Amyotrophic Lateral Sclerosis, Motor Neuron Disease, Nervous System Diseases, Neurodegenerative Diseases, Spinal Cord Diseases, TDP-43 Proteinopathies
Conditions
Keywords
Quality of life, Overall quality of life, Health related quality of life, Disease specific quality of life, Ventilation support, Non-invasive ventilation support, Invasive ventilation support
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.
Interventions
DEVICELong term mechanical ventilation support
Patients that choose life prolonging treatment with long term mechanical ventilation support
DEVICENo long term mechanical ventilation support
Patients that decline life prolonging treatment with long term mechanical ventilation support
Sponsors
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
Haukeland University Hospital
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
8 Years to No maximum
Healthy volunteers
Yes
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Overall quality of life assessed by the Quality of Life Scale | At inclusion | Quality of Life Scale total score (range 16-112). Higher score indicates better quality of life. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Health-related quality of life assessed by the EQ-5D-5L | At 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) | At inclusion | ALSAQ-5 score (range 0-100), with 0 reflecting the best health state |
| Health-related quality of life assessed by Severe Respiratory Insufficiency Questionnaire | At inclusion | Severe Respiratory Insufficiency Questionnaire score (range 0-100), with 100 reflecting the best health state. |
| Health-related quality of life assessed by Kidsscreen-27 | At 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 | At inclusion | Zarit Burden Interview total score (range 0-88 where a higher score indicates greater burden). |
Countries
Norway
Contacts
Primary ContactOle-Bjørn Tysnes
Backup ContactTale Litlere Bjerknes
Outcome results
None listed