Steroid-dependent patients have a lifelong dependency on replacement therapy and a lifelong vulnerability to sudden death from under treated adrenal crisis. Yet evidence about the adequacy of medical response to adrenal crisis within the UK is largely anecdotal.
We invited members of the main UK support groups representing steroid-dependent patients to complete an online questionnaire identifying the frequency, causes and location of their adrenal crises (episodes needing injected steroids and/or IV fluids). Respondents were asked to describe the nature and timeliness of their medical treatment and to provide demographic information that explored predisposing factors. 1042 patients belonging to the UK support groups for pituitary conditions, Addisons, endocrine cancer and congenital adrenal hypoplasia gave responses.
Patients with comorbidities reported more frequent crisis episodes. Asthma and diabetes were the co-morbidities that acted to destabilize steroid-dependence most strongly. Those patients whose fluid balance is medication-dependent primary adrenal insufficiency and diabetes insipidus were less stable and more vulnerable to adrenal crisis, than those with secondary adrenal insufficiency and intact fluid homeostasis.
Vomiting was the overwhelming factor triggering crisis episodes, reported in around 80% of cases. The most common location, at 70%, was the home. 9% reported they were already a hospital inpatient and their adrenal crisis was iatrogenic that is, triggered by insufficient steroid medication during surgical recovery or post-labour.
For their most recent crisis, over one-third either gave themselves an initial injection of 100 mg hydrocortisone IM, or received this from a partner, parent, child, friend or neighbour. Two-thirds of all respondents were happy with the quality of the medical treatment they received for their most recent adrenal emergency. The largest factor influencing satisfaction levels was the timeliness of the medical response. Less than two-thirds thought they had received prompt medical treatment.
Good patient education and readiness to self-treat remain important for the steroid-dependent patient, as delays in the medical response can be predicted for roughly one-third of patients experiencing adrenal crisis.