Barriers to stress dosing in acute Addison disease

[Correction Notice: this article has been updated to exclude amendments made by the editors to the quotes provided by Professor Louise Rushworth, and to remove the term “steroid phobia” which was added by the editor. We apologise to Professor Rushworth and to readers for the inaccuracies.]

Adrenal crises are rare events but they continue to occur in part because patients with primary adrenal insufficiency are not stress dosing during episodes of acute illness.

An audit of acute Addison disease in the Hunter New England area of NSW identified 252 hospital attendances by 56 patients during the period from 2000 to 2017.

Infection was the principal diagnosis in nearly half of patients (45.2%), followed by adrenal insufficiency (34.1%), gastroenteritis (12.3%) and trauma (7.5%). Nearly half of the patients (48.4%) arrived at the hospital by ambulance.

Common comorbidities included thyroid disease (52%) and type 2 diabetes (32.5%).

Two-thirds of patients (66.1%) were found to have never used any kind of stress dosing prior to any attendance at the study hospital.

“Of those patients who had stress dosed, 11 (19.6%) had one recorded attempt at dose escalation, 5 (8.9%) had a record of dose escalation at 2 presentations; and there was 1 patient (1.8%) for each of 3, 6 and 16 separate presentations in which there was stress dosing before arrival,” the study said.

Although vomiting was a common presenting symptom, oral stress dosing was the most  common route of self-administration (15.9%) compared to intramuscular hydrocortisone administration (2.8%). There was no record of subcutaneous hydrocortisone administration.

Almost all patients with clinician-diagnosed adrenal crisis (93.7%) were administered IV hydrocortisone.

“When all attendances at hospital by these patients were considered, oral stress dosing was used in fewer than one in five presentations and in only 2.8% of the total attendances did a patient use IM hydrocortisone prior to arrival,” the study said

Senior author on the paper Professor Louise Rushworth told the limbic the barriers to patient-initiated stress dosing were not well understood but the lack of action explained to some extent why adrenal crises continue to occur.

The study, published in the Journal of the Endocrine Society, found younger patients, those with vomiting and those with more frequent hospital presentations were more likely to self administer hydrocortisone.

Professor Rushworth, from the University of Notre Dame’s School of Medicine, said older patients with multiple comorbidities might find it harder to recognise that they were becoming ill or enact dose escalation.

“If you are young, otherwise well and only have one problem, it can be easier to identify an acute illness and act on it than if you have a number of comorbidities, such as heart disease, diabetes, adrenal failure, and you are feeling unwell,” she said.

The study also noted that there may be some confusion regarding the diagnosis of adrenal crisis.

“It is a rare event and many doctors would not have managed a patient with adrenal insufficiency.  Therefore they may not be aware of the difference between symptomatic adrenal insufficiency and an adrenal crisis.”

“There can be confusion about giving glucocorticoids to patients. As doctors we have been strongly educated about the dangers of glucocorticoids and so some practitioners may be reluctant to give hydrocortisone.  However, patients with adrenal insufficiency must be given hydrocortisone urgently to prevent or treat an adrenal crisis.”

She reinforced that patients with adrenal insufficiency who were ill had a definite need.

“It is vital.  An adrenal crisis must be treated immediately with IV hydrocortisone.”

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