Table 1.

Model input parameters

Input parameterValueProbability distributionReferences
Utilities    
Baseline HA utility, prophylaxis 0.9378-0.0026 × age β-PERT (varied by ±0.05 from base case) 14,15,16  
Baseline HA utility, no prophylaxis 0.6705-0.0019 × age β-PERT (varied by ±0.05 from base case) 14,15,16  
Disutility of infusion, assuming 12 FVIII infusions per month 0.005 β-PERT (0.004, 0.006) 17  
Disutility of CVAD infection 0.045 β-PERT (0.036, 0.054) 18  
Utilities by mRS    
mRS 0 0.936  19  
mRS 1 0.817   
mRS 2 0.681   
mRS 3 0.558   
mRS 4 0.265   
mRS 5 –0.054   
Utility for no disability/mild disability after ICH, mRS 0-2 0.78 β-PERT (0.63, 0.94) Calculation (supplemental, see “Disability-specific utilities”) 
Utility for disability after ICH, mRS 3-5 0.35 β-PERT (0.28, 0.42) Calculation (supplemental, see “Disability-specific utilities”) 
Transition probabilities    
Probability of ICH on emicizumab, yearly, age 0-1 year β-PERT (0, 0.00000001) 6,9 
Probability of ICH on standard of care, yearly, age 0-1 year 0.024 β-PERT (varied by ±20%) 6,9  
Probability of death after ICH, age 0-1 year 0.0714 β-PERT (0.05712, 0.08568) 7  
Probability of disability after ICH, age 0-1 year 0.38 β-PERT (0.304, 0.456) 7  
Background mortality for males Age dependent  US life tables 
Disability mortality hazard, relative to background mortality 151% Fixed 10  
Distribution of outcomes across the mRS after ICH, 3 months after ICH    
mRS 0 0.038 (5/132)  20  
mRS 1 0.22 (29/132)   
mRS 2 0.129 (17/132)   
mRS 3 0.234 (31/132)   
mRS 4 0.318 (42/132)   
mRS 5 0.061 (8/132)   
Probability of CVAD infection 0.0196 β-PERT (0.0131, 0.0287) 4  
Costs    
Cost of emicizumab prophylaxis, monthly per kg $610.10 Fixed CMS 
Cost of Advate prophylaxis, monthly per kg $454.2 Fixed CMS 
Male body weight, kg Age dependent  21 (age 0-2 years)
22 (age 2-20 years)
23 (age 20-100 years) 
Cost of treating ICH    
Cost of Advate to treat ICH, per kg $3 179.40 Fixed CMS 
Inpatient costs of ICH $14 230.77 γ (225, 63.2) CMS 
General population male yearly health care expenditures Age dependent γ (varied by ±20% from base case) MEPS 
Health care expenditures by mRS, 2010-2014    
mRS 0 $10 500  24  
mRS 1 $10 500   
mRS 2 $10 500   
mRS 3 $11 484   
mRS 4 $11 484   
mRS 5 $11 484   
Average baseline health care expenditures for males, 2010-2014 $6 870 Fixed MEPS 
Post-ICH no-disability/mild-disability baseline cost multiplier 1.53 Fixed Calculation (supplemental, see “cost multipliers”) 
Post-ICH disability baseline cost multiplier 1.67 Fixed Calculation (supplemental, see “cost multipliers”) 
Hourly wage for a working adult $31.48 Fixed 25  
Cost of death: child, adjusted to 2024 USD $124 530 γ (225.1, 553.2) 26  
Cost of death: adult, adjusted to 2024 USD $64 117 γ (225.5, 284.4) 27  
Cost of CVAD insertion $1 144.1 Fixed CMS 
Cost of CVAD removal $214.46 Fixed CMS 
Input parameterValueProbability distributionReferences
Utilities    
Baseline HA utility, prophylaxis 0.9378-0.0026 × age β-PERT (varied by ±0.05 from base case) 14,15,16  
Baseline HA utility, no prophylaxis 0.6705-0.0019 × age β-PERT (varied by ±0.05 from base case) 14,15,16  
Disutility of infusion, assuming 12 FVIII infusions per month 0.005 β-PERT (0.004, 0.006) 17  
Disutility of CVAD infection 0.045 β-PERT (0.036, 0.054) 18  
Utilities by mRS    
mRS 0 0.936  19  
mRS 1 0.817   
mRS 2 0.681   
mRS 3 0.558   
mRS 4 0.265   
mRS 5 –0.054   
Utility for no disability/mild disability after ICH, mRS 0-2 0.78 β-PERT (0.63, 0.94) Calculation (supplemental, see “Disability-specific utilities”) 
Utility for disability after ICH, mRS 3-5 0.35 β-PERT (0.28, 0.42) Calculation (supplemental, see “Disability-specific utilities”) 
Transition probabilities    
Probability of ICH on emicizumab, yearly, age 0-1 year β-PERT (0, 0.00000001) 6,9 
Probability of ICH on standard of care, yearly, age 0-1 year 0.024 β-PERT (varied by ±20%) 6,9  
Probability of death after ICH, age 0-1 year 0.0714 β-PERT (0.05712, 0.08568) 7  
Probability of disability after ICH, age 0-1 year 0.38 β-PERT (0.304, 0.456) 7  
Background mortality for males Age dependent  US life tables 
Disability mortality hazard, relative to background mortality 151% Fixed 10  
Distribution of outcomes across the mRS after ICH, 3 months after ICH    
mRS 0 0.038 (5/132)  20  
mRS 1 0.22 (29/132)   
mRS 2 0.129 (17/132)   
mRS 3 0.234 (31/132)   
mRS 4 0.318 (42/132)   
mRS 5 0.061 (8/132)   
Probability of CVAD infection 0.0196 β-PERT (0.0131, 0.0287) 4  
Costs    
Cost of emicizumab prophylaxis, monthly per kg $610.10 Fixed CMS 
Cost of Advate prophylaxis, monthly per kg $454.2 Fixed CMS 
Male body weight, kg Age dependent  21 (age 0-2 years)
22 (age 2-20 years)
23 (age 20-100 years) 
Cost of treating ICH    
Cost of Advate to treat ICH, per kg $3 179.40 Fixed CMS 
Inpatient costs of ICH $14 230.77 γ (225, 63.2) CMS 
General population male yearly health care expenditures Age dependent γ (varied by ±20% from base case) MEPS 
Health care expenditures by mRS, 2010-2014    
mRS 0 $10 500  24  
mRS 1 $10 500   
mRS 2 $10 500   
mRS 3 $11 484   
mRS 4 $11 484   
mRS 5 $11 484   
Average baseline health care expenditures for males, 2010-2014 $6 870 Fixed MEPS 
Post-ICH no-disability/mild-disability baseline cost multiplier 1.53 Fixed Calculation (supplemental, see “cost multipliers”) 
Post-ICH disability baseline cost multiplier 1.67 Fixed Calculation (supplemental, see “cost multipliers”) 
Hourly wage for a working adult $31.48 Fixed 25  
Cost of death: child, adjusted to 2024 USD $124 530 γ (225.1, 553.2) 26  
Cost of death: adult, adjusted to 2024 USD $64 117 γ (225.5, 284.4) 27  
Cost of CVAD insertion $1 144.1 Fixed CMS 
Cost of CVAD removal $214.46 Fixed CMS 

CDC, Centers for Disease Control and Prevention; CMS, Centers for Medicare and Medicaid Services; MEPS, Medical Expenditure Panel Survey; USD, US dollar.

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