Deficiency of Benzoylcholinesterase

Benzoylcholinesterase (also called pseudocholinesterase or butyrylcholinesterase) is an enzyme made in the liver and released into your blood. Its job is to help break down some anesthesia drugs, especially succinylcholine and mivacurium, and a few “ester” local anesthetics. When the enzyme is too low or works poorly, those drugs last much longer than normal. The result can be hours of weakness and not breathing well after anesthesia. Most people feel normal again once the drug wears off, but they may need a breathing machine for a while. This condition can be inherited (genetic) or acquired (caused by illness, pregnancy, or medicines). NCBI+1

Benzoylcholinesterase (also called pseudocholinesterase or butyrylcholinesterase) is a blood enzyme that helps the body break down certain anesthetic medicines (like succinylcholine and mivacurium) and some ester-type local anesthetics (for example, procaine, tetracaine, chloroprocaine, benzocaine, cocaine). When this enzyme is low or works poorly, these drugs last much longer than usual. After surgery or an emergency procedure, a person can stay paralyzed and stop breathing on their own for hours, even after the operation ends. Most people feel normal day to day and only learn about the problem after anesthesia. The condition can be inherited (changes in the BCHE gene) or acquired (liver disease, pregnancy, certain medicines, malnutrition). Management is mainly prevention (avoid the trigger drugs), careful anesthesia planning, and supportive breathing until the drugs wear off. (Evidence: StatPearls clinical review; MedlinePlus Genetics overview.) NCBI+2NCBI+2

Doctors also call this butyrylcholinesterase deficiency or pseudocholinesterase deficiency. It is uncommon, but it is important because it strongly changes how your body clears some anesthesia drugs. If you or a family member has it, you should tell your doctors before any surgery. NCBI+1

Other names

This condition has several names that mean the same thing. You may see: pseudocholinesterase deficiency, butyrylcholinesterase deficiency, BCHE deficiency, atypical cholinesterase, dibucaine-resistant cholinesterase, or older terms like suxamethonium apnea. All point to the same clinical problem: very slow breakdown of certain muscle relaxants used in anesthesia. NCBI+1

Types

1) Hereditary (genetic) type.
This happens when both copies of the BCHE gene are changed (autosomal recessive). The enzyme is absent or works poorly, so succinylcholine and mivacurium last much longer. People with only one changed copy (carriers) may also be slow to clear these drugs, but usually less so. MedlinePlus+1

2) Acquired type.
Here the enzyme level or activity falls because of medical conditions, pregnancy, poor nutrition, or some medicines and toxins. The gene is normal, but the amount or function of enzyme is reduced. The effect is often temporary and improves when the cause is removed or treated. PMC+1

3) Phenotypic variants identified by testing.
Laboratories sometimes classify variants by how the enzyme reacts to the inhibitor dibucaine (the “dibucaine number”) or fluoride. Typical patterns include usual (normal), heterozygous atypical, homozygous atypical, fluoride-resistant, silent, and other named variants. These patterns help predict how long paralysis may last after succinylcholine. ltd.aruplab.com+1

Causes

1) BCHE gene variants (hereditary).
Changes in the BCHE gene lower enzyme activity. This is the classic cause and passes in families. MedlinePlus

2) Carrier state (one altered BCHE copy).
Carriers can still clear the drugs, but more slowly than average. Effects are milder than in people with two altered copies. MedlinePlus

3) Liver disease.
The enzyme is made in the liver. Advanced liver disease reduces production and lowers levels in blood. UW Faculty Web Server

4) Kidney (uremic) disease.
Severe kidney problems are linked to reduced activity. OpenAnesthesia

5) Malnutrition and low protein states (hypoproteinemia).
Poor nutrition or low serum proteins reduce enzyme levels. Semantic Scholar

6) Pregnancy (especially third trimester) and early postpartum.
Physiologic changes lower activity by roughly up to one-third and may prolong drug action. Wiley Online Library

7) Newborn period.
Newborns have naturally lower activity, so effects can be longer. OpenAnesthesia

8) Major burns.
Burn injuries are associated with reduced levels. Semantic Scholar

9) Cancer and chemotherapy.
Some cancers and treatments can reduce activity. Semantic Scholar

10) Organophosphate exposure (insecticides).
These chemicals inhibit cholinesterases and can markedly lower function. MedlinePlus

11) Carbamate insecticides and echothiophate eye drops.
These agents also inhibit the enzyme and prolong effects of succinylcholine. ResearchGate

