PREGNANCY AND DELIVERY
Almost without exception,
female patients with porphyria (of any
sort) have normal pregnancies and deliver
healthy babies without experiencing acute
attacks. However, pregnancy is associated
with increased levels of hormones such
as progesterone which potentially may
aggravate porphyria. Severe attacks during
pregnancy are now very rare, and even
mild attacks are most uncommon.
Our advice therefore is
that patients wishing to have children
should embark on pregnancy without fear.
It is wise to choose a gynaecologist who
is prepared to manage the pregnancy intelligently
and who will liaise with a physician interested
in porphyria as necessary.
The major risk is in
fact that medication unsafe for porphyria
may be prescribed inadvertently either
during the pregnancy or at the time of
delivery. This must be avoided. Where
any doubt exists, the gynaecologist should
consult us about the safety of any medication
he/she wishes to prescribe. Should an
attack arise during pregnancy, it should
be treated promptly according to our usual
protocol. In particular, haem arginate
would appear to be safe during pregnancy.
We have encountered only
two patients who have experienced acute
attacks during, and apparently precipitated
by, pregnancy. In one, this was restricted
to a single attack in the early phases
of each pregnancy. These attacks responded
well to standard treatment with haem arginate
and pregnancy thereafter was uncomplicated
and resulted in a healthy baby. In the
second, a patient with AIP, both her pregnancies
were complicated by a number of recurrent
acute attacks during the second trimester.
Each responded well to standard treatment
with haem arginate and she was induced
at 30-32 weeks in order to forestall further
attacks. Both mother and infants are healthy.
We must stress that this pattern of attacks
during pregnancy is extremely unusual
and, even so, was fairly easily and safely
managed with a satisfactory outcome.
TESTING CORD BLOOD FOR INHERITANCE OF
PORPHYRIA IN THE INFANT
Cord blood can be
tested to determine whether the
infant has inherited porphyria from
a parent. (Though antenatal diagnosis
is technically possible, it is not
indicated for a relatively benign
disorder such as porphyria.) Read
of porphyria in infants and children.
DRUG SAFETY IN OBSTETRIC PRACTICE
Selecting a safe antibiotic
for a pregnant porphyric is usually
simple. Read the page Treating
infections in porphyric patients.
Prostaglandins given intravaginally
are safe. Oxytocin (Syntocinon®, Pitocin®)
is safe. Ergometrine is dangerous and
must be avoided, therefore syntocinon/ergometrine
combinations must be avoided.
Opiates are safe. Epidural
analgesia is safe, preferably using bupivacaine
rather than mepivacaine. General anaesthesia
requires the selection of agents safe
in porphyria and if so, is not a problem.
such as hexaprenaline, and
atosiban are safe. Dexamethasone,
for fetal maturation, is safe.
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