Coping with the Burden of Chronic Skin Disease
Paediatric skin disease burden
The secondary impact of paediatric skin disease
This chapter will describe the burden of paediatric skin disease on afflicted children and their families. The burden of atopic dermatitis, acne and infantile haemangiomas will be reviewed in depth. These skin disorders generally occur in different age groups and are representative of different disease-specific burdens. The related literature includes both qualitative descriptive reports and quality-of-life measurement to report the impact on both the affected child and their family. Opportunities for medical and psychosocial support for those affected by paediatric skin disease will be reviewed.
In addition, the burden of skin disease will be examined through biopsychosocial theory using a multidimensional framework, which covers the burden on patient, caregivers and society. We will explain biopsychosocial theory and examine how it encompasses the multifactorial nature of the paediatric pathology phenomenon and healing potential.
Quality of life is defined broadly as a patient’s ability to enjoy normal life activities. More precisely, health-related quality of life is an outcome that extends beyond traditional views of mortality and morbidity and includes the health dimensions of symptoms, the functional impact, and the broad psychological, social and emotional impact . For paediatric patients, this outcome is not only relevant for the affected child but for their parents and siblings as well. Burden of disease can be defined as how quantity and quality of life are affected by disease. Lastly, coping strategies are defined as the specific efforts, both behavioural and psychological, that people employ to master, tolerate, reduce or minimize stressful events .
1 VanBeek M, Beach S, Braslow JB et al. Highlights from the report of the working group on ‘core measure of the burden of skin disease’. J Invest Dermatol 2007;127:2701–6.
2 Folkman S, Lazarus RS. An analysis of coping in a middle-aged community sample. J Health Social Behav 1980;21:219–39.
Paediatric Skin Disease Burden
The effects of paediatric skin disease on children and their families can be measured using quality-of-life scales, measures of psychological disturbance and general health scales. Quality-of-life scales for children with skin disease address this outcome from a disease-specific, skin-specific or a general health perspective . In addition, for the paediatric population, many scales are age specific to allow more accurate measurement of disease impact. In the past several years, many disease- and skin-specific scales have been developed and validated .
The Children’s Dermatology Life Quality Index (CDLQI) is one such skin-specific instrument that was developed to measure the effect of skin disease on children from 3 to 16 years of age. With this scale, the greatest quality-of-life effects were seen in children with scabies, eczema, psoriasis and acne . This intuitively makes sense, with diseases with greater symptomatology having a higher impact on quality of life. Disease-specific quality-of-life scales have also been developed. For example, for atopic dermatitis, these include the Dermatitis Family Impact questionnaire (DFI), the Infants’ Dermatitis Quality of Life Index (IDQOL), the Childhood Atopic Dermatitis Impact Scale (CADIS) and the Parents’ Index of Quality of Life in Atopic Dermatitis (PIQoL-AD) [4–7]. Moreover, the impact of skin disease on the family can also be quantified. The skin-specific Family Dermatology Life Quality Index (FDLQI) was developed for this purpose . While disease-specific scales such as the DFI and CADIS also measure family impact, the FDLQI scale can be used to measure this important outcome for any skin disease [4,6].
Measures of psychological disturbance are also applied to patients, paediatric and adult, with skin disease. One such study which included teens and adults with acne, psoriasis and atopic dermatitis reported that individuals with skin disease exhibited higher levels of minor psychological disturbances and public self-consciousness, and were more neurotic than control patients . This finding of increased self-consciousness has also been reported in several other investigations of individuals with acne, psoriasis and atopic eczema [10–12].
Self-esteem is the difference between how one actually sees oneself and how one would like to be , making this an important outcome for individuals with disease that affects their appearance. Self-esteem and other psychological sequelae have been investigated in adolescents and young adults with skin disease, acne vulgaris primarily, with reports describing low self-esteem, self-consciousness, frustration, anger, increased depressive symptoms, impaired self-image, feelings of uselessness, embarrassment, anxiety, fewer feelings of pride, and lower self-worth [14,15]. Moreover, appearance-related teasing and bullying is a considerable problem for some children with acne, psoriasis and eczema and has been an under-recognized and seldom reported morbidity in this population . In addition, suicidal ideation attributed to their skin disease has been reported by some patients .
The Burden of Atopic Dermatitis
Atopic dermatitis (AD) is extremely common and presents early in childhood during a critical period for physical and psychosocial development . AD presenting during this period may disrupt the establishment of normal sleep patterns, behaviour and relationships. The multidimensional effects of AD affect not only the afflicted child but the entire family. AD affects the child with symptoms (predominantly itch), activity limitations and behaviour changes, and affects the parents by causing sleep dysfunction, creating an emotional burden and imposing limitations on family and social functioning and activities . Several disease-specific quality-of-life instruments have been developed to quantify this multidimensional impact on children and their families, as previously mentioned [3–7].
