Inherited Bleeding Disorders

Question 1

What level of factors 8/9 should we see in :

A) ANC
B) Labour
C) post partum
D) regional anaesthesia
E) LMWH

Answer 1

Above 0.5

If 0.5, treat and maintain above 1

Above 1

Above 0.5

Above 0.5

Below 0.6

Question 2

VWF levels are the same except for :

Answer 2

Labour

> 0.5

Question 3

Neonatal testing for Haemophilia

Answer 3

Female babies don’t need testing
Male babies - diagnostic test
Mild cases retesting at 3-6 months
Coed blood test, coags and F levels, cranial USS for mod/ severe
Cranial MRI (symptomatic ICH, even if normal cranial USS)
If low F levels = oral vitamin K 1mg (AVOID IM VIT K)
Short term primary prophylaxis of factor concentrate (Mod/Sev haemophilia with traumatic delivery / prematurity / at risk of bleeding not in normal pregnancy

Question 4

Neonatal Mx vWF

Answer 4

Do cord bloods for vWF activity
Give oral Vit K
If type III suspected = do cranial imaging
Give prophylaxis with factor concentrate, especially if traumatic injury

Question 5

Difference between vWF and Haemophilia

Answer 5

For vWF always give PO Vit K and if type III do cranial USS

Question 6

Thalassaemia classifications

Answer 6

Major : > 7 Tx / year

Intermedia : less than or equal to 7 transfusions / year

Minor : mild to moderate microcytic anaemia with no significant effect on overall health ( no transfusions)

Question 7

SCD screening for end organ damage

Answer 7

Screen for p HTN with echo
HTN / proteinuria - BP and urinalysis
Annual RFTs / LFTs
Retinal screening - proliferative retinopathy specially on HBSC
Iron overload - cardiac MRI (women with multiple transfusions / high ferritin

Question 8

B Thal screening for end organ damage

Answer 8

Reduced fertility in Rx dependent due to organ overload causing anterior pituitary damage (requiring GnRH as ovulation induction)

Pancreas : known DM, fructose one < 300 nmol/l for min 3 months before conception (equivalent to HbA1c 43 mol/mol)

HbA1c not reliable as diluted by transfusion blood

Thyroid - must be euthyroid

Treat if HypoT

Cardiology - ECHO, ECG, T2 Cardiac MRI

Question 9

Vaccines advised in patients with SCD and those with thalassaemia who have undergone spelenectomy

Answer 9

1) pneumococcal vaccine
2) hepatitis B
3) H Influenzae type B
4) Influenza and swine flu
5) conj men C
6) meningitis B & ACWY

Question 10

Antenatal SCD complications in pregnancy

Answer 10

Anaemia
Blood Tx
Painful crisis
CCU admission
ACA
UTI

Question 11

Sickle related complications in pregnancy - pregnancy related/ exacerbated

Answer 11

Pregnancy induced hypertensive disease

Renal insufficiency

VTE

Ante partum hospital admission

Postpartum infection

Question 12

Feral sickle related complications in pregnancy

Answer 12

Miscarriage

Preterm delivery

Stillbirth

FGR