12) Anticholinesterase medicines (e.g., neostigmine).
These drugs inhibit cholinesterases and can worsen or unmask the problem. OpenAnesthesia

13) Esmolol.
This beta-blocker can lower activity. Medscape

14) Metoclopramide.
This anti-nausea drug is reported to reduce activity. Medscape

15) Cyclophosphamide and other cytotoxic agents.
Some chemotherapy drugs decrease activity. Semantic Scholar

16) Oral contraceptives / estrogen exposure.
Hormonal contraceptives are linked to lower activity. Medscape

17) Hypothyroidism / myxedema.
Low thyroid states can reduce activity. Semantic Scholar

18) Heart failure.
Severe heart failure is reported among acquired causes. Semantic Scholar

19) Radiotherapy.
Radiation treatment has been associated with reduced levels in some reports. Semantic Scholar

20) Severe infection or hyperpyrexia.
Serious illness and very high fevers appear in acquired lists. avesis.gazi.edu.tr

Symptoms

Most people do not know they have this condition until they receive succinylcholine or mivacurium during anesthesia. Then they experience prolonged effects. Symptoms reflect long-lasting muscle paralysis and trouble breathing until the drug wears off. Cleveland Clinic

1. Cannot move arms or legs for much longer than expected after surgery. You feel weak and cannot sit up. Cleveland Clinic

2. Trouble breathing or no breathing on your own after the operation. You may need a ventilator. Cleveland Clinic

3. Very slow return of muscle strength even with time. NCBI

4. Need for continued sedation because you are awake but too weak to breathe or move comfortably. NCBI

5. Low oxygen if support is not provided. Monitors may show desaturation without assisted ventilation. NCBI

6. Prolonged time in recovery or ICU. You stay until the block resolves. ClinMed Journals

7. Family history of similar events (“a relative took a long time to wake up after anesthesia”). MedlinePlus

8. Normal feeling before surgery (there are no day-to-day symptoms when you avoid triggering drugs). Cleveland Clinic

9. Normal nerve and muscle exam once the drug clears. There is no permanent weakness. NCBI

10. Possible anxiety or distress if awake yet paralyzed before sedation is given. ClinMed Journals

11. Need for extended oxygen support even after extubation if weakness lingers. WFSA Resource Library

12. Prolonged need for monitoring of train-of-four (TOF) responses in the operating room/PACU. Orphan Anesthesia

13. No symptoms in daily life with routine activities, unless exposed to certain drugs or toxins. MedlinePlus

14. In newborns of affected mothers, possible longer drug effect if succinylcholine is used. OpenAnesthesia

15. Mild cases (carriers) may only have slightly slow recovery, which is easy to miss. MedlinePlus

Diagnostic tests

A) Physical examination

1) Focused post-anesthesia exam for prolonged weakness and apnea.
Doctor checks whether you can move, breathe, and keep oxygen levels up after surgery. Long paralysis after succinylcholine points to this condition. NCBI

2) Family history and past anesthesia history.
A story of “took many hours to wake or breathe after surgery” in you or relatives raises suspicion of a hereditary form. MedlinePlus

3) Nutritional and pregnancy assessment.
Findings suggesting malnutrition or third-trimester pregnancy support an acquired cause. Semantic Scholar+1

B) Manual/bedside tests

4) Five-second head-lift test.
Trying to hold the head up for 5 seconds is a classic bedside check for residual block, but it is not reliable on its own and should not replace objective monitors. SpringerLink+1

5) Grip-strength or tongue-depressor tests.
Simple checks of muscle power; also unreliable alone for safe extubation. PMC+1

6) Sustained tetanus/TOF fade by peripheral nerve stimulator (qualitative).
A small handheld stimulator tests how muscles respond to nerve pulses. Fade suggests ongoing block. Qualitative assessment helps but should be backed by quantitative monitoring. anesthesiaejournal.com

7) Clinical observation of breathing pattern and tidal volume.
Shallow breaths suggest persistent block; again, not specific without objective monitoring. WFSA Resource Library

8) Single-twitch height recovery trend (if TOF monitoring unavailable).
Some centers trend single-twitch response after succinylcholine when TOF is not feasible. This is a pragmatic bedside approach but less precise. blinkdc.com

C) Laboratory and pathological tests

9) Serum pseudocholinesterase (butyrylcholinesterase) activity level.
A simple blood test. Low activity during or after the event supports the diagnosis. Some labs list typical ranges (e.g., ~4,000–13,500 U/L). PMC

