Historical perspective

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1 Bronchiectasis Dilated airways with frequently thickened walls Faculty/Presenter Disclosure Faculty: Dr. Alan Kaplan Relationships with commercial interests (12 months) Grants/research support: N/A Speakers bureau/honoraria: Grifols, J&J, Merck, Purdue, Trudel Consulting fees: Aerocrine, AstraZeneca, Boehringer Ingelheim, GSK, Meda, Novartis, Pfizer, Sanofi Clinical trials: Novartis Disclosure of Commercial Support This program has no commercial support Mitigating Potential Bias No pharmacotherapies discussed here today other than by classes of medication Historical perspective Case Robyn Nurse Occupational Asthma Recurrent chest and sinus infections Continued wheezing despite ICS/LABA 1

2 Investigations CXR? Bronchiectasis CT confirmed bronchiectasis Spirometry showed some reversibility 10% and 200 ml FENO low Blood eosinophils 100 Weaned her down off ICS watching her FENO FENO stayed low, she was NO different off her ICS Out of interest She was sent to a Respirologist by another treating doctor looking after her for a different issue Said she had Asthma due to the spirometry being reversible ICS or not? CMAJ 2017 BRONCHIECTASIS Definition: Abnormal and permanent dilation of bronchi. Focal or diffuse distribution Clinical consequences chronic and recurrent infection and Pooling of secretions in dilated airways. Classification: 1. Cylindrical (fusiform) 2. Saccular 3. Varicose How does it present? Pathophysiology 2 Prerequisites: Infectious insult Impairment of drainage, airway obstruction, and/or a defect in host defense. 2

3 Pathophys Continued Infection: Bacterial, mycobacterial, esp. ABPA central airway bronchiectasis Airway obstruction: intraluminal tumor, foreign body, lymph nodes, COPD Etiology: A. Infections-Microorganisms Measles and Pertussis Adeno & Influenza virus Bacterial infection with virulent organisms: S.aureus, Klebsiella Anaerobes Atypical mycobacteria Mycoplasma HIV Tuberculosis Fungi Immunodeficiency: ciliary dyskinesia, HIV, hypogammaglobulinemia, cystic fibrosis (obstruction and immunodef.) ETIOLOGY : IMPAIRED HOST DEFENCE Local causes: Endobronchial obstruction Generalised impairment: 1. Immunoglobulin deficiency 2. Primary ciliary disorders 3. Cystic fibrosis ETIOLOGY : NON-INFECTIOUS Toxins or toxic substances NH 3 ; gastric contents Immune responses, ABPA Inflammatory diseases: ulcerative colitis, rheumatoid arthritis, Sjögren syndrome. -1-Antitrypsin deficiency Yellow nail syndrome primary Reference: Up to Date 3

4 4. Diagnosis - CXR dilated airways thickened airway walls irregular periph. opacities (mucus) General Management of Bronchiectasis Respiratory care treatment protocols Oxygen prn Bronchopulmonary hygiene Hyperinflation therapy Aerosolized medication Mechanical ventilation Why do patients require Airway Clearance? Normal function of the airways facilitates the movement and removal of mucus from the lungs Coughing vibrates and contracts the airways aiding in loosening of mucus and airway clearance Cilia beat back and forth carrying mucus to the central airways where it can be coughed out Compromised function of the airways can result from Overproduction of thick, sticky mucus Impaired or ineffective function of the cilia Ineffective cough Fixed or diminished lung volumes/capacities Chronic respiratory infections Which is the most effective form of Airway Clearance? Manual chest physiotherapy (CPT) is often the standard, however it has some negative aspects Requires caregiver assistance Physically demanding Time consuming Can be uncomfortable or unpleasant Long-term compliance may be low No compelling evidence that one form is more effective than any other Irwin RS, Boulet LP, Cloutier MM, Gold PM, Ing AJ, O Byrne P, Prakash UBS, Pratter MR, Rubin BK. Managing Cough as a Defense Mechanism and as a Symptom: A Consensus Panel Report of the American College of Chest Physicians. Chest 1998;114(2):133S-181S. McCool FD, Rosen MJ. Nonpharmacologic Airway Clearance Therapies: ACCP Evidence-Based Clinical Practice Guidelines. Chest 2006;129(1):250S-259S. Flume PA, Robinson KA, O Sullivan BP, Finder JD, Vender RL, Willey-Courand DB, White TV, Marshall BC and the Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic Fibrosis Pulmonary Guidelines: Airway Clearance Therapies. Resp Care 2009;54(4): McIlwaine MP, Alarie N, Davidson GF, Lands LC, Ratjen F, Milner R, Owen B, Agnew J. Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis. Thorax 2013;0:1-6. 4

