"یک اتاق پر از غریبه ها در هر روز": تاثیر روانی اجتماعی پروزوپاگنوزیا تکاملی بر روی کودکان و خانواده های آنها

Abstract Objective Individuals with developmental prosopagnosia (‘face blindness’) have severe face recognition difficulties due to a failure to develop the necessary visual mechanisms for recognizing faces. These difficulties occur in the absence of brain damage and despite normal low-level vision and intellect. Adults with developmental prosopagnosia report serious personal and emotional consequences from their inability to recognize faces, but little is known about the psychosocial consequences in childhood. Given the importance of face recognition in daily life, and the potential for unique social consequences of impaired face recognition in childhood, we sought to evaluate the impact of developmental prosopagnosia on children and their families. Methods We conducted semi-structured interviews with 8 children with developmental prosopagnosia and their parents. A battery of face recognition tests was used to confirm the face recognition impairment reported by the parents of each child. We used thematic analysis to develop common themes among the psychosocial experiences of the children and their parents.

Introduction Faces are the most distinctive cue to a person's identity [1] and, arguably the most important visual stimulus in our lives [2]. Very early in life we use faces to recognize our caregivers and interact with them. In childhood, face recognition is important for making friends and developing social skills. As children mature, face recognition plays a role in finding partners, building careers, and maintaining social relationships. Thus, face recognition is important for both interpersonal development and status within the social world, beginning early in life and extending throughout adulthood. The importance of face recognition is highlighted by cases of developmental prosopagnosia (DP), a neurodevelopmental disorder characterized by severe face recognition difficulties in the absence of deficits to low-level vision and intellect [3], [4] and [5]. Although DP was once considered rare, recent reports suggest that it affects 2% of the population [6], [7] and [8]. Despite this relatively high prevalence, only one study has directly examined the psychosocial consequences of DP in adults [9]. Participants reported feelings of embarrassment, guilt, and failure as a result of their face recognition difficulties. They indicated fear and avoidance of social situations, and, in extreme cases, chronic anxiety leading to long-term social isolation, limited employment opportunities, and loss of self-confidence. The authors likened the psychosocial consequences of DP to those resulting from other disorders like stuttering and dyslexia, that are commonly afforded special support and accommodations [9]. These findings led the authors to conclude that DP can have a lasting effect on formation and maintenance of social relationships. Only one case study has examined the social consequences of DP in childhood [10]. ‘Steve’ (13-years-old) depended on non-facial visual cues such as clothing and hairstyle to identify others. Via semi-structured interview he reported specific concerns with academics, social interactions, and safety. The author concluded that, “children with prosopagnosia rarely have a wide circle of friends because friendships are difficult to develop and keep” (p.285) [10]. In the present study, we conducted semi-structured interviews with eight children and their parents. The goal was to use thematic analysis to extract themes that would provide insight in to the psychosocial consequences of impaired face recognition in a group of children with DP.

Results Analysis of the child data set identified three main themes that covered their awareness of their difficulties, the extensive use of coping strategies, and the social implications of their face recognition deficits. These themes were classified as: 1) I am aware, 2) My coping strategies, and 3) It's not funny. Three parallel themes were also found in the parent data set: 1) I wish I knew, 2) Helping my child cope, and 3) It's difficult knowing that my child is struggling (Fig. 1). A brief narrative of each theme, with example quotations, is provided below. More extensive quotations are included in the Supplementary material 4 and Supplementary material 5. Child and parent themes. Illustration of the relationship between child and ... Fig. 1. Child and parent themes. Illustration of the relationship between child and parent themes. Figure options Quantitative measures of the prevalence of shared experiences can be misleading in non-random sample studies where participants may not be representative of the population of interest. Thus, rather than quantifying the number of participants whose reports contributed to a given theme, we use qualitative measures (e.g. all, some, many), to convey general impressions [9]. Children's themes 1) I am aware This theme represents the rather acute awareness of the majority of the children about their face recognition difficulties. All but the youngest child interviewed expressed a direct awareness of their face recognition difficulties. Some children offered information about their difficulties directly: KF: ….and what are you not so good at? Rose (12) 2: … recognizing people, that's the main thing (L99/290) 3 Other children required an introduction to the topic of face recognition, at which point they spoke in depth about their difficulties. Some children described a difficulty remembering names, possibly misattributing their face recognition difficulties. For example, John (10-years-old), initially reported, “…I'm not good at remembering names” (L107/269), but when specifically asked about face recognition, he explained, “Ermm… faces are what I actually get mixed up”, (L144/269). 