Pregnancy complicated by Acute Intermittent Porphyria
(AIP) poses a multitude of ethical conflicts for the patient, family, and
physician alike. In pregnancy, fluctuating hormones precipitate exacerbation of
attacks that predominate as life-threatening seizures and acute abdominal pain [1].
The rarity of this high-risk obstetric complication emphasizes the
under-representation of this concomitant etiology in scientific literature and
therefore limited treatment and management options in obstetric practice. This
reality presents conflicting views for both the equally susceptible patient and
physician. In general, AIP studies provide evidence for avoidance of
precipitating factors (hormones, drugs, starvation or dieting, and infections)
in treatment of the disorder. However, naturally occurring hormone increases
are inevitable in the pregnant patient predisposing her to neurotoxic effects
that manifest as neuropsychiatric and neurovisceral symptoms [1]. The deficiency
of porphobilinogen deaminase coupled with precipitating factors (such as
hormone fluctuation in pregnancy) causes increased Haem biosynthesis and
subsequent accumulation of porphyrin precursors [1].
The primary treatment for the AIP patient is limited to
avoidance of the otherwise precipitating factors and has shown minuscule improvement in pregnancy outcomes [1]. In addition, this diagnosis in pregnancy
complicates routine management of the otherwise healthy obstetrics patient as
general and local anaesthetic and intravenous induction agents are
contraindicated [1]. The limitation of resources for the physician to treat the
patient’s condition has yielded poor fetal and maternal prognosis overall. This
unfortunate reality has led the physician to review medical termination of
pregnancy as an option in the management of the patient’s condition during
initial obstetrics education and counseling with the patient [1]. This
is one implication of how underrepresented patient populations face injustice in medicine. What do you feel should be done to improve the maternal prognosis for
the AIP patient?
[1] Sahu,
M. T., Rajaram, S., Saxena, A. K., Goel, N., & Ghumman, S. (2006). Medical
Termination
of Pregnancy in Acute Intermittent Porphyria. Gynecologic
and Obstetric Investigation, 62(1), 38–40. https://doi.org/10.1159/000091835
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