Bronchiectasis
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
Early and effective
management reduces
short- and long-term morbidity
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 bronchitis
Protracted 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
Definitions
• ?chronic suppurative lung disease best
overarching term
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)
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Congenital
CF
Immune deficiency
Primary ciliary dyskinesia
Aspiration, recurrent small volume
Post-infection
(Systemic inflammatory diseases)
Investigations
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FBC
Immunoglobins
Sweat test
Sputum
PCD – exhaled fractional nasal nitric oxide
and/or nasal ciliary brushings
• Spirometry and lung volumes (>6yo)
Invx additional
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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 of severity 1
• Clinical
– Cough
– Sputum
– Exacerbation rate
– Well-being
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
Microbiology
• Common pathogens children:
– H influenzae
– S pneumoniae
– M catarrhalis
Management
Early and effective mgmt
reduces
short- and long-term morbidity
Management 1
• Airway clearance
Chest 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 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
Northland burden
General
prevalence
NZ Maori
(only)
Pasifika only
NZ Maori and
Pasifika
Northland
23/32751
10/15138
1:1424
children (0-14)
1:1514
1/2079
17/17217
1:2079
1:1013
National
1:3000
1:1700
1:650
Northland
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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
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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
Questions?