Kim Johnson has Rett Syndrome, a condition unknown in the 1960s when she was born, and although she developed normally for nearly two years, it has led to her being severely dyspraxic and without speech. Kim lives in Melbourne, where she has spent the past four years studying for the VCE - Victorian Certificate of Education, awarded after successful completion of the final year of secondary school -- after DEAL Communication Centre (now the Anne McDonald Centre) showed her how to communicate by typing with a headpointer. From that time her family and friends gradually came to know a totally new Kim, who has led them all into uncharted waters.
Kim finally gained her VCE in December, 1997. Since then she has studied various tertiary courses.
Here's an up-to-date piece on what Kim has been doing lately.
Lady from Down Under: An interview with a person with Rett Syndrome
by Pam Cole, Autism Research Monthly
Riding the surf in a blow-up boat, exploring unusual places, horse-back riding, hot-air-ballooning, going out with friends for some coffee, or staying in with a good read... Sounds like a list of activities of someone with quite a sense of adventure. It is, actually, and this person is Kim. She has Rett Syndrome, a condition which didn’t stop her from going airborne in a helicopter for her fortieth birthday.
Rett Syndrome is a developmental condition that affects oneself pervasively and inhibits intended movement, including speech and mobility. It often renders the hands next to useless. Sometimes it is causes breathing irregularities, namely hyperventilation with apneas.
So, what's it like to have Rett's? Kim answers: “I feel that love has lessened (the) damning effects of Rett Syndrome. I feel my terror mostly at night. I feel sad that I do not live with my husband in my own city apartment with expansive views.” She also needs around-the-clock care, as well as help with activities of daily living, such as feeding, dressing, and walking. While irregular breathing may cause anxiety in some, Kim says that it helps her to “gain control.”
Rett Syndrome also affects sensory processing, the way we collect and process information about “the world.” Jill, Kim’s mother, explains, “Kim doesn't have touch perception over most of her body – just head, hands, toes, and a few joints. She feels pain and temperature, and is very sensitive to both, but not much to touch.” Also, Kim needs to be seated no more than several feet away from the television – or else her visual perception breaks into “bits.” In order to see the whole picture, she has to be close. She enjoys concerts, live shows and films, but needs to sit close to her TV set for her view of the screen to be unified – beyond a couple of meters she sees the scene “in bits”.
Her sensory issues also influence her taste. Kim writes, “I feel lulled by dominant melody and strong rhythm. I love quivering drumming music – lively and energetic, but not too loud.” Jill adds that baroque is one of Kim's favorite musical styles. At home, they greatly enjoy listening to classical, because “pop...is so pervasive.” Also, “Kim loves to dance to lively music – we hold her, and she rocks from foot to foot.”
Kim communicates with a head-pointer attached to a small keyboard, which is linked to her laptop, because she is nonverbal. The program she uses has a word-prediction program to ease typing. In the past, she used to need Jill to hold her head a bit to type. Now, though, Jill makes contact with her back for some sensory input. When she is not near her laptop, she uses her Cannon Communicator, which prints out slips of paper with typed words. When speech is expected, she has her Lightwriter, which holds phrases and speaks typed words aloud.
Kim's education is a major aspect of her life. She taught herself to read as a child by watching Sesame Street and through interactions with her family. When her family contacted Dr. Rosemary Crossley, at the DEAL Communication Center, in Australia, it was discovered that Kim was not “profoundly mentally retarded,” but already understood the intricacies of the written English language. At age 22, Kim became communicative. She went on to earn her Victorian Certificates in English, History of Revolutions – French and Russian, Australian History, and Psychology. She completed her 11th grade math exam without facilitation and earned a score of 82%. Math is one of her favorite subjects, but she couldn't continue due to not being able to use the required programmable calculator because of its small buttons and screen. She chose to study the arts at university for this reason.
Kim's interest in politics has refined into a desire to help those with Rett Syndrome. Like a true leader, Kim leads by example. She decided to get her degree for herself and to show people with Rett's that they can advance their education. Kim was the first person with Rett Syndrome to be considered to have intelligence via Rettnet, a world-wide email network for families with a person with Retts. This was groundbreaking, because before then and even now, Retts has been associated with intellectual disability. In her own words, “Justice means nobody gets left behind and I try to help those I can.” She also wants to encourage Rett Syndrome people and their parents by her example to gain an education. As she said “I once toyed with the idea of writing a book, but then thought getting a degree better,” because a book could be discarded and forgotten about, but her education records are permanent. However, they did write that book and are currently looking into getting it published.
