Self-management in adrenal insufficiency — towards a better understanding

Abstract

CORTISOL is an essential stress hormone produced by the adrenal glands and tightly regulated via the hypothalamic-pituitary-adrenal axis. Cortisol deficiency is a rare, but life-threatening condition, requiring immediate diagnosis and treatment. The most common cause of primary adrenal insufficiency (AI) is an autoimmune process [1], but also infections, tissue infiltration, drugs or surgery may lead to insufficient production of glucocorticoids (GC) in the adrenal glands [2]. Secondary and tertiary AI are most often due to a pituitary adenoma or traumatic injury [3].Regardless of the aetiology, patients with cortisol deficiency need an individual substitution treatment plan, which has to be adjusted in stressful situations. Abstract. Patients with adrenal insufficiency (AI) require life-long glucocorticoid (GC) replacement treatment and dose adjustment in stress situations to prevent life-threatening adrenal crises. Herein this study we evaluated the patients' healthcare situation and their knowledge on AI, comparing various aspects to a prior survey in 209 physicians. Using a questionnaire, we conducted a comprehensive survey among 33 AI patients who were treated at the endocrine outpatient clinics of two University Hospitals in Germany. The majority of AI patients (97%) named their treating physician as main source for information. Overall, 89.7% of interviewees were satisfied with their medical treatment; however, about 1/3 reported controversies with healthcare professionals regarding GC replacement in various situation. Two thirds of AI patients increased their substitution dose temporarily within the last 12 months. However, not all patients had an emergency ID, and only 64.5% an emergency kit. None of the interviewed patients identified the need for adjustment in all given situations correctly. Almost 80% of patients did not correctly identify all symptoms of GC over-and under-replacement. Interestingly, we found no significant differences between patients and physicians regarding specific aspects of GC replacement. We showed that: (i) AI patients have some knowledge gaps on modalities and adequacy of GC replacement therapy; (ii) long-term management of patients with AI remains a challenge requiring an experienced specialist; and (iii) further education of physicians as primary source of information is necessary. Additional education may help AI patients to empower them to adequate self-treatment. Self-management in adrenal insufficiency

Management of adrenal insufficiency

“…[1] The role of the patient in recognizing when an increase in glucocorticoid doses is required is central to minimize the risk to themselves; thus, the importance of patient education is repeatedly emphasized. [2,3] Published existing literature has identified deficiencies in the patients' understanding of steroid sick day rules, [2,4] how to administer parenteral hydrocortisone, [2,4,5] and when to seek medical attention. [2] However, reports highlight gaps in clinician knowledge, particularly on the recognition, and the management of AI.…”

“…[2] However, reports highlight gaps in clinician knowledge, particularly on the recognition, and the management of AI. [4,[6][7][8] Given that healthcare professionals have a central role in patient education, it is essential that clinicians, irrespective of grade are familiar with steroid sick day rules and pharmacokinetics to avoid preventable adverse patient outcomes. Furthermore, the increasing use of drugs such as immune checkpoint inhibitors and the endocrinopathy side effects associated with these further supports the need for all physicians, not just endocrinologists, to recognize, and promptly initiate treatment AI.…”

Novel insights into glucocorticoid replacement therapy for pediatric and adult adrenal insufficiency

“…42,57 Guidelines stipulate that patients need to be equipped not only with an emergency card but also with an emergency kit for parenteral glucocorticoid self-administration. 83 A recent survey 89 performed in Germany on 33 patients showed that less than 70% carried an emergency kit, almost 80% were unable to correctly adjust the glucocorticoid dose during common illnesses and only 20% could identify the symptoms of over- and undertreatment.…”

“…2,88 Its potency stems from the inadequate level of cortisol that is unable to modulate the immune response, thus favoring the inflammatory cytokines excess, and to enhance vascular reactivity to catecholamines. 2,89 Other precipitators are surgical procedures, physical or psychological stress, or abrupt interruption of corticosteroid replacement. 2,88 As for risk factors, patients with PAI are much more prone because of the deficit of mineralocorticoid hormones.…”

Assessing the Knowledge of Sick-Day Rules Among Patients on Long-Term Glucocorticoids and Healthcare Professionals in a UK District General Hospital

“…Knowledge about "sick-day" rules, the importance of "steroid cards," and the utility of parenteral steroids is shown to be suboptimal. 14,15…”