Bleeding disorders Flashcards
1
Q
Haemophilia types
A
- Haemophilia A- factor VII def
- Haemophilia B- Factor IX def
2
Q
Haemophilia inheritance
A
- X linked
- 50% have no FHx, new mutation
- If mum is a carrier-
50% sons will be affected
50% daughters will be carriers
3
Q
Testing pre- conception
A
- Test women who have Fhx- boys have the condition in the family
4
Q
Severity
A
- Factor <0.01 - severe
- Factor 0.01-0.05 - Mod
- Factor 0.06-0.4 - Mild
- Factor >0.4 - Normal
5
Q
Haemophilia carrier risk
A
- Higher risk of bleeding after procedures/miscarriage
- No inc risk of miscarriage itself
6
Q
Haemophilia neonate risk
A
- Male babies- higher risk of ICH, bleeding and extracranial bleed
7
Q
Haemophilia fetal precautions
A
- No FSE, FBS, ventouse, mid cavity forceps
- No ECV
8
Q
Haemophilia AN mx
A
- Baseline factor VIII and IX levels for mum
- Gender screen w ffDNA
- Counseling-
Discussed invasive testing, genetic dx, pre-natal testing, termination if wanted.
9
Q
Haemophilia factor levels AN
A
- Check factor level at booking, pre-procedure and 3rd T
- Factor VIII inc in preg
- Factor IX stable
10
Q
Levels to aim for (haemophilias)
A
- Aim factor level >0.5iu/ml pre-procedure
- If treatment is needed- aim level of 1.0 iu/ml and not to fall <0.5iu during a procedure
11
Q
Treatment for Haemophilia
A
TXA
- If bleeding/procedure-for all women any factor level.
- For miscarriage- cont till bleeding stops
- If <0.5iu/ml, TXA + DDAVP (only for Factor VIII)
- Facotr IX, non-responder or insuf rise- Give Recombinant factor
12
Q
DDAVP doses and SE
A
- 0.3mcg/kg
- Repeat doses 12-24h
- SE- anti- diuretic
- Limit fluid to 1L/24h
- Risk of low Na- seizures
- DO NOT USE IN PET
13
Q
Delivery -Haemophilia
A
- MDT- Obs, NNU, anaesthetics.
- Consider CS for affected male fetus.
- AVOID mid cavity forceps, any ventouse.
14
Q
Haemophilia and anaesthetic in labour
A
- Factor level >0.5 for insertion and removal
- Antenatal plan for best anaesthetic options
- Avoid IM inj if level <0.5
15
Q
Postnatal mx- Haemophilia
A
High risk time for bleeding
- SVD aim levels >0.5 for 3 days
- CS/ instrumental- level >0.5 for 5 days
- TXA- cont till lochia minimal/ 7 days for CS
- LWMH- check RF
Try to avoid if factor level <0.6
16
Q
Neonatal mx- Haemophilia
A
- AN plan
- Bloods inc coag post del
- Cord blood samples
- Female babies do not need testing
- Repeat testing at 3-6 months- esp for Haem B
- Oral Vit K - red bleeding risk
17
Q
Baby w Mod to severe haemophilia
A
- Refer to haemophilia centre
- Cranial US
- MRI if signs of ICH
- Consider short term prophylaxsis if high bleeding risk- Preterm, traumatic birth
18
Q
Types of VWD
A
- Type 1- Partial quantitative
- Type 2- Qualitative
- Type 3- Severe quantitative
19
Q
Inheritance of VWD
A
Autosomal dominant or recessive depends on type
20
Q
Type 1 VWD
A
- VWF <0.3iu/ml
- Mostly commonly due to mutation in VWF gene
21
Q
Type 2 VWD
A
- 2A, 2B - Dominant
- 2N- recessive
- Reduction in functional activity of VWF
- Can cause low plts (esp 2B)
22
Q
Type 3 VWD
A
- Absent VWF
- Low factor VII
- Common in consanguineous families
- Recessive inheri
23
Q
Risks of VWB
A
- 10x Inc risk of bleeding
- 10-15% APH
- 25% PPH
no inc risk of miscarriage
24
Q
Course of VWB in preg
A
- Type 1- can improve- levels inc in preg
- Might normalise by term
- Less likely to normalise if factor <0.15 at start
- Type 2- Get worse
- 2B- also have low plts
- Type 3- Minimal or no VWF or factor VIII
- T2 and 3 or severe T1 should deliver in high risk centre.
25
Neonate risk
- Low risk, factor levels are physiologically high at birth
26
Management of VWD
- Assess bleeding phenotype
- Check VWF and VIII levels at booking, 3rd T, and before any procedure.
27
Treatment for VWD
- As close to delivery as possible
- TXA for all women
- If levels <0.5 pre procedure, give DDAVP- DO NOT Give for T2B and T3
- VWF+Fac VIII concentrate instead
- In T2B- can develop low plts after DDAVP
- Target level 1.0iu/ml, maintain >0.5 till haemostasis
28
DDAVP for VWD
- Do a test dose once T2B is excluded
- Inc VWF by 3-5x
- DO NOT Give for T2B and T3
29
Anaesthesia in VWD
- T1- if levels normal, can have regional
- T2- Avoid unless VWF >0.5 and plts >50
- T3- No regional
30
PN mx VWD
- VWF >0.5 for 3 days post SVD
- 5 days post CS/instrumental
- Give TXA for 7-14days
- Check LMWH risks
- Might need repeat rx for VWF levels
31
Neonate plan for VWD
- Testing- bloods +cord blood
- Oral Vit K if risk of T2/3
- Cr USS and Short term concentrate if T3
32
Factor XI def
- Uncommon autosomal recessive and dominant
- High risk in Jews
- Factor XI is antifibrinolytic
- Levels do not correlate to bleeding tendency
33
Testing of low Factor XI
- Check at booking, 3rd T and pre-proceudre
- Check for other clotting disorders
- No special precautions for baby
34
Mx Factor XI
- Inc risk of PPH
- TXA
- FFP
- If severe bleeding then for facotr XI concentrate
**DO NOT give TXA and factor XI together, high clot risk**
35
Anaesthesia for factor XI
- No regional if XI is low
- High risk of bleeding
