Evaluating the effectiveness of a Steroid Therapy Card in the management of children with Congenital Adrenal Hyperplasia (CAH) at home

Background: Congenital Adrenal Hyperplasia (CAH) is the most common cause of childhood primary adrenal insufficiency, which can predispose a child to the risk of adrenal crisis and possible increased morbidity and mortality,... [ view full abstract ]

Background:

Congenital Adrenal Hyperplasia (CAH) is the most common cause of childhood primary adrenal insufficiency, which can predispose a child to the risk of adrenal crisis and possible increased morbidity and mortality, in the event of illness, stress or injury. CAH is an autosomal recessively inherited condition causing inadequate production of cortisol, aldosterone and excess adrenal androgens. Glucocorticoid dosing is integral to this condition and optimal management relies on parents and carers understanding the principles of prompt action and sick day rules to prevent adrenal crisis.

Aim and Objective

To evaluate the effectiveness of steroid therapy cards as information tools for parents of children with CAH.

Description of Innovation:

A literature review revealed a lack of consensus to ‘Sick Day Rules' and a steroid therapy card for adrenal insufficiency containing personal information and dosing regimens was developed cross-site for use by families and their specialist centres. A concurrent audit in the form of an anonymous questionnaire was distributed to parents in late 2014 at the author's institution.

Impact of Innovation:

Benefits of the steroid therapy card include individualised information for families on how to respond when their child is unwell and standard guidelines for local hospitals on how to treat adrenal crisis. Overall, feedback has been positive.

Findings also highlighted the need for continuing parental education and a 50% non-compliance with the recommended wearing of medi-alert bracelets.

Conclusion and implications:

Recommendations proposed include:
 (1) Ongoing education and information sharing sessions with parents to revise ‘Sick Day Rules’.
 (2) Strategies to improve uptake of medi-alerts bracelets.

A feedback sheet with the findings was distribured at clinic highlighting both the importance of sick day management and the recommended wearing of medi-alert bracelets with details of 'budget friendly' suppliers.

Optimal control relies on parents and carers abilities to manage their child's condition at home and can greatly influence a better and safer outcome for a child with CAH.