b-Thal Pregnancy Plan (GTG 2014)
Pre-Conception
Annual clinic review should include discussion of family planning
Screen for end organ damage
Aggressive iron chelation preconception reduces end-organ damage
Diabetes common in b-Thal – HbA1c <43 mmol/mol for 3 months prior to conception
TFT – ensure euthyroid
Cardiac – ECG, Echo and T2* MRI
Liver iron – Ferriscan, ideally liver iron should be <7mg per gram of dry weight
Abdo USS – gallstones, liver cirrhosis, hepatitis
Bone density – Offer bone density scan and ensure vitamin D replaced
Vaccination
Hep B vaccine (+ Hib and pneumococcus if hyposplenic)
Pen V prophylaxis if hyposplenic
Review Medication
Folic acid 5mg daily
Discontinue teratogenic medications
Stop deferiprone and deferasirox 3 months prior to conception
Avoid desferrioxamine in 1st trimester. Can be used safely at low doses after 20 weeks
Stop bisphosphonates 3 months prior to conception
Haemoglobinopathy screening
Screen partner
Offer IVF/ICSI with pre-implantation genetic diagnosis to avoid homozygous / compound heterozygous pregnancies if both partners have significant hbpathy.
Antenatal
Schedule
Monthly review to 28 weeks then fortnightly
Monthly HbA1c if diabetes
Cardiac assessment at 28 weeks if b-Thal Major
Scans
Offer viability scan at 7-9 weeks
Routine 1st trimester scan at 11-14 weeks
Routine anomaly scan at 20 weeks
Additional monthly growth scans from 24 weeks
Transfusion
If b-Thal Major , regular transfusion to maintain pre-transfusion Hb >100g/l
b-Thal Intermedia, start transfusion if worsening maternal anaemia or growth restriction
Thromboprophylaxis
Splenectomy or Plt >600 – Aspirin
Splenectomy & Plt >600 – Aspirin + LMWH
Intrapartum
If known red cell antibodies, crossmatch blood for delivery in advance
Intrapartum fetal heart rate monitoring is recommended (increased risk of fetal distress)
b-Thal Major – desferrioxamine should be infused during labour
Active management of third stage of labour to reduce blood loss
Postpartum
Consider as high risk for thrombosis
Breast feeding is safe