Description of the condition
Improvement in neonatal and paediatric care in recent decades has resulted in the survival of increasing numbers of children with multiple and profound impairment. Babies born early or in frail condition are being successfully treated and surviving into childhood. Young children suffering serious neurological impairment as a result of infection, illness or trauma are also surviving longer, as are children with various genetic, neuromuscular and metabolic disorders (Blucker 2011).
While some of these children go on to lead normal lives, others are left with severe impairment in all aspects of functioning. While the causes of these impairments are varied, these children bear the illness burden of profound and multiple disabilities, and for research purposes may be grouped under the label of 'severe global developmental delay' (SGDD).
SGDD is therefore an umbrella term, broadly capturing a population of children with severe intellectual disability and severe motor impairment, most of whom will be extremely limited in their functional movement, and are dependent upon others for all activities of daily living.
Their physical handicap and associated musculoskeletal deformities lead to high levels of respiratory morbidity as a consequence of the combination of several factors which may include; excessive salivation (sialorrhoea), swallowing problems (dysphagia), gastro-oesophageal reflux (GOR), ineffective cough, immobility, chest wall deformity, weakness of respiratory muscles, small airways collapse (atelectasis), damaged lung tissue (bronchiectasis), sleep disordered breathing (nocturnal hypoventilation),aspiration and respiratory failure.
Description of the intervention
The respiratory problems experienced by this patient group are often multi-faceted and complex, and a myriad of interventions are applied in clinical practice, including chest physiotherapy, suction, mechanical insufflation-exsufflation (MIE), non-invasive ventilation (NIV), postural management and tracheostomy.
How the intervention might work
The rationale by which these interventions may decrease respiratory morbidity broadly include improved ventilation, enhanced ventilation-perfusion (V/Q) matching, and assistance in mobilisation and expectoration of secretions.
Why it is important to do this review
Patients with SGDD do not share a specific diagnosis and some have no definitive diagnosis - representative statistics are difficult to obtain. As a result of the heterogeneity of these patients and the breadth of factors impacting upon their respiratory health, comprehensive review of the literature exists.
Current research into paediatric respiratory care tends to focus on very specific groups of patients, such as those with cystic fibrosis, spinal muscular atrophy or Duchenne's muscular dystrophy, with consensus guidelines published for the respiratory care of some of these groups (Finder 2004; Hull 2012; Wang 2007). The available literature reflects that respiratory compromise is a major, if not the largest, cause of mortality for individuals with cerebral palsy (Augustine 2010; Fitzgerald 2009; Healy 2010; Littleton 2011; Somerville 2008; Sullivan 2006), neuromuscular disorders (Katz 2004; Panitch 2009; Simonds 2006) and severe developmental disability (Mestrovic 2006; O'Loughlin 2009). It is also a major cause of morbidity (Chatwin 2003; Fitzgerald 2009; Healy 2010; Seddon 2003; Yuan 2010) requiring hospital admissions and impacting upon quality of life.
In the United Kingdom (UK), children with SGDD often end up in costly acute care for prolonged periods of time. In the current economic and political climate, there is a drive toward community-based care for long-term conditions. Improved access to specialist equipment and trained staff would allow treatment of sub-acute and chronic respiratory conditions in the community, facilitate timely discharge and prevent hospital readmissions. A systematic review that brings together the range of management options in a way which can inform best practice is therefore timely.
Quality research is necessary to ensure that this growing population of vulnerable children receive equity of care and that treatment is safe and effective and enhances quality of life for them and their families.