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Rapid Responses: Rapid Responses published:
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![[Read Rapid Response]](/icons/misc/sectionDOWN.gif)
Addison's disease: failure to diagnose can be fatal
- Katherine G White, Les Christian, Nick Willson (trustees) (24 July 2009)
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Adrenal Disease - Personal Experience
- Gabriel Antony (24 July 2009)
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Authors Response
- Ali J Chakera, Bijay Vaidya, Catherine Dick (18 August 2009)
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Katherine G White, Chair Addison's Disease Self-Help Group, Les Christian, Nick Willson (trustees)
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Our thanks to Vaidya, Chakera and Dick for their concise and balanced overview of the complexities of diagnosing Addison’s disease and for their important reminder that failure to diagnose can be fatal. Death from undiagnosed or under-treated adrenal failure is now thankfully rare, but still occurs each year in a few tragic cases. The Office of National Statistics records that an average of 30 people died from adrenal failure every year from 2001 to 2007. Four per cent of these deaths were in children and teenagers, 9% in adults aged 20 – 39, 12% in adults aged 40 – 59, 14% in adults in their 60s, 23% in adults in their 70s, and 38% in adults aged 80 or over. As the case scenario offered by Vaidya, Chakera and Dick illustrates, most adult patients with adrenal failure experience a lengthy and debilitating illness before they are diagnosed. Many will have their symptoms attributed to other illnesses, including psychiatric disorders. Almost all will make repeated visits to their GP with a variety of non- specific symptoms, in which overwhelming fatigue is the common denominator. So most deaths from undiagnosed adrenal failure could, arguably, be avoided through greater medical awareness of this rare condition. To assist GPs in the early detection of adrenal failure, the Addison’s Disease Self-Help Group’s (ADSHG) Clinical Advisory Panel has recently published a free guide 'Diagnosing Addison's: a guide for GPs'. It describes the investigations that can be readily conducted in primary care, and lists those symptoms which should act as a warning sign that urgent referral to an endocrine specialist is advisable. You can read these GP guidelines on the ADSHG website at www.addisons.org.uk along with other free guides for GPs, surgeons, paramedics and other medical practitioners. Competing interests: None declared |
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Gabriel Antony, Endocrinologist South Coogee 2034 New South Wales Australia
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VAIDYA el al state that "a short synachen test is the investigation of choice to confirm or exclude Addison's Disease" and that "random measurement of serum cortisol is inadequate". By the time a patient with Addison's Disease comes to clinical attention, as is detailed in the article, the condition has likely existed for quite some time. As a result, the ACTH concentration will be elevated at all times and disproportionately so to the cortisol concentration. A paired random measurement of the two, in my view, is the investigation of choice. By definition, the "pulsatile nature and diurnal variation of cortisol secretion" are irrelevant in this situation. The amount of useless material printed again and yet again over many years on the choice and interpretation of so-called "adrenal-stimulation tests" (stimulation of the adrenal axis and testing of the adrenal reserve, usually being lumped together under this term) makes me, for one, avoid them and make the documenting the actual parameters of the "adrenal system", as suggested above, my first choice. Leave the "stimulation" to the feedback = "nature"! Regarding treatment, in the companion article (2), in my view, a disconcertingly brief and only passing mention is made under a "doctor's perspective" of the need to have an "emergency injection kit of hydrocortisone at home". (While the ancient and venerable - and potentially lethal - advice is faithfully recited in the other paper to "double or triple the replacement dose of hydrocortisone"(1)). It does take a great deal of time and convincing a patient to accept the vital necessity to learn to give themselves an injection and even more to do so, but the licence to try to avoid it, as given to them in this advice, can cost them their lives. By the time they recognise that this advice did not work, it can be too late! "Mixed messages" and "convenience medicine" (i.e: "pill-popping" vs giving oneself an injection) do kill patients! People usually choose the "soft option", regardless of the consequences. While one person's clinical experience is, of necessity, limited, the above comments are a "doctor's perspective" and current personal practice, as it evolved, who has treated patients with adrenal diseases over forty years. References: 1. Vaidya, B, Chakera, AJ, Dick C. Easily Missed? Addison's Disease. BMJ 2009; 339:104. 2. Baker, SJK, Wass, JAH. A patient's journey Addison's Disease. BMJ 2009; 339:106. Competing interests: endocrinology |
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Ali J Chakera, Specialist Registrar, Diabetes and Endocrinology Department of Endocrinology, Royal Devon and Exeter Hospital, Exeter, EX2 5DW, Bijay Vaidya, Catherine Dick
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In a rapid response to our paper, Gabriel Antony comments that a paired measurement of random serum cortisol and plasma adrenocorticotrophin (ACTH), and not the short synacthen test, is the investigation of choice for the diagnosis of Addison’s disease (1). Although we agree that a low random cortisol together with a raised ACTH level suggests the diagnosis, we would still advocate a short synacthen test to diagnose hypoadrenalism. There are several reasons why: • The short synacthen test has been extensively studied in both healthy individuals as well as in patients with primary and secondary hypoadrenalism, which have allowed to derive validated cut-off levels for normal response (2). In contrast, there are no validated cut-off levels are for a paired random cortisol and ACTH. Extreme cases of low cortisol and high ACTH may be diagnostic, but more subtle changes may lead to confusion. Accepting that the symptoms of Addison’s disease are non- specific, it is important to have clear diagnosis before committing a patient to lifelong glucocorticoid replacement. • A short-synacthen test is a quick, easy test. Analysing an ACTH level is more complicated – requiring the sample to be taken immediately (on ice) to the laboratory and having a much longer turn-around time for the results. As well as practical difficulties in performing the test in primary care, it may lead to an inappropriate delay in diagnosis and thus treatment. • A low random cortisol with a normal ACTH could represent secondary hypoadrenalism. A patient or clinician could thus be falsely reassured that these tests are normal. Regarding the question of glucocorticoid replacement at times of physiological stress in patients with Addison’s disease, treatment strategy should vary according to the nature and severity of the illness. We agree that it is important for patients with Addison’s disease and their next of kin to learn when and how to inject intramuscular hydrocortisone as timely parenteral administration of hydrocortisone at the time of a serious illness particularly if the patient is unable to tolerate oral medication could be life-saving. However, there is no justification to recommend hydrocortisone injections during every minor illnesses or stress. The optimum balance can be achieved through patient education. References: 1. Vaidya B, Chakera AJ, Dick C. Easily Missed? Addison’s disease. BMJ 2009; 339:b2385 2. Dorin RI, Qualls CR, Crapo LM. Diagnosis of Adrenal Insufficiency. Annals of Internal Medicine. 2003; 139(3):194-204 Competing interests: None declared |
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