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PubMed This is a summary of 38 peer-reviewed journal articles Updated
Anesthesiology

Your Guide to Hereditary Butyrylcholinesterase Deficiency

At a Glance

Hereditary butyrylcholinesterase deficiency (pseudocholinesterase deficiency) is a genetic trait that causes dangerously prolonged paralysis after receiving certain anesthesia drugs. Patients must strictly avoid succinylcholine and mivacurium, and should wear a medical ID to alert surgical teams.

Welcome to the comprehensive guide for Hereditary Butyrylcholinesterase Deficiency (also known as pseudocholinesterase deficiency). If you or a loved one recently experienced a terrifying moment in the recovery room—being told you “didn’t wake up” or “couldn’t breathe” for several hours after surgery—you are likely feeling shaken. It is normal to feel anxious after such an event. However, it is important to know that what occurred is a well-understood, manageable genetic trait.

This guide will empower you to understand your condition, ensure your medical records are complete, and confidently advocate for safe anesthesia care in the future.

Please explore the following sections to learn more:

01

What is Hereditary Butyrylcholinesterase Deficiency?

Learn about hereditary butyrylcholinesterase deficiency. Understand why this silent trait causes prolonged paralysis after anesthesia and what it means for you.

02

The BCHE Gene and Diagnosis

Learn how hereditary butyrylcholinesterase deficiency (BCHE) is diagnosed. Understand genetic testing, the Dibucaine Number, and what your lab report means.

03

Anesthesia Management and Safe Alternatives

Learn the safe anesthesia options for hereditary butyrylcholinesterase deficiency. Discover which muscle relaxants to avoid and how to prepare for safe surgery.

04

Protecting Yourself and Your Family

Learn how to manage BChE deficiency safely. Discover the triple-alert system, triggers to avoid like succinylcholine, and why family screening is vital.

Common questions in this guide

Why did I experience prolonged paralysis after surgery?
This condition is caused by an inherited variation in the BCHE gene. It prevents your body from quickly breaking down specific muscle relaxants used during anesthesia, which causes you to remain temporarily paralyzed and unable to breathe on your own after surgery.
Which anesthesia drugs should I avoid if I have BCHE deficiency?
If you have this genetic trait, you must avoid the muscle relaxant drugs succinylcholine and mivacurium. Your anesthesiologist has many safe alternative medications they can use to ensure you are comfortably and safely sedated during surgery.
Should my family members be tested for pseudocholinesterase deficiency?
Yes, because this is an inherited genetic trait, you should inform your immediate family members about your diagnosis. They should be tested before undergoing any surgical procedures to prevent a potentially dangerous anesthesia reaction.
How can I make sure future surgical teams know about my condition?
You should ask your doctor to place a specific allergy alert for succinylcholine and mivacurium in your electronic health record. It is also highly recommended that you wear a medical ID bracelet or necklace that clearly states your condition.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is the specific name of my genetic variant, and is it documented clearly in my chart?
  2. 2.Can you place an 'allergy' alert for succinylcholine and mivacurium in my electronic health record?
  3. 3.Are there any specific medical ID brands or formats you recommend for this condition?

Questions For You

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This guide is for informational purposes only and does not replace professional medical advice. Always discuss your hereditary butyrylcholinesterase deficiency diagnosis with your anesthesiologist and surgical team prior to any procedure.

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