Respiratory morbidity is common in children with neuromuscular diseases (NMD) owing to chronic hypoventilation and impaired cough. Optimal, cost-effective respiratory management requires implementation of clinical practice guidelines and a coordinated multidisciplinary team approach.
To explore South African physiotherapists’ knowledge, perception and implementation of respiratory clinical practice guidelines for non-ventilated children with NMD.
An online survey was conducted amongst members of the South African Society of Physiotherapy’s Cardiopulmonary Rehabilitation (CPRG) and Paediatric special interest groups and purposive sampling of non-member South African physiotherapists with respiratory paediatrics expertise (
Most respondents worked in private healthcare, with 1–10 years’ experience treating patients with NMD. For acute and chronic management, most participants recommended nebulisation and 24-h postural management for general respiratory care. Percussions, vibrations, positioning, adapted postural drainage, breathing exercises and manually assisted cough were favoured as airway clearance techniques. In addition, participants supported non-invasive ventilation, oscillatory devices and respiratory muscle training for chronic management.
Respondents seemed aware of internationally-endorsed NMD clinical practice guidelines and recommendations, but traditional manual airway clearance techniques were favoured. This survey provided novel insight into the knowledge, perspectives and implementation of NMD clinical practice guidelines amongst South African physiotherapists.
There is an urgent need to increase the abilities of South African physiotherapists who manage children with NMD, as well as the establishment of specialised centres with the relevant equipment, ventilatory support and expertise in order to provide safe, cost-effective and individualised patient care.
Neuromuscular diseases (NMD) are a heterogeneous group of disorders that include pathology of the muscle (e.g. myopathies, muscular dystrophies), neuromuscular junction, peripheral nerves and motor neurons (e.g. anterior horn cell) (Yang & Finkel
Characterised by progressive muscle weakness, including cardiac and respiratory muscles, children with NMD often present with respiratory morbidity because of hypoventilation and an impaired cough (Chatwin et al. 2018; Farrero et al.
Even with new drug therapies changing the disease course and respiratory function over time in children with NMD, adequate symptomatic and preventative, pro-active respiratory management strategies are still recommended (Farrero et al.
Mechanical ventilation, oxygen supplementation as well as peripheral and proximal airway clearance techniques (ACT) can address the respiratory complications experienced by children with NMD (Chatwin et al. 2018; Finder
Peripheral and proximal airway clearance techniques, including cough augmentation.
Airway clearance techniques (
Neuromuscular disease clinical practice guidelines, recommendations and updates for respiratory management have been published since 2004 and, if implemented, can minimise healthcare expenses whilst optimising patient outcome (Birnkrant et al.
A quantitative, cross-sectional descriptive study within a non-probability purposive sampling frame was used. The target population consisted of all physiotherapists registered with the South African Society of Physiotherapy (SASP), with a self-identified special interest in cardiopulmonary rehabilitation (CPRG) and/or paediatrics, who were members of either one or both of these special interest groups.
South African physiotherapists with expertise in respiratory paediatrics, who were not members of the SASP special interest groups, were identified by the authors and invited to take part in the survey as their input could provide valuable information. Respondents without either academic, research or clinical expertise in the respiratory management of children with NMD were excluded from our study.
The self-constructed questionnaire was based on existing clinical practice guidelines and clinical expertise (Finder et al.
The final questionnaire consisted of four sections and participants were asked to indicate their support of specific respiratory management techniques (‘yes’, ‘no’, ‘unsure’, ‘patient dependent’ and open-text responses for additional comments).
Vocational background and clinical experience (in the field of NMD) of the participant.
Evidence-based respiratory management strategies were recommended for children with NMD in an acute setting (hospital), based on a case scenario:
Patient X has been diagnosed with a NMD and is older than five years of age and has recently become wheelchair-bound (non-ambulant). He has been admitted to hospital because of a respiratory infection and is presenting with a weak cough. He is haemodynamically stable, but oxygen saturation is 91%, and he is retaining secretions because of an ineffective cough.
The four subdivisions of Section B included questions relating to general respiratory care (including ventilatory support), peripheral ACT (secretion mobilisation), proximal ACT (cough augmentation) as well as other physiotherapy and general management strategies related to children with NMD during acute care.
Evidence-based respiratory management strategies were recommended for children with NMD in a chronic setting (home or school), based on a case scenario:
Patient Y has been diagnosed with a NMD and is older than five years of age. He recently became wheelchair-bound (non-ambulant) and attends follow-up visits at the neuro clinic every 3–6 months, depending on the need. Currently his vital capacity is < 80% predicted value for his age and he presents with a weak cough.
The four subdivisions of Section C enquired about ventilatory support, peripheral ACT and proximal ACT, LVR techniques (such as breath-stacking), breathing exercises, respiratory muscle training as well as other physiotherapy and general management strategies related to children with NMD during chronic management.
