Symptomatic treatment, care, and support of CJD patients.

Prion diseases (e.g., Creutzfeldt-Jakob disease) are rapidly progressive neurodegenerative diseases that are invariably fatal. Diagnosing prion disease can be difficult and can lead to frustration. There is no currently available disease-altering treatment for prion diseases and the care and management of affected patients are directed towards symptomatic relief and quality of life. In this chapter, we highlight the many unique challenges of prion disease and how they affect care and management strategies. Symptomatic treatment follows many of the same principles observed in geriatric and/or hospice care, with some important differences due to disease-specific characteristics. We provide an overview of pharmacologic and nonpharmacologic strategies for managing symptoms of prion disease. Education and psychosocial support are also very important in managing patients and families affected by the illness and are discussed in detail. Readers of this chapter will understand the context of caring for a patient with prion disease and will be supplied with practical tools for managing symptoms and educating other healthcare personnel and caregivers. Additional resources for assistance in the care of prion disease patients are also discussed.