2) My coping strategies All children volunteered information about their coping strategies, which ranged in complexity from asking a person's name, to attending to context, and/or remembering non-facial information about appearance (Table 4). Interestingly, many children reported using more than one strategy. For example, Lorraine described a rather complex process of deduction: Lorraine (6): So all I do to find the right Holly is to look at both Hollys and say OK, so I want the one with [pause] a striped shirt [pause], light colored skin [pause], the hair like I have, and also no bracelets… (L19/312) Table 4. Summary of coping strategies discussed by the children Asking names outright Voice Clothing/style Listen for parents using names Skin color Asking parents to tell names Hair style Relying on parents to tell names Jewelry Identity badges Teeth Not using names Eyes Face shapes and sizes Table options Other children reported memorizing clothing, and hair color, style, and length. Despite serving as a practical solution to recognition difficulties, some children reported that these visual cues were unreliable: John (10): … [my math teacher] got her haircut and it confused me a lot (L239/269) Perhaps to overcome the drawbacks of the visual coping strategies above, other children reported relying on more stable visual clues such as face shape and size, or voice recognition. The children also described using contextual clues or resources in their environment to identify others. For example, Lorraine reported using name badges when available. Other children discussed how their parents played a role in buffering their face recognition difficulties: Harry (9): …I usually listen to what Dad or Mom says and follow them (L262/365) Due to the fallibility of visual, verbal, and environmental cues, most children described the avoidance of names as the most reliable strategy. 3) It's not funny Most children expressed negative feelings about their face recognition difficulties, explaining that, while others might find it humorous, they do not. The eldest boy, Thomas, described his experiences plainly: KF: …how does it make you feel? Thomas (14): Embarrassed mostly yeah KF: Why do you think you feel embarrassed? Thomas: It's more when I, say, call them by the wrong name or something and they say, “that isn't my name” or something like that, but it's just awkward you know (L60/179) Rose also reported finding the difficulties upsetting: Rose (12): … It does bother me and it does get on my nerves if it happens over and over (L108/290) KF: Does it stop you from doing anything? Rose: … sometimes it feels like I can't hang out or do what my friends wanna do, yeah Overall, many of the children, especially the older ones, reported negative feelings associated with an inability to recognize faces. When describing their experiences, they expressed that although recognition mistakes might be perceived as humorous to others, they did not find it funny themselves and were oftentimes emotionally hurt by these experiences. Furthermore, some children explicitly described situations in which failure to recognize others encouraged them to remove themselves from, or avoid, social situations. Parents' themes Parent interviews provided particular insight into how the family copes with a child's face recognition difficulties. For two families (Lorraine's and Thomas'), these experiences may have been affected by the presence of prosopagnosia in the parent as well. Three themes were developed following interaction with the data, all closely paralleling themes from the children's interview (see Fig. 1). 1) I wish I knew There was an overwhelming sense that parents struggled to understand their child's face recognition difficulties. Many parents reflected on their initial feelings that something was not quite right: Joanne (John, 10) 4: …when he was younger…he came across as not very friendly… kids… would say hello to him and he would not even not say their names…at some point down the line, he mentioned that he didn't even know who they were… then it started to kind of come up in my mind that maybe this was an issue for him (L64/279) Some parents also expressed a degree of doubt regarding their child's face recognition impairment and occasionally described frustration about not understanding their child's experiences: Sophie (David, 10): … I wish I could see what he saw… I wish I knew (L126/249) To help confirm suspicions or dispel doubt, parents drew on examples of occasions in which their child mistook a stranger for a parent, failed to recognize someone familiar, or even misidentified a photograph of themselves (see Supplementary Material 5). When more clarity was gained, many parents described empathy for their child: Joanne (John, 10): …and I thought, ‘well no wonder you don't want to be friendly and talk to some of these kids because you don't know who they are’, it's a room full of strangers every day (L71/279) as well as vindication: Lucy (Thomas, 14): … you know once you learn the word [prosopagnosia] then I just felt vindicated, like now I know what it is; now I can deal with it (L71/593) Given the limited awareness of prosopagnosia in the general public, some parents reported fearing that they would be judged: Emma (Chloe, 5): I mentioned it and they were, like, ‘Oh how odd’, cuz most people have never heard of it, right? They're all, like, ‘Oh, weird’, but then … they all thought, like, maybe I was the crazy mom (L168/227) In summary, all parents expressed experiencing some degree of distress related to either their uncertainty regarding their child's face recognition difficulties, and/or the lack of understanding of what their child is experiencing both perceptually and emotionally. In addition, parents expressed anxiety about how their concerns are perceived by other parents and caregivers. 