Respiratory management strategies used by South African physiotherapists in the acute and chronic settings, 6 months prior to the survey.
The questionnaire (
Flow chart of combined responses for pilot study participants and electronic responses.
The survey system on Survey Monkey was opened for the participation of SASP members in June 2016 and reminder emails were sent approximately every 2 weeks, for a duration of 8 weeks.
Data were entered into Excel spreadsheets, therafter the completed survey data (including pilot study responses) were exported to statistical programmes for a combined analysis STATA® (StataCorp,
Depending on the normality of continuous data (tested using the Shapiro–Wilk
Because of the distribution of responses and a variety of answers from participants for ‘repetitions’ and ‘frequency’ of treatment techniques, parametric tests could not be applied. Frequency tables, proportions and histograms or bar graphs illustrate descriptive statistics.
The Institutional Review Board at the University of Cape Town provided ethical clearance (513/2015) and the SASP Executive Committee (president), as well as the chairpersons of CPRG and Paediatric Special Interest Group granted permission to distribute the questionnaire to their members. Informed consent was obtained from participants by including study information and a confirming consent statement on the opening page of the Survey Monkey questionnaire.
A flow chart of survey responses is presented in
The total number of recruited participants consisted of 74 physiotherapists who responded to the online survey, as well as five out of the six pilot study participants with a response rate of 16.4% (79/481).
Three of the initial respondents did not provide consent; two provided incomplete vocational information (Section A) and 10 reported that they had not previously worked with children with NMD or had no experience in the field. These 15 participants were therefore excluded from the original 79 responses. The final sample of
Questions on vocational background (area of work) allowed for more than one option to be chosen. The majority of the 64 participants worked in the private sector which included out-patients and hospital wards (general and specialised) (58%;
Most participants (42%;
No statistically significant association was found between years of experience in NMD and clinical practice trends such as ventilatory support, oxygen supplementation, ACT and respiratory muscle training. The use of LVR (breath-stacking) during chronic management of children with NMD, however, showed a significant association with the place of work (public vs. private healthcare sector) (Yates χ2, [1;
In this section of the questionnaire, participants were asked about recommended evidence-based respiratory management strategies specifically related to general respiratory care as well as peripheral and proximal ACT during acute management of children with NMD.
A varying number of participants (
Acute respiratory management in children and adolescents with neuromuscular diseases (
Under general respiratory management (indicated in Spotted,
Peripheral ACT (indicated in Zigzag,
Manually assisted cough, with a combination of thoraco-abdominal compression, was mostly indicated as proximal ACT, whilst suctioning was also well supported (Striped,
Summary of suggested treatment duration and frequencies for peripheral and proximal airway clearance techniques (acute care).
Treatment | Duration per treatment (min) | Proportion | % | Frequency (per day) | Proportion | % |
---|---|---|---|---|---|---|
Percussions | 10 or 15 | 9/31 | 29 | 2 | 23/31 | 74 |
Vibrations | 5 or 10 | 12/33 | 36 | 2 | 24/33 | 73 |
Postural drainage (Trendelenburg) | 1–20 | N/A | 2 | 6/8 | 75 | |
Adapted postural drainage (No Trendelenburg positioning) | 10 or 20 | 7/30 | 23 | 2 | 18/28 | 64 |
Positioning | 10 or 20 or 30 | 8/32 | 25 | 2 | 15/29 | 52 |
Autogenic drainage (Including assisted autogenic drainage) | 5 or 10 | 6/19 | 32 | 2 | 13/18 | 72 |
Breathing exercises: |
5–10 | 15/35 | 43 | 2 | 18/32 | 56 |
Manually assisted cough | Frequency per treatment: |
6/32 | 19 | Patient dependent | 27/38 | 71 |
Suctioning | Frequency per treatment: |
8/22 | 36 | 2 | 10/22 | 46 |
ACBT, active cycle of breathing technique.
Only a minority of participants (
Additional recommendations for ACT during acute management provided by survey participants as open-text responses included mobilisation (change of position, thoracic or upper limb mobility), breathing exercises (huffing, breath-stacking, ACBT, PEP therapy using blow bottle or windmill blowing and respiratory muscle training) as well as caregiver education and/or support.
The responses (
Chronic management in children and adolescents with neuromuscular diseases (
Under ventilatory support during chronic management (indicated in Spotted,
The majority of the participants recommended oscillatory devices, positioning and adaptive PD for peripheral ACT (Zigzag,
Similar to acute care, MAC was strongly recommended for cough augmentation as part of proximal ACT, favouring a combined thoraco-abdominal technique. Most of the participants were unsure of the use of cough assist devices (MI-E) (Striped,
Regarding the use of lung compliance (LVR), breathing exercises and respiratory muscle training (Checkered,
Treatment duration and frequency of peripheral and proximal ACT as well as respiratory muscle training during chronic care of children with NMD, as recommended by the majority of participants, are depicted in
Summary of suggested treatment duration and frequencies for respiratory muscle training, lung compliance and airway clearance techniques (chronic care).
