1. Bronchiectasis

2. Northland 2013 - 10 known paediatric patients with bronchiectasis in Whangarei and 4 in greater Northland. Now 27 confirmed non cystic fibrosis bronchiectatic patients in Northland

3. Early and effective management reduces short- and long-term morbidity

4. Definition Irreversible bronchial dilatation Radiological or pathological diagnosis HRCT scan current gold standard

5. Chronic Suppurative Lung Disease Symptoms of chronic endobronchial suppuration +/- radiological evidence of bronchiectasis

6. Chronic infective bronchitis Protracted bacterial bronchitis Prolonged wet cough Resolves completely after treatment If untreated may progress to bronchiectasis

7. Bx, CSLD, Protracted bacterial bronchitis Symptoms and signs overlap and lack specificity Absolute reliance on radiology-based definition unsatisfactory –When to do imaging –Age related changes in bronchoarterial ratio uncertainty –2 HRCT scans to fulfil irreversible defn –Influence of acute illness

8. Definitions ?chronic suppurative lung disease best overarching term

12. Pathogenesis Obstruction Chronic inflammation, progressive wall damage, dilatation Abnormal cartilage formation (congenital causes) Common thread: difficulty clearing secretions + recurrent infections Resulting airway injury and remodelling

13. Pathogenesis 2 Infections and an ineffective host immune response involving uncontrolled recruitment and activation of inflammatory cells within lower airways Release of mediators, eg proteases and free radicals Causing bronchial-wall injury and dilatation

14. Causes (paeds) Congenital CF Immune deficiency Primary ciliary dyskinesia Aspiration, recurrent small volume Post-infection (Systemic inflammatory diseases)

15. Investigations FBC Immunoglobins Sweat test Sputum PCD – exhaled fractional nasal nitric oxide and/or nasal ciliary brushings Spirometry and lung volumes (>6yo)

16. Invx additional CF gene mutations Bronchoscopy – FB/ airway abnormality Ba swallow/ video fluoroscopy Further immune tests –IgE, neut fnc test, lymphocyte subsets, ab resp to vaccinations HIV Echo (esp adults, ?pulm hypertension)

17. Assessment of severity 1 Clinical –Cough –Sputum –Exacerbation rate –Well-being

18. Assessment Severity 2 Lung function Spirometry –Classically obstructive –Repeated at each review –Relatively insensitive in mild disease, and in children –Spirometric volumes can stabilize and improve in children 6 minute walk –Assessment functional impairment

19. Microbiology Common pathogens children: –H influenzae –S pneumoniae –M catarrhalis

20. Management Early and effective mgmt reduces short- and long-term morbidity

21. Management 1 Airway clearance Chest physiotherapy Nutrition Fitness and activity Avoidance of environmental pollutants –TOBACCO Assessment for co-morbidities Annual ‘flu immunisation

22. Management 2 Intensive antibiotic treatments –Reduce microbial load –Oral Abx and ambulatory care initially –Hospital and IV Abx + intensified physio more severe/ unresponsive oral

23. Burden of disease Incidence – non-CF Bx/CSLD NZ <15yo 3.7/100 000 per year (2x CF incidence) Central Australian Indigenous children 1470/1000 000/year US 18-34 yo 4.2/100 000

24. Northland burden

26. Northland 27 children 0-16 Almost all post-infection x1 with unsafe swallow x1 with IgA deficiency 2 other children with PCD but not Bx

27. Paediatric Bronchiectasis Clinic Quarterly multidisciplinary clinic Currently only at Whangarei Physio, nurse, doctor Team meeting at the conclusion of each clinic to discuss patient’s plans and monitoring and discussion of issues. Same physiotherapist in clinic as on ward –aids with continuity of care –outreach nurse also follows patient both in the community and on admissions.

28. Aims of Multidisciplinary clinic: To provide standardised care to children with bronchiectasis To provide ongoing monitoring in accordance with guidelines for bronchiectasis To prevent/reduce hospital admissions To provide a continuum of physiotherapy techniques in the management of bronchiectasis through their childhood

29. Aims of Multidisciplinary clinic To develop a proactive application to deliver health care for these children and their families to reduce disease progression To provide education and promotion of healthy lifestyles for families with the aim of reducing disease progression To provide a central point of contact for patients and family with bronchiectasis and thus patient centred care To provide holistic care To reduce inequalities of health care access

30. Presentation Chronic or recurrent wet cough Children do not usually expectorate Cough often temporarily resolves after treatment

31. Primary care input 1 Index of suspicion –Two or more episodes of chronic (>4 wks) wet cough/year that respond to Abx –CXR abnormalities persisting at least 6 wks after appropriate therapy Specialist referral

32. Primary care input 2 Management of exacerbations –Appropriate antibiotic for patient –Appropriate length of course –Low threshold for referral for admission if not improving Routine immunisations, plus annual ‘flu Smoking cessation advice and support

33. Questions?