BRONCHIECTASIS
DEFINITION
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
BRONCHIECTASIS
COMMON CAUSES
Common causes of Bronchiectasis
Post-infective
Tuberculosis
Measles
Whooping cough
Mucociliary clearance defect
CF
PCD
Young’s syndrome
Immune defects
Immunoglobulin deficiency
Cellular defects
ABPA
Localized bronchial obstruction
Gastric aspiration
Foreign body
Benign tumour
External compression
CLINICAL FEATURES
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
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DIAGNOSIS AND INVESTIGATIONS
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Assessment
X-ray
CT
Sputum specimen
Bronchoscopy
Lung function
Serum immunoglobulins
ABPA
Gene mutation analysis
Electron microscopy
MEDICAL MANAGEMENT
Clearance of
infected
secretions
Physiotherapy
Treating
infections
Antibiotics
Oral, intravenous,
nebulized
OTHER TREAMENT MEASURES
Influenza vaccination
 Treatment of rhinosinusitis
 Immunoglobulin replacement therapy
 Surgical resection
 Inhaled human deoxyribonuclease (rhDNase)
 Inhaled steroids and bronchodilators
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PHYSIOTHERAPY
Problems:
 Excess bronchial secretions
 Dyspnoea
 ↓ exercise tolerance
 Chest wall pain (musculoskeletal)
EXCESS BRONCHIAL SECRETIONS
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
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ACUTE EXACERBATION
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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
DYSPNOEA
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↓ EXERCISE TOLERANCE
Inhalation with
bronchodilator
Relaxation positions and
breathing control
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Exercise to ↑ fitness and
↓ secretions
Group pulmonary
rehab programme
IMT
CHEST WALL PAIN - MUSCULOSKELETAL
Anti-inflammatory drugs and analgesics
 Heat
 IF
 TENS
 Acupuncture
 Manual therapy
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EVALUATION OF PHYSIOTHERAPY
Effective treatment: ↓ amount and purulence of
sputum
no fever
↑ spirometry
↑ exercise tolerance
↑ energy levels
↓ dyspnoea
↓ chest wall pain
PRIMARY CILIARY DISKINESIA (PCD)
PCD
PCD
Abnormal cilia
structure
Normal structure
abnormal
function
No cilia
PCD (CONTINUED)
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
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PCD - SYMPTOMS
Pneumonia, rhinitis, asthma
 Otitis media
 GOR
 Infertility and ectopic pregnancy
 Investigations: nasal mucociliary clearance test
genetic testing
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MEDICAL
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Antibiotics
Assess and monitor
hearing
Inhaled B2-agonist
GOR – proton pump
inhibitor
PHYSIOTHERAPY
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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
EVALUATION OF PHYSIO
Effective Rx:
minimal coughing on exertion
↓ dyspnoea, coughing, wheezing
↓ fever
↓ secretions (back to usual
amount)
CYSTIC FIBROSIS
CF
CF
Chronic
pulmonary
disease
Pancreatic
insufficiency
↑ electrolites
in sweat
CF (CONTINUED)
Autosomal recessive
 Caucasian populations
 Life expectancy was
2 years, now 31 years
 Faulty gene - CFTR
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CF (CONTINUED)
Abnormality in protein (CFTR)
Changes in ion transport
Changes in mucus and serous secretions produced by
exocrine glands, respiratory system and digestive tract
CF (CONTINUED)
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Ion transport → absorption of sodium ions
from mucosal surface → movement of water
into epithelial cells.
