scholarly journals MON-160 The Burdens of Adrenal Insufficiency: A Survey Study from Two Tertiary Care Centers in the United States

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Natalia Genere ◽  
Dingfeng Li ◽  
Majlinda Xhikola ◽  
Emma Behnken ◽  
Tiffany Abbondanza ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Burden Of Disease ◽  
General Health ◽  
Well Being ◽  
The United States ◽  
Comfort Level ◽  
Survey Study ◽  
Adrenal Crisis ◽  
Glucocorticoid Replacement Therapy

Abstract Background: Adrenal insufficiency (AI) is a chronic disorder necessitating life-long replacement. Patients’ quality of life and health outcomes depend on knowledge and comfort level with self-management. Objective: To determine patients’ knowledge in regards to diagnosis and management, estimate burden of disease and to identify predictors of adverse outcomes in patients with AI Methods: Survey study of patients with AI evaluated at two tertiary medical centers between 2015 and 2019. Collected variables included data on circumstances of AI diagnosis, symptoms, management, burden of disease, and overall well-being. Results: Among 785 patients (mean age at diagnosis 44.2 ± 18.0, 64% women, and 92% Caucasian), 310 (40%) had primary AI (PAI), 255 (33%) had secondary AI (SAI) not related to glucocorticoid use, and 211 (27%) had steroid-induced AI (SIAI). Patients were diagnosed with AI after presenting with symptoms for a median of 1 year (0-6), 28% with symptoms lasting >2 years, 44% visiting emergency room (ER) at least once prior to diagnosis. A third of patients reported a discordant diagnosis from their medical record. Baseline glucocorticoid replacement therapy included hydrocortisone (HC) in 447 (59%), median of 20 mg (IQR 15 - 25mg), prednisone in 190 (25%), median of 5 mg (IQR 4 - 7.5mg), other regimens in 38 (5%), and no steroids in the remainder (85, 11%); 197 (26%) patients reported daily equivalent HC dose of >25 mg. Overall, 549 (73%) of patients reported use of stress dose steroids at least once per year, higher in patients taking HC >25 mg/day (3.2 vs 2.7 times per year if HC<25mg/day, p=0.01). Improper use of stress steroids was reported in 193, 25% patients. Patients taking HC>25 mg /day reported a higher number of adrenal crises (1.6 vs 1.3 in patients on HC<25 mg/day, p=0.04). Among 314 (41%) patients who reported ER visits due to adrenal crisis, only a third received prompt glucocorticoids. One third of patients described their general health as fair or poor. Predictors of negative perceptions of overall health included SIAI (OR 6.2 and 2.5, vs PAI and SAI respectively), poor understanding of diagnosis (OR 2.6), daily HC>25 mg (OR 2.1), and presence of at least one adrenal crisis (OR 2.3) (p<0.001 for all). Conclusion: Patients with AI experience delay in diagnosis, and a third do not fully understand their diagnosis. In addition to patient education, interventions to improve general health and outcomes may include selecting a physiological glucocorticoid replacement therapy, prevention of adrenal crisis, and improving ER care.

scholarly journals Quality of Life and Its Determinants in Patients With Adrenal Insufficiency: A Survey Study From Three Tertiary Centers in the United States

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A92-A93
Author(s):  
Dingfeng Li ◽  
Malavika Suresh ◽  
Tobias Else ◽  
Sarah Brand ◽  
Oksana Hamidi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Financial Burden ◽  
The United States ◽  
Survey Study ◽  
Current Evidence ◽  
Adrenal Crisis ◽  
The Usa