Currently, she is attending a day program and lives in a residency for severely disabled adults. What she wants to share with the community is this: “Talk with the people. Just because they don't answer, doesn't mean they don't understand.” Certainly, this is poignant, as many people are afflicted with communication impairments. As is often the case with people who have trouble communicating, they can perceive a lot better than they can express, so it is always good to talk to people. Certainly, the ability to communicate does not equate intelligence.
And here's one we prepared earlier.
Hi! I'm Kim's mother, Jill.
Kim would like me to tell you something about herself, as her communication is very slow and tires her rapidly. She also wants to tell you quite a bit herself, though, and her words will be found typed in UPPER CASE BOLD. Often you'll be able to tell by the style who is doing the talking; Kim's expression is very concise, as she has a lot of time to think but not much energy to type. Rosie Crossley has described it in her latest book "Speechless" as ...."as if she were writing a telegram and being charged by the word"!
Kim is having a lot of fun catching up on the education she missed as a child and teenager. Somehow she managed to teach herself to read - FROM TV AT HOME at the usual age .... SESAME ST .... and adores maths: ADDING WAKES MY MIND
Kim is also very interested in politics, and would be happy to have (short!) discussions on any political subject.
Owning a dog is her present aim, while not necessarily that of her family!
In 1993 Kim undertook her first ever real education at the Collingwood TAFE college where she studied literacy and maths. An article would be read and discussed by the class, and then some questions answered in writing. One such topic was about disputes between neighbours, the causes of such disputes, and how they could be dealt with in Mediation Centres rather than by expensive litigation in the Courts. The question was asked "Why do you think Mediation Centres are necessary?" to which Kim replied:
BECAUSE NEIGHBOURS DON'T CO-EDIFY, DISDAIN APPEARS. CLOSE CONFINES DEEPEN RIFTS.
Another time the class read a Neighbourhood Watch article about how to protect people and property. The question was asked: "How can you protect yourself in a public place?" Kim's response:
ESCHEW LONELY PLACES and COHABIT UTILITIES.
The class was learning how to write a letter of complaint - how to set it out etc
I wish to complain about BEING CALLED INTELLECTUALLY DISABLED AS I ADVOCATE MY DAILY AFFAIRS BEST. EVERYONE DEMEANS MY ABILITIES.
And in class, writing for self-exploration:
IF I HAD THE SKILL I'D BE A DOCTOR.
After this wonderful year Kim wanted something more serious and challenging, so began VCE studies at a TAFE college(at the rate of one subject per year since her communication is so slow). VCE is the final year of school study, usually taken at about 17-18 years of age. Kim used a mixture of essays and multiple choice questions in her written work. Subjects were partly chosen for convenience of timetable! She works best in the mornings, for instance, and cannot stay up for evening courses, so was been unable to take Politics, her favourite subject. She was finally awarded her VCE in December, 1997.
Each year has seen expansion in the amount of written work produced. For English self-expression she composed a short poem:-
"I, lonely being, eulogise each persevering friend reaching to me.
Gates were often opened
Writing gives me the niche I need to utilise my voice.
Love makes diffidence over my actions bleach, egging me on with ever viable activities which add to confidence.-'
Kim also rides the police horses at the barracks in South Melbourne, with an organisation called Riding for the Disabled. This is a great group of people who give their time and expertise to helping everyone ride - and I mean everyone! One delightful young man, whose body was severely distorted by cerebral palsy, was carried in the arms of a rider sifting on a sheepskin! He didn't stop smiling! Some are blind, and because of her dyspraxia Kim may or may not hold on, so helpers walk along beside her just in case (she hasn't needed anyone yet, except during trotting!!) The highlights have been trail rides in the country followed by a barbeque.
Many people have asked Kim just how she communicates - what do we all do to help her express herself?
It became apparent quite early that hand-pointing was going to be difficult. Girls and women with Rett Syndrome spend a considerable amount of time with their hands together in the mid-line. This is not a voluntary action. They are driven to do it, and it interrupts almost every other activity, even eating!! So Kim prefers to point with her head.
She sits on a fairly comfortable upright chair (I LIKE COMFORTABLE FURNITURE) with her feet on the floor. The light comes from behind to illuminate the keyboard, and I often prop a mirror on the chair in front of her so that I can see her eyes. Often Kim will very obviously be attending to the keyboard, at other times her gaze may wander up and to the side in a fairly characteristic manner, and I wait for her complete this seemingly necessary activity before gently bringing her head back to the midline and asking her to look again at the keyboard.
Another Rett Syndrome factor affecting pointing is breath-holding. Research has shown that during this process there is a tendency to normalisation of the EEG - Kim has said it helps her to GAIN CONTROL. This may happen frequently during a communication session - Kim uses this to position her headpointer, and on expiration lowers the tip onto the desired key as she relaxes.
