1EM © 2012 Copyright Electromed, Inc. Bronchiectasis William Grimm, RRT 33 rd Annual Mountain Air Symposum 10/9/2014

2EM © 2012 Copyright Electromed, Inc. The Challenge 

3EM © 2012 Copyright Electromed, Inc. Presentation Agenda Bronchiectasis ­ Types ­ Causes ­ Symptoms ­ Effects on the Body ­ Diagnosis ­ Treatment ­ Bronchiectasis Research Registry Airway Clearance High Frequency Chest Wall Oscillation (HFCWO) Patient Satisfaction with the SmartVest ® Airway Clearance System

4EM © 2012 Copyright Electromed, Inc. What is Bronchiectasis? Abnormal stretching and enlarging of the respiratory passages caused by mucus blockage The blockage and accompanying infection cause inflammation, leading to the weakening and widening of the passages* Bronchiectasis = dilation of proximal and medium-sized bronchi >2mm with transmural inflammation, edema scarring, and ulceration –Laennec, 1819 –Osler, late 1800s –Reid, 1950s *Source: American Lung Association

5EM © 2012 Copyright Electromed, Inc. A. Normal lung and airway Source: Barker AF. Bronchiectasis. NEJM 2002; 346(18): B. Bronchiectasis lower lobes, most common, saccular dilatations and grape-like clusters with pools of mucus Bronchiectasis

6EM © 2012 Copyright Electromed, Inc. COPD Education

7EM © 2012 Copyright Electromed, Inc. Types of Bronchiectasis Focal Bronchiectasis –Occurs in a single lobe of the lung and can be the result of an obstruction by a large foreign object, a tumor, or an unusual development in the airway Diffuse Bronchiectasis –Occurs in more than a single lobe –An underlying immune deficiency, infection, or toxic exposure that involves multiple lobes –Cystic Fibrosis and Primary Ciliary Dyskinesia

8EM © 2012 Copyright Electromed, Inc. What Historically Caused Bronchiectasis? The most common causes have been severe lung infections during childhood such as whooping cough or severe viral infections

9EM © 2012 Copyright Electromed, Inc. What Causes Bronchiectasis Today? Tuberculosis is the single leading cause worldwide Genetic disorders such as CF and PCD are the leading causes in Western countries Aspiration (focal bronchiectasis) GERD Complications of primary infections such as bronchitis, pneumonia, whooping cough, or tuberculosis Not all cases are caused by infection or genetic defect –Exact cause is never determined in many cases Higher incidence in older women and native American population in Alaska

10EM © 2012 Copyright Electromed, Inc. Prevalence of Bronchiectasis USA - 52 in 100,000 South Korea Study –1,409 patients ages 23 to 86 –HR CT Scan »129 of study group had bronchiectasis on HR CT Scan (9.1%) »53% of the 9.1% above exhibited respiratory Symptoms Source: Kwak, et al., Highprevalence of bronchiectasis in adults: analysis of CT findings in a health screening program, Department of Internal Medicine, Hanyang University of Medicine, Seongdong-gu, Seoul, Korea

11EM © 2012 Copyright Electromed, Inc. Economic Burden of Bronchiectasis in the USA Annual cost of care in 2001 = $13,244 ­ Greater than the cost of heart disease ($12,000) and COPD ($11,000 to $13,000) For the estimated 110,000 patients with Bronchiectasis the total medical care cost annually = $1.4 billion

12EM © 2012 Copyright Electromed, Inc. Chronic Respiratory Symptoms Cough and sputum production (90 to 100%) Areas of scarring Mucus gland enlargement Coughing up blood occasionally Wheezing, possibly from asthma Shortness of breath (75%) Pleurisy or chest pain with breathing (50%) Source: Barker AF. Bronchiectasis. NEJM 2002; 346(18):

13EM © 2012 Copyright Electromed, Inc. Bronchiectasis Symptoms Persistent productive cough Production of large quantities of purulent (infected) sputum Chronic respiratory infection, including infection with rare and/or opportunistic organisms Shortness of breath Cyanosis

14EM © 2012 Copyright Electromed, Inc. Effects on the Body Vicious Cycles –Once bronchiectasis is established in the lobe or lobes of the lung, it compromises airway clearance which leads to respiratory infections –These infections take up in the nooks, crannies, and pockets of the airway which the bronchiectasis has created Inflammation –Loss of structural integrity of muscle, elastic tissue, and sometimes bronchial cartilage making the airway susceptible to infection which leads to inflammation

