Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline.

Physiotherapy management is a key element of care for people with cystic fibrosis (CF) throughout the lifespan. Although considerable evidence exists to support physiotherapy management of CF, there is documented variation in practice. The aim of this guideline is to optimize the physiotherapy management of people with CF in Australia and New Zealand. A systematic review of the literature in key areas of physiotherapy practice for CF was undertaken. Recommendations were formulated based on National Health and Medical Research Council (Australia) guidelines and considered the quality, quantity and level of the evidence; the consistency of the body of evidence; the likely clinical impact; and applicability to physiotherapy practice in Australia and New Zealand. A total of 30 recommendations were made for airway clearance therapy, inhalation therapy, exercise assessment and training, musculoskeletal management, management of urinary incontinence, managing the newly diagnosed patient with CF, delivery of non-invasive ventilation, and physiotherapy management before and after lung transplantation. These recommendations can be used to underpin the provision of evidence-based physiotherapy care to people with CF in Australia and New Zealand.

Lifestyle Implications of Home Mechanical Insufflation-Exsufflation for Children With Neuromuscular Disease and Their Families.

BACKGROUND: Mechanical insufflation-exsufflation (MI-E) is increasingly used in the home management of children with neuromuscular disease. Research to date has focused on the effect of MI-E on physical health. The aim of this study was to qualitatively investigate the impact of home MI-E on the child and family's lifestyle. METHODS: Eight parents and 3 children participated in semistructured interviews. RESULTS: Five themes emerged from parent interviews demonstrating: (1) lifestyle implications, (2) parents becoming experts, (3) parents developing a sense of control over their child's condition, (4) an element of extra care, and (5) impacts on the parent-child relationship. Developing themes from the child interviews showed them adjusting to and then relying on the device. Home MI-E medicalized the home, but the overall lifestyle impact was positive. CONCLUSIONS: Although involving a small number of subjects, this study demonstrated a mixture of opposing impacts of home MI-E on lifestyle, both enabling and disabling, which need to be considered when introducing home MI-E. The positive impacts included greater ability to manage the child's health, including avoidance of hospital admissions. Negative impacts were greatest for those parents who were sole operators of the device, including a frequently disrupted lifestyle.

Effect of home mechanical in-exsufflation on hospitalisation and life-style in neuromuscular disease: a pilot study.

AIM: Mechanical in-exsufflation (MI-E) augments the weakened cough of patients with neuromuscular disease (NMD), clearing secretions and overcoming atelectasis. Little has been published on the impact of MI-E alone on rates of hospitalisation and quality of life (QOL). The aim of this study was to assess the impact of home MI-E on hospital admissions and life-style in children with NMD. METHODS: A retrospective chart review was performed on children using MI-E, including data on the number of admissions to hospital, length of stay and hours of ventilation. A parental survey was used to gather information on the impact of MI-E on life-style for the child and family. RESULTS: Ten children with NMD (seven spinal muscular atrophy, two Duchenne muscular dystrophy and one centronuclear myopathy) using MI-E at home were identified. MI-E use commenced at mean age of 8.5 years (range 1.1-16.9) with 1.4 years of use (range 0.3-3.8). MI-E pressures ranged from +/-30 to 40 cmH2 O with no complications reported. There was a significant reduction in hospital days at 6 (P = 0.036) and 12 (P = 0.028) months following commencement of home MI-E compared with the same period preceding MI-E use. The survey highlighted positive benefits of MI-E use, in particular the ability to treat many pulmonary exacerbations at home. CONCLUSIONS: Home MI-E use by children with NMD can reduce hospitalisation and benefit families by maintaining their child at home.