Cystic Fibrosis Physiotherapy Title of Guideline (must include the word Guideline (not protocol, policy, procedure etc) Contact Name and Job Title (author) Directorate & Speciality Date of submission December 2016 Date when guideline reviewed December 2021 Guideline Number Explicit definition of patient group to which it applies (e.g. inclusion and exclusion criteria, diagnosis) Cystic Fibrosis Management Guidelines Section 11 - Physiotherapy Dr Jayesh Bhatt (supervising editor) Consultant in Paediatric Respiratory Directorate: Family Health Children Speciality: Respiratory Children and young people suspected of having cystic fibrosis and those with confirmed diagnosis Abstract Key Words Paediatrics. Children. Cystic Fibrosis Statement of the evidence base of the guideline has the guideline been peer reviewed by colleagues? 1a meta analysis of randomised controlled X trials 2a at least one well-designed controlled study without randomisation 2b at least one other type of well-designed quasi-experimental study 3 well designed non-experimental descriptive studies (ie comparative / correlation and case studies) 4 expert committee reports or opinions and / or clinical experiences of respected authorities 5 recommended best practise based on the clinical experience of the guideline developer Consultation Process Staff at Nottingham Children s Hospital via the Guidelines E-mail process. Target audience Staff at the Nottingham Children s Hospital This guideline has been registered with the trust. However, clinical guidelines are guidelines only. The interpretation and application of clinical guidelines will remain the responsibility of the individual clinician. If in doubt contact a senior colleague or expert. Caution is advised when using guidelines after the review date. Dr Jayesh Bhatt Page 1 of 10 Issued: December 2016
Document Control Document Amendment Record Version Issue Date Author V1 November 2012 V2 September 2015 Dr Jayesh Bhatt Professor Alan Smyth Dr Carol Bertenshaw V3 December 2016 Dr Jayesh Bhatt (supervising editor) General Notes: Summary of changes for new version: No significant changes Statement of Compliance with Child Health Guidelines SOP This guideline refers to activities of only one specific team and consultation has taken place with relevant members of that team. Therefore this version has not been circulated for wider review. Martin Hewitt Clinical Guideline Lead 14 December 2016 Dr Jayesh Bhatt Page 2 of 10 Issued: December 2016
NOTTINGHAM UNIVERSITY HOSPITALS NHS TRUST CHILDREN AND YOUNG PERSONS CYSTIC FIBROSIS UNIT CYSTIC FIBROSIS MANAGEMENT GUIDELINES Section 11 Physiotherapy (Revised by Lisa Godfrey & Marie Bolton) Dr Jayesh Bhatt Page 3 of 10 Issued: December 2016
Section 11 - Physiotherapy 11.1 Introduction and principles 11.2 Airways Clearance Techniques 11.2.1 Techniques in pre-school children 11.2.2 Incentive Spirometer 11.2.3 Active Cycle of Breathing 11.2.4 Positive Expiratory Pressure (PEP) 11.2.5 Oscillating PEP Divices (The Acapella & The Flutter) 11.2.6 Autogenic Drainage 11.2.7 Intermittent Positive Pressure Breathing (IPPB) 11.2.8 High Frequency Chest Wall Oscillation (Vest) 11.2.9 Exercise 11.3 Physiotherapy for postural and musculosketal problems 11.4 Physiotherapy for urinary incontinence 11.5 Physiotherapy and the Upper Respiratory Tract 11.6 Physiotherapy Services - Inpatients 11.7 Physiotherapy Services - CF Clinic 11.8 Physiotherapy Services - Community Dr Jayesh Bhatt Page 4 of 10 Issued: December 2016
11.1 Introduction and Principles Chest physiotherapy is an essential part of the management of CF. Airway Clearance Techniques (ACT) aid the clearance of excessive bronchial secretions. By removing obstructive secretions they aim, in the short-term, to reduce airway obstruction, airway resistance and improve ventilation. In the long-term chest physiotherapy aims to delay the progression of respiratory disease and maintain optimal respiratory function, as removal of mucopurulent secretions may help to reduce the elastase mediated damage to the airways. A programme of daily prophylactic physiotherapy is initiated soon after diagnosis, and continues even when lungs appear clinically clear and the child is asymptomatic. Completing daily prophylactic physiotherapy in this way may help to maintain clear lungs by preventing the build up of secretions and therefore preventing lung damage. Physiotherapy includes aerobic exercise, which increases sputum clearance and maintains/improves exercise tolerance. In addition, assessing and treating complications of CF such as urinary incontinence, musculoskeletal pain and postural abnormalities arising from chronic lung disease may also be required. All CF patients and their families/carers should have access to advice from a senior physiotherapist who has specialised knowledge of CF. It is important that all patients/carers are seen by an experienced physiotherapist as soon as is appropriate at diagnosis in order to formulate an effective management plan, which is suitable for the age, background and disease severity of the individual. The treatment regimens should be reviewed at regular intervals through clinic visits, community visits and ward reviews, and adapted according to their changing needs. 