Permanent dilatation of one or more bronchi

Elastic and muscular tissue of bronchial walls destroyed by acute and chronic infection

Impaired drainage of secretions

Secretions chronically infected

Chronic inflammatory response

Progressive destructive lung disease

Common causes of Bronchiectasis

Post-infective TuberculosisMeaslesWhooping cough

Mucociliary clearance defect CFPCDYoung’s syndrome

Immune defects Immunoglobulin deficiencyCellular defects

ABPA

Localized bronchial obstruction Foreign bodyBenign tumourExternal compression

Gastric aspiration

Totally asymptomatic to severe disease Productive cough with large amounts of

purulent secretions, sometimes haemoptysis Frequently admitted to hospital Exacerbations – chest pain, dyspnoea, fever If accompanied by CF or PCD – sinus disease

with nasal blockage, purulent discharge, and facial pain

Auscultation – coarse crepitations, wheezing Usually no clubbing

Assessment X-ray CT Sputum specimen Bronchoscopy Lung function Serum immunoglobulins ABPA Gene mutation analysis Electron microscopy

Physiotherapy Antibiotics Oral,

intravenous, nebulized

Clearance of infected

secretions

Treating infections

Influenza vaccination Treatment of rhinosinusitis Immunoglobulin replacement therapy Surgical resection Inhaled human deoxyribonuclease

(rhDNase) Inhaled steroids and bronchodilators

Problems: Excess bronchial secretions Dyspnoea ↓ exercise tolerance Chest wall pain (musculoskeletal)

Patient must understand pathology and reason for treatment

ACBT, AD, Flutter Becareful of head-down tip - GOR Self treatment important daily Time of day? Physio techniques reassessed Improved ventilation Hypertonic saline

Hospitalised - ↑ secretions or more purulent, dehydrated, dyspnoea.

Haemoptysis and pleuritic pain Nebulized bronchodilator and humidification IPPB - ↓ work of breathing Post-resection – changed anatomy of bronchial

tree – find optimal position Blood streaking in sputum – continue Rx. Frank haemoptysis – discontinue Continue Rx when secretions mildly bloodstained

Inhalation with bronchodilator

Relaxation positions and breathing control

Exercise to ↑ fitness and ↓ secretions

Group pulmonary rehab programme

IMT

Anti-inflammatory drugs and analgesics Heat IF TENS Acupuncture Manual therapy

Effective treatment: ↓ amount and purulence of sputum

no fever↑ spirometry↑ exercise tolerance↑ energy levels↓ dyspnoea↓ chest wall pain

Infections in nose, ears, sinuses and lungs

Fertility affected (fallopian tubes and sperm motility)

Dextrocardia or situs inversus Previously immotile cilia syndrome Chronic sputum production and nasal

symptoms

Pneumonia, rhinitis, asthma Otitis media GOR Infertility and ectopic pregnancy Investigations: nasal mucociliary

clearance test genetic testing

Antibiotics Assess and monitor

hearing Inhaled B2-agonist GOR – proton pump

inhibitor

Daily physio Teach parents early signs

of infection Lethargy, “off colour”,

fever Secretions mostly in

dependant areas Airway clearance

techniques Huffing games Exercises Nasopharyngeal

suctioning

Effective Rx: minimal coughing on exertion↓ dyspnoea, coughing, wheezing↓ fever ↓ secretions (back to usual amount)

Autosomal recessive Caucasian populations Life expectancy was 2 years, now 31 years Faulty gene - CFTR

Ion transport → absorption of sodium ions from mucosal surface → movement of water into epithelial cells.

Balance between movement of sodium and chloride → volume and composition of of airway surface liquid and mucociliary clearance

Newborn screening DNA testing Symptoms of respiratory and GI symptoms Failure to pass meconium (meconium ileus) Healthy apeptite, but failure to thrive

(malabsorption and hyposecretion of enzymes by pancreas)

Streatorrhoea (fatty and offensive stools) ↑ concentration of sweat chloride

Productive cough Chest pain – musculo-

skeletal or pleuritic Dyspnoea (infection or

as disease progresses) Pneumothorax Haemoptysis Clubbing Coarse crepitations Pleural rub Nasal polyps

