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What is the basic principles of the treatment of haemophilia?

- Treatment centre/multidisciplinary
- Treat early (if in doubt treat)
- Fast track triage in A&E
- Do not wait for clinical signs to develop
- Take care of veins
- Avoid drugs like aspirin
- Early home therapy
- RICE - rest, immobilise, cool, elevate


What is the treatment for haemophilia A?

- Factor VIII
- Amount needed = (rise x weight)/2
- Half-life 8 hrs so 1-3x daily


What is the treatment for haemophilia B (christmas disease)?

- Factor IX
- Amount needed = rise x weight (smaller molecule so doesn't need to be divided by 2)
- Half-life 18-24 hours (OD)
- Treat with recombinant factor IX


What is the other treatment for haemophilia A?

Desmopressin (DDAVP)
This releases stored factor VIII and so is useful for mild haemophiliacs.


What drug is given for prophylaxis of haemophilia A + B?

Tranexamic acid - anti-fibrinolytic given orally
For prophylaxis start 18 months to 3 years when joint bleeds occur.


What drugs should be avoided in patients with coagulation problems?

- Aspirin
- Anti-coagulants


How do you manage type 1 VW disease?

- Treat with DDAVP and tranexamic acid
- Watch for diminishing returns in major surgery, may need VW factor
- consider iron due to blood loss


How do you manage type 2 VW disease?

- 20-30%
- Underactive > VW factor/DDAVP
- Overactive (type 2b) > VW factor concentrate, not DDAVP


How do you manage type 3 VW disease?

- 1-5%
- VW factor
- Consider prophylaxis
- Genetic advice


What mechanical prevention is there for thromboembolism?

- Anti-embolism stockings (AES)
- Intermittent pneumatic compression sleeves (IPC)
- Do not affect coagulation system
- May exacerbate pre-existing arterial insufficiency
- Less effective than pharmacological management


What is the pharmacological prevention for thromboembolism?

- Low dose LMWH (SC)
- Low dose UFH (IV) - can be immediately reversed with protamine sulfate
- Direct anti-Xa and anti-thrombin drugs (O) - DOACs
- Cons - risk of bleeding, allergies, heparin induced thrombocytopenia and thrombosis (HITT)
- Do not use warfarin (too unpredictable)


How long do you have to investigate an acute VTE before treatment?

1 hour for PE
4 hours for DVT


What is the management of an acute VTE?

Start LMWH (in post-op UFH might be preferred) and oral warfarin (except in some post-op patients). Warfarin takes 48-72 hours to reach its therapeutic window at which time the heparin can be discontinued.


What is the treatment for DVT or PE?

- Rivaroxaban, apixaban, edoxaban - direct inhibitors of activated factor X (factor Xa), given for long-term anti-coagulation
Give for 3 months, if not enough review at 6 months
- Contra-indications for rivaroxaban - pregnancy and cirrhosis with coagulopathy


What is the prophylaxis for VTE?

- Anti-embolus stockings - contraindicated in patients with intermittent claudication
- LMWH VTE prophylaxis - increase the risk of operative haemorrhage
- Potassium levels should be monitored for LMWH (>7days), diabetes, CKD and some medication mean more susceptible to hyperkalaemia
- Take off HRT, oral contraceptives


What is the treatment if anticoagulation therapy is contraindicated?

Vena caval filter in IVC - when there is major trauma, severe bleeding disorder.


What is the management for a large PE?

1. O2 if hypoxic (10-15L/min)
2. Morphine 5-10mg IV with anti-emetic if the patient is in pain or v distressed
3. IV access and start LMWH/fondaparinux
4. If low BP give 500ml IV fluid bolus, get ICU input
5. - If haemodynamically unstable consider thombolysis (e.g. alteplase 10mg IV bolus then IV 90mg/2h)
- Haemodynamically stable - if persistent low BP consider vasopressin e.g. dobutamine 2.5-10mcg/kg/min IV or noradrenaline; aim for systolic BP > 90mmHg
6. Initiate long-term coagulation


What is the management for haemophilia A?

- Minor bleeds: pressure and elevation. Desmopressin raises factor VIII levels and may be sufficient.
- Major bleeds e.g. haemarthrosis: increase factor VIII levels to 50% of normal e.g. with recombinant factor VIII. Life-threatening bleeds, e.g. obstructing airway, need levels of 100%.


What is the management of VW disease?

- Desmopressin in mild bleeding
- VMF - containing factor VIII concentrate for surgery or major bleeds - avoid NSAIDs


What are the warfarin drug interactions?

- Warfarin is metabolised in the liver by cytochrome P450 enzyme system
- Clarithromycin inhibits one of the enzymes in this system (CYP3A4) potentiating the effect of warfarin and should be stopped or changed to alternative.
- Warfarin is a vitamin K antagonist