Primary adrenal insufficiency or Addison’s disease is a rare chronic condition with an incidence of 4 -11/100,000 people. The management of Addison’s disease during pregnancy is extremely difficult and rarely encountered by endocrinologists and obstetricians. Dysregulation of the maternal hypothalamic-pituitary-adrenal (HPA) axis determines foetal exposure to stress hormones influencing development and birth outcomes. In healthy pregnant women cortisol, ACTH, CRH and CBG increase throughout pregnancy whilst the placenta itself can produce active CRH and ACTH stimulating the maternal pituitary and adrenal glands, in Addison’s it takes careful consideration to mimic the normal hormonal changes in pregnancy. Historically Addison’s disease was associated with a high maternal mortality and women were strongly discouraged from becoming pregnant. Modern glucocorticoid replacement therapy and improved obstetric care, have reduced both maternal and foetal morbidity and mortality.
Addisonian crises are life threatening events in pregnant women. They can be precipitated by pregnancy specific stressors such as hyperemesis gravidrum, labour and surgery, as well as the same precipitants that occur outside of pregnancy.
This presentation describes the case of Miss B, a 24 year old woman. Following her diagnosis with Addison’s disease. The first 18 months after her diagnosis and subsequent pregnancy will be explored, and the issues our team faced with her complex care needs.
Addison’s disease in pregnancy requires a high degree of self-management to prevent Addisonian crises. The endocrine clinical nurse specialist (CNS) is vital to ensure the patient has the knowledge and support they need to continue pregnancy safely. The role of a CNS is to provide clinical expertise and support for patients. This case highlights the CNS role within the multidisciplinary team and the ongoing support we were able to provide within this complex case.