Effects of Long Term Ventilation Support on the Quality of Life of ALS Patients and Their Families (ALS-LTMV)

Oslo University Hospital, University Hospital, Akershus, St. Olavs Hospital, Helse Stavanger HF, University Hospital of North Norway

Amyotrophic lateral sclerosis (ALS) is a serious rapidly progressive disease of the nervous system. The average survival from the time of diagnosis is two to three years. The patient physical and psychological sufferings in ALS are immense, and apart from Riluzole, there is no effective treatment. Care of advanced ALS have an estimated cost of 4-8 million NOK per year. Perhaps the most challenging topic of ALS care is the decision to extend ventilation support into the stages of disease that require treatment both during day and night. In these cases, treatment is clearly life-sustaining and although quality of life may be maintained, the burden of caregiving imposed upon family or health care workers is huge, regardless of tracheostomy (TIV) or non-invasive (NIV) modality. The present study is a longitudinal questionnaire study in Norway measuring overall quality of life, health-related quality of life, and disease-specific quality of life in ALS patients, partners and children before and after the introduction of life sustaining ventilation support. The investigators aim to increase the knowledge on how life-sustaining ventilation support with NIV or TIV affects the quality of life in ALS patients, life partners and children. The results from the study may provide crucial information for clinicians and patients on one of the most difficult ethical issues of ALS treatment. The investigators anticipate that this information will facilitate a shared decision making processes, weighing benefits and disadvantages in a wider perspective.

Amyotrophic Lateral Sclerosis, Motor Neuron Disease, Nervous System Diseases, Spinal Cord Diseases, Neurodegenerative Diseases, TDP-43 Proteinopathies

Quality of life, Overall quality of life, Health related quality of life, Disease specific quality of life, Ventilation support, Non-invasive ventilation support, Invasive ventilation support

"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.

"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.

"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.

"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.

"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.

Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

"Kidsscreen-27" score where mean (±SD) scores of 50 ± 10 define normality for children and adolescents aged 8-18 years across Europe. Higher scores indicate a better health-related quality of life.

"Kidsscreen-27" score where mean (±SD) scores of 50 ± 10 define normality for children and adolescents aged 8-18 years across Europe. Higher scores indicate a better health-related quality of life.

"Kidsscreen-27" score where mean (±SD) scores of 50 ± 10 define normality for children and adolescents aged 8-18 years across Europe. Higher scores indicate a better health-related quality of life.

"Kidsscreen-27" score where mean (±SD) scores of 50 ± 10 define normality for children and adolescents aged 8-18 years across Europe. Higher scores indicate a better health-related quality of life.

"Kidsscreen-27" score where mean (±SD) scores of 50 ± 10 define normality for children and adolescents aged 8-18 years across Europe. Higher scores indicate a better health-related quality of life.

Inclusion criteria for patients: A clinical diagnosis of probable ALS according to the revised El Escorial criteria Progression of the illness leading the consulting physician to offer treatment with LTMV Can communicate in Norwegian Inclusion criteria for partners of ALS patients: Partner of a patient with ALS with progression of the illness leading the consulting physician to offer treatment with LTMV Can communicate in Norwegian Inclusion criteria for children: Children from 8 years and older having a parent who suffers from ALS with progression of the illness leading the consulting physician to offer treatment with LTMV Can communicate in Norwegian Exclusion criteria for patients, partners and children of ALS patients: 1. Potential participants with cognitive impairment or dementia.