respiratory
Cystic fibrosis

CF airborne cross-infection risk extended

Gram-negative bacteria and Staphylococcus aureus in the sputum of patients with cystic fibrosis can travel up to four metres during coughing and remain viable for up to 45 minutes. The findings suggest...

Gram-negative bacteria and Staphylococcus aureus in the sputum of patients with...

Read more
cystic-fibrosis

Parents want better communication when children are diagnosed with CF

Parents with an infant who has just been diagnosed with cystic fibrosis want facts – and don’t want to learn of the diagnosis over the phone. These are some of the findings from a survey of 26 parents at two...

Parents with an infant who has just been diagnosed with cystic fibrosis want facts – and don’t want to learn...
cystic-fibrosis

Call for more awareness of genetic carrier screening

One of Kirsty McConnell’s favourite memories of her youngest daughter Lily is her first time at the beach. The water is sparkling and cold and her big blue eyes widen as she’s carefully lowered into the water in a baby seat. She...

One of Kirsty McConnell’s favourite memories of her youngest daughter Lily is her first time at the beach. The water is...
cystic-fibrosis

New nutrition guidelines for cystic fibrosis

New nutritional guidelines for people with cystic fibrosis have advised against the routine use of complementary therapies, and highlighted a growing overweight and obesity problem among patients with the disease. The comprehensive 284-page guidelines also recommend a high salt diet...

New nutritional guidelines for people with cystic fibrosis have advised against the routine use of complementary therapies, and highlighted a...
cystic-fibrosis

CF drug knocked back for the third time

The Pharmaceutical Benefits Advisory Committee has rejected, for the third time, an application to have the potentially life saving cystic fibrosis treatment Orkambi listed on the Pharmaceutical Benefits Scheme (PBS). The drug’s manufacturer, Vertex, is seeking an Authority Required listing for...

The Pharmaceutical Benefits Advisory Committee has rejected, for the third time, an application to have the potentially life saving cystic...
cystic-fibrosis

Sweating on new tests (and therapies) for cystic fibrosis

A number of metabolites in the sweat of newborns screened positive for cystic fibrosis could provide the basis for complementary diagnostic tests to sweat chloride, according to Canadian research. As well as potentially improving the diagnosis of cystic fibrosis, the...

A number of metabolites in the sweat of newborns screened positive for cystic fibrosis could provide the basis for complementary...
cystic-fibrosis

Early combo treatment benefits kids with CF

Young children with cystic fibrosis treated with a combination of the CFTR-correcting therapies ivacaftor (Kalydeco) and lumacaftor (Orkambi) have seen rapid improvements in their symptoms in a phase III randomised controlled trial. The fixed dose combination treatment with lumacaftor (100mg) and ivacaftor...

Young children with cystic fibrosis treated with a combination of the CFTR-correcting therapies ivacaftor (Kalydeco) and lumacaftor (Orkambi) have seen rapid...
cystic-fibrosis

Access to cystic fibrosis drug widens

Children aged 2 to 5 living with cystic fibrosis will be able to access ivacaftor (Kalydeco) on the PBS, the federal government has announced. Prior to today’s announcement only children six years of age and older with the G551D or other...

Children aged 2 to 5 living with cystic fibrosis will be able to access ivacaftor (Kalydeco) on the PBS, the...
cystic-fibrosis

Mycobacterium spreading among CF patients globally

Whole genome sequencing of multi-drug resistant Mycobacterium abscessus isolates has shown the organism is spreading worldwide in the cystic fibrosis community possibly via cross-infection. The similarity between over 1,000 isolates from patients at cystic fibrosis (CF) clinics in Europe, the US...

Whole genome sequencing of multi-drug resistant Mycobacterium abscessus isolates has shown the organism is spreading worldwide in the cystic fibrosis...
cystic-fibrosis

Holy Grail: Understanding CF related diabetes

  Paediatric respiratory fellow Dr Bernadette Prentice tells us about her Holy Grail: to be able to detect which patients with CF will go on to develop diabetes at an earlier age. ...