11.2 Airway Clearance Techniques Different techniques are used for different ages with a gradual change from the passive treatment of babies towards independence in adolescence and adulthood. Several studies have been conducted looking at the effectiveness of different techniques, but results are sometimes conflicting and vary in quality. When choosing a technique it is vital to consider patient preference as this will ultimately determine adherence to treatment and therefore effectiveness. 11.2.1 Techniques in Pre-school Children All babies with CF commence on a daily programme of ACT. Parents are taught how to assess for signs of respiratory exacerbations and to increase treatment frequency as appropriate. Initial treatment techniques consist mainly of modified positioning/postural drainage and chest percussion. Parents are also taught to incorporate daily movement positioning/assisted exercise from diagnosis. At an appropriate point during infancy, baby/infant PEP is introduced. From around the age of two, blowing games to encourage and teach deep breathing exercises and directed coughing are introduced into the treatment session. At an appropriate point a child will be taught the forced expiratory technique (FET). At around the age of three to five years the incentive spirometer can be used (see below). These techniques are then incorporated at a later age into the Active Cycle of Breathing Techniques (ACBT). Dr Jayesh Bhatt Page 5 of 10 Issued: December 2016
11.2.2 Incentive Spirometer This is a device that encourages and teaches thoracic expansion exercises/ deep breaths, whilst monitoring flow rate and volume and providing visual feedback to the child and carers. It can be incorporated into the ACBT technique and modified postural drainage. This device can be started with children from the age of about three to five. 11.2.3 Active Cycle of Breathing This consists of three breathing techniques: - Thoracic expansion exercises - to loosen secretions Breathing control - to encourage relaxation Forced expiration technique - to mobilise and clear secretions This is not a rigid treatment technique but is modified to suit each patient. The cycle is repeated as often as necessary and is often combined with modified postural drainage positions, or in conjunction with other treatment adjuncts. 11.2.4 Positive Expiratory Pressure (PEP) This technique involves breathing out through a PEP mask or mouthpiece, against a resistance. PEP therapy increases intra-bronchial pressure in central and peripheral airways splinting the airways open and preventing compression induced by airway collapse. This promotes inflow of air behind mucus obstructions either via a bronchial route or collateral airway channels. Smaller bronchial airways are prevented from collapse thus permitting the continuing upward movement of secretions. This treatment can be performed in sitting or modified postural drainage positions. The PEP mask is usually introduced in infancy, in the form of baby/ infant PEP, using tidal volume breathing. At an appropriate age the technique will be adapted to involve deep breathing through the mask followed by huffing, coughing and breathing control. The correct expiratory pressure can be measured with a manometer. 11.2.5 Oscillating PEP Devices (The Acapella & The Flutter) The Acapella is a plastic hand held device that works similarly but using a plastic arm and a magnet. These devices combine the effects of PEP, as explained above, with a cyclic oscillatory and vibratory effect during expiration that is transmitted through the tracheobronchial tree. This helps to break up and reduce the viscosity of secretions, so they can be cleared more easily. The Flutter is a pipe like device containing a large ball bearing. During expiration through the device, the steel ball initially impedes airflow. As expiratory pressure increases and exceeds the effect of gravity the ball is displaced, the pressure is released the ball drops back down. A treatment session consists of a series of deep breaths through the device, usually in sitting, followed by a combination of huffing, coughing and breathing control. The acapella can be used in any position whereas the flutter can only be used in sitting. These devices are usually used with the older children / adolescents as the technique is more difficult to perfect and monitor than using a PEP device. 11.2.6 Autogenic Drainage This technique consists of a three phase breathing regimen, which uses high expiratory flow rates while avoiding airway closure. The utilisation of high airflow produces shearing forces within the airways that may tear mucus from bronchial walls. The treatment can be performed in any position, and involves breathing at different lung volumes, which maximises the expiratory flow at that particular generation of the bronchial tree. The technique is combined with the forced expiratory technique, coughing and breathing control. This technique again is used with older children / adolescents as good selfawareness of breathing control, lung volumes and secretions are essential. Dr Jayesh Bhatt Page 6 of 10 Issued: December 2016
