Bleeding disorders

Question 1

Haemophilia types

Answer 1

• Haemophilia A- factor VII def
• Haemophilia B- Factor IX def

Question 2

Haemophilia inheritance

Answer 2

• X linked
• 50% have no FHx, new mutation
• If mum is a carrier-
  50% sons will be affected
  50% daughters will be carriers

Question 3

Testing pre- conception

Answer 3

• Test women who have Fhx- boys have the condition in the family

Question 4

Severity

Answer 4

• Factor <0.01 - severe
• Factor 0.01-0.05 - Mod
• Factor 0.06-0.4 - Mild
• Factor >0.4 - Normal

Question 5

Haemophilia carrier risk

Answer 5

• Higher risk of bleeding after procedures/miscarriage
• No inc risk of miscarriage itself

Question 6

Haemophilia neonate risk

Answer 6

• Male babies- higher risk of ICH, bleeding and extracranial bleed

Question 7

Haemophilia fetal precautions

Answer 7

• No FSE, FBS, ventouse, mid cavity forceps
• No ECV

Question 8

Haemophilia AN mx

Answer 8

• Baseline factor VIII and IX levels for mum
• Gender screen w ffDNA
• Counseling-
  Discussed invasive testing, genetic dx, pre-natal testing, termination if wanted.

Question 9

Haemophilia factor levels AN

Answer 9

• Check factor level at booking, pre-procedure and 3rd T
• Factor VIII inc in preg
• Factor IX stable

Question 10

Levels to aim for (haemophilias)

Answer 10

• 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

Question 11

Treatment for Haemophilia

Answer 11

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

Question 12

DDAVP doses and SE

Answer 12

• 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

Question 13

Delivery -Haemophilia

Answer 13

• MDT- Obs, NNU, anaesthetics.
• Consider CS for affected male fetus.
• AVOID mid cavity forceps, any ventouse.

Question 14

Haemophilia and anaesthetic in labour

Answer 14

• Factor level >0.5 for insertion and removal
• Antenatal plan for best anaesthetic options
• Avoid IM inj if level <0.5

Question 15

Postnatal mx- Haemophilia

Answer 15

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

Question 16

Neonatal mx- Haemophilia

Answer 16

• 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

Question 17

Baby w Mod to severe haemophilia

Answer 17

• Refer to haemophilia centre
• Cranial US
• MRI if signs of ICH
• Consider short term prophylaxsis if high bleeding risk- Preterm, traumatic birth

Question 18

Types of VWD

Answer 18

• Type 1- Partial quantitative
• Type 2- Qualitative
• Type 3- Severe quantitative

Question 19

Inheritance of VWD

Answer 19

Autosomal dominant or recessive depends on type

Question 20

Type 1 VWD

Answer 20

• VWF <0.3iu/ml
• Mostly commonly due to mutation in VWF gene

Question 21

Type 2 VWD

Answer 21

• 2A, 2B - Dominant
• 2N- recessive
• Reduction in functional activity of VWF
• Can cause low plts (esp 2B)

Question 22

Type 3 VWD

Answer 22

• Absent VWF
• Low factor VII
• Common in consanguineous families
• Recessive inheri

Question 23

Risks of VWB

Answer 23

• 10x Inc risk of bleeding
• 10-15% APH
• 25% PPH

no inc risk of miscarriage

Question 24

Course of VWB in preg

Answer 24

• 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.

Question 25

Neonate risk

Answer 25

- Low risk, factor levels are physiologically high at birth

Question 26

Management of VWD

Answer 26

- Assess bleeding phenotype - Check VWF and VIII levels at booking, 3rd T, and before any procedure.

Question 27

Treatment for VWD

Answer 27

- 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

Question 28

DDAVP for VWD

Answer 28

- Do a test dose once T2B is excluded - Inc VWF by 3-5x - DO NOT Give for T2B and T3

Question 29

Anaesthesia in VWD

Answer 29

- T1- if levels normal, can have regional - T2- Avoid unless VWF >0.5 and plts >50 - T3- No regional

Question 30

PN mx VWD

Answer 30

- 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

Question 31

Neonate plan for VWD

Answer 31

- Testing- bloods +cord blood - Oral Vit K if risk of T2/3 - Cr USS and Short term concentrate if T3

Question 32

Factor XI def

Answer 32

- Uncommon autosomal recessive and dominant - High risk in Jews - Factor XI is antifibrinolytic - Levels do not correlate to bleeding tendency

Question 33

Testing of low Factor XI

Answer 33

- Check at booking, 3rd T and pre-proceudre - Check for other clotting disorders - No special precautions for baby

Question 34

Mx Factor XI

Answer 34

- 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**

Question 35

Anaesthesia for factor XI

Answer 35

- No regional if XI is low - High risk of bleeding