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Letter
Prion disease: clinical pathway development for the terminally ill
  1. Roseanagh Hogg1,2,
  2. John Centola3,
  3. Kirsty Durley1,
  4. Chloe Antoinette Chin4,
  5. Rachel Quibell5,
  6. Helena Spriggs6,
  7. Matthew Carey2,7,
  8. Tomasz Bajorek8,
  9. Mary Miller2,
  10. Victoria Bradley2 and
  11. Suvankar Pal3
  1. 1Palliative Medicine, Health Education Thames Valley - HETV, Oxford, UK
  2. 2Palliative Medicine, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  3. 3Neurology, National CJD Research and Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK
  4. 4Palliative Medicine, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  5. 5Palliative Medicine, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK
  6. 6Health Education North East, Newcastle upon Tyne, England, UK
  7. 7Palliative Medicine, Sir Michael Sobell House Hospice, Oxford, UK
  8. 8Psychological Medicine, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  1. Correspondence to Dr Roseanagh Hogg, Palliative Medicine, Oxford University Hospitals NHS Foundation Trust, Oxford, Oxfordshire, UK; r.hogg1{at}nhs.net

https://doi.org/10.1136/spcare-2022-003877

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    Dear Editor,

    Prion diseases are devastating, rapidly progressive and uniformly fatal neurodegenerative conditions for which there is no cure.1 BMJ Best Practice advocates a focus on symptom management and quality of life and provides suggestions as to how to approach some more common symptoms of prion disease.2 However, to our knowledge, there are currently no published comprehensive clinical guidelines for management of prion diseases. While a general supportive approach and early involvement of palliative care may benefit, there is an urgent unmet need for an evidence-based and consensus-based approach for best management of people with these complex disorders.1 We have endeavoured to explore current practice across three large tertiary centres in collaboration with the UK National CJD Research and Surveillance Unit. This comprised a systematic and structured retrospective review of case records of people with prion disease recently assessed across the centres to understand current trends and inform future direction. We extracted information from case records of 30 individuals including type of Creutzfeldt-Jakob disease, presenting symptom complex, latency of referral to palliative care services, reasons for referral, overall prognosis, members of the interdisciplinary team involved, treatment approaches implemented and final place of death. Our overall aim was to highlight common …

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    Footnotes

    • Twitter @drchlo, @dr_mary_miller, @suvankarpal

    • Contributors Data collection was completed by KD, RQ, HS and CAC. RH reviewed the data and drafted the manuscript. The manuscript was reviewed and edited by SP, VB, MM, TB, JC and MC in addition to the other authors listed above. All authors are part of a working group currently looking at symptom management in prion disease, and working towards developing an evidence base and clinical guideline.

    • 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.

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

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.