Prevention and Management of Adrenal Crisis in Children and Adults

Abstract

Adrenal crisis (AC) is a potentially life-threatening event in patients with adrenal insufficiency (AI). Patients with AI are at risk of developing AC if glucocorticoid replacement is reduced or stopped, or if the dose is not increased during periods of intercurrent illness or major emotional and physical stress. Infections and gastroenteritis are the main precipitating factors for AC which must be treated immediately with parenteral hydrocortisone administration. Mortality is twofold in patients with AI and 1 in 12 patients have at least one hospital admission per year due to AC. Most hospital admissions and deaths from AC can be prevented with prompt management. Education and support for self-management for patients and their families is of paramount importance to achieve optimal adherence with “sick day rules” and to prevent AC. Every patient should be provided with an emergency steroid card, a hydrocortisone injection pack and regular training on self-administration. Patients and families should be encouraged to access information and resources available via the patient advocacy groups. Moreover, raising awareness and training of healthcare professionals in the acute service and developing appropriate care pathways such as ambulance “red flag” protocols ensures that patients are treated immediately when presenting with AC. This chapter presents a comprehensive overview of the causes, clinical presentation, treatment, and prevention of AC. Specific sections in the chapter focus on the care and unique needs of the child presenting with AC and their families. A great emphasis is given on patient education with a focus on the key role of the endocrine nurse as an advanced practitioner in this aspect of patient care. This chapter follows on from Chap. 37 in the adrenal section and we strongly recommend the reader refers to it for a comprehensive overview of the diagnosis and management of adrenal insufficiency.