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Cystic fibrosis and the transition to adult health services
  1. Simon C Langton Hewer1,
  2. Jennifer Tyrrell2
  1. 1
    Department of CF and Respiratory Medicine, Bristol Royal Hospital for Children, Bristol, UK
  2. 2
    Department of Paediatrics, Royal United Hospital, Bath, UK
  1. Dr S C Langton Hewer, Department of CF and Respiratory Medicine, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol BS2 8NB, UK; simon.langtonhewer{at}

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There is no other condition quite like cystic fibrosis (CF). Faulty genes that the parents usually did not know they carried have caused it. The newborn baby will usually be free of any problems, but the young child is likely to have problems with weight gain and with frequent respiratory infections. Once the diagnosis has been made, the family must learn a complex new set of rules: medication needs to be given several times every day, and chest physiotherapy must be learned and performed twice a day, sometimes more. The family and later the child need to become medical experts: they will need to recognise when they need to seek extra help from their CF team—how do they know when the child is coughing enough to need another appointment? They will need to learn to work with a whole range of willing and enthusiastic practitioners including doctors, nurses, physiotherapists, dieticians, psychologists, social workers and pharmacists. Not to mention the additional worries of school absences for inpatient and outpatient consultations. Then on top of that there are the family crises—not just the normal ones that all families will go through, but those when the child may grow Pseudomonas aeruginosa for the first time and a 2-week hospital admission is required, often with minimal advance notice. As the child grows up, they will be expected to learn more about their health and how to maintain it, with a gradual reduction in responsibility from their parents and the expectation that they will become largely independent of parental input.

There is convincing evidence in favour of improving survival for patients with CF, despite the fact that there is as yet no definitive treatment to cure the defect at a cellular level.1 2 This improved survival is a reflection in part of the improving …

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