5 Benefits of Oscillating PEP Therapy Enhances mucus mobilization and removal Aids in lung hygiene/helps prevent infections Improves gas transfer and lowers air trapping Better patient compliance and acceptance Shorter treatment times than CPT Easy to learn for independent use Inexpensive and portable device Little interruption to daily living At least as effective as manual chest physiotherapy What is Oscillating Positive Expiratory Pressure (PEP) Therapy? Airway with a mucus plug, normal exhalation with no positive expiratory pressure applied. Airway stays closed. PEP Only Airway with positive expiratory pressure applied. Airway is opened allowing pressure to build behind the mucus plug. OPENS AIRWAYS DOES NOT AFFECT MUCUS Airway with oscillating positive expiratory pressure applied. Airways stay open allowing pressure to build behind the mucus plug while vibrations help thin, shear and mobilize mucus. OPENS AIRWAYS CAN GET BEHIND PLUGS THINS, SHEARS AND DISLODGES MUCUS SUPPORTS NATURAL CILIA CLEARANCE McCool FD, Rosen MJ. Nonpharmacologic Airway Clearance Therapies: ACCP Evidence-Based Clinical Practice Guidelines. Chest 2006;129(1):250S-259S. Marks HJ. Airway Clearance Devices in Cystic Fibrosis. Paediatric Respiratory Reviews 2007;8: Nebulizer Port Standard 22 mm fitting accommodates small volume nebulizers for combined aerosol treatments Exhalation Ports Exhaled breath is directed away from the user One-Way Valve Low-resistance valve allows inhalation and exhalation without removing from mouth Resistance Indicator 5 resistance settings adjust to each patient s capacity; one device for all patient groups Aerobika Intelligent Design Designed to ensure correct assembly every time; easy to clean/disinfect and dishwasher safe Lightweight, ergonomic design fits comfortably in the hand; Resistance is not position dependent so patients can hold the device in the most natural position Exacerbation Exacerbation: Etiology +Rx Colonization/infection: Hemophilus Pseudomonas MAI Aspergillus Very difficult to distinguish colonization from acute infection with these bugs. Psuedomonas colonized more bronchiectasis on CT; increased number of hospitalizations vs H. flu colonization Treatment: Fluoroquinolone Prevention Antibiotics-Controversial: Consider Macrolide TIW Cipro qd X 7-14 D/ month Bronchial Hygiene, physiotherapy, pulmonary rehab, airway clearnace?bronchodilators, not ICS! Surgery? Effect of sputum bacteriology on the quality of life of patients with bronchiectasis. Wilson CB; Jones PW; O'Leary CJ; Hansell DM; Cole PJ; Wilson R Eur Respir J 1997 Aug;10(8):

6 Back to Robyn FENO and blood eosinophils stayed low Spirometry is a FALSE POSITIVE Other things can also cause reversibility ICS discontinued as she did not get benefit from it (and possible harm ) Uses a LABA/LAMA for symptoms of wheeze as well as the airway clearance device Summary Non specific presentation Insult + infection is cause Airway obstruction is not uncommon CT scan makes diagnosis, but not cause Symptoms treated with bronchodilators and airway clearance devices Role of antibiotics.. 6