Download Cystic Fibrosis - 11 - Physiotherapy

Cystic Fibrosis – Physiotherapy
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Cystic Fibrosis Management Guidelines –
Section 11 - Physiotherapy
Dr Jayesh Bhatt (supervising editor)
Consultant in Paediatric Respiratory
Directorate: Family Health – Children
Speciality: Respiratory
December 2016
December 2021
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Children and young people suspected of having
to which it applies (e.g. inclusion
cystic fibrosis and those with confirmed diagnosis
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Abstract
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Paediatrics. Children. Cystic Fibrosis
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expert committee reports or opinions and /
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recommended best practise based on the
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Staff at Nottingham Children’s Hospital via the
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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
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Consultation Process
Dr Jayesh Bhatt
Page 1 of 10
Issued: December 2016
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Dr Jayesh Bhatt
Professor Alan Smyth
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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
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Issued: December 2016
Section 11 - Physiotherapy
11.1
11.2
11.2.1
11.2.2
11.2.3
11.2.4
11.2.5
11.2.6
11.2.7
11.2.8
11.2.9
11.3
11.4
11.5
11.6
11.7
11.8
Dr Jayesh Bhatt
Introduction and principles
Airways Clearance Techniques
Techniques in pre-school children
Incentive Spirometer
Active Cycle of Breathing
Positive Expiratory Pressure (PEP)
Oscillating PEP Divices (The Acapella & The Flutter)
Autogenic Drainage
Intermittent Positive Pressure Breathing (IPPB)
High Frequency Chest Wall Oscillation (Vest)
Exercise
Physiotherapy for postural and musculosketal problems
Physiotherapy for urinary incontinence
Physiotherapy and the Upper Respiratory Tract
Physiotherapy Services - Inpatients
Physiotherapy Services - CF Clinic
Physiotherapy Services - Community
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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
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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
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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
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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
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 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
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Care and Good Clinical Practice for the Physiotherapy Management of Cystic
Fibrosis.Second Edition. 2011.
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Dr Jayesh Bhatt
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Dr Jayesh Bhatt
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