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Severe Cystic Fibrosis and the Obstetric Patient: Perioperative Optimization and Anesthetic Planning
Abstract Number: F4C-7
Abstract Type: Case Report/Case Series
Cystic fibrosis is a lethal, autosomal recessive disease. Due to advances in treatment however, an increasing number of patients are surviving to reproductive age. As a result, Obstetricians and Obstetric Anesthesiologists are encountering more patients with CF each year. According to the CF Foundation, the number of pregnancies in women with CF has doubled between 1990 and 2015 (1). CF patients have an increased risk of peripartum complications including respiratory deterioration, pulmonary hypertension and preterm birth and so antepartum care should focus on pulmonary optimization (2). In addition, a multidisciplinary team should be prepared to provide a higher level of cardiopulmonary support in the peripartum period.
We present a case of a 29 year old female with severe cystic fibrosis who was admitted at 33 weeks with a CF exacerbation. Pre-admission PFTs demonstrated an FEV1 of 37% predicted. At baseline, she used 4L NC O2 however she was requiring increasing levels of O2 in the setting of worsening dyspnea, productive cough and hypoxia. She received a pulmonary tune-up, including broad-spectrum antibiotics and an aggressive pulmonary toilet regimen. At 34 weeks, monitoring demonstrated fetal distress and she was consented for induction. In early labor, she received an epidural which was placed easily and achieved a bilateral T11 level. Her induction was complicated by fetal heart rate decelerations, near emergent cesarean delivery and subsequent urgent section for fetal intolerance of labor. Her epidural was carefully dosed with 2% lido with epinephrine to achieve a T6 surgical level. She required 6L NC O2 to maintain a saturation of 95%. Her surgery was uneventful and she recovered well post-operatively. She experienced no additional respiratory issues and was discharged home on post-operative day three.
Our case demonstrates that a T6 anesthetic level provides adequate cesarean section conditions without compromising pulmonary function. She may not have tolerated additional weakening of her intercostal muscles from a higher thoracic block. We were able to avoid general anesthesia, which was our and our patient’s goal. Case series demonstrate that patients with cystic fibrosis with an FEV1 <50% predicted, as with our patient, carry a mortality risk up to 15% at 15 months postpartum (3). Our patient’s carefully titrated neuraxial anesthetic helped to decrease her risk of peripartum complications, primarily those that would have been attributed to airway manipulation, mechanical ventilation and potentially prolonged ventilator dependence.
1. Chestnut, David H. Chestnut's Obstetric Anesthesia: Principles and Practice. Fifth edition. Philadelphia, PA: Elsevier/Saunders, 2014.
2. Cystic Fibrosis Foundation Patient Registry. Annual Data Report 2015. Page 9.
3. Deighan M, Ash S, McMorrow R. Anaesthesia for parturients with severe cystic fibrosis: a case series. Int J Obstet Anesth. 2014 23(1):75-9.