10) Dibucaine number (DN).
Reports the percent inhibition of your enzyme by dibucaine. Normal DN is high (~≥80). Heterozygous atypical often ~40–60. Homozygous atypical often ~≤20. Low DN indicates higher risk of long paralysis with succinylcholine. DN is interpreted together with the activity level. ltd.aruplab.com+1

11) Fluoride number.
Similar concept using fluoride to identify other resistant variants. Helps phenotype unusual patterns. ltd.aruplab.com

12) BCHE genetic testing.
DNA testing confirms hereditary forms and clarifies family risk. Useful after recovery, not in the middle of surgery. MedlinePlus

13) Liver function tests and serum albumin.
Help identify acquired causes like liver disease or hypoproteinemia. UW Faculty Web Server+1

14) Pregnancy test (when appropriate).
Supports pregnancy-related reduction in activity. Wiley Online Library

15) Toxin/drug exposure testing (e.g., organophosphate exposure).
If suspected, testing for exposures that inhibit cholinesterases may explain a sudden drop. MedlinePlus

16) Repeat enzyme activity and DN several weeks later.
Levels may recover after an acquired cause resolves; repeating helps distinguish temporary from genetic deficiency. repositorio.ulssa.pt

D) Electrodiagnostic tests

17) Quantitative train-of-four (TOF) monitoring.
Objective devices (e.g., acceleromyography) measure the TOF ratio. Safe recovery usually means TOF ratio ≥0.9. Persistent low ratios after succinylcholine suggest deficiency. PMC

18) Post-tetanic count (PTC) with peripheral nerve stimulator.
If no twitches are seen, PTC estimates depth of block and tracks recovery over time. Useful when paralysis is very deep and prolonged. anesthesiaejournal.com

19) Intraoperative neuromuscular monitoring protocol.
Continuous monitoring guides ventilation, sedation, and timing of extubation; it is key in suspected cases to avoid premature awakening while still paralyzed. Orphan Anesthesia

E) Imaging tests

20) Imaging is usually not required for diagnosis.
However, if breathing problems persist and the cause is unclear, doctors may order tests like a chest X-ray to look for other reasons (e.g., pneumonia or atelectasis) while the enzyme problem is being confirmed by labs. The enzyme deficiency itself is diagnosed by blood tests and neuromuscular monitoring, not by imaging. NCBI

Non-pharmacological treatments

1. Strict drug avoidance plan.
   Before any surgery or emergency procedure, the anesthesia team should avoid succinylcholine and mivacurium, and usually avoid ester local anesthetics (e.g., procaine, tetracaine, chloroprocaine, benzocaine). This plan is written in the chart and communicated to all staff.
   Purpose: To prevent dangerously prolonged paralysis and apnea.
   Mechanism: Avoids drugs that depend on plasma cholinesterase for breakdown. (Evidence: MedlinePlus; Mayo Clinic.) MedlinePlus+1

2. Use safe alternatives for muscle relaxation.
   Description: Choose non-depolarizing agents that do not rely on plasma cholinesterase, such as rocuronium, cisatracurium, atracurium, or vecuronium, with standard monitoring and planned reversal.
   Purpose: Provide needed muscle relaxation without prolonged apnea.
   Mechanism: These agents are metabolized by hepatic/renal pathways or Hofmann elimination (cisatracurium/atracurium). (Evidence: FDA labels for rocuronium, cisatracurium; StatPearls.) FDA Access Data+2FDA Access Data+2

3. Careful airway and breathing support if exposure happens.
   Description: If the wrong drug is given and the patient remains paralyzed, keep the airway secure and continue mechanical ventilation with sedation until spontaneous breathing returns.
   Purpose: Prevent low oxygen and complications while the drug wears off.
   Mechanism: Supportive ventilation substitutes for breathing until neuromuscular junction function returns. (Evidence: Anectine label; StatPearls.) FDA Access Data+1

4. Full neuromuscular monitoring (train-of-four).
   Description: Use objective monitoring to dose relaxants and confirm recovery to a train-of-four ratio ≥0.9 before extubation.
   Purpose: Reduce residual paralysis and unplanned ICU stays.
   Mechanism: Quantifies blockade rather than guessing clinical recovery. (Evidence: StatPearls.) NCBI

5. Medical alert identification.
   Description: Wear a medical alert bracelet/necklace and carry a wallet card stating “Pseudocholinesterase (butyrylcholinesterase) deficiency—avoid succinylcholine/mivacurium; use non-depolarizing relaxants.”
   Purpose: Protects you during emergencies when you cannot speak.
   Mechanism: Alerts clinicians to avoid triggers and plan safe anesthesia. (Evidence: Mayo Clinic; Medscape; American Journal of the Medical Sciences review.) Mayo Clinic+2Medscape+2

