European Journal of Internal Medicine 26 (2015) 75–76
Contents lists available at ScienceDirect
European Journal of Internal Medicine journal homepage: www.elsevier.com/locate/ejim
Letter to the Editor A European Emergency Card for adrenal insufficiency can save lives Keywords: Addison's disease Adrenal insufficiency Medical alert card
Adrenal insufficiency (AI) is a life threatening disease that has several underlying causes. In primary AI (PAI) the adrenal cortex itself is destroyed and the most common cause is autoimmune adrenalitis resulting in impaired synthesis and release of cortisol and aldosterone. A failure of the pituitary or hypothalamic control centers lead to secondary (SAI) and tertiary AI, respectively, manifested clinically as isolated cortisol insufficiency. Typical symptoms of lack of cortisol are tiredness, weakness, loss of energy, weight loss, nausea, vomiting, abdominal pain and muscle and joint pain. These symptoms are non-specific and therefore AI patients are often misdiagnosed [1]. Hyperpigmentation and salt craving are typical symptoms and signs in PAI.
Once diagnosed, a life-long, daily medical treatment with glucocorticoids is essential for patients with AI. Hydrocortisone (HC) or cortisone acetate (CA) are the drugs of choice [2,3]. Due to their short half-life, these are often administered in two to four divided daily doses, totaling 15–25 mg HC or 25–37.5 mg CA per day. However, these amounts do not suffice in situations of stress and illnesses. Therefore, AI patients are trained to double, triple or even quadruple glucocorticoid doses in cases of severe physical stress such as infections with high fever, to avoid an adrenal crisis. Vomiting and diarrhea are particularly serious, since administered drugs are insufficiently absorbed. In these situations a life-threatening adrenal crisis can develop within a few hours [4] even in the event of a mild stomach upset. Adrenal crisis occur with a frequency of 6–14 per 100 patient years [4,5]. Typical clinical features are extreme weakness, nausea, and abdominal pain; eventually leading to hypotension, shock and death. Adrenal crisis is most probably the major cause of increased mortality in patients with PAI [6,7] and SAI [8]. Intravenous hydrocortisone (100 mg) and saline must be given without delay, followed by intermittent hydrocortisone (200–400 mg per 24 h) and i.v. saline infusion.
Fig. 1. Original Swedish Emergency Card [10].
http://dx.doi.org/10.1016/j.ejim.2014.11.006 0953-6205/© 2014 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
76
Letter to the Editor
Survival of patients presenting with acute adrenal crisis depends on prompt replacement treatment. Unfortunately, acute treatment is often delayed despite patient education and availability of a plethora of cards and leaflets. A common European Emergency Card could overcome the current short-comings of multiple national cards. This card should be designed solely to provide quick and easy information principally to the healthcare worker in an emergency situation [9]. An independent panel of European endocrinologists reviewed all available national emergency cards and information leaflets across Europe [9]. The Swedish Emergency Card [10] (Fig. 1) was felt to fulfill the purpose of a European Emergency Card. The red and white two-sided plastic card in credit card size provides information in English and the national language (Supplemental Fig. 1). The endocrine societies of Norway, Sweden, Germany, and Italy have already implemented and endorsed the card, and others (e.g. UK, Finland and Spain) are in the process of implementation (Supplemental Fig. 2). Separate cards for children are also available. There is also an ongoing work in the European Society of Endocrinology inviting all European countries to gather around the common European card. The message of the card is clear for all to see: Treat immediately an AI patient in an emergency situation with intravenous hydrocortisone and saline independently of the underlying cause! We hereby encourage all national endocrine societies in Europe to make national versions of the European Emergency Card for Adrenal Insufficiency. We are convinced that the card will improve treatment and save lives of AI patients. Conflict of interests Nothing to disclose. Acknowledgments The board members of the Swedish Addison Registry. Appendix A. Supplementary data Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ejim.2014.11.006. References [1] Bleicken B, Hahner S, Ventz M, Quinkler M. Delayed diagnosis of adrenal insufficiency is common: a cross-sectional study in 216 patients. Am J Med Sci 2010 Jun;339(6): 525–31.
