TEAMWORK: Evie Marshall's cystic fibrosis might rule her life but she has her devoted mum Sonia by her side every step of the way.
TEAMWORK: Evie Marshall's cystic fibrosis might rule her life but she has her devoted mum Sonia by her side every step of the way. Patrick Woods

Sonia's daily battle for the love of Evie

HAVING a child diagnosed with the chronic disease Cystic Fibrosis (CF) is "like being hit by a bus, then the bus reverses and runs over you again".

Every parent's life changes after they have their first child but Sunshine Coast mother Sonia Marshall was confronted with a full-time role of caring for her new daughter, Evie.

The overwhelming responsibilities led to the breakdown of her marriage, inability to work despite her PhD qualifications and learning how to take care of a child with little guidance or support and a condition she did not understand.

Every four days, a newborn is diagnosed with CF.

The incurable genetic condition manifests in various symptoms, including coughing, abdominal pain from a failing digestive system, difficulty breathing, permanent lung damage, difficulty exercising, chronic head pain, fatigue, repetitive lung infections, possible osteoporosis, infertility, developing CF-related diabetes, shortened life expectancy and an inability to gain weight, which can lead to requiring a feeding peg in the stomach.

 

 

Sophie and Evie in 2007, preparing for the Great Walk For Cystic Fibrosis at Mooloolaba.
Sophie and Evie in 2007, preparing for the Great Walk For Cystic Fibrosis at Mooloolaba. WARREN LYNAM/169852

Eleven years on from the diagnosis, Sonia's eldest daughter Evie undergoes an intensive annual treatment program of 15,500 tablets, 1100 hours of physiotherapy and nebulisers, 730 insulin injections and 3660 hours connected to her nutrition feed pump - approximately three hours of treatment per day.

"Everybody with CF is different. Evie is probably middle of the road. She's not on the lung transplant list, but they're not great," Sonia says.

"She does physiotherapy every day at home; it is probably the most time consuming and boring bit.

"I've been trained on how to do that and then when she has an admission in hospital, the physiotherapist does it twice a day and I do it once, so we bump it up to three times a day. We call it a tune up."

Sonia joined a group of Sunshine Coast mothers with CF children and initiated the charity marathon "65km for Cystic Fibrosis" to raise awareness and funds for the CF community.

"Through having a daughter with CF, I have met lots of other people, particularly children, families and young adults living with CF, I saw what they go through and I see what my daughter goes through.

 

 

Evie Marshall with her doggie and best friend Squirt in 2013.
Evie Marshall with her doggie and best friend Squirt in 2013. John McCutcheon

"It's really awful. It is such a horrible disease. I'm just the person who can't sit by and do nothing; I've got to do something about it. 65kms for Cystic Fibrosis is just one way of doing something about it and it also makes me feel very empowered."

Most of Sonia's time is taken up with looking after Evie and she is unable to work full-time, making it difficult to pay for various expensive medication.

"There's no break and it is hard because I can't work full-time.

"Things like owning a house or even paying rent, the cost of the medication and the equipment is pretty high as well.

"Even the travel costs of going from here to Brisbane and back again. Financially it's a real struggle and that's just ongoing."

Sonia even struggles to balance mundane household chores with Evie's medical needs.

"When Evie was little, she was too little to keep her in the house by herself but I couldn't take her out while I mowed the lawn because of all the dust and fungi and stuff in the air.

"It's a bit easier now that she's a bit older but it was really hard."

 

 

Huge volumes of drugs have always been needed to help Evie cope with her cystic fibrosis.
Huge volumes of drugs have always been needed to help Evie cope with her cystic fibrosis. Brett Wortman

She said Evie's father struggled with his daughter's diagnosis.

"He had a really hard time coping with the diagnosis so he spiraled into depression and couldn't go to work, couldn't get off the couch, couldn't help me with this newborn baby who had CF.

"It was just really unfortunate. I'm not bitter towards him or anything like that, it was just really difficult for me to handle."

The couple wanted a second child but knew that there was a one-in-four chance of it also having Cystic Fibrosis.

It was a difficult decision and they eventually had their second daughter, Liv, who was not born with CF.

