Timelines in the Management of Adrenal Crisis
Timelines in the Management of Adrenal Crisis
Objective To evaluate current management timelines in adrenal crisis (AC) and to establish time targets and time limits for emergency treatment.
Design/patients Patients from a prospective study who had reported an AC (n = 46) were contacted and asked about management of their AC. A survey among 24 European endocrinologists collected expert recommendations concerning time targets and time limits for contact–arrival time of emergency health professionals and presentation of emergency card–glucocorticoid (GC) injection time.
Results Median time targets and time limits regarded by experts as adequate for contact–arrival time were 45 and 90 min, respectively, and for card–injection time 15 and 30 min, respectively. Thirty-seven of 46 patients could be interviewed. All patients were equipped with an emergency card but only 23 (62%) with an emergency kit. Seven patients (19%) were trained in GC self-injection. The median time interval between contacting a health professional and arrival was 20 min (range 2–2880 min); ≤45 min: n = 32 (86%), <90 min: n = 34 (92%). The median time interval between arrival and administration of GC was 30 min (range 2–2400 min); ≤15 min: n = 17 (46%), ≤30 min: n = 20 (54%).
Conclusion While the time between contacting health professionals and their arrival was within the limits set by experts, initiation of GC administration was delayed in 46% of patients. Thus, improved management of AC needs to focus on shortening the presentation of card–injection time. Given the current reality in the management of AC, promotion of self-injection of GC (s.c. or i.m.) is warranted.
Adrenal crisis (AC) is a life-threatening emergency that may occur in patients with chronic adrenal insufficiency (AI). Precipitating factors are stressful conditions like infectious disease and trauma, but also psychological distress, leading to increased glucocorticoid demand. Thus, it is of high importance that patients with AI adjust their glucocorticoid dose under these conditions to prevent AC. Accordingly, patient education is considered a key step for crisis prevention. However, even in well-educated patients, AC occurs, and retrospective data demonstrate that about half of the patients with chronic AI have already experienced at least one AC since diagnosis, with an incidence around 6·8 per 100 patient years. A survey of the UK Addison's disease self-help group reported that 8% of the patients needed hospital treatment with injected hydrocortisone within the past 12 months and the 2013 follow-up survey indicates that an even higher number of almost 15% of patients had received an emergency injection or intravenous fluids.
Importantly, recent data revealed increased mortality in chronic AI. In a Norwegian analysis, 'adrenal failure' was the second most frequent cause of death in 130 patients with Addison's disease (15%). Furthermore, infection and sudden death were more common than in the general population. An analysis of the National Swedish Hospital and Cause of Death Registry documented an increased mortality in patients with Addison's disease compared to normative data. Of these deaths, 12·6% were attributed to 'endocrine causes' with 7·1% as death from AI per se. In this study, the risk ratio for death from infectious disease, which is a main precipitating factor for AC, was 6·6 [95% confidence interval (CI) 2·6–15·2] in men and 5·6 (CI 2–12) in women. Another Swedish analysis of 755 patients with secondary AI found an even higher standard mortality ratio for death from infectious disease of 8·9 (CI 4·7–15·2). Infectious disease was found to be associated with signs of AC in most cases. These data suggest that patients with chronic AI are particularly threatened by infectious disease and that AC substantially contributes to the increased mortality in AI. Based on these findings, prevention and optimal management of AC is likely to have a major impact on patient outcomes in AI.
Patient education is currently the most important measure for prevention of AC. However, in case of emergency, patients may depend on rapid initiation of professional care with immediate administration of parenteral glucocorticoids. Due to the rarity of AI, physicians are often unfamiliar with the correct emergency treatment of AC. Furthermore, the clinical features of impending AC are nonspecific, leading to a delay in emergency glucocorticoid administration. Anecdotal evidence suggests that the management of AC is frequently insufficient, contributing to the mortality rate of AC. Accordingly, patient self-help groups and endocrinologists often perceive the current management of AC outside of specialised centres as not being satisfactory. However, objective data supporting this assumption are lacking.
The aim of this study, therefore, was to assess current management of AC with a special focus on the time needed for initiation of parenteral glucocorticoid treatment in a well characterised cohort of patients with chronic AI and to compare the results with expert opinion on optimal time targets and time limits in AC.
