'UK heatwave could be a death sentence for our toddler - there's no cure'

Two-year-old Aurora Bryant-Saunders’ massive smile belies a terrifying truth - the coming heatwave could kill her. Sizzling temperatures of up to 37.3 degrees, putting parts of the UK on red alert last month, forced her parents to spend £1,200 on moving the family - including her big sister Bea, seven, into an air-conditioned flat. Her mum, Allie Saunders, says: “Degrees are a matter of life and death. The four-day heatwave cost us over £1,200 - but it could have cost us Aurora’s life.” Aurora has an ultra-rare genetic form of epilepsy, known as PCDH19, affecting just three in every million people. Allie, of Manchester, says: “Aurora struggles to control her core temperature. When the heat gets to a point you or I would find uncomfortable, her body’s natural reaction is to go into seizures. She starts to struggle around 25-27 degrees. “When the temperature rose to over 33 degrees in her bedroom, we had to flee to an air-conditioned apartment in central Manchester.” Doctors believe Aurora’s condition was triggered by a cold at two months old. Allie says: “The first two months of her life were normal. Then she got a cold and began to seize. The seizures have continued ever since. She has them daily, but we manage them with medication given every 12 hours. “Certain things bring them on, such as infections and viruses like common colds, teething and heat. Even with an expensive fan on, last week the temperature in her room reached 33 degrees and she began seizing.” Knowing the seizures were becoming dangerous, Allie and her partner Ross Bryant, 34, a drinks specialist, had no option but to move out. Allie says: “The seizures can cause permanent damage to her brain and affect her body for life. “All the affordable air conditioning units in the country had sold out. Going outside was out of the question, apart from at night. We had no choice but to check into an air conditioned apartment in town. It was life or death.” Allie, who works in events, continues: “Day to day, Aurora is much like any other toddler. She is a happy, loving, funny little girl. She loves playing with cars and her favourite song is ‘row row row your boat,’ because she thinks it’s about her. When it’s not boiling hot in the UK, she lives a pretty normal life with us, going swimming and having playdates with her friends.” Rushing her to hospital after her first seizure, initially, doctors thought it was ordinary epilepsy, or febrile convulsions, which children often grow out of. But the seizures continued and two months later, thanks to the help of a senior neurologist at Royal Manchester Children’s Hospital (RMCH) who was present during an episode, genetic testing was rushed through, and Aurora was diagnosed with PCDH19. Allie explains: “It predominantly shows up in girls and neither parent needs to be a carrier of the gene - we aren’t. It can’t be cured with gene therapy and surgery would have no effect, as it occurs in her entire brain. She won’t grow out of it like some childhood epilepsy, this is for life.” Warned that the future for little Aurora was uncertain, Allie says: “Aurora has regular seizures, which we manage with medication. She’s on a seizure plan, so we can manage them mostly at home by upping her meds by a certain dosage. When this doesn’t work, we take her to hospital for IV meds. We tend to have to go in for a few days every three months.” While Aurora’s diagnosis rocked the family’s world, they’ve been supported every step of the way by medical teams at the RMCH and Roald Dahl’s children's charity. Describing Aurora as a “joyful little girl,” Allie says: “Aurora can walk, albeit being wobbly, and has learned a few words. ‘Bread stick’, ‘naughty’ and ‘hi’ are her favourites, but her first word was ‘Nini’ - her neurologist's name. “As she grows, she’ll most likely be autistic. There’s a high possibility of regression when she has severe seizures that may affect her ability to walk, speak and feed herself.” Luckily, Bea is there to hold her hand. Allie says: “Bea’s a great big sister and is very protective. Their age gap helps and we try to make sure that Bea gets enough time with us all. We are so incredibly proud of Aurora. With the right care, she should be able to live a long and happy life. But avoiding her triggers is hard, especially heat, as we can’t control the weather - and the UK is getting hotter and hotter.” Caring for Aurora is a full-time job. Allie says: “Ross had to give up his job entirely, as there wasn’t enough flexibility if an emergency arose. It’s been hard to run my business too. We have to pay for a specialised nanny to look after Aurora, which has cost us £20K in the last year, so we can work and get some respite. “For the first few years, she wasn’t able to go to nursery as they wouldn’t be able to give her the one to one care she would need. Now we’ve been given the green light by her team, but we’re unable to get a place at a nursery that would be suitable for her. “ Allie says any nursery would need a specialist worker to cater for any medical emergencies for Aurora. While her medical team would help to support the training for nursery staff, most have no place or won’t take her. Money is tight for the family. Allie says: “There’s a cost of living crisis, we can’t afford to pay £1,200 to stay in a hotel every time it’s hot. We haven’t even paid rent yet, because we can’t. And it’s only July. Summer has only just begun.” Allie hopes that by sharing her story, it will help other parents in the same position feel less alone. She says: “We wouldn’t want to change Aurora, but the UK infrastructure isn’t built for children and families with disabilities. The thing about Aurora is that she’s fine, until she’s not. The idea that she could have died last week because it was hotter than usual, really scared us.” Fearing another heatwave, Allie and Ross have launched a GoFundMe page, asking for help to cover costs of future emergency accommodation, plus further treatment for Aurora, including physiotherapy. Meanwhile, they are hoping that pioneering gene therapy, which is in the works, will soon mean Aurora’s condition can be alleviated. Allie says: “There are advances in medicine all the time, and we’re keen to raise as much awareness of PCDH19 as possible, to hopefully secure more funding for gene therapy research.” The devoted parents have now bought an air conditioning unit for Aurora’s bedroom. Allie says: “When the temperature hots up, we’ll retreat to her bedroom. But it’s not exactly sustainable. If these heatwaves really are the new normal we’ll have to move away. We’ve spoken about Scotland, because at least it tends to be a bit cooler. For most people, 33 degrees is picnic weather. But for Aurora, it could be a death sentence.” *Donate at https://www.gofundme.com/f/auroras-fund-the-reason-why What is PCDH19? PCDH19 refers to a gene located on the X chromosome that provides instructions for making a protein involved in cell-to-cell adhesion and signalling in the brain. Pathogenic variants or mutations in this gene cause PCDH19-related epilepsy, a rare childhood-onset disorder primarily affecting females. The hallmark is brief, recurrent clusters of seizures, often triggered by fever, illness or heat.