Treatment | Repetitions per day (min) | Proportion | % | Frequency per day | Proportion | % |
---|---|---|---|---|---|---|
Percussions | Time per treatment: |
5/12 | 42 | 2 | 8/12 | 67 |
Vibrations | Time per treatment: |
4/12 | 33 | 2 | 5/12 | 42 |
Oscillatory devices | Frequency per treatment: |
4/18 | 22 | 2 | 7/18 | 39 |
Adapted postural drainage |
Time per treatment: |
4/14 | 29 | 2 | 6/14 | 43 |
Positioning | Time per treatment: |
4/19 | 21 | 2 | 5/17 | 29 |
Autogenic drainage | Time per treatment: |
3/10 | 30 | 2 | 5/10 | 50 |
Manually assisted cough | Coughs per treatment: |
9/17 | 53 | Patient dependent | 19/26 | 73 |
Suctioning | Frequency per treatment: |
6/12 | 50 | As required; as needed; patient dependent | 6/9 | 67 |
Inspiratory muscle training |
5 |
2/19 |
11 |
2 |
6/19 |
32 |
Expiratory muscle training (Five participants were unsure) | 5 |
2/16 |
13 |
2 |
5/16 |
31 |
Breathing exercises | Time per treatment: |
5/22 | 23 | 2 | 11/22 | 50 |
Additional comments under the open-text responses related to ACT included the use of hydrotherapy for improved respiratory function, breathing exercises (especially ACBT), percussions, the use of nebulised normal saline for secretion mobilisation, suctioning only if needed and the use of MI-E using both manual and automatic settings. Furthermore, general recommendations for chronic management in NMD included pharmacological intervention, nutrition and hygiene as well as including exercise or games or play therapy in the patients’ home programme to improve their respiratory function.
A summary of the favoured respiratory management treatment strategies, for both acute and chronic management, is provided in
Summary of preferred techniques in acute and chronic management of children with neuromuscular diseases.
Management | Acute ( |
Chronic ( |
---|---|---|
General respiratory care or respiratory support | Non-invasive ventilation |
Non-invasive ventilation |
Secretion mobilisation | Percussions |
Nebulisation |
Airway clearance and cough augmentation or assistance | Manually assisted cough |
Manually assisted cough |
Lung compliance exercises and respiratory muscle training | Not included as part of acute management | Breathing exercises |
O2, oxygen supplementation.
, The majority of the participants were either not aware of MI-E as an alternative cough augmentation option or were aware of the device, but had never used it before.
, 50% of participants were unsure about the use of LVR such as breath-stacking or GPB.
Six (10%) participants responded to this section, as they treated children with NMD on a regular basis and had used respiratory management strategies in this patient cohort within 6 months prior to the survey.
All respondents (
Mechanical insufflation-exsufflation and LVR or lung compliance techniques such as breath-stacking and GPB were not used for either acute or chronic management.
All practicing South African physiotherapists are obligated to register with the Health Professionals Council of South Africa (HPCSA), whilst membership of other professional bodies such as the SASP is voluntary and requires additional annual membership fees. Of the South African physiotherapists registered with the HPCSA, approximately 54% are members of the SASP, most of whom work in private healthcare (Fourie
Most respondents (66%) indicated 1–10 years of NMD experience, but we were unable to show an association between years of experience and clinical practice trends. There was one statistically significant association (
The low response rate (13.3%) limits the generalisability of the survey results, however, the response rate was similar to another survey conducted amongst SASP special interest groups and higher than other online surveys conducted amongst physiotherapists (Clenzos, Naidoo & Parker
The case scenarios both described non-ambulant children presenting with decreased pulmonary function and a poor cough. Similar to clinical recommendations for non-ambulant children with NMD, presenting with associated restrictive lung disease and pulmonary function regression, most survey participants supported NIV during acute (14/44) and especially chronic management (16/27) (Birnkrant et al.
Participants also recommended the use of nebulisation, 24-h postural management and oxygen supplementation during acute and chronic management. Clinical practice guidelines on nebulisation and postural management strategies in children with NMD are limited and are, therefore, usually based on clinical reasoning and patient presentation, as indicated by many participants. Survey participants also seemed to be aware that the chronic use of mycolytics such as hypertonic saline in children with NMD is not advisable, but that inhaled bronchodilators may be considered in children presenting with asthma or bronchial hyperresponsiveness (bronchospasm) (Finkel et al.