2) Helping my child cope The children's theme, ‘My coping strategies’, illustrated that the children are aware of the methods they use to compensate for face recognition difficulties. All parents discussed noticing and encouraging their child's strategies: Sophie (David, 10): …I think if he doesn't know someone, he's very good, he just doesn't use names (L106/249) They also reported elements that they thought were important to the children, but that were not disclosed by the children themselves. For example, some parents suggested that their child is drawn to other children with distinctive characteristics: Julia (Andrew, 8): …there was like one Indian kid in his class and everybody was white so he was friends with the Indian kid (L13/188) Chloe and Lorraine's moms both noticed that their children often use friendliness as a strategy to disguise difficulties: Abigail (Lorraine, 6): … she's so just generally universally friendly to everybody, umm, maybe that is her tactic: if you're just always consistently nice and chatty, and have your guard down with every single person in your classroom, then… it doesn't matter who they are maybe (L225/285) As reported by some of the children, several parents recounted that their child checks with them about a person's identity. For example: Joanne (John, 10): …I know he's comfortable talking to me or, you know, just the fact that he'll stop and go ‘Mom, do you think I'll recognize them?’ or ‘Mom, which one…’(L170/279) Parents described trying to help their child recognize others by providing hints, such as where particular children are seated, pointing out distinctive clothing, and stating the names. Some parents even reported trying to teach coping strategies: Emma (Chloe, 5): …cuz I said, ‘well how did you know that was Miss Roberts?’, and she said, ‘oh, when I heard her voice,’ and then, you know, helping her, say, ‘okay, then you gotta get people talking’… (L61/227) Because of the difficulties with face recognition, mothers felt the need to protect their children, often reporting coming to their defense when others perceive them as rude or “stand-offish,” encouraging empathy from other children, and preventing situations in which their child might experience anxiety related to face recognition. However, some parents reported that they felt their attempts at doing so had failed: Julia (Andrew, 8): … I guess I've learned that I can't protect him (L75/188) Other parents felt it was important to find the right balance between helping their child and allowing him or her to develop the skills for themselves: Emma (Chloe, 5): … I want to [tell her who someone is] sometimes so she doesn't feel awkward, but also learning to step back a little bit and let her work it out (L45/227) Within the context of helping their child to cope, many parents reported feeling concerned about their child's safety in terms of public events, such as getting lost on field trips, and exposure to strangers: Jill (Rose, 12): … a stranger pulled up to the next door neighbor's house and got out of the car and she approached him and said, “hey, you wanna have a look at my bugs?”… I brought her [into the house] and was very upset with her and said, “Rose, I've just told you we don't talk to people that we don't know” and she said, “well that's Mr. Jones” … she said, “he's got glasses on, aren't those his glasses?” (L64/221) Jill also gave an honest account of the impact this had on her parenting of Rose: Jill (Rose, 12): I've limited her social opportunities in order to keep her safe, so I think socially [children with DP] are a bit hindered when you have to worry about safety….(L10/221) 3) It's difficult knowing that my child is struggling Many children interviewed reported negative emotions associated with face recognition difficulties. Similarly, parents expressed concerns about the emotional impact of DP on their children. Part of this theme was derived from the parents' tone and apparent swelling up of emotion that occurred during the interviews: Jill (Rose, 12): …we're still trying to figure out how to help our child cope emotionally …. I try not to ask her too much because she does get emotional about it, I mean just instantly just the swelling up of emotion because she- I think it's hard for her (L110/221) Joanne (John, 10): … when I tried to broach the subject with him… he was upset, he didn't want to talk about it; he didn't like it…he got upset, he actually cried a little bit (L137/279) However, some parents felt unsure about the emotional impact, particularly when considering their child's social skills: Jane (Lorraine, 6): …I don't seem to see any avoidance, Lorraine is so social and just outgoing and so far if it bothers her I don't see any evidence of it… (L227/174) All parents felt a need to understand their child's experiences to help them to cope and build long-lasting friendships: Joanne (John, 10): …I feel that there's a lot of potential for friendships in his life that perhaps don't get to be fulfilled because he doesn't always come across as friendly as maybe he would if he were able to recognize faces. (L18/279) The parents clearly expressed a degree of anxiety and sadness about their child's struggles. Furthermore, parents emphasized the need for increased awareness of DP among educators, healthcare professionals, and the general public.