Palliative care in Creutzfeldt-Jakob disease: looking back, thinking ahead

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disease for which there is no cure. However, it is difficult to diagnose and is unique in that it is both a genetic and transmissible disease. The disease is characterised by symptoms of a rapidly progressive dementia. Palliation is the only treatment and early diagnosis is an important aspect in relation to gaining speedy access to palliative and end-of-life care services. People with CJD may be cared for in a diversity of settings including; general hospital wards, neurological units, hospices; care homes and in their own home. Management of physical and psychosocial symptoms and dealing with family bereavement is complex and challenging. Due to the complexity of the physical symptoms input from clinicians with palliative care expertise is an important consideration. Given transmission risk and the latent incidence of infection in the general population, following the emergence of variant CJD; plus the recent hypothesis of a potential relationship between immune responses to COVID-19 and the acceleration of preclinical or evident neurodegenerative disease, there is a need for renewed interest in research in this field. Over the past 20 years, many thousands of articles have been published on CJD. These have been predominately in the medical and science literature and very few publications have addressed the nursing care of persons and families dealing with CJD. There is a need for renewed interest in the management of the disease by supportive and palliative care services.