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

Not Applicable

Recruiting

• Conditions: Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Nervous System Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Neurodegenerative Diseases
• Interventions: Device: Long term mechanical ventilation support; Device: No long term mechanical ventilation support

• Registration Number: NCT05744310
• Lead Sponsor: Haukeland University Hospital

Brief 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.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2023-01-30
• Study First Submitted QC: 2023-02-21
• Study First Posted: 2023-02-27
• Study Start: 2023-04-21
• Status Verified: 2024-10
• Last Update Submitted: 2025-03-26
• Last Update Posted: 2025-04-01
• Primary Completion: 2032-08-21
• Study Completion: 2032-08-21

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 200

Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
ALS patients that choose life prolonging treatment with LTMV and their families	Long term mechanical ventilation support	-
ALS patients that decline life prolonging treatment with LTMV and their families	No long term mechanical ventilation support	-

Primary Outcome Measures

Name	Time	Method
Overall quality of life assessed by the "Quality of Life Scale"	21 months after inclusion	"Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.

Secondary Outcome Measures

Name	Time	Method
Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"	21 months after inclusion	"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.
Health-related quality of life assessed by the "EQ-5D-5L"	21 months after inclusion	"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)"	21 months after inclusion	"ALSAQ-5" score (range 0-100), with 0 reflecting the best health state
Health-related quality of life assessed by "Kidsscreen-27"	21 months after inclusion	"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.
Caregiver burden assessed by "Zarit Burden Interview"	21 months after inclusion	"Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).

Trial Locations

Locations (7)

Sørlandet Hospital Trust; 🇳🇴 Kristiansand, Vest Agder, Norway

Haukeland University Hospital; 🇳🇴 Bergen, Norway

Akershus University Hospital; 🇳🇴 Lørenskog, Norway

Oslo University Hospital; 🇳🇴 Oslo, Norway

Stavanger University Hospital; 🇳🇴 Stavanger, Norway

Universitetssykehuset Nord-Norge; 🇳🇴 Tromsø, Norway

St. Olavs Hospital; 🇳🇴 Trondheim, Norway