Cystic fibrosis

• Cystic fibrosis with any of the following concerning features:
  • respiratory distress
  • new haemoptysis (clots or more than streaks)
  • pleural effusion
  • consolidation/pneumonia/fever
  • pneumothorax
  • non-response to antibiotics for chest infection
  • respiratory failure and pulmonary hypertension/cor pulmonale
• Cystic fibrosis related abdominal complications (e.g. renal colic, distal intestinal obstruction syndrome, acute pancreatitis or biliary cholic/cholecystitis)
• Any cystic fibrosis related deterioration, respiratory or other, should be referred onto tertiary cystic fibrosis clinic urgently or emergency

• Nil

To contact the relevant service, see Clinician Assist WA: Acute Respiratory assessment (external site).

• Chronic suppurative lung disease with airflow obstruction and chronic airway infection
• Allergic bronchopulmonary aspergillosis
• Chronic rhinosinusitis with nasal polyposis

Referrals missing 'mandatory information' with no explanation provided may not be accepted by site. If 'mandatory information' is not included, the explanation must be provided in the body of the referral (e.g. patient unable to access test in regional or remote areas or due to financial reason).

This information is required to inform accurate and timely triage. If unable to attach reports, please include relevant information/findings in the body of the referral and advise where (provider) investigation/imaging was completed.

• Duration and severity of symptoms including:
  • non-pulmonary CF problems
  • recent admissions
• Current medication list
• Any known allergies
• Previous centre of care (if transitioning patient)

• Nil

• Nil

• Family history
• Weight history/trend

• Nil

• FBC
• U&Es
• LFTs
• Ca
• 25 (OH) Vitamin D3
• Fat soluble vitamin levels (Vitamin A, D, E)
• Fe results
• Coagulation profile
• Fasting BGL
• Spirometry
• CXR/CT and any other relevant imaging
• Any recent sputum culture results (within last 12 months)
• Genotype
• Sweat test            

Category 1

Appointment within 30 days

• Newly diagnosed cystic fibrosis
• Patients with known cystic fibrosis transitioning from a paediatric or other adult centre who have recent clinical instability and/or severe lung disease (FEV1<40%)

Category 2

Appointment within 90 days

• Suspected but undiagnosed cystic fibrosis
• Patients with known cystic fibrosis transitioning from a paediatric or other adult centre who have recent clinical stability or moderate lung disease (FEV1>40%)

Category 3

Appointment within 365 days

•  No defined category 3 criteria

• Nil

• All patients diagnosed with cystic fibrosis should be managed by a cystic fibrosis service in a tertiary facility

Spirometry: Bulk-billed spirometry can be obtained via Asthma WA. A list of other providers undertaking lung function testing is provided on Clinician Assist WA: Respiratory Function Testing.
See MBS: Item 11505 and MBS: Item 11506 for information on completing spirometry.

Clinician resources

• Cystic Fibrosis Australia: Standard of Care for Cystic Fibrosis in Australia 2008
• The Thoracic Society of Australia & New Zealand: Nutritional guidelines for cystic fibrosis 2017
• CF Physio