Atopic dermatitis affects the emotions and behaviour of children in an age-dependent manner. The most commonly reported emotional symptoms for the young child with AD include irritability, fussiness and increased crying . Parents most often attribute these emotions to the symptom of pruritus which may be central to quality-of-life impairment and the burden imposed by AD. For example, sleep disturbance from pruritus can affect daytime behaviour and productivity . In addition, parents of young children with AD describe their children as being more clingy, fearful and frustrated, and wanting to be held more [19,21]. These psychological disturbances may increase with increasing severity of disease [21,22]. Of note, an association between attention deficit/hyperactivity disorder and eczema has been suggested . Although the authors recognize that this association may be secondary to other disease-related factors such as itching, sleep disturbance or other psychosocial impairment, this finding further defines the potential increased life burden of AD for the affected child and their family .
Limited data exist describing the psychosocial effects of AD on adolescents. Adolescence is a critical time for the development of self-identity and self-esteem  and looking different due to skin disease adversely affects teens during this developmental stage. Adolescents and adults with acne vulgaris report increased anxiety, embarrassment, interpersonal difficulties, social isolation, shame and self-consciousness [26–28]. Adolescents affected by AD probably have similar or greater emotional impact due to their skin disease .
Coincident with its impact on the child, childhood AD affects the emotional, financial and social well-being of their parents [29,30]. Mothers of young children with AD report a decrease in employment outside the home, poor social support, stress about parenting and difficulty with discipline . In addition, parents of children with AD have many worries relating to disease triggers and medications use, including fear of using topical corticosteroids . Increased AD disease severity is strongly associated with a higher impact on the family, and of note, family impact decreases as disease severity lessens [29,32]. This finding highlights the importance of understanding and measuring the burden of disease on the entire family.
The Burden of Acne Vulgaris
Acne is quite prevalent among adolescents and young adults, with over 90% of males and 80% of females being affected by the time they are 21 years old [33,34]. Adolescence is a critical period of life for the development of self-esteem, and when acne or other disfiguring disease occurs during this developmental stage, there is often a negative effect on self-esteem and other psychological outcomes, as mentioned previously [26–28]. Of note, there is an association with depression and suicidal ideation related to acne  and this has been shown in cases of both mild and moderate acne . Additional psychosocial morbidity, including embarrassment with social inhibition and isolation, has also been reported . Moreover, chronic stress and anxiety have been credited with exacerbating the disease process itself .
Quality of life has been measured in patients with acne using the disease-specific Cardiff Acne Disability Index (CADI), which was developed and tested for this purpose . This scale and others, such as the Acne Disability Index and the Acne-QoL, have been used in clinical trials to evaluate response to treatment [38–40].
The Burden of Infantile Haemangiomas and Congenital Malformations
Haemangiomas are among the most common congenital defects, with >60% occurring in a visible location on the head and neck. Complex psychosocial issues exist for parents of children with such a congenital defect and for the child themselves it is comparable to other congenital defects such as cleft lip/palate . As with other skin disease, the degree of parent stress from their child’s haemangioma is variable and not correlated with the surface area or location of the lesion . One mother described her experiences of having a child with a large facial hemangioma thus: ‘Our first years were filled with doctor visits, staring strangers, rude comments and pitying looks. Comments like, “What did you do during your pregnancy to cause that?” were an everyday occurrence’ .
Drotar et al. have studied reaction patterns and coping mechanisms for parents of children with congenital malformations. In a study of 20 parents of children with various malformations, the authors noted a predictable course of parental reactions. Despite a wide variation in the type of underlying malformation, each parent evolved through five stages: shock, denial, sadness and anger, gradual adaptation and finally reorganization with variable time spent in each stage and varying success in the reorganization period. Some parents reported difficulties in becoming attached to these children and reported a fear of not being able to care for their child appropriately. Some continued to search endlessly for a cause and remained isolated from support while others accepted the malformation as a chance occurrence and were able to seek support from friends, family and support groups . The feelings of parents immediately after the birth of children with malformations include grief, guilt, sadness and a sense of loss of their expected ‘normal’ child [44,45]. The heterogeneity of disease and the uncertain and often unpredictable course may lead to feelings of anxiety and a loss of control. The reactions of strangers to children with congenital malformations are another significant source of stress and anxiety for parents. This parental anxiety, along with a perception that the child is vulnerable due to their malformation, may trigger the development of indulgent and overprotective behaviours toward the affected child . The adverse effects on a parent’s quality of life have also been quantified using a haemangioma-specific survey, with high scores noted for parental feelings of disbelief and panic .
Studies investigating the impact of appearance on behaviour and self-esteem suggest that children with craniofacial anomalies are treated differently from children without these defects. The affected children have been shown to be more introverted and to express a more negative self-concept than unaffected children and these negative self-perceptions and lack of self-esteem continue to pose problems for those with congenital anomalies as they become adults [48,49]. These psychosocial difficulties have a negative impact on social functioning and quality of life.
Tanner et al. sought to understand the coping and adaptation mechanisms for children with haemangiomas and their parents . The reactions of parents were similar to those observed by Drotar in parents of children with various malformations as outlined above, including feelings of loss and grief, despite the fact that haemangiomas generally follow a benign course. One particularly distressing aspect for parents noted in this and other similar studies was the reaction of strangers. In addition to negative reactions by strangers regarding the child’s appearance, many parents have reported actual accusations of child abuse. Such social stigmatization, along with a sense of panic or fear associated with the presence of the haemangioma, has a significant psychological impact on these parents . Parents may choose to minimize contact with strangers and maximize contact with familiar adults as a coping mechanism for both themselves and their children.