Psychiatric Symptoms and Quality of Life in Patients Affected by Epidermolysis Bullosa

Margari, Francesco; Lecce, Paola Alessandra; Santamato, Wanda; Ventura, Paolo; Sportelli, Nicola; Annicchiarico, Giuseppina; Bonifazi, E.

doi:10.1007/s10880-010-9205-4

Cited by 49 publications

(60 citation statements)

References 13 publications

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“…In the Dutch QOLEB significant differences were seen on the functioning and overall scale, but not on the emotions scale, suggesting one of 3 explanations: either that the mental burden of EB is similar in milder and more severe subtypes, that patients with more severe EB subtypes have acquired emotional resilience due to the severe impact of living with EB, or that the QOLEB is not appropriate to distinguish between emotions. Such decreased prevalence of emotional burden is also seen with results from a study by Margari et al (13), who found that 80% of EB patients experienced sub-threshold psychiatric symptoms, in particular depression, anxiety, and behaviour disturbances, but that there was no close correlation between these symptoms and the clinical severity of EB (13). Emotional resilience was not explored by Margari et al (13) but has been proposed by Frew et al (3) to explain the lack of consistent quantitative data regarding emotional burden in EB.…”

Section: Discussionsupporting

confidence: 64%

“…Several studies have focused on qualitatively describing the impact of EB on patients' lives (7)(8)(9)(10)(11). Quantitative measurement of HRQoL has been performed using both generic and dermatology-specific instruments in EB patients (6,(12)(13)(14)(15). However, due to ceiling effects and content validity issues of these instruments, the accuracy of the measurements is questionable.…”

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confidence: 99%

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Health-related Quality of Life in Epidermolysis Bullosa: Validation of the Dutch QOLEB Questionnaire and Assessment in the Dutch Population

Yuen¹,

Frew²,

Veerman³

et al. 2014

Acta Derm Venerol

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Wing Yan Yuen, Department of Dermatology, University Medical Center Groningen, Hanzeplein 1, NL-9700 RB Groningen, The Netherlands. E-mail: w.y.yuen@umcg.nl Epidermolysis bullosa (EB) consists of a spectrum of heritable trauma-induced blistering diseases of skin and mucous membranes (1). Many subtypes exist, and their clinical severity is variable, ranging from an occasional palmoplantar blister to childhood lethality (1). The health-related quality of life (HRQoL) is the impact of a disease on the physical, psychological, and social health of a patient (2). Defining HRQoL in EB patients is important in patient care and management (3). Furthermore, in recent years more focus has been placed on finding specific treatments for EB and measuring HRQoL can help in assessing the efficacy of these new treatment modalities (4, 5). Also, measuring and comparing HRQoL to other diseases can help assign funding to this rather unknown and rare disorder (4, 6). Measuring HRQoL is complicated by the wide range of phenotypes in EB, which all have their own clinical severity (3). Several studies have focused on qualitatively describing the impact of EB on patients' lives (7-11). Quantitative measurement of HRQoL has been performed using both generic and dermatology-specific instruments in EB patients (6, 12-15). However, due to ceiling effects and content validity issues of these instruments, the accuracy of the measurements is questionable. It has been hypothesised that, especially in the more severe EB subtypes, there has been an overestimation of the HRQoL (3). To overcome these problems, Frew et al. (3) developed and validated an EB-specific HRQoL measurement tool for older children and adults that can be used across all EB subtypes, called the QOLEB (quality of life in EB). Translation of the QOLEB, which was created and validated in English in Australia, to other cultures and languages may provide reliable comparisons of HRQoL in EB and the efficacy of clinical interventions across different countries (3). The aim of this study was to develop a validated and reliable QOLEB in Dutch, and to assess HRQoL in Dutch EB patients. MATERIALS AND METHODSThis study was performed at the Center for Blistering Diseases at the University Medical Center Groningen, which is the single national referral centre for EB in the Netherlands. Ethical approval was granted by the Medical Ethical Committee of the University Medical Center Groningen in the Netherlands. A signed consent form was obtained from all participating patients. Study measurement toolsThe English QOLEB is a validated and reliable adult EBspecific HRQoL tool consisting of a 17-item questionnaire, and it was developed by 2 of the authors (JWF and DFM) (3). The QOLEB measures 2 factors: functioning (questions 1-7, 9-10, 12-13, 15) and emotions (question 8, 11, 14, 16-17). For each question 4 optional answers exist that are scored from 0 to 3 points, in which a higher score represents a worse HRQoL. The functioning scale ranged from 0-36, the emotions scale from 0-15, and the ove...

