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State of the science
Living and dying with cystic fibrosis
  1. Mary Miller1,2
  1. 1 Palliative Care, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  2. 2 Honorary Senior Clinical Lecturer, Nuffield Department of Medicine, Oxford University, Oxford, UK
  1. Correspondence to Dr Mary Miller, Department of Palliative Care, Sobell House, Oxford University Hospitals NHS Foundation Trust, Oxford OX3 7LE, UK; mary.miller{at}ouh.nhs.uk

https://doi.org/10.1136/bmjspcare-2020-002631

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    Throughout history, it has been recognised a ‘salty’ child would die in infancy. Over the past 80 years, knowledge about cystic fibrosis (CF), including genetic mutations and healthcare interventions, has developed at pace. Dr Dorothy Andersen described the pathological changes in the pancreas in 1938. In November 2019, a phase 3 randomised controlled trial of triple therapy (elexacaftor–tezacaftor–ivacaftor) suggested that it will benefit 90% of all people with CF.1

    The CF specialty thinks critically about the care it delivers, measures outcomes and conducts research.2 It has achieved much, pushing the age of death from childhood in the 1960s to middle age now. Of the 137 people who died with CF in the UK in 2018, the median age was 32 years.3 It is hoped a child born today will live to 47.3 years (44.1—women, 51—men).3 Triple therapy and further research developments will further extend lifespan towards late middle age in this incurable disease. There remains much to learn about triple therapy. Some patients will be ineligible; there are diverse CF genetic mutations and globally many will not have access to these expensive drugs.

    What is known of symptom prevalence and management in CF?

    Symptoms are common and troublesome—a median of 10 symptoms were reported when the Memorial Symptom Assessment Scale was administered to 303 people with CF.4 Cough was reported by 94%, breathlessness by 77%, lack of energy by 77% and pain by …

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    Footnotes

    • Twitter @dr_mary_miller

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Not required.

    • Provenance and peer review Not commissioned; internally peer reviewed.