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Balance between movement of sodium and
chloride → volume and composition of of
airway surface liquid and mucociliary clearance
CF (CONTINUED)
Normal lung at
birth
Inflammation
and infection
Mucus secretion
and obstruction
Destroys lung
tissue
(bronchiectasis)
Peptides and
neutrophil
elastase
Neutrophil
bronchiolitis
↓ ciliary function
and mucus
clearance
Chronic hypoxia
and pulmonary
hypertension
Respiratory
failure
DIAGNOSIS AN PRESENTATION
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
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SIGNS AND SYMPTOMS - RESPIRATORY
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Productive cough
Chest pain – musculoskeletal or pleuritic
Dyspnoea (infection or as
disease progresses)
Pneumothorax
Haemoptysis
Clubbing
Coarse crepitations
Pleural rub
Nasal polyps
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Chronic sinusitis
Bronchial wall thickening
Hyperinflation
Nodular shadows
Pulmonary function – initially
obstructive, later restrictive
Ventilation/perfusion
imbalance
Hypoxaemia, CO2 retention
Pulmonary hypertension
ABPA
SIGNS AND SYMPTOMS – GI AND OTHER
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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
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MEDICAL MANAGEMENT
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
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MEDICAL MANAGEMENT (CONTINUED)
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
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HOME TREATMENT
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
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PHYSIOTHERAPY MANAGEMENT
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
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PHYSIO – INFANTS AND SMALL CHILDREN
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
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PHYSIO – INFANTS AND SMALL CHILDREN
Physical activity very important – something
they would enjoy
 Play active role in Rx
 Encourage child to expectorate
 Learn to blow nose
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AIRWAY CLEARANCE
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
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AIRWAY CLEARANCE (CONTINUED)
Patient preference
 Airway clearance once a day with exercise
 Some patients may require Rx 2-3x a day
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EXERCISES
↑ 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
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EXERCISES (CONTINUED)
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
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PRECAUTIONS
No absolute contraindications but exercise
should not be done if patient has:
 Abdominal obstruction
 Acute bronchopulmonary exacerbation with
fever
 Arthralgia and athritis
 Pneumothorax
 Persistent haemoptysis
 Surgery
PRECAUTIONS (CONTINUED)
Exercise –induced bronchoconstriction
 Hot climates
 DM
 Sport:
contact sports
bungee jumping
parachute jumping
scuba
altitude (skiing)
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EXERCISE WITH ADVANCING DISEASE
Not excluded
 Maintenance
 Oxygen – before and after exercise
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INHALATION THERAPY
Beta-adrenergic drugs
 B2-agoniste
 Hypertonic saline with ultrasonic nebulizer
 Bronchoconstriction – test dose
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ACUTE BRONCHOPULMONARY INFECTION
↑ cough and sputum, ↓ in spirometry
 ↓ exercise tolerance
 Weight loss
 Lack of energy
 Dyspnoea
 Fever
 Chest pain
 ↑ duration and frequency of Rx – manual
techniques
 Positioning
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OXYGEN THERAPY
↑ than normal drive to breathe - ↓ PCO2
 Inspiratory time↑ which ↑work of breathing
 Don’t chronically retain PCO2
 Hypoventilate at night - oxygen
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COMPLICATIONS
Advanced CF
 ABPA :
narrowing of airways
gas trapping
small airways disease
mucus plugs (collapse)
wheezing
 Arthropathy: pain, swelling
hot joints, effusions
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COMPLICATIONS (CONTINUED)
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Diabetes:
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DIOS:
polyuria→dehydration→
sticky secretions
insulin requirements change
during exercise
abdominal pain
distension
vomiting
palpable fecal masses
obstruction
COMPLICATIONS (CONTINUED)
GOR
 Haemoptysis
 Liver disease
 Low bone mineral density
 Musculoskeletal dysfunction
 Pneumothorax
 Pregnancy
 Surgery
 Transplantation
 Incontinence
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EVALUATION OF PHYSIO
Sputum weight
 Lung function
 Blood gases
 VAS, Borg scale, QOL
 Adherence!!
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REFERENCES
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Pryor, J.A. and Prasad, S.A. 2008.
Physiotherapy for respiratory and cardiac
problems. Adults and Paediatrics. Edinburgh:
Churchill Livingstone