Management of Beta-Thalassaemia in pregnancy GTG Flashcards
What is Thalassaemia major?
Homozygous for B thalassaemia
Severe transfusion dependant anaemia, normall Dx 2-6 years, growth and development may be impaired
What is thalassaemia minor?
Heterozygous for B thalassamia - mild to moderate microcytic anaemia, no detrimental impacts on health
Clinical manifestations of thalassaemias
Anaemia – varying severity
* Skeletal deformities
* Osteoporosis
* Organomegaly
* Hypercoagulable state
* Iron overload
* Transfusion related iron overload complications; including hepatic, cardiac and endocrine dysfunction
What is the mainstay of treatment for thalassaemia
Blood transfusion and iron chelation
What impact can iron overload have
Hepatic, cardiac and endocrine (DM, hypothyroid, hypoparathyroid)
The anterior pituitary gland is sensitive to iron overload, puberty can be delayed/incomplete, low bone mass
Which cause of subfertility is seen in women with thalassaemia?
hypogonadotropic hypogonadism – may require ovulation induction with gonadotrophins.
What is the most common cause of death in people with thalassaemia?
- Cardiac failure is cause of death in 50%.
Screening for haemoglobinopathies in UK
Initial Family origin screening
Red cell index check in low prevalence areas
Haemoglinopathy screen in high prevalence areas
If patient has haemoglinopathy, screening offered to partner
Screen for neonate - heel prick test
What pre-conception care should be offered to women with thalassaemia?
ECHO (low LVEF, CI pregnancy)
ECG
T2 Cardiac MRI (Aim T2 >20mls. If <10mls - increased risk cardiac failure)
Ferriscan or Liver T2 0 ideally iron <7mg/kg, if > 15 increase risk cardiac loading + liver USS
If DM Fructosamine < 300nmol/L and HbA1c < 43 for 3 months
Test partner
Aggressive chelation desferrioxamine
Bone density scan
Review iron chelators
If early pregnancy and EF <55% or Sx of heart failure?
Recommend TOP
Which iron chelators should be stopped? What other mediation should be stopped?
– deferasiroc and deferiprone should be stopped 3 months before conception
- Stop bisphosphaonates 3 months pre-pregnancy
What medications should be started in AN period?
Hep B vaccination
Folic acid 5mg
If splenectomy - pen V
Consider Vit D
When can desferrioxamine be given?
Use 3/12 pre-pregnancy
Stop in 1st trimester
Restart at 20 weeks at low dose if high iron burden
In partner testing which conditions have high risk of serious haemoglinopathy?
Beta Thal
HbS
HbE
Delta Beta thalassaemia
Hb Lepore
HbO Arab
Hb Constant Spring
In partner testing which conditions have high risk of mild- moderate haemoglinopathy?
Hb C
Other variant haemoglobin
what proportion of transfusion dependant thalassaemia are subfertile
80-90%
Success with ART 80% as gondal function preserves
How often should women with B Thal be reviewed?
Monthly until 28 weeks, then fortnightly
When should women with B Thal be offered growth
7-9 weeks
Routine 11-14 weeks
Detailed anomaly 18-20+6
Growth USS every 4 weeks from 24 weeks
Which women should be offer low dose desferrioxamine 4-5days a week from 20-24 week?
Women at high risk of cardiac decompensation
- Cardiac T2 <10mls
- Liver iron >15mg/g
20mg/kg/day 4-5 days per week
Women with thalasaemia should have what additional testing?
Test DM
Monitor TFT
Specialist cardiac assessment at 28 weeks
Regular Hb testing aiming Hb >100
If Thalassaemia and DM, what testing?
Monthly frustosamine conc
Review in JANC
If splenectomy or platelets > 600
Give aspirin
If splenectomy and platelets > 600
Aspirin + LMWH
What intrapartum care?
No specific advice re timing of delivery
Cont CTG in labour
Input - MW, Obs, anaesthetic and game
If red cell antibody - have blood X matched
If Hb <100 cross match blood
If thal major - IV desferrioxamine 2g over 24 hours should be given during labour.
Cont CTG
Active 3rd stage
VTE consideration PP?
High risk VTE
LMWH 6 days if SVD
6/52 CS
Is breast feeding safe with desferrioxamine?
Yes
If BT major and breastfeeding when to restart desferrixamine?
o If major + BF – restart desferrioxamine as soon as 24 hour infusion stops. Secreted in breast milk but not harmful to baby
If BT major and no breastfeeding when to restart desferrixamine?
IV/Subcut desferrioxamine are continued until discharge
Consider Thalassaemia on each system
Pituitary: Hypogondatrophic hypogonadism
Heart - cardiomytopathy
Thyroid: Hypo/hyper
Bones Osteoporosis
Pancreas DM
Liver cirrhosis
Spleen Splenectomy/hyposplenuc
Transfusion: Hepatitis/HIV/antibdoies
Vaccine
5mg Folic acid
