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

Overview

Official title

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

Brief title

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

Registration type

ClinicalTrials.gov

NCT number

NCT05744310

Status

Recruiting

Study category

Interventional, Device

Sponsor / center

Haukeland University Hospital

Research center

Not stated in the official study record.

Collaborators

Oslo University Hospital, University Hospital, Akershus, Helse Stavanger HF, University Hospital of North Norway, St. Olavs Hospital, Nordland Hospital, Bodø, Hospital of Southern Norway Kristiansand, Østfold Hospital Kalnes

Study type

Interventional study

Phase

Not applicable / observational

Enrollment

200

Start date

04-21-2023

Primary completion date

08-21-2032

Estimated / actual completion date

08-21-2032

Last source update

09-21-2026

What is being studied?

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.

Intervention(s)

Who may be eligible?

This is a simplified summary. The official study team or research center determines eligibility.

Population / disease status

Diagnosed ALS

Diagnosis / conditions

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

Age

8 Years to Not stated in the official study record.

Disease duration

Not stated in the official study record.

Respiratory criteria

Not stated in the official study record.

ALSFRS-R criteria

Not stated in the official study record.

Genetic criteria

Not stated in the official study record.

Medication requirements

Not stated in the official study record.

Major inclusion and exclusion criteria from the official record

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.

Genetics

Gene-specific study?

No

Gene or variant

Any / Not gene-specific

Genetic test required?

Not stated in the official study record.

Known carrier required?

Not stated

Confirmed pathogenic variant required?

Not stated in the official study record.

Presymptomatic carriers eligible?

Not stated in the official study record.

At-risk relative eligible?

Not stated

Genetic test provided?

Not stated in the official study record.

Genetic counseling provided?

Not stated in the official study record.

Results returned / offered?

Not stated

Family history required?

Not stated in the official study record.

Study design

Randomized?

Not stated in the official study record.

Allocation

Not stated in the official study record.

Intervention model

Not stated in the official study record.

Masking

Not stated in the official study record.

Placebo

Not stated in the official study record.

Primary purpose

Not stated in the official study record.

Endpoints

Primary outcome measures

• Overall quality of life assessed by the "Quality of Life Scale"
• Overall quality of life assessed by the "Quality of Life Scale"
• Overall quality of life assessed by the "Quality of Life Scale"
• Overall quality of life assessed by the "Quality of Life Scale"
• Overall quality of life assessed by the "Quality of Life Scale"

Secondary outcome measures

• Health-related quality of life assessed by the "EQ-5D-5L"
• Health-related quality of life assessed by the "EQ-5D-5L"
• Health-related quality of life assessed by the "EQ-5D-5L"
• Health-related quality of life assessed by the "EQ-5D-5L"
• Health-related quality of life assessed by the "EQ-5D-5L"
• 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)"
• Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
• Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
• Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
• Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
• Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
• Health-related quality of life assessed by "Kidsscreen-27"
• Health-related quality of life assessed by "Kidsscreen-27"
• Health-related quality of life assessed by "Kidsscreen-27"
• Health-related quality of life assessed by "Kidsscreen-27"
• Health-related quality of life assessed by "Kidsscreen-27"
• Caregiver burden assessed by "Zarit Burden Interview"
• Caregiver burden assessed by "Zarit Burden Interview"
• Caregiver burden assessed by "Zarit Burden Interview"
• Caregiver burden assessed by "Zarit Burden Interview"
• Caregiver burden assessed by "Zarit Burden Interview"

A biomarker outcome should not automatically be interpreted as a clinical outcome.

Locations

Contact

Central contact: Ole-Bjørn Tysnes · ole-bjorn.tysnes@helse-bergen.no · + 47 55975063; Tale Litlere Bjerknes · tale.litlere.bjerknes@helse-bergen.no · +47 55975045

Study official: Ole-Bjørn Tysnes · Haukeland University Hospital · PRINCIPAL_INVESTIGATOR