Chronic sinusitis Bronchial wall thickening Hyperinflation Nodular shadows Pulmonary function –

initially obstructive, later restrictive

Ventilation/perfusion imbalance

Hypoxaemia, CO2 retention Pulmonary hypertension ABPA

Obstruction of small bowel with

Abdominal distension and discomfort

Vomiting and ↓ or no bowel signs

CFRD Biliary cirrhosis Portal hypertension Bleeding Liver transplant

Puberty delayed Normal or near normal

fertility in women Men infertile Rheumatic symptoms Joint pain, swelling, ↓ ROM of knees, ankles

and wrists Low bone mineral

density Fractures, rib fractures

Pulmonary function and nutrition important Interdisciplinary team Morbidity and mortality related to chronic

infection → oral, nebulized and intravenous antibiotics

Important to wash hands between patients, contamination of nebulizers

Inhaled bronchodilators and steroids Hypertonic saline

High energy intake Fat-soluble vitamins and vitamin K,

pancreatic enzymes Cortcosteroid nasal spray Haemoptysis – will stop spontaneously,

embolization Pneumothorax – resolve without Rx or with

ICD Heart-lung and double lung transplant Palliative care

Home treatment less disrupting than hospitalisation

IV antibiotics at home Home visits Physio doing home Rx Patient must take responsibility for own Rx Future: Gene therapy

Stem cell therapy

Accurate assessment and Rx for every individual patient

↓ secretions, ↑ exercises Education with regards to inhalation

therapy / oxygen therapy Musculoskletal pain, low bone density Urinary incontinence Work with patient and family / carers –

realistic Rx plan

Before feeds for 10-15 minutes ↑ frequency and duration during infection PEP facemask AD Physical activity Head-down tip - ↑ GOR Routine daily airway clearance – not

required if no symptoms

Physical activity very important – something they would enjoy

Play active role in Rx Encourage child to expectorate Learn to blow nose

Main aim CF secretions - ↑ viscoelastisity,

dehydrated, hyperadhesive Mobilize secretions without ↑

obstruction or fatigue ↑ airflow, ↑ long volumes, alter

properties of secretions Huffing Rather ↑ ventilation than ↑ drainage

Patient preference Airway clearance once a day with

exercise Some patients may require Rx 2-3x a

day

↑ exercise tolerance Make a given level of exercise more

comfortable and ↑ADL Endurance: swimming, cycling, running Strength training: weights Interval training Intensity 20-30 min, 3-4x per week

Weight that can be lifted comfortably 10-15x, progress to 20-30x and then ↑ weight

15-30 minutes, every second day Warm-up, stretches and cool down Be careful with strengthening training

in children 8-12 repititions without fatigue

No absolute contraindications but exerciseshould not be done if patient has: Abdominal obstruction Acute bronchopulmonary exacerbation with

fever Arthralgia and athritis Pneumothorax Persistent haemoptysis Surgery

Exercise –induced bronchoconstriction Hot climates DM Sport: contact sports

bungee jumpingparachute jumpingscubaaltitude (skiing)

Not excluded Maintenance Oxygen – before and after exercise

Beta-adrenergic drugs B2-agoniste Hypertonic saline with ultrasonic

nebulizer Bronchoconstriction – test dose

↑ cough and sputum, ↓ in spirometry ↓ exercise tolerance Weight loss Lack of energy Dyspnoea Fever Chest pain ↑ duration and frequency of Rx – manual

techniques Positioning

↑ than normal drive to breathe - ↓ PCO2

Inspiratory time↑ which ↑work of breathing

Don’t chronically retain PCO2

Hypoventilate at night - oxygen

Advanced CF ABPA : narrowing of airways

gas trappingsmall airways diseasemucus plugs (collapse)wheezing

Arthropathy: pain, swellinghot joints, effusions

Diabetes: polyuria→dehydration→sticky secretionsinsulin requirements change

during exercise DIOS: abdominal pain

distensionvomitingpalpable fecal masses

obstruction

GOR Haemoptysis Liver disease Low bone mineral density Musculoskeletal dysfunction Pneumothorax Pregnancy Surgery Transplantation Incontinence

Sputum weight Lung function Blood gases VAS, Borg scale, QOL Adherence!!

Pryor, J.A. and Prasad, S.A. 2008. Physiotherapy for respiratory and cardiac problems. Adults and Paediatrics. Edinburgh: Churchill Livingstone