Challenge for Habilitation: The Child with Congenital Rubella Syndrome (Texas Children’s Hosp, 1977)

[Narrator:] Challenge for habilitation: the child with the congenital rubella syndrome. [Narrator:] A maternal viral infection during pregnancy, especially in the first three months, has the potential for producing a number of harmful effects in the developing fetus. An estimated 20,000 to 30,000 children were born with the congenital rubella syndrome as the result of the 1964-65 rubella epidemic in the United States. This group of one-and-a-half to four-year-old children in the clinic reception area are all in the rubella program at the Texas Children’s Hospital in Houston. When the 146 patients were reviewed in 1967, 90 had hearing loss, 39 were visually impaired, and 50 had significant motor handicap. Of these, there were 20 deaf-blind with impairment of both hearing and vision. The use of more definitive diagnostic studies and clinical observation showed that the 17 considered normal probably did not have the congenital rubella syndrome. Comprehensive evaluations were done on 80 children at age two and a half years in order to project their educational needs. Those with primarily an auditory or visual handicap were eligible for existing preschool programs. The staff of the child development clinic initiated a diagnostic therapeutic nursery for the 25 multiply-handicapped children. Their arbitrary classification as mild, moderate, or severe was based on physical, developmental, and behavioral deviations. The clinic offers interdisciplinary services for the parents and child. Individual sessions with the social worker focus on current stresses produced by the presence of a handicapped child in the family. The persistence of rubella virus may cause progression of known physical abnormalities and development of new ones. The pediatrician must therefore evaluate all systems in his periodic examination. During each regular eye examination, the ophthalmologist looks for evidence of congenital cataract, glaucoma, microphthalmus, and rubella retinopathy. Similar in-depth studies are done by the cardiologist, otologist, audiologist, and orthopedist. Maturation, cognition, and behavior, while related to the degree of structural abnormality, cannot be defined by physical examination alone. Developmental and psychological profiles are used by the psychologist to determine the mental age and to identify specific areas of functional delay or deficit. The psychologists have selected different test instruments for three children of comparable chronological age based on prior knowledge of their medical disability and direct observation of their behavior. Excitability and inattention, rather than the severity of visual and hearing impairment, prevent this deaf-blind girl from performing on the Gesell Developmental Test. The hearing-impaired, but otherwise normal child, is being tested with the non-verbal Leiter International Scale. Her intellectual potential, as well as affect and behavior, are appropriate. Performance on the Stanford-Binet indicates that this physically normal boy is also of average intelligence. At five years of age however, immunological tests showed that his original diagnosis of rubella was probably incorrect. Motor skills, particularly creeping, crawling, and walking, did not develop in an orderly progression for most of the congenital rubella patients. For some children, athetosis, spasticity, or hypotonia resulting from central nervous system involvement, was a significant handicap. This girl with spastic diplegia, in addition to correctable auditory and visual impairment, has had individual physical therapy and has been fitted with long leg braces and crutches. Overcoming architectural barriers, such as stairs at school, is a part of her gait training. The earlier motor handicap of the boy at the table is not evident since he no longer needs a short leg brace. Both of these children have progressed from the multi-handicapped program to a pre-school deaf class. Their functional vision and auditory amplification allow them to interact and to respond with pleasure to the sight and sound of the barking toy dog. An area of baldness is seen on the back of the heads of many rubella children. This resulted from the type of bizarre movement and self-stimulatory activity demonstrated by this 18-month-old deaf-blind motor-delayed child. The multi-handicapped girl in the parallel bars has only minimal muscle weakness and is at a readiness stage for walking. Her distractibility, short attention span, and her poor vision, even with contact lenses, are preventing her independent locomotion. She later progressed to walking alone with one hand on the wall, but would not cross an open doorway until provided with a yardstick with which to maintain contact with the floor or wall. Hypotonia and muscle weakness, although initially present in many of the children, usually improved by the age of two and a half years. This severely hypotonic boy is still unable to hold his head erect at age four years. The physical therapist is positioning him so as to utilize his minimal muscle power to help develop motor function. The therapist attempts to stimulate head control by holding his attention with a flashlight and a spinning musical top. Head control would have to be established in the normal neurodevelopmental sequence before he could coordinate movements of his arms, legs, and trunk. His inability to maintain a given posture is fatiguing and soon results in loss of motivation. Although this little