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Identifying and managing respiratory disorders associated with cerebral palsy: protocols for monitoring respiratory health
Evidence review C1
NICE Guideline, No. 119
National Guideline Alliance (UK).
Identifying and managing respiratory disorders
Review question
C1 What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
Introduction
Adults with cerebral palsy are at increased risk of respiratory health problems. This may be due to a variety of co-morbidities, including gastro-oesophageal reflux, aspiration of feed or secretions, reduced functional lung volume, muscle tone in the form of respiratory muscle weakness and some side effects of regularly used medication. Identifying adults with these risks and re-appraising risk may help prevent infection, and delay respiratory failure. This review question looks at the evidence of clinical and cost effectiveness for methods of identification and monitoring respiratory disorders.
PICO/PIRO table
Please see Table 1 for a summary of the Population, Intervention/Index test, Comparison/Reference standard and Outcome (PICO/PIRO) characteristics of this review.
Table 1
Summary of the protocol (PICO/PIRO table).
For full details see review protocol in appendix A.
Methods and process
This evidence review was developed using the methods and process described in Developing NICE guidelines: the manual 2014. Methods specific to this review question are described in the review protocol in appendix A and for a full description of the methods see supplementary document C.
As GRADE is designed only for RCTs and observational studies, a modified version of this tool was used in order to appraise the confidence in the included diagnostic test accuracy evidence. The QUADAS-2 checklist risk of bias and applicability items were used for evaluating the risk of bias and indirectness, respectively, of the studies. The quality assessment of inconsistency and imprecision were adapted to take into account the methodological features of diagnostic studies.
GRADE was not used for evidence about clinimetric properties (such as reliability or construct validity), methodological quality was summarised for each publication individually using the consensus-based standards for the selection of health status measurement instruments (COSMIN) checklist for individual studies or the CASP checklist for systematic reviews.
Declaration of interests were recorded according to NICE’s 2014 conflicts of interest policy from May 2016 until April 2018. From April 2018 onwards they were recorded according to NICE’s 2018 conflicts of interest policy. Those interests declared until April 2018 were reclassified according to NICE’s 2018 conflicts of interest policy (see Interests Register).
Clinical evidence
Included studies
Two studies were included. One was a cross-sectional study (Lampe 2014; number of participants in study, N=46), the other was a systematic review including 7 cross-sectional studies (Lennon 2014; N=117).
Lampe 2014 compared chest expansion measured with a tape, lung capacity measured using spirometry and oxygen saturation measured using pulse-oximetry in adults with cerebral palsy. Lennon 2014 was a systematic review of the clinical usefulness of aerobic or anaerobic fitness measures in adults with cerebral palsy.
Lampe 2014 did not report the sensitivity or specificity of reduced lung capacity for low oxygen saturation, but provided sufficient information for the NGA team to calculate these statistics for a number of threshold values (see appendix E, Figure 3). The threshold value closest to the threshold agreed in the review protocol for high sensitivity (90%; see appendix A) was used in the modified GRADE analysis.
The clinical studies included in this evidence review are summarised in Table 2 and evidence from these is summarised in the clinical evidence profiles below (Table 3 and Table 4).
See also the literature search strategy in appendix B, study selection flow chart in appendix C, sensitivity/specificity plots in appendix E and study evidence tables in appendix D.
Excluded studies
No studies were excluded from this review.
Summary of clinical studies included in the evidence review
Table 2 summarises the characteristics of the included studies
Table 2
Summary of included studies.
Quality assessment of clinical studies included in the evidence review
The clinical evidence profiles for this review question are presented in Table 3 and Table 4.
Table 3
Clinical evidence profile for diagnostic accuracy of reduced lung vital capacity (1 litre or more lower than normal) with spirometry for prediction of low oxygen saturation (<96%) in adults with cerebral palsy.
Table 4
Clinimetric properties of bicycle ergometers, wheelchair ergometers and the 6 minute walk test to determine maximal aerobic capacity in adults with cerebral palsy.
Economic evidence
Included studies
A systematic review of the economic literature was conducted but no studies were identified which were applicable to this review question.
Excluded studies
No studies were identified which were applicable to this review question.
Summary of studies included in the economic evidence review
No economic evaluations were included in this review
Economic model
This question was not prioritised for economic modelling as the committee considered that it was unlikely that any recommendation made would place significant additional costs on NHS or PSS budgets.
Resource impact
No unit costs were presented to the committee as these were not prioritised for decision making purposes.
Evidence statements
Monitoring of respiratory health
No evidence was found about the impact of regular monitoring of respiratory health on outcomes in adults with cerebral palsy.
Lung vital capacity measured using spirometry
Critical outcomes
Respiratory health
No evidence was found for this outcome.
Overall survival
No evidence was found for this outcome.
Hospital admission
No evidence was found for this outcome.
Important outcomes
Secondary conditions
No evidence was found for this outcome.
Respiratory function
No evidence was found for this outcome.
Health related quality of life
No evidence was found for this outcome.
Satisfaction
No evidence was found for this outcome.
