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A Study to Evaluate Overall Health, Physical Activity, and Joint Outcomes in Participants With Severe or Moderate Hemophilia A Without Factor VIII Inhibitors on Emicizumab Prophylaxis

Phase 4
Active, not recruiting
Conditions
Severe Hemophilia A
Moderate Hemophilia A
Interventions
Registration Number
NCT05181618
Lead Sponsor
Hoffmann-La Roche
Brief Summary

Study MO42623 is a Phase IV, multicenter, open-label, three cohort study designed to evaluate the impact of emicizumab prophylaxis on overall health, physical activity, and joint outcomes in participants aged ≥13 and \<70 years with severe hemophilia A without factor VIII (FVIII) inhibitors or moderate hemophilia A without FVIII inhibitors who are receiving FVIII prophylaxis and who will start emicizumab treatment as part of this study.

Detailed Description

Not available

Recruitment & Eligibility

Status
ACTIVE_NOT_RECRUITING
Sex
All
Target Recruitment
136
Inclusion Criteria
  • Diagnosis of severe congenital hemophilia A (intrinsic factor VIII [FVIII] level <1%) or moderate congenital hemophilia A (intrinsic FVIII level ≤5%) if previously prescribed prophylaxis
  • A negative test for FVIII inhibitor (i.e., <0.6 Bethesda Units) during screening period
  • No history of FVIII inhibitory antibodies (<0.6 BU/mL using the Bethesda assay) in the last 5 years. Participants who completed successful immune tolerance induction (ITI) at least 5 years before screening are eligible, provided they have had no evidence of inhibitor recurrence (permanent or temporary) as may be indicated by detection of an inhibitor, FVIII half-life <6 hours, or FVIII recovery <66% since completing ITI
  • Participants who were on standard FVIII prophylaxis, defined as the regular administration of FVIII to prevent bleeding, for at least the last 24 weeks, can be enrolled regardless of the number of bleeds during this period
  • Adequate hematologic, hepatic and renal function
  • For women of childbearing potential: agreement to remain abstinent (refrain from heterosexual intercourse) or use contraception during the treatment period and for at least 24 weeks after the final dose of emicizumab
Exclusion Criteria
  • Inherited or acquired bleeding disorder other than severe congenital hemophilia A (intrinsic FVIII level <1%) or moderate congenital hemophilia A (intrinsic FVIII level ≤5%) without FVIII inhibitors who were previously prescribed prophylaxis for at least 24 weeks
  • Participants who have previously received emicizumab prophylaxis
  • Participants that plan to have joint replacement, joint procedure, synovectomy or synoviorthesis at screening
  • Participants who had joint replacement, joint procedure, synovectomy or synoviorthesis: Less than 2 years ago; OR, More than 3 years ago and are still experiencing pain in the joint. For participants who had joint replacement, joint procedure, synovectomy or synoviorthesis more than 2 years ago who are not experiencing pain in the joint(s), the participant may be enrolled but the specific joint(s) in which the procedure was conducted will be excluded from the study
  • Participants who have conditions other than hemophilia A that can affect joint health and structure (e.g., osteoarthritis) or with severely impaired mobility due to conditions other than hemophilia A
  • Participants with known reduced bone mineral density defined as clinically relevant vitamin D deficiency
  • Participants with pre-existing uncontrolled or unstable cardiovascular disease not receiving targeted medication or in a stable condition
  • Participants not eligible for MRI
  • History of illicit drug or alcohol abuse within 48 weeks prior to screening in the investigator's judgement
  • Participants who are at high risk for thrombotic microangiopathy (TMA)
  • Previous (within the last 12 months) or current treatment for thromboembolic disease (with the exception of previous catheter-associated thrombosis for which anti-thrombotic treatment is not currently ongoing) or signs of thromboembolic disease
  • Other conditions (e.g., certain autoimmune diseases) that may currently increase the risk of bleeding or thrombosis
  • History of clinically significant hypersensitivity associated with monoclonal antibody therapies or components of the emicizumab injection
  • Planned surgery during the emicizumab loading dose phase
  • Known HIV infection not controlled by medication
  • Concomitant disease, condition, significant abnormality on screening evaluation or laboratory tests, or treatment that could interfere with the conduct of the study, or that would in the opinion of the investigator, pose an additional unacceptable risk in administering study drug to the participant
  • Receipt of any of the following: An investigational drug to treat or reduce the risk of hemophilic bleeds within 5 half-lives of last drug administration at screening; A non-hemophilia-related investigational drug within last 30 days or 5 half-lives at screening, whichever is shorter; or, Any other investigational drug currently being administered or planned to be administered
  • Inability to comply with the study protocol
  • Pregnant or breastfeeding, or intending to become pregnant during the study