Abstract Context: Quality of life (QoL) is impaired in patients with chronic adrenal insufficiency (AI) despite standard glucocorticoid (GC) replacement therapy. Current evidence on the determinants of QoL is scarce and limited in very few European countries, and how it relates to AI subtypes remains underexplored. Objective: We conducted the first survey study in patients with AI in the USA to determine the correlations between clinical parameters, adverse outcomes, patient education, socioeconomic factors, and QoL in different subtypes of AI. Design, Setting and Participants: Cross-sectional survey study of 529 patients with AI between 2015 and 2020, at three tertiary centers in the USA. Intervention: Patient-centered questionnaires. Main Outcome Measures: QoL scores using Short-Form 36. Results: Of 529 participants, 223 (42.2%) had primary AI (PAI), 190 (35.9%) had secondary AI (SAI), and 116 (21.9%) had glucocorticoid induced AI (GIAI). Median age at the time of survey was 58 years (IQR: 43–68), 342 (64.8%) were women and 483 (91.3%) were Caucasians. Median duration of AI was 6 years (IQR: 3–14.5), longest in patients with PAI (11 vs 4 years in SAI and GIAI, p=0.0001). Overall, Physical Composite Summary (PCS) score was lower than the Mental Composite Summary (MCS) (38.1±12.9 vs 46.5±11.8). Across the eight dimensions, each individual decade- and sex-adjusted Z-score (using the normative data of USA population) in patients with PAI was significantly higher than that of the other two AI subtypes (all p<0.05), although all Z-scores were still below 0. In multivariate analyses, patients were more likely to report a worse PCS score (<40) if they were women (OR: 3.3, CI 95%: 1.8–6.0), had SAI or GIAI (OR: 2.5, CI 95%: 1.4–4.3), had shorter duration of AI (<6 years) (OR: 2.0, CI 95%: 1.1–3.6), were treated with >25 mg hydrocortisone equivalent daily (OR: 2.3, CI 95%: 1.2–4.6), had more comorbidities related to GC excess (OR: 2.3, CI 95%: 1.3–4.0), reported higher financial burden due to AI (OR: 2.1, CI 95%: 1.3–3.6), and reported difficulties with AI management (OR: 2.5, CI 95%: 1.2–5.2). Women (OR: 2.1, CI 95%: 1.08–4.0), shorter duration of AI (OR: 2.4, CI 95%: 1.4–4.3), higher financial burden due to AI (OR: 2.3, CI 95%: 1.3–4.0), reporting difficulties with AI management (OR: 2.6, CI 95%: 1.4–4.9), and lack of family support during adrenal crisis (OR: 9.1, CI 95%: 2.3–33.3), were predictors of a worse MCS score (<40). Conclusions: Patients with AI have substantially impaired QoL despite GC replacement therapy. Certain determinants of QoL are modifiable and achievable, such as avoiding GC over-replacement, offering detailed hands-on education in self-management, more comprehensive insurance coverage, and more robust domestic support. Our study calls for a multidimensional effort from patients, clinicians, and society to improve QoL in this vulnerable patient population.

scholarly journals MON-164 Determinants of Quality of Life in Primary and Secondary Adrenal Insufficiency from Two Large Tertiary Care Centers in the United States

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Dingfeng Li ◽  
Natalia Genere ◽  
Majlinda Xhikola ◽  
Emma Behnken ◽  
Tiffany Abbondanza ◽  
...  
Keyword(s):  
Quality Of Life ◽  
United States ◽  
Adrenal Insufficiency ◽  
The United States ◽  
Comfort Level ◽  
Adrenal Crisis ◽  
Secondary Adrenal Insufficiency ◽  
Number Of Patients ◽  
Duration Of Disease

