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Effects of Oral Levosimendan on Respiratory Function in Patients With Amyotrophic Lateral Sclerosis (ALS): Open-Label Extension

Phase 3
Terminated
Conditions
Amyotrophic Lateral Sclerosis
Interventions
Registration Number
NCT03948178
Lead Sponsor
Orion Corporation, Orion Pharma
Brief Summary

This study provides an opportunity for subjects in the REFALS (3119002; NCT03505021) study to continue treatment with oral levosimendan. The study will also provide more information about long-term safety and effectiveness of oral levosimendan in patients with ALS.

This is an open-label study, so that all eligible subjects that complete the double-blind REFALS study (48-weeks of treatment) will have the opportunity to receive oral levosimendan treatment. The primary objective, in addition to continuing treatment for subjects enrolled in the REFALS study, is to evaluate long-term safety of oral levosimendan in ALS patients. Another important objective is to explore long-term effectiveness of oral levosimendan in the treatment of patients with ALS.

This study is open only to patients taking part in the REFALS study.

Detailed Description

Not available

Recruitment & Eligibility

Status
TERMINATED
Sex
All
Target Recruitment
227
Inclusion Criteria
  • Written or verbal informed consent (IC) for participation in the study
  • Subjects who completed 48 weeks of treatment according to the REFALS study protocol
  • Able to swallow study treatment capsules at the time of completing 48 weeks dosing in the REFALS study
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Exclusion Criteria
  • Development (or significant worsening from baseline of the REFALS study) of serious cardiovascular disease (e.g.: myocardial infarction, heart failure, arrhythmia, stroke, or second or third degree atrioventricular (AV) block)
  • Pulse/heart rate repeatedly >100 bpm after 5-minute rest at baseline. If the pulse/heart rate is >100 bpm in the first recording, then a second recording must be done after another 5 min rest to confirm pulse/heart rate >100 bpm
  • Systolic blood pressure (SBP) <90 mmHg
  • Severe renal impairment (creatinine clearance < 30ml/min or creatine >170 µmol/l at 48 week visit of the REFALS study, or on dialysis
  • Severe hepatic impairment at the discretion of the investigator
  • Women of reproductive age without a negative pregnancy test and without a commitment to using a highly effective method of contraception (e.g.: oral hormonal contraceptive associated with inhibition of ovulation, intrauterine devices and long acting progestin agent), if sexually active during the study, and for 1 month after the last dose of the study treatment. Women who are postmenopausal (1 year since last menstrual cycle), surgically sterilised or who have undergone a hysterectomy are considered not to be reproductive and can be included
  • Subject judged to be actively suicidal by the investigator
  • Any other clinical significant cardiovascular, gastrointestinal, hepatic, renal, neurological or psychiatric disorder or any other major concurrent illness that in the opinion of the investigator could interfere with the interpretation of the study results or constitute a health risk for the subject if he/she took part in the study
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Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
LevosimendanLevosimendanOral Levosimendan; Levosimendan 1mg capsules for oral administration, once to twice a day, continued as long as clinically beneficial. The total study duration is up to 3 years.
Primary Outcome Measures
NameTimeMethod
Adverse Events RecordingFrom signing informed consent until 14-25 days after the last study treatment for all patients, an average of 23.5 weeks.

Adverse Events as subject counts and proportions (%) of subject per Adverse Event

12-lead Electrocardiogram AssessmentsBaseline, week 2, week 4, month 3, month 6, end-of-study(subject's last visit, 2-48 weeks after study entry)

Summarisation of any abnormal 12-lead ECG findings using descriptive statistics.

Pulse/Heart Rate AssessmentChange in pulse and heart rate(from ECG recording) from Baseline, week 2, week 4, week 6 (pulse rate only), Month 3, Month 6, end of study (subject's last visit, 2-48 weeks after study entry)

Actual values and changes from baseline in supine pre-dose pulse/heart rate were summarised using descriptive statistics .

Secondary Outcome Measures
NameTimeMethod
Need for Respiratory Support DeviceTime to event at study completion (subject's last visit, 2-48 weeks after study entry)

Time to respiratory device support (non invasive) or death

Subject's Status for Tracheostomy and SurvivalBaseline to end of study (average 2-48 weeks after study entry

Number of patients with the need for tracheostomy or who died whilst on treatment from baseline to the end of the study was summarised using descriptive statistics.

