What is the impact of winning the BSPED Nurse and AHP Award by Kate Davies

As we plan to launch the 2025 Awards we asked Kate Davies, (BSPED Nurse and AHP Award winner in 2022) to tell us how her project “The clinical validity and acceptability of alternative methods of monitoring for young people with Congenital Adrenal Hyperplasia (CAH)” was impacted by winning the award. 

The most common form of Primary Adrenal Insufficiency is Congenital Adrenal Hyperplasia (CAH), occurring in approximately 1 in 15,000 live births. In CAH due to 21-hydroxylase deficiency, blocks occur in the steroidogenesis pathway leading to a lack of cortisol formation, with mineralocorticoid production also affected. 

Treatment is in the form of oral fludrocortisone to replace the salt loss, and oral glucocorticoid (GC) replacement therapy, such as hydrocortisone. This is frequently prescribed at 10-12 mg/m²/day in divided doses, but higher dosages may sometimes be required to suppress the adrenocorticotrophic hormone (ACTH) driven androgen production in CAH. 

The signs and symptoms of under or over treatment may take months to detect, and could remain undetected in current monitoring programmes, which only gives a single snapshot view of a single timepoint. The goal of treatment is to achieve the best clinical outcome for the patient, with the lowest dose of GC possible, in order to reduce the potential side effects. Underdosing can result in a life threatening adrenal crisis, leading to hypovolaemic shock and ultimately death. Overdosing, especially in children, can result in growth failure, weight gain, hypertension, and the potential for cardiovascular disease as the child grows older. If replacement is not adequate, potentially due to non-adherence, this can lead to hyperandrogenism, leading to early puberty and later infertility, and testicular adrenal rest tumours (TARTs) in boys. If overtreated, hypercortisolism can result, leading to insulin resistance and obesity).  It is therefore vital for children and young people (CYP) to adhere to monitoring and medication regimes, and understand the processes involved during the transitional process from paediatric to adult services.

Current monitoring in the UK involves regular biochemical analysis to assess the adequacy of replacement therapy. Practice varies between 24-hour cortisol assessments with 2 hourly cortisol and adrenal androgen measurements via an intravenous cannula, to occasional measurement of individual androgens: there is no consensus for optimum, effective, yet acceptable monitoring. Ultimately, there are unmet needs in the monitoring and long-term outcomes of CAH in young people. 

However, there is growing interest in using alternative sampling methods to measure cortisol. Conventional methodologies for monitoring and management of CAH can potentially be replaced and / or used in conjunction by hair and / or salivary cortisol measurement, alongside focused anthropometrical measurements. This has a number of potential advantages including that it gives a long-term measure of cortisol replacement, is not invasive and could be performed without a need to visit the hospital clinic.

Hair cortisol analysis provides a longer read-out of cortisol exposure: the exposure time frame is dependent on the length of hair analysed, with each centimetre of hair being estimated to represent one month of exposure. This compares to monitoring in Type 1 diabetes, where HbA1c (glycated haemoglobin) measurements are taken every few months in order to gain an understanding of long-term glucose control. 

Likewise for saliva cortisol analysis, which has also been shown as a non-invasive monitoring tool, although these do focus more on specific cortisol concentrations throughout the day. Nevertheless, salivary cortisol day curves can be undertaken throughout the day in the patient’s home, compared to 24-hour venous sampling which would necessitate overnight hospital stays. 

Anthropometric (non-painful and non-invasive) measurements during adolescence highlight inadequacies in cortisol replacement, which can invoke earlier nursing input in order to reduce the risk of metabolic syndrome and obesity long-term. However, this is not mainstream current practice, with body measurements only focusing on height, weight, and Tanner staging during puberty, alongside bone-age assessment.

The award from the BSPED has enabled me to undertake specific anthropometric training with the International Society for the Advancement of Kinanthropometry (ISAK), and also purchase the necessary equipment, such as a stadiometer and skinfold callipers. In addition, the funding will also go towards the development of a website for recruitment of patients within the two hospital Trusts I will be working with, which can be accessed through a QR code on specifically designed recruitment posters.