The design and psychometric evaluation of a health-related quality-of-life outcome measure (QoLTEN) for adults with Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) are devastating conditions with a sudden onset. Patients are catapulted into a rapid and unexpected form of acute skin failure with a significant risk to their life. They become critically ill within a short period of time and are treated as a medical emergency with the focus of care being on the preservation of life. The cause of these conditions is usually an abnormal reaction to medication. Initially, the patient presents with flu like symptoms, high temperature, and a red rash. The rash spreads and blisters, whereupon the top layer of the skin dies and is eventually shed. The lungs, genitals and the eyes may be involved leaving the patient with many long-lasting physical and psychological complications. Current treatments revolve around the identification and withdrawal of the causative drug, as well as intensive care support of the different failing organ systems. The survival of patients is increased when they are cared for within the Intensive Care Unit or Burns Unit setting.

Survivors of SJS/TEN are left with many psychological and physiological complications leading to a substantial impact on their quality of life e.g., inability to work and relationship problems. Many survivors are afraid of taking medication, with 50% of survivors still avoiding medication five years post discharge. Patients, family carers and clinicians have noted that the general Quality of Life (QoL) measures do not capture the specific experiences of these patients. Consequently, this project aims to develop and test an SJS/TEN specific QoL outcome measure to be used to assess QoL experiences of this group. It is envisaged that this QoL measure may also be used as a communication aid between clinicians and patients, during follow up care to monitor changes in QoL and provide appropriate care to allay distress.

Award Date
01 July 2022
Award Value
€355,335.88
Principal Investigator
Dr Pauline O'Reilly
Host Institution
University of Limerick
Scheme
ILP