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Table 1 Overview of recommendations for discussing personalized prognosis with people with ALS and their families

From: Discussing personalized prognosis in amyotrophic lateral sclerosis: development of a communication guide

1. Interpreting the ENCALS survival model
 The ENCALS survival model provides three outcomes: 1) survival curve; 2) risk group (i.e. very short, short, intermediate, long , or very long); 3) survival probability and interquartile range.
  1. Do not use the survival curve to discuss personalized prognosis, this may overwhelm the patient.
  2. Discuss the personalized prognosis based on the risk group, the group median, or the interquartile range of the survival probability (see 3.1 below).
2. Tailoring discussion to individual patient needs
2.1 General
  1. Tailor discussion of personalized prognosis to patient readiness and individual information needs.
  2. The patient has a right not to know their prognosis.
2.2 Family and next of kin
  1. Stimulate patients to bring family or next of kin with them for support.
  2. If the patient requests it, discuss their prognosis first with their family or next of kin.
2.3 Diverging information needs
  1. If the patient does not want to know their prognosis, but their family or next of kin does, only discuss prognosis with family or next of kin after obtaining the patient’s permission.
2.4 Non-western patients with an immigrant background in the Netherlands
  1. If there is a language barrier, use a professional translator.
  2. Similar to all patients, explore the needs and preferences of patients with a different cultural background, and their families or next of kin, with regard to discussing their prognosis.
  3. Family or next of kin of non-western patients might try to shield the patient from their prognosis. If the patient requests it, discuss their prognosis with their family or next of kin.
2.5 Patients with serious cognitive impairments/FTD
  1. If due to cognitive impairment/FTD the patient is suspected of lacking decisional capacity to decide whether they want to discuss their prognosis, a cognitive screener like the Edinburgh Cognitive and Behavioral ALS Screen can be used to gain insight into affected cognitive domains.
  2. If the patient is judged to lack decisional capacity to decide whether they want to discuss their prognosis, ask their family or next of kin if they want information about the prognosis.
3. Discussing personalized prognosis
3.1 General
  1. Ask the patient how much they would like to know and tailor discussion to their preferences.
  2. Differentiate between three steps of increasing detail
   i. Risk groups without a time indication: very short, short, intermediate, long, or very long.
   ii. Group average as a time indication: very short (1.5 years), short (2 years), intermediate (3 years), long (3.5 years), or very long (7.5 years).
   iii. Interquartile range of the survival probability if the patient requests a more individual estimation of their prognosis.
  3. Emphasize that the prognosis is not an exact time frame, but an estimation and that individual disease progression varies per patient. Point out the long tail (on the graph) and explain that half of the patients live longer, some of whom much longer.
3.2 Example prognostic discussion
  1. Risk group:
   “Looking at your disease characteristics, you fall into the group with a [much shorter than average / shorter than average / intermediate / longer than average / much longer than average] life expectancy.”
   “Half of the patients in every group live longer than the average, some of whom much longer.”
  2. Group average:
   “In this group, half of the people die within the first [1.5 years (much shorter) / 2 years (shorter) / 3 years (average) / 3.5 years (long) / 7.5 years (much longer)] of their disease.”
   “The other half live longer, some of whom much longer.”
  3. Interquartile range
   “Of the patients with your disease characteristics, two out of four die between … months [75th percentile] and … months [25th percentile].”
   “However, one in four patients dies earlier, but one in four lives longer, some of whom much longer.”
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