[2] Quinkler M, Hahner S. What is the best long-term management strategy for patients with primary adrenal insufficiency? Clin Endocrinol (Oxf) 2012 Jan;76(1):21–5. [3] Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med 2014 Feb;275(2):104–15. [4] Hahner S, Loeffler M, Bleickenx B, Drechsler C, Milovanovic D, Fassnacht M, et al. Epidemiology of adrenal crisis in chronic adrenal insufficiency — the need for new prevention strategies. Eur J Endocrinol 2010;162(3):597–602. [5] Meyer G, Neumann K, Badenhoop K, Linder R. Increasing prevalence of Addison's disease in German females: health insurance data 2008–2012. Eur J Endocrinol 2014 Feb 4;170(3):367–73. [6] Bergthorsdottir R, Leonsson-Zachrisson M, Oden A, Johannsson G. Premature mortality in patients with Addison's disease: a population-based study. J Clin Endocrinol Metab 2006 Dec;91(12):4849–53. [7] Bensing S, Brandt L, Tabaroj F, Sjöberg O, Nilsson B, Ekbom A, et al. Increased death risk and altered cancer incidence pattern in patients with isolated or combined autoimmune primary adrenocortical insufficiency. Clin Endocrinol (Oxf) 2008;69(5): 697–704. [8] Burman P, Mattsson AF, Johannsson G, Höybye C, Holmer H, Dahlqvist P, et al. Deaths among adult patients with hypopituitarism: hypocortisolism during acute stress, and de novo malignant brain tumors contribute to an increased mortality. J Clin Endocrinol Metab 2013 Apr;98(4):1466–75. [9] Quinkler M, Hahner S, Johannsson G, Stewart PM. Saving lives of patients with adrenal insufficiency: a pan-European initiative? Clin Endocrinol (Oxf) 2014 Mar; 80(3):319–21. [10] Dahlqvist P, Bensing S, Ekwall O, Wahlberg J, Bergthorsdottir R, Hulting AL. A national medical emergency card for adrenal insufficiency. A new warning card for better management and patient safety. Lakartidningen 2011 Nov 2;108(44):2226–7.
Marcus Quinkler Endocrinology in Charlottenburg, Berlin, Germany Per Dahlqvist Department of Public Health and Clinical Medicine, University of Umeå, Umeå, Sweden Eystein S. Husebye Department of Clinical Science, University of Bergen, Bergen, Norway Department of Medicine, Haukeland University Hospital, Bergen, Norway
Olle Kämpe Department of Medicine (Solna), Karolinska Institutet, Stockholm, Sweden Corresponding author. Tel.: +46 8 524 800 00, +46 708 15 14 00; fax: +46 8 517 730 96. E-mail address: [email protected].
18 November 2014
Contents lists available at ScienceDirect
European Journal of Internal Medicine journal homepage: www.elsevier.com/locate/ejim
Letter to the Editor A European Emergency Card for adrenal insufficiency can save lives Keywords: Addison's disease Adrenal insufficiency Medical alert card
Adrenal insufficiency (AI) is a life threatening disease that has several underlying causes. In primary AI (PAI) the adrenal cortex itself is destroyed and the most common cause is autoimmune adrenalitis resulting in impaired synthesis and release of cortisol and aldosterone. A failure of the pituitary or hypothalamic control centers lead to secondary (SAI) and tertiary AI, respectively, manifested clinically as isolated cortisol insufficiency. Typical symptoms of lack of cortisol are tiredness, weakness, loss of energy, weight loss, nausea, vomiting, abdominal pain and muscle and joint pain. These symptoms are non-specific and therefore AI patients are often misdiagnosed [1]. Hyperpigmentation and salt craving are typical symptoms and signs in PAI.
Once diagnosed, a life-long, daily medical treatment with glucocorticoids is essential for patients with AI. Hydrocortisone (HC) or cortisone acetate (CA) are the drugs of choice [2,3]. Due to their short half-life, these are often administered in two to four divided daily doses, totaling 15–25 mg HC or 25–37.5 mg CA per day. However, these amounts do not suffice in situations of stress and illnesses. Therefore, AI patients are trained to double, triple or even quadruple glucocorticoid doses in cases of severe physical stress such as infections with high fever, to avoid an adrenal crisis. Vomiting and diarrhea are particularly serious, since administered drugs are insufficiently absorbed. In these situations a life-threatening adrenal crisis can develop within a few hours [4] even in the event of a mild stomach upset. Adrenal crisis occur with a frequency of 6–14 per 100 patient years [4,5]. Typical clinical features are extreme weakness, nausea, and abdominal pain; eventually leading to hypotension, shock and death. Adrenal crisis is most probably the major cause of increased mortality in patients with PAI [6,7] and SAI [8]. Intravenous hydrocortisone (100 mg) and saline must be given without delay, followed by intermittent hydrocortisone (200–400 mg per 24 h) and i.v. saline infusion.
