Patient Experiences of SJS/TEN in Children and Young People
Research type
Research Study
Full title
A Qualitative Study of the Psychological Morbidity in Paediatric Survivors of Stevens Johnson Syndrome and Toxic Epidermal Necrolysis
IRAS ID
275144
Contact name
Tess McPherson
Contact email
Sponsor organisation
Oxford University Hospitals NHS Foundation Trust
Duration of Study in the UK
1 years, 0 months, 30 days
Research summary
Research Summary -
Stevens Johnson Syndrome (SJS) and its more severe counterpart Toxic Epidermal Necrolysis (TEN) are rare, life-threatening skin conditions which affect 5 people per million population per year. They can affect people at any age, and at the Oxford University Hospitals Dermatology Department, we see approximately one case per year in children and young people (under the age of 18). Whilst children and young people are much more likely to survive this illness than adults, they can be affected by significant long-term complications, such as skin changes including scarring, eye problems and lung damage. In adults, we know that SJS and TEN can cause long term psychological problems. However, as the latest guidelines on SJS and TEN highlights, there has been no research into the psychological effects of these conditions in children.
We propose running a questionnaire and telephone interview study to investigate the patient experiences and psychological effects of SJS and TEN in children and young people who have been treated at Oxford University Hospitals for SJS or TEN over the last 10 years. Questions will focus on their memories of the illness, any sleep disturbance or nightmares about SJS / TEN, their understanding of the condition and its causes, and how this has affected them longer term, including how they feel about doctors, hospitals, and medicines since the event, as well as screen for symptoms of post traumatic stress, anxiety and depression.
This research will help us understand what the experiences of SJS and TEN were for this population, and whether it differs from the adult population. As the psychological effects of skin conditions in children and young people are becoming more appreciated and understood, clinics are beginning to introduce counselling and psychological support for affected patients – this research may highlight another population in whom this support could be beneficial.Lay Summary of Results -
A Qualitative Study of the Psychological Morbidity in Paediatric Survivors of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Dear Editor, Stevens-Johnson Syndrome (SJS) and Toxic epidermal necrolysis (TEN) are rare but devastating skin conditions that lead to significant morbidity and mortality. Previous research has demonstrated psychological sequelae in surviving adults1 . Guidelines were produced for the management of SJS/TEN in children and young people (CYP) which demonstrated important differences between adults and CYP and stated a need for further research into long term outcomes in young patients2. This qualitative study investigates psychological impact that SJS/TEN has on CYP and their families.
All CYP under the age of 18 who had been treated in Oxford over a 12-year period (2007-2019) were identified (23). Of these, 13 (10 male, 3 female) consented to semi-structured interviews. Mean age at time of diagnosis was 6.6 years. The cause was identified as an infection in seven cases, medication in one case and inconclusive in five cases. Duration of hospital(s) stay ranged between 7 and 42 days. Mean time between diagnosis and interview was 8.3 years. Interview questions explored experiences of the event, care during and after discharge and long term sequelae. Interviews were conducted individually and with both child and parent present. This report focuses on thematic analysis of these interviews with representative quotes outlined in Table 1.
CYPs’ physical experiences of their acute illness centred around significant pain and disruption of basic functions such as eating, talking and passing urine. Long-term, most still have ongoing physical complaints, most commonly ophthalmic. Skin scarring or pigment change is frequently a problem and 3 required subsequent circumcision.
Some of the most traumatic memories for both parents and children whilst in hospital were iatrogenic in nature combined with experiences of poor communications. This was frequently associated with venesection but included other interventions such as nasogastric tubes, which one CYP said was inserted without adequate explanation despite feeling she was old enough to understand. Parents also felt invasive interventions surrounding eye care occurred without sufficient communication.
CYP’s subsequent psychological morbidity commonly manifested as night terrors and symptoms of post-traumatic stress disorder (PTSD), as well as symptoms of depression and anxiety for many years afterwards. A longer hospital stay was also more likely to correlate with psychological morbidity. Only 3 went onto get professional psychological support, with a further six retrospectively identifying that they likely had one or more of these psychological diagnoses and expressed a wish that there had been appropriate support available.
Parents were able to recall the events with more clarity than the CYPs themselves. All parents interviewed exhibited health anxiety towards their child subsequent to the event which took various forms. For example, they were quick to present to emergency services for more minor ailments and generally described themselves as being overprotective, acknowledging this as a negative consequence. Some parents suffered more significant symptoms such as generalised anxiety, PTSD and depression for many years after the event.
General understanding of SJS/TEN was limited among interviewees and they reported that resources for this age group were lacking. Parents and CYPs were aware that medications and viruses were generally implicated but most felt that uncertainty around the specific cause and likelihood of recurrence was an additional source of anxiety. Notably, even in cases where infections were reported to be the likely aetiology, most CYPs and parents showed a fear of medications with one claiming to be “terrified” of medication and some avoiding medications entirely.
Families perceived a lack of aftercare feeling they went from intense care in hospital to an abrupt discharge with insufficient information and follow-up. Many reported they would benefit from more ongoing support for their child’s physical needs and the emotional needs of both the parent and child.
The study’s limitations are the relatively small sample size due to the rare nature of this condition. However, response rate and engagement was good and saturation of themes was achieved after ten interviews. The long lag-time between the acute event and interview could be also seen as a limitation. Conversely, this may also allow for more balance and reflection in the responses and allows us to identify longer-term impacts.
This rich dataset of patients’ voices highlights the significant long-term psychological trauma experienced by both child and parent after an episode of SJS/TEN. This was attributed to multiple factors including severity of the illness, inpatient procedures, subsequent long-term physical morbidity, lack of clarity around cause and uncertainty around risk of recurrence. Resources have since been developed for this age-group including a template discharge letter and BAD information leaflet which may address some issues3. However, this research reveals a significant unmet need and an important opportunity to improve services and support for the physical and psychological needs for families who experience SJS/TEN.REC name
London - Hampstead Research Ethics Committee
REC reference
22/PR/0660
Date of REC Opinion
15 Jun 2022
REC opinion
Favourable Opinion