Diagnosis of cystic fibrosis in adults: Australian Cystic Fibrosis Data Registry data, 2000–2019

Lin, Amelia Alice; Wong, Kai; Visser, S.; Jo, Helen; Al-Hindawi, Y.; Kosbab-Jackson, Katrin; Cocks, Molly; Volovets, Anastasia; Haber, Paul; Hammond, Kirsten J. L.; Taylor, Nicole; Yozghatlian, V.; Lau, Edmund; Marshall, Nathaniel S.; Aquino-Salomon, Tara; Sivam, Sheila

doi:10.5694/mja2.51797

Cited by 2 publications

(4 citation statements)

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“…Cystic fibrosis (CF) is one of the most prevalent inherited genetic disorders in Australia and it is estimated that the number of individuals living with CF will rise by 70.0% by 2025 in specific global regions 1,2 . Data from the Australian CF Data Registry (ACFDR) currently records 3538 individuals living with CF, indicating an incidence of 1 in 2500 births [3][4][5] . CF is a multi-organ condition primarily characterised by pulmonary infections leading to progressive reduction in lung function 1,2 .…”

Section: Introductionmentioning

confidence: 99%

Cystic Fibrosis Australia and Phage Australia survey: Understanding clinical needs and attitudes towards phage therapy in the CF community

Lynch,

Sinclair,

Khatami

et al. 2024

Preprint

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Cystic fibrosis (CF) is the most prevalent serious inherited disease in Australia, imposing significant health risks. CF is characterised by chronic lung inflammation and recurrent pulmonary infections that increase morbidity and premature mortality rates. The emergence of antimicrobial resistance (AMR) places further challenges on the treatment and management of CF, necessitating research into alternative strategies for treatment of bacterial infections . Bacteriophage therapy, involving bacterial-specific viruses, is a potential avenue for AMR infections in patients with CF. Existing literature supports the feasibility of phage therapy in CF but there has been a gap in investigating attitudes of the CF community including affected individuals and their caregivers, regarding phage therapy. Understanding perspectives and needs of the CF community is essential for successful implementation and acceptance of novel therapies including phage therapy. We conducted a survey that encompasses responses from 112 consumers from across Australia, comprising people living with CF (38.4%), parents of affected children (49.6%), carers (6.4%), and family members (3%). The findings showed a significant reliance on antibiotics with 51.4% requiring oral, 43.4% nebulised, and 11.4% intravenous (IV) antibiotics within the preceding 2 weeks. Respondents highlighted the availability of new treatments, duration of hospitalisations and costs associated with treatment as important priorities to address. Despite an awareness of phage therapy among 62.4% of respondents, 86.4% expressed interest in obtaining more information, primarily from medical staff (66.7%). Notably, 96.0% of respondents expressed willingness to participate in phage therapy trials. The results of this survey highlighted the CF community's strong interest in advanced therapeutic approaches, specifically phage therapy. The findings reveal a notable recognition and acceptance of phage therapy as a viable treatment option for pulmonary infections associated with CF.

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Section: Introductionmentioning

confidence: 99%

Cystic Fibrosis Australia and Phage Australia survey: Understanding clinical needs and attitudes towards phage therapy in the CF community

Lynch,

Sinclair,

Khatami

et al. 2024

Preprint

View full text Add to dashboard Cite

show abstract

“…The report by Lin and colleagues in this issue of the Journal on cystic fibrosis diagnosed during adulthood 1 is a timely reminder of the importance of the disorder. More than 4000 people in Australia have cystic fibrosis, 2 and one in 25 carry disease‐relevant gene mutations 3 .…”

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confidence: 97%

“…Lin and colleagues analysed data from the Australian Cystic Fibrosis Data Registry, which captures data for more than 90% of people in Australia with cystic fibrosis, highlighting the importance of such databases for understanding disease epidemiology 2 . The disorder is usually diagnosed in infants after newborn screening, but the authors report that 20% of people were more than 50 years old when diagnosed 1 . Adults may be diagnosed with cystic fibrosis because they were missed by newborn screening (about 5% of people with cystic fibrosis), were born before newborn screening commenced (in 1981 in New South Wales, 2001 in Western Australia), or were born in countries where newborn screening for cystic fibrosis is not undertaken 9 …”

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Overcoming the burden of cystic fibrosis

Massie

2023

Medical Journal of Australia

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Diagnosis of cystic fibrosis in adults: Australian Cystic Fibrosis Data Registry data, 2000–2019

Cited by 2 publications

References 3 publications

Cystic Fibrosis Australia and Phage Australia survey: Understanding clinical needs and attitudes towards phage therapy in the CF community

Cystic Fibrosis Australia and Phage Australia survey: Understanding clinical needs and attitudes towards phage therapy in the CF community

Overcoming the burden of cystic fibrosis

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