Chepstow mum's local 'Light Up for Mito' in daughter's memory

Helen Ray’s daughter Tirion was diagnosed with a form of Mitochondrial disease at the age of 18, after suffering unexplained explosive seizures out of the blue for many months.

Tizzy, as she was known to family and friends, spent many hours in the specialist neurology ward at Cardiff University Hospital, and it took six weeks for blood test results and a brain biopsy to confirm she had adult onset Alpers Mitochondrial disease.

Tizzy adored younger brother Dylan, who was eight when she was diagnosed (Image: Helen Ray)

Mrs Ray said: “She was a medical mystery for a while, until they confirmed her diagnosis and told us the devasting news that she likely wouldn’t survive to see her 20’s as there is no known cure.

“Her form of mito can either impact babies or hide until later life. The usual symptoms are seizures, mobility issues and liver failure, although it presents differently in everyone so it’s very hard to diagnose.

“Tizzy was still the same stubborn drama queen post-diagnosis, and she was so determined to carry on and walk again.

"She beat liver failure and continued to amaze her medical teams at the Aneurin Bevan Trust and a specialist mitochondrial disease service in Newcastle, even though the odds were stacked against her.”

Tizzy had a good few years, using a strict medication and diet routine to control her seizures. She was able to participate in all her previous activities, including horse riding, swimming, gym, and socialising with her friends.

Even after her diagnosis, Tizzy was active and took up horse-riding (Image: Helen Ray)

Sadly, by 2017, the seizures began to get stronger and the medication stopped working. Tirion passed away in April 2018, just seven weeks short of her 23rd birthday.

Mitochondrial disease is genetic, and both of Tizzy’s parents carry the faulty gene. Thankfully, Tizzy's brother Dylan did not get the gene and so doesn't have the disease. 

Mrs Ray said: “Her father Steve and I were completely unaware we had this gene until we were tested after Tizzy’s diagnosis. 

“It was awful to see the impact mito had on her. It took just six months for her to go from a fairly independent young lady to being fully bedbound and unable to do anything for herself."

She continued: “This year, I wanted to do something in Tizzy’s memory, so I’ve asked local landmarks to join in with lighting up green across the country for International Mitochondrial Disease Awareness week.

“Her wish was that mito didn’t exist and I owe it to her to continue to raise awareness of her story and the disease. It keeps her memory alive and helps me to feel like I can use her death for something useful.”

Some of the buildings that have been lit up for Mitochondrial Awareness Week across the country (Image: Liz Curtis / Helen Ray)The Rays discovered the Lily Foundation, a charity for people with mitochondrial disease, in 2014. Both during treatment and after Tizzy’s death, the charity has been a massive support, becoming “like one big extended family”.

Founder Liz Curtis, who lost her daughter Lily to mitrochondrial disease in 2007 at just eight months old, said: “I knew Tizzy more or less throughout her whole journey with mito. I can't think about her without a smile on my face - she was unique!

"She amazed me with how she managed her condition, she certainly didn't allow it to rule her life, when she was well enough. She was truly one in a million and I feel fortunate to have been a small part of her journey.”

The annual Light up for Mito will take place around the world this Saturday, September 23 and will help to raise awareness of the disease on a global scale.

Newport’s clock tower will be lit up, and a council spokesperson said: “This is our way of paying tribute to Tizzy, her family, friends and all those whose lives have been impacted by the disease.”

Mitochondrial disease was once considered rare but is now believed to affect 1 in 5,000 people, according to the NHS, making it the second most commonly diagnosed serious genetic condition after cystic fibrosis.