Effect of Angiofibromas on Quality of Life and Access to Care in Tuberous Sclerosis Patients and Their Caregivers.

BACKGROUND/OBJECTIVES: Facial angiofibromas (AF) have the potential to cause disfigurement in children with tuberous sclerosis complex (TSC). Facial disfigurement can impact the quality of life (QoL) of individuals and their families, leading to negative psychosocial outcomes. QoL has not been studied in TSC patients with AF. METHODS: We conducted a cross-sectional survey study to investigate QoL of TSC patients with AF and their caregivers and to explore the current state of access to treatment for AF. TSC patients and caregivers in TSC clinic at Boston Children's Hospital and through the Tuberous Sclerosis Alliance were recruited to complete QoL surveys including the CADIS, CDLQI, and Skindex-teen questionnaires, and a survey on access to treatment of AF. RESULTS: Fifty-eight patients with TSC and 161 caregivers participated in the study. Caregivers of patients with AF had significantly poorer QoL scores compared to caregivers of those without AF, as measured by a modified CADIS questionnaire (mean 31.7 vs. 11.7, p = 0.004). Among patients with AF, those who received treatment had significantly better QoL scores compared with those without treatment, as measured by the CDLQI (mean 3.8 vs. 9.5, p = 0.001). Forty-one and two-tenths percent of subjects reported never receiving treatment for AF. Forty-seven and three-tenths percent of subjects were prescribed topical rapamycin, 47.7% of whom experienced difficulty with insurance coverage. CONCLUSIONS: Presence and lack of treatment of AF significantly impacts QoL in TSC patients and their caregivers. Access to care for AF is limited by multiple factors and should be addressed by clinicians working with this patient population.

Exploring supportive care needs and experiences of facial surgery patients.

Research suggests that an important determinant of psychosocial outcomes following disfiguring surgery is social support. Healthcare professionals are in a position to offer emotional support to patients undergoing surgery, but evidence regarding the experience of supportive care among facial surgery patients is sparse. This study explored the experiences of facial surgery patients and their friends or family with regard to support from healthcare professionals. Twenty-nine facial surgery patients and nine significant others (eight marital partners and one close family member) participated in indepth interviews and focus groups, and the data were analysed using a grounded theory form of coding. Analysis identified three main categories of support: informational, practical and emotional. Findings also showed discrepancies between patients' need for emotional support and provision by healthcare staff.

Excision of squamous cell carcinoma of forehead.

By the time 77-year-old Mr Mack was admitted to the plastic surgery ward, the 10-week-old recurrent swelling on the right side of his forehead was about 6 cm in diameter and cystic in appearance. Histologically confirmed as a squamous cell carcinoma, the growth did not appear to be fixed to the underlying bone, but nor was it fully mobile which suggested that the malignancy may have spread to the deep fascia. Red and shiny on top, two or three discharging puncti were evident.