  Paediatric respiratory fellow Dr Bernadette Prentice tells us about her Holy Grail: to be able to detect which patients with CF...
cystic-fibrosis

Pulmonary vascular disease predicts exacerbation risk in cystic fibrosis

Pulmonary artery enlargement, suggestive of pulmonary arterial hypertension, is very common in patients with cystic fibrosis and an independent marker of exacerbation risk, a Brisbane-based study has concluded. Writing in a paper published in the Lancet Respiratory Medicine the international study...

Pulmonary artery enlargement, suggestive of pulmonary arterial hypertension, is very common in patients with cystic fibrosis and an independent marker...
cystic-fibrosis

Evidence for CFTR combination therapy in advanced CF

Lumacaftor/ivacaftor combination therapy benefits patients with cystic fibrosis homozygous for the Phe508del CFTR mutation with moderate to severe lung dysfunction, a pooled analysis of data from the TRAFFIC and TRANSPORT studies shows. The research team including Dr Stuart Elborn from...

Lumacaftor/ivacaftor combination therapy benefits patients with cystic fibrosis homozygous for the Phe508del CFTR mutation with moderate to severe lung dysfunction,...
cystic-fibrosis

National approach needed for CF screening

Australia needs a national system to coordinate carrier screening, pregnancy screening and newborn screening for cystic fibrosis, says a leading Melbourne paediatric respiratory physician and researcher. Professor John Massie believes a disconnect between the states, the lack of national guidelines and...

Australia needs a national system to coordinate carrier screening, pregnancy screening and newborn screening for cystic fibrosis, says a leading...
cystic-fibrosis

Infants with CF have different nasal microbiota: study

Microbiota in the respiratory tract of infants born with cystic fibrosis are different to healthy infants even in the first year of life, the largest study to date reveals. Members of the Swiss Cystic Fibrosis Infant Lung Development (SCLID) study group...

Microbiota in the respiratory tract of infants born with cystic fibrosis are different to healthy infants even in the first...
cystic-fibrosis

Moving toward precision medicine with stem cells

Inducing the formation of pluripotent stem cells from a patient’s blood or skin cells provides the ultimate personalised tool for researching lung diseases including cystic fibrosis, delegates have heard in a session on precision medicine in lung disease. Presenting during a...

Inducing the formation of pluripotent stem cells from a patient’s blood or skin cells provides the ultimate personalised tool for...
cystic-fibrosis

Oral NSAIDs promising on paper, but CF clinicians wary

The release of a Cochrane review showing high dose oral NSAIDs can slow the progression of lung damage in people with cystic fibrosis is unlikely to change clinical practice, says a leading Australian respiratory physician and researcher. Associate Professor Sarath Ranganathan,...

The release of a Cochrane review showing high dose oral NSAIDs can slow the progression of lung damage in people...
cystic-fibrosis

Pseudomonas aeruginosa cells explode

  An international team of microbiologists have made a breakthrough in understanding how the superbug Pseudomonas aeruginosa causes infection. Associate Professor Cynthia Whitchurch, a microbiologist from UTS Science’s ithree Institute and colleagues found that Pseudomonas aeruginosa cells explode violently, a finding that...

  An international team of microbiologists have made a breakthrough in understanding how the superbug Pseudomonas aeruginosa causes infection. Associate Professor Cynthia...
cystic-fibrosis

Three questions on the long-term treatment of Staph infection in CF

Although methicillin-sensitive Staphylococcus aureus is a common cause of lung infection in people with cystic fibrosis there is not enough evidence to guide its long-term treatment, Cochrane reviewers say. A review of the literature revealed reports of long-term antibiotic treatment and successful...

Although methicillin-sensitive Staphylococcus aureus is a common cause of lung infection in people with cystic fibrosis there is not enough evidence to...
cystic-fibrosis

Hypertonic saline well tolerated, effective, in CF exacerbations

Hypertonic saline is well tolerated, improves lung function and speeds the resolution of symptoms in people with cystic fibrosis who have been hospitalised with a pulmonary exacerbation, an Australian randomised trial finds. Published in Thorax the trial led by physiotherapist...