Her preferred keyboard is a Canon Communicator, which is supplemented by Yes/No/Don't Know etc boards. The keyboard is small - some 12x8 cm (4x5') and contains the alphanumeric characters, some punctuation, upper and lower case, space and most importantly - backspace! It is essential to be able to erase mistakes. Output is a small printed paper tape. This is the fastest means for her so far, and output is not so great that a computer is an advantage - indeed the keyboards are too big, and even using switches and word-prediction programmes gives no appreciable advantage. So I turn my hand at word-prediction, asking if my guess is correct using the Yes/No board. Often the answer is NO, so she continues with the word.
So, we sit side by side, my chair a little higher than Kim's as I gently rest my hand on her head to give her some proprioceptive feedback of where her head is in space. She has always told us of balance problems, which we came to understand better when she told us she had no touch perception over most of her body - heat, cold and pain Yes! but not touch except for brisk rubbing. So her own feeling of herself in space is limited to her head, hands and a few joints. She cannot feel the ground beneath her feet, and when asked by a physio if she could feel the chair she sat on, replied YES, BUT I CAN'T ENTIRELY BE SURE LENGTH OF CHAIR FROM FLOOR, OR IF I'M EVEN HIGH OR LOW IN THE ROOM.
To prevent her hands from coming together during a session, I sit on the side of her dominant hand (left) and put it between us. For Kim, handwringing is a serious distraction and must be prevented during communication, which is fragile. (However it is a different matter when walking - BRINGING HANDS TO MIDFRONT GIVES ADVANTAGE OF BEING ABLE TO GAUGE DISTANCE OF NEXT STEP) I then hold the Canon so that the keyboard lies in the plane of movement of the tip of Kim's headpointer, and so that the tip starts at the centre of the top row of characters. Then I wait for her to move to the desired letter and then depress the key. The keyboard has a guard over it with holes over each character, a little larger than the rubber tip on the headpointer. This means a double action is required to produce a printed character, which increases the certainty of a correct hit.
Checking against errors and facilitator influence:
It is vital that a typing error is not allowed to persist, as Kim may try to change her writing to accommodate it, thereby not saying what she intended, or she may simply give up. I say each letter as it is typed (more than once as a prompt if she pauses for long), and in addition, each sentence. I check at least every sentence, and each guessed word. Kim has asked me to GUESS MORE to save her time and energy this can be quite easy if you know what the conversation is about.
Also it is necessary to be aware of the many ways it is possible for the facilitator to influence Kim's communication. The most obvious is for the facilitator, say, me, to actually push Kim's headpointer onto the keys, or push the keyboard onto the headpointer. This would be fairly easy, as I am stronger than Kim, but why would I bother? And if I did, I wouldn't spend one and a half hours extracting 10-20 words; I'd have it all done in 2 minutes and be off!
There are, however, more subtle influences to guard against: encouraging movement in the "right' direction rather than any other. preventing hitting the "wrong" key for various reasons. tensing the fingers of my right hand in excitement as she approaches the "right' key, which might give her a clue to my thoughts, if she wanted it.
So, the pressure of the right hand must be steady and even, and appropriate for the day - some days more, some days less. There will inevitably be changes in pressure on my fingers as Kim moves her head, so I must pay constant attention to maintain evenness. Some days slight backwards pressure on her forehead is helpful; at other times this may be sufficient to completely prevent her pointing.
Last year Kim studied Psychology, which was quite an eye-opener for us both. She learned a lot about visual perception principles, and about behaviourist psychologists! As she said in an essay on the topic:
HOW EVENTS ARE INTERPRETED IS DEPENDENT ON OUR PRIOR EXPERIENCE AND INFLUENCES. EXAMPLE OF THIS IS MY INTENTION. SOME OBSERVING MY COMMUNICATION PERCEIVE MY INTENTIONAL MOVEMENTS, BUT OTHERS PERCEIVE CONTROL BY THE RECEIVER.
This year Kim wants to write up Rett Syndrome from her point of view, so if anyone has questions they would like to ask her, and are not in a hurry for the answer, or anything they would like to say to her about anything at all, please e-mail her on firstname.lastname@example.org
Kim's headpointer consists of a 3cm band of softly-padded pigskin, with 2 straps crossing the head and secured with velcro for ease of adjustment. Another pair of straps passes under her chin - also secured with velcro. A 27cm rod is attached to the front of the band with a wingnut. This rod can be shaped so that the rubber tip is in easy view of the user.