In a small group of patients with AD, AC was highly prevalent occurring in 63.8%, in the previous five years. Knowledge in preventing an AC was poor and particularly in the areas of managing diarrhoea, intercurrent stress and surgery. Despite having had an AC, knowledge was poorer in respect of prevention strategies relative to having a cold, fever, infection, diarrhoea, vomiting and intercurrent stress. Vulnerability was highly prevalent and interestingly, AD duration was negatively correlated with vulnerability, whereas the number of comorbidities was positively associated with vulnerability. Knowledge score was negatively correlated with the development of an AC, and increased vulnerability was associated with the development of an AC.
This study supports importance of knowledge of prevention strategies, to reduce the likelihood of an AC. Poorer knowledge scores were associated with a crisis in the previous 5 years. Logistic regression revealed that poorer knowledge was independently associated with the development of a crisis in the previous 5 years. Our survey demonstrated that knowledge was patchy, specifically, in the domains of addressing a cold, fever, infection, diarrhoea and vomiting. Of concern, was that fewer than 50% of this surveyed population offered correct answers. A German study, examined the source of knowledge transfer in patients with all forms of hypoadrenalism, and were asked to rate their knowledge in being able to cope with AD. In 30% they indicated that they were sufficiently informed, whereas 64% suggested that they had deficits [24]. Our patients who never experienced, compared with those who had experienced a crisis, had better knowledge of prevention strategies in managing a cold, fever, infection, diarrhoea, vomiting or intercurrent stress, implying a protective effect of knowledge. Recovery from AC may have been a missed opportunity for education or that the crisis per se, or it made patients less receptive to instruction on how to take evasive action to prevent an AC in the future. This was not probed but would have been subject to recall bias.
Our patients are deficient in specific domains, thus emphasis of these, during all clinic visits, may be lifesaving. Our results are tangible evidence for enhancing knowledge-based strategies in preventing AC. The minority of participants (21%) had contacted a support group, which is similar to UK patients with AD attending a self-help group [25], explaining the overall poor knowledge. A German survey found 70% of patients with all forms of hypoadrenalism acquired counselling from their physicians, 65% acquired additional knowledge from books, brochures and guidebooks, 59% obtained information from interacting with other members of supportive groups and 48% preferred the internet [24]. Our participants were not evaluated as to the extent of training that they received during their interactions with health-care providers or degree of engagement with support groups. This hampers our determination as to whether this is a health-systems deficiency, due to being overloaded or a problem relating to cognition and poorer memory [26]. The minority (49%) wore some form of identification, indicating that they suffer from AD requiring stress doses of hydrocortisone in case of an emergency, which represents a gross implementation deficiency.
We attempted to identify contributing factors for an AC. There was disproportionately greater number of patients with primary hypothyroidism, osteoporosis, type 1 diabetes and type 2 diabetes who developed a crisis. Coexisting type 1 diabetes, for example, may predispose to the development of an AC. Primary hypothyroidism and type 2 diabetes may also contribute to the development of an AC, albeit that it remains elusive, how osteoporosis may culminate in an AC. Either initiation or overtreatment of hypothyroidism has been shown to precipitate an AC in vulnerable persons for example, autoimmune polyglandular syndrome where hypothyroidism and AD can coexist, raising the cortisol demands [27]. In a Swedish national in-patient cohort, coexistence of diabetes and AD represented a 4-fold increased risk of all-cause mortality, compared with matched controls of diabetes alone and although cardiovascular risk was the most common cause of death, infections and potentially an AC, were more common than in diabetics alone [28]. Specific factors in developing an AC, including gastrointestinal infection, fever, sexually transmitted diseases, psychological stress and pregnancy, have been identified. Similar to our study, Hahner et al, demonstrated an increased number of comorbidities, particularly, non-endocrine, was predictive for the development of an AC, with an odds ratio of 2.02 (95% confidence interval of 1.05–3.89; p = 0.035) [4]. Our survey did not interrogate whether they used inadequate replacement doses of glucocorticoids or if there was a history of poor adherence [7]. Unplanned surgery is a predisposing factor and by pro-actively addressing this in patients with AD, we may be avert a crisis in the future.
The majority (66%) of our cohort suffered from at least one crisis in the previous 5 years, which is considerably higher than reported by Meyer et al [6], in which there is a 5%-17% likelihood of having an AC and 6 per 100 patient years, as described by Arlt et al [7]. Explanations for this vastly increased risk in SA are not apparent, but low doctor patient ratios, competing communicable disease demands, poor allocation of health-care resources, and unmet needs, may account for this [19]. Patients living in SA are at increased vulnerability, due to their poor economic circumstances, exposure to suboptimal healthcare delivery, and widespread crime. Greater vulnerability may affect mental health adversely and could affect patient adherence to medication and self-care.
It is expected that AD for a shorter duration may result in greater vulnerability scores, and a greater fear of the unknown. Living longer with AD may have a mitigating effect against vulnerability. It is expected that having several comorbidities, may predispose to vulnerability. Patients who had an AC felt more vulnerable, but vulnerability was associated with the development of a crisis, in the previous year. It remains uncertain as to whether vulnerability may predispose to a crisis or whether a recent crisis in the preceding year results in greater vulnerability. Our study has demonstrated a substantial burden of vulnerability. Health-care practitioners caring for these patients should pay attention to necessary psychological and if required, psychiatric support, as a comprehensive care plan. This recommendation is supported by the burden of depression in AD [29]. Henry (2019) in her PhD documented greater anxiety in patients than controls, (10.68 versus 2.5) using the Beck Anxiety Inventory; (p < 0.001) [30].