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Section: Discussionsupporting

confidence: 64%

mentioning

confidence: 99%

Health-related Quality of Life in Epidermolysis Bullosa: Validation of the Dutch QOLEB Questionnaire and Assessment in the Dutch Population

Yuen¹,

Frew²,

Veerman³

et al. 2014

Acta Derm Venerol

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“…A large proportion of persons suffering from the disease consists of children and young adults. The studies are conducted on: -the demand for multi-dimensional care [5], including psychological support [3,4,6]; -the feeling of differentness and exclusion from peer groups [6,7]; -the influence of EB on the quality of life [2,8,9], -the influence of EB on the patient's family [8][9][10].…”

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confidence: 99%

The need of social support, life attitudes and life satisfaction among parents of children suffering from epidermolysis bullosa

Mącik¹,

Kowalska-Dąbrowska²

2015

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Introduction. Epidermolysis bullosa (EB) is a group of rare genetic skin disorders. Much is known about the disease from the medical perspective, but there are still only a few studies about the psychological condition of the patient or his family. The birth of a child with EB may become a trauma for the parents. Parents' feelings also vary depending on the type of EB. Objective. The main aim of the study was to determine whether, and in what way, having a child suffering from EB influences parents' life and psychological well-being. Due to this aim, life satisfaction, life attitudes and the need for social support were measured. Material and methods. Twenty-one parents of ill children were studied (13 mothers, 8 fathers; 16 parents had one child with EB and 5 parents two). Six children suffered from EB simplex, 1 child from junctional EB and 14 from dystrophic EB. The course of the disease was mild in 9 children and severe in 12. As the control group 22 parents of healthy children were chosen. Results. The parents of ill children have a greater need for social support from family, both general, and instrumental, appraisal and emotional. The need for support from medical institutions is at a low level. In life attitudes there are also significant differences. Parents of ill children have a higher level of existential vacuum, and a lower level of life control and life purpose. Also the life satisfaction is lower. Conclusions. The results of our study indicate that a child suffering from EB is a heavy burden for the parents. streszczenie Wprowadzenie. Epidermolysis bullosa (EB) jest grupą rzadkich genetycznych chorób skóry, pojawiających się raz na kilkaset urodzeń. Z medycznego punktu widzenia choroba jest dobrze poznana, ale wciąż niewiele jest opracowań dotyczących problemów psychologicznych chorego i jego rodziny. Narodziny dziecka z EB mogą stać się specyficznym rodzajem traumy dla jego rodziców, a ich odczucia zależą również od typu i przebiegu choroby. W części piśmiennictwa sugeruje się, że EB jest obciążeniem nie tylko dla chorego, lecz także dla jego rodziny. Cel pracy. Głównym celem prezentowanych badań było sprawdzenie, czy i w jaki sposób posiadanie dziecka chorego na EB wiąże się z różny-the need of social support, life attitudes and life satisfaction among parents of children suffering from epidermolysis bullosa zapotrzebowanie na wsparcie społeczne, postawy życiowe i satysfakcja z życia rodziców dzieci chorych na epidermolysis bullosa

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“…Several studies have investigated this aspect and concluded that general quality of life in EB patients can be poor (Horn et al, 2002;Tabolli et al, 2009;Jeon et al, 2016;Angelis et al, 2016), but data on psychological aspects of EB have been conflicting. Some have reported no manifestation of anxiety and/or depressive symptoms (Fortuna et al, 2016) or other psychiatric disorders (Andreoli et al, 2002), whereas others have found a high prevalence of psychosocial problems and psychiatric symptoms (Margari et al, 2010). …”

Section: Discussionmentioning

confidence: 99%

Oral Health-related Quality of Life in Patients with Dystrophic Epidermolysis Bullosa

Fortuna¹,

Aria²,

Whitmire³

et al. 2016

AJOM

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Background: Dystrophic epidermolysis bullosa (DEB) is a devastating mucocutaneous inherited disorder that can have a remarkable impact on the oral cavity.Objective: To understand the relationships between oral health-related quality of life and general quality of life in DEB patients versus a control group.Methods: Twenty-eight DEB patients and 26 healthy individuals completed the following battery of scales: Oral Health Impact Profile (OHIP-49), the RAND Short Form-36 (SF-36), the Hamilton Rating Scale for anxiety (HAM-A) and depression (HAM-D).Results: All 7 dimensions in OHIP-49 demonstrated a statistically significant difference in functional limitations (Median:13.0; IQR:10.3-20.0 vs Median:7.0; IQR:2.0-9.0; p<0.001) and physical disability (Median:11.5; IQR:4.0-15.0 vs Median:0.0; IQR:0.0-2.3; p<0.001) in DEB patients vs controls, respectively. In RAND SF-36, physical functioning and general health were highly significant (p<0.001), whereas limitation due to physical health (p=0.043) and pain (p=0.010) were moderately significant. A positive correlation was found between oral health-related quality of life and anxiety (p=0.005 and p=0.03 in DEB patients and controls, respectively) and depression (p=0.045 and p=0.001 in DEB patients and controls, respectively). In 44 DEB patients, no correlation was found between general quality of life and anxiety and depression, oral health-related quality of life and oral-pharyngeal severity score, number of oral-pharyngeal sites involved and general quality of life, as well as oral health-related quality of life between patients with dominant and recessive DEB forms (p>0.05). Conclusions:The oral health-related quality of life is highly impaired in patients with DEB. General quality of life was affected only for some dimensions and was not significantly correlated with oral health-related quality of life.

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Psychiatric Symptoms and Quality of Life in Patients Affected by Epidermolysis Bullosa

Cited by 49 publications

References 13 publications

Health-related Quality of Life in Epidermolysis Bullosa: Validation of the Dutch QOLEB Questionnaire and Assessment in the Dutch Population

Health-related Quality of Life in Epidermolysis Bullosa: Validation of the Dutch QOLEB Questionnaire and Assessment in the Dutch Population

The need of social support, life attitudes and life satisfaction among parents of children suffering from epidermolysis bullosa

Oral Health-related Quality of Life in Patients with Dystrophic Epidermolysis Bullosa

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