Addison's, adrenal insufficiency and patient safety

The Addison’s Disease Self-Help Group (ADSHG) is the Patient Support Group (PSG) for Addison’s disease and adrenal insufficiency in the UK. Supporting both patients and healthcare professionals (HCPs), the ADSHG provides expert, trusted guidance alongside peer support, advised by a Clinical Advisory Panel and affiliated with the Society for Endocrinology (SfE) and the European Society of Endocrinology (ESE).

There is a clear clinical gap in the treatment and management of these rare conditions. The management of adrenal insufficiency has evolved little over the last 30–40 years with a one-size-fits-all approach common. Adrenal insufficiency-related hospital admissions and adrenal crises (with their associated morbidity and mortality) have increased considerably in the last two decades; for every 200 incidents of adrenal crisis, there will be one death.

To address the identification and management of adrenal insufficiency, the first-ever NICE guidance for AI was published in August 2024 and its importance should not be underestimated. The guidelines cover the care of people with primary (Addison’s), secondary, or tertiary adrenal insufficiency (including glucocorticoid-induced adrenal insufficiency). Their detailed and comprehensive analysis of the available clinical data can now inform best practice.

NICE Topic Advisor Dr Helen Simpson’s (UCLH) webinar on the NICE Guidelines updates HCPs on the latest practices in diagnosing and managing this critical condition. Dr Simpson breaks down the guidelines, highlights key changes, and provides practical advice on implementation in clinical settings.  

Adrenal crisis – a preventable death

In an emergency situation, non-expert HCPs often do not realise the urgency of treatment for acute adrenal crisis or fail to heed the requests of well-informed patients for hydrocortisone. Rapid treatment can shorten hospital stays, reduce emotional trauma and avoid unnecessary deaths from this eminently treatable medical problem.

The NICE guidelines address this, providing life-saving information over elements such as education, the need for steroid emergency cards, a minimum of two emergency injection kits to be issued to all patients and access to sufficient corticosteroid medication to enable patients to self-manage and follow the ‘Sick Day Rules’.

The need for the NICE guidelines is further shown as deaths associated with a lack of understanding amongst HCPs take place, despite the NHS National Patient Safety Alert and guidelines for the management of these patients being available from the Society for Endocrinology and the Addison’s Disease Self Help Group, and for emergency care the standalone steroid-dependent guidelines in the JRCALC.

Tragically in a recent Regulation 28: Report to Prevent Future Deaths, the coroner reported the death was contributed to by delays in identifying the patient required exogenous steroid medication, alongside delays in the prescription and administration. The coroner expressed concern over the lack of understanding from GPs to paramedics, all in managing this treatable problem. The NICE guideline provides information to help prevent future tragic, avoidable deaths for the HCPs, with the ADSHG providing resources to equip the patients to highlight their steroid dependency when at their most vulnerable.

Ambulance care – moving away from “red flagging”

Communicating change of practice within rare conditions raises another area of concern. Whilst the NHS is so stretched, often vital updates in processes don’t reach those impacted. In February 2024 the Association of Ambulance Chief Executives (AACE) recommended that 'Red Flags' on the addresses of those with adrenal insufficiency should no longer be used and Trusts have either ceased or will cease the use of 'Red Flags'. Confusion remains as not all Trusts have yet implemented the change, and Scotland, Wales and Northern Ireland are reviewing their own processes. In light of the change, the AACE encouraged that Summary Care Records (SCR) should be accessed. Yet many patients and HCPs are unaware of this update, or indeed whether it is the best solution.

There is no doubt that continued development of patient safety solutions for steroid dependency is required for emergency situations. In the case of Regulation 28: Report to Prevent Future Deaths from February 2024, the expectation was placed on the patient to volunteer crucial information about their condition, rather than on the NHS Pathways system or Call Handlers to access the SCR. Consequently, this led to the tragic and preventable death of a 24-year-old male - a father, fiancé and son.

Due to rare conditions being lifelong and complex, the need to be an ‘expert patient’ is clear. Working together, HCPs and PSGs can have a powerful impact in ensuring the provision of high-quality and well-coordinated care, and communicating change – such as disseminating the new NICE Guidelines and changes in ambulance processes. The joint aim is to engage, educate, equip and empower all HCPs, patients and those who support them.

The new NICE guidelines for adrenal insufficiency bring positive change and voice to the patients’ needs. Their release shines a light on the importance of steroid dependency; with the hope of further developments to meet clinical needs, research gaps, and preventing deaths.

We are very grateful to Dr Simpson and all involved on the guideline committee, for their hard work and dedication to improving patient safety and the lives of those affected by Addison’s and adrenal insufficiency. Please get in touch with Addison's disease if we can support you or your patients in any way.