Against this general background of research, special consideration will now be given to totally blind children who have specifically shown delays and impairments in their interpersonal relating and social understanding, which are the focus of this project. The sub-sections are as follows:
(i) Case presentations;
(ii) Organic impairments and developmental progress;
(iii) Congenital blindness and autism a) specific conditions - Leber's amaurosis; Retinopathy of prematurity b) behavioural observations;
(iv) Developmental improvement;
(i) Case presentations
Keeler (1958) provided a series of detailed case histories of pre-school children totally blind through one of the major reported causes of that particular time-period, retrolental fibroplasia (now known as ROP). At this time the term 'childhood schizophrenia' was used as a general diagnostic label for many developmental pathologies. Within this classification was a particular condition, what Kanner (1943) called "autistic disturbance of affective contact." Keeler's research programme, begun in 1953 in Toronto, was concerned with a group of five referred blind children who presented with symptoms strikingly similar to this phenotype.
The children studied were all premature, ranging from six to seven months gestation at birth. There were three girls and two boys. All were totally blind, or at most with extremely low light perception in one eye. All showed a behavioural predilection towards "self isolation" and did not use language appropriately in interpersonal communication, neither initiating verbal contact nor responding suitably to approaches. However, they demonstrated comprehension of some spoken language, and according to Keeler, "good memory". Also odd was their disinclination to distinguish between their primary caregivers and strangers. Two showed such a reluctance to interact that they would reject any physical contact at all. They demonstrated typical autistic patterns of behaviour: rhythmic rocking, hand and arm waving with rhythmical opposition of the finger tips of both hands, particularly to music, with which they showed an unusual preoccupation. Their play was solitary, usually involving repetitive physical routines with mechanical objects and those that made noise, e.g. spirming a pot lid, or screwing on and off a jar top. There was much echolalia (word repetition out of context), or the adoption of approximations of heard words using the same tone and rhythm. In this regard, they were noted to learn early to hum (by about age two) and to remember and sing "fairly complicated" songs, sometimes with the correct words. These children would not feed themselves, throwing the spoon away when encouraged to learn to self-feed, and remaining on strained baby foods up to age four or five. Neurological assessment showed normal encephalograms.
Keeler commented that "the psychological environment in all five cases had not been good". All of the children had spent their first month in a hospital incubator, and then when at home the mothers had all tended to leave the children to fend for themselves in cribs or playpens for long periods, attending only to their imperative physical needs. These parents showed great distress at their children's blindness, particularly at the time of diagnosis, and more than one repeatedly stated that they "would rather see their children dead than the way they were."
Of the children, Keeler presented two case descriptions. The first was Cheryl, who was referred at age six. She was bom at 6 months, 21bs in weight, and was not discharged from hospital until two months old. She was diagnosed as having retrolental fibroplasia at six-months-old and to be totally blind. Her mother complained that she showed no attention and remained aloof, never used language to communicate but had "decent" receptive language (though no example of this was actually provided in th% report). As an infant she was reported as preferring to be by herself, not wanting to be picked up, and having developed a range of bizarre hand and arm movements, along with persistent rocking and humming. She had a very good spatial memory, remembering the practical geography of places visited only once, and could find her way around the house easily. At age six she would still only use her potty toilet. When her mother suggested she "go to the po-po" Cheryl would mimic the word *po- po' and remain sitting until her mother took her physically to her potty. Another example of her echolalia came in response to Keeler's question to her at the end of the interview. "Do you want to go home. Cheryl?" was simply echoed back.
Her mother's main concern was Cheryl's reluctance to use speech, "If only she would talk with me, we could get along so well together and she would be happy." Keeler commented that it was unclear what attempts Cheryl's mother made to "bring her out" and how early these had been made in her life; Keeler felt it is was as though she expected her daughter to take all the initiatives and demonstrate her needs. She seemed not to realise her part in engaging and helping Cheryl to discern and appreciate her mother's presence and identity, and to develop a mutually defining relationship. She left Cheryl in a playpen for much of the time, and worked on and off while paying for child-care. She had stayed at
home for one year with Cheiyl in the hope that it would improve things, but when no "dramatic" progress was seen she became discouraged.