6. Family notification and optional testing.
   Description: First-degree relatives can consider testing because the trait is often inherited.
   Purpose: Prevent anesthesia complications in relatives.
   Mechanism: Identifies carriers or affected people before surgery. (Evidence: MedlinePlus; StatPearls.) MedlinePlus+1

7. Document allergy/critical alert in the medical record.
   Description: Add “Critical: prolonged paralysis with succinylcholine/mivacurium” to the EHR problem list.
   Purpose: Forces pop-up alerts for prescribers.
   Mechanism: Electronic safety check. (Evidence: Standard safety practice summarized in Mayo Clinic guidance.) Mayo Clinic

8. Optimize nutrition and liver health.
   Description: Treat malnutrition and liver disease when possible.
   Purpose: Improve enzyme production and reduce risk of acquired deficiency.
   Mechanism: The enzyme is made in the liver and needs adequate protein. (Evidence: StatPearls.) NCBI

9. Pre-op anesthesia consult.
   Description: Plan medication choices, monitoring, and reversal strategy before elective procedures.
   Purpose: Avoid last-minute errors.
   Mechanism: Risk assessment and individualized plan. (Evidence: OrphanAnesthesia guideline.) Orphan Anesthesia

10. Peri-operative communication checklist.
    Description: Use a safety checklist calling out “BChE deficiency—no sux/no mivacurium.”
    Purpose: Shared mental model across team.
    Mechanism: Standardizes handoff language. (Evidence: OrphanAnesthesia.) Orphan Anesthesia

11. Post-op sedation if paralyzed but awake.
    Description: Give adequate sedation and analgesia during mechanical ventilation until recovery.
    Purpose: Prevent awareness and distress.
    Mechanism: Maintains comfort while block persists. (Evidence: StatPearls.) NCBI

12. ICU/step-down monitoring when needed.
    Description: If recovery is slow or complications occur, monitor closely.
    Purpose: Early detection of breathing or airway problems.
    Mechanism: Continuous observation. (Evidence: StatPearls.) NCBI

13. Temperature control and normothermia.
    Description: Maintain normal body temperature; avoid hypothermia which can prolong drug effects.
    Purpose: Faster recovery of neuromuscular transmission.
    Mechanism: Enzyme and receptor function are temperature sensitive. (Evidence: anesthesia practice texts summarized in StatPearls.) NCBI

14. Avoid ester topical anesthetics when alternatives exist.
    Description: Prefer amide local anesthetics (e.g., lidocaine, bupivacaine) when appropriate.
    Purpose: Lower risk of prolonged or unpredictable effect.
    Mechanism: Amides are metabolized by liver, not by plasma cholinesterase. (Evidence: Mayo Clinic; bupivacaine label background.) Mayo Clinic+1

15. Educate patient about over-the-counter products (e.g., benzocaine gels/sprays).
    Purpose: Avoid unexpected long action or toxicity.
    Mechanism: Many OTC numbing products are esters. (Evidence: Mayo Clinic.) Mayo Clinic

16. Genetic counseling (optional).
    Description: Discuss inheritance, testing choices, and family risk.
    Purpose: Support informed decisions for relatives.
    Mechanism: Clarifies autosomal recessive transmission. (Evidence: MedlinePlus.) MedlinePlus

17. If inadvertent exposure occurs, consider blood product strategies (case-by-case).
    Description: Some reports describe using fresh frozen plasma or human serum cholinesterase to supply enzyme when paralysis is very prolonged; this is not routine and carries transfusion risks.
    Purpose: Potentially speed recovery.
    Mechanism: Provides the missing enzyme to break down drug. (Evidence: OrphanAnesthesia guideline; Medscape treatment note; case literature.) Orphan Anesthesia+2Medscape+2

18. Pre-op stop or review interacting medicines that lower cholinesterase.
    Purpose: Reduce acquired deficiency risk.
    Mechanism: Remove reversible contributors. (Evidence: Mivacurium label—reduced plasma cholinesterase activity section.) FDA Access Data

19. Clear discharge instructions after any anesthesia.
    Purpose: Warn about signs of residual weakness and when to seek help.
    Mechanism: Safety net after hospital stay. (Evidence: StatPearls.) NCBI

20. Maintain a personal anesthesia record.
    Purpose: Give future teams the exact agents that were safe or unsafe.
    Mechanism: Learning from prior exposures. (Evidence: Mayo Clinic patient guidance.) Mayo Clinic

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Drug treatments

Key idea: there is no drug that fixes the enzyme. Drugs are used to avoid unsafe agents and to safely induce, maintain, and reverse anesthesia.