11.2.7 Intermittent Positive Pressure Breathing (IPPB) IPPB is a technique used to provide positive pressure throughout inspiration. The Bird Mark 7 ventilator is the pressure limited ventilator normally used to provide IPPB triggered by the patient s spontaneous effort, and is a useful adjunct to physiotherapy. Through providing intermittent mechanical ventilation it has been shown to augment lung expansion, reduce work of breathing, and assist in the clearance of secretions when more simple airway clearance techniques alone are not maximally effective. This is only used during inpatient admissions and the need for IPPB will be based on careful assessment and consideration by an experienced Physiotherapist, and by agreement of the Consultant. There is a full hospital guideline on IPPB to refer to for more information. 11.2.8 High Frequency Chest Wall Oscillation (Vest) Some parents may ask about use of High Frequency Chest Wall Oscillation (HFCWO) as a treatment alternative. HFCWO consists of a wearable vest surrounded by an air chamber which delivers rapidly repeating pulses of air to the chest wall. The most robust research at present comparing the use of HFCWO and PEP demonstrated that PEP was significantly favourable to HFCWO in reducing pulmonary exacerbations, increasing length of time to pulmonary exacerbation, reducing use of antibiotics and reducing treatment times. Based on the evidence available we would encourage PEP or an oscillatory PEP device as the main form of ACT. HFCWO may be considered in some circumstances, but always in conjunction with other physiotherapy adjuncts. 11.2.9 Exercise The importance of regular exercise in maintaining a healthy life style is recognised in both health and disease. It has long been recognised that physical activity is a key aspect in the care of patients with cystic fibrosis. It is recommended that all children and young people with cystic fibrosis should participate in developmentally appropriate physical activity for a minimum of 60 minutes on a daily basis. Aerobic exercise for 30-60minutes, 3 times a week, should form part of this from the age of 7, and it is from this age that all children will participate in sub-maximal exercise testing (in the form if an incremental step-test) as part of their annual assessment. Discussions around exercise levels and recommendations for physical activity take place at every clinic review. Special consideration may be necessary when giving advice with regards to exercise to children and young people with CF complications such as those with moderate to severe lung disease, CF related diabetes and those with a pulmonary exacerbation. 11.3 Physiotherapy for Postural and Musculoskeletal Problems Children with CF are at risk of developing postural problems as a consequence of lung disease, together with the risk of suffering CF related arthropathy. From about the age of seven postural screening is performed at annual assessment. Children and parents are educated about the importance of good posture to optimise lung expansion and prevent secondary musculoskeletal problems. Specific postural correction exercises and thoracic stretches are taught if required. Out-patient musculoskeletal physiotherapy can be arranged for more complex problems. 11.4 Physiotherapy for Urinary Incontinence Urinary incontinence is recognised as a common problem in girls with CF, and although may cause significant problems may not be readily reported. Screening for this is included in the physiotherapy annual assessment in girls over the age of seven. If problems are identified advice is provided about self management during ACT and daily activities, and Dr Jayesh Bhatt Page 7 of 10 Issued: December 2016
pelvic floor exercises can be taught. Patients can also be referred to the paediatric specialist urology nurse. 11.5 Physiotherapy and the Upper Respiratory Tract Although a high percentage of children with CF will have some sinonasal involvement, very few are symptomatic. The physiotherapy team may be involved in helping manage upper respiratory tract signs if a child is symptomatic. This may include simply encouraging effective nose blowing as part of their airway clearance routine, but may sometimes include the additional use of nasal wash-outs and sinus nebulisation, alongside any other medical management that has been deemed necessary. 