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 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

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)

• 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 of severity 1

• Clinical– Cough– Sputum– Exacerbation rate– Well-being

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

Microbiology

• Common pathogens children:– H influenzae– S pneumoniae– M catarrhalis

Management

Early and effective mgmt

reduces

short- and long-term morbidity

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

Questions?