Beta thal GTG Flashcards
Main treatments for beta thalassaemia?
Blood transfusion and iron chelation therapy
Splenectomy is not longer the mainstay of treatment, but many women have already undergone splenectomy
What complications can occur due to multiple transfusions?
Iron overload causing hepatic, cardiac and endocrine dysfunction
e.g. cardiac failure, subfertility, osteoporosis, diabetes mellitus, hypothyroid, osteoporosis
Risks to baby in beta thal pregnancy?
FGR
What needs to be considered prior to pregnancy in beta thalassaemia?
May require ovulation induction with gonadotrophins
Consider aggressive chelation in the preconception stage to reduce iron burden and end-organ damage in pregnancy
TFTs, Diabetes, echo, ECG, T2 cardiac MRI, liver USS, liver ferriscan or T2 MRI to assess [iron]
How to assess for diabetes in beta thalassaemia?
serum fructosamine <300 is normal
can’t use HbA1c because it’s diluted by transfused blood causing underestimation
Cardiac risks in beta thal pregnancy?
Cardiac arrythmias due to previous myocardiac iron overload
Cardiomyopathy
Which iron chelator can be used in pregnancy?
Desferrioxamine BUT not in 1st trimester
Encapsulated bacteria examples?
Neisseria meningitidis
Strep pneumoniae
H. influenzae
Who gets AN aspirin 75mg in beta thal?
splenectomy OR platelets >600
Who gets AN VTE prophylaxis in beta thal?
splenectomy AND platelets >600
They also get aspirin 75mg
Considerations for intrapartum care in beta thal?
Give IV desgerrioxamine 2g over 24h
Not an incidcation for CS
Active management 3rd stage
What Hb are you aiming for in pregnant women with beta thal?
100g/L pre-transfusion
How does beta thalassemia manifest in baby?
No fetal disease as fetal Hb made of 2 HbF and 2 HbA
Maternal blood findings in thalassemia minor?
reduced MCV
reduced mean cell Hb (MCH)
normal or slightly reduced mean cell Hb concentration (MCHC)
Mild-to-moderate microcytic anemia
