Section 4 - Management of diagnosed conditions

1. What is bronchiectasis? ^1–5

• A chronic lung condition, defined as the permanent dilatation of the bronchi and bronchioles where the elastic and muscular tissue is destroyed by re-occurring inflammation and infection
• The damage impairs the natural drainage of bronchial secretions resulting in airway obstruction and progressive lung damage characterised by persistent:
  • cough
  • sputum production
  • recurrent respiratory infections
• Symptoms may occur for many years before a diagnosis is confirmed
• Haemophilis influenzae and Pseudomonas aeruginosa pathogens are a primary cause of bronchiectasis airway infections
• Nearly 2% of Aboriginal and Torres Strait Islander children will develop bronchiectasis
• No definite cause can be established in up to half of all patients
• Up to 50% of patients will also have Chronic obstructive pulmonary disease

2. Diagnosis of bronchiectasis ^1–5

• Bronchiectasis relies on both a clinical and radiological diagnosis
• Predicting mortality and exacerbation rates in bronchiectasis can be undertaken with an online bronchiectasis prediction tool. See Resource 1.

Flowchart 1. Diagnosing bronchiectasis

3. Management of bronchiectasis ¹

• Management involves improving mucus clearance, while reducing airway bacterial colonisation, inflammation, and structural damage by:
  • minimising symptoms (i.e. cough)
  • reducing hospital admissions
  • preventing lung infections
  • improving quality of life
  • improving exercise tolerance
  • maintaining lung function
  • reducing frequency and severity of exacerbations
  • prolonging survival
  • aggressively identifying and managing comorbidities, including:
    • Chronic obstructive pulmonary disease
    • severe respiratory infections
    • GORD
    • Asthma (adults and children > 12)
    • chronic bronchitis

1. Support patient self-management
   • Discuss bronchiectasis and:
     • airway clearance manoeuvres. See Resource 2.
     • how to control breathlessness and Anxiety disorders
     • develop an action plan. See 3.10 Action plan
     • medicine usage, effects and adherence
     • provide supportive resources. See Resource 3.
   • If the patient also has COPD refer them to SMoCC, a phone service that supports patients manage their condition. See Resource 4.
   • Encourage the patient to identify barriers to adequate lifestyle modification and
     medical adherence and create goals to overcome those barriers. See Engaging our patients
2. Social-emotional support
   • See Social-emotional wellbeing
3. Smoking cessation ^1–4
   • Patients who stop smoking reduce the likelihood of lung infections and bronchiectasis progression
   • See Smoking cessation
4. Prevent respiratory infections ^1–4
   • Respiratory illnesses contribute to bronchiectasis exacerbations and progression
   • Provide Influenza, pneumococcal and COVID-19 vaccines as per the Australian Immunisation Handbook
5. Avoid environmental pollutants ²
   • Patients should avoid environmental pollutants (see Table 1.) which can exacerbate:
     • coughing
     • sputum volume, consistency and purulence
     • shortness of breath
     • exercise intolerance
     • fatigue
     • haemoptysis

