Disease progression

Prion diseases progress and lead to death rapidly, but the various forms of the disease may have different life expectancies.  

Creutzfeldt-Jakob disease has a vital prognosis of around 6 months from the onset of symptoms. About 90% of patients die within one year of disease onset, although patients with a longer life expectancy have been reported.

Planning guidelines and care. Given how quickly prion diseases develop, it is very important to plan early on how the patient wants or would want to be cared for. Where possible, the recommendation is that an advance directive document or equivalent is drawn up in preparation for the point at which the person is no longer able to make decisions for themselves. These future guidelines include situations requiring medical procedures that could be considered aggressive, such as nasogastric tube feeding, intravenous treatments, or being admitted to hospital.  

Care precautions. A prion disease patient cannot transmit the disease through contact with skin or fluids, or through respiration. Therefore, the patient's relatives do not have to take any special measures, and isolation is not required. Nevertheless, patients with prion diseases cannot be blood, organ or tissue donors.