Hypertonic saline is well tolerated, improves lung function and speeds the resolution of symptoms in people with cystic fibrosis who...
cystic-fibrosis

Cystic Fibrosis: A call for papers

The Lancet Respiratory Medicine is looking for high-quality submissions in the area of cystic fibrosis that will have an effect on the management of patients. This is...

The Lancet Respiratory Medicine is looking...
cystic-fibrosis

‘Window of opportunity’ for children with CF

A study exploring the use of the oral drug ivacaftor in very young children with a CFTR gating mutation suggests there may be a window in early life where at least partial restoration of pancreatic function might be possible. The ...

A study exploring the use of the oral drug ivacaftor in very young children with a CFTR gating mutation suggests...
cystic-fibrosis

Experts caution against using HbA1c as a screening tool in CF

Despite a large prospective study recently suggesting that HbA1c is effective as a screening tool for cystic fibrosis related diabetes (CFRD), a group of Australian experts have warned against its widespread use for now. In April this year a study published...

Despite a large prospective study recently suggesting that HbA1c is effective as a screening tool for cystic fibrosis related diabetes...
cystic-fibrosis

Heightened melioidosis risk in CF patients

Respiratory physicians have been warned to have a high index of suspicion for infection with Burkholderia pseudomallei - the cause of melioidosis – in patients with cystic fibrosis who have travelled in northern Australia or South-East Asia. Respiratory physician Dr James...

Respiratory physicians have been warned to have a high index of suspicion for infection with Burkholderia pseudomallei - the cause...
cystic-fibrosis

Guidelines address mental health in CF

Clinicians have been urged to systematically address mental health issues in people with cystic fibrosis to improve health outcomes. New guidelines developed by the International Committee on Mental Health in CF recommend that all people with CF receive an annual mental...

Clinicians have been urged to systematically address mental health issues in people with cystic fibrosis to improve health outcomes. New guidelines...
cystic-fibrosis

Task Force calls for more adult CF centres

There is an urgent need for more adult CF centres that are equipped to deliver multidisciplinary care addressing the complexity of the disease, concludes a report published by a joint Task Force from European Respiratory Society and the European Cystic...

There is an urgent need for more adult CF centres that are equipped to deliver multidisciplinary care addressing the complexity...
cystic-fibrosis

Some evidence for mannitol in CF: Cochrane

People with cystic fibrosis who are treated with inhaled dry powder mannitol over a six-month period have improvements in some measures of lung function, a Cochrane review concludes.   However there was no evidence to suggest...

People with cystic fibrosis who are treated with inhaled dry powder mannitol over a six-month period have improvements...
cystic-fibrosis

Lung clearance index could reduce reliance on CT

The lung clearance index can identify the extent of structural lung disease in children with cystic fibrosis and may prove helpful in reducing the reliance on CT imaging, Perth researchers have found. Professor Graham Hall, from the Telethon Kids Institute in...

The lung clearance index can identify the extent of structural lung disease in children with cystic fibrosis and may prove...
cystic-fibrosis

Release P. aeruginosa vaccine data: Cochrane

A Cochrane review that has found no evidence that Pseudomonas aeruginosa vaccine is effective in patients with cystic fibrosis has called for unpublished data to be released. The Cystic Fibrosis and Genetic Disorders Group said results from...

A Cochrane review that has found no evidence that Pseudomonas aeruginosa vaccine is effective in patients with cystic fibrosis has called...
cystic-fibrosis

Route of antibiotics in CF open to debate: Cochrane

There is no evidence to suggest that any route of antibiotic administration is superior to any other in the treatment of exacerbations in people with CF, concludes a Cochrane review. "While there is unlikely to be...

There is no evidence to suggest that any route of antibiotic administration is superior to any other...
cystic-fibrosis

Gene study paves the way for other trials: Expert

The findings of a recent gene therapy trial in CF may have been modest but it represents years of work that will lay the path for other trials, an expert says. The randomised double blind placebo controlled phase 2 study of...

The findings of a recent gene therapy trial in CF may have been modest but it represents years of work...
cystic-fibrosis

P aeruginosa travels between CF centres

The movement of patients between cystic fibrosis centres is an important risk factor for acquiring P aeruginosa strain infections, Australian researchers say. Led by Scott Bell from the Child Health Research Centre at The University of Queensland, Brisbane...