Abstract Background: Patients with primary adrenal insufficiency (PAI) and secondary adrenal insufficiency (SAI) report impaired quality of life. Predictors of adverse outcomes include duration of disease, supraphysiologic glucocorticoid (GC) use, and presence of other comorbidities. There is limited evidence to suggest that quality of life is disproportionately affected in one subtype of adrenal insufficiency. Objective: To identify determinants of quality of life in patients with PAI vs SAI. Method: Survey study of patients with the diagnosis of AI between 2015 and 2019 was evaluated at two large tertiary medical centers in the United States. Collected variables included data on circumstances of AI diagnosis, symptoms, management, burden of disease, and overall well-being. Patients with AI due to exogenous glucocorticoid use were excluded. Results: Patients with PAI (n=310, 65% women) were diagnosed at a younger age (37 ± 19 vs 48 ± 16 years, p<.01) than patients with SAI (n=255, 57% women). Patients with SAI were more likely not to understand their diagnosis (13% vs 4% in PAI, p<.01), or to report a discordant diagnosis (16% vs 9% in PAI, p=.02). Patients with PAI were on a higher dose of daily GC (23.8 mg vs 18.5 mg hydrocortisone (HC) equivalent, p <.01), with higher number of patients on HC> 25 mg/day (33% vs 15%, p<.01). Patients with PAI reported a higher compliance with wearing medical alert ID (79% vs 64%, p=.01) and higher availability of injectable GC (74% vs 58%, p<.01). Patients with PAI reported a higher frequency of at least one adrenal crisis within the last 12 months that required self- injectable GC (32% vs 16%, p<.01), or prompted ER visits (56% vs 29%, p<.01) compared to those with SAI. Nevertheless, patients with PAI were more likely to report good general health (78% vs 65% in SAI, p<.01). When adjusted for current age, sex, duration of disease, supraphysiologic GC use, number of current symptoms and recent adrenal crises, patients with SAI had HR of 2.6 (CI 95% 1.5-4.3) for poor health, 1.6 (CI 95% 0.95-2.8) for physical limitations, 1.7 (CI 95% 1.1-2.7) for fatigue, and 2.7 (CI 95% 1.5-4.9) for social limitations compared to those with PAI. Conclusions: Patients with PAI receive higher daily GC and experience a higher number of adrenal crises when compared to patients with SAI. However, patients with PAI report a better general perception of health, possibly due to a more robust knowledge of their disease and higher comfort level managing AI. More effort is needed to ensure patients with AI are appropriately educated regarding their medical condition and therapy.

Adrenal insufficiency: review of clinical outcomes with current glucocorticoid replacement therapy

2016 ◽  
Author(s):  
Gudmundur Johannsson ◽  
Alberto Falorni ◽  
Stanko Skrtic ◽  
Hans Lennernas ◽  
Marcus Quinkler ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Clinical Outcomes ◽  
Glucocorticoid Replacement Therapy

Adrenal insufficiency: review of clinical outcomes with current glucocorticoid replacement therapy

2014 ◽  
Vol 82 (1) ◽  
pp. 2-11 ◽  
Author(s):  
Gudmundur Johannsson ◽  
Alberto Falorni ◽  
Stanko Skrtic ◽  
Hans Lennernäs ◽  
Marcus Quinkler ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Clinical Outcomes ◽  
Glucocorticoid Replacement Therapy

scholarly journals EXTENSIVE EXPERTISE IN ENDOCRINOLOGY: Adrenal crisis

2015 ◽  
Vol 172 (3) ◽  
pp. R115-R124 ◽  
Author(s):  
Bruno Allolio
Keyword(s):  
Patient Education ◽  
Adrenal Insufficiency ◽  
Well Being ◽  
Stressful Events ◽  
Major Surgery ◽  
Adrenal Crisis ◽  
Self Administration ◽  
Necrosis Factor Alpha ◽  
Hydrocortisone Administration ◽  
Glucocorticoid Deficiency