Diagnostic accuracy
- Very low quality evidence from one cross-sectional study in 46 adults with cerebral palsy indicates that lung vital capacity (measured using spirometry) is reduced in adults with cerebral palsy compared to people without cerebral palsy, particularly in those with higher GMFCS scores or scoliosis. Reduced lung vital capacity (of at least 1 litre below the predicted normal value), however, is not a good predictor of oxygen saturation with sensitivity of 86% and specificity of 26% for low oxygen saturation. The positive and negative likelihood ratios of 1.16 and 0.55 respectively suggest this test is not useful for ruling low oxygen saturation in or out.
Clinimetric properties
No evidence was found for this outcome.
Tests for maximal aerobic capacity
Critical outcomes
Respiratory health
No evidence was found for this outcome.
Overall survival
No evidence was found for this outcome.
Hospital admission
No evidence was found for this outcome.
Important outcomes
Secondary conditions (e.g. colds, asthma, sleep apnoea, daytime sleepiness)
No evidence was found for this outcome.
Respiratory function
No evidence was found for this outcome.
Health related quality of life
No evidence was found for this outcome.
Satisfaction
No evidence was found for this outcome.
Diagnostic accuracy
No evidence was found for this outcome.
Clinimetric properties
Very low quality evidence from one systematic review including 7 cohort studies in 117 teenagers or adults with cerebral palsy who were ambulatory or self-propelled wheelchair users indicates that lab-based maximal or sub-maximal bicycle and wheelchair ergometer tests are valid tests of aerobic fitness. The 6-minute walk test however is not a valid measure of maximal aerobic capacity.
The committee’s discussion of the evidence
Interpreting the evidence
The outcomes that matter most
The critical outcomes were respiratory health, overall survival and hospital admission because poor respiratory function can lead to life threatening illnesses requiring hospital admission. There was a lack of evidence about the impact of regular monitoring of respiratory function on these critical outcomes so the committee instead considered evidence about the accuracy of tests for respiratory function with the assumption that early diagnosis and treatment of respiratory problems should improve overall health. Important outcomes were secondary conditions (such as colds), respiratory function, health related quality of life and satisfaction
The quality of the evidence
For outcomes from one study a GRADE approach was used that was modified for diagnostic accuracy measures. Outcomes for the other study could only be assessed using the CASP quality checklist. Evidence for all outcomes was rated as very low quality. The included study measured oxygen saturation measured at a single point during the day and the committee agreed this would be less informative about early respiratory failure than a nocturnal monitoring protocol.
The committee considered that evidence about maximal aerobic capacity tests was not relevant to the general population of adults with cerebral palsy, because such tests would be typically used for monitoring the cardiorespiratory fitness of athletes as part of their training programme.
Due to the limitations of the evidence the committee based their recommendations on their expertise and experience.
Benefits and harms
The committee based on their experience and knowledge, agreed that there is a lack of awareness about some of the signs and symptoms that may indicate respiratory impairment. They therefore wanted to describe some of the presentations associated with respiratory impairment to improve recognition and identification of the condition. Timely assessment would also ensure prompt discussion about treatment options.
The committee agreed, based on their experience that respiratory impairment coupled with certain comorbidities could result in respiratory complications. The committee agreed that a referral for a full respiratory assessment was likely to be beneficial in this group as it would afford the chance to prevent or treat respiratory complications. This would also lead to effective management and prevention of further complications. The committee recognised that this recommendation may lead to an increase in the number of referrals, but the resource impact will be balanced by reduced number of complications.
There was also some evidence that oxygen saturation and lung vital capacity measured using spirometry were reduced in those with GMFCS IV to V or with kyphoscoliosis. However, they could not make a strong recommendation for this because the evidence was very limited and of poor quality.
Due to the lack of evidence, the committee made a research recommendation on the methods of detection and management of respiratory impairment in adults with cerebral palsy in the community. Based on their experience, the committee were aware that adults with cerebral palsy are at increased risk of respiratory problems particularly people with some pre-existing respiratory conditions and those with high Gross Motor Function Classification System (GMFCS) and are at a high risk of serious adverse effects. Early detection of respiratory impairment, management and appropriate referral for specialist assessment would enable prevention or treatment of respiratory complications in this high-risk group.
Cost effectiveness and resource use
The committee noted that no relevant published economic evaluations had been identified for this topic.
The committee acknowledge that the recommendations would lead to an increase in referrals to a limited number of respiratory specialists with experience in adults with cerebral palsy potentially increasing waiting times or diverting the resource from elsewhere. However, improved outcomes, especially in regards to respiration will lead to a significant increase in quality of life. Some additional resource use will be recouped though a decrease in hospital admissions especially expensive unplanned admissions.
