Rare Disease Day takes place annually on 28 February (or 29 February in leap years), chosen because 29 February is the rarest day of the year. The day raises awareness of rare diseases and their impact on the 3.5 million in the UK affected by a rare condition. So whilst individually rare, rare conditions are collectively common, and our voices are stronger when we work together. 

An issue which affects all rare diseases is care coordination. We all know the positive impact well-coordinated care has on our quality of life as people living with rare health conditions. But the benefits are also clear for healthcare budgets, and providers of services outside of the healthcare environment. However coordinated care is in itself rare. So how could this be improved?

Thanks to the hard work of the Endocrine Team at University Hospitals Birmingham NHS Foundation Trust, they have developed and put in place in their hospitals ‘The 4E’, to support people living with Addison’s disease, adrenal insufficiency, carers and clinicians.

Many rare diseases are lifelong and complex. Being engaged, educated, equipped and empowered is essential to help people living with these conditions. Management of life-long conditions requires ongoing education and training of not only those who have the condition themselves but those who support them, as well as all healthcare providers to ensure the provision of high-quality and well-coordinated care.   

Here we speak to Sherwin Criseno, one of the experts behind The 4E framework and the Nurse Consultant in Endocrinology at University Hospitals Birmingham NHS Foundation Trust and Chair of the European Society of Endocrinology Nurse Committee. He explains The 4E initiative, how it supports people with Addison’s and adrenal insufficiency and the potential for it to be applied to not only other Endocrine units in hospital trusts across the UK, but other rare health conditions.

The 4E: Engage, Educate, Equip and Empower

    4E is a framework that provides a systematic and effective system of supporting people with a long-term condition to prevent complications (such as adrenal crisis), recognise and manage early signs of deterioration to prevent avoidable serious and fatal consequences, such as death.

    The 4E framework puts emphasis on the importance of empowerment of patients, their loved ones and carers as well as healthcare providers as key to the effective management of long-term conditions by engaging, educating and equipping all stakeholders on all aspects of the care of people living with Addison's or adrenal insufficiency.

    How has it been applied in your hospital trust?

    The 4E framework was used to implement the National Patient Safety Alert (2020) of the NHS Steroid Emergency Card. A working group led by an Endocrine Consultant and Endocrine Nurse was established consisting of key stakeholders including: emergency care and critical care clinicians; medical and surgical clinicians; pharmacists; management teams; non-clinical staff (patient safety team, communications team and IT services), as well as patient representatives.

    The working group met 2-weekly during the first 3 months of project planning and developed a strategy for implementation, education and monitoring of NPSA Patient Safety Alert provisions across the organisation.

    1. First, an engagement session with patient representatives and clinicians revealed key areas of focus in terms of clinical practice, education and training as well as audit and research.
    2. Education was then focused to non-Endocrine clinicians as well as Emergency Care and Critical Care staff on the prevention and management of adrenal insufficiency and adrenal crisis.
    3. To support clinicians in clinical practice, they were equipped with clinical guidelines consisting of easy-to-follow practical guides, patient information leaflets, supplied with copies of the NHS Steroid Emergency Cards, as well as advice and guidance for escalation and support.

    To evaluate the effectiveness of the implementation and the degree of empowerment, an audit was carried out after 6 months which revealed a high degree of utilisation of the clinical guidelines, high usage of the NHS Steroid Emergency Card, high level of awareness of adrenal insufficiency and its management (as identified through clinical record during patient’s admission) and increased level of interaction between Endocrine and non-Endocrine clinicians on the management of patients with adrenal insufficiency. This was further evidenced by an increased number of referrals for in-house advice and guidance.

    Why is The 4E initiative helpful for people living with Addison’s disease and adrenal insufficiency?

    Adrenal insufficiency is a life-long condition. As such patients are the main caretaker of their health. Therefore effective and safe self-management is critical in both preventing and managing adrenal crisis. For patients to be able to manage their long-term conditions safely and effectively, they need to be engaged with all aspects of their care, educated by professionals constantly on self-management techniques and equipped with tools to prevent and manage adrenal crisis. The 4E framework is there to support them and other clinicians involved in their care to achieve this.

    Tell us more about your work in Endocrinology

    I am a Nurse Consultant in Endocrinology at the University Hospitals Birmingham NHS Foundation Trust, UK. I currently lead the largest team of Specialist Nurses in Endocrinology in Europe and am the chair of the European Society of Endocrinology Nurse Committee. I'm a visiting lecturer at the University of Birmingham and also an NIHR Clinical Doctoral Research Fellow, currently pursuing my doctoral research on the effects of growth hormone treatment discontinuation in adults.

    "Adrenal insufficiency has always been an area of interest for me. In particular, patient empowerment through effective self-management has been one of the main focuses of my clinical practice."

    To help raise awareness of adrenal insufficiency and The 4E framework among healthcare professionals, I attended Endocrine meetings including the Society for Endocrinology Congress held in November 2021 and am grateful to have been awarded the ADSHG Annette Louise Seal Memorial Award, which is given for a nurse-led research/project that advances steroid awareness and patient safety.

    I was delighted to be invited by the ADSHG to speak at their Patient Voice session at the European Society of Endocrinology (ESE) Conference live in Italy in May 2022. Working with the ADSHG, alongside The Pituitary Foundation, CAH Support Group and Alex TLC for The Patient’s Voice session allowed me to speak directly to other healthcare professionals in Europe on The 4E through our session titled: “Preventing Adrenal Crisis, the importance of dialogue between patient and clinician.”

    Do you have any advice that you would give to healthcare professionals looking to undertake research or put frameworks in place to create patient resources?

    When thinking of service development to improve the care and support for people living with long-term conditions, remember The 4E: Engage, Educate, Equip and Empower!

    Author: Sherwin Criseno

    Nurse Consultant in Endocrinology at University Hospitals Birmingham NHS Foundation Trust, HEE/NIHR Clinical Doctoral Research Fellow and Chair of the European Society of Endocrinology Nurse Committee.

    Twitter: @CrisenoSherwin

    'The 4E (Engage, Educate, Equip and Empower): A framework for supporting the approach in the prevention, early recognition and effective management of adrenal crisis in adults.'

    Endocrine Abstracts (2021) 77 P17 | DOI: 10.1530/endoabs.77.P17

    Thank you Sherwin for speaking with the ADSHG in support of Rare Disease DayWe hope that increased awareness of best practice initiatives such as The 4E among healthcare professionals will help raise awareness of well-coordinated care to not only support people with Addison’s and adrenal insufficiency but all rare conditions.



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