Fig. 1. Original Swedish Emergency Card [10].
http://dx.doi.org/10.1016/j.ejim.2014.11.006 0953-6205/© 2014 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
76
Letter to the Editor
Survival of patients presenting with acute adrenal crisis depends on prompt replacement treatment. Unfortunately, acute treatment is often delayed despite patient education and availability of a plethora of cards and leaflets. A common European Emergency Card could overcome the current short-comings of multiple national cards. This card should be designed solely to provide quick and easy information principally to the healthcare worker in an emergency situation [9]. An independent panel of European endocrinologists reviewed all available national emergency cards and information leaflets across Europe [9]. The Swedish Emergency Card [10] (Fig. 1) was felt to fulfill the purpose of a European Emergency Card. The red and white two-sided plastic card in credit card size provides information in English and the national language (Supplemental Fig. 1). The endocrine societies of Norway, Sweden, Germany, and Italy have already implemented and endorsed the card, and others (e.g. UK, Finland and Spain) are in the process of implementation (Supplemental Fig. 2). Separate cards for children are also available. There is also an ongoing work in the European Society of Endocrinology inviting all European countries to gather around the common European card. The message of the card is clear for all to see: Treat immediately an AI patient in an emergency situation with intravenous hydrocortisone and saline independently of the underlying cause! We hereby encourage all national endocrine societies in Europe to make national versions of the European Emergency Card for Adrenal Insufficiency. We are convinced that the card will improve treatment and save lives of AI patients. Conflict of interests Nothing to disclose. Acknowledgments The board members of the Swedish Addison Registry. Appendix A. Supplementary data Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ejim.2014.11.006. References [1] Bleicken B, Hahner S, Ventz M, Quinkler M. Delayed diagnosis of adrenal insufficiency is common: a cross-sectional study in 216 patients. Am J Med Sci 2010 Jun;339(6): 525–31.
[2] Quinkler M, Hahner S. What is the best long-term management strategy for patients with primary adrenal insufficiency? Clin Endocrinol (Oxf) 2012 Jan;76(1):21–5. [3] Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med 2014 Feb;275(2):104–15. [4] Hahner S, Loeffler M, Bleickenx B, Drechsler C, Milovanovic D, Fassnacht M, et al. Epidemiology of adrenal crisis in chronic adrenal insufficiency — the need for new prevention strategies. Eur J Endocrinol 2010;162(3):597–602. [5] Meyer G, Neumann K, Badenhoop K, Linder R. Increasing prevalence of Addison's disease in German females: health insurance data 2008–2012. Eur J Endocrinol 2014 Feb 4;170(3):367–73. [6] Bergthorsdottir R, Leonsson-Zachrisson M, Oden A, Johannsson G. Premature mortality in patients with Addison's disease: a population-based study. J Clin Endocrinol Metab 2006 Dec;91(12):4849–53. [7] Bensing S, Brandt L, Tabaroj F, Sjöberg O, Nilsson B, Ekbom A, et al. Increased death risk and altered cancer incidence pattern in patients with isolated or combined autoimmune primary adrenocortical insufficiency. Clin Endocrinol (Oxf) 2008;69(5): 697–704. [8] Burman P, Mattsson AF, Johannsson G, Höybye C, Holmer H, Dahlqvist P, et al. Deaths among adult patients with hypopituitarism: hypocortisolism during acute stress, and de novo malignant brain tumors contribute to an increased mortality. J Clin Endocrinol Metab 2013 Apr;98(4):1466–75. [9] Quinkler M, Hahner S, Johannsson G, Stewart PM. Saving lives of patients with adrenal insufficiency: a pan-European initiative? Clin Endocrinol (Oxf) 2014 Mar; 80(3):319–21. [10] Dahlqvist P, Bensing S, Ekwall O, Wahlberg J, Bergthorsdottir R, Hulting AL. A national medical emergency card for adrenal insufficiency. A new warning card for better management and patient safety. Lakartidningen 2011 Nov 2;108(44):2226–7.
Marcus Quinkler Endocrinology in Charlottenburg, Berlin, Germany Per Dahlqvist Department of Public Health and Clinical Medicine, University of Umeå, Umeå, Sweden Eystein S. Husebye Department of Clinical Science, University of Bergen, Bergen, Norway Department of Medicine, Haukeland University Hospital, Bergen, Norway
Olle Kämpe Department of Medicine (Solna), Karolinska Institutet, Stockholm, Sweden Corresponding author. Tel.: +46 8 524 800 00, +46 708 15 14 00; fax: +46 8 517 730 96. E-mail address: [email protected].
18 November 2014
