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The use of rIX-FP in patients with haemophilia B: a nurse's perspective

Kara Garner

Kara Garner

St Luke's Hemophilia CenterBoise, US
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Garner, Kara
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Christine Guelcher

Christine Guelcher

Children's National HospitalWashington, US
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Guelcher, Christine
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Debra Pollard

Debra Pollard

Royal Free London NHS Foundation TrustLondon, UK
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Pollard, Debra
  
Aug 18, 2021
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Article Category: Clinical Practice
Published Online: Aug 18, 2021
Page range: 86 - 97
DOI: https://doi.org/10.17225/jhp00180
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© 2021 Kara Garner et al., published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.
With an extended half-life that enables a flexible dosing regimen, rFIX-FP can benefit haemophilia B patients by offering effective treatment with reduced treatment burden. Nurse specialists have a complex but key role in ensuring that patients and caregivers are well informed, supported and empowered to choose a treatment approach that works best for them.© Shutterstock
With an extended half-life that enables a flexible dosing regimen, rFIX-FP can benefit haemophilia B patients by offering effective treatment with reduced treatment burden. Nurse specialists have a complex but key role in ensuring that patients and caregivers are well informed, supported and empowered to choose a treatment approach that works best for them.© Shutterstock

Figure 1

Process of subclinical bleeding [20]
Process of subclinical bleeding [20]

Figure 2

PROLONG-9FP clinical trial programme [37]Figure updated from Santagostino et al., 2016*PUPs study was halted following guidance that safety data in this population should be gained from disease registries
PROLONG-9FP clinical trial programme [37]Figure updated from Santagostino et al., 2016*PUPs study was halted following guidance that safety data in this population should be gained from disease registries

Figure 3

rIX-FP phase III/IIIb extension study treatment programme in adult/adolescent and paediatric patients*Only patients ≥18 years were able to switch to the 21-day regimen if they were well controlled on the 14-day regimen for at least 6 months
rIX-FP phase III/IIIb extension study treatment programme in adult/adolescent and paediatric patients*Only patients ≥18 years were able to switch to the 21-day regimen if they were well controlled on the 14-day regimen for at least 6 months

Pharmacokinetics of rIX-FP compared with rFIX reported in phase III studies[11,31]

rIX-FP rFIX
50 IU/kg (N=46) [40] 75 IU/kg (N=8) [33] 50 IU/kg (N=11) [31] 75 IU/kg (N=17) [58]
Half-life (hours) 101.7 104 18.1 22.5
Clearance (mL/h/kg) 0.769 0.84 0.84 7.5*
AUC∞ (IU*h/dL) 7176 9345 548 1070
IR (IU/dL)(IU/kg) 1.27 1.08 0.84 0.80

A comparison between dosing intervals, ABR and annual infusions in adult/adolescent and paediatric patients

rFIX rIX-FP
ADULT/ADOLESCENTS [59] ADULT/ADOLESCENTS [60] PAEDIATRICS [61] ADULT/ADOLESCENTS [42] PAEDIATRICS [43]
Dosing intervals* (days) 3.5 7 3.5–7 7 10 14 21‡ 7 10 14
Number of patients (n) 44 25 22 22 17 41 11 21 8 8
Dose (IU/kg) 50 100§ 64.6 35–50 50–75 50–75 100 25–50 50–75 50–75
Annual infusions (n)† 104 52 52–104 52 37 26 17 52 37 26
ABR (median) 2.6 2.0 3.7 1.33 0.80 0.92 0.32 2.00 3.50 5.60
AsBR (median) 1.7 1.0 0.58 0.00 0.28 0.37 0.00 0.00 0.00 1.10
Steady-state trough FIX (%) mean (SD) 3.57 (3.11) 2.0 (N/A) N/A 22.0 (8.4) 19.8 (16.0) 13.6 (6.4) 7.6 (2.3) 15.1 (4.1) 8.8 (5.7) 7.2 (2.9)
Language:
English
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1 times per year
Journal Subjects:
Medicine, Basic Medical Science, Basic Medical Science, other, Clinical Medicine, Clinical Medicine, other, Pharmacy, Pharmacology
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