Mixed Method Study to Assess Behcet’s Syndrome Impacts on Quality of Life of Children

Abstract

Introduction: Behcet’s Syndrome (BS) is a rare, chronic inflammatory condition that can significantly affect the daily lives of children and young people (CYP). While BS is well-documented in adults, there is limited research on how it impacts CYP, particularly in terms of their quality of life (QoL) and access to appropriate healthcare, including dental care. Oral ulcers, a hallmark symptom, can cause considerable pain and difficulties with eating, speaking, and maintaining oral hygiene. This thesis explores the challenges faced by CYP with BS, the gaps in healthcare services, and the need for including a paediatric dentist within the multidisciplinary care. Method: This research is based on three interconnected studies: Literature Review – A comprehensive review of existing research on BS in CYP, highlighting the condition’s clinical manifestations, diagnostic challenges, and its impact on QoL, with a focus on oral health. A retrospective service evaluation was conducted using medical records of 34 CYP diagnosed with BS who attended the Multidisciplinary Clinic (MDT) at Alder Hey Hospital (AHH) between January 1, 2012, and December 31, 2021. The primary aim was to assess the oral health needs of these children. To achieve this, data were collected on their symptoms, clinical characteristics, referral pathways, and management approaches. As this was a service evaluation, ethical approval was not required, and the project was formally registered with the local governance team. Qualitative Study: Semi-structured interviews were conducted remotely via Zoom with CYP aged 8-18 years diagnosed with BS. Recruitment was facilitated by Behcet’s UK charity and AHH. Thematic analysis was conducted using an inductive approach to identify key themes. Ethical approval was granted by the Health Research Authority (REC; IRAS: 318222). Results: Service evaluation: Thirty-four children diagnosed with BS attended the MDT at AHH between 2012 and 2021, with an average age of 12.8 years (range: 5-17). A family history of BS was present in 26.5% (n=9) of cases. Oral ulceration was the most common initial symptom, affecting 97.1% (n=33), with frequent recurrences every 2-3 weeks. Other common findings included joint pain (79.4%), genital ulceration (44.1%), gastrointestinal symptoms (82.4%), and ocular involvement (41.1%). Only 14.7% (n=5) had a confirmed BS diagnosis, while 85.3% (n=29) remained probable cases. General paediatrics (47%) and rheumatology (17.6%) were the primary referral sources, with ophthalmology (35.3%) and physiotherapy (29.4%) receiving the highest number of onward referrals. Paediatric dental assessments were documented in only 52.2% (n=18), highlighting potential gaps in oral health management. Qualitative interviews: revealed Five key themes: (1) Living with BS Symptoms: CYP experienced disruptions to daily functions, including eating, sleeping, and maintaining hygiene, driven by symptoms such as oral and genital ulcers, fatigue, and joint pain. These challenges hindered their ability to socialise and participate in routine activities. (2) Living with Uncertainty: The unpredictable nature of BS fostered anxiety, making it difficult to plan routines or attend school consistently. (3) Fear of Disbelief and Misjudgement: Participants reported being dismissed by teachers, peers, and healthcare professionals due to the invisibility of symptoms, exacerbating feelings of isolation. (4) Support Systems: Mixed experiences with school accommodations and healthcare highlighted systemic gaps, including inconsistent support and delayed diagnoses. (5) Walking the Tightrope of Treatment and Relief: CYP expressed frustration over limited relief despite ongoing treatment, coupled with the burden of medication side effects. Conclusion: The findings from this thesis sheds light on the challenges faced by CYP with BS, particularly in relation to their daily lives, healthcare experiences, and oral health needs. The service evaluation revealed that while oral ulcers were the most common symptom, paediatric dental assessments were not consistently recorded, suggesting gaps in oral healthcare within the MDT. The qualitative study highlighted how BS disrupts essential daily activities, causing physical discomfort, emotional distress, and social isolation. Many CYP struggled with the unpredictability of their symptoms, difficulties accessing appropriate care, and a lack of understanding from professionals. These findings underscore the need for a more integrated approach to care, with paediatric dentists playing a key role in the MDT. Addressing oral health early could help prevent long term dental complications and improve overall well-being. Greater awareness, better referral pathways, and improved support systems are essential to ensuring CYP with BS receive the care they need. Future research should explore ways to enhance early diagnosis, personalised treatment, and coordinated multidisciplinary management.