Unsafe / to avoid or use only with extreme caution

• Succinylcholine (suxamethonium).
  Class: Depolarizing neuromuscular blocker.
  Typical dose/time: 1–1.5 mg/kg IV for rapid intubation; action usually minutes but can last hours in deficiency.
  Purpose: Rapid muscle relaxation for intubation.
  Mechanism: Persistent depolarization at nicotinic receptors; normally hydrolyzed by plasma cholinesterase.
  Side effects: Prolonged apnea in deficiency, hyperkalemia risk, bradycardia, malignant hyperthermia trigger in susceptible patients. Not recommended when plasma cholinesterase is reduced. (Evidence: FDA labels ANECTINE/Quelicin and 2021 succinylcholine injection label.) FDA Access Data+2FDA Access Data+2

• Mivacurium.
  Class: Short-acting non-depolarizing NMBA, metabolized by plasma cholinesterase.
  Dose/time: Individualized; action can be greatly prolonged in deficiency.
  Purpose: Surgical relaxation.
  Mechanism: Competitive block of nicotinic receptors; relies on PChE for breakdown.
  Side effects: Prolonged block/apnea if cholinesterase low; histamine release. Use is risky in deficiency. (Evidence: FDA labels.) FDA Access Data+1

Preferred alternatives / supportive drugs

1. Rocuronium.
   Class: Non-depolarizing NMBA.
   Dose/time: 0.6–1.2 mg/kg IV; duration intermediate; can be reversed with sugammadex.
   Purpose: Intubation and surgical relaxation as a succinylcholine alternative.
   Mechanism: Competitive antagonist at nicotinic receptors; not dependent on plasma cholinesterase.
   Main adverse effects: Tachycardia, hypotension, anaphylaxis (rare). (Evidence: FDA labels and NDA approval.) FDA Access Data+1

2. Cisatracurium (Nimbex).
   Class: Non-depolarizing NMBA.
   Dose/time: 0.1–0.2 mg/kg IV; intermediate duration; organ-independent Hofmann elimination.
   Purpose: Muscle relaxation when hepatic/renal function is limited.
   Mechanism: Competitive block; spontaneous degradation (temperature and pH dependent).
   Side effects: Bradycardia, hypotension, rare anaphylaxis. (Evidence: FDA label.) FDA Access Data

3. Atracurium.
   Class: Non-depolarizing NMBA.
   Dose/time: 0.4–0.5 mg/kg IV; intermediate action; Hofmann elimination/ester hydrolysis (nonspecific).
   Purpose: Alternative relaxant not reliant on plasma cholinesterase.
   Mechanism: Competitive block; organ-independent breakdown.
   Side effects: Histamine release, hypotension; laudanosine metabolite (high doses) may lower seizure threshold. (Evidence: FDA ANDA label set.) FDA Access Data

4. Vecuronium.
   Class: Non-depolarizing NMBA.
   Dose/time: 0.08–0.1 mg/kg IV; intermediate duration; hepatic/renal elimination.
   Purpose: Another alternative to sux/mivacurium.
   Mechanism: Competitive block, no reliance on plasma cholinesterase.
   Side effects: Prolonged effect in organ failure. (Evidence: NMBA class labeling context; sugammadex indication covers vecuronium.) FDA Access Data

5. Sugammadex (Bridion).
   Class: Selective relaxant binding agent.
   Dose/time: Dosed by depth of block; rapidly reverses rocuronium/vecuronium.
   Purpose: Fast, reliable reversal when non-depolarizers are used.
   Mechanism: Encapsulates rocuronium/vecuronium molecules, removing them from the neuromuscular junction.
   Side effects: Hypersensitivity, bradycardia, anaphylaxis (rare), interactions with hormonal contraceptives. (Evidence: FDA label and clinical pharmacology review; 2024 pediatric update.) FDA Access Data+2FDA Access Data+2

6. Neostigmine (± glycopyrrolate) for reversal of other non-depolarizers (not sux).
   Class: Acetylcholinesterase inhibitor; antimuscarinic co-agent.
   Dose/time: Neostigmine up to 0.07 mg/kg IV with glycopyrrolate 0.01 mg/kg IV; reverse shallow-to-moderate block.
   Purpose: Reverse residual non-depolarizing block when sugammadex is not used/available.
   Mechanism: Raises acetylcholine at the neuromuscular junction; glycopyrrolate blunts muscarinic effects.
   Side effects: Bradycardia, secretions, bowel cramps; use antimuscarinic. (Evidence: BLOXIVERZ/Neostigmine labels; PREVDUO (fixed-dose neostigmine+glycopyrrolate).) FDA Access Data+2FDA Access Data+2

