RUTH Rogers says it has always been a family affair when it comes to raising money to help fund both research into Cystic Fibrosis and improving the quality of life for young children receiving treatment at the John Hunter Hospital.
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“It helps the unit purchase things they can’t afford, like portable DVD players or Xboxes, and even a refrigerator to store medicines in,” she explains.
“We also give money to the Arts program; it is wonderful and it gives children something to do while they are in hospital.”
But it was actually her daughter, who started the ball rolling not long after she received the devastating news both her children had a rare form of the disease.
However, due to the deteriorating health of her son, Myles, Karen Hobson had to hand the reigns over to her mum.
At that time he was a very sick little boy.
Ruth says his lung capacity had dropped to 42 per cent, he was at the doctor all the time and there was talk of Myles being put on the lung transplant list.
“It was a daunting thought; he was only 10 and-a-half years old.”
And things did not improve until he began taking the drug, Kalydeco.
Myles was a last minute inclusion in the drug trial, and the early signs were encouraging but now he has reached the end, the results are speaking for themselves.
An energetic Myles, who can’t sit still and now has a healthy appetite, says he has put on about 10 kilos, has grown 7cm and his lung capacity is sitting at around 92 per cent.
Ruth says prior to this he was lucky to grow one to two centimetres a year and could not put on weight.
In fact, before the trial the 12-year-old weighed 22.26 kilos despite eating 180g of fat per day then ingesting another 2000 calories per night via his PEG (feeding) tube.
This is because CF causes a thick-build up mucus in the lungs, pancreas and other organs.
The build-up decreases pancreatic function making it hard for sufferers to put on any weight and, ultimately, grow.
It also traps bacteria in the lungs, resulting in recurrent infections which lead to irreversible damage. Lung failure is the major cause of death for someone with CF.
Now, Kalydeco is available on the Pharmaceutical Benefits Scheme, his sister Emily is also taking the small blue “miracle” pill and benefitting from it.
The siblings were diagnosed within a week of each other; at the time Emily was three months old and Myles three-and-a-half.
Myles adds he has started back at hip-hop classes and can run around.
Thankfully, Emily has never had to stop her busy dancing schedule and hopefully, now, never will.
Her mum describes her as a dancing diva.
Kalydeco (ivacaftor) is the first medicine to treat the underlying cause of CF in people with gating mutations in the CFTR gene, like Emily and Myles.
Cystic fibrosis is a rare genetic disease for which there is no cure.
It is caused by a defective or missing CFTR protein, resulting from mutations in the CFTR gene.
In people with gating mutations, the CFTR protein reaches the cell surface but does not function properly.
The new drug, which is taken orally, is known as a CFTR potentiator and it helps the defective protein function more normally at the cell surface to help hydrate and clear mucus from the airways.
Approximately 250 Australians are eligible for the drug.
Although Karen is no longer in a constant state of panic, and can sleep at night without listening out for “that cough”, she is mindful the drug is still not a cure and more research needs to occur.
The young mum is also well aware the drug doesn’t work for all forms of the disease and knows a lot more money is still needed to sustain ongoing treatments for all sufferers.
“We never fundraise for personal gain, we only ever raise money for CF as it only gets a small amount of funding from the government,” she explains.
Cystic fibrosis is the most common, genetically acquired, life-shortening chronic illness affecting young people in Australia today.
In 2011, the average age of death due to CF was 27 years, and 20 per cent of those who died were under the age of 18.
One in 2500 babies in Australia, or one baby every four days, is born with CF.
Nearly half (47 per cent) of Australians with CF were hospitalised at least once in 2011 and of, these patients, approximately half (49 percent) spent at least 14 days in the hospital.
Ruth says this year’s family fun day is shaping up to be a big one despite an associated event being cancelled and some unexpected surgery in the lead up, which involved a bone graft.
Although the 2015 Cystic Fibrosis Postie Bike Challenge has been postponed until next year due to the harsh economic climate, the boys have still managed to make a contribution to her annual fundraising efforts.
“I am very grateful to have received a cheque for $3289 from the proceeds of the postie bike raffle the boys had at the Caledonian,” she says.
The family fun day starts at 10am and there will be a variety of activities on offer at the Singleton Golf Club.
From jumping castles and the Hunter Valley Zoo to a performance from The Voice’s Sarah De Bono and local stalls, as the name suggests Ruth guarantees it will be a “fun” day.
With proud mum Karen pointing out the Taylor Made Academy of Dance will also be taking to the stage.
And that her dancing diva, Emily, happens to be a member of the academy.