The Evolving landscape of CF Treatments
In the past decade, the development of ground-breaking medicines called CFTR (cystic fibrosis transmembrane conductance regulator) modulators have enabled treatment targeting the root cause of Cystic Fibrosis – the CFTR gene defect. These disease-modifying drugs have revolutionised the mainstay of CF treatment.1
Since their adoption, modulators have had an observed effect on Cystic Fibrosis (CF) patients in various ways, such as improved lung function and respiratory symptoms, however much is still unknown.1
For instance, although significant improvements in lung function have been observed, many people with CF remain pancreatic insufficient requiring pancreatic enzyme replacement therapy, as well as additional supplementation of fat-soluble vitamins.
However, it is worth noting that research has suggested that absorption of nutrients, including fat-soluble vitamins, is improving in some CF patients being treated with modulator therapy, with varying results across studies.
In these individuals, there may be a need for reduced vitamin dosages. Although, currently, the guidelines recommend that fat-soluble vitamins should be supplemented according to the individual’s level of pancreatic insufficiency.2
We have recently collaborated with the British Dietetic Association (BDA) Cystic Fibrosis Specialist Group to hear from experts on the impact of modulators on vitamin status in adults and children with CF. Expert speakers Laura Schembri, Specialist Paediatric Respiratory Dietitian at the Royal Brompton Hospital, and Maeve O’Driscoll, Adult Cystic Fibrosis Dietitian and Adult CF Clinical Speciality Lead at the Royal Brompton, provide insights on the latest research and share their learnings from within their own patient populations.
To view the webinar on the BDA website,
- Regard L, et al. CFTR Modulators: Current Status and Evolving Knowledge. Semin Respir Crit Care Med. 2023 Apr;44(2):186-195. Epub 2022 Dec 19.
- Wilschanski M, et al. ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis. Clinical Nutrition 2024; 413-44
UK-PVI-2400021|Jun 24