Abstract and Introduction
Abstract
Objective To evaluate current management timelines in adrenal crisis (AC) and to establish time targets and time limits for emergency treatment.
Design/patients Patients from a prospective study who had reported an AC (n = 46) were contacted and asked about management of their AC. A survey among 24 European endocrinologists collected expert recommendations concerning time targets and time limits for contact–arrival time of emergency health professionals and presentation of emergency card–glucocorticoid (GC) injection time.
Results Median time targets and time limits regarded by experts as adequate for contact–arrival time were 45 and 90 min, respectively, and for card–injection time 15 and 30 min, respectively. Thirty-seven of 46 patients could be interviewed. All patients were equipped with an emergency card but only 23 (62%) with an emergency kit. Seven patients (19%) were trained in GC self-injection. The median time interval between contacting a health professional and arrival was 20 min (range 2–2880 min); ≤45 min: n = 32 (86%), <90 min: n = 34 (92%). The median time interval between arrival and administration of GC was 30 min (range 2–2400 min); ≤15 min: n = 17 (46%), ≤30 min: n = 20 (54%).
Conclusion While the time between contacting health professionals and their arrival was within the limits set by experts, initiation of GC administration was delayed in 46% of patients. Thus, improved management of AC needs to focus on shortening the presentation of card–injection time. Given the current reality in the management of AC, promotion of self-injection of GC (s.c. or i.m.) is warranted.
Introduction
Adrenal crisis (AC) is a life-threatening emergency that may occur in patients with chronic adrenal insufficiency (AI). Precipitating factors are stressful conditions like infectious disease and trauma, but also psychological distress, leading to increased glucocorticoid demand. Thus, it is of high importance that patients with AI adjust their glucocorticoid dose under these conditions to prevent AC. Accordingly, patient education is considered a key step for crisis prevention. However, even in well-educated patients, AC occurs, and retrospective data demonstrate that about half of the patients with chronic AI have already experienced at least one AC since diagnosis, with an incidence around 6·8 per 100 patient years. A survey of the UK Addison's disease self-help group reported that 8% of the patients needed hospital treatment with injected hydrocortisone within the past 12 months and the 2013 follow-up survey indicates that an even higher number of almost 15% of patients had received an emergency injection or intravenous fluids.
Importantly, recent data revealed increased mortality in chronic AI. In a Norwegian analysis, 'adrenal failure' was the second most frequent cause of death in 130 patients with Addison's disease (15%). Furthermore, infection and sudden death were more common than in the general population. An analysis of the National Swedish Hospital and Cause of Death Registry documented an increased mortality in patients with Addison's disease compared to normative data. Of these deaths, 12·6% were attributed to 'endocrine causes' with 7·1% as death from AI per se. In this study, the risk ratio for death from infectious disease, which is a main precipitating factor for AC, was 6·6 [95% confidence interval (CI) 2·6–15·2] in men and 5·6 (CI 2–12) in women. Another Swedish analysis of 755 patients with secondary AI found an even higher standard mortality ratio for death from infectious disease of 8·9 (CI 4·7–15·2). Infectious disease was found to be associated with signs of AC in most cases. These data suggest that patients with chronic AI are particularly threatened by infectious disease and that AC substantially contributes to the increased mortality in AI. Based on these findings, prevention and optimal management of AC is likely to have a major impact on patient outcomes in AI.
Patient education is currently the most important measure for prevention of AC. However, in case of emergency, patients may depend on rapid initiation of professional care with immediate administration of parenteral glucocorticoids. Due to the rarity of AI, physicians are often unfamiliar with the correct emergency treatment of AC. Furthermore, the clinical features of impending AC are nonspecific, leading to a delay in emergency glucocorticoid administration. Anecdotal evidence suggests that the management of AC is frequently insufficient, contributing to the mortality rate of AC. Accordingly, patient self-help groups and endocrinologists often perceive the current management of AC outside of specialised centres as not being satisfactory. However, objective data supporting this assumption are lacking.
The aim of this study, therefore, was to assess current management of AC with a special focus on the time needed for initiation of parenteral glucocorticoid treatment in a well characterised cohort of patients with chronic AI and to compare the results with expert opinion on optimal time targets and time limits in AC.
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