Positioning of patients such as side-lying and a variety of sitting positions during acute and chronic management was strongly supported by participants, most likely for the benefit of regional lung ventilation redistribution, resolving unilateral and/or isolated lung infiltration, improved secretion mobilisation and delay of secondary complications (Lupton-Smith et al.
The acute case scenario indicated desaturation (SpO2 < 95%), which could warrant the use of supplemental oxygen. However, hypoxaemia in children with NMD is usually caused by hypoventilation, mucus plugging, atelectasis and/or a respiratory tract infection. Supplemental oxygen should then rather be combined with ventilatory support such as NIV and cough augmentation (proximal ACT) in order to address the underlying cause(s) of the desaturation (Birnkrant et al.
Secretion management with ACT is advocated in order to maintain health, improve health-related quality of life and minimise respiratory complications, which may explain why these techniques were well supported by respondents (Birnkrant et al.
Utilising oscillatory PEP devices for secretion mobilisation was also better supported by respondents during chronic management than during acute management. All the South African physiotherapists who recently worked with children with NMD (
Survey participants correctly indicated the need for cough assistance based on the patient presentation (decreased vital capacity, desaturation and poor cough), which aligns with clinical practice guidelines (Birnkrant et al.
On the contrary, MI-E devices are recommended and used in many developed, high-income countries as an alternative cough augmentation method to curb respiratory deterioration (Chatwin et al.
Another cough augmentation technique, LVR or breath-stacking, can be performed manually (e.g. with an ambubag) or mechanically (with a ventilator or cough assist device) and is recommended in patients with NMD. The benefits of LVR include secretion mobilisation, improved ventilation, cough ability and maintaining lung and chest compliance with the aim of decreasing pulmonary function regression and preventing respiratory morbidity (Castrillo et al.
Besides these advantages, half the survey participants were unsure about the use of LVR during chronic management and only a minority supported the use of cough augmentation techniques such as breath-stacking and GPB (Castrillo et al.
The chronic case scenario depicted a non-ambulant patient with progressed disease and a poor cough effort. Possibly because of the clinical presentation, survey participants could have assumed that the patient would present with severe respiratory muscle weakness and possibly decreased oral control. Not all patients are able to perform GPB or spontaneous breath-stacking as these techniques require good oral control and coordination (Farrero et al.
In SA, physiotherapists as first-line practitioners play a primary role in the multidisciplinary team responsible for evidence-based respiratory management of children with NMD in order to prevent, delay and/or manage respiratory complications (Birnkrant et al.
Specialist care for NMD in SA is limited and there is an urgent need to increase the abilities of South African physiotherapists as well as the establishment of specialised centres with the relevant equipment, ventilatory support and expertise (Birnkrant et al.
This survey provided novel insight into the knowledge, perspectives and implementation of NMD clinical practice guidelines amongst South African physiotherapists. Results from this survey can also inform healthcare managers and policymakers, especially in light of the proposed changes to the South African healthcare system, as well as planning for future clinical trials.
The authors would like to acknowledge Prof. Jennifer Jelsma for assistance with the development of the protocol and initial data analysis.
The authors acknowledge Dr Janine Verstraete and Dr Alison Lupton-Smith for assistance with uploading and correcting the questionnaire on Survey Monkey and assisting with the technicalities of the programme. The authors also acknowledge Dr Verstraete for her valuable input as a critical reader of the manuscript.
The authors would like to acknowledge Prof. H.S. Schoeman for assistance with the statistical analysis on STATA.
The authors would like to acknowledge Mrs Zanne Jordaan for assisting with the analysis of the open-text responses.
The authors acknowledge the SASP (in particular Dr Ina Diener, Chantelle van den Bergh, Dr Alison Lupton-Smith) that assisted in distributing the Survey Monkey link to its members as well as sending reminders on a regular basis.
The authors would also like to thank all the members of the CPRG and Paediatric special interest groups as well as the pilot study participants and experts in the field who took time to complete the survey and provide valuable information.
The authors declare that they have no financial or personal relationships that may have inappropriately influenced them in writing this article.
A.H. was responsible for the conception, design and compilation of the protocol, designing, collation and uploading of the questionnaire (Survey Monkey), collecting data, analysing and interpreting the data (with the assistance of the statistician, primary supervisor and colleague), drafting and correcting the manuscript. L.C. was responsible for correction of the protocol, assistance with correction and collation of questionnaire, interpreting of data and revising the manuscript. B.M. was the primary supervisor who assisted with the conception and design of the protocol, advisory capacity (questionnaire), analysing and interpreting the data, revising the manuscript.
This work forms part of a PhD study that was supported by the URC Equipment Grant (Western Cape), Sefako Makgatho Health Sciences University Research Development Grant and the South African Society of Physiotherapy (PhD grant).
The data that support the findings of this study are available from the corresponding author, A.H., upon reasonable request.
The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of any affiliated agency of the authors.