Adrenal crisis is a life-threatening emergency contributing to the excess mortality of patients with adrenal insufficiency. Studies in patients on chronic replacement therapy for adrenal insufficiency have revealed an incidence of 5–10 adrenal crises/100 patient years and suggested a mortality rate from adrenal crisis of 0.5/100 patient years. Patients with adrenal crisis typically present with profoundly impaired well-being, hypotension, nausea and vomiting, and fever responding well to parenteral hydrocortisone administration. Infections are the major precipitating causes of adrenal crisis. Lack of increased cortisol concentrations during infection enhances pro-inflammatory cytokine release and sensitivity to the toxic effects of these cytokines (e.g. tumour necrosis factor alpha). Furthermore, pro-inflammatory cytokines may impair glucocorticoid receptor function aggravating glucocorticoid deficiency. Treatment of adrenal crisis is simple and highly effective consisting of i.v. hydrocortisone (initial bolus of 100 mg followed by 200 mg over 24 h as continuous infusion) and 0.9% saline (1000 ml within the first hour). Prevention of adrenal crisis requires appropriate hydrocortisone dose adjustments to stressful medical procedures (e.g. major surgery) and other stressful events (e.g. infection). Patient education is a key for such dose adjustments but current education concepts are not sufficiently effective. Thus, improved education strategies are needed. Every patient should carry an emergency card and should be provided with an emergency kit for parenteral hydrocortisone self-administration. A hydrocortisone pen would hold a great potential to lower the current barriers to hydrocortisone self-injection. Improved patient education and measures to facilitate parenteral hydrocortisone self-administration in impending crisis are expected to significantly reduce morbidity and mortality from adrenal crisis.

scholarly journals Therapeutic Patient Education for Adrenal Insufficiency under COVID-19 Pandemic Conditions

2020 ◽  
Author(s):  
Tina Kienitz ◽  
Stefanie Hahner ◽  
Stephanie Burger-Stritt ◽  
Marcus Quinkler
Keyword(s):  
Patient Education ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Medical Therapy ◽  
Medical Personnel ◽  
Daily Basis ◽  
Background Information ◽  
Therapeutic Patient Education ◽  
Dose Adaptation ◽  
Glucocorticoid Replacement Therapy

AbstractAdrenal insufficiency (AI) requires life-long treatment with glucocorticoid replacement therapy. Over- or under-substitution carries the risk of increased morbidity in the form of side effects or adrenal crises. Glucocorticoid replacement therapy needs to be flexible with dose adaptation in special situations. This could not be managed by medical personnel on a daily basis, but requires an educated patient who has a good knowledge of the disease, understands his medical therapy and is able to perform situational dose adaptation. The rarity of the disease in combination with the need to respond to stressful situations with rapid glucocorticoid dose adjustment underlines that a well-trained patient is crucial for optimal management of the disease.In this literature review we provide background information further clarifying the need of education in patients with AI including the current shortcomings of medical therapy and of the treatment of patients with AI. We outline the aims of therapeutic patient education, present the concept of structured patient education in Germany, and discuss available results of patient group education programs. Furthermore, we propose strategies how therapeutic patient education for adrenal insufficiency can be organized under COVID-19 pandemic conditions.

scholarly journals Determinants of Self-reported Health Outcomes in Adrenal Insufficiency: A Multisite Survey Study

2020 ◽  
Author(s):  
Dingfeng Li ◽  
Natalia Genere ◽  
Emma Behnken ◽  
Majlinda Xhikola ◽  
Tiffany Abbondanza ◽  
...  
Keyword(s):  
Health Outcomes ◽  
Adrenal Insufficiency ◽  
Perceived Health ◽  
Adverse Outcomes ◽  
Survey Study ◽  
Self Management ◽  
Current Evidence ◽  
Adrenal Crisis ◽  
Cross Sectional Survey ◽  
Prompt Treatment

Abstract Context Current evidence on determinants of adverse health outcomes in patients with adrenal insufficiency (AI) is scarce, especially in regards to AI subtypes. Objective To determine predictors of adverse outcomes in different subtypes of AI. Design and Setting Cross-sectional survey study at 2 tertiary centers. Participants A total of 696 patients with AI: primary AI (PAI, 42%), secondary AI (SAI, 32%), and glucocorticoid-induced AI (GIAI, 26%). Intervention Patient-centered questionnaire. Main Outcome Measures Patients’ knowledge, self-management of AI, self-perceived health, and adverse outcomes. Results The incidence rate of adrenal crisis was 24/100 patient-years with 44% experiencing at least 1 adrenal crisis since diagnosis (59% in PAI vs 31% in SAI vs 37% in GIAI, P < .0001). All patients described high degrees of discomfort with self-management and receiving prompt treatment. Patients with PAI were most likely to develop adrenal crises (adjusted OR 2.8, 95% CI 1.9-4.0) despite reporting better self-perceived health (adjusted OR 3.3, 95% CI 2.1-5.3), understanding of their diagnosis (89% vs 74-81% in other subtypes, P = .002), higher comfort with self-management (62% vs 52-61% in other sub types, P = .005), and higher likelihood to receive prompt treatment for adrenal crises in the emergency department (42% vs 19-30% in other subtypes, P < .0001). Conclusions Patients with AI reported high degrees of discomfort with self-management and treatment delays when presenting with adrenal crises. Despite better self-perceived health and understanding of diagnosis, patients with PAI experienced the highest frequency of adrenal crises. A multidimensional educational effort is needed for patients and providers to improve the outcomes of all subtypes of AI.

