People With Cystic Fibrosis
The review includes 13 studies (five new at this update) representing data from 529 participants. Studies mainly assessed behavioural and educational interventions:1. gene pre-test education counselling for relatives of those with CF;2. biofeedback, massage and music therapy to assist physiotherapy;3. behavioural and educational interventions to improve dietary intake and airway clearance;4. self-administration of medication and education to promote independence, knowledge and quality of life; and5. systemic interventions promoting psychosocial functioning.A substantial proportion of outcomes were educational or behavioural relating to issues of adherence, change in physical status or other specific treatment concerns during the chronic phase of the disease. Some evidence was found for relative's acceptance of a genetic test for carrier status when using home-based rather than clinic-based information leaflets and testing. There is some evidence that behavioural interventions improve emotional outcomes in people with CF and their carers, and that psychoeducational interventions improve knowledge in the short term. There was no consistent effect on lung function, although one small study showed that biofeedback-assisted breathing re-training helped improve some lung function measurements. Some studies point to educational and behavioural interventions aiding nutrition and growth in people with CF. Currently there is insufficient evidence for interventions aimed at other aspects of the disease process.