References
Lampe 2014
Lampe, R., Blumenstein, T., Turova, V., Alves-Pinto, A., Lung vital capacity and oxygen saturation in adults with cerebral palsy, Patient Preference and Adherence, 8, 1691–1697, 2014 [PMC free article: PMC4267512] [PubMed: 25525345]Lennon 2015
Lennon, N., Thorpe, D., Balemans, A. C., Fragala-Pinkham, M., O’Neil, M., Bjornson, K., Boyd, R., Dallmeijer, A. J., The clinimetric properties of aerobic and anaerobic fitness measures in adults with cerebral palsy: A systematic review of the literature, Research in Developmental Disabilities, 45–46, 316–28, 2015 [PubMed: 26296079]
Appendices
Appendix A. Review protocols
Review protocol for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
Table 5. Review protocol for monitoring respiratory health in adults with cerebral palsy (PDF, 336K)
Appendix B. Literature search strategies
Literature search strategies for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
This appendix is a combined search strategy and will be the same for all the evidence reviews for the C review questions as listed below:
- C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
- C2: Does assisted ventilation improve quality of life for adults with cerebral palsy who have a chronic respiratory disorder (including respiratory failure)?
- C3: Are prophylactic treatments (for example, antibiotics, chest physiotherapy, cough assistance) effective in preventing respiratory infections in adults with cerebral palsy?
Database: Medline & Embase (Multifile)
Database(s): Embase 1974 to 2018 March 22, Ovid MEDLINE(R) In-Process & Other Non-Indexed Citations and Ovid MEDLINE(R) 1946 to Present
Database: Cochrane Library
Database: Web of Science
Appendix C. Clinical evidence study selection
Clinical evidence study selection for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
Figure 1. Flow diagram of clinical article selection for this review
Appendix D. Clinical evidence tables
Clinical evidence tables for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
Table 9. Studies included in monitoring respiratory health in adults with cerebral palsy (PDF, 320K)
Appendix E. Forest plots
Forest plots for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
Lung vital capacity measured with spirometry
Appendix F. GRADE tables
GRADE tables for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
Appendix G. Economic evidence study selection
Economic evidence study selection for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
No economic evidence was identified for this review.
Appendix H. Economic evidence tables
Economic evidence tables for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
No economic evidence was identified for this review.
Appendix I. Health economic evidence profiles
Health economic evidence profiles for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
No economic evidence was identified for this review.
Appendix J. Health economic analysis
Health economic analysis for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
No economic analysis was included in this review.
Appendix K. Excluded studies
Clinical and economic lists of excluded studies for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
Clinical studies
No studies were excluded from this review.
Economic studies
No economic evidence was identified for this review.
Appendix L. Research recommendations
Research recommendations for review question C1: What is the most effective protocol for monitoring respiratory health in adults with cerebral palsy?
Can detection and management of respiratory disorder in adults with cerebral palsy be improved in primary and community care?
Final
Evidence reviews
These evidence reviews were developed by the National Guideline Alliance, hosted by the Royal College of Obstetricians and Gynaecologists
Disclaimer: The recommendations in this guideline represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, professionals are expected to take this guideline fully into account, alongside the individual needs, preferences and values of their patients or service users. The recommendations in this guideline are not mandatory and the guideline does not override the responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or their carer or guardian.
Local commissioners and/or providers have a responsibility to enable the guideline to be applied when individual health professionals and their patients or service users wish to use it. They should do so in the context of local and national priorities for funding and developing services, and in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities. Nothing in this guideline should be interpreted in a way that would be inconsistent with compliance with those duties.
NICE guidelines cover health and care in England. Decisions on how they apply in other UK countries are made by ministers in the Welsh Government, Scottish Government, and Northern Ireland Executive. All NICE guidance is subject to regular review and may be updated or withdrawn.
- Review Assessing and monitoring complications and comorbidities: feeding and nutritional problems: Cerebral palsy in adults: Evidence review B3[ 2019]Review Assessing and monitoring complications and comorbidities: feeding and nutritional problems: Cerebral palsy in adults: Evidence review B3National Guideline Alliance (UK). 2019 Jan
- Review Identifying and managing respiratory disorders associated with cerebral palsy: prophylactic treatments: Cerebral palsy in adults: Evidence review C3[ 2019]Review Identifying and managing respiratory disorders associated with cerebral palsy: prophylactic treatments: Cerebral palsy in adults: Evidence review C3National Guideline Alliance (UK). 2019 Jan
- Review Dyspepsia and Gastro-oesophageal Reflux: A Systematic Review[ 2007]Review Dyspepsia and Gastro-oesophageal Reflux: A Systematic ReviewSwedish Council on Health Technology Assessment. 2007 Oct
- Review Feed thickener for infants up to six months of age with gastro-oesophageal reflux.[Cochrane Database Syst Rev. 2017]Review Feed thickener for infants up to six months of age with gastro-oesophageal reflux.Kwok TC, Ojha S, Dorling J. Cochrane Database Syst Rev. 2017 Dec 5; 12(12):CD003211. Epub 2017 Dec 5.
- Review Gastro-oesophageal reflux treatment for asthma in adults and children.[Cochrane Database Syst Rev. 2003]Review Gastro-oesophageal reflux treatment for asthma in adults and children.Gibson PG, Henry RL, Coughlan JL. Cochrane Database Syst Rev. 2003; (2):CD001496.
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