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Cohort 1, Hemophilia A and Without Arthropathy: EmicizumabEmicizumabCohort 1 comprises participants with severe or moderate hemophilia A and with no synovitis and no osteochondral damage (Haemophilia Early Arthropathy Detection with Ultrasound \[HEAD-US\] score of 0) in all index joints.
Cohort 2, Hemophilia A and with Synovitis Only: EmicizumabEmicizumabCohort 2 comprises participants with severe or moderate hemophilia A and with synovitis (HEAD-US synovitis score of ≥1) in at least one index joint and no osteochondral damage (HEAD-US bone and cartilage score of 0).
Cohort 3, Hemophilia A and with Osteochondral Damage: EmicizumabEmicizumabCohort 3 comprises participants with severe or moderate hemophilia A and with osteochondral damage (HEAD-US bone and cartilage score of ≥1) in at least one index joint and with any synovitis score.
Primary Outcome Measures
NameTimeMethod
Joint Status at 12 Months, Based on Centrally Reviewed Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) Scores with a Specific Focus on the Synovitis Score in Participants with Synovitis12 Months
Percentage of Joints That are Problem Joints at 6 Months6 Months
Daily Time Spent in Moderate to Vigorous Physical Activity (MVPA) Over Time, as per the Activity Tracker Default CategorizationFrom Baseline until end of treatment period (up to 36 months)
Model-Based Annualized Bleed Rates for All Bleeds, Treated Bleeds, Spontaneous Bleeds, Joint Bleeds, Treated Joint Bleeds, and Target Joint BleedsFrom Baseline until end of treatment period (up to 36 months)
Mean Calculated Annualized Bleed Rates for All Bleeds, Treated Bleeds, Spontaneous Bleeds, Joint Bleeds, Treated Joint Bleeds, and Target Joint BleedsFrom Baseline until end of treatment period (up to 36 months)
Median Calculated Annualized Bleed Rates for All Bleeds, Treated Bleeds, Spontaneous Bleeds, Joint Bleeds, Treated Joint Bleeds, and Target Joint BleedsFrom Baseline until end of treatment period (up to 36 months)
Joint Status at 6 Months, Based on Centrally Reviewed Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) Scores with a Specific Focus on the Synovitis Score in Participants with Synovitis6 Months
Joint Status at 36 Months, Based on Centrally Reviewed Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) Scores with a Specific Focus on the Synovitis Score in Participants with Synovitis36 Months
Clinical Joint Status at 6 Months, Based on the Hemophilia Joint Health Score (HJHS v2.1) Excluding Gait Assessment6 Months
Number of Problem Joints at 24 Months24 Months

Problem joints are defined as joints having chronic joint pain and/or limited range of movement due to compromised joint integrity (i.e., chronic synovitis and/or hemophilic arthropathy) with or without persistent bleeding.

Number of Problem Joints at 36 Months36 Months

Problem joints are defined as joints having chronic joint pain and/or limited range of movement due to compromised joint integrity (i.e., chronic synovitis and/or hemophilic arthropathy) with or without persistent bleeding.

Percentage of Joints That are Problem Joints at 12 Months12 Months
Percentage of Joints That are Problem Joints at 24 Months24 Months
Change from Baseline in the CATCH Domain Scores Over Time, as Assessed with the Comprehensive Assessment Tool of Challenges in Hemophilia (CATCH) Questionnaire for Adult ParticipantsAt Baseline (Day 1), Months 3, 6, 9, 12, 18, 24, 30, and 36
Clinical Joint Status at 24 Months, Based on the Hemophilia Joint Health Score (HJHS v2.1) Excluding Gait Assessment24 Months
Joint Status at 24 Months, Based on Centrally Reviewed Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) Scores with a Specific Focus on the Synovitis Score in Participants with Synovitis24 Months
Change from Baseline in the CATCH Domain Scores Over Time, as Assessed with the CATCH Questionnaire for Pediatric ParticipantsAt Baseline (Day 1), Months 3, 6, 9, 12, 18, 24, 30, and 36
Change from Baseline in the Average Daily Time Spent Doing Physical Activities by Intensity Level Over Time, as Assessed by Participant Responses to the International Physical Activity Questionnaire Short Format (IPAQ-SF)At Baseline (Day 1), Months 3, 6, 9, 12, 18, 24, 30, and 36
Clinical Joint Status at 12 Months, Based on the Hemophilia Joint Health Score (HJHS v2.1) Excluding Gait Assessment12 Months
Clinical Joint Status at 36 Months, Based on the Hemophilia Joint Health Score (HJHS v2.1) Excluding Gait Assessment36 Months
Joint Status at 36 Months, Based on Centrally Reviewed International Prophylaxis Study Group (IPSG) Score (with MRI)36 Months
Number of Problem Joints at 6 Months6 Months

Problem joints are defined as joints having chronic joint pain and/or limited range of movement due to compromised joint integrity (i.e., chronic synovitis and/or hemophilic arthropathy) with or without persistent bleeding.

