Pregnancy in Cystic Fibrosis with Lung Transplant: A Case Report

 

Objective: This case report highlights the complexities and challenges of managing pregnancy in a woman with cystic fibrosis (CF) who has undergone lung transplant. It underscores the need for careful multidisciplinary coordination in managing such high-risk pregnancies, and the unique considerations required for optimizing maternal and fetal outcomes.

Case Report: A 31-year-old woman, G5P0 with cystic fibrosis and lung transplantation presented for antenatal care at 15 weeks of gestation. She had undergone bilateral lung transplant 6 years prior due to end-stage pulmonary disease associated with CF. Post-transplant, she was maintained on immunosuppressive therapy, including Tacrolimus, Azathioprine, and Prednisolone.

She underwent 5 IVF cycles for current conception and Preimplantation Genetic Diagnosis (PGD) to confirm the embryo was not affected. The patient was closely monitored by a multidisciplinary team, including obstetrics – maternal fetal medicine, pulmonology, and neonatology; and managed for potential risks of preterm labor, respiratory compromise, and rejection of the transplanted lungs. She required frequent obstetric ultrasounds, pulmonary assessments, with adjustments to her medications to minimize the risk of infection and rejection while safeguarding fetal development. Her blood pressure, renal function, and glucose levels were closely monitored due to the side effects of immunosuppressants. Lung function remained stable, however she developed Pulmonary Embolism (PE) managed with Enoxaparin, hypertension, and renal impairment with Chronic Kidney Disease (CKD) stage 1.

Patient was followed up by tertiary centre and birthed by Caesarean section at 35 weeks of gestation.

Discussion: Pregnancy in women with cystic fibrosis and prior lung transplant presents significant challenges. The potential risks of pulmonary deterioration, graft rejection, and complications from immunosuppressive drugs require a carefully coordinated approach. Additionally, management during pregnancy is particularly complex due to the potential teratogenic effects and risks of infection. This case emphasizes the importance of individualized care and collaboration across specialties to optimize outcomes for both mother and baby.

Conclusions: This case underscores feasibility of successful pregnancy in women with cystic fibrosis who have undergone lung transplantation. Multidisciplinary management and regular monitoring of lung function, immunosuppressive therapy, and fetal development are crucial. Future research should focus on optimizing therapeutic regimens for these high-risk pregnancies and understanding the long-term outcomes for both mothers and children. This case also highlights the need for clear guidelines in managing such pregnancies, especially in terms of medication management, timing of delivery, and long-term maternal health post-pregnancy.