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

Study Purpose:

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.

Study Status:

Recruiting

Disease:

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

Study Type:

Interventional

Type of Intervention:

Device

Intervention Name:

Long term mechanical ventilation support, No long term mechanical ventilation support

Placebo:

Phase:

N/A

Study Chair(s)/Principal Investigator(s):

Ole-Bjørn Tysnes, Haukeland University Hospital

Clinicaltrials.gov ID:

NCT05744310

Neals Affiliated?

No

Coordinating Center Contact Information

Study Sponsor:

Haukeland University Hospital

Estimated Enrollment:

200

Estimated Study Start Date:

04 / 12 / 2023

Estimated Study Completion Date:

08 / 21 / 2032

Posting Last Modified Date:

04 / 12 / 2023

Date Study Added to neals.org:

02 / 27 / 2023