Australian Clinical Practice Guidelines for ... - ResearchGate

Physiotherapy for Cystic Fibrosis in Australia and New Zealand:
A Clinical Practice Guideline Online Supplement* Foreword
This Clinical Practice Guideline has been written by physiotherapists who
are experienced in the management of cystic fibrosis in Australia and New
Zealand. Chairperson: Associate Professor Brenda Button
Senior Clinician Physiotherapist
Alfred Hospital and Monash University, Melbourne Editor: Professor Anne Holland
Clinical Chair, Physiotherapy
La Trobe University / Alfred Health, Melbourne Writing group:
Jenny Bishop (group leader) Westmead Hospital, NSW
Ryan Black (group leader) Lady Cilento Children's Hospital,
Brisbane
Summar Bowen Princess Margaret Hospital, Western Australia
Kate Burton (group leader) The Prince Charles Hospital, Queensland
Brenda Button (group leader) Alfred Hospital, Victoria
Robyn Cobb (group leader) The Prince Charles Hospital, Queensland
Narelle Cox (group leader) La Trobe University, Victoria
Rosie Day John Hunter Children's Hospital, NSW
Julie Depiazzi Princess Margaret Hospital, Western
Australia
Ruth Dentice (group leader) Royal Prince Alfred Hospital, NSW
Katherine Doiron Bond University/Pindara Private
Hospital, Qld
Michael Doumit Sydney Children's Hospital, NSW
Tiffany Dwyer Central Clinical School, Sydney Medical
School, University of Sydney; Royal Prince Alfred Hospital, NSW.
Alison Elliot The Children's Hospital at Westmead, NSW
Louise Fuller Alfred Hospital, Victoria
Kathleen Hall The Prince Charles Hospital, Queensland
Anne Holland La Trobe University / Alfred Health,
Victoria
Matthew Hutchins Mater Health Services
Melinda Kerr Royal Children's Hospital, Victoria
Annemarie Lee Alfred Hospital, Victoria
Christina Mans Waikato DHB, Hamilton NZ
Anna Middleton (group leader) Children's Hospital at Westmead, NSW
Fiona Moran (group leader) The Royal Children's Hospital,Victoria. Lauren O'Connor Gold Cost University Hospital, Queensland
Ranjana Steward Alfred Hospital, Victoria
Angela Potter Women's & Children's Hospital, SA
Tshepo Rasekaba Alfred Hospital, Victoria
Rebecca Scoones Starship Children's Health, New Zealand
Esta-Lee Tannenbaum (group leader) Royal Children's Hospital, Victoria
Ben Tarrant Alfred Hospital, Victoria
Nathan Ward Royal Adelaide Hospital, SA
Samantha West Westmead Hospital, NSW
Dianne White Royal Adelaide Hospital, South Australia
Christine Wilson (group leader) Royal Children's Hospital, Queensland
Lisa Wilson Alfred Hospital, Victoria
Jamie Wood Sir Charles Gairdner Hospital and Institute for
Respiratory Health, Western Australia
Michelle Wood (group leader) The Prince Charles Hospital, Queensland Our thanks are extended to the following individuals for their valuable
comments and review of this document: Ms Nardia Robertson, Dr Audrey
Tierney, Dr Dominic Keating, Dr Kathy Stiller.