girl has normal vision and aided hearing within the speech range, coordinated upper extremity movement is extremely difficult for her due to severe spasticity and athetosis. The occupational therapist is demonstrating an activity which requires the child to use the fine muscles of her hand and forearms for grasping, and the gross muscles of her shoulders and arms for reaching and placing the block on the pedestal. This demands a great effort from the child, who is rewarded by an approving facial expression and verbal praise from the therapist. Psychomotor retardation and maladaptive behavior prevent establishment of the oral mechanisms of sucking, chewing, and swallowing, the essential antecedents of eating and drinking. The occupational therapist is attempting to teach this three-and-a-half-year-old boy each step in the hand-to-mouth progression of self-feeding. First, he must learn to grasp the spoon or cup, hold onto it, and then move it through space from the table to his mouth with increasing dexterity and minimal spilling. He rejects not only the utensils, but any interaction with the therapist herself. Even in children who are attentive, upper extremity weakness, lack of motor control, and poor eye-hand coordination may delay the acquisition of self-help skills. The speech therapist must consider both mental and chronological age in programming for multiply-handicapped children. She teaches the mothers of children under three years of age how to provide language stimulation through daily activities. A bell is used by this mother to make her 18-month-old girl aware of environmental sounds. Such awareness is a forerunner to comprehension of the spoken voice. This three-year-old boy prefers to sight through the hole in the plastic ring rather than use the ring appropriately. When his mother has his attention, he can comprehend her directions. A standing board is used for positioning and total body support of the hypotonic boy shown previously on the mat. This allows him to participate in a small group conducted by the speech therapist. He responds appropriately by laughing at the barking dog and attempts to use his arms and hands to grasp the color rings. On the other hand, the deaf-blind girl shows no more interest in auditory stimulation than she did in independent locomotion. The nursery groupings for peer interaction are based on the degree of impairment and the cumulative effect of multiple handicaps on behavior. Those classified as severe are immature and have inappropriate emotional responses, lack of environmental awareness and self-stimulation, which prevent optimal group participation. Many of these deaf-blind children are absorbed in stereotyped activities frequently referred to as blindisms. Waving their hand in front of their eyes seems to produce a hypnotic flicker effect. Jabbing the thumb or forefinger into the globe of the eye appears to be pleasurable rather than painful for them. The therapeutic program is designed to develop the child’s attention span, his awareness of others, and his own self concept. This goal-directed activity is most difficult to achieve with these autistic-like children who do not interact with the group. For this reason, one-to-one behavior modification techniques are a more successful training method. Children in the moderate group function at about the one-year language level by Gesell developmental norms. Although they are able to understand and execute a verbal command, their attention span is short. Distraction by pleasurable visual stimulation often prevents completion of the task directed by the therapist. Sighting through the translucent cup into a direct light results in amplification of the child’s existing nystagmus and represents another example of a pleasurable blindism. The children in the mild multi-handicapped group are able to comprehend directions and carry out a fine motor activity emphasizing color matching and number concepts. This represents a level of function which approaches their chronological age. They have a longer attention span and watch and interact with the other children. Most have a moderate to severe neurosensory hearing loss, but have adequate vision to lip-read and make maximal use of visual clues given by the teacher. Imagery play is used by the nursery teacher to initiate group interaction with three children. They are over three and a half years of age but are still at the parallel play stage of social development. The little boy was placed in the nursery program for habilitation of multiple handicaps resulting from head injury. Although his physical appearance and combination of handicaps is somewhat different from the children with congenital rubella, the therapeutic approach is essentially the same. Over the past three and a half years, the parents and professional staff have mutually benefited from the discussion and educational groups focused on the management of these handicapped children. The nursery teacher is demonstrating how to make an edible play dough from flour, water, and food coloring. The speech therapist then shows the parents how they may use the play dough for language stimulation by the use of simple phrases such as “take it,” “roll it,” and “pull it,” which are clearly enunciated so that the child can read their lips. Continuous monitoring and discussion of each child’s progress at interdisciplinary staffings provides the basis for projecting short and long-term functional and educational goals for optimal habilitation.

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