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Cystic Fibrosis

Trikafta’s second PBAC deferral disappoints CF advocates

A decision on PBS listing for Trikafta (elexacaftor/tezacaftor/ivacaftor) for treatment of cystic fibrosis has been deferred for a second time by the Pharmaceutical Benefits Advisory Committee (PBAC). At a May intracycle meeting the PBAC again deferred on making a recommendation...

A decision on PBS listing for Trikafta (elexacaftor/tezacaftor/ivacaftor) for treatment of cystic fibrosis has been deferred for a second time...

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cystic-fibrosis

High prevalence of asymptomatic C. diff colonisation in CF

People with cystic fibrosis have a high prevalence of asymptomatic carriage of toxigenic C. difficile, an Australian study has shown. However, while 30% of asymptomatic CF patients tested at a WA centre had toxigenic C. difficile colonisation, there was no evidence...

People with cystic fibrosis have a high prevalence of asymptomatic carriage of toxigenic C. difficile, an Australian study has shown. However,...
cystic-fibrosis

Inflammation may persist in kids with early CF despite P. aeruginosa eradication

Anti-inflammatory approaches are needed in early CF because some children have a propensity for persistent inflammation and bronchiectasis progression despite successful P. aeruginosa eradication, Australian research shows. A study involving 88 infants enrolled in the Australian Respiratory Early Surveillance Team for...

Anti-inflammatory approaches are needed in early CF because some children have a propensity for persistent inflammation and bronchiectasis progression despite...
cystic-fibrosis

Benefits of cystic fibrosis combination therapies maintained in the long-term: trials

Treatment with lumacaftor-ivacaftor (Orkambi) was tolerated well and maintained its efficacy in children aged 6-11 years out to 120 weeks, according to long-term trial data. Previous trials have demonstrated success with the combination therapy in children homozygous for the F508del-CFTR mutation...

Treatment with lumacaftor-ivacaftor (Orkambi) was tolerated well and maintained its efficacy in children aged 6-11 years out to 120 weeks,...
cystic-fibrosis

Can CFTR therapy SIMPLIFY the treatment burden in CF?

Two concurrent new clinical trials will examine the feasibility of discontinuing the chronic therapies hypertonic saline or dornase alfa in patients with cystic fibrosis who have begun therapy with the elexacaftor/tezacaftor/ivacaftor (ETI) combination. The combination CF transmembrane regulator (CFTR) modulator therapy...

Two concurrent new clinical trials will examine the feasibility of discontinuing the chronic therapies hypertonic saline or dornase alfa in...
cystic-fibrosis

Study supports use of ivacaftor in first year of life for children with CF

The CFTR modulator therapy ivacaftor (Kalydeco) may be safely used in the first year of life to treat the underlying cause of cystic fibrosis, latest results from the ARRIVAL study suggest. Currently listed on the PBS for use in children...

The CFTR modulator therapy ivacaftor (Kalydeco) may be safely used in the first year of life to treat the underlying...
cystic-fibrosis

Reproductive screening for CF recommended for Medicare funding

Reproductive carrier screening for cystic fibrosis (CF) should listed on the MBS, the Medical Services Advisory Committee (MSAC) has advised the Health Minister, Greg Hunt. The recommendation was made in response to an application by the Royal College of Pathologists...

Reproductive carrier screening for cystic fibrosis (CF) should listed on the MBS, the Medical Services Advisory Committee (MSAC) has advised...
cystic-fibrosis

8 predictors of lung function trajectory revealed by Australian CF registry

Decline in FEV1pp among people with CF is predicted by eight demographic and clinical predictor variables, an analysis of the Australian Cystic Fibrosis Data Registry has found. Using data from 3655 patients in the registry with a median of 21.7 years...

Decline in FEV1pp among people with CF is predicted by eight demographic and clinical predictor variables, an analysis of the...
cystic-fibrosis

We’re not going back to how it was before: how respiratory teams adapt to COVID-19

Emma Wilkinson talks to respiratory healthcare professionals in the UK about how they have adapted their services to the COVID-19 pandemic - and how they have discovered that the new ways of working are often an improvement on ‘traditional models’ Now...