scholarly journals Steroid withdrawal syndrome after successful treatment of Cushing’s syndrome: a reminder

2005 ◽  
Vol 153 (2) ◽  
pp. 207-210 ◽  
Author(s):  
A Bhattacharyya ◽  
K Kaushal ◽  
D J Tymms ◽  
J R E Davis
Keyword(s):  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Cushing’S Syndrome ◽  
Withdrawal Syndrome ◽  
Pituitary Surgery ◽  
Cushing's Syndrome ◽  
Temporary Increase ◽  
Steroid Withdrawal ◽  
Glucocorticoid Replacement Therapy ◽  
Cortisol Levels

Steroid withdrawal syndrome (SWS) usually refers to relapse of the disease being treated after withdrawal of glucocorticoid therapy, or the symptoms of adrenal insufficiency which occur when glucocorticoids are rapidly reduced or stopped. A less well-recognised form of SWS is that which develops when patients experience a symptom complex similar to that of adrenal insufficiency despite acceptable cortisol levels. We describe three patients who presented with this form of SWS following surgical treatment for endogenous Cushing’s syndrome. All responded well to a short-term increase in the dose of glucocorticoid replacement therapy, with the median duration of the syndrome being 10 months (range 6–10 months). Trough serum cortisol levels above 100 nmol/l, with peaks between 460 and 750 nmol/l were documented in the first two patients at presentation with SWS. It is thought that the syndrome may result from development of tolerance to glucocorticoids, and mediators considered to be important in its development include interleukin-6, corticotrophin-releasing hormone, vasopressin, and central noradrenergic and dopaminergic systems. The exact underlying mechanism for SWS remains unclear. However, with increasing recommendations for use of lower doses of replacement glucocorticoids, its incidence may increase. Physicians need to be aware of this condition, which is self-limiting and easily treated by a temporary increase in the dose of glucocorticoid replacement therapy. It is possible that a slower glucocorticoid tapering regimen than that used in the standard postoperative management of patients undergoing pituitary surgery may reduce the risk of development of SWS.

scholarly journals Isolated Adrenocorticotropin Deficiency Associated with Delirium and Takotsubo Cardiomyopathy

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Masanori Murakami ◽  
Noriko Matsushita ◽  
Rie Arai ◽  
Naohiro Takahashi ◽  
Ryuki Kawamura ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Takotsubo Cardiomyopathy ◽  
Mental State ◽  
Adrenocorticotropic Hormone ◽  
Myocardial Dysfunction ◽  
Laboratory Data ◽  
Adrenal Crisis ◽  
Isolated Acth Deficiency ◽  
Acth Deficiency

We report a 65-year-old woman with isolated adrenocorticotropic hormone (ACTH) deficiency. The patient was transported to the emergency outpatient department by ambulance complaining of malaise and nausea. Because her laboratory data revealed hyponatremia, we performed endocrinological examinations and diagnosed isolated ACTH deficiency. After admission, she went into a delirious state and suffered from takotsubo cardiomyopathy due to adrenal insufficiency. Replacement therapy with hydrocortisone sufficiently improved her delirium and cardiomyopathy. We conclude that her unstable mental state and myocardial dysfunction were closely related to adrenal insufficiency and suggest that adrenal crisis may cause delirium and Takotsubo cardiomyopathy.

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