11.6 Physiotherapy Services- Inpatients A normal physiotherapy service is provided to the CF patients between the hours of 8.30am and 4.30pm Monday to Friday. All CF patients are assessed and an appropriate treatment plan is devised. This is reviewed and adapted throughout the admission as appropriate, and may include airway clearance techniques and exercise. Contact details: Senior Paediatric CF Physiotherapist: Phone extension: 65326/ 61887 Bleep: 780 7413 Mobile: 07812269773/07812269774 Referral: An open referral system to physiotherapy operates for the CF patients during normal working hours, but it is essential that physiotherapy staffs are made aware of an admission. A referral should be made to the physiotherapy service via the nerve centre by the nurse caring for the patient on admission to hospital. Weekends: A weekend physiotherapy service is provided to the CF patients between 8.30am and 4.30pm as part of the paediatric physiotherapy weekend rota. If patients are admitted over the weekend a referral should be made to the Paediatric Physiotherapy Team via the Nerve Centre. On-Call / Emergency physiotherapy: An emergency service is provided between the hours of 4.30pm and 8.30am each day. The physiotherapist can be contacted through switchboard. The emergency physiotherapy policy applies, indicating that a patient may be seen if they would significantly deteriorate if not seen by physiotherapy prior to the start of the next working day. Planned call outs may be arranged if the ward physiotherapist deems this appropriate. 11.7 Physiotherapy Services - CF Clinic All patients are assessed by an experienced physiotherapist every CF clinic where possible. The aims of the service are to assess, review and progress physiotherapy airway clearance techniques and exercise as appropriate, provide equipment as required, and to give advice and educate children and carers as necessary. The physiotherapist will closely liaise with the CF multi-disciplinary team and shared care teams as appropriate. A Physiotherapy Annual Assessment is performed around the time of the child s birthday at their Annual Assessment appointment. This assessment will include: Dr Jayesh Bhatt Page 8 of 10 Issued: December 2016
A thorough subjective and objective respiratory assessment. A review and modification of current techniques including demonstration. A review of current exercise/activity. An exercise test is performed from the age of seven. Reviewing, supplying and updating equipment as appropriate. Screening for musculoskeletal and postural problems. Screening for urinary incontinence. Advice and education. 11.8 Physiotherapy Services - Community Visits by the community CF physiotherapist are offered and arranged for a variety of reasons. These may include, but are not limited to: - Patients receiving IV antibiotic therapy at home. - Newly diagnosed patients. - Support for patients with severe lung disease - Support for those with end-stage disease - Support for patients with difficult social situations e.g. parental illness, poor compliance - Organising and monitoring the provision of physiotherapy within schools. - Establishing and monitoring any changes in treatment Contact details: Community CF Physiotherapist: 07812269774 1. Association of Chartered Physiotherapists in Cystic Fibrosis (ACPCF). Standards of Care and Good Clinical Practice for the Physiotherapy Management of Cystic Fibrosis.Second Edition. 2011. 2. CF Trust Clinical Guidelines for the physiotherapy management of CF. 2002. 3. Elkins S, Jones A, Van der Schans CP. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2006, Issue 2. Art. No: CD003147. DOI: 10.1002/14651858. CD003147.pub3 4. Flume, PA, Robinson K, O Sullivan B, Finder JD, Vender FL, Willey-Courand D, White TB, Marshall BC & The Clinical Practice Guidelines Committee. Cystic Fibrosis Pulmonary Guidelines: Airway Clearance Therapies. Resp Care 2009; 54; 522-537 5. Hyeona Kang S, de Tarso Roth Daclin P, Bejzman Piltecher O, de Oliveira Migliavacca R. Chronic rhinosinusitis and nasal polyposis in cystic fibrosis: update on diagnosis and treatment. Jornal Brasileiro de Pneumologia 2015; 41(1): 65-76 6. Main E, Prasad A, Van der Schans C. Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis. The Cochrane Database of Systematic Reviews 2005, Issue1. Art. No: CD002011. DOI: 10.1002/14651858. CD002011.pub2 7. Massery M. Musculoskeletal and neuromuscular interventions: a physical approach to cystic fibrosis. J Royal Soc Med 2005; 98 (Suppl 45): 55-66 8. Mcllwaine MP, Alarie N, Davidson GF, Lands LC, Ratjen F, Milner R, Owen B, Agnew JL. Long-term multicentre randomised control study of high frequency chest wall oscillation versus positive expiratory pressure in cystic fibrosis. Thorax 2013; 68(8); 746-751 Dr Jayesh Bhatt Page 9 of 10 Issued: December 2016
9. McVean RJ, Orr A, Webb AK, Bradbury A, Kay L, Phillips E, Dodd ME. Treatment of urinary incontinence in cystic fibrosis. J Cyst Fibros 2003; 2: 171-176 10. Morrison L, Agnew L. Oscillating devices for airway clearance in people with cystic fibrosis. Cochrane Database for Systematic Reviews 2014, Issue 1. Art. No: CD006842. doi: 1002/14651858.CD006842.pub3. 11. Nankivell G, Caldwell P, Follett J. Urinary incontinence in adolescent females with cystic fibrosis. Paediatric Respiratory Reviews 2010; 11: 95-99 12. Prasad SA, Main E, Dodd ME. Finding consensus on the physiotherapy management of asymptomatic infants with cystic fibrosis. Paed Pulmonol 2008; 43; 236-244 13. Swisher AK, Hebestreit H, Mejia-Downs A, Lowman JD, Gruber W, Nippins M, Alison J and Schneiderman. Exercise and habitual physical activity for people with cystic fibrosis: Expert consensus, evidence-based guide for advising patients. Cardiopulmonary Physical Therapy Journal 2015; 00; 1-14. Dr Jayesh Bhatt Page 10 of 10 Issued: December 2016