Mortality among family members of patients with amyotrophic lateral sclerosis – a Swedish register-based study

Abstract

Objective: To test two hypotheses: (1) partners of ALS patients have higher mortality due to outcomes related to psychological distress, and (2) parents and siblings of ALS patients have higher mortality due to diseases that co-occur with ALS. Methods: We performed a nationwide, register-based cohort study in Sweden. We included ALS-free partners, biological parents and full siblings (N ¼ 11,704) of ALS patients, as well as ALS-free partners, biological parents and full siblings (N ¼ 14,460,150) of ALS-free individuals, and followed them during 1961-2013. Hazard ratios (HRs) and 95% confidence intervals (CIs) of overall and cause-specific mortality were derived from Cox regression. Results: Partners of ALS patients, compared to partners of ALS-free individuals, displayed higher mortality due to external causes (HR 2.14; 95% CI 1.35-3.41), including suicide (HR 2.44; 95% CI 1.09-5.44) and accidents (HR 2.09; 95% CI 1.12-3.90), after diagnosis of the ALS patients. Parents of ALS patients had a slightly higher overall mortality (HR 1.03; 95% CI 1.00-1.07), compared with parents of ALS-free individuals. This was driven by mortality due to dementias and cardiovascular, respiratory, and skin diseases. Parents of ALS patients had, however, lower mortality than parents of ALS-free individuals due to neoplasms. Siblings of ALS patients had higher mortality due to dementias, and digestive and skin diseases. Conclusions: Increased mortality due to suicide and accidents among partners of ALS patients is likely attributable to severe psychological distress following the ALS diagnosis. Increased mortality due to dementias among parents and full siblings of ALS patients suggests shared mechanisms between neurodegenerative diseases.

Exploring amyotrophic lateral sclerosis patients’ experiences of psychological distress during the disease course in China: a qualitative study

“…This has contributed to the rising mortality rates among partners of ALS patients in recent years. 49 Furthermore, ALS presents specific genetic characteristics, 50 51 prompting patients to worry about passing the disease to their offspring and causing them psychological distress. Compared with children from typical households, children of ALS patients are more prone to health and socioemotional issues, including somatic symptoms, social isolation, anxiety, depression, and low self-esteem.…”

Partly unequal receipt of healthcare in last month of life in amyotrophic lateral sclerosis: a retrospective cohort study of the Stockholm region

“…Previous studies have shown that symptom relief in the last week in life is inferior in patients with ALS than in cancer (6,8). It has also been shown that partners of patients with ALS have a higher mortality due to external causes, including suicide and accidents, after the ALS diagnosis (38), and that both patients and relatives are emotionally and existentially affected by the disease (11)(12)(13). Thus, it might be beneficial with SPC from the patient's perspective, to reduce suffering and increase symptom relief, but possibly also from a family perspective, as family support is an integral part of SPC (14,15,39).…”

Nurses’ challenges when supporting the family of patients with ALS in specialized palliative home care: A qualitative study

“…Their fears may also be related to a preconceived notion of the disease that may be obtained through media portrayals (A. Ozanne & Graneheim, 2018 ). Spouses of persons with ALS have an especially higher mortality in the form of accidents and suicide than family members of patients with other diagnoses (Kläppe et al, 2021 ). The families situation can be eased by support from healthcare or if they have the idea that it is a matter of time because death is imminent (de Wit et al, 2019 ).…”