Haemophilia A: Management Basics

Feedback00:52 Intro02:35 Structure of Haemophilia A care05:10 Key aspects of management (On-demand vs prophylaxis)07:00 Prophylaxis: reduce death rates from ICH and reducing joint bleeds- Primary prophylaxis: before the 2nd joint bleedSevere haemophiliaAny child spontaneous ICHModerate haemophilia A (1-3 IU/dL)- Secondary prophylaxis:After the 2nd joint bleedLimit joint damage and maximize long term functionESPRIT trial- Tertiary prophylaxis: If joint disease already establishedSlow progression, reduce pain and improve QOLSPINART study13:10 Phases of treatment in Primary Prophylaxis- Modify dose during according to needs at that stage of life- By adulthood, 30% of severe patients can safely stop primary prophylaxis!15:50 Prophylactic medications- IV Recombinant FVIII, 1 IU/kg increases by 2 IU/dL (2%)- Half life: 8-12 hrs - Primary Prophylaxis: (needs CVC)Aim trough level 1-3 IU/dL25-40 IU/kg approx 3-4x per weekTitrate clinically which is individual to the patient- Extended half life...ratio of regular:half-life should be at least 1:1.3- Cannot START them on this as can cause inhibitor25:40 Efanesoctocog (EFA) only needs once weekly IV dosing- Very extended half life- XTEND 1 and XTEND-KIDS trials27:52 Emicisimab: Bi-specific Ab, SC, half life 30 days- Binds FIXa to FX thereby replacing FVIII- Phenotypically makes patients have mild haemophilia A- Used for ANY haemophilia with inhibitor OR severe haemophilia without an inhibitor- HAVEN 3 study- Breakthrough bleeds ?management challenges at home- FIBA can cause MAHA (don't use together) - Thrombosis risk- Reduces APTT and interferes with measuring FVIII and inhibitor45:50 On-demand therapy (inhibitor dependent)47:50 Joint bleeds- Moderate bleed: Aim peak of 50- 60 IU/dL- Severe bleed: Aim peak of 60-80 IU/dL- Daily dosing- Assess within 15 mins , treat within 30 mins- TXA + analgesia, PT + PRICE 52:20 Other bleeds- Peak 80-100 IU/dL: Iliopsoas, ICH, GI bleeds, Neck/throat- Deep cut: aim peak 50 IU/dL- Keep at peak for 1-3 days then 50% decrease in peak level for the next week54:10 Case-study: 24M, swollen knee, FVIII 0.3 IU/L- Patients usually have an emergency plan- Assume severe if no info.01:00:15 Case study: 38M, Appendicectomy, FVIII 30 IU/dL- Hari tricks David, David is tricked- Give DDAVP because rFVIII can cause an inhibitor01:03:05 DDAVP (Vasopressin)- IV/SC- 3-5 fold increase of FVIII- 0.3micrograms/kg- Releases a pool of FVIII from endothelium *lung*- tachyphylaxis- Check protocol, side effects and contraindications1:08:36 Management planning in elective surgery (MDT)- TXA!- Calculate dose of Recomb. FVIII- Check levels at 15mins(pre-op), 4hrs (post-op), next morning- May need VTEp01:12:05 Comprehensive Care Centre Annual Review checklist- exam pearlNB: Target joint- 3 or more bleeds into one joint in a 6 month 'Basics to Brilliance: Haematology Podcast' has been accredited for CPD credit by the Royal College of Pathologists UK. Medical professionals and clinical scientists holding career-grade positions, who are registered with any of the Royal Colleges for CPD, will be eligible to earn 1 credit for every hour of learning. Email: [email protected] Insta: BasicstoBrilliance X: @basics_2_brill Send us your feedback!

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