A Primigravida with Common Variable Immunodeficiency, Chronic Liver Disease, and Shwachman- Diamond Syndrome: A Case Report

Common Variable Immunodeficiency (CVID) is a heterogeneous primary antibody deficiency characterized by markedly reduced serum immunoglobulins and impaired vaccine responses. Its co-occurrence with Shwachman-Diamond Syndrome (SDS), an autosomal recessive disorder caused by biallelic SBDS gene mutations, is exceptionally rare. This case report examines the management of a 32-year-old primigravida facing a rare triad of CVID, Chronic Liver Disease (CLD), and SDS. It highlights the specialized nursing vigilance required when multiple immunological and hematological vulnerabilities converge during pregnancy. The patient presented at 35 weeks and 6 days of gestation with a three day history of non-productive cough and concern regarding low amniotic fluid volume (AFI 8 cm). Primary diagnoses included CVID, CLD with portal hypertension (Grade II esophageal varices), SDS-1, and Gestational Diabetes Mellitus (GDM). Key interventions included antibiotic therapy for respiratory symptoms, monthly IVIg infusions, strict bleeding and infection precautions, and comprehensive nursing surveillance encompassing maternal-fetal monitoring, patient education, and coordinated discharge planning. The primary take-away is that pregnancies involving rare multisystemic immunodeficiencies and liver cirrhosis require a highly coordinated, multidisciplinary nursing approach to prevent life threatening infections and hemorrhage