Table of Contents Foreword i Table of Contents iv List of Abbreviations x Overview xii 1 Introduction 1 Background to the Clinical Guideline 1 Purpose and Scope 1 Process 1 Methods 2 Dissemination 3 Review process 3 Disclosure statement 5 2 Airway Clearance Techniques 7 Rationale for Airway Clearance Techniques in Cystic Fibrosis 7 Active Cycle of Breathing Technique 8 Autogenic Drainage 9 Positive Expiratory Pressure (PEP) Therapy 10 High Pressure PEP 11 Oscillating PEP 12 The Flutter® 13 Acapella® 14 RC-Cornet® 14 Postural Drainage 15 Postural Drainage in gravity-assisted positions 15 Modified postural drainage 17 Percussion and vibration 17 Other airway clearance techniques 19 High Frequency Chest Wall Oscillation (HFCWO) 19 Intrapulmonary Percussive Vibration (IPV) 20 Physical Exercise as Airway Clearance 20 Clinical Decision Making regarding Airway Clearance Techniques 22 Recommendations 23 3 Inhalation Therapy as an Adjunct to Physiotherapy 24 Inhalation Therapy Technique (including Adjuncts to Inhalation Therapy) 24 Mode of Delivery 26 Mixing Inhaled Medications 27 Timing and Order of Inhaled Medications 28 Nebuliser Maintenance 29 Clinical Monitoring of Inhalation Therapy 30 Recommendations 31 4 Exercise 33 Assessment of exercise capacity 33 Six minute walk test 34 The 3-minute step test 35 Modified Shuttle test (walk/run) 36 Choosing a test of exercise capacity 37 Exercise prescription in cystic fibrosis 38 Facilitating physical activity in people with CF 40 Recommendations 41 5 Musculoskeletal Complications of Cystic Fibrosis 41 Pain 42 Low Bone Mineral Density 42 Vertebral compression and rib fractures 43 Increased thoracic kyphosis 44 Muscle strength 44 Muscle length 45 CF-related arthropathy 46 Physiotherapy intervention for musculoskeletal problems 46 Optimise physical activity to maintain bone mineral density and muscle
mass 47 Optimise muscle strength 48 Optimise muscle length 48 Manual therapy and pain management 49 Recommendations 52 6 Physiotherapy management of the complex patient 53 Pneumothorax 55 Allergic Bronchopulmonary Aspergillosis 57 Cystic fibrosis related diabetes 58 7 Physiotherapy for pregnancy, labour and the post-natal period 61 Physiological changes during pregnancy 61 Pre-pregnancy planning 62 Airway Clearance Therapy during pregnancy in CF 62 Pelvic floor function 63 Exercise during Pregnancy 63 Other physiotherapy interventions during pregnancy 64 Physiotherapy during labour in CF 64 Physiotherapy post-Caesarian section in CF 64 Physiotherapy in the post-natal period 65 8 Physiotherapy management of continence 66 Recommendations 68 9 Physiotherapy management of the newly diagnosed patient 69 The newly diagnosed infant 69 The newly diagnosed adult 70 Recommendations 71 10 Transition from paediatric to adult care - physiotherapy role 72 11 Physiotherapy management for end-stage disease 74 Non-Invasive Ventilation in Cystic Fibrosis 74 Acute respiratory failure and bridge to transplant 74 Sleep disordered breathing and chronic respiratory failure 75 Non-invasive ventilation as an adjunct to airway clearance 76 Exercise and non-invasive ventilation 77 General considerations for NIV 77 Recommendations 78 Physiotherapy and Lung Transplantation 79 Transplant assessment 79 Preparation for transplantation 79 Post-operative period 80 Rehabilitation post transplant 81 Transplant-related problems 81 Recommendations 82 Cystic Fibrosis and the Intensive Care Unit 83 Intensive Care for Reversible CF Complications 83 Intensive Care for Respiratory Failure 83 Extra-corporeal Membrane Oxygenation (ECMO) 83 Physiotherapy for CF in Intensive Care 84 Palliative or end of life care in cystic fibrosis 85 When to switch from active treatment to palliative care 86 The impact of lung transplantation on end of life care 86 Physiotherapy treatment at end of life in CF 87 12 Adherence to physiotherapy in cystic fibrosis 89 13 Infection Control during physiotherapy in cystic fibrosis 92 Airway clearance and inhalation therapy 92 Gym Sessions and Exercise 93 Outpatient Practice 94 14 Delivery of physiotherapy treatment to inpatients and outpatients
with cystic fibrosis 95 Physiotherapy treatment for inpatients 95 Physiotherapy treatment for outpatients 96 15 Directions for Future Research 98 16 References 99 APPENDICES 129 Appendix 1 Protocols for airway clearance techniques 129 Appendix 2: Three-minute step test protocol 130 Appendix 3: Modified shuttle test protocol 132 Appendix 4: Musculoskeletal Screening Tool 134 Appendix 5: Pelvic floor exercises for people with CF 135 Appendix 6: Conflict of Interest Statements 136
List of Abbreviations 3MST three minute step test 6MWT six minute walk test ABPA allergic bronchopulmonary aspergillosis ACBT active cycle of breathing technique ACT airway clearance technique AD autogenic drainage BC breathing control BGL blood glucose level BMD bone mineral density CF cystic fibrosis CFRD cystic fibrosis related diabetes CPAP continuous positive airway pressure FEV1 forced expiratory volume in one second FET forced expiration technique FVC forced vital capacity GOR gastro-oesophageal reflux HFCWO high frequency chest wall oscillation HPOA hypertrophic pulmonary osteoarthropathy IPG/CF International Physiotherapy Group / Cystic Fibrosis IPV intrapulmonary percussive ventilation MST modified shuttle test NHLBI National Heart, Lung, and Blood Institute NIV non-invasive ventilation PaCO2 partial pressure of ca