ESPE2021 ePoster Category 2 Adrenals and HPA Axis (57 abstracts)
Variability of advice and education for steroid sick day dosing in Duchenne Muscular Dystrophy and the impact of the COVID-19 pandemic: Results of a UK wide patient survey
S Thakrar 1 , MS Cheung 2 , A Johnson 1 , H Katugampola 3 , A Manzur 4 , F Muntoni 4 , T Mushtaq 5 , C Turner 6 , C Wood 7 , M Guglieri 6 & SC Wong 8,9
1Duchenne UK, United Kingdom, United Kingdom; 2Department of Endocrinology and Diabetes, Evelina Children’s Hospital, London, United Kingdom; 3Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children, London, United Kingdom; 4Dubowitz Neuromuscular Centre, Great Ormond Street Hospital for Children, London, United Kingdom; 5Department of Paediatric Endocrinology, Leeds Teaching Hospital NHS Trust, Leeds, United Kingdom; 6John Walton Muscular Dystrophy Research Centre, Newcastle, United Kingdom; 7Newcastle University Translational and Clinical Research Institute, Newcastle, United Kingdom; 8Department of Paediatric Endocrinology, Royal Hospital for Children Glasgow, Glasgow, United Kingdom; 9School of Medicine, University of Glasgow, Glasgow, United Kingdom
Introduction: The use of long-term oral corticosteroid in DMD is associated with a range of side effects including secondary adrenal insufficiency. The updated international care consensus (2018) recognizes this important issue and recommends emergency plans to be in place.
Aim: This online UK-wide patient survey aims to determine the advice and education given for sick day dosing plans and the impact of the COVID-19 pandemic in corticosteroid-treated young people with DMD.
Methods: The survey was circulated between November 2020 and January 2021.
Results: A total of 130 responses were obtained from 129 parents/guardians of a person with DMD. One parent provided responses for twins. Median age of the person with DMD was 11.5 years (Range: 5, 26). Eighty-three of 130(64%) were on daily corticosteroid. Sixty of the 130(46%) had access to hydrocortisone for injection with severe and/or vomiting illness. Of the 60 with access to injectable hydrocortisone, 44/60(73%) also had an oral stress dose plan for mild illness. However, 48/70(69%) with no access to injectable hydrocortisone did not have an oral stress dose plan. Of the 60 with access to injectable hydrocortisone, 42/60(70%) had received some form of training. Twenty-one of the 42(50%) of those who received training performed a practice injection. Thirty-four of the 42(81%) who received training felt confident about administering an injection as opposed to 9/18(50%) who did not receive any training. Forty-one of the 42(98%) who received training were clear on when injectable hydrocortisone is needed as opposed to 14/18(78%) who did not receive training. Prior to the COVID-19 pandemic [February 2020], 43/130(33%) were provided with an oral emergency stress dose plan for mild-moderate acute illness. After February 2020, 66/130(51%) were given an oral emergency stress dose plan for mild-moderate acute illness. A total of seven different oral stress dose plans were identified for those on daily corticosteroid and a greater number of combinations of plans in those on the intermittent regimen.
Conclusion: Despite the risk of adrenal crisis in patients who are treated with long-term corticosteroid and recent international guidance (2018), emergency sick day plans were not consistently in place in this national survey of boys with DMD. Solutions could be challenging but must take a multi-dimensional and multi-disciplinary approach. Guidance at a national level and the development of structured education for hospital clinicians and patients maybe steps towards improving clinical outcomes.
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