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Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional studyCommentary: Management in paediatric and adult cystic fibrosis centres improves clinical outcome

BMJ 1998; 316 doi: https://doi.org/10.1136/bmj.316.7147.1771 (Published 13 June 1998) Cite this as: BMJ 1998;316:1771

Abstract

Objectives: To assess the effect on clinical outcome of managing paediatric and adult patients with cystic fibrosis at specialised cystic fibrosis centres.

Design: Cross sectional study.

Setting: Two adult cystic fibrosis centres in the United Kingdom.

Subjects: Patients from an adult cystic fibrosis centre in Manchester were subdivided into those who had received continuous care from paediatric and adult cystic fibrosis centres (group A), and those who had received paediatric care in a centre not specialising in cystic fibrosis followed by adult care in a cystic fibrosis centre (group B). Group C were referrals to the new adult cystic fibrosis centre in Cambridge who had received neither paediatric nor adult centre care for their cystic fibrosis.

Main outcome measures: Body mass index (weight (kg)/height (m2)), lung function (forced expiratory volume in one second (FEV1 percentage of predicted)), the Northern chest x ray film score, and age at colonisation with Pseudomonas aeruginosa.

Results: A prominent stepwise increase in body mass index was associated with increasing amounts of care at a cystic fibrosis centre; 18.3, 20.2, and 21.3 for groups C, B, and A respectively (P<0.001). Improved nutritional status was correlated with a higher FEV1 and better (lower) chest x ray film scores; r=0.52 and −0.45 respectively (P<0.001 for both).

Conclusion: These findings provide the first direct evidence that management of cystic fibrosis in paediatric and adult cystic fibrosis centres results in a better clinical outcome, and strongly supports the provision of these specialist services.

Key messages

  • Management of patients with cystic fibrosis in paediatric and adult cystic fibrosis centres results in an improved clinical outcome

  • Improved clinical outcome occurred in cystic fibrosis centres despite earlier and more frequent colonisation with Pseudomonas aeruginosa

  • Nutritional status is an important predictor of lung disease in cystic fibrosis

Footnotes

    • Accepted 4 February 1998

    Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional study

    1. Ravi Mahadeva, Anglia and Oxford research fellowa,
    2. Kevin Webb, consultant physicianb,
    3. Roger C Westerbeek, biostatisticianc,
    4. Nick R Carroll, senior registrard,
    5. Mary E Dodd, senior physiotherapistb,
    6. Diana Bilton, consultant physiciane,
    7. David A Lomas, honorary consultant physiciana
    1. aDepartment of Haematology, University of Cambridge, Medical Research Council Centre, Cambridge CB2 2QH,
    2. bBradbury Cystic Fibrosis Unit, Wythenshawe Hospital NHS Trust, Manchester M23 9LT,
    3. cMedical Research Council Biostatistics Unit, University of Cambridge CB2 2QQ,
    4. dAddenbrooke's Hospital NHS Trust, Cambridge CB2 2QQ,
    5. eCystic Fibrosis Unit, Papworth Hospital NHS Trust, Papworth Everard, Cambridge CB3 8RE
    6. Department of Child Health, Singleton Hospital, Swansea, West Glamorgan SA2 8QA
    1. Correspondence to: Dr Bilton
    • Accepted 4 February 1998

    Abstract

    Objectives: To assess the effect on clinical outcome of managing paediatric and adult patients with cystic fibrosis at specialised cystic fibrosis centres.

    Design: Cross sectional study.

    Setting: Two adult cystic fibrosis centres in the United Kingdom.

    Subjects: Patients from an adult cystic fibrosis centre in Manchester were subdivided into those who had received continuous care from paediatric and adult cystic fibrosis centres (group A), and those who had received paediatric care in a centre not specialising in cystic fibrosis followed by adult care in a cystic fibrosis centre (group B). Group C were referrals to the new adult cystic fibrosis centre in Cambridge who had received neither paediatric nor adult centre care for their cystic fibrosis.

    Main outcome measures: Body mass index (weight (kg)/height (m2)), lung function (forced expiratory volume in one second (FEV1 percentage of predicted)), the Northern chest x ray film score, and age at colonisation with Pseudomonas aeruginosa.

    Results: A prominent stepwise increase in body mass index was associated with increasing amounts of care at a cystic fibrosis centre; 18.3, 20.2, and 21.3 for groups C, B, and A respectively (P<0.001). Improved nutritional status was correlated with a higher FEV1 and better (lower) chest x ray film scores; r=0.52 and −0.45 respectively (P<0.001 for both).

    Conclusion: These findings provide the first direct evidence that management of cystic fibrosis in paediatric and adult cystic fibrosis centres results in a better clinical outcome, and strongly supports the provision of these specialist services.

    Key messages

    • Management of patients with cystic fibrosis in paediatric and adult cystic fibrosis centres results in an improved clinical outcome

    • Improved clinical outcome occurred in cystic fibrosis centres despite earlier and more frequent colonisation with Pseudomonas aeruginosa

    • Nutritional status is an important predictor of lung disease in cystic fibrosis

    Footnotes

      • Accepted 4 February 1998

      Commentary: Management in paediatric and adult cystic fibrosis centres improves clinical outcome

      1. J A Dodge, honorary consultant
      1. aDepartment of Haematology, University of Cambridge, Medical Research Council Centre, Cambridge CB2 2QH,
      2. bBradbury Cystic Fibrosis Unit, Wythenshawe Hospital NHS Trust, Manchester M23 9LT,
      3. cMedical Research Council Biostatistics Unit, University of Cambridge CB2 2QQ,
      4. dAddenbrooke's Hospital NHS Trust, Cambridge CB2 2QQ,
      5. eCystic Fibrosis Unit, Papworth Hospital NHS Trust, Papworth Everard, Cambridge CB3 8RE
      6. Department of Child Health, Singleton Hospital, Swansea, West Glamorgan SA2 8QA
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