Tuesday, October 30, 2018

Emergency Room Guidelines for Acute Porphyrias


Emergency Room Guidelines for Acute Porphyrias
These Emergency Room Guidelines (download PDF) cover essential information for the emergency physician treating a patient in an acute porphyria attack, including common precipitating factors, typical presentation and other diagnostic clues, making the initial diagnosis, common sequelae and best practices for treatment. A PowerPoint presentation (as PDF) for instruction is also available.
Please note: These Guidelines are for Physician Use Only. The APF sells a separate, personalized Emergency Room and Primary Care Physician Kit that contains all the information acute porphyria patients need to have with them in the Emergency Room (medical journal articles, information about diagnostic labs and Panhematin®, room for your own diagnostic test results).
Neville R Pimstone MD, PhD, Head Liver Diseases Greater West Los Angeles Veterans Affairs, Professor Emeritus UC Davis
Karl E. Anderson MD, Professor, Departments of Preventive Medicine and Community Health, Internal Medicine, Pharmacology and Toxicology; Associate Director, General Clinical Research Center, Director, Porphyria Center and Laboratory, University of Texas Medical Branch, Galveston, Texas
Bradley Freilich, MD, Kansas City Gastroenterology and Hepatology, LLC

KEY POINTS
1. The human porphyrias are clinical disorders reflecting defects in heme biosynthesis.
2. Acute porphyrias cause acute attacks of neurological symptoms that can be life-threatening.
3. Acute attacks are triggered by certain drugs, sex steroid hormones, reduced intake of calories and carbohydrate, alcohol and unknown factors.
4. Many of these factors stimulate heme synthesis in the liver, which in the face of a metabolic enzyme defect, leads to increased production of heme precursors that may be neurotoxic.
5. Delta-aminolevulinic acid (ALA) and porphobilinogen (PBG), are porphyrin precursors and intermediates in the heme biosynthetic pathway.
6. ALA and porphobilinogen (PBG) are almost always elevated in urine during an acute attack of porphyria.
7. The most common emergency room (ER) clinical presentation is acute abdominal pain. Other features may include seizures, confusion and hallucinations, and a progressive polyaxonal motor neuropathy, which can progress to paralysis and respiratory failure requiring a ventilator.
8. A high index of suspicion in the presence of nonspecific symptoms is important for diagnosis. A family history of porphyria, female sex, onset during the luteal phase of the menstrual cycle, or recent use of a porphyrinogenic drug may be diagnostic clues.
9. A new diagnosis of porphyria as the cause of acute symptoms must be substantiated by finding a substantial increase in urine porphobilinogen (PBG).
10. Treatment should start promptly after the diagnosis is made. Mild attacks are sometimes treated with glucose loading (e.g. 3L of 10% glucose daily by vein).
11. Most acute attacks should be treated with hemin (Panhematin®, Recordati Rare Diseases at: www.recordatirarediseases.com or 866-654-0539) 3-4mg/kg into a large peripheral vein or venous access port daily for 4 days. Reconstituting Panhematin® with human serum albumin rather than sterile water is recommended prior to infusion. This helps prevent phlebitis at the site of intravenous infusion.
12. Hospitalization is usually required for symptomatic treatment of pain, nausea and vomiting, correction of electrolyte imbalance and observation for respiratory impairment, either to a general medical service or ICU.

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Judy Snyder Personal Story of AIP

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