1、Bronchiectasis,Definition,Permanent dilatation of one or more bronchiElastic and muscular tissue of bronchial walls destroyed by acute and chronic infectionImpaired drainage of secretionsSecretions chronically infectedChronic inflammatory responseProgressive destructive lung disease,Bronchiectasis,C
2、ommon causes,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 di
3、scharge, and facial pain Auscultation coarse crepitations, wheezing Usually no clubbing,Diagnosis and investigations,Assessment X-ray CT Sputum specimen Bronchoscopy Lung function Serum immunoglobulins ABPA Gene mutation analysis Electron microscopy,Medical management,Physiotherapy AntibioticsOral,
4、intravenous,nebulized,Clearance of infected secretions,Treating infections,Other treament measures,Influenza vaccination Treatment of rhinosinusitis Immunoglobulin replacement therapy Surgical resection Inhaled human deoxyribonuclease (rhDNase) Inhaled steroids and bronchodilators,physiotherapy,Prob
5、lems: 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 reas
6、sessed Improved ventilation Hypertonic saline,Acute exacerbation,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 positio
7、n Blood streaking in sputum continue Rx. Frank haemoptysis discontinue Continue Rx when secretions mildly bloodstained,Dyspnoea Exercise tolerance,Inhalation with bronchodilator Relaxation positions and breathing control,Exercise to fitness and secretions Group pulmonary rehab programme IMT,Chest wa
8、ll pain - musculoskeletal,Anti-inflammatory drugs and analgesics Heat IF TENS Acupuncture Manual therapy,Evaluation of physiotherapy,Effective treatment: amount and purulence of sputumno fever spirometry exercise tolerance energy levels dyspnoea chest wall pain,Primary ciliary diskinesia (PCD),PCD,P
9、CD (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,PCD - symptoms,Pneumonia, rhinitis, asthma Otitis media GOR Infertility an
10、d ectopic pregnancy Investigations: nasal mucociliary clearance testgenetic testing,Medical Physiotherapy,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 dependa
11、nt 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 (continued),Autosomal recessive Caucasian populations Life
12、 expectancy was2 years, now 31 years Faulty gene - CFTR,CF (continued),CF (continued),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 mucociliar
13、y clearance,CF (continued),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 sto
14、ols) concentration of sweat chloride,Signs and symptoms - Respiratory,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 Hyp
15、erinflation Nodular shadows Pulmonary function initially obstructive, later restrictive Ventilation/perfusion imbalance Hypoxaemia, CO2 retention Pulmonary hypertension ABPA,Signs and symptoms GI and other,Obstruction of small bowel with Abdominal distension and discomfort Vomiting and or no bowel s
16、igns 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,Medical management,Pulmonary fun
17、ction 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,Medical management (conti
18、nued),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,Home treatment less disrupting
19、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,Physiotherapy management,Accurate assessment and Rx for every individual patient secretions, exercises Education with regards to inhalation
20、therapy / oxygen therapy Musculoskletal pain, low bone density Urinary incontinence Work with patient and family / carers realistic Rx plan,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 R
21、outine daily airway clearance not required if no symptoms,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,Airway clearance,Main aim CF secretions - viscoelastisity, dehydrated, hype
22、radhesive Mobilize secretions without obstruction or fatigue airflow, long volumes, alter properties of secretions Huffing Rather ventilation than drainage,Airway clearance (continued),Patient preference Airway clearance once a day with exercise Some patients may require Rx 2-3x a day,exercises, exe
23、rcise 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,Exercises (continued),Weight that can be lifted comfortably 10-15x, progress to 20-30x and then weight 15-30 m
24、inutes, every second day Warm-up, stretches and cool down Be careful with strengthening training in children 8-12 repititions without fatigue,Precautions,No absolute contraindications but exercise should not be done if patient has: Abdominal obstruction Acute bronchopulmonary exacerbation with fever
25、 Arthralgia and athritis Pneumothorax Persistent haemoptysis Surgery,Precautions (continued),Exercise induced bronchoconstriction Hot climates DM Sport: contact sportsbungee jumpingparachute jumpingscubaaltitude (skiing),Exercise with advancing disease,Not excluded Maintenance Oxygen before and afte
26、r exercise,Inhalation therapy,Beta-adrenergic drugs B2-agoniste Hypertonic saline with ultrasonic nebulizer Bronchoconstriction test dose,Acute bronchopulmonary infection, cough and sputum, in spirometry exercise tolerance Weight loss Lack of energy Dyspnoea Fever Chest pain duration and frequency o
27、f Rx manual techniques Positioning,Oxygen therapy, than normal drive to breathe - PCO2 Inspiratory time which work of breathing Dont chronically retain PCO2 Hypoventilate at night - oxygen,complications,Advanced CF ABPA : narrowing of airwaysgas trappingsmall airways diseasemucus plugs (collapse)whe
28、ezing Arthropathy: pain, swellinghot joints, effusions,Complications (continued),Diabetes: polyuriadehydrationsticky secretionsinsulin requirements change during exercise DIOS: abdominal paindistensionvomitingpalpable fecal masses obstruction,Complications (continued),GOR Haemoptysis Liver disease L
29、ow bone mineral density Musculoskeletal dysfunction Pneumothorax Pregnancy Surgery Transplantation Incontinence,Evaluation of physio,Sputum weight Lung function Blood gases VAS, Borg scale, QOL Adherence!,References,Pryor, J.A. and Prasad, S.A. 2008. Physiotherapy for respiratory and cardiac problems. Adults and Paediatrics. Edinburgh: Churchill Livingstone,
