1. Cystic Fibrosis and Physiotherapy Linda Lapointe P.T. CHEO March 3 rd, 2015

2. Treatment Areas Core Activities: – Secretion clearance – Facilitate exercise – Education (to child and parents) – Support to child and family Other Important Roles: – Musculoskeletal problems, Posture – Urinary incontinence issues – Inhalation therapy education

3.  Facilitate mucus clearance  Generally sessions last from 15 minutes to 40 minutes  Once daily, twice daily or TID  Coordinated with inhalations (mucolytics, bronchodilators and/or antibiotics)  Initiated at diagnosis  Appropriate techniques introduced at appropriate ages Airway Clearance Techniques

4. Airway clearance techniques Most Common: Percussions and modified postural drainage * PEP (Positive Expiratory Pressure)* High Frequency Chest Wall Oscillation Other: Oscillating PEP * Autogenic Drainage * Active Cycle of Breathing * *techniques recognized by the International Physiotherapy Group for Cystic Fibrosis (IPG/CF) http://www.cfww.orghttp://www.cfww.org

5.  Principle:  Ketchup Bottle o Use assistance of gravity o Vibrate to liquefy gels (secretions) Percussions and modified postural drainage

6. Modified Postural Drainage Positions

7. Positive Expiratory Pressure (PEP) Basic Principle: breathe against a resistor (at FRC) –Positive pressure proximal to building up in airways splints bronchiectatic airways open, facilitating removal of distal mucous plugs –Positive alveolar pressure transmitted between alveoli (Pores of Kohn, Ducts of Lambert) DISTAL to the mucous plug pushes mucous plug proximally Each treatment cycle: 12-15 breaths followed by forced expiratory technique, or active cycle of breathing to clear secretions Each treatment session consists of 6 cycles (20-30 minutes total)

8. Variation on a Theme Flutter (OscillatingPEP) Basic Principle: PEP valve; device also contains a metal ball bearing that oscillates during expiration, so secretions are also vibrated (to liquefy them) In randomized trials, not shown to be as effective as PEP for maintaining FEV 1 in CF patients (McIIwaine, et al. Journal of Pediatrics 2001)

9. High Frequency Chest Wall Oscillation (HFCWO) An inflatable vest attached to an air pulse- generating compressor producing oscillation of the chest wall Enhances mucociliary transport by creating a cough like expiratory flow that shears mucous from the airway wall and alters the properties of the mucus Basic Principle: vibration, to liquefy secretions Long-term multicentre randomised controlled study of high frequency chest wall oscillation vs PEP in CF (McIIwaine, et al. Thorax 2013): results favour PEP and do not support the use of HFCWO as the primary form of airway clearance in CF Inflatable Vest Programmable compressor