The movement of patients between cystic fibrosis centres is an important risk factor for acquiring P aeruginosa strain infections, Australian researchers...
cystic-fibrosis

Another step along the path of translational gene therapy

A proof of concept trial has shown that non-viral gene therapy can stabilise FEV1 in cystic fibrosis patients. The results provide "another step along the path" of translational cystic fibrosis gene therapy and should encourage the rapid introduction of more potent...

A proof of concept trial has shown that non-viral gene therapy can stabilise FEV1 in cystic fibrosis patients. The results provide...
cystic-fibrosis

Take control sample before bronchoscopy

Taking a control sample before bronchoalveolar lavage might be a good idea say Irish respiratory physicians after finding contamination in a bronchoscope that was considered clean. Writing in a letter to Thorax the researchers from several hospitals in Ireland detail...

Taking a control sample before bronchoalveolar lavage might be a good idea say Irish respiratory physicians after finding contamination in...
cystic-fibrosis

One size does not fit all for IV antibiotic duration

ECFS delegates voted a resounding 'NO' during a head to head debate on whether the duration of IV antibiotics should be 14 days for all cystic fibrosis patients. Leading a very convincing 'NO' debate Barry Plant from Cork in Ireland noted...

ECFS delegates voted a resounding 'NO' during a head to head debate on whether the duration of IV antibiotics should...
cystic-fibrosis

High-dose Vit D maintains levels in winter

A one-off oral high dose of vitamin D is effective in maintaining vitamin D levels during the winter months in children with CF, researchers from Melbourne report. The study included 41 children with CF who were treated with a single supra-physiological...

A one-off oral high dose of vitamin D is effective in maintaining vitamin D levels during the winter months in...
cystic-fibrosis

Ivacaftor improves nutritional status

Treatment with ivacaftor can lead to weight gain in CF patients with a G551D mutation, a small Australian study shows. Weight gain was significantly related to improvement in fat intake at 16 weeks and younger age at 34 weeks, the open...

Treatment with ivacaftor can lead to weight gain in CF patients with a G551D mutation, a small Australian study shows. Weight...
cystic-fibrosis

Mild anxiety can benefit CF patients

Mild symptoms of anxiety can actually drive adherence to inhaled therapy, researchers have discovered. Patients who reported mild symptoms of anxiety were less likely to report adherence issues with inhaled antibiotic treatment compared to patients with no anxiety or elevated levels of...

Mild symptoms of anxiety can actually drive adherence to inhaled therapy, researchers have discovered. Patients who reported mild symptoms of anxiety...
cystic-fibrosis

Pseudomonas infection ‘scary’

A fear of acquiring Pseudomonas aeruginosa causes considerable anxiety to a large number of CF patients and their families, a survey reveals. Concerns around how infection would increase the burden of treatment impact daily life were particularly widespread, the survey of almost...

A fear of acquiring Pseudomonas aeruginosa causes considerable anxiety to a large number of CF patients and their families, a survey...
cystic-fibrosis

Four challenges in the next five years: Elborn

In the closing plenary to wrap up this year’s conference outgoing ECFS President Stuart Elborn gave an insightful talk outlining what he thinks are the biggest challenges in treating and managing CF over the next five years. Challenges according...

In the closing plenary to wrap up this year’s conference outgoing ECFS President Stuart Elborn gave an insightful talk outlining...
cystic-fibrosis

CF doctors will need to become “super generalists”

Adult CF doctors will need to be come “super generalists” if they are to effectively manage the complexities of the disease that comes with an increasing life expectancy, experts have said. In a session held today on Growing old with CF...

Adult CF doctors will need to be come “super generalists” if they are to effectively manage the complexities of the...
cystic-fibrosis

CFTR modulation improves bacterial clearance

Addressing CFTR dysfunction improves overall bacterial clearance from sputum, according to research presented here at ECSF 2015. The study looked at the short and long term effects of ivacaftor on the sputum microbiota of 12 adult patients with at least one...

