Overcoming therapeutic nihilism. Breaking bad news of amyotrophic lateral sclerosis—a patient-centred perspective in rare diseases

Amyotrophic lateral sclerosis (ALS) is a rare, incurable, and fatal neurodegenerative disease with median survival time from onset to death ranging from 20 to 48 months. Breaking bad news about ALS diagnosis is a challenging task for physicians and a life-changing experience for patients. Several protocols for delivering difficult information are available, including SPIKES and EMPATHY. Our goal was to assess to what extent these guidelines are followed in Polish ALS patients’ experience as well as to identify any other patients’ preferences not addressed by the guidelines. Participants of our study were recruited via a neurology clinic. Twenty-four patients with confirmed ALS diagnosis were interviewed using in-depth interview and a self-constructed questionnaire: 9 females, 15 males in age ranging from 30–39 to 60–69. The analysis showed a pattern of shortcomings and fundamental violations of available protocols reported by ALS patients. Patients also had to deal with therapeutic nihilism, as they were perceived as “hopeless cases”; unlike in oncological setting, their end-of-life needs were not accommodated by some standard schemes. As a conclusion, we recommend using extended breaking bad news protocols with special emphasis on preparing a treatment plan, giving the patient hope and sense of purpose, offering psychological support and counselling directed to patients and caregivers, and providing the patient with meaningful information about the disease, social support, treatment options, and referral to appropriate health care centres. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10072-022-05931-1.