Growing up with juvenile vulvar lichen sclerosus: the experiences and care needs of adult women with lichen sclerosus since childhood - a qualitative exploration.

BACKGROUND: Vulvar lichen sclerosus (VLS) is a chronic remitting condition that affects the genital skin of females of all ages. Although qualitative studies have been conducted that have focused on women with VLS in mid-life or beyond, less is known about the experiences of individuals with VLS from childhood or adolescence onward. OBJECTIVES: To gain an understanding of the experiences of women with a history of juvenile VLS (JVLS) with regard to the impact of the disease on their personal lives, and their experiences and needs regarding care and guidance. METHODS: A qualitative study was conducted consisting of 27 in-depth face-to-face interviews with adult women with a histologically confirmed history of JVLS, striving for maximum variation and saturation. Interviews were audio recorded and transcribed verbatim. A thorough thematic content analysis was performed. RESULTS: Three main themes were identified. Theme 1 was named 'Varying impact of living with JVLS': women experienced diverse emotional and physical impacts, from shame and denial to complete acceptance, from restrictions in daily functioning to no limitations; they felt hindered by their own lack of knowledge about JVLS and generally expressed positivity in sharing their experiences with people close to them. Theme 2 was entitled 'Finding one's way in care and guidance': while navigating care and guidance, women often felt hindered by knowledge gaps among healthcare professionals (HCPs), lack of continuity in care and guidance, lack of life phase-adjusted and future-oriented information provision, inadequate guidance around life events and insufficient monitoring of determinants of treatment adherence. Theme 3 was named 'Need for patient-tailored care involving appropriate and compassionate care and guidance': patients stressed the need for age-appropriate and life phase-adjusted information, guidance around life events and compassionate contact with knowledgeable HCPs aware of the determinants of treatment adherence and influencing factors. CONCLUSIONS: Age-appropriate, life phase-adjusted, individually tailored care for women diagnosed with VLS in childhood or adolescence is needed. Care and guidance from childhood onward should encompass a standard of care adapted to the individual as their needs change over time. This involves taking interpersonal differences into account, including differences in support network and coping strategies. These findings demonstrate the need to improve awareness and knowledge about JVLS/VLS among HCPs, especially primary care providers, and among the general public.

Vulvar lichen sclerosus (VLS) affects up to 1 in 30 women and at least 1 in 900 girls. Contrary to what was thought before, it is now known that childhood VLS generally does not disappear at puberty. This study was done to better understand the experience of women diagnosed with VLS at a young age. Adult women who had VLS as a child or adolescent took part, regardless of whether they still had the disease or not. Women were interviewed about living with VLS from childhood up to the present and their experience with the care they had received. They were also asked to provide their thoughts on how care and guidance could be improved. Recurring themes were considered. Our findings showed that adult women diagnosed in childhood or adolescence experienced a range of emotional and physical impacts of living with JVLS. Care and guidance had many shortcomings from the time of diagnosis onward. VLS often went unrecognized or untreated due to the lack of knowledge of healthcare providers. Many women felt they could not ask questions about treatment, sexuality or childbirth at consultations. When given the opportunity to ask questions during regular check-ups, women felt supported by the help they received. Overall, suggestions were made to improve healthcare professionals' knowledge and understanding of JVLS and VLS. Improving public knowledge of vulvar disease and the vulva in general was suggested.

Outcome Measures in Adult Vulvar Lichen Sclerosus: A Case Series of Women Diagnosed as Juveniles.

OBJECTIVES: Studies on the consequences of juvenile vulvar lichen sclerosus (JVLS) in adulthood are limited. A number of measuring tools are available for analyzing adult vulvar lichen sclerosus (VLS), but these have not been applied in studies on JVLS. The aim is to study physical findings, quality of life, sexual well-being, and self-image in adult women with a history of juvenile VLS. MATERIALS AND METHODS: Adult women with a biopsy proven history of JVLS were recruited to be examined and surveyed using available standardized measurement tools. This took place in an outpatient setting by physicians who were not involved in the treatment of participants. RESULTS: Twenty-seven women (median age 29 years) with a history of JVLS and median time since biopsy of 19.5 years were recruited. Of these women, 59% currently had symptoms, 63% had signs of active disease, and 85% had moderate to severe architectural changes. Despite these residual signs, vulvar specific-quality of life and vulvar self-image scored favorably while generic health-related quality of life was somewhat effected. CONCLUSIONS: JVLS has consequences in adulthood involving physical findings and vulvar quality of life. The use of standardized outcome measures for clinical practice and research purposes facilitates a better understanding of the sequelae to JVLS.

Gonadal function in boys with newly diagnosed cancer before the start of treatment.

STUDY QUESTION: Are Inhibin B and testosterone levels reduced in boys with newly diagnosed cancer prior to therapy? SUMMARY ANSWER: Pretreatment serum levels of Inhibin B and testosterone are significantly reduced in boys with newly diagnosed cancer, compared to reference values. WHAT IS ALREADY KNOWN: Disease-related gonadal impairment has been demonstrated in girls and young women diagnosed with cancer, prior to therapy. STUDY DESIGN, SIZE, DURATION: We conducted a descriptive study in boys newly diagnosed with cancer between January 2006 and February 2014. PARTICIPANTS/MATERIALS, SETTING, METHODS: Serum Inhibin B and testosterone levels were determined in 224 boys, up to the age of 18 years, with newly diagnosed cancer prior to therapy. Hormone levels were compared with age-matched reference values. The cohort consisted of patients with acute lymphoblastic leukaemia (ALL), acute myeloid leukaemia (AML), Hodgkin lymphoma (HL), non-Hodgkin lym-phoma (NHL), nephroblastoma, neuroblastoma and sarcoma. MAIN RESULTS AND THE ROLE OF CHANCE: This study demonstrates reduced serum levels of Inhibin B in boys with newly diagnosed cancer, compared to reference values (standard deviation score (SDS) -0.9, P < 0.001). Median Inhibin B level in patients was 103.5 ng/l (range 20-422). Of all patients, 78.6% showed Inhibin B levels below the 50th percentile, and 58.5% had Inhibin B levels below the 25th percentile. Serum testosterone levels were significantly lower than the reference range population (SDS -1.2, P < 0.001). Median testosterone level in pubertal patients was 7.3 nmol/l (range 0.1-23.6). No correlation with clinical signs of general illness and hormone levels were observed. LIMITATIONS, REASONS FOR CAUTION: In this study, reproductive hormone levels were compared with age-matched reference values. Future studies may compare reproductive hormone levels with case controls. WIDER IMPLICATIONS OF THE FINDINGS: Future longitudinal studies are necessary to determine whether pretreatment impaired gonadal function at the time of cancer diagnosis is an important determinant of ultimate recovery of spermatogenesis after treatment and later on in adulthood. STUDY FUNDING/COMPETING INTERESTS: W.v.D. was supported by the Pediatric Oncology Center Society for Research (KOCR), Rotterdam, The Netherlands. A.-L.L.F.v.d.K. was supported by EU FP7 PanCare LIFE study. The authors have no conflicts of interest.