When should I refer a patient with cystic fibrosis to a specialist clinic for further management?

Refer a patient with cystic fibrosis to a specialist cystic fibrosis centre for further management if:

• They have a positive or equivocal sweat test result.
• The clinical assessment suggests cystic fibrosis but test results (sweat or gene tests) are normal.
• Gene testing reveals one or more cystic fibrosis mutations.

Specialist cystic fibrosis centres provide multidisciplinary care including specialist paediatricians or adult physicians, nurses, physiotherapists, dietitians, pharmacists, and clinical psychologists with expertise in cystic fibrosis.

Referral should also be considered for ongoing management, support, and monitoring of complications, as well as for access to specialist interventions such as intravenous antibiotic therapy and exercise programmes.

Additionally, specialist advice should be sought for specific complications such as bone mineral density issues (Z score below -2.0 or T score below -2.5).

Patients and their families should be offered information, support, and opportunities to discuss their care with healthcare professionals experienced in cystic fibrosis.