///2019 Abstract Details
2019 Abstract Details2019-07-13T07:45:15-05:00

The importance of a multidisciplinary approach in caring for a pregnant patient diagnosed with Creutzfeldt-Jakob Disease

Abstract Number: RF3AC-356
Abstract Type: Case Report Case Series

Lynn Squires MD1 ; Adrian Koziak MD, MSc, FRCPC2; Valerie Sim MD, FRCPC3; Allison Thiele MD, PhD, FRCSC4

Introduction

Sporadic Creutzfeldt-Jakob Disease (CJD) is a rare degenerative prion disease affecting one per million people annually(1). While it typically affects people later in life, there have been seven reported case studies of CJD in pregnant women(2). We present a case of a 35 year old G2P1 woman diagnosed with CJD at 14+3 weeks gestation. We will outline the successful multidisciplinary approach to her care.

Patient History

The patient initially presented with new onset memory deficits and challenges with daily tasks. CJD was suspected due to characteristic MRI findings and confirmed with CSF testing. As the patient’s cognitive status had declined, her family made the decision to undertake all lifesaving interventions until delivery. She was managed at home until 28 weeks gestation when she was admitted for fetal and maternal monitoring, as well as nasogastric feeds. At 32 weeks she developed autonomic instability manifested as maternal fever and tachycardia. At time of delivery, the patient was non-communicative, deconditioned, bedridden with contractures, and had developed intermittent trismus.

Discussion

Multidisciplinary meetings occurred from 17 weeks gestation onward to ensure clear communication and preparation. This included representatives from obstetrics, maternal fetal medicine, neonatology, anesthesia, intensive care, neurology, palliative medicine, infection control and ethics. Protocols were developed for both emergent and elective delivery, along with readily available kits containing disposable anesthesia and OR equipment.

The scheduled cesarean section proceeded under general anesthesia at 36+3 weeks gestation. Personal protective equipment was donned by all staff and non essential equipment and personnel were removed from the operative theatre. After a slowly titrated induction maintaining spontaneous ventilation and avoiding depolarizing neuromuscular blockade, the patient’s airway was secured with the aid of a bite block. A healthy neonate was delivered and uterine tone established.

The patient was transferred to a private room after reversal of all anesthetic medications and extubated while spontaneously breathing in the company of her family and newborn. This successful outcome was made possible through extensive communication with all staff involved in her care. This case demonstrates how patients with advanced CJD present unique anesthetic, ethical, and infection control challenges which require multidisciplinary approaches to fully optimize care.

References

1. Sperling, R., et al., Creutzfeldt-Jakob disease in the obstetric patient. J Obstet Gynecol Neonatal Nurs, 2005. 34(5): p. 546-50.

2. Di Gangi, S., et al., Obstetric dilemma on the most appropriate management of Creutzfeldt-Jakob disease in pregnancy: seventh case presentation, literature review and new insight. J Matern Fetal Neonatal Med, 2015. 28(3): p. 254-61.

SOAP 2019