Emma Wilkinson talks to respiratory healthcare professionals in the UK about how they have adapted their services to the COVID-19...
cystic-fibrosis

CF patients with liver disease at high risk of bone loss and diabetes

Patients with CF-related liver disease have a worse disease burden than other CF patients - including more hospitalisations and being at higher risk of other complications such as bone disease and diabetes. A study from the Australian CF Data Registry...

Patients with CF-related liver disease have a worse disease burden than other CF patients - including more hospitalisations and being...
cystic-fibrosis

Few reported cases of COVID-19 in cystic fibrosis patients

Early data from Europe suggests that patients with cystic fibrosis are doing an exceptional job of avoiding SARS-CoV-19 infection but there is no room for complacency, experts warn. In a letter to Lancet Respiratory...

Early data from Europe suggests that patients with cystic fibrosis are doing an exceptional job of avoiding SARS-CoV-19...
cystic-fibrosis

Disease modulators are not the end of the story in cystic fibrosis, experts warn

Disease modulator drugs are a major step forward in the treatment of cystic fibrosis but increasingly wider availability “won’t turn the clock back” for the growing numbers of adult patients whose lungs have been damaged by the disease, respiratory physicians...

Disease modulator drugs are a major step forward in the treatment of cystic fibrosis but increasingly wider availability “won’t turn...
cystic-fibrosis

Novel therapy success for ‘untreatable’ P. aeruginosa in CF lung transplant patient

Doctors in Queensland have successfully treated a life-threatening P. aeruginosa infection in a post–lung transplant patient with cystic fibrosis by using used plasmapheresis to eliminate inhibitory IgG2 antibodies that were blocking the effects of antibiotics. The 33-year old man Jordan Trieger had...

Doctors in Queensland have successfully treated a life-threatening P. aeruginosa infection in a post–lung transplant patient with cystic fibrosis by using...
cystic-fibrosis

Symdeko and Orkambi get PBS subsidies for CF patients

The combination therapy for cystic fibrosis  - tezacaftor with ivacaftor (Symdeko) - is to be PBS reimbursed for patients 12 years and older who are homozygous for the F508del  mutation in the CFTR gene. Symdeko will also be made available for people...

The combination therapy for cystic fibrosis  - tezacaftor with ivacaftor (Symdeko) - is to be PBS reimbursed for patients 12...
cystic-fibrosis

Children with bronchiectasis deserve MDT model of care clinics

Children with bronchiectasis should have access to dedicated multidisciplinary team (MDT) clinics similar to those routinely provided for children with cystic fibrosis (CF), NSW clinicians say. A study of care provided for 22 children with bronchiectasis at a tertiary centre...

Children with bronchiectasis should have access to dedicated multidisciplinary team (MDT) clinics similar to those routinely provided for children with...
cystic-fibrosis

Sniffer dogs can detect CF pseudomonas infections

Sniffer dogs are able to detect the presence of Pseudomonas aeruginosa at very low levels, opening up the possibility for their use in screening and early antibiotic treatment to prevent lung...

Sniffer dogs are able to detect the presence of Pseudomonas aeruginosa...
cystic-fibrosis

Kalydeco listed on PBS for children aged 12-24 months

Respiratory medicine specialists have welcome the announcement that ivacaftor (Kalydeco) will be subsidised on the PBS for children aged 12-24 months with cystic fibrosis (CF) from 1 August. The drug is currently subsidised for about 280 patients over two years of...

Respiratory medicine specialists have welcome the announcement that ivacaftor (Kalydeco) will be subsidised on the PBS for children aged 12-24...
cystic-fibrosis

PBS moves for COPD inhalers and new CF treatments

In a major change for corticosteroid/LABA inhalers for COPD, the Pharmaceutical Benefits Advisory Committee (PBAC) has recommended removal of the requirement for patients to have a FEV1 less than 50% of predicted normal prior to therapy. At its March meeting the...

In a major change for corticosteroid/LABA inhalers for COPD, the Pharmaceutical Benefits Advisory Committee (PBAC) has recommended removal of the...
cystic-fibrosis

CMV linked to lung damage in CF

Cytomegalovirus (CMV) may have an impact on disease progression in cystic fibrosis (CF) leading to earlier lung transplant and increased mortality. A Canadian study, published in the European Respiratory Journal, found that CMV seropositive patients with CF who received a...

