A lesson from Cystic Fibrosis and Anesthesia: Perioperative Management Strategies

Anesthesia Maintenance in Cystic Fibrosis: Volatile Agents and Ventilation

General anesthesia with the CF patient provides a safe and effective way to maintain adequate oxygenation.   Using volatile agents facilitates the delivery of high-inspired oxygen concentrations, effective bronchodilation, and a decrease in hyperreactive airways.  The proper usage of volatile agents also decreases the use of neuromuscular-blocking agents.   Caution should be used with the administration of desflurane.  Due to its strong odor, this agent can cause a reactive airway and increase the potential for bronchoconstriction.

Postoperative ileus is a common complication in the CF patient who receives excessive intraoperative opioids.   Well-balanced intraoperative analgesia that minimizes the use of narcotics will reduce the risk of ileus and respiratory depression.   Nonsteroidal anti-inflammatory agents should be used judiciously as an adjunct to opioids. Intraoperative infiltration of a local anesthetic at the surgical site will reduce the need for postoperative pain control, which, in turn, reduces the risk of ileus and respiratory depression.  

Spontaneous respirations during the surgical procedure benefit the patient. The use of an anticholinergic agent, when used as a reversal adjunct in the presence of muscle relaxants, can cause an increase in the viscosity of mucus.  A surgical procedure without muscle relaxants facilitates respiratory-muscle recovery and faster recovery from surgery.  

Calculating and implementing a proper fluid plan is imperative to maintain adequate hydration in the CF patient.  Proper hydration preserves respiratory secretions in a less dense state, which decreases the risk of developing mucous plugs.   To achieve adequate hydration, the anesthesia provider must consider insensible losses of 2ml/kg/hr and add this to the fluid plan.  

Using humidified inspired oxygen also decreases the risk of mucous plugs by keeping the secretions less dense. Using a heat-and-moisture exchanger (HME) must be weighed against the increased dead space created when this device is added and the increased work of breathing developed by the HME.   The increased dead space and work of breathing can potentially be harmful, leading to a high retention of carbon dioxide. The decision to use an HME should be patient-specific.

Intermittent endotracheal suctioning during surgery will minimize the accumulation of bronchial secretions.  Oropharyngeal suctioning will also decrease the risk of bronchospasm and aspiration on emergence.  Attempts should be made so the patient avoids coughing while intubated to reduce the risk of bronchospasm.  Using 1-1.5mg/kg of lidocaine intravenously before extubation facilitates smooth muscle relaxation and will help decrease the risk of coughing. 

During long surgeries, the CF patient’s medication and schedule must be maintained.  The anesthesia provider must be incredibly familiar with the patient’s antibiotic regimen and adhere to it during the maintenance phase of anesthesia.  As always, the goal should be constant vigilance on the part of the anesthesia provider to avoid patient distress and provide a safe anesthesia experience.

InterventionRationale or caveat
Volatile agentsDeliver high inspired oxygen, bronchodilation and reduced airway hyperreactivity, and lower neuromuscular blocker use
DesfluraneUse with caution — its strong odor can cause a reactive airway and bronchoconstriction
OpioidsExcessive intraoperative opioids cause postoperative ileus; balance analgesia and use NSAIDs judiciously as an adjunct
Local anesthetic at the surgical siteReduces postoperative pain control needs and therefore ileus and respiratory depression
Muscle relaxantsAvoiding them favors spontaneous respiration and respiratory-muscle recovery; anticholinergic reversal adjuncts thicken mucus
Fluid planAdd insensible losses of 2 mL/kg/hr; hydration keeps secretions less dense and lowers mucous plug risk
Humidified inspired oxygenKeeps secretions less dense; an HME adds dead space and work of breathing, so the decision is patient-specific
Endotracheal and oropharyngeal suctioningIntermittent suctioning limits secretion accumulation and lowers bronchospasm and aspiration risk on emergence
Lidocaine 1–1.5 mg/kg IV before extubationFacilitates smooth muscle relaxation and decreases coughing
Home antibiotic regimenMaintain the patient’s medication schedule through long procedures

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