The second case was that of Betty. She was referred at age 4, and bom at 6!/4 months, weighing 31bs, and spent two months in hospital special care, receiving a diagnosis of retrolental fibroplasia at 3 months. At the time of referral she was with foster parents. As far as was known second-hand, Betty started to walk at age 2. As an infant she was unconcerned about others, not seeking aJffection, and at present remained often alone for long periods. She would not interact with other children, yet was tirelessly and fearlessly keen on rough-and tumble play with adults, often showing anger and sulking whenever it was stopped. She followed instructions, showing adequate associative receptive language, with a sense of 'right and wrong', which seemed to be behaviourally learned by reward and punishment. Finding out her wants was on a trial and error basis, in that she would only react to offers of the item or action that she desired, e.g. "Do you want to go to the toilet?" receiving a positive response, while she ignored suggestions of a glass of milk or cookies. She was unlikely to take her own initiatives, sitting and wetting herself unless asked, rather than going to the toilet unaided. She was reported by her foster mother as vocalising early and using words at 12 months, saying "Mama" and "Dada"', but thereafter she did not develop in using language communicatively, instead appropriating received phrases into repetitious verbalisms, e.g. "Get down, Betty!" In her third year she began to rock, and awoke in the night chattering to herself, "to hear her own voice" as her foster mother said. She was slow in learning to feed and at five years still preferred strained baby foods. Most things presented to her Betty took immediately to her nose to smell them, and preferred objects that made a noise. Betty's foster mother felt she was "not stupid by any means", and that if "someone were to spend a lot of time with her and she was with other children, I think she could come on wonderfiilly."
The picture here was of someone intent to make contact with others, but perhaps lacking the means, with receptive language skills superior to expressive ones. She was unable to initiate and name her specific wants, but was easily responsive when what she wanted was offered. Again, Keeler noted that, notwithstanding Betty's first two months spent in hospital, she had never received very much
individual attention, her foster mother working periodically. In her first year, particularly, she was left in a playpen for long periods.
A further group of 25 pre-school and 10 school-age (i.e. 6 - 12) blind children with the same condition were also observed. Although not presenting such a full range of difficulties, their collected histories showed patchily similar symptomology, though less pronounced or persistent. There was delayed motor development, delayed language development, preoccupation with music, over dependence on smell in exploration, blindism-like bizarre body movements, and unspecified "autistic patterns". Although all of these 35 were considered at school to be learning at an appropriate level, they were usually thought "slower" than their classmates, blind through other conditions.
In considering a comparison group of children of similar ages blind through other conditions, such as cataracts and glaucoma (though these children all had some vision, albeit less than 20/200; n = 18) there was not "the same degree of autistic patterns of behaviour" (p. 73). Although some children were withdrawn and some demonstrated blindisms, it was never with the same severity as the children above.
Another group of children were compared, those who lost their vision late in infancy or in early childhood through trauma infection, neuromyelitis optical and particularly retinoblastoma (n = 17). Such abnormalities were even less conspicuous in these cases. Whether or not their motor or language development was becoming established at the time of vision loss, they were developing along normal lines. Keeler noted, though, that children in this group were typically anxious and became especially distressed at environmental changes. In contrast with the less visually impaired group they were also more likely to develop blindisms such as fingers in the eye, head and body twisting.
Keeler (1958) characterised the index children's condition as an aversion to affection, while Hallenbeck (1954) characterised those blind children of nursery and primary school age in his study as showing an emotional coldness and withdrawal from human contact. Keeler evaluated the
debatable). He judged all but those of the first five children to be between "average” and "excellent". In these cases Keeler gave etiological focus to the early months spent isolated in incubators, without social contact. Freedman (1971) also thought that the most fundamental sensoiy deprivation, of primary somatosensory stimulation, was crucial. However, an investigation by Chase (1972) found somewhat contradictorily that there was no simple relationship between longer lengths of time in special hospital care and higher levels of affective disturbance; in fact she saw the opposite, that children spending longer times in hospital were less prone to these social difficulties.
Beyond this, though, Keeler pointed to what he felt were deficient, withdrawn patterns of parental care and encouragement, and the core problem being the children's emotional deprivation - not being closely held, caressed, spoken to, nor adequately stimulated - rather than components of the medical condition such as hypothesised cortical oxygen damage, or "vascular involvement to the brain" in addition to the retinal pathology. Green and Schechter (1957) also reported on similar behaviour patterns in a group of totally blind children. Yet they characterised the maternal behaviour in their study as overprotective: the mothers "interfered and did many things for their children, thus preventing them from learning from their own efforts." (p. 637). Hallenbeck (1954) suggested there was a vicious cycle whereby new parents were told of the medical team's low expectations for the baby, visually, mentally and physically, which along with the factors of separation and subsequent slow development, serve to undermine the parents' expectations and skills in caring for the infant.
As to comparisons with autism, which he saw in terms of a "lack of distinction between themselves and the outside world, i.e. between the I and the not-I", Keeler concluded against simple co-morbidity, but rather felt that the symptoms "simulated" the syndrome, even though, or perhaps because, there was a prevalence of autistic symptoms to lesser degrees in most of the children with retrolental fibroplasia. Instead he referred to the importance of visual and emotional cues in a developing conceptual differentiation of body image, and beyond that of self and other. He speculated finally about the influence of brain damage associated with prematurity, with central nervous system involvement, and psychogenetic causation, particularly considering the five severe cases against the other 35 with retrolental fibroplasia. With "favourable mothering" and "good psychological
environments'' the syndrome would not occur with such behavioural severity. He called for corrective intervention with these older pre-school children, involving a specialised "close, warm" nursery programme, working at individual and group levels.