Health Care Service Use During the Study(Visits to the Emergency Room)From baseline to the end of the study(2-48 weeks after study entry)

The number of visits to the emergency room were recorded throughout the study using a diary given to the study subjects

Supine Slow Vital Capacity (SVC)The change from Baseline, week 2, week 4, month 3, month 6, end-of-study (subject's last visit, 2-48 weeks after study entry)

Change from baseline in supine and sitting SVC (all devices) through to the end of the study, expressed as a % of predicted normal

Borg Category Ratio 10 Scale (CR 10)Baseline through study completion (week 2, week 4, month 3, month 6, end of study (subject's last visit, 2-48 weeks after study entry)

Patients rated their perception of the severity of their dyspnea using the Borg Category Ration 10 scale (CR 10). The scale ranges from 0(no dysponea) to 10 (maximal dyspnea). each category is numbered and most but not all have verbal cues. At each assessment the patient scored the category they felt best described their symptoms. The analysis measured change from baseline to the end of the study in both a supine and sitting position where a negative score indicates improvement and a positive score reflects worsening.

Number of Subjects Requiring Health and Home Care Resource UseBaseline through study completion (2- 48 weeks after study entry)

The number of study subjects requiring Health and home care resource use was aggregated over the course of the study for each subject and summarised using descriptive statistics.

Health Care Service Use During the Study(Stays in Hospital)From baseline to the end of the study(2-48 weeks after study entry)

The number of night stays in hospital were recorded throughout the study using a diary given to the study subjects

Health Care Service Use During the Study (Days Spent in an Institutional Facility)From baseline to the end of the study(2-48 weeks after study entry)

The number of days spent in an institutional facility were recorded throughout the study using a diary given to the study subjects

Disease ProgressionFrom Baseline through study completion(subject's last visit, 2-48 weeks after study entry)

Count of study withdrawals due to disease progression

Revised ALS Functional Rating Scale (ALSFRS-R)Change from Baseline in respiratory function of ALSFRS-R at study completion (subject's last visit, 2-48 weeks after study entry)

ALSFRS-R scale contains 3 parameters related to respiratory function: Severity of dyspnea, occurrence of orthopnea (shortness of breath when in supine position i.e. lying flat), and the use of mechanical ventilation for respiratory in sufficiency. These 3 parameters are combined to create the respiratory domain with a score of 0-12(where 12 is normal function). Although individual items and patients vary, ALSFRS-R typically declines at a relatively constant rate over time. Plotted over time the slope of the line obtained indicates the speed of progression and thus an effective treatment might be expected ro reduce the slope of decline.

Trial Locations

Locations (79)