Cytomegalovirus (CMV) may have an impact on disease progression in cystic fibrosis (CF) leading to earlier lung transplant and increased...
cystic-fibrosis

New CF drug gets TGA approval

The TGA has approved a new cystic fibrosis treatment tezacaftor/ivacaftor (Symdeko) for patients aged 12 and older. Manufacturer Vertex announced  on 12 March that its drug (tezacaftor 100 mg/ivacaftor 150 mg tablet and ivacaftor 150 mg tablets) had been ...

The TGA has approved a new cystic fibrosis treatment tezacaftor/ivacaftor (Symdeko) for patients aged 12 and older. Manufacturer Vertex announced...
cystic-fibrosis

CRP levels can’t guide antibiotics in CF

C-reactive protein (CRP) levels are not a good marker of acute respiratory infections in patients with CF and will be of little use in guiding antibiotic use, a Victorian study has found. While CRP levels have been used as a...

C-reactive protein (CRP) levels are not a good marker of acute respiratory infections in patients with CF and will be...
cystic-fibrosis

CFTR modulator triple therapy offers ‘breakthrough’ for 90% of CF patients

Positive early results with new generation CFTR corrector triple therapy are a “major breakthrough” for most cystic fibrosis patients, researchers say. Published in the NEJM, two parallel trials of triple therapy regimens of the small-molecule CFTR correctors VX-659 or VX445 in...

Positive early results with new generation CFTR corrector triple therapy are a “major breakthrough” for most cystic fibrosis patients, researchers...
cystic-fibrosis

Indeterminate role of Aspergillus in CF, Australasian study finds

About 18% of young children with cystic fibrosis are returning BAL cultures positive for Aspergillus species although there seems to be no association with FEV1% predicted or with lung function decline into adolescence. Data from 156 patients in the Australasian Cystic...

About 18% of young children with cystic fibrosis are returning BAL cultures positive for Aspergillus species although there seems to...
cystic-fibrosis

GLP-1 drug could be better alternative to insulin for CF-related diabetes

The GLP-1 receptor agonist exenatide may be a better alternative to insulin for the management of cystic fibrosis-related diabetes (CFRD), a pilot study carried out by respiratory physicians and endocrinologists in South Australia suggests. In a study involving six young...

The GLP-1 receptor agonist exenatide may be a better alternative to insulin for the management of cystic fibrosis-related diabetes (CFRD),...
cystic-fibrosis

Promising therapies and overlooked problems in CF management

There are both promising therapies and emerging problems in the future management of cystic fibrosis, according to a UK paediatric respiratory physician. Dr Ian Balfour-Lynn, director of paediatric CF at the Royal Brompton Hospital, told ERS 2018 that phase 2 studies...

There are both promising therapies and emerging problems in the future management of cystic fibrosis, according to a UK paediatric...
cystic-fibrosis

Why it’s time to rebut milk-mucus myth

The widely held belief that drinking milk promotes excessive mucus production in the lungs needs to be rebutted, a UK paediatric respiratory specialist says. Dr Ian Balfour-Lynn, of the Royal Brompton Hospital in London, says his department's staff are constantly told by...

The widely held belief that drinking milk promotes excessive mucus production in the lungs needs to be rebutted, a UK paediatric...
cystic-fibrosis

Could lower airway microbiota serve as a biomarker for CF progression?

Young adults with cystic fibrosis with low microbiome diversity in their sputum have a greater risk of early end-stage lung disease, a novel study finds. According to the researchers from the Canada their findings could be used to ...

Young adults with cystic fibrosis with low microbiome diversity in their sputum have a greater risk of early end-stage...
cystic-fibrosis

CF research wins top science prize

Cough research by a team of scientists and clinicians from Queensland has taken out one of Australia’s top science awards  - the Eureka Prize - for their pivotal work in reducing transmission of pathogen in cystic fibrosis. The CF Air team, led...

Cough research by a team of scientists and clinicians from Queensland has taken out one of Australia’s top science awards  -...
cystic-fibrosis

Be vigilant for cryptococcal infection in lung transplant patients

Immunosuppression and environmental exposures mean that cryptococcal infection is an ongoing risk for lung transplant recipients, according to the authors of a major case series from Victoria. Already known to be one of the most common invasive fungal infections in solid...