The stress took its toll on the couple and they separated around seven years ago.

"That was really difficult. I think back and think if we didn't have a child with CF, would we still be together?" Sonia says.

"Maybe we would and maybe we wouldn't but it was certainly a factor in the family breakdown."

 

Two super heroes in 2013.
Two super heroes in 2013. John McCutcheon

Evie has had her own struggles, moving through her schooling years with a serious chronic illness.

"For her it's a big deal to get through a week of school. We always joke and say she goes to school part-time; it's like a part-time job.

"She goes when she can handle going."

Despite the peg in her stomach, frequent coughing and inability to maintain weight, Evie could easily be perceived as a healthy girl.

She is frequently bullied and told she "doesn't look sick" or "there's nothing wrong with you".

"She doesn't look sick, she looks fine," Sonia said.

"They just cannot see it and because we don't make a big deal about it, we don't whine about it and that's to our detriment some times to be honest.

"People come over to our house and Evie will just disappear for a moment and do a treatment and come back - it is not a big saga about having to do this or that."

Their unusual day-to-day life has become the norm to their immediate family but Sonia said many still did not understand.

"They don't understand how time consuming it is and how restricting it is.

"I get invited to a barbecue and at the last minute we have to cancel because Evie isn't feeling well, or an emergency happens and we end up in hospital or somebody at the barbecue has a really bad virus and we have to leave.

"There are a million things we do automatically and respond to automatically that other people just have no idea about."

 

LITTLE CUTIE: Evie Marshall, aged two, with her mum Sonia.
LITTLE CUTIE: Evie Marshall, aged two, with her mum Sonia. Brett Wortman/bw168665a

She said she felt "empowered" when she found support with a community of mothers who established 65km for Cystic Fibrosis.

"Everybody is different, every parent is different.

"Some parents prefer to stay out of the CF community. I was like that and now I've gone completely the other way.

"It is really great to connect with other parents. I feel like nobody gets it - it's a very socially isolating position to be in.

"People think they understand but they don't really walk in our shoes, they don't understand how hard it is.

"It's really important for me to connect with other people who walk in our shoes and have the same fears and desires and emotional rollercoasters."

Drugs that target specific genotypes are being developed and introduced in the Cystic Fibrosis community.

Orkambi targets Evie's genotype, Delta 508, which will in turn improve her lung function, but it does not have the economic justification to be placed on the Pharmaceutical Benefits Scheme (PBS).

 

 

Evie and mum in 2007.
Evie and mum in 2007. Brett Wortman/bw168665c

"The Pharmaceutical Benefits Committee and Minister for Health, Greg Hunt all said the same thing, it is what I read in the Pharmaceutical Benefits Advisory Committee (PBAC) outcomes as well.

"The price the drug company is asking for is really unachievable in the house budget and it's a small patient population but its still over a $100,000,000 of medication."

The PBAC saw the clinical benefits of Orkambi in the short-term but the long-term improvements were not demonstrated clearly based on what the drug company, Vertex, submitted as evidence.

The Marshalls have been discouraged by Vertex pricing the drug out of the market and not allowing it to be worth the investment, as other CF related drugs, such as Kalydeco, have been for Australia.

"It almost seems Vertex have been a bit slack and they haven't submitted the data for the PBAC to make a decision based off the price they are asking.

"We're urging Vertex, the PBAC and the Minister for Health to get cracking with it, get on with it and come to an agreement where patients can get access now as considerations of price continues.

"Every day is another day of damage for people with CF so why can't we have it now and you guys go away and go sort it out."

 

 

Evie all smiles in 2008 to promote the Great Strides Walkathon, a fundraiser for cystic fibrosis.
Evie all smiles in 2008 to promote the Great Strides Walkathon, a fundraiser for cystic fibrosis. Warren Lynam/178902

While others are currently experiencing the life changing effects of Vertex's creations, Sonia still holds out hope for Evie's future.

"There is so much hope with all these new drugs coming out of the woodwork everywhere and we just have to keep working hard, keep Evie's lungs and body in good shape and sometime within the next five to 10 years there will be something that will significantly change her life."


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