Cystic fibrosis awareness month: Northland toddler one of 540 Kiwis living with CF

Nick Juretich and Bex Blatherwick with sons Coen and Liam.

For one week, Northland parents Bex Blatherwick and Nick Juretich were blissfully ensconced in their newborn family bubble oblivious it was about to be shattered.

That came with abnormal results of the routine heel prick test from their new baby son Liam with following tests confirming six weeks later that he had cystic fibrosis (CF).

"He seemed fine," said Bex. "We wouldn't have known anything different. I personally was in denial. We thought the following tests would come back negative and that he would just be a carrier."

The mother of two said she then started "freaking out" and researching a topic she knew nothing about.

"I had to get the midwife to write it down so I could search it."

The energetic, fun and fearless two-year-old takes Cystic Fibrosis in his stride and doesn't know any different.

What she learnt was that her baby had a serious and ultimately terminal genetic condition affecting around 540 Kiwis. It is caused by a faulty gene passed down from a baby's mum and dad and is usually diagnosed soon after birth. One in 25 people carry the faulty gene that causes it, often without knowing.

"I felt guilt as this is a genetic disease and we both have to be carriers to pass it on. We didn't know," said Bex.

People with CF secrete thick, sticky mucus in their bodies. The mucus in their lungs traps bacteria which can result in chronic infections, meaning that people with CF struggle with reduced lung function, progressive lung damage and possible respiratory failure. In the digestive system, CF reduces the amount of insulin produced and stops the digestive enzymes that aid digestion, leading to poor growth and physical weakness.

To combat this, daily routines are carried out and Bex and Nick soon adapted to a new norm. This entails Bex carrying out a 40-minute morning physio session to break up the mucous in Liam's lungs, involving patting his back and front, along with administering hypertonic saline nebuliser. If Liam gets sick, this is repeated in the evening. Throughout the day, he needs a diet high in fat and salt to maintain a healthy weight, along with enzymes to help digest the fat.

"I'm constantly looking on the back of packets to see how much fat is in them," said Bex. "I never thought I'd be one of those people.

Dargaville couple Nick Juretich and Bex Blatherwick's lives were turned upside-down when their son Liam was diagnosed with Cystic Fibrosis shortly after birth.

"But we have adapted to this way of living; weighing food, giving enzymes, physio and specialist appointments. I'm also always on the lookout for stagnant water if we are out as the bacteria would be harmful if he breathed it in."

She said hygiene is important to stop Liam from getting sick.

"He has frequent antibiotics and gets sore tummies often but Covid's been really good for that because a lot of people are aware of washing their hands. I've found this year we haven't had many illnesses."

To the observer, it is not obvious Liam lives with a condition facing a lifetime of treatments, frequent hospitalisation and a significantly reduced life expectancy. His mum said he takes it all in his stride because he doesn't know any different.

Two-year-old Liam is one of 540 Kiwis living with Cystic Fibrosis.

The energetic, fun and fearless two-year-old loves rough and tumble with his brother Coen, 4.

"They love each other's company."

This month is the Cystic Fibrosis Annual Appeal when New Zealanders are asked to help give Kiwis living with CF a brighter future. Living with CF is tough, time-consuming, and an emotional rollercoaster. It doesn't get easier for those affected. Life-saving medications such as Trikafta are still not funded in New Zealand and costs more than $450,000 per year. There is no cure, and only half of those with CF reach 31 years of age.

Cystic Fibrosis New Zealand (CFNZ) chief executive Jane Bollard said, "CF imposes a heavy burden, with significant quality of life, social and economic impacts on people with CF, their families, whānau and communities.

"Proceeds from the appeal will be used to advocate for access to Trikafta and other life-saving medications, provide counselling and emotional support, financial help during hospitalisations and transplants, and medical equipment to improve quality of life."

New Zealanders who have accessed Trikafta have experienced significant improvements in their health, in particular lung function, such as transitioning from the active lung transplant list to returning to school and participating in sports. Another has seen his lung function increase and is now able to return to work, run and think about starting a family.

Bex and Nick have been prolific fundraisers for CFNZ in the hope that one day their son will have access to the life-saving drug.

"We remember being told [with the diagnosis] technology is moving fast in Liam's generation and life-saving treatment won't be too far away. We are hoping by the time Liam is 12 [old enough to take Trikafta] that it will be available in New Zealand.

"We are very lucky to have a community like ours in Dargaville. Everyone seems to get behind a cause."

Last month the community raised $6020 for CFNZ at the Dargaville Bake-off where contestants paid a gold coin to enter their baking with the final from each week competing in the grand final, the day finishing with an auction with goods donated from local businesses. Last year, Bex took part in Sweatember, setting a goal of 10,000 steps for every day in September and raising $3700.

As well as selling chocolate fish, this Saturday they are holding a raffle and collection box for CF Awareness Month at Countdown Dargaville.

Donations can also be made online at www.cfnz.org.nz/donate, or by texting FUTURE to 4462 to make an instant $3 donation.

About Cystic Fibrosis

● There are over 540 Kiwis with CF. The condition is usually diagnosed through the heel prick test as part of newborn screening.

● People with CF secrete thick, sticky mucus in their bodies. The mucus in their lungs traps bacteria which can result in chronic infections, meaning that people with CF struggle with reduced lung function, progressive lung damage and possible respiratory failure. In the digestive system, CF reduces the amount of insulin produced and stops the digestive enzymes that aid digestion, leading to poor growth and physical weakness.

● The median age for life expectancy over the last seven years is 31, ie only half of those with CF lived to 31 or more. Life expectancy for a baby born today would be higher.

● Current treatments for most people with CF in New Zealand treat only the symptoms and not the underlying cause. These treatments include physiotherapy throughout the day to dislodge the mucus, taking nebulised treatments each day and many medications and supplements.

● CF modulators, such as Kalydeco and Trikafta, provide the first treatments to address the underlying cause of CF. Kalydeco is funded in New Zealand but only works for about 5 per cent of people with CF – it has provided significant benefits for the 30-plus eligible people in New Zealand. Trikafta works for about 90 per cent of those with CF and provides significant and immediate improvements by increasing lung function and weight gain, reducing the burden of treatment, improving quality of life, and increasing life expectancy.

About Cystic Fibrosis NZ

● CFNZ is the only charity in New Zealand dedicated to supporting and improving quality of life for people with CF and their families.

● CFNZ also funds CF research and advocates on behalf of the community for better access to services and care.

● CFNZ receives less than 5 per cent government funding and relies on the New Zealand public to fund its support service.