Non-drug management of breathing problems for children with severe physical and intellectual impairment

Background For a variety of reasons, some children live with very severe intellectual and physical problems; they are unable to walk or talk and require a lot of care. In this study we refer to them as children with severe global developmental delay (SGDD); this is not a specific diagnosis but is rather an 'umbrella term' used to describe a group of children with similar problems. These children may have weak or stiff muscles and deformities of their skeleton; often they have problems with swallowing, resulting in food or saliva going into their lungs. Frequently they have a poor cough reflex and lack the strength required to expel secretions when they do cough. When we sleep, our breathing becomes shallower; for some children with SGDD whose breathing is already shallow when awake, falling asleep means that they do not breathe sufficiently deeply to take in enough oxygen and breathe out enough carbon dioxide. The consequence of these problems is that their respiratory system becomes weakened; they are more likely to develop chest infections, and relatively minor infections can make them very unwell. This can result in their spending a lot of time in hospital. This affects the quality of life for these children and families and is very expensive. Many types of treatment could help, but no good summary of studies has been prepared to tell healthcare professionals which treatments are best and when they should be used; this is the reason for this review.

Review question The aim of our review was to discover how effective each type of treatment is for managing breathing problems in children with severe global developmental delay. As so many treatments are available, we decided to look only at treatments that do not involve drugs.

Study characteristics We carried out a wide database search to look for studies of interventions for the management of breathing problems in children with severe neurological impairment. We found 15 studies of interest, which included a number of different types of treatment.

Key results The results showed that several different treatments provided potential benefits, and for most interventions no serious adverse effects were reported. However, the quality of the studies was not good enough to inspire confidence in the findings. Night-time positioning equipment and spinal bracing were shown to have a potentially negative effect in some participants. Although some studies looked at the same type of treatment, they used it in different ways or used different measures to assess effectiveness, so we could not put the results together.

Quality of the evidence Of the 15 studies included here, only four used the 'gold standard' study design for health interventions. The remainder of the studies used less robust study designs, which limits the strength of the results. Further well-designed randomised studies including larger numbers of participants are required to guide healthcare professionals to select the most effective treatments.

Authors' conclusions: 

This review found no high-quality evidence for any single intervention for the management of respiratory morbidity in children with severe global developmental delay. Our search yielded data on a wide range of interventions of interest. Significant differences in study design and in outcome measures precluded the possibility of meta-analysis. No conclusions on efficacy or safety of interventions for respiratory morbidity in children with severe global developmental delay can be made based upon the findings of this review.

A co-ordinated approach to future research is vital to ensure that high-quality evidence becomes available to guide treatment for this vulnerable patient group.

Read the full abstract...

Children with severe global developmental delay (SGDD) have significant intellectual disability and severe motor impairment; they are extremely limited in their functional movement and are dependent upon others for all activities of daily living. SGDD does not directly cause lung dysfunction, but the combination of immobility, weakness, skeletal deformity and parenchymal damage from aspiration can lead to significant prevalence of respiratory illness. Respiratory pathology is a significant cause of morbidity and mortality for children with SGDD; it can result in frequent hospital admissions and impacts upon quality of life. Although many treatment approaches are available, there currently exists no comprehensive review of the literature to inform best practice. A broad range of treatment options exist; to focus the scope of this review and allow in-depth analysis, we have excluded pharmaceutical interventions.


To assess the effects of non-pharmaceutical treatment modalities for the management of respiratory morbidity in children with severe global developmental delay.

Search strategy: 

We conducted comprehensive searches of the following databases from inception to November 2013: the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, the Allied and Complementary Medicine Database (AMED) and the Cumulative Index to Nursing and Allied Health Literature (CINAHL). We searched the Web of Science and clinical trials registries for grey literature and for planned, ongoing and unpublished trials. We checked the reference lists of all primary included studies for additional relevant references.

Selection criteria: 

Randomised controlled trials, controlled trials and cohort studies of children up to 18 years of age with a diagnosis of severe neurological impairment and respiratory morbidity were included. Studies of airways clearance techniques, suction, assisted coughing, non-invasive ventilation, tracheostomy and postural management were eligible for inclusion.

Data collection and analysis: 

We used standard methodological procedures as expected by The Cochrane Collaboration. As the result of heterogeneity, we could not perform meta-analysis. We have therefore presented our results using a narrative approach.

Main results: 

Fifteen studies were included in the review. Studies included children with a range of severe neurological impairments in differing settings, for example, home and critical care. Several different treatment modalities were assessed, and a wide range of outcome measures were used. Most studies used a non-randomised design and included small sample groups. Only four randomised controlled trials were identified. Non-randomised design, lack of information about how participants were selected and who completed outcome measures and incomplete reporting led to high or unclear risk of bias in many studies. Results from low-quality studies suggest that use of non-invasive ventilation, mechanically assisted coughing, high-frequency chest wall oscillation (HFCWO), positive expiratory pressure and supportive seating may confer potential benefits. No serious adverse effects were reported for ventilatory support or airway clearance interventions other than one incident in a clinically unstable child following mechanically assisted coughing. Night-time positioning equipment and spinal bracing were shown to have a potentially negative effect for some participants. However, these findings must be considered as tentative and require testing in future randomised trials.