Addressing CFTR dysfunction improves overall bacterial clearance from sputum, according to research presented here at ECSF 2015. The study looked at...
cystic-fibrosis

Going beyond BAL for surveillance

BAL surveillance has taught us a lot about early drivers of CF lung disease but there’s an urgent need to find less invasive biomarkers of pulmonary inflammation, the conference has heard. Taking the audience through the results from AREST CF study...

BAL surveillance has taught us a lot about early drivers of CF lung disease but there’s an urgent need to find...
cystic-fibrosis

End-of-life planning essential in CF

Despite an improving life expectancy cystic fibrosis is still a life-limiting non-curable illness that requires end of life planning, says Lieven Dupont from Leuven in Belgium. There were many barriers to having an end-of-life discussion with patients, Lieven told delegates attending...

Despite an improving life expectancy cystic fibrosis is still a life-limiting non-curable illness that requires end of life planning, says...
cystic-fibrosis

CF patients not consuming enough fruit and veg

CF patients have a diet that is lacking in fruit and veg compared to the general population, an Australian study reveals. The analysis of diets of 24 adult outpatients attending Alfred Health in Melbourne found no patient met the recommended serve...

CF patients have a diet that is lacking in fruit and veg compared to the general population, an Australian study...
cystic-fibrosis

Manage physical and mental health together

Cystic Fibrosis centres need to offer patients comprehensive care that can treat the entire spectrum of disease associated complications including mental health, the conference has heard. Presenting to delegates in a session on standards of care: building a bridge to the...

Cystic Fibrosis centres need to offer patients comprehensive care that can treat the entire spectrum of disease associated complications including...
cystic-fibrosis

In varietate concordia

This winner of this year's ECFS award is Dr Carlo Castellani from the Verona Cystic Fibrosis Centre. Accepting his award at last night's opening plenary session Castellani told delegates he chose the title of his acceptance speech In varietate concordia (united in...

This winner of this year's ECFS award is Dr Carlo Castellani from the Verona Cystic Fibrosis Centre. Accepting his award at...
cystic-fibrosis

Drug combo a “game-changer” for cystic fibrosis

Lumacaftor in combination with ivacaftor provides clinically important reductions in the rate of pulmonary exacerbations in patients with cystic fibrosis homozygous for the Phe508del CFTR mutation, new ground-breaking research shows. The phase 3 randomised double blind placebo controlled TRAFFIC and...

Lumacaftor in combination with ivacaftor provides clinically important reductions in the rate of pulmonary exacerbations in patients with cystic fibrosis...
cystic-fibrosis

Genetically modified human embryo raises ethical concerns

It all started with a rumour. Then just six weeks ago, a warning rang out in the scientific journal Nature, expressing “grave concerns regarding the ethical and safety implications” of creating the world’s first genetically-modified human embryo. Then last...

It all started with a rumour. Then just six weeks ago, a warning rang out in the scientific journal...
cystic-fibrosis

Stop using the term ‘non-CF bronchiectasis’

Clinicians and researchers need to stop describing bronchiectasis by what it is not and instead embrace the name it has held for over 150 years, say respiratory...

Clinicians and researchers need to stop describing bronchiectasis by what...
cystic-fibrosis

Two blows better than three

A third forced spirometry blow is unnecessary in some patients say researchers whose study findings challenge existing guidelines. The study of 49 cystic fibrosis patients and 64 lung transplant patients compared the best FEV1 measurement from trials 1 and 2 with trial...

A third forced spirometry blow is unnecessary in some patients say researchers whose study findings challenge existing guidelines. The study of...
cystic-fibrosis

Lung disease can be measured in under 6’s: PRAGMA-CF

Clinical trials involving very young children with cystic fibrosis may now be possible as researchers from Western Australia have developed a CT outcome measure that is sensitive to detecting the early progression of lung disease. Chest CT is the gold standard...

Clinical trials involving very young children with cystic fibrosis may now be possible as researchers from Western Australia have developed...
cystic-fibrosis

Increasing CF survival age may impact care

The number of people with cystic fibrosis living into adulthood is expected to increase 75% by 2025, a life expectancy forecast shows. While this is clearly good news it could have an impact on patient care, say the...

The number of people with cystic fibrosis living into adulthood is expected to increase 75% by 2025, a life expectancy...