Rebalancing agents represent a new class of therapies with specific safety monitoring requirements, particularly around thrombotic risk. As with gene therapy patients, the Patient Timeline (Section 3) allows centres to review the full monitoring history for each patient and identify missing assessments or laboratory results. The protocols below define the data collection schedule for CBDR.

This section covers monitoring for concizumab, fitusiran, and marstacimab as rebalancing agents for Hemophilia A or B with or without inhibitors (CDA DRD priority level 3).

Evidence Gaps Being Addressed

  • Limited real-world effectiveness data compared to traditional therapies
  • Insufficient long-term safety data, particularly thrombotic risk
  • Uncertainty regarding patient selection and treatment sequencing
  • Limited comparative patient-reported outcome data
  • Inadequate data on optimal dosing strategies

Product-Specific Information

Agent

Mechanism

Indication

Approval Status

Concizumab

Anti-TFPI monoclonal antibody

Hem A/B with or without inhibitors

FDA approved Dec 2024

Fitusiran

siRNA targeting antithrombin

Hem A/B with or without inhibitors

FDA approved Mar 2025

Marstacimab

Anti-TFPI monoclonal antibody

Hem A/B without inhibitors

FDA approved Oct 2024

 

Baseline Data Collection

Complete prior to initiating rebalancing agent:

Category

Required Data

Patient Demographics

Age, weight, diagnosis (Hem A or B), severity, inhibitor status (current and historical)

Prior Treatment History

Previous prophylaxis regimens, products used, reasons for switching, history of ITI if applicable

Bleeding History

12-month ABR (total and joint bleeds), target joints, spontaneous vs. traumatic bleeds

Thrombotic Risk Assessment

History of thrombosis, cardiovascular disease, use of hormonal therapy, immobility risk factors

Laboratory Baseline

Factor level, inhibitor titer, D-dimer, fibrinogen, platelet count, liver/renal function

Joint Health

HJHS total and individual joint scores

Quality of Life

PROBE score, EQ-5D, pain VAS

 

Ongoing Monitoring Schedule

Timepoint

Assessments

Focus

Week 1-4

Bleeding episodes, injection site reactions

Early safety monitoring

Monthly (M1-6)

Bleed count, adverse events, adherence

Efficacy signal

Month 3

PROBE, pain VAS, bleed rate calculation

Early PRO and efficacy

Month 6

HJHS, PROBE, EQ-5D, 6-month ABR, laboratory panel

Comprehensive 6-month review

Month 12

Full assessment: HJHS, PROBE, EQ-5D, 12-month ABR, factor consumption, lab panel

Annual efficacy, comparative data

Ongoing

All bleeds, adverse events, treatment for bleeds (bypass agents)

Real-time safety and efficacy

 

Critical Safety Monitoring

Report immediately. Thrombotic events are a class concern:

  • Venous thromboembolism (DVT, PE)
  • Arterial thrombosis (stroke, MI, peripheral arterial)
  • Catheter-related thrombosis
  • Thrombotic microangiopathy

Also report:

  • Injection site reactions (severity, duration)
  • Hypersensitivity reactions
  • Breakthrough bleeds requiring bypass agent use
  • Any hospitalization