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Epidermolysis Bullosa in Paediatric Palliative Care: A Case Series
Oral Presentation
October 13th 2022 at 11:45am
Institution: The Royal Children's Hospital - SA, Australia
Background
Epidermolysis bullosa (EB) comprises a group of rare genetic conditions which are characterised by fragility of the skin and mucous membranes and formation of blisters with minor trauma. EB can be associated with a range of multi-system effects. Severe forms of EB can be life-limiting. There is a lack of studies which describe the Palliative Care needs of children with EB.
Epidermolysis bullosa (EB) comprises a group of rare genetic conditions which are characterised by fragility of the skin and mucous membranes and formation of blisters with minor trauma. EB can be associated with a range of multi-system effects. Severe forms of EB can be life-limiting. There is a lack of studies which describe the Palliative Care needs of children with EB.
As a state-wide Paediatric Palliative Care service, we have been uniquely placed to care for a number of children with EB.
Aim
The aim of this case series was to describe the experience of children with severe forms of EB and define the role of the Paediatric Palliative Care service in caring for these children and their families.
The aim of this case series was to describe the experience of children with severe forms of EB and define the role of the Paediatric Palliative Care service in caring for these children and their families.
Methods
We present a series of five cases of children with severe forms of EB who were known to the state-wide Paediatric Palliative Care service from 2011 to the present day, with a discussion of our learnings in caring for these children and their families. We conducted a retrospective review using electronic medical records. Parents and guardians gave consent for their child to be included in this case series.
We present a series of five cases of children with severe forms of EB who were known to the state-wide Paediatric Palliative Care service from 2011 to the present day, with a discussion of our learnings in caring for these children and their families. We conducted a retrospective review using electronic medical records. Parents and guardians gave consent for their child to be included in this case series.
Results
Medical decision-making and provision of healthcare in EB provokes complex ethical, psychological, personal and professional dilemmas. This can be confronting for health professionals as well as for children with EB and their families. Our case series highlights the diversity of management approaches that may be considered, each tailored to the unique context of the child and family.
Medical care of children with EB must be collaborative and cohesive and is best provided by a multidisciplinary team. The Paediatric Palliative Care team can play an important role in promoting quality of life and supporting complex decision making, in addition to symptom management and care coordination.
