Background: We present a case of a 10 year old female with T1DM, referred to our tertiary centre for complex diabetes care due to her other severe chronic dermatological condition. We discuss the difficulties with optimizing diabetes control in such a chronic debilitating condition which has a pervasive effect on T1DM treatment.
Case: To our knowledge this is only the second reported case of a child with T1DM and Epidermolysis Bullosa. Severe Recessive Dystrophic Epidermolysis Bullosa (RDEB) is characterized by a lack of adhesion under the basement membrane of the skin, leading to blisters which heal with scarring, contraction of the joints, fusion of the fingers and toes, contraction of the mouth membranes and narrowing of the oesophagus. Additionally, RDEB patients have a high chance of developing squamous cell carcinoma. The particular issues that have arisen in relation to T1DM care include:
1) Poor skin integrity affecting insulin administration and glucose monitoring (including both continuous and sporadic testing).
2) Pain for multiple reasons; In particular, pain on swallowing due to oesophageal strictures which in turn lead to variation in oral intake and reliance on gastrostomy feeds. This impacts on carbohydrate counting, carbohydrate ratios and overall effectiveness of insulin requirements/absorption.
3) Limitations of physical ability, particularly reduced hand dexterity, effecting ability to provide her own diabetes care and resulting in reliance on others. This lack of autonomy had led to emotional difficulties especially around school.
4) Language barriers due to relocation for care of her RDEB, which has made relationships between healthcare centres and recognition of the patient and her familys diabetes knowledge more complex. This has impacted on the treating diabetes team deciding the best course of treatment in her condition.
In addition, there are deep psycho-social implications of this life limiting condition combined with the new diagnosis of T1DM.
Discussion: This particular case highlights the complexities of management of a patient with T1DM and a severe dermatological condition. Discussion around this case helps to highlight those aspects of care that can best support patients, families and healthcare professions in caring for these patients.
27 - 29 Nov 2019
British Society for Paediatric Endocrinology and Diabetes