Acute Intermittent Porphyria during Pregnancy: A Case Outlining the Safety of Administering Hematin Infusions.

Abstract Background : Acute Intermittent Porphyria (AIP) is one of the genetic disorders of heme biosynthesis characterized by acute life-threatening attacks of nonspecific neurologic symptoms (1). Gastrointestinal symptoms include abdominal pain, nausea, and vomiting. Neurologic symptoms include pain in extremities, back, chest, or head. Little has been written on pregnancy and porphyria. Neilson and Neilson (1985) (2) reviewed literature for the outcome of pregnancy in porphyria and was able to gather data on 76 pregnancies occurring among 40 patients. They report AIP attacks in 95% of patients. Case Report : The subject is a 35 year-old female, who underwent 2 previous pregnancies. In each pregnancy, she experienced acute episodes of AIP and received intravenous (IV) hematin. The patient was diagnosed in 1995 and became pregnant in 1998. During pregnancy # 1, at 23 wks gestation, the patient presented to the ER and was admitted with AIP. Symptoms included severe abdominal pain and nausea and vomiting. She received 165mg of hematin IV daily 3 days and then was discharged when symptoms resolved. At 25 wks gestation, the patient was admitted for AIP and received 4 doses of hematin. The patient was discharged after 4 days, when symptoms abated. At 32 wks gestation, the patient was admitted for the third time with AIP and given 3 doses of hematin. Her symptoms were included acute abdominal pain, back pain and uncontrolled nausea and vomiting. The patient received a total of 13 doses of hematin during her 4 admissions. The baby was delivered vaginally at term with normal APGAR scores and normal birth weight. The patient was negative for hepatitis and HIV. During pregnancy # 2, the patient presented to the ER at 26 wks gestation. Symptoms included severe abdominal and back pain, uncontrolled nausea and vomiting and upper extremity weakness. Patient received 220mg of hematin and was discharged later that day when symptoms subsided. The patient returned to the ER at 28 wks gestation with complaints of severe abdominal pain, back pain, nausea and vomiting. Patient again received 220mg of hematin but was admitted to the hospital for IV hematin once a day for three days. At 30 wks gestation, the patient presented to the ER with acute abdominal pain, nausea and vomiting and was given 220mg and was discharged later that day. At 32 wks gestation, the patient entered the ER with upper and lower abdominal pain. She received 220 mg of hematin and later was discharged. The patient delivered a healthy baby at 35 weeks gestation. The baby had a normal APGAR and normal birth weight. During her last trimester, the patient received a total of 6 doses of hematin during her second pregnancy. The patient continues to be negative for hepatitis and HIV. Conclusion : Hematin is safe for pregnant women and the fetus. Hematin effectively treated symptoms of AIP. Hematin Dosing Total # Hematin Doses During Pregnancy Dosing/Hematin Infusion Symptoms Pregnancy # 1 13 165 mg Abdominal and Back Pain, Nausea, Vomiting Pregnancy # 2 6 220 mg Abdominal and Back Pain, Nausea, Vomiting Baby Weight Milestones 2 Weeks Check Up 2 Months Check Up 4 Months Check Up Baby 1 6.3 lbs 9.0 lbs 12.0 lbs Baby 2 6.5 lbs 9.1 lbs 11.2 lbs