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

Haukeland University Hospital

Status: Enrolling

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

Treatments: Device: Long term mechanical ventilation support; Device: No long term mechanical ventilation support

Study type: Interventional

Funder types: Other

Identifiers

NCT05744310

492102

Details and patient eligibility

About

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.

Enrollment: 200 estimated patients

Sex: All

Ages: 8+ years old

Volunteers: Accepts Healthy Volunteers

Inclusion and exclusion criteria

Inclusion criteria for patients:

1. A clinical diagnosis of probable ALS according to the revised El Escorial criteria
2. Progression of the illness leading the consulting physician to offer treatment with LTMV
3. Can communicate in Norwegian

Inclusion criteria for partners of ALS patients:

1. Partner of a patient with ALS with progression of the illness leading the consulting physician to offer treatment with LTMV
2. Can communicate in Norwegian

Inclusion criteria for children:

1. 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
2. Can communicate in Norwegian

Exclusion criteria for patients, partners and children of ALS patients:

1. Potential participants with cognitive impairment or dementia.

Trial design

Primary purpose: Treatment

Allocation: Non-Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

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200 participants in 2 patient groups

ALS patients that choose life prolonging treatment with LTMV and their families

Active Comparator group

Treatment:

Device: Long term mechanical ventilation support

ALS patients that decline life prolonging treatment with LTMV and their families

Active Comparator group

Treatment:

Device: No long term mechanical ventilation support