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 Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
200
Patients that choose life prolonging treatment with long term mechanical ventilation support
Patients that decline life prolonging treatment with long term mechanical ventilation support
Nordland Hospital Bodø
Bodø, Nordland, Norway
RECRUITINGSørlandet Hospital Trust
Kristiansand, Vest Agder, Norway
RECRUITINGHaukeland University Hospital
Bergen, Norway
RECRUITINGAkershus University Hospital
Lørenskog, Norway
RECRUITINGOslo University Hospital
Oslo, Norway
RECRUITINGØstfold Hospital Kalnes
Sarpsborg, Norway
NOT_YET_RECRUITINGStavanger University Hospital
Stavanger, Norway
RECRUITINGUniversitetssykehuset Nord-Norge
Tromsø, Norway
RECRUITINGSt. Olavs Hospital
Trondheim, Norway
RECRUITINGOverall quality of life assessed by the "Quality of Life Scale"
"Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.
Time frame: At inclusion
Overall quality of life assessed by the "Quality of Life Scale"
"Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.
Time frame: 3 months after inclusion
Overall quality of life assessed by the "Quality of Life Scale"
"Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.
Time frame: 9 months after inclusion
Overall quality of life assessed by the "Quality of Life Scale"
"Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.
Time frame: 15 months after inclusion
Overall quality of life assessed by the "Quality of Life Scale"
"Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.
Time frame: 21 months after inclusion
Health-related quality of life assessed by the "EQ-5D-5L"
"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.
Time frame: At inclusion
Health-related quality of life assessed by the "EQ-5D-5L"
"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.
Time frame: 3 months after inclusion
Health-related quality of life assessed by the "EQ-5D-5L"
"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.
Time frame: 9 months after inclusion
Health-related quality of life assessed by the "EQ-5D-5L"
"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.
Time frame: 15 months after inclusion
Health-related quality of life assessed by the "EQ-5D-5L"
"EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.
Time frame: 21 months after inclusion
Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"
"ALSAQ-5" score (range 0-100), with 0 reflecting the best health state
Time frame: At inclusion
Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"
"ALSAQ-5" score (range 0-100), with 0 reflecting the best health state
Time frame: 3 months after inclusion
Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"
"ALSAQ-5" score (range 0-100), with 0 reflecting the best health state
Time frame: 9 months after inclusion
Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"
"ALSAQ-5" score (range 0-100), with 0 reflecting the best health state
Time frame: 15 months after inclusion
Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"
"ALSAQ-5" score (range 0-100), with 0 reflecting the best health state
Time frame: 21 months after inclusion
Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.
Time frame: At inclusion
Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.
Time frame: 3 months after inclusion
Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.
Time frame: 9 months after inclusion
Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.
Time frame: 15 months after inclusion
Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"
"Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.
Time frame: 21 months after inclusion
Health-related quality of life assessed by "Kidsscreen-27"
"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.
Time frame: At inclusion
Health-related quality of life assessed by "Kidsscreen-27"
"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.
Time frame: 3 months after inclusion
Health-related quality of life assessed by "Kidsscreen-27"
"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.
Time frame: 9 months after inclusion
Health-related quality of life assessed by "Kidsscreen-27"
"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.
Time frame: 15 months after inclusion
Health-related quality of life assessed by "Kidsscreen-27"
"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.
Time frame: 21 months after inclusion
Caregiver burden assessed by "Zarit Burden Interview"
"Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).
Time frame: At inclusion
Caregiver burden assessed by "Zarit Burden Interview"
"Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).
Time frame: 3 months after inclusion
Caregiver burden assessed by "Zarit Burden Interview"
"Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).
Time frame: 9 months after inclusion
Caregiver burden assessed by "Zarit Burden Interview"
"Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).
Time frame: 15 months after inclusion
Caregiver burden assessed by "Zarit Burden Interview"
"Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).
Time frame: 21 months after inclusion
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