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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
Definition
• Irreversible bronchial dilatation
• Radiological or pathological diagnosis
• HRCT scan current gold standard
Chronic Suppurative Lung Disease
• Symptoms of chronic endobronchial suppuration
+/- radiological evidence of bronchiectasis
Chronic infective bronchitisProtracted bacterial bronchitis
• Prolonged wet cough
• Resolves completely after treatment
• If untreated may progress to bronchiectasis
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
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
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
Causes (paeds)
• Congenital
• CF
• Immune deficiency
• Primary ciliary dyskinesia
• Aspiration, recurrent small volume
• Post-infection
• (Systemic inflammatory diseases)
Investigations
• FBC
• Immunoglobins
• Sweat test
• Sputum
• PCD – exhaled fractional nasal nitric oxide and/or nasal ciliary brushings
• Spirometry and lung volumes (>6yo)
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)
Assessment Severity 2Lung 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
Management 1
• Airway clearanceChest physiotherapy
• Nutrition
• Fitness and activity
• Avoidance of environmental pollutants– TOBACCO
• Assessment for co-morbidities
• Annual ‘flu immunisation
Management 2
• Intensive antibiotic treatments– Reduce microbial load– Oral Abx and ambulatory care initially– Hospital and IV Abx + intensified physio
• more severe/ unresponsive oral
Burden of diseaseIncidence – 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
Northland burden
Northland National General prevalence
23/32751 1:1424 children (0-14)
1:3000
NZ Maori (only)
10/15138 1:1514 1:1700
Pasifika only 1/2079 1:2079 1:650 NZ Maori and Pasifika
17/17217 1:1013
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
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.
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
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
Presentation
• Chronic or recurrent wet cough
• Children do not usually expectorate
• Cough often temporarily resolves after treatment
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
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