Parmalee. et al. (1959) conducted a similar project - "The Development of ten children with retrolental fibroplasia: A four year study". All of their subjects were bom premature, weighing less than 1.8g,There were ten premature, sighted control subjects, none of whom showed any degree of "retardation". Six had no vision; four had some in at least one eye (i.e. at most perception of colour and shape close to the eye); three of the six totally blind were "socially retarded", and also delayed by comparison in toilet training, language and feeding, though not in walking without support. These three had the lowest birth weights, though their gestational ages were similar to the other, more developmentally normal children. A Gesell test in the first year showed a normal level of achievement for all ten, but thereafter the three children in question began to slip behind the others on developmental measures. Their cases are summarised here:
Case 1 was bom to Japanese parents, with retrolental fibroplasia, diagnosed at 3 months. The parents were said to feel shame, and had not informed some relatives of their son's blindness. For the first year he was kept in a crib, surrounded by pillows; then at 12 months he was allowed more freedom on the floor. At this point he seemed happy and responsive to his brother. In the second year he walked freely around the house, but only banged new toys in exploring them. He was apprehensive in new situations, and Parmalee commented that he "seemed to regress in the second year." In his third year he showed little play, still only banging toys; though he did enjoy a particular rough-and-tumble game of running with his mother, holding her skirt. It was "difBcult to penetrate his repetitive activities." At 32 months he permitted more social contact, and said "Okay", "Car", "Cookie" (i.e. a few object labels). In his fourth year he tolerated being placed on the toilet, but wouldn't feed himself. On the whole he was more responsive to people generally, and his mother particularly. She talked though about diSiculty in establishing a relationship with him.
Case 2 was diagnosed at two months of age; he had no siblings. At first both parents were very happy with their new child, however disabled; though as things subsequently developed, the father became "aggressively defensive" and generally "unable to be critical". His wife was inhibited by him in expressing opinions about the baby. In the first twelve months the child showed a normal rate of achievement, except sitting unaided - he was confined to a folded sheet on a rug, the mother afraid for him to move about for fear of injury, not being allowed to touch food himself as his mother resented any "mess". At nineteen-months he was walking, but wanting to go everywhere holding mother's hand. He was kept for most of the time in a playpen, and invented a game of rolling a ball up a slide and then catching it. His parents reported that at this time his mother felt closer to him, though in comparison he was increasingly demanding of his father, who began to resent his demands. At 24 months he began to talk, feed himself and begin toilet training. His third year brought "impressive" walking and exploration of toys, some words but no combinations. His mother described fears of toileting and resistance to training, reluctance to sleep at night or to co-operate in eating, and she seemed overwhelmed and in continual conflict with him, becoming indecisive in her actions. He was considered to show "normal mental potential, but significant emotional disturbances." Brain damage was suspected, compounded by difficulties in relationships. From age three-to-four, however, he "made greater effort to be self-critical." He started school, tolerating other children, though not participating in their activities. He was described as hyperactive, "negative" and aggressive, and was still using only single words in communication. Parmalee described this family's behaviour during these research visits. The father usually entertained and amused the child, but there was no development in his manner; he seemed afraid to have him move about.
Case 3 was diagnosed at three-months-old. Her parents were initially very close and enjoyed their daughter, but then became concerned with others' opinions of her and the mother became as though a "distant observer". The marriage became strained, with separations. There was a one-year younger sibling, though the relationship was not close. At seven-months she bounced when held supported, laughed, and played with her hands. She was not, though, sleeping on a normal day-night schedule, and this was left uncorrected by her parents. In her second year she could drink out of a cup, and said "Dada", "Mama", "Pat-a-cake", "Bye-bye". By this age intraocular tension had increased in both eyes.
with great pain. She had surgery but still seemed distressed, slept poorly and lost weight. Her parents found this period "veritable hell". At 21 months she was thought happier, with her sleeping problem less severe. She used phrases and short sentences, and physically was quite active. At this point the family was then forced temporarily to move, due to family illness. On return she seemed to regress, preferring solitary play; she had developed a large vocabulary but now mainly talked to herself. Her parents felt that this mood of withdrawal was due to periodic eye pain. In her fourth year she became very ill with pneumonia, with long periods of crying, and her speech became barely understandable; she "climbed on her parents and other people as if they were furniture." During the interview she ignored others, preoccupied in her own play; though she did enjoy a spell of rough-and-tumble. Otherwise any motor activity involved mainly rhythmic and circular body movements. She began to bite herself. From age four to five there was improvement: she was more aware of the environment and interactive; her perseverative play could be interrupted and redirected.