Treatment Flashcards

1
Q

What is the basic principles of the treatment of haemophilia?

A
  • 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
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2
Q

What is the treatment for haemophilia A?

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

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

A
  • 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
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4
Q

What is the other treatment for haemophilia A?

A

Desmopressin (DDAVP)

This releases stored factor VIII and so is useful for mild haemophiliacs.

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5
Q

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

A

Tranexamic acid - anti-fibrinolytic given orally

For prophylaxis start 18 months to 3 years when joint bleeds occur.

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6
Q

What drugs should be avoided in patients with coagulation problems?

A
  • NSAIDs
  • Aspirin
  • Anti-coagulants
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7
Q

How do you manage type 1 VW disease?

A
  • 60-80%
  • Treat with DDAVP and tranexamic acid
  • Watch for diminishing returns in major surgery, may need VW factor
  • consider iron due to blood loss
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8
Q

How do you manage type 2 VW disease?

A
  • 20-30%
  • Underactive > VW factor/DDAVP
  • Overactive (type 2b) > VW factor concentrate, not DDAVP
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9
Q

How do you manage type 3 VW disease?

A
  • 1-5%
  • VW factor
  • Consider prophylaxis
  • Genetic advice
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10
Q

What mechanical prevention is there for thromboembolism?

A
  • 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
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11
Q

What is the pharmacological prevention for thromboembolism?

A
  • 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)
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12
Q

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

A

1 hour for PE

4 hours for DVT

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13
Q

What is the management of an acute VTE?

A

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.

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14
Q

What is the treatment for DVT or PE?

A
  • 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
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15
Q

What is the prophylaxis for VTE?

A
  • 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
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16
Q

What is the treatment if anticoagulation therapy is contraindicated?

A

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

17
Q

What is the management for a large PE?

A
  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
    • 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
  5. Initiate long-term coagulation
18
Q

What is the management for haemophilia A?

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%.
19
Q

What is the management of VW disease?

A
  • Desmopressin in mild bleeding

- VMF - containing factor VIII concentrate for surgery or major bleeds - avoid NSAIDs

20
Q

What are the warfarin drug interactions?

A
  • 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