Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature

Tiemensma, Jitske; Andela, Cornelie D.; Kaptein, Ad A.; Romijn, Johannes A.; Mast, Roos C. van der; Biermasz, Nienke R.; Pereira, Alberto M.

doi:10.1530/eje-14-0023

Cited by 60 publications

(42 citation statements)

References 58 publications

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“…The impact of AI on affected patients is high and impairment of quality of life is a frequent observation [17][18][19]. Our data confirm that physicians are still the main source of information for AI patients [10], despite the widespread availability of professional and semi-professional sources in the internet era.…”

Section: Discussionsupporting

confidence: 70%

Self-management in adrenal insufficiency — towards a better understanding

et al. 2017

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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

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Section: Discussionsupporting

confidence: 70%

Self-management in adrenal insufficiency — towards a better understanding

et al. 2017

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“…Patients on replacement therapy reproducibly selfreport impairment in particular dimensions of general well-being questionnaires such as SF-36 (8,29,30,31,32,33,34,35,36). General health and vitality perception are low for all patient subgroups, although the reductions are particularly pronounced for patients with concomitant type-1 diabetes (T1D).…”

Section: Health-related Quality Of Lifementioning

confidence: 99%

“…General health and vitality perception are low for all patient subgroups, although the reductions are particularly pronounced for patients with concomitant type-1 diabetes (T1D). The quality of life (QoL) impairments have further been associated with female gender, increasing glucocorticoid dose, maladaptive personality traits, lower education and membership in patients' organisations (34,35,37). One registry study indicated increased prevalence of affective disorders associated with PAI (38), and a cross-sectional study including 54 patients with PAI reported more irritability and somatic arousal (34).…”

Section: Health-related Quality Of Lifementioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Epidemiology, quality of life and complications of primary adrenal insufficiency: a review

Bensing

Hulting

Husebye

et al. 2016

European Journal of Endocrinology

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In this article, we review published studies covering epidemiology, natural course and mortality in primary adrenal insufficiency (PAI) or Addison's disease. Autoimmune PAI is a rare disease with a prevalence of 100-220 per million inhabitants. It occurs as part of an autoimmune polyendocrine syndrome in more than half of the cases. The patients experience impaired quality of life, reduced parity and increased risk of preterm delivery. Following a conventional glucocorticoid replacement regimen leads to a reduction in bone mineral density and an increase in the prevalence of fractures. Registry studies indicate increased mortality, especially evident in patients diagnosed with PAI at a young age and in patients with the rare disease autoimmune polyendocrine syndrome type-1. Most notably, unnecessary deaths still occur because of adrenal crises. All these data imply the need to improve the therapy and care of patients with PAI.

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“…This initial tremendous improvement in well-being has deceived expert physicians for some decades and led them to believe that treated patients with adrenal insufficiency lead a largely normal life with a normal life expectancy (1,2,3). Only in recent years, it became evident that restoration of well-being remains incomplete (4,5,6,7,8,9). The reasons for this incomplete recovery of well-being with current replacement regimens are still not fully understood and comprise among others a non-physiological glucocorticoid replacement with an altered diurnal rhythm of cortisol availability (10,11,12,13,14), lack of DHEA (15,16,17) and reduced adrenaline secretion from the adrenal medulla (18,19).…”

Section: Introductionmentioning

confidence: 99%

EXTENSIVE EXPERTISE IN ENDOCRINOLOGY: Adrenal crisis

Allolio

2015

European Journal of Endocrinology

184

230

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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.

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Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature

Cited by 60 publications

References 58 publications

Self-management in adrenal insufficiency — towards a better understanding

Self-management in adrenal insufficiency — towards a better understanding

MANAGEMENT OF ENDOCRINE DISEASE: Epidemiology, quality of life and complications of primary adrenal insufficiency: a review

EXTENSIVE EXPERTISE IN ENDOCRINOLOGY: Adrenal crisis

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Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature

Cited by 60 publications

References 58 publications

Self-management in adrenal insufficiency &mdash; towards a better understanding

Self-management in adrenal insufficiency &mdash; towards a better understanding

MANAGEMENT OF ENDOCRINE DISEASE: Epidemiology, quality of life and complications of primary adrenal insufficiency: a review

EXTENSIVE EXPERTISE IN ENDOCRINOLOGY: Adrenal crisis

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Self-management in adrenal insufficiency — towards a better understanding

Self-management in adrenal insufficiency — towards a better understanding