6. Airway clearance technique ^1–4
   • Main therapy to clear excess lung secretions to improve ventilation and reduce hospital presentations
   • Technique:
     • start with 5 deep abdominal breaths. Expand chest fully, starting with the
       diaphragm and lower ribs. Avoid lifting or shrugging shoulders
     • do 30–60 seconds of relaxed breathing. Breathe from the diaphragm. The patient should feel their stomach rising and falling with each breath. Shoulders should be kept as relaxed as possible
     • do another 5 deep abdominal breaths
     • follow this with 30–60 seconds of relaxed breathing
     • take a medium sized breath in and huff the air out a little more forcefully
     • start with 3 cycles of gentle huffs. Finish with 2 cycles of more forceful huffs
     • finish with a cough to clear any secretions left in the main airways
     • repeat above cycle 2–3 times or until no more secretions can be removed
   • Refer to a physiotherapist if patient is unable to clear lung secretions
   • See Resource 2. for further information
7. Improve physical activity tolerance ^1–4
   • Enhances airways clearance
   • Should include moderate to high intensity aerobic exercises, strength training and mobility exercises
   • Refer to an exercise physiologist for pulmonary rehabilitation or exercise program
   • See Physical activity and sleep
8. Pulmonary rehabilitation program
   • An important hospital avoidance strategy offered to all patients with:
     • poor physical activity tolerance
     • > 2 exacerbations per year
   • If no local program available:
     • advocate for a service
     • refer to the Pulmonary Rehabilitation Toolkit. See Resource 5.
     • contact the chronic condition coordinator or the Lung Foundation for
       rehabilitation program details and training. See Resource 6.
9. Nutrition
   • Lung disease increases the risk of poor nutrition, weight loss and reduced muscle strength because of:
     • increased energy needs
     • decreased appetite
     • lack of energy to shop, cook or eat meals
     • an increased need for certain vitamins, minerals and antioxidants
   • Refer to MO/NP or dietitian if patient has unintended weight loss or weight gain
   • See Diet and nutrition
10. Action plan ²
    • Develop an action plan (Resource 7.) with the patient so they can:
      • recognise and monitor exacerbations and severity. See Table 3.
      • intervene early to prevent exacerbations
      • understand and feel comfortable using it
    • Review and update action plan each visit, especially when changing medicines. See Table 2.

4. Medicines for bronchiectasis

1. Sputum sample ^1–4
   • Always take sputum samples and treat early
   • Bronchiectasis patients often have positive sputum culture results. This does not mandate antibiotic use unless:
     • the patient has an exacerbation (see Table 3.) or
     • results show a new isolation of P. aeruginosa
   • Exclude NTM infection by collecting at least 3 sputum samples for mycobacterial culture, in all patients before azithromycin use

2. Eradication of Pseudomonas aeruginosa (P. aeruginosa) ^1–4
   • The presence of P. aeruginosa in the airways is associated with increased:
     • exacerbations
     • risk of hospitalisation
     • risk of mortality
   • If a patient is clinically stable when P. aeruginosa is first identified, do not treat. Consult the Antimicrobial Stewardship (AMS) or a respiratory specialist to avoid promoting antibiotic resistance
3. Long-term antibiotics to reduce exacerbation frequency and symptoms in adults ^1,2,4
   • Seek advice from AMS or a respiratory specialist if a patient has:
     • ≥ 6 exacerbations over 12 months or
     • ≥ 2 hospitalisations over 12 months or
     • > 6 months of continuous symptoms
   • Routine long-term (6–12 months) oral or nebulised antibiotics are not recommended as antibiotic resistance is a common outcome
4. Azithromycin prophylaxis in children with non-cystic fibrosis (non-CF) bronchiectasis or chronic suppurative lung disease (CSLD) ^6,7
   • Prior to initiation of azithromycin as maintenance therapy, the following are required:
     • child has been reviewed by a respiratory consultant
     • presence of bronchiectasis or CSLD
     • ≥ 3 exacerbations and/or ≥ 2 hospitalisations in previous 12 months
     • failed trial of long-term non-macrolide antibiotics for at least three months
     • documented evidence of NTM exclusion in the lower airways
     • non-pharmacological interventions are optimised and adhered to
     • documented baseline liver function test and ECG
   • Azithromycin is not initiated if:
     • evidence of NTM infection
     • allergy to macrolides
     • abnormal liver function test
     • medicine interactions e.g. antiarrhythmics
     • See Table 4. for dosing and follow-up in children

5. Cycle of care

6. References

• All Chronic Conditions Manual references are available via the downloadable References PDF

7. Resources

1. Bronchiectasis prediction tools for predicting mortality and exacerbation rates in non-cf bronchiectasis
2. Airway clearance manoeuvres resources
3. Bronchiectasis patient resources
4. Self-Management of Chronic Conditions (SMoCC) service
5. The Australian Lung Foundation Pulmonary Rehabilitation Toolkit
6. The Lung Foundation training and education website
7. A bronchiectasis action plan