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NCT Number: NCT05744310

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

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.

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Key information

Conditions

Amyotrophic Lateral Sclerosis Central Nervous System Diseases Metabolic Diseases Motor Neuron Disease Nervous System Diseases Neurodegenerative Diseases Neuromuscular Diseases Nutritional and Metabolic Diseases Proteostasis Deficiencies Spinal Cord Diseases TDP-43 Proteinopathies

Age range

8 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Primary location

Nordland Hospital Bodø, Bodø, Nordland, Norway

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Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

for patients:

  • A clinical diagnosis of probable ALS according to the revised El Escorial criteria
  • Progression of the illness leading the consulting physician to offer treatment with LTMV
  • Can communicate in Norwegian

Inclusion criteria

for partners of ALS patients:

  • Partner of a patient with ALS with progression of the illness leading the consulting physician to offer treatment with LTMV
  • Can communicate in Norwegian

Inclusion criteria

for children:

  • 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
  • Can communicate in Norwegian

Exclusion criteria

for patients, partners and children of ALS patients:

  • Potential participants with cognitive impairment or dementia.

Treatment and study plan

Long term mechanical ventilation support

Device

Patients that choose life prolonging treatment with long term mechanical ventilation support

No long term mechanical ventilation support

Device

Patients that decline life prolonging treatment with long term mechanical ventilation support

Primary outcomes

  1. Overall quality of life assessed by the "Quality of Life Scale"

    Time frame: At inclusion

    "Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.

  2. Overall quality of life assessed by the "Quality of Life Scale"

    Time frame: 3 months after inclusion

    "Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.

  3. Overall quality of life assessed by the "Quality of Life Scale"

    Time frame: 9 months after inclusion

    "Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.

  4. Overall quality of life assessed by the "Quality of Life Scale"

    Time frame: 15 months after inclusion

    "Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.

  5. Overall quality of life assessed by the "Quality of Life Scale"

    Time frame: 21 months after inclusion

    "Quality of Life Scale" total score (range 16-112). Higher score indicates better quality of life.

Secondary outcomes

  1. Health-related quality of life assessed by the "EQ-5D-5L"

    Time frame: At inclusion

    "EQ-5D-5L" index score and EQ-VAS score (range 0-100) where higher scores indicates better health-related quality of life.

  2. Health-related quality of life assessed by the "EQ-5D-5L"

    Time frame: 3 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.

  3. Health-related quality of life assessed by the "EQ-5D-5L"

    Time frame: 9 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.

  4. Health-related quality of life assessed by the "EQ-5D-5L"

    Time frame: 15 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.

  5. Health-related quality of life assessed by the "EQ-5D-5L"

    Time frame: 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.

  6. Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

    Time frame: At inclusion

    "ALSAQ-5" score (range 0-100), with 0 reflecting the best health state

  7. Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

    Time frame: 3 months after inclusion

    "ALSAQ-5" score (range 0-100), with 0 reflecting the best health state

  8. Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

    Time frame: 9 months after inclusion

    "ALSAQ-5" score (range 0-100), with 0 reflecting the best health state

  9. Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

    Time frame: 15 months after inclusion

    "ALSAQ-5" score (range 0-100), with 0 reflecting the best health state

  10. Disease-specific quality of life assessed by "Amyotrophic lateral sclerosis assessment scale - 5 items (ALSAQ-5)"

    Time frame: 21 months after inclusion

    "ALSAQ-5" score (range 0-100), with 0 reflecting the best health state

  11. Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"

    Time frame: At inclusion

    "Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

  12. Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"

    Time frame: 3 months after inclusion

    "Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

  13. Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"

    Time frame: 9 months after inclusion

    "Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

  14. Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"

    Time frame: 15 months after inclusion

    "Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

  15. Health-related quality of life assessed by "Severe Respiratory Insufficiency Questionnaire"

    Time frame: 21 months after inclusion

    "Severe Respiratory Insufficiency Questionnaire" score (range 0-100), with 100 reflecting the best health state.

  16. Health-related quality of life assessed by "Kidsscreen-27"

    Time frame: 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.

  17. Health-related quality of life assessed by "Kidsscreen-27"

    Time frame: 3 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.

  18. Health-related quality of life assessed by "Kidsscreen-27"

    Time frame: 9 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.

  19. Health-related quality of life assessed by "Kidsscreen-27"

    Time frame: 15 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.

  20. Health-related quality of life assessed by "Kidsscreen-27"

    Time frame: 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.

  21. Caregiver burden assessed by "Zarit Burden Interview"

    Time frame: At inclusion

    "Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).

  22. Caregiver burden assessed by "Zarit Burden Interview"

    Time frame: 3 months after inclusion

    "Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).

  23. Caregiver burden assessed by "Zarit Burden Interview"

    Time frame: 9 months after inclusion

    "Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).

  24. Caregiver burden assessed by "Zarit Burden Interview"

    Time frame: 15 months after inclusion

    "Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).

  25. Caregiver burden assessed by "Zarit Burden Interview"

    Time frame: 21 months after inclusion

    "Zarit Burden Interview" total score (range 0-88 where a higher score indicates greater burden).

Study contacts

Contact information is provided by the study sponsor or research team.

Ole-Bjørn Tysnes

CONTACT

[email protected]

+ 47 55975063

Tale Litlere Bjerknes

CONTACT

[email protected]

+47 55975045

Sponsors and collaborators

Lead sponsor

Haukeland University Hospital

Other

Collaborators

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

Registry information

Acronym: ALS-LTMV

Important dates

Study start
2023
Primary completion
2032
Study completion
2032
First posted
Feb 27, 2023
Registry last updated
Aug 28, 2025

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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