7. Propofol (hypnotic for induction/maintenance).
   Class: General anesthetic (IV).
   Purpose: Sedation/induction without affecting plasma cholinesterase metabolism of relaxants.
   Mechanism: GABA-A modulation; not related to BCHE deficiency.
   Side effects: Hypotension, apnea (central), pain on injection. (Evidence: standard anesthetic practice; see ULTIVA label cross-references listing Diprivan trademark). FDA Access Data

8. Opioid analgesics (e.g., remifentanil, fentanyl) as adjuncts.
   Class: Opioid agonists.
   Purpose: Analgesia and blunting of intubation response without affecting BChE metabolism.
   Mechanism: μ-opioid receptor agonism.
   Side effects: Respiratory depression—use with monitoring. (Evidence: ULTIVA (remifentanil) label for perioperative use.) FDA Access Data

9. Amide local anesthetics (e.g., lidocaine, bupivacaine).
   Class: Local anesthetic—amide type.
   Purpose: Regional/field blocks without relying on plasma cholinesterase.
   Mechanism: Sodium channel blockade; hepatic metabolism.
   Side effects: Local anesthetic systemic toxicity if overdosed. (Evidence: Bupivacaine labeling.) FDA Access Data

10. Phenylephrine/ephedrine (hemodynamic support) as needed.
    Class: Vasopressor/sympathomimetic.
    Purpose: Treat anesthesia-related hypotension safely.
    Mechanism: α-1 agonism (phenylephrine) or mixed action (ephedrine).
    Side effects: Hypertension, tachycardia. (Evidence: standard perioperative pharmacology; adjunct to labels above.) NCBI

11. Antiemetics (ondansetron, dexamethasone).
    Purpose: Reduce nausea/vomiting; no effect on BChE metabolism. (Evidence: standard perioperative practice.) NCBI

12. Sedatives (midazolam) for comfort during unexpected prolonged ventilation.
    Purpose: Comfort and amnesia while the block resolves. (Evidence: StatPearls.) NCBI

Notes: Some centers may consider FFP or purified human cholinesterase in exceptional cases of very prolonged paralysis, but this is not routine because transfusion carries risks and many patients recover safely with ventilation and time. (Evidence: OrphanAnesthesia; Medscape.) Orphan Anesthesia+1

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Dietary molecular supplements

There is no supplement that “fixes” the genetic enzyme problem. These ideas support general liver function, healing, and safe recovery after surgery. Always discuss with your clinician.

1. Balanced protein (essential amino acids).
   Dose: meet daily protein needs (e.g., 1.0–1.2 g/kg/day if medically appropriate).
   Function/mechanism: The enzyme is a liver-made protein; adequate protein supports hepatic synthesis and overall recovery. (Evidence: StatPearls—acquired deficiency worsens with malnutrition.) NCBI

2. Omega-3 fatty acids (fish oil).
   Dose: commonly 1–2 g/day EPA+DHA if appropriate.
   Function/mechanism: General anti-inflammatory support; may help recovery after surgery. (Background nutritional evidence; not disease-specific.) NCBI

3. Multivitamin with B-complex.
   Dose: as labeled.
   Function/mechanism: Corrects common deficits that may worsen malnutrition-related enzyme reductions. (Evidence: StatPearls—malnutrition link.) NCBI

4. Vitamin D.
   Dose: per level-guided replacement.
   Function/mechanism: Supports muscle and immune function during recovery. (General perioperative nutrition rationale.) NCBI

5. Vitamin C.
   Dose: 200–500 mg/day typical.
   Function/mechanism: Antioxidant support during surgical recovery. (General evidence.) NCBI

6. Zinc.
   Dose: 8–11 mg/day dietary; short courses if deficient.
   Function/mechanism: Supports wound healing and protein synthesis. (General surgical nutrition.) NCBI

7. Selenium.
   Dose: ~55 mcg/day dietary; supplement if low.
   Function/mechanism: Antioxidant enzyme cofactor; supports recovery. (General nutrition.) NCBI

8. Probiotics (as tolerated).
   Dose: per product label.
   Function/mechanism: May reduce post-op GI upset; no direct effect on BChE. (General GI support.) NCBI

9. Electrolyte repletion (magnesium, potassium) under supervision.
   Function/mechanism: Stable electrolytes aid muscle and nerve function while recovering from block. (Perioperative basics.) NCBI

10. Avoid unregulated “cholinesterase boosters.”
    Function/mechanism: Claims are unproven; could interact with anesthesia. (Safety guidance.) NCBI

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Immunity-booster / regenerative / stem cell

There are no approved immunotherapy or stem-cell drugs that treat benzoylcholinesterase deficiency. Below are supportive pharmacologic ideas used around anesthesia; they do not regenerate the enzyme.

