A teenage cystic fibrosis sufferer who was warned she’d be dead by 17 if her condition went untreated is pleading with the government to subsidise the only medication which has offered her any relief.
Isabella Powell, from Auckland in New Zealand, was diagnosed with the genetic disease as a newborn and spent the first year of her life in hospital.
At 17, she and her family were introduced to Trikafta, a ‘miracle drug’ which helped Bella feel like she could breathe easier just six hours after taking her first dose.
Within two weeks, she was feeling great and could walk around, despite struggling to stand up earlier, and was able to sleep through a full night without waking up in a fit of coughs.
‘It’s basically a miracle drug,’ her mother, Alley, told Daily Mail Australia.
‘[It’s] more than a miracle. If there’s a word that’s bigger than a miracle, it’s that.’
Isabella Powell (pictured with her mother Alley), from Auckland in New Zealand, was diagnosed with the genetic disease as a newborn, and spent the first year of her life fighting to survive in hospital
But the medication costs about $470,000 each year and is not subsidised at all by the New Zealand government – despite being the best chance at survival for the 503 local cystic fibrosis sufferers.
Alley explained that Bella grew up feeling like she was ‘alive but not living’ as she missed out on birthday parties, school formals and camps when battling her condition.
‘There are no words as a mum to describe watching your daughter struggle to breathe,’ Alley explained.
Pictured: Bella with Sir Bob Elliott
At the height of New Zealand’s Covid lockdown, Alley feared Bella was ‘slipping away’.
‘If I was to be honest I did not think she would see Christmas,’ she admitted.
But the new medication has changed her life, and the Powell family understand that Bella is one of the ‘lucky ones’ who was able to afford the drug.
Bella requires three pills a day – two in the morning and one at night.
Bella’s first three months of treatment was funded by Sir Bob Elliott, who paved the way for cystic fibrosis treatment and founded the ‘heel prick test’ to identify the disease at birth.
Elliott, who was knighted at his home this year, had terminal cancer and told Alley he ‘couldn’t stand by and see Bella die’ knowing there was a drug that could potentially have treated her.
He died on August 21, after decades of campaigning and searching for a treatment for cystic fibrosis.
Prior to his death, Elliott said it would be like ‘completing his life work’ to see the price of Trikafta lowered to become affordable for Kiwi families.
Bella is on the waiting list for a lung transplant, but she and her family know the risks associated with the operation are high.
For starters, even if she finds a right match, they mightn’t work in her body. Then her family have to consider the real risk of her dying either on the waiting list or operating table.
Her family have also struggled with knowing that a donor would have to die ‘so that Bella could live’.
Based on the funds available to them at the moment, Bella’s medication will run out in July 2021.
Within two weeks of starting treatment, she was feeling great and could walk around, despite struggling to stand up earlier, and was able to sleep through a full night without waking up in a fit of coughs
Alley is desperate to find a way to keep funding her daughter’s treatment after watching her live the life she always dreamed of.
‘She has energy again to do the things she loves,’ Alley said. ‘She has not missed school and has had no hospital admissions which is an absolute miracle as we used to spend at least three months a year in hospital.’
A GiveALittle fundraising page has been set up to help fund her treatment, but Bella’s family are ultimately hoping to raise awareness about the drugs and are working on getting it subsidised by the New Zealand government.
Back in October, Bella had the opportunity to meet with Prime Minister Jacinda Ardern about the necessity of the drug.
She shared a photograph of the pair together alongside a touching message asking the PM to ‘make choices with people like me in mind’ while representing New Zealand on the world stage.
‘I am Bella and I want to live. I want to have just a chance to contribute my potential to our country… I just need to breathe.’
Pictured: Bella and her two sisters, Maggie (left) and Harriet (right). Bella’s mother Alley said life has somewhat returned to normal for her siblings too since she began medication
Bella said Ms Ardern was ‘vivacious, beautiful and kind’ when they met and made her hopeful for the future to know that ‘anything is possible’.
But she also acknowledged that at the moment, her future relies on the charity of people who are in a position to help her fund her expensive treatment, and is hoping that this will be amended in the near future.
The 17-year-old explained that living with the condition is like ‘being alive but not really living’.
‘Your ribs can’t move, you can’t get enough air in. Your body is always sore.’
Bella described her condition as constantly feeling like she was ‘breathing through one of those teeny straws’.
Prior to taking the drug, Bella’s goal was to be able to do what other 17-year-old girls could do.
‘When you just want to walk with your friends to class. The really small things. And you can’t do that,’ she explained.
Now, she’s living a life she never dreamed of and is now gearing up to feature in a live rendition of High School Musical in 2021.
Bella described her condition as constantly feeling like she was ‘breathing through one of those teeny straws’. Pictured with one of her sisters during a hospital stay
Bella’s first three months of treatment was funded by Sir Bob Elliott, who paved the way for cystic fibrosis treatment and founded the ‘heel prick test’ to identify the disease at birth. Pictured: Bella with Sir Bob Elliott
She hopes to study theatre management and is slowly reintroducing herself to circus training after she had to stop when her condition made her weight plummet.
In total, there are about 503 patients in New Zealand who are campaigning for Trikafta to become cheaper.
Without any subsidies, for each patient to receive the lifesaving treatment it would cost $236 million a year.
‘Our wish is that every person living with CF and every family who watches their child struggle has an opportunity to experience the miracle that is Trikafta,’ Alley said.
‘We know it comes at a huge cost and things like this take time but time is something our children do not have a lot of.’
Elliott described the unattainable price point as ‘cruel’.
‘I think it’s cruel actually, and unethical. I’ve been treating cystic fibrosis most of my professional life. This is what we’ve been waiting for and it works.’
Elliott made a scientific breakthrough in the medical industry in the 1970s when he created the ‘heel prick test’ to identify cystic fibrosis at birth.
He dedicated most of his career from that moment onwards to finding a cure.
Bella spent upwards of three months every year in hospital with health issues prior to discovering the medication
WHAT IS CYSTIC FIBROSIS AND THE NEW ‘MIRACLE DRUG’?
Cystic fibrosis is a progressive disease that causes lung infections.
The disease is genetic and can be discovered at birth.
It limits the sufferers ability to breathe over time and creates thick mucus which clogs the airways and various organs.
Symptoms include shortness of breath, frequent lung infectious, poor growth and difficulty to gain weight.
Approximately 1,000 cases of cystic fibrosis are diagnosed internationally each year.
In October 2019, Trikafta was approved in the US as a tentative treatment to cystic fibrosis.
It is a triple combination therapy treating sufferers aged 12 and older which helps defective genes to work more appropriately.
In September 2020, Vertex, the drug provider, announced a trial of the drug on children aged 6-11 proved effective, as well.