Cystic fibrosis survival lags in Australia

6 minute read

We trail behind France by more than a decade in life expectancy, thanks in part to transplantations and medication.

People with cystic fibrosis in France have a life expectancy that is more than 10 years higher than those in Australia, according to a country-to-country comparison in Thorax.  

The analysis of four countries with single-payer healthcare systems found that survival was highest in France, with a median survival age of almost 66 years, compared with Canada and New Zealand at 55, and Australia at 53. 

“Our study has shown significantly higher survival in France compared with countries with comparable healthcare systems,” Professor Scott Bell, a senior thoracic physician at the Prince Charles Hospital, and colleagues wrote.  

People with cystic fibrosis in France appeared to live the longest, despite having the highest proportion of underweight patients, they found.  

This may be due to differences in availability of lung transplantation, as France had the highest proportion of transplant recipients over the study period.  

But it was unlikely there was a single reason why one country had better survival rates than another, the authors said. Other than transplants, another factor could be differences in access to medications between the countries, they said.  

“France has an extensive, government-funded medication program that minimises barriers to accessing CF medications,” the authors wrote.   

“Such differential access to medications could explain differences in health outcomes as therapies have been linked to improved lung function and reduced exacerbations, both of which are closely associated with survival.” 

Professor Bell told TMR there were several potential reasons why survival was better in France.  

“Even though the groupings of the genotypes or the genetic variants in the French population looked, on average, much the same as the other three countries, there may be subtle differences or greater numbers of patients with milder gene variants,” the University of Queensland professor said. 

However, he said access to lung transplants was likely to be a key factor.  

“The proportion of patients from France who died after transplantation was almost 60%, versus just over 40% who died without a transplant,” he said. “Those percentages are roughly the opposite in Australia and Canada. 

“One of the ways of prolonging life in patients with end-stage lung disease in cystic fibrosis is to offer them a transplant. The elevation in survival seen in France is probably because of easier access to transplant that then prolongs life, on average, 10 years.” 

Professor Bell said the highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies became available in France earlier than in Canada and Australia, and much earlier than in New Zealand, where fewer modulators were available. 

The earlier administration of CFTR modulators to prevent long-term complications in younger children could greatly increase survival in the future, he said. 

Professor Bell said Australia was the first country to implement national newborn screening for cystic fibrosis, which led to improved nutrition and earlier diagnosis. 

Along with the evolution of sophisticated multidisciplinary cystic fibrosis care teams over the last 30 years, survival has improved from around 25 in the 1980s to around 50 now, he said. 

“The next step is around access to CF modulator drugs for younger patients for longer periods of time. I predict that we’ll see this survival rapidly increase over the next decade or so, because those patients with the most severe disease will live much longer than they have done previously as a result of taking the modulator drugs.” 

Perth cystic fibrosis expert Dr Anna Tai, who was not involved in the study, said the 10-year gap this robust study found between France and Australia and Canada was surprising.  

Dr Tai said that in addition to France’s better access to transplantation and medications, survival rates could also be affected by disparity in socioeconomics, ethnicity, mental health, patient adherence and resources between countries. 

Encouraging drug adherence in young patients was a challenge for clinicians, said the Sir Charles Gairdner Hospital respiratory physician.  

“This is further impacted by mental health burden and financial constraints,” she said.  

“Medical teams have the responsibility to monitor and support adherence to prescribed medications and identify and remove barriers to adherence. 

“Vigilant screening, diagnosis and management of mental health issues which affect adherence is a crucial part of CF care.”    

Dr Tai said the CFTR modulator elexacaftor/tezacaftor/ivacaftor (Trikafta) had been shown to significantly reduce exacerbations, reduce lung function decline and improve nutrition, and would likely lead to substantial improvements in survival. 

“As survival in CF improves, there will be a growing population of adults with CF who live with unique and complex physical and mental health care needs. 

“There is an ongoing, existing under-resourcing of CF care in Australia, particularly in adult CF care. This has now become a pressing issue in the setting of a rapidly expanding adult population with increasingly complex healthcare needs.” 

Meanwhile, an Australian study in the Journal of Cystic Fibrosis found that adults and children with cystic fibrosis had better clinical outcomes during the first 12 months of the covid pandemic. 

The study reported that virtual consultations increased from 8% to 47% after the pandemic began. There was also a 26% reduction in courses of intravenous antibiotics after the 12-month period, and an increase in the average number of consultations per patient from four to five. 

“In the 12 months following the onset of the covid-19 pandemic, restrictions on movement of people led to reduced community interaction, reduced circulating respiratory viruses and a change in model of care to incorporate more remote consultations,” the authors wrote. 

“The covid-19 pandemic forced a change in the CF model of clinical care delivery. Clinical outcomes, including lung function, BMI and hospitalisations for CF related issues were improved in the 12 months following the onset of the pandemic.”

The authors said a potential explanation for fewer pulmonary exacerbations was the reduction in exposure to respiratory viruses such as rhinovirus, RSV and influenza. 

Thorax 2022, online 15 September 

Journal of Cystic Fibrosis 2022, online 16 September 

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