Breaking bad news, the diagnosis of cystic fibrosis in childhood.

BACKGROUND: The day parents are told their child has cystic fibrosis (CF) is imprinted in their memory. Parents often show strong emotions (e.g. shock, anxiety); they need to cope with bad news and restructure their lives taking into account CF. AIMS: The aims of this study are (1) to explore how parents recall circumstances of the CF diagnosis and the information they received and (2) to investigate their current coping styles. METHODS: Parents (n=38) of 20 children (diagnosed during the past 5 years) were interviewed using a semi-structured interview. Coping was assessed using the Utrecht Coping List. The association between coping and time since diagnosis/severity of illness was investigated. RESULTS: Fifteen parents first heard the term 'CF' from their local pediatrician or GP. All were informed in detail by the CF specialist. All parents recalled specifics about the information, the attitude of the doctor, their thoughts and emotions. Most parents were satisfied with the content and manner in which they had received information. Nineteen appreciated the doctor showing some emotions during the talks. One couple criticized the doctor for not showing emotions. Parents reported higher use (than normative scores) of the active coping style 'social support seeking' and the accommodative coping styles 'palliative reaction pattern' and 'comforting cognitions'. Perception of severity of illness was associated with higher scores on palliative coping. CONCLUSIONS: This study shows the importance of physicians and CF teams to tailor the way of providing bad news to parents' needs and preferences. It is important to help and encourage parents to use active or accommodative coping strategies. The diagnosis is the starting point of a long-term relationship. 'Doing things well from the start' helps families to learn to live with CF and treatment.

European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre.

A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those who are not. Key to the effectiveness of the specialist CF Centre is the multidisciplinary team (MDT), which should include consultants, clinical nurse specialist, microbiologist, physiotherapist, dietitian, pharmacist, clinical psychologist, social worker, clinical geneticist and allied healthcare professionals, all of whom should be experienced in CF care. Members of the MDT are also expected to keep up to date with developments in CF through continued professional development, attendance at conferences, auditing and involvement in research. Specialists CF Centres should also network with other Centres both nationally and internationally, and feed Centre data to registries in order to further the understanding of the disease. This paper provides a framework for the specialist CF Centre, including the organisation of the Centre and the individual roles of MDT members, as well as highlighting the value of CF organisations and disease registries.