Anesthesia Implications for the Lumbee Native American Population
Lumbee Native Americans have a significant risk of malignant hyperthermia due to Native American Myopathy (NAM), requiring avoidance of triggering agents and preparation for MH treatment during anesthesia.
Native American Myopathy in the Lumbee Population
Native American Myopathy (NAM) is a rare autosomal recessive disorder first reported in the Lumbee Indians of North Carolina 1. This condition has important anesthetic implications:
- Prevalence estimated at approximately 2:10,000 in the Lumbee population 1
- Associated with a 36% mortality rate by age 18 1
- Linked to mutations in the STAC3 gene, particularly the homozygous missense variant (c.851G > C; p.Trp284Ser) 2
Key Clinical Features of NAM
- Congenital weakness and arthrogryposis
- Cleft palate
- Ptosis
- Short stature
- Kyphoscoliosis
- Talipes deformities
- Susceptibility to malignant hyperthermia triggered by anesthesia 1, 2
Anesthetic Management Recommendations
Preoperative Assessment
- Thorough evaluation for signs of NAM (physical abnormalities, family history)
- Assess for difficult airway due to potential craniofacial abnormalities
- Document any previous anesthetic exposures and complications
- Consider genetic testing for STAC3 mutations in patients with family history or clinical features of NAM
Anesthetic Technique Selection
Avoid MH-triggering agents:
- Avoid all volatile anesthetics (sevoflurane, desflurane, isoflurane)
- Avoid succinylcholine
Preferred anesthetic approaches:
- Total intravenous anesthesia (TIVA) with propofol when general anesthesia is required
- Regional or neuraxial anesthesia when appropriate for the procedure
- Spinal anesthesia has been associated with decreased risk of complications in orthopedic procedures 3
Monitoring requirements:
- Standard ASA monitoring (ECG, SpO2, NIBP, capnography)
- Temperature monitoring is essential
- Consider more invasive monitoring based on procedure complexity
Preparation for Potential MH Crisis
- Ensure dantrolene is immediately available (minimum 36 vials/720mg for a 70kg patient)
- Prepare ice, cold saline, and cooling blankets
- Have MH protocol readily accessible in the operating room
- Ensure capability for rapid arterial blood gas analysis
Postoperative Considerations
- Extended PACU observation period
- Continued temperature monitoring
- Vigilance for delayed MH reactions
- Clear documentation of NAM diagnosis in medical records for future anesthetic encounters
Additional Anesthetic Considerations
Airway Management
- Higher incidence of difficult airway due to craniofacial abnormalities
- Have difficult airway equipment readily available
- Consider videolaryngoscopy for first attempt at intubation
- For patients with cleft palate, special attention to tube positioning and fixation
Cardiovascular Effects
- Patients with NAM may have increased sensitivity to the cardiovascular depressant effects of anesthetics 4
- Titrate anesthetic agents carefully to maintain hemodynamic stability
- Consider using ketamine for its sympathomimetic properties when appropriate, but monitor for emergence reactions 5
Pain Management
- Maximize multimodal non-opioid analgesics (NSAIDs, acetaminophen)
- Regional anesthesia techniques when possible
- Careful titration of opioids if required
Conclusion
Anesthesia providers caring for Lumbee Native Americans must maintain a high index of suspicion for NAM and implement appropriate precautions to prevent malignant hyperthermia. A non-triggering anesthetic technique with careful monitoring is essential to reduce morbidity and mortality in this population.