Effects of Long Term Ventilation Support on the Quality of Life of ALS Patients and Their Families

Summary

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.

Conditions

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

Interventions

DEVICE

Long term mechanical ventilation support

Patients that choose life prolonging treatment with long term mechanical ventilation support

DEVICE

No long term mechanical ventilation support

Patients that decline life prolonging treatment with long term mechanical ventilation support

Sponsors & Collaborators

• Oslo University Hospital

  collaborator OTHER

• University Hospital, Akershus

  collaborator OTHER

• Helse Stavanger HF

  collaborator OTHER_GOV

• University Hospital of North Norway

  collaborator OTHER

• St. Olavs Hospital

  collaborator OTHER

• Nordland Hospital, Bodø

  collaborator UNKNOWN

• Hospital of Southern Norway Kristiansand

  collaborator UNKNOWN

• Østfold Hospital Kalnes

  collaborator UNKNOWN

• Haukeland University Hospital

  lead OTHER

Principal Investigators

• Ole-Bjørn Tysnes · Haukeland University Hospital

Study Design

Allocation

NON_RANDOMIZED

Purpose

TREATMENT

Masking

NONE

Model

PARALLEL

Eligibility

Min Age

8 Years

Sex

ALL

Healthy Volunteers

Yes

Timeline & Regulatory

Start

2023-04-21

Primary Completion

2032-08-21

Completion

2032-08-21

Countries

• Norway

Study Locations