Fresh air for children with Cystic Fibrosis.


Supplied by CFWA

A child with cystic fibrosis using an eFlow nebuliser.

Joshua Smith, Reporter

A Perth-based biotech company has received over $20m in funding to develop a new treatment for children suffering from Cystic Fibrosis, more commonly referred to as CF.

The treatment is being developed by Respirion Pharmaceuticals, a spin-off company from Telethon Kids Institute, and will use an inhaled drug to target antibiotic-resistant infections that restrict breathing.

Matthew Casey, a spokesperson for Cystic Fibrosis WA (CFWA), said the treatment has already shown promising results in its early testing stage.

“The initial trial of the treatment has shown a significant lung function improvement in patients,” he said.

People who suffer from CF have a defective CFTR gene, which results in a lack of natural salt movement around the cells. Consequently, the body is unable to break down and get rid of phlegm and other mucus which builds up in the lungs and airways.

This can cause severe breathing problems for sufferers and requires constant chest physiotherapy in order to remove the excess mucus in the lungs. Therapy is continued throughout the patient’s life.

Medication, vitamins and salt replacements are also often taken via a nebuliser: a machine that turns liquid medicine into a vapour so that it can be inhaled through a mouthpiece.

Currently, treatments for children with CF can take half an hour or more every day, and sometimes several hours a day if the patient is on a course of antibiotics.

According to Casey, the aim of the new treatment is to more effectively provide the same kinds of medication that would ordinarily be inhaled.

“The Respirion treatment that has been announced will hopefully improve the efficacy of this type of nebulised treatment.”

At present, there is no cure for Cystic Fibrosis.

For sufferers, preventative measures are of the highest priority.

Scott Arnold-Eyers, who has a daughter with CF, said on top of the frequent treatments, one of the biggest challenges faced by his family is to avoid common illnesses.

Arnold-Eyers said: “If you catch the flu, for example, you’re going to have a lot of phlegm build up. But if someone with Cystic Fibrosis catches the flu from you, then they’re not going to be able to get rid of the phlegm.”

When asked for his advice on how to best avoid sickness, his answer was simple:

“Stay away from people who are sick.”

According to CFWA, the issue of cross-infection is often one of the most insidious parts of living with CF. Sufferers need to keep a small distance from one another, subsequently, they are unable to share their experiences and challenges face to face.

This issue has been highlighted by the newly-released film Five Feet Apart, which follows the story of two young adults with Cystic Fibrosis who want to be together but can’t be for risk of cross-infection.

In Australia, the recommended distance that people with Cystic Fibrosis keep from each other is four metres or 13 feet – roughly the length of a small car. As a result, CFWA have produced a range of online resources for young people to share their experiences and navigate the trickier parts of living with CF

One in 25 people in Australia suffer from CF and May is Cystic Fibrosis awareness month. Friday 24 May is CFWA’s 65 Roses Day in Australia where individuals can help raise funds for CF research by selling roses in their local community or gifting roses to be delivered on the day.

For more information about Cystic Fibrosis WA and to learn how you can get involved in raising funds on 65 Roses Day, click here.