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Flashcards in Treatment Deck (20)
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1

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

2

What is the treatment for haemophilia A?

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

3

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

4

What is the other treatment for haemophilia A?

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

5

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.

6

What drugs should be avoided in patients with coagulation problems?

- NSAIDs
- Aspirin
- Anti-coagulants

7

How do you manage type 1 VW disease?

-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

8

How do you manage type 2 VW disease?

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

9

How do you manage type 3 VW disease?

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

10

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

11

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)

12

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

1 hour for PE
4 hours for DVT

13

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.

14

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

15

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

16

What is the treatment if anticoagulation therapy is contraindicated?

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

17

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

18

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%.

19

What is the management of VW disease?

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

20

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