Johns Hopkins Hospital

🇺🇸

Baltimore, Maryland, United States

Augusta University, Medical Centre

🇺🇸

Augusta, Georgia, United States

Neurology Associates

🇺🇸

Lincoln, Nebraska, United States

The Ohio State University Wexner Medical center

🇺🇸

Columbus, Ohio, United States

Alleghenay General hospital

🇺🇸

Pittsburgh, Pennsylvania, United States

University of Pittsburgh Medical Center

🇺🇸

Pittsburgh, Pennsylvania, United States

Massachusetts General Hospital

🇺🇸

Boston, Massachusetts, United States

Nerve and Muscle Centre of Texas

🇺🇸

Houston, Texas, United States

University of Washington Medical Center

🇺🇸

Seattle, Washington, United States

Perron Institute for Neurological and Translational Science

🇦🇺

Nedlands, Western Australia, Australia

Centre Hospitalier Regional de la Vitadelle

🇧🇪

Liège, Belgium

Medizinische Universitat Innsbruck

🇦🇹

Innsbruck, Tyrol, Austria

Hospital for Special Care

🇺🇸

New Britain, Connecticut, United States

University of California San Diego

🇺🇸

La Jolla, California, United States

Algemeen Ziekenhuis St Lucas Gent

🇧🇪

Gent, Belgium

Calvary Health Care Bethlehem

🇦🇺

Parkdale, Victoria, Australia

Universitair Ziekenhuis Leuven

🇧🇪

Leuven, Belgium

Univeritair Medisch Centrum Utrech

🇳🇱

Utrecht, Netherlands

Georgetown University

🇺🇸

Washington, District of Columbia, United States

The George Washington Medical Faculty Associates

🇺🇸

Washington, District of Columbia, United States

University of Alberta, Division of Neurology

🇨🇦

Edmonton, Alberta, Canada

Alberta Health Services-Neuromuscular Clinic

🇨🇦

Calgary, Alberta, Canada

Norrlanda University Hospital Neuro-huvud-hals-centrum Vasterbotten

🇸🇪

Umeå, Sweden

The Walton Centre NHs Foundation Trust, Neurology and Neurosurgery

🇬🇧

Liverpool, United Kingdom

Barts Health NHS Trust Royal London hospital

🇬🇧

London, United Kingdom

Medizinische Universitat wein Universitatsklinik ffur Neurologie

🇦🇹

Wein, Austria

Charite Universitatmedizin Berlin- Campus Virchow-Klinikum

🇩🇪

Berlin, Germany

Montreal Neurological Institute and Hospital

🇨🇦

Montréal, Quebec, Canada

Centre Hospitalier Affilie Universitaire de Quebec

🇨🇦

Québec, Quebec, Canada

Turku University Hospital

🇫🇮

Turku, Finland

Beaumont Hospital, Clinical Research Centre

🇮🇪

Dublin, Ireland

Azienda Ospedaliera Universitaria-maggiore della Carita di Novara

🇮🇹

Novara, Italy

Hospital Universitario Reina Sofia Servicio Neurologia

🇪🇸

Córdoba, Spain

Sunnybrook Health Sciences Centre

🇨🇦

Toronto, Ontario, Canada

Karlstad Central Hospital Neurology and Rehabilitation

🇸🇪

Karlstad, Sweden

Hospital Universitario y Politecnico de La Fe

🇪🇸

Valencia, Spain

Stan Cassidy Centre for Rehabilitation

🇨🇦

Fredericton, New Brunswick, Canada

Universitatsklinikum Jena, Klinik fur Neurologie

🇩🇪

Jena, Germany

Helsinki University Central Hospital, Neurology Outpatients Clinic

🇫🇮

Helsinki, Finland

Azienda Policlinico San Martino

🇮🇹

Genova, Italy

Hospital Universitari de Bellvitge

🇪🇸

Barcelona, Spain

Hospital San Rafael

🇪🇸

Madrid, Spain

Hopital Gui de Chauliac Service de Neurologie

🇫🇷

Montpellier, France

Hopital Pasteur Centre de reference des Malades Neuromusculaires et SLA

🇫🇷

Nice, France

ICS Maugeri Spa S UO Riabilitazione Nurologica

🇮🇹

Milan, Italy

Azienda Ospedaliero Universitaria Pisana Ospedale Santa Chiara

🇮🇹

Pisa, Italy

Policlinico Umberto I di Roma Clinica Neurologica

🇮🇹

Rome, Italy

Medizinische Hochschule Hannover

🇩🇪

Hannover, Germany

Azienda Ospedaliera Universitaria Citta della Salute e della Scienza di Torino

🇮🇹

Torino, Italy

Karolinska University Horpital Huddinge Neurology Clinic

🇸🇪

Stockholm, Sweden

Centre Hospitalier Universitaire de Limoges Service de Neurologie

🇫🇷

Limoges, France

Universitatsklinikum Munster, Institut fur Schalfmedizin und Neuromuskalaire Erkrankungen

🇩🇪

Münster, Germany

Universitatsmedizin Rostock, Klinik und Poliklinik fuer Neurologie

🇩🇪

Rostock, Germany

Universitatsklinikum Ulm, Poliklinik fur Neurologie

🇩🇪

Ulm, Germany

Deutsche Klinik fur Daignostik

🇩🇪

Wiesbaden, Germany

Neuromuscular research Centre and Neuromuscular Clinic of Arizona

🇺🇸

Phoenix, Arizona, United States

University of South Florida/USF Health

🇺🇸

Tampa, Florida, United States

Oregon Health and Science University

🇺🇸

Portland, Oregon, United States

Providence Brain and Spine Institute

🇺🇸

Portland, Oregon, United States

University of Utah Health-Imaging & Neurosciences Center in research Park

🇺🇸

Salt Lake City, Utah, United States

University of California Irvine Medical Center

🇺🇸

Orange, California, United States

Holy Cross Hospital Neuroscience Institute

🇺🇸

Fort Lauderdale, Florida, United States

Mayo Clinic Jacksonville

🇺🇸

Jacksonville, Florida, United States

Northwestern University Feinberg School of Medicine

🇺🇸

Chicago, Illinois, United States

University of Chicago

🇺🇸

Chicago, Illinois, United States

Health Partners Speciality Center

🇺🇸

Saint Paul, Minnesota, United States

Neurosciences Institute - Neurology Charlotte

🇺🇸

Charlotte, North Carolina, United States

Washington University School of Medicine

🇺🇸

Saint Louis, Missouri, United States

Columbia Presbyterian Hospital

🇺🇸

New York, New York, United States

Hospital for Special Surgery

🇺🇸

New York, New York, United States

Royal Brisbane and Women's Hospital

🇦🇺

Herston, Queensland, Australia

Brain and Mind Centre

🇦🇺

Camperdown, New South Wales, Australia

Flinders Medical Centre

🇦🇺

Bedford Park, South Australia, Australia

Salzqammergut-klinikum Vocklabruck, Neurologie

🇦🇹

Vocklabruck, Upper Austria, Austria

Hospital Universitario de Basurto

🇪🇸

Bilbao, Spain

University of Michigan, Michigan Medicine University Hospital

🇺🇸

Ann Arbor, Michigan, United States

University of Florida McKnight Brain Institute

🇺🇸

Gainesville, Florida, United States

Wake Forest University Baptist Medical Center

🇺🇸

Winston-Salem, North Carolina, United States

McMaster University Medical Centre

🇨🇦

Hamilton, Ontario, Canada

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