1. Balanced parenteral/enteral nutrition (medical therapy).
   ~100 words: Supports hepatic protein production; corrects malnutrition that can lower enzyme levels. Dose: individualized by dietitian. Function/mechanism: restores substrates for synthesis. (Evidence: StatPearls—malnutrition link.) NCBI

2. Antioxidant vitamins (A/C/E) if deficient.
   Dose: replacement only. Function: reduce oxidative stress during recovery; mechanism: antioxidant pathways. (General nutrition.) NCBI

3. Selenium/Zinc repletion if low.
   Dose: standard RDA unless deficiency documented. Function: co-factors in protein/antioxidant enzymes. (General nutrition.) NCBI

4. Physiologic steroids (only if clinically indicated, e.g., adrenal insufficiency).
   Function: maintain hemodynamic stability; mechanism: genomic modulation of inflammation. (Perioperative standards.) NCBI

5. No stem-cell therapy is indicated.
   Function/mechanism: Not studied or recommended for this enzyme deficiency. (Evidence: consensus/absence in guidelines.) Orphan Anesthesia

6. Vaccinations per schedule (general immune health).
   Function: prevent infections that could worsen catabolic states. (Public-health standards.) NCBI

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Surgeries (procedures) and why they are done

The deficiency does not require surgery. These are common procedures where special anesthesia planning is most important.

1. Emergency intubation for airway protection.
   Why: Needed for trauma, sepsis, or status asthmaticus; plan rocuronium (with possible sugammadex) instead of succinylcholine. (Evidence: rocuronium/sugammadex labels.) FDA Access Data+1

2. Elective general surgery (e.g., hernia repair).
   Why: Requires muscle relaxation and ventilation; choose non-depolarizers and objective reversal. (Evidence: NMBA labels; StatPearls.) FDA Access Data+1

3. Cesarean delivery or obstetric procedures.
   Why: If general anesthesia is required, avoid sux/mivacurium; regional techniques with amide locals may be preferred. (Evidence: Mayo patient guidance; bupivacaine labeling.) Mayo Clinic+1

4. Endoscopy or short procedures with sedation.
   Why: Often possible without any neuromuscular blocker; reduces risk entirely. (Evidence: StatPearls.) NCBI

5. Regional anesthesia/nerve blocks.
   Why: When appropriate, use amide local anesthetics (lidocaine/bupivacaine) to avoid ester-drug issues. (Evidence: Mayo Clinic; bupivacaine label.) Mayo Clinic+1

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Preventions

1. Wear a medical alert ID. (Evidence: Mayo; Medscape; AJMS review.) Mayo Clinic+2Medscape+2

2. Put a critical allergy/alert in your medical record. (Evidence: Mayo guidance.) Mayo Clinic

3. Tell every anesthesia team before any procedure. (Evidence: MedlinePlus.) MedlinePlus

4. Avoid succinylcholine and mivacurium. (Evidence: FDA labels; MedlinePlus.) FDA Access Data+2FDA Access Data+2

5. Prefer non-depolarizers (rocuronium/cisatracurium/atracurium), with sugammadex or neostigmine+antimuscarinic for reversal as appropriate. (Evidence: labels.) FDA Access Data+2FDA Access Data+2

6. Avoid or minimize ester local anesthetics; prefer amide agents when suitable. (Evidence: Mayo guidance; bupivacaine label.) Mayo Clinic+1

7. Keep nutrition and liver health optimized. (Evidence: StatPearls.) NCBI

8. Share information with family; consider testing. (Evidence: MedlinePlus.) MedlinePlus

9. Use objective neuromuscular monitoring in any case using relaxants. (Evidence: StatPearls.) NCBI

10. Have a clear post-op plan for ventilation if exposure occurs. (Evidence: Anectine label; StatPearls.) FDA Access Data+1