Number of Problem Joints at 12 Months12 Months

Problem joints are defined as joints having chronic joint pain and/or limited range of movement due to compromised joint integrity (i.e., chronic synovitis and/or hemophilic arthropathy) with or without persistent bleeding.

Percentage of Joints That are Problem Joints at 36 Months36 Months
Daily Step Count Over Time, as Measured with a Wearable Activity TrackerFrom Baseline until end of treatment period (up to 36 months)
Daily Metabolic Equivalents of Tasks (METs) Over Time, as Measured with a Wearable Activity TrackerFrom Baseline until end of treatment period (up to 36 months)
Daily Active Minutes of Physical Activity Over Time, as Measured with a Wearable Activity TrackerFrom Baseline until end of treatment period (up to 36 months)
Number of Participants who Prefer Emicizumab SC Treatment, Their Previous Hemophilia IV Treatment, or Have No Preference, as Assessed Through Use of the Emicizumab Preference Survey at Month 6At Month 6
Secondary Outcome Measures
NameTimeMethod
Number of Participants with at Least One Severe Hypersensitivity, Anaphylaxis, and Anaphylactoid EventFrom Baseline until 24 weeks after the final dose of emicizumab (up to 3.5 years)
Number of Participants with at Least One Injection-Site ReactionFrom Baseline until 24 weeks after the final dose of emicizumab (up to 3.5 years)
Number of Participants with at Least One Adverse Event, with Severity Determined According to the World Health Organization (WHO) Toxicity ScaleFrom Baseline until 24 weeks after the final dose of emicizumab (up to 3.5 years)
Number of Participants with at Least One Thromboembolic EventFrom Baseline until 24 weeks after the final dose of emicizumab (up to 3.5 years)
Number of Participants with at Least One Event of Thrombotic Microangiopathy (TMA)From Baseline until 24 weeks after the final dose of emicizumab (up to 3.5 years)
Number of Participants with Anti-Drug Antibodies (ADAs) Against Emicizumab at Baseline and During the StudyAt Baseline, Months 6, 12, 24, and 36
Number of Participants who Develop Anti-FVIII Inhibitors During the StudyAt Months 6, 12, 24, and 36

Trial Locations

Locations (27)

Policlinico Univ. A. Gemelli

🇮🇹

Roma, Lazio, Italy

AOU Careggi

🇮🇹

Firenze, Toscana, Italy

Complejo Hospitalario Universitario A Coruña (CHUAC)

🇪🇸

La Coruna, Spain

Hospital Regional Universitario Carlos Haya

🇪🇸

Malaga, Spain

CHU Farhat Hached

🇹🇳

Sousse, Tunisia

Aziza Othmana Hospital

🇹🇳

Tunis, Tunisia

Gazi Universitesi Tip Fakultesi

🇹🇷

Ankara, Turkey

Akdeniz Uni School of Medicine

🇹🇷

Antalya, Turkey

St Thomas Westminster

🇬🇧

London, United Kingdom

Istanbul University Cerrahpasa Medical Faculty

🇹🇷

Istanbul, Turkey

Ege Uni Medical School

🇹🇷

Izmir, Turkey

Hospital das Clinicas - UNICAMP

🇧🇷

Campinas, São Paulo, Brazil

Mailman Center for Child Development

🇺🇸

Miami, Florida, United States

Orthopaedic Institute for Children

🇺🇸

Los Angeles, California, United States

Oklahoma Children's Hospital ? Jimmy Everest Center

🇺🇸

Oklahoma City, Oklahoma, United States

Hospital das Clínicas Faculdades Médicas de Ribeirão Preto

🇧🇷

Ribeirao Preto, São Paulo, Brazil

Hamilton Health Sciences Corporation

🇨🇦

Hamilton, Ontario, Canada

Charité Universitätsklinikum Berlin

🇩🇪

Berlin, Germany

Universitätsklinikum Bonn

🇩🇪

Bonn, Germany

Észak-Pesti Centrumkórház - Honvédkórház

🇭🇺

Budapest, Hungary

AOU Federico II

🇮🇹

Napoli, Campania, Italy

Hôpital d'enfants de Rabat - Service d'hémato-oncologie pédiatrique

🇲🇦

Rabat, Morocco

University Clinical Centre of Serbia

🇷🇸

Belgrade, Serbia

Hospital de la Santa Creu i Sant Pau

🇪🇸

Barcelona, Spain

Hospital Universitario Vall de Hebron

🇪🇸

Barcelona, Spain

Hospital Universitario la Paz

🇪🇸

Madrid, Spain

Manchester University NHS Foundation Trust (MFT)

🇬🇧

Manchester, United Kingdom

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