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Anesthetic management of a parturient with end-stage cystic fibrosis
Abstract Number: T1D-8
Abstract Type: Case Report/Case Series
Cystic fibrosis (CF) is an autosomal recessive disorder defined by thickened secretions that leads to multi-organ dysfunction, most notably in the GI and pulmonary systems. Despite some published literature, specific guidelines for the anesthetic management of CF parturients with severe disease do not exist. We report a case of successful management of a patient with severe CF undergoing Caesarean section.
A 24yo, 48kg (BMI: 20.6 kg/m2) G2P1 known CF female presented at 30&5/7 weeks with several days of marked dyspnea, cough, fever, and hypoxia requiring initiation of continuous BiPAP prior to transfer to our institution. Her pregnancy had already been complicated by 2 infective exacerbations, each requiring a 2-week hospital course. At baseline, she required 4-5L nasal oxygen and BiPAP at night. Pre-pregnancy PFTs revealed FEV1 0.58L (19% predicted) and FVC 1.25L (35% predicted). A multidisciplinary team of internal medicine, obstetrics, pulmonary/critical care, and obstetric anesthesia determined it best to attempt medical optimization prior to a Caesarean delivery at 34 weeks. With aggressive medical therapy, she was weaned off BiPAP and stabilized on 10L nasal oximizer, yet remained unable to lay supine and had a persistent, violent cough. We pursued an anesthetic plan that avoided narcotics and intubation given the risks of respiratory depression and terminal postoperative ventilator dependence. To achieve this goal, we placed a pre-operative epidural in her labor room and slowly dosed it with a 0.15% bupivacaine infusion. Once proper epidural function was confirmed, she was transferred to the OR. The infusion was discontinued, and a T4 surgical blockade was achieved via slow epidural boluses of 2% lidocaine with epinephrine (5 mcg/ml). Supplemental narcotics or benzodiazepines were not required. She had an uneventful operative course on 10L nasal oximizer and was transferred to the ICU for close observation. She was weaned to her home oxygen requirements and transferred to the floor by POD 1. Her epidural was continued for post-operative analgesia to avoid narcotics and was removed POD 1 per her request. The remainder of her postpartum course was uneventful, and she was discharged POD 7.
Advancements in fertility medicine, management of high-risk pregnancies, and CF treatment have led to more CF patients successfully carrying gestations to viability. CF poses significant challenges for the obstetric anesthesiologist, who must synthesize the inputs of a multidisciplinary team into a safe and effective anesthetic approach. The few previously published reports of anesthesia for Caesarean in severe CF all utilize regional anesthesia, predominantly CSE, in addition to intravenous anxiolysis and/or analgesia. Due to our patient’s severe pulmonary compromise, worse than described in most reports, we elected for a slow and carefully targeted purely epidural anesthetic with excellent result for mother, fetus, and their healthcare providers.