1.1.1. Introduction

In times of physiological stress (increased physical need for cortisol/glucocorticoid replacement), individuals with adrenal insufficiency are unable to produce enough cortisol to meet these requirements. A failure to adjust glucocorticoid therapy in times of physiological stress may place individuals at risk of adrenal crisis or even death.

Patients, their family, or carers, may require the necessary information and training to develop the knowledge and skills to adjust their glucocorticoids in this way. Currently, there is variation in practice on the provision of information and in the level of content made available.

This review explores non-pharmacological strategies for managing periods of physiological stress in people with adrenal insufficiency.

1.1.2. Summary of the protocol

For full details see the review protocol in Appendix A.

Table 1

PICO characteristics of review question.

1.1.3. Methods and process

This evidence review was developed using the methods and process described in Developing NICE guidelines: the manual. Methods specific to this review question are described in the review protocol in Appendix A and the methods document.

Declarations of interest were recorded according to NICE’s conflicts of interest policy.

1.1.4. Effectiveness evidence

1.1.5. Summary of studies included in the effectiveness evidence

No relevant clinical studies were identified.

1.1.6. Summary of the effectiveness evidence

No relevant clinical studies were identified.

1.1.7. Economic evidence

1.1.8. Economic model

This area was not prioritised for new cost-effectiveness analysis.

1.1.9. Unit costs

Relevant unit costs are provided below to aid the consideration of cost-effectiveness.

Table 2

Staff costs associated with non-pharmacological interventions.

Table 3

Other costs associated with non-pharmacological interventions.

1.2.1. The outcomes that matter most

The committee considered all outcomes listed in the protocol to be critical and of equal importance in decision-making. These outcomes included mortality, Health-related Quality of Life, incidence of adrenal crisis, admission to hospital or ITU and psychological morbidities such as incidence of stress or PTSD.

1.2.2. The quality of the evidence

No clinical evidence was identified for this review.

1.2.3. Benefits and harms

In the absence of any identified evidence, the committee decided to make consensus recommendations to reflect best current clinical practice for non-pharmacological management of adrenal insufficiency. They wished to highlight emergency cards that should be given to people who are taking glucocorticoids or who have adrenal insufficiency and are at risk of serious complications.

The NHS Steroid Emergency Card is given to all adult patients at risk of adrenal crisis including people with primary adrenal insufficiency and people who may develop secondary adrenal insufficiency and become steroid dependent. Its purpose is to prompt all healthcare professionals to consider adrenal crises in people carrying the card, initiate appropriate management for surgery or invasive procedures and treat patients rapidly and appropriately when presenting as an emergency. It includes a summary of the management and emergency treatment of adrenal crisis and provides a link to the Society for Endocrinology emergency management guidelines. It is provided by health care professionals prescribing glucocorticoids or looking after patients with adrenal insufficiency.

The British Society of Paediatric Endocrinology and Diabetes (BSPED) has developed an emergency steroid card which summarises the emergency management and sick-day rules in children and young people. This card is given to children and young adults who have adrenal insufficiency or who are at high risk of adrenal insufficiency due to exogenous steroids.

A Steroid treatment card (blue card) is also available and is provided to people who are at risk of tertiary adrenal insufficiency or can be ordered from NHS Forms at NHS Business Services Authority (NHS BSA). This generally affects people with non-endocrine conditions who are on exogenous steroids where dose and duration could lead to adrenal suppression. The card includes guidance on minimising the risks when taking steroids, such as not stopping glucocorticoids abruptly, or changes in dose if the person is experiencing any physiological stress, undergoing surgery or invasive procedures. It provides details of an individual’s prescriber, drug, dosage, and duration of treatment for healthcare professionals treating the patient.

The committee emphasised the importance of providing information to people with or at high risk of adrenal insufficiency on how to manage their treatment at times of physiological stress such as intercurrent illness and on sick days. They also highlighted additional non-pharmacological methods that are available to alert health care professionals about a patient’s condition, and which are easy to implement. These include medical alert jewellery such as bracelets, apps, and mobile phone medical IDs.

1.2.4. Cost-effectiveness and resource use

No economic evaluations were identified for this review; therefore, unit costs were presented to aid the committee’s consideration of cost-effectiveness. Unit costs were obtained for a range of healthcare professionals who may deliver non-pharmacological interventions listed in the protocol. In addition, the cost of Steroid Emergency Cards was also presented.

In current best clinical practice, all people with a diagnosis of adrenal insufficiency are provided education about daily dosing, sick-day rules and crisis management. Adults with adrenal insufficiency are given steroid emergency cards which provide healthcare staff important information on when to prescribe emergency hydrocortisone and the person’s personal information (such as NHS number and why they are prescribed steroids). Children with adrenal insufficiency are given a BSPED (British Society for Paediatric Endocrinology and Diabetes) adrenal insufficiency card which provides parents, carers, and healthcare staff a child’s steroid care plan for sick-days and emergencies. People at risk of adrenal suppression because of corticosteroid use are also provided a blue steroid card which provides information and advice for healthcare professionals and people at risk of adrenal insufficiency.

Education on daily dosing, sick-day rules and crisis management is provided at the time of diagnosis and throughout a person’s treatment. Education can be provided by various healthcare professionals as this information is continually relayed to a person with AI when they present in a healthcare setting. In current practice, the structure of endocrinology departments varies; making it challenging to estimate the cost of providing this education. However, because the provision of this education is best clinical practice and the cost of providing additional education when people are in a healthcare setting is minimal (a couple of minutes of extra staff time), this recommendation is not expected to result in a significant resource impact. In addition, this provision of information is highly likely to be cost-effective as without this information people are at increased risk of experiencing an adrenal crisis. An adrenal crisis not only negatively impacts a person’s quality and life (and may result in death if not treated quickly enough), but also has large cost implications associated with it. Costs of an adrenal crisis will vary – and can range from a hospital admission in intensive care to a day case hospital admission.

Adult steroid emergency cards cost £2.65 (for 100 cards). The provision of BSPED adrenal insufficiency cards is current practice. The cost of providing BSPED adrenal insufficiency cards is likely already included in administrative budgets as these are downloaded from the BSPED website and printed by endocrinology departments to be given to parents and carers. Blue steroid cards are provided by pharmacists when the person picks up their steroid prescription.

In best practice people are also provided information on the use of patient held alerts (such as medic alert bracelets) and directed to recognised patient support groups. Patient-held alerts are either free (for example, mobile phone medical ID) or the cost is borne by the person with adrenal insufficiency. Patient support groups are run by charities and therefore no cost is incurred to the NHS.

Overall, the recommendations made for non-pharmacological interventions for managing periods of physiological stress are largely reflective of clinical practice and will therefore not result in a significant resource impact.

1.2.5. Recommendations supported by this evidence review

This evidence review supports recommendations 1.4.10 – 1.4.11.

J.1. Clinical studies

J.2. Health Economic studies

None.