose her, Sam cancelled their planned wedding, instead booking a registrar to marry them in the hospice the following week, on April 3.
“We had room for 10 guests and the hospice cafe was going to do some food," he said. “Esmee's whole outfit had been picked and chosen. I never got to see it but I know she had the whole kit – pretty dress and tiara."
“Esmee visited, giving her a Mother's Day card, but Amy didn't even register what was happening. She was drifting in and out of consciousness," said Sam. “The last thing she said to me that Sunday was, 'Please don't leave me on my own.'"
“I slept that night in the armchair in her room. The following morning, she would not respond to anything. I sat there holding her hand and, over the course of an hour and a half, her breathing just slowed down and stopped. She didn't seem in pain and it was very peaceful at the end."
It was heartbreaking.
“I had to break Esmee's heart by telling her that her mom was dead," he said. “She knew she wasn't well – in January her teachers had asked her to write down a prayer and she put, 'Please God make my mommy better.' Like all of us, Esmee struggled to accept Amy was going to die. Because she'd seen the doctors sort me out, she expected that for her mummy too."
Movingly, when Amy was cremated on April 24 at Memorial Woodlands, Bristol, she was dressed in her white lace-necked bridal gown.
“Esmee asked me a few months later, 'Are you sad that you and Mummy didn't get to marry? I said 'of course' and Esmee replied, 'I'll marry you Daddy.'"
“She has been a complete rock," he said. “She misses her mom so much, but we talk about her all the time and have a shelf with pictures of Amy on it. Luckily, I have a lot of support from Amy's mom, who lives close by as, with my Ataxia, I worry about having a setback in case I'm not here for Esmee."
Sam only knows that his heart donor was a man in his early 20s but remains eternally grateful to him and his family.
“I wrote them a letter as there's barely an hour in the day when I don't think about what they've done for me. If they knew that I'm still here to look after Esmee because of them, I hope they would be really proud of themselves and of their son," he said.