Caracterización de MEFs doblemente deficientes en Cdh1 y Skp

Koos lives on a smallholding about 20 kilometres from town, where his parents run a guest house. He attends the same school as Jan and Jani. His mother is a qualified high school teacher, but has been in the guest house trade for the past thirteen years, since the family's return from the United States of America. His father had previously been a missionary director and currently has his own estate agency business. There are four children. Koos is the youngest of the three brothers. His sister, the youngest of the children, is in Grade 8 and is home-schooled. His two elder brothers are both students at different universities.

 Koos's understanding of his own giftedness

Koos defines a gifted person as someone who is exceptional and excels. Such individuals will be recognized by their outstanding leadership qualities. Their natural abilities and talents in any field of expertise will be, even without any practice or effort, far above others' performance. He also explains that giftedness has many faces, and different people may show different aptitudes and strengths.

 Inner world

Koos has many interesting hobbies. He keeps a chameleon as a pet and enjoys listening to gospel music such as for example Jesus Culture. He plays pool and snooker for relaxation and enjoys fishing. He sets time aside for reading mystery or detective stories and has a passion for words, possessing a comprehensive vocabulary. Koos regards words as powerful: they can build or destroy another human being and should be used with great wisdom and discernment. He has a talent for creative writing and in his personal time attempts to write essays and mini-novels. He visualizes what he reads and then re-creates his own story from an existing one. Discussing his personality, he mentions that he rates being honest and having a witty sense of humour as part of being a balanced and unique individual. He also describes himself as kind-hearted and observant. He is intuitively aware of others' emotions, even when they themselves are unaware. Empathy and harmony are qualities that he values. He describes himself as particularly time-oriented and does his school assignments well ahead of time to avoid unnecessary pressure:

I always wear a watch; I always want to know what the time is … I don't like pressure, so if I have to do something like a task then I want to do it early, otherwise I think about it constantly and I stress.

Koos describes himself as active and good at sport, participating in cricket and rugby. His intellectual ability and his ability to relate, his interpersonal skills and qualities such as being trustworthy and empathic are some of his assets. His integrity is recognized by others as a strength. He is also a leader who does not compromise on values and advocates for social justice. He explains his leadership style as follows:

I am a leader … I lead at school … I think a leader has strong values … I go against the stream. Fairness is important to me … I am honest … direct, yes …

Koos regards religion as an essential part of his existence. He believes that his sense of values is guided by spiritual principles. The Bible seems to be the foundation for his daily choices, and he describes his mind-set

and thoughts as innocent and kept 'pure', though sometimes he lacks the courage explicitly to display these qualities.

I was told from a young age by my parents about Jesus and I also have strong values about how I live and … my thoughts are very pure and according to the Bible, but then I don't have the guts to live my actions … but I do have very strong values according to which I try to live every day … to just have a relationship with Him will change your life 100%, just to talk to Him, you don't have to feel guilty if you don't do it, that's also fine.

He may also sometimes lack drive and be laid-back, and he regards these as two areas in which he still needs to develop.

Koos's main subjects of interest are Biology and Mathematics. He seems to be very conscientious and diligently prepares his work well in advance, in contrast to other male peers in his class who does not attend to their schoolwork. He further explains that he experiences exams as very stressful. He does not like to cram his academic work. When he prepares for an exam, he learns all his work and especially his content work off by heart like an oral. He does not summarize when preparing for exams; instead, he merely memorizes by reading through the content.

 Relationship with the parents

Koos describes his relationship with his family as very close. His father is his role-model, who supports and guides him with wisdom and is his confidant. His two older brothers are also his role-models and encourage him to be diligent in his academic work. They have set an example for him with their own attitude towards academics. His parents support him by constantly affirming and encouraging him. He admits that he thrives on compliments and recognition in his quest to do well at school:

My parents support me a lot … compliments and acknowledgement motivate me … but they still give me freedom, they will not be angry at me if I don't study. But they create the environment to learn.

 Relationship with peers

Koos shares that he has a close group of friends who support one another, have a lot of fun together and are building memories as a group. Among the other boys there is however a lot of tension and inter-group conflicts. He has various friends from different age groups in sport and he is also friends with university students.

 Relationship with the school

Koos enjoys school and experiences it as a safe environment which is conducive to his personal development. He also has positive relationships with the teachers and his close group of friends. The school does have computer and internet facilities, but for logistical reasons he never uses these facilities.

I enjoy the relationships at school with my teachers and my friends, and I enjoy the safe environment that it brings, it's comfortable for me, I enjoy sport a lot, I enjoy orals …

Koos, like Jan and Jani, takes both his national languages at first-language level, and these classes are never overcrowded. He therefore feels that he receives sufficient individual attention and support. Despite this advantage, he says that due to the new curriculum some teachers rush through the work at the expense of those learners who might need more support. He agrees with the notion of presenting subjects on higher and lower grades. Koos also expresses his concern over gifted education in the current school system. He feels that, despite some changes in the curriculum, adequate provision is not made for their specific needs.

Sometimes it frustrates me … the new curriculum is CAPS, which is a lot more work, so they run through the work without, if there is one child who understands, then the teacher assumes she can continue, so that is quite frustrating. There are many people behind me who don't understand anything, but they won't tell the teacher. I think they should bring back higher and standard grade … the needs are definitely not addressed sufficiently in the new curriculum … that is what they are changing now … the new curriculum is probably a step in the right direction, I don't know whether it's at the finish line yet but it is moving.

Koos feels strongly that the school should give learners the opportunity to work from old examination papers, as well as allowing them to view their end-of-year exam answering sheets, so that they can learn from their mistakes. During the focus group interview, he argued that classes should not be divided according to intellectual ability, as this would be to the detriment of both the more intellectual and the less intellectual groups. He explained that the bright learners would be more pressured to achieve academically, while the other groups would not be challenged and could be neglected because of prejudice on the teacher's part. He argues as follows:

… there are upsides and downsides, the downsides will be that those learners, if they are in an intelligent class, will be pushed much more to work harder … if they're put in a class where everyone is equally stupid then they will simply not really work, there won't be the more intelligent learners who will push them and the teachers won't really care, because they know the children don't care.

 The role of the community

As indicated above, Koos lives outside town on a smallholding. He is not close to the public library and therefore cannot utilize this facility. At home, they have one computer which is shared by all the members of the household. He has access to the internet and uses it for all his research. He also mentions that he totally lacks any kind of communal support. He is not able to visit the public library and he does not have support from his community, as is the case with Queen (as told in the focus group interview). He says he would have loved this.

In conclusion, Koos shared that his dream is to become a veterinarian. He presented as a sensitive and imaginative boy who is mature for his age, honest and prepared to accept responsibility for his future. He is also considerate and caring, with a real empathy for others.