An Evaluation of Procedural Sedation Techniques in Duchenne Muscular Dystrophy Patients Undergoing Stem Cell Therapy

Keywords

Duchenne muscular dystrophy - procedural sedation - cardiorespiratory depression

Introduction: Anesthesia in Duchenne muscular dystrophy (DMD) poses many challenges, because of poor cardiorespiratory function, weak airway muscles, macroglossia, or obstructive sleep apnea. The present study was undertaken to evaluate safety as well as efficacy of procedural sedation techniques, and to assess the effect on hemodynamic and respiratory parameters in patients of DMD.

Methodology/Description: The present prospective, observational study was performed in 54 consecutive male patients of DMD presenting for stem cell therapy. After institutional ethics committee approval, patients coming for elective bone marrow aspiration and intrathecal catheterization as a part of stem cell therapy with age > 5 years were included. Patients unwilling for consent and patients requiring general anesthesia were excluded. Drugs and dosages used were noted. Hemodynamic parameters were noted every 5 minutes. Sedation levels were monitored using Ramsay sedation score every 10 minutes. Statistical analysis was done using the unpaired “t” test and p value of < 0.05 was considered significant.

Results: The age range was from 6 to 32 years with average of 11.59 years. Most commonly used drugs for procedural sedation were midazolam, dexmedetomidine infusion, and ketamine. Hemodynamic stability was maintained in all patients. Respiratory rate and end-tidal CO₂ were maintained close to baseline (p > 0.05). No cardiorespiratory adverse events were noted.

Conclusion: Dexmedetomidine and ketamine provide good procedural sedation without causing cardiore-spiratory depression, maintain airway reflexes, and offer adequate analgesia along with local anesthesia. The study subject draws attention to an often-neglected area and has scope for change in future practice.

• References

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• 2 Sethna NF, Rockoff MA, Worthen HM, Rosnow JM. Anesthesia-related complications in children with Duchenne muscular dystrophy.. Anesthesiology 1988; 68 (03) 462-465