15EM © 2012 Copyright Electromed, Inc. 1.Top - Foamy mucus 2.Middle - Watery layer 3.Bottom - Cell debris 3-Layer Sputum Source: Barker AF and Albert: Clinical Respiratory Medicine, 2nd ed., © 2004 Mosby

16EM © 2012 Copyright Electromed, Inc. Infections Associated with Bronchiectasis Staphylococcus aureus Haemophilus influenza Mycoplasma pneumoniae Mycobaterium tuberculosis Nontuberculous Mycobacterium (avium-complex) Pseudomonas aeruginosa

17EM © 2012 Copyright Electromed, Inc. How is Bronchiectasis Diagnosed? Computed Tomography (CT Scan) of the chest, required by Medicare Chest X-ray Bronchoscopy Lung function tests Blood tests Sputum culture

18EM © 2012 Copyright Electromed, Inc. Physical Exam Adventitious breath sounds Crackles (70%) Rhonchi (44%) Wheezing (34%) Clubbing (3%) Findings of primary causative disease (eg. rash, joint deformities, situs inversus, lymphadenopathy, dwarfism, yellow nails, IV needle tracks, etc.) Source: Barker AF. Bronchiectasis. NEJM 2002; 346(18):

19EM © 2012 Copyright Electromed, Inc. Treating Bronchiectasis Airway Clearance Therapy (ACT) –Reduces obstruction and infection of the bronchi –Examples: exercise, CPT, inhaled medications, and mechanical devices Antibiotics –To control infections –Tobi, which is inhaled, for P. Aeruginosa –Azithromycin, which is taken orally Lung Transplants –For those who are approaching respiratory failure –For focal bronchiectasis, possible treatments include a lobectomy, or partial lung resection

20EM © 2012 Copyright Electromed, Inc. Bronchiectasis Registry Consolidated database of non-CF bronchiectasis patients from multiple clinic institutions –Stores information about bronchiectasis patients and the disease itself –Enrolled 10,000 patients in October 2011 Source: COPDFoundation.org

21EM © 2012 Copyright Electromed, Inc. Bronchiectasis Study via Registry 54 Stable COPD Patients Mean age = 69 Mean FEV1 = % had Bronchiectasis in HRCT Scan Increased in reported COPD exacerbations, sputum markers, and bacterial colonization

22EM © 2012 Copyright Electromed, Inc. Natural airway clearance includes three essential components Normal Airway Clearance Mucus traps impurities Cilia beat carry mucus to large airways A strong cough clears mobilized secretions In most individuals, these three components work in harmony to keep our airways clear

23EM © 2012 Copyright Electromed, Inc. Inadequate Airway Clearance Inadequate clearance Infections Scarring Decrease pulmonary function Increased health costs Loss of productivity

24EM © 2012 Copyright Electromed, Inc. Mucociliary System Disorders Cystic Fibrosis (CF) Primary Ciliary Dyskinesia Immotile Ciliary Syndromes Obstructive Pulmonary Conditions COPD ­ Chronic Bronchitis ­ Emphysema Bronchiectasis Neuromuscular Disease ALS Muscular Dystrophy Spinal Muscular Atrophy Post Polio Neuromotor Disease/Conditions Cerebral Palsy Multiple Sclerosis Spinal Cord Injuries Restrictive Airway Disorders Scoliosis Kyphoscoliosis Acute Care in Hospital setting Post surgical Ventilator Etc. Inadequate Airway Clearance Problems may occur with diseases or conditions characterized by

25EM © 2012 Copyright Electromed, Inc. Complications of Retained Secretions Atelectasis Increased Work of Breathing Hypoxemia Infection Lung Damage

26EM © 2012 Copyright Electromed, Inc. High Frequency Chest Wall Oscillation (HFCWO) - Considerations for Use High Frequency Chest Wall Oscillation is designed specifically for airway clearance therapy to enhance mucus transport and improve bronchial drainage. Indications for HFCWO are not diagnosis specific; however, it can be considered when retained secretions cause pulmonary compromise. Thus, individual assessment of each patient is appropriate.

27EM © 2012 Copyright Electromed, Inc. Patient Satisfaction with HFCWO via the SmartVest ® Airway Clearance System The SmartVest ® incorporates many features designed for the comfort and convenience of the user. These features are intended to increase the ease of use to encourage better cooperation and compliance for all ages – small children to adults. What do patients say they like about the SmartVest ® ? The SmartVest ® is gentler on the body, with comparable efficacy Only soft, breathable, washable fabrics touch the skin (SmartVest Wrap ® must be spot cleaned) All Velcro™-type closures make adjustments and “on and off” simple A single offset hose improves maneuverability during treatment

28EM © 2012 Copyright Electromed, Inc. THANK YOU QUESTIONS?