Unique perioperative challenges in a parturient with Cystic Fibrosis and Cystic Fibrosis–Related Diabetes Using a Hybrid Closed-Loop Insulin Pump

Dr Shilpi Sethi ST5 Anaesthetic trainee

Dr Kate Bosworth Consultant Anaesthetist

Introduction

• Cystic fibrosis (CF) is a multisystem autosomal recessive disorder

• Mutations in the CF transmembrane conductance regulator protein [1]

• Viscous secretions and progressive pulmonary and extrapulmonary complications, including cystic fibrosis–related diabetes (CFRD)

• Physiological changes of pregnancy coupled with pathological manifestations of CF pose unique challenges

• Predicting outcomes and managing associated pathologies are crucial in management of CF

• As advances in CF therapy improve life expectancy, pregnancies in individuals with CF—and concomitant use of rtCGM and insulin pump technologies—are becoming increasingly common [2]

Case Report

• 35-year-old primigravida at 38 + 1 weeks’ gestation with CF complicated by chronic Burkholderia cepacia colonisation and pregestational CFRD managed with a hybrid closed-loop insulin pump

• History of neonatal colostomy and ileostomy

• Clinically stable on inhaled bronchodilators and aerosolised antibiotics, with normal spirometry.

• Pelvic MRI demonstrated dense adhesions between the uterus, abdominal wall, and anterior bladder dome, prompting a recommendation for elective cesarean delivery

• Comprehensive preoperative counselling was undertaken regarding anaesthetic options (spinal, combined spinal–epidural, or general anaesthesia) given the anticipated prolonged surgical duration

• A shared management plan with endocrinology supported continued patient-led pump use with preparedness to transition to a variable-rate intravenous insulin infusion (VRII) if glycemic instability occurred

• Cesarean delivery proceeded uneventfully under spinal anaesthesia

• Postpartum haemorrhage requiring return to theatre necessitated conversion to VRII

• The remainder of her postoperative course was uncomplicated

Discussion

• For women with CF, pregnancy carries added health risks, including higher rates of pneumonia, renal failure, and rarely death, with a mortality rate of 1% [3]

• Severe pulmonary disease (FEV1 < 50%), pulmonary hypertension, Burkholderia Cepacia infection, and exocrine pancreatic insufficiency have been shown to be important predictors of poor maternal outcomes

• During pregnancy, women with CF experience increased frequency of pulmonary exacerbations, hospitalizations and illness-related visits

• Early mobilisation and assisted physiotherapy is critical in the postnatal period to help in clearance with clearance of the airways and improved ventilation

Cystic Fibrosis-Related Diabetes

• Be aware of insulin sensitivity [4]

• Dysglycemia, post prandial hyperglycemia, reactive hypoglycemia

• VRII cannot be put through portocath

• Early liaison/low threshold with anaesthetics team for vascular access

• For women with diabetes / gestational diabetes who are having a planned caesarean section, the need for a variable rate intravenous insulin infusion (VRIII) should be discussed on an individual basis

Conclusion

• Highlights the unique perioperative challenges posed by the intersection of CF, CFRD, and closed-loop insulin delivery systems in obstetric anaesthesia

• Thorough preoperative assessment, structured shared decision-making, and close multidisciplinary collaboration are essential to optimise maternal and fetal outcomes

References

• [1] Deighan M, Ash S, McMorrow R. Anaesthesia for parturients with severe cystic fibrosis: a case series. Int J Obstet Anesth. 2014 Feb;23(1):75-9

• [2] Marks BE, Kilberg MJ, Aliaj E, Fredkin K, Hudson J, Riva D, Román C, Kelly A, Putman MS. Perceptions of Diabetes Technology Use in Cystic Fibrosis-Related Diabetes Management. Diabetes Technol Ther. 2021 Nov;23(11):753-759. doi: 10.1089/dia.2021.0201. Epub 2021 Jul 20. PMID: 34185606.

• [3] Oxman R, Roe AH, Ullal J, Putman MS. Gestational and pregestational diabetes in pregnant women with cystic fibrosis. J Clin Transl Endocrinol. 2021 Dec 5;27:100289. doi: 10.1016/j.jcte.2021.100289. PMID: 34984172; PMCID: PMC8693285.

• [4] Rogers WK, Chugaieva I, Moheet A, Wernimont SA. Diabetes Mellitus in Pregnancy: Implications for Obstetric Anesthesia. Anesthesiology. 2025 Aug 1;143(2):424-443