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When to see a doctor

See a doctor (and tell anesthesia) before any surgery or procedure that might use general anesthesia or local numbing medicines. Seek urgent care if you recently had anesthesia and feel short of breath, weak, cannot swallow well, or cannot lift your head, or if a family member had “trouble waking up” after surgery and you plan to have anesthesia. These steps help you avoid the trigger drugs and plan safe alternatives. (Evidence: MedlinePlus; StatPearls.) MedlinePlus+1

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What to eat and what to avoid

1. Eat: Balanced diet with adequate protein (supports liver protein synthesis). Avoid: crash diets around surgery. (Evidence: StatPearls—malnutrition risk.) NCBI

2. Eat: Fruits/vegetables for micronutrients. Avoid: excessive alcohol (harms liver). (Evidence: StatPearls.) NCBI

3. Eat: Healthy fats (e.g., fish, nuts). Avoid: unregulated “enzyme-boosting” supplements. (Evidence: StatPearls.) NCBI

4. Hydration: Maintain fluids unless your doctor restricts them. Avoid: dehydration before elective procedures. (Evidence: perioperative standards.) NCBI

5. Post-op: Small, high-protein meals as tolerated. Avoid: heavy alcohol/sedatives unless prescribed. (Evidence: StatPearls.) NCBI

(Diet does not cure the enzyme problem; it supports recovery and general health.)

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Frequently asked questions (FAQs)

1) Is this the same as “suxamethonium apnea”?
Yes. That term means breathing stops for a long time after succinylcholine because the enzyme that clears it is missing or weak. (Evidence: StatPearls; FDA label.) NCBI+1

2) Can I live a normal life?
Yes. Most people have no daily symptoms. The key is to avoid the trigger drugs and tell every anesthesia team in advance. (Evidence: StatPearls; MedlinePlus.) NCBI+1

3) What test confirms it?
A dibucaine number and serum cholinesterase activity test confirm risk; genetic testing can identify BCHE variants. (Evidence: OpenAnesthesia; ARUP; MedlinePlus.) OpenAnesthesia+2ltd.aruplab.com+2

4) What numbers are typical?
Normal dibucaine number ≈ 80; carriers ~50–60; severely affected ~20–30. (Evidence: OpenAnesthesia; Medscape.) OpenAnesthesia+1

5) Which drugs are unsafe?
Succinylcholine and mivacurium; many ester local anesthetics may last longer (procaine, tetracaine, benzocaine, chloroprocaine, cocaine). (Evidence: MedlinePlus; Mayo Clinic; FDA labels.) MedlinePlus+2Mayo Clinic+2

6) Which muscle relaxants are safer?
Non-depolarizers such as rocuronium, cisatracurium, atracurium, vecuronium, with proper monitoring and reversal. (Evidence: FDA labels.) FDA Access Data+1

7) How is prolonged paralysis treated if it happens?
Secure the airway, give mechanical ventilation and sedation until the drug wears off. Rarely, blood products like FFP are considered case-by-case. (Evidence: Anectine label; OrphanAnesthesia; Medscape.) FDA Access Data+2Orphan Anesthesia+2

8) Is sugammadex useful?
Yes—for rocuronium or vecuronium reversal; it does not reverse succinylcholine. (Evidence: FDA Bridion label.) FDA Access Data

9) Do I need to avoid all local anesthetics?
No. You mainly avoid ester types; amide types (e.g., lidocaine, bupivacaine) are usually fine when dosed properly. (Evidence: Mayo Clinic; bupivacaine label.) Mayo Clinic+1

10) Could pregnancy make it worse?
Yes, enzyme levels can be lower in pregnancy, so anesthesia plans should be adjusted. (Evidence: StatPearls.) NCBI

11) Should my family be tested?
It can help, especially if a close relative had anesthesia problems. (Evidence: MedlinePlus.) MedlinePlus

12) Is there a medicine to replace the enzyme?
Not as a routine therapy. Human serum cholinesterase or FFP has been reported in rare, severe cases. (Evidence: OrphanAnesthesia; Medscape.) Orphan Anesthesia+1

13) Can I have rapid sequence intubation safely?
Yes—use rocuronium (with appropriate dosing and sugammadex available) instead of succinylcholine. (Evidence: rocuronium and sugammadex labeling.) FDA Access Data+1

14) Will this affect dental care?
Tell your dentist; avoid benzocaine sprays/gels and other ester anesthetics when possible. (Evidence: Mayo Clinic.) Mayo Clinic

15) Where should I store my documents?
Keep a visible medical alert and a copy of test results in your phone and wallet. (Evidence: Mayo Clinic; Medscape.) Mayo Clinic+1

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: November 07, 2025.

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