Parents of children with cystic fibrosis live in constant fear their children may contract life-threatening, difficult-to-treat lung infections – but cutting-edge work being undertaken by Wal-yan Respiratory Research Centre researchers is offering new hope.
The word ‘pseudomonas’ lives, rent-free, in the back of Perth mum Lisa Hopkins’ mind pretty much 24/7.
Lisa’s 9-year-old daughter Emma lives with cystic fibrosis (CF) and the prospect of her contracting pseudomonas – a harmful bacteria that causes serious lung infections in children with CF and can cause irreversible lung damage – is one of Lisa’s most persistent fears.
Just as scary is the idea that the medications families like the Hopkins rely on so heavily to keep their children healthy may one day not work.
“We’re always worried about Emma’s risk of developing an infection – particularly with stagnant water at the park, swimming pool, weekends away camping, in the school yard – it’s constant,” Lisa said.
“She’s been on antibiotics since she was six weeks old and fortunately has only been admitted for IV treatments a couple of times, but we are scared about antibiotic resistance.
“It’s a constant fear every time she becomes unwell. As a parent I don’t know how many antibiotics the doctors have up their sleeves to treat these infections and whether they’ll work.”
But knowing there is cutting-edge research on a new treatment – happening right here in Perth – gives Lisa hope.
The work taking place in the The Kids lab is bacteriophage therapy (more commonly known as phage therapy), which involves turning predatory phage viruses against bacteria like pseudomonas. Already showing great success in the lab, the treatment could improve the quality and length of life for children, including Emma, who live with CF.
“It’s great to see people like Associate Professor Anthony Kicic using their passion to help people like Emma,” Lisa said.
“If you’re being told current treatments aren’t working, you feel like you’re at a loss. But phage therapy will literally be a lifesaver for so many people with CF and their families. It’s such a game changer. It gives us hope and reduces our stress levels – it is reassuring to know the researchers are working hard to develop another weapon in the tool belt.”
The Hopkins family consider themselves lucky that Emma is healthy and has not, at this stage, developed pseudomonas. However, in most cases, colonisation with this bacterium is inevitable. For some children it can sadly develop in the first few months of life.
Treating pseudomonas is multi-faceted and includes at least two weeks in hospital for IV antibiotics, as well as lung physiotherapy.
To reduce the treatment burden, the aim is to administer phage therapy in a similar way to an asthma puffer.
“It would be life-changing to have medication that is less taxing on Emma’s body,” Lisa said.
“The medications we make them go through are not pleasant – you feel so bad. You’re telling them ‘I’m sorry, but you have to do this’. You want to stop the pain, but they have to go through it to stop the infection.
“With IV antibiotics, we run the risk of damaging her veins and there is also a high risk of wound infection – so a puffer treatment would be so much less risk and less invasive.
“With the amount of trauma kids with CF experience, not to mention time away from school and friends, to have something that doesn’t require long stints in hospital would be incredible.”
Lisa said the family were big supporters of the research being conducted at the Wal-yan Respiratory Research Centre, with Emma involved in several CF research projects and clinical trials, including AREST CF.
“We try and be a part of any clinical trial that we reasonably can be. We explain to Emma that we are helping be part of the process to find a cure, just as the people before her helped develop enzymes.”