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00:52 Intro
02:35 Structure of Haemophilia A care
05:10 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 bleed
- Severe haemophilia
- Any child spontaneous ICH
- Moderate haemophilia A (1-3 IU/dL)
- Secondary prophylaxis:
- After the 2nd joint bleed
- Limit joint damage and maximize long term function
- ESPRIT trial
- Tertiary prophylaxis:
- If joint disease already established
- Slow progression, reduce pain and improve QOL
- SPINART study
13:10 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/dL
- 25-40 IU/kg approx 3-4x per week
- Titrate 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 inhibitor
25:40 Efanesoctocog (EFA) only needs once weekly IV dosing
- Very extended half life
- XTEND 1 and XTEND-KIDS trials
27:52 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 inhibitor
45:50 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 week
54:10 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 inhibitor
01:03:05 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 contraindications
1:08:36 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 VTEp
01:12:05 Comprehensive Care Centre Annual Review checklist- exam pearl
NB: Target joint- 3 or more bleeds into one joint in a 6 month
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