Immunosuppression and environmental exposures mean that cryptococcal infection is an ongoing risk for lung transplant recipients, according to the authors...
cystic-fibrosis

Fourth time lucky for CF drug’s PBS listing bid

After being knocked back three times for PBS listing, the cystic fibrosis treatment lumacaftor with ivacaftor (Orkambi) has received a positive recommendation from the Pharmaceutical Benefits Advisory Committee At its July meeting the PBAC made  a recommendation for a Section...

After being knocked back three times for PBS listing, the cystic fibrosis treatment lumacaftor with ivacaftor (Orkambi) has received a...
cystic-fibrosis

OPTIMIZE trial: long term azithromycin preserves lung function in CF children

The longest randomised controlled trial of azithromycin treatment in children with cystic fibrosis has shown a 44% reduction in exacerbations over 18 months. The OPTIMIZE trial conducted in 221 US children with CF aged 6 months-18 years with recent Pseudomanas...

The longest randomised controlled trial of azithromycin treatment in children with cystic fibrosis has shown a 44% reduction in exacerbations...
cystic-fibrosis

Prolonged wear of face masks protects CF patients from cross infection

Surgical face masks prevent person-to-person cough transmission of cystic fibrosis pathogens when worn for forty minutes, Queensland respiratory specialists have shown. While paper masks have previously been shown to prevent the spread of Pseudomonas aeruginosa aerosols during short term wear (ten...

Surgical face masks prevent person-to-person cough transmission of cystic fibrosis pathogens when worn for forty minutes, Queensland respiratory specialists have...
cystic-fibrosis

Calling time on length of antibiotic treatment for exacerbations in CF

Adult cystic fibrosis patients who need antibiotics for a pulmonary exacerbation may be at risk of overtreatment and undertreatment if the duration of treatment is stipulated in guidelines, an expert warns.Writing in a comment piece published in the...

Adult cystic fibrosis patients who need antibiotics for a pulmonary exacerbation may be at risk of overtreatment and undertreatment...
cystic-fibrosis

PPI use linked to lung disease progression in children with CF

Proton pump inhibitors (PPI) use has been identified as a risk factor for future exacerbations and accelerated decline in FEV1 in children with cystic fibrosis. The findings, from a Dutch longitudinal observational study involving 545 children with CF aged 5-18 years...

Proton pump inhibitors (PPI) use has been identified as a risk factor for future exacerbations and accelerated decline in FEV1...
cystic-fibrosis

Ivacaftor ‘window of opportunity’ for CF treatment in children under two

Ivacaftor (Kalydeco) appears safe and effective in treating cystic fibrosis in children under two and its early use may help preserve pancreatic function, according to results from a phase 3 trial involving two Australian centres. Currently listed on the PBS for...

Ivacaftor (Kalydeco) appears safe and effective in treating cystic fibrosis in children under two and its early use may help...
cystic-fibrosis

Why macrolides should remain a last resort in respiratory patients

Macrolide antibiotics are an important growth area in prescribing for respiratory disease but should be kept as back-up therapy, delegates at the British Thoracic Society Summer Meeting have heard. Ten years ago almost no respiratory patients would have been on long...

Macrolide antibiotics are an important growth area in prescribing for respiratory disease but should be kept as back-up therapy, delegates...
cystic-fibrosis

Respiratory physicians in Queen’s Birthday Honours List

Several prominent respiratory and sleep medicine clinicians have been recognised in the 2018 Queen’s Birthday Honours list.Professor Christine McDonald, Director of the Department of Respiratory and Sleep Medicine at Austin Health, Melbourne, is named as a Member in the General...

Several prominent respiratory and sleep medicine clinicians have been recognised in the 2018 Queen’s Birthday Honours list.Professor Christine McDonald, Director...
cystic-fibrosis

The great debate: can telemedicine reduce infections in CF?

Telehealth approaches to patients with cystic fibrosis (CF) may not just be a solution to the tyranny of distance in a big country but can also help minimise infections, an Australian paediatric respiratory physician argues. In an ATS 2018 debate about...

Telehealth approaches to patients with cystic fibrosis (CF) may not just be a solution to the tyranny of distance in...
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...
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...