Maternal medicine Flashcards
(109 cards)
In beta thalassaemia, what is used for monitoring of sugar control?
HbA1C - under reads due to defective global chains and transfusions
Serum Fructosamine used instead
Should be <300 for 3 months prior to conceiving (equivocal to HbA1c 43)
What are the cardiac requirements in pregnancy for women with beta thalassaemia?
Ideally no cardiac iron but this can take years to achieve in prenatal planning
Otherwise, aim for cardiac T2* > 20 ms (on cardiac MRI) wherever possible as this reflects minimal iron in the heart.
A T2* < 10 ms is associated with an increased risk of cardiac failure.
Note that reduced ejection fraction is a relative contraindication to pregnancy
Cardiac failure accounts for 50% of deaths in patients with B-thal
What is target dry weight of iron for the liver in b-thal?
7mg/g
If exceeds 15 , then need to start iron chelation therapy in second to third trimester to reduce risk of cardiac overload of iron
(desferrioxamine)
What iron chelators are safe in pregnancy?
Limited safety data
Use of desferrioxamine between 20-28 weeks where necessary
deferasirox and deferiprone should be stopped 3 months before pregnancy
OK post-natally
What special precautions should be taken for splenectomy patients?
Daily penicillin or erythromycin to protect against encapsulated organisms Neisseria meningitidis, Streptococcus pneumoniae and Haemophilus influenzae type b.
Haem IB and Menc C vaccines if not already had
What is recommended schedule of scans in pregnancy for b-thal?
Early scan 7-9 weeks
Growth scans from 24 weeks (4 weekly)
What is the management of transfusions for patients with b-thal in pregnancy?
Continue transfusions with target of Hb >100
Transfused 2-3 units, 2 weekly as required - will fall at different rates depending on patient
If Hb >80 at 36 weeks then can avoid further transfusion until after delivery
What are VTE requirements of b-thal in pregnancy?
Splenectomy AND platelets > 600 - LMWH and Aspirin
Splenectomy OR platelets >600 - Aspirin
When IP - LMWH
What are the recommendations for intrapartum care of b-thal?
Continuous CTG
Not an indication for C/S
Iron chelation (stress response of labour) 2g over 24 hours
Active 3rd stage
Riks of transmission of Parvovirus
Risk of vertical transmission
<15 weeks gestation - 15%
15 - 20 weeks - 25%
Term - 70%
Risk of fetal infection is negligible after 20 weeks
7 days incubation on average
Risk of fetal death 5-10% of infected fetuses
What time of virus is varicella?
What is incubation time and infectivity?
Varicella (chickenpox) is a DNA virus
Incubation 1-3 weeks
Infections from 48 hours before rash appears to after the vesicles crust over - typically about 5 days
Should you be vaccinated against varicella in pregnancy?
Not during but safe prepregnancy or postpartum
Safe whilst breastfeeding
Live attenuated vaccine
What are the criteria for receiving VZIG?
Made from donated blood products - limited resource
If significant exposure in pregnant woman offer as soon as possible for up to 10 days after contact
Significant exposure: contact in the same room for 15 minutes or more, face-to-face contact or contact in the setting of a large open ward.
Non-immune pregnant women who have been exposed should be treated as infective between days 8-28 following exposure or days 8-21 if had VZIG
What are maternal risks of varicella in pregnancy?
What is treatment?
Affects 0.3% of pregnancies in UK (most women immune)
Pneumonia - around 5%
Hepatitis
Encephalitis
If presents within 24 hours of rash - oral aciclovir 800mg 5 times/day for 7 days
IV aciclovir if severely unwell
Discuss timing of delivery in varicella
Ideally at least 7 days after onset of rash
Risks: disseminated disease, thrombo/coagulopathy, neonatal transmission
Sometimes indicated if severely unwell
What are the fetal implications of varicella in pregnancy?
No known increased risk of miscarriage
If before 28 week then increased risk of fetal varicella syndrome
FVS: skin scarring in a dermatomal distribution; eye defects (microphthalmia, chorioretinitis or cataracts); hypoplasia of the limbs; and neurological abnormalities (microcephaly, cortical atrophy, mental retardation or dysfunction of bowel and bladder sphincters).
It does not occur at the time of initial fetal infection but results from a subsequent herpes zoster reactivation in utero and only occurs in a minority of infected fetuses.
See FMU at 16-20 weeks or 5 weeks after initial infection
If infection in last 4-5 weeks of pregnancy then risk of neonatal varicella - 50% affected, 25% clinical varicella (chickenpox)
What is the method of inheritance for haemophilia ?
Haemophilia A (Factor VIII) and B (Factor IX) are both X-linked
If mother is a carrier then son has 50% chance of being affected and daughter 50% of being carrier
40-50% of haemophilia is de novo mutation
Therefore, new cases of severe haemophilia usually arise from mutation during spermatogenesis in the maternal grandfather, conferring obligate carrier status on the mother. This theory is correct in 90% of cases and thus, the risk to the next male baby after a spontaneously affected sibling is 45%.
How is severity of haemophilia assessed?
Severity is categorised according to the plasma concentration of factor VIII or IX.
Severe <0.01 iu/ml
Moderate haemophilia 0.01–0.05 iu/ml
Mild haemophilia, 0.06–0.40 iu/ml.
What is the cut off of factor VIII/IX for obstetric procedures in haemophilia?
0.5iu/ml is needed for
Amnio/CVS
Any surgical procedure
Epidural/spinal
Aim for factor VIII/IX levels of at least 0.5 iu/ml to cover surgical or invasive procedures, or spontaneous miscarriage. If treatment is required, factor levels of 1.0 iu/ml should be aimed for and not allowed to fall below 0.5 iu/ml until haemostasis is secure
What are treatment options in the antenatal period for haemophilia?
Tranexamic Acid
DDAVP (Desmopressin - diuretic effect) only for Haemophilia A, not in PET
Recombinant factors
How do levels of factor XIII and IX change during pregnancy?
Synthesis of factor VIII is increased in pregnancy with plasma levels rising from 6 weeks of gestation to two to three times the baseline by term.
Factor IX levels are relatively unaltered
What considerations need to be taken into account during labour for babies predicted to have haemophilia?
Avoid:
ECV
FSE/FBS
Midcavity forceps or ventouse
From GTG: Low-cavity forceps may be used and are likely to be preferable to caesarean section in the second stage of labour.
Active management of 3rd stage
Give TXA in labour
Levels of factor VIII/IX should be maintained above 0.5 iu/ml for at least 3 days following an uncomplicated vaginal delivery or 5 days following instrumental delivery or caesarean section.
TXA should be continued postpartum until lochia is minimal.
LMWH should generally be avoided where the factor level is 0.6 iu/ml or less, but will need to be considered in women with thrombotic risk factors, with careful balance of risks.
What are neonatal considerations with haemophilia?
Offer all male born foetuses cord blood testing if born to known female carriers
- may need retesting at 3/6 months
Administer Vitamin K orally if low factor levels
Consider cranial US/MRI
Factor VIII - same as adult level
Factor IX - 1/2 of adult level
What is mechanism of inheritance for von willebrand disease?
Dependent on type
Type 1 - partial reduction in amount of vWF
Type 2 - Dysfunctional vWF, dominant inheritance
Can have thrombocytopenia with DDAVP treatment, generally avoided
Type 3- large reduction in amount of vWF, recessive inheritance