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Studies in the psychology of dwarfism
T h e Journal o[ P E D I A T R I C S
381
Studies in doepsychology of d vmfism II. Personality maturation and response to growth hormone treatment in hypopituitary dwarfs
Seventeen dwarfs, who were treated with human growth hormone, were investigated psychologically. The degree of psychomaturation achieved tended to parallel the degree to which the dwarf had been treated socially according to age and not size. Psychopathology, when present, was of the inhibitive, dissociative type, with constriction o[ the cognitive field; aggression and acting out were conspicuously absent. Psychosis was absent. The expectancies and consequences of treatment, including the increased growth, constitute a readjustment syndrome which is helped by psychologic counseling.
John Money, Ph.D.,* and Ernesto Pollitt B A L T I M O R t ~ , ]VID.
A L L W H O COlVIE in contact with a dwarf, whether professional people, parents, relatives, or strangers, find that impulsively and intuitively they gear their reactions and expectancies of the dwarf first to his size and not his age. It is only on second thought, or through habituation, that allowance is made for age and social maturity. The effect of silhouette and visual gestalt in determining social response is apparent even on viewing a photograph of a dwarf (Fig. 1). The effect is equally apparent in reverse, that is, From the Departments of Psychiatry and Pediatrics, The Johns Hopkins University School of Medicine. Supported in research by Research Grant No. HD-00325 and Research Career Development Award No. HD-K3-18,635, The National Institute of Child Health and Human Development, United States Public Health Service. *Address, Phlpps 400, The Johns Hopkins Hospital, Baltimore, Md. 21205.
in one's social response to viewing a child with precocious physique and sexual maturation. In another context, the silhouette effect also applies to the sexual differentiation of one's response, as is evident when dealing with hermaphroditic ambiguity, or with transvestism and impersonation. All dwarfs are obliged to deal with some degree of babying in their social-behavioral and personality development as a result of the silhouette effect. One expects a complementary relationship between being babied and responding immaturely. This relationship is also a matter of degree; that is, the more a dwarf is babied, the greater is the immaturity or lag in social-behavioral and personality development.
PURPOSE The purpose of this paper is to r e p o r t that parental attitudes and expectancies, on
382
Money and Pollitt
March 1966
Fig. 1. Two 17-year-old boys: one of average height and one dwarfed. The dwarfed patient (J.W.) had gained 1~ inches during 8 months of treatment at the time of photography.
the one hand, and the dwarfed child's psychomaturational (social-behavioral and personality) level, on the other, complement one another; to report the various phenomena of personality disturbance encountered when psychom~ituration becomes distorted; and to report the readjustment reactions of the patients to the growth that results from
treatment with H G H (human growth hormone or Somatotropin). This paper is the second 4 from a longitudinal study of dwarfed children receiving investigative therapy with H G H in the pediatric growth study at T h e Johns Hopkins Hospital. The sample comprises 17 of the 21 dwarfed
Volume 68 Number 3
children who were selected between late 1960 and mid 1964 for a trial test with human growth hormone and subsequent investigative therapy. The 17 cases were diagnosed as: isolated growth hormone deficiency, 3; hypopituitarism, 6; panhypopituitarism, 5; and undiagnosed, with positive H G H response, 3. Three children were disqualified on account of diagnosis (gonadal dysgenesis, 2; primordial dwarfism, 1) and one eligible patient was lost as a result of scheduling falltires. PROCEDURES
Endocrine. Initially, the patients were hospitalized for approximately a 4 week period for special tests and evaluation. Additional, briefer, evaluative admissions followed at 4 or 8 month intervals, dependent on the investigative cycle of therapy. The medication for the 17 patients varied slightly in dosage. Generally, however, the hormone was administered in a dosage of 2 mg. a day for a 2 week period, followed by a 2 week interval without medication for an 8 month period. A 4 month period followed, during which no medication was received. Variations in the procedure were dependent on the patients' response to growth hormone. A detailed report of H G H treatment on 11 of the 17 patients considered in this paper is given elsewhere. 2, 5 In most cases a normal rate of growth per annum was achieved, the m a x i m u m rate for the first year being around 6 inches. There was a tendency toward diminished effectiveness of treatment after an initial spurt of catch-up growth, so that the patients m a y mature as short adults. Psychologic. All 17 patients were interviewed in the psychohormonal research unit. One boy was followed for as long as 10 years, 6 of which were prior to H G H treatment. The follow-up intervals were yearly, or longer, except during the active period of the H G H program during which all patients returned to the hospital more often. Only one boy was followed as briefly as 8 months, that is, the interval between initial evaluation and first posttreatment return.
Psychology o/ dwarfism
383
A minimum of two interview hours per patient per follow-up was established; the interviews were longer for the initial workup, and longer when special problems required attention. Using both direct and oblique questioning techniques, the interviews were oriented to obtain information about the patients' attitudes, feelings, dreams, thoughts, and experiences with respect to their size, physical and recreational activities, social and romantic acceptance, academic and family life, and their experience with growth hormone. The majority of interviews, or a portion of them, were recorded and transcribed. Otherwise, at the conclusion of the interview, a note was dictated for inclusion in the patient's history. In all cases an attempt was made to interview the parents or other responsible relatives. In 6 cases, only the mother was seen, once, only the father. In 9 cases, both parents were seen and in one case, neither, because the child traveled alone from a distant state. In addition to the interviews with patients and parents, observations were made of the children's behavior and way of relating to their peers, parents, and ward personnel during the period of hospitalization. These observations were carried out by the head nurse of the pediatric clinical research unit. The school authorities sent information about behavior, social and physical activities, and study habits of 13 children. From the foregoing data, statements relative to the following topics were excerpted as they pertained first, to the child and second, to the parents: (1) short stature, (2) physical capabilities, (3) social relations, and (4) H G H treatment. A one-page summary of parent-child complementarity (or its absence) was formulated, concluding with a notation on prominent personality mechanisms in evidence and an estimate of psychomaturation as adequate or deficient for age, or as in part adequate but in part "distorted" or disturbed by psychopathologic mechanisms; or as deficient and dis-_ totted.
3 84
Money and Pollitt
March 1966
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FINDINGS Psychomaturation: Parent-child complementarity. I n all 17 cases (except No. 111 25 84 whose work-up and treatment was undertaken with the parents in absentia) it was possible to see that the child's psychom a t u r i t y level (Table I) complemented and was complemented by the rearing practices, attitudes, and expectancies of the parents. This correspondence is readily illustrated in representative psychologic summaries, of which, owing to space limitations, only one is here reproduced. It is a case of severe maturational lag complicated by neurotic mechanisms. B. D., No. 91 02 74. This l l-year-old child was first seen at the age of 8 years and followed for 3 years. The informants were the patient, mother, and father. When the boy was 9, the father was first interviewed and said that neither he nor his wife had been fooled by the boy's short stature. They had treated him according to his age. For example, if the boy were to fight, they would not fight for him, but left him to defend himself. The father actually participated little in the handling of the 2 children, as he considered himself too ill-tempered and not as competent as his wife. The wife concurred in this opinion stating that her husband was too dependent on her, relied on her excessively, and made mountains out of molehills. The marriage and family life had been very stormy. The husband had a long psychiatric history, marked by volatile fluctuations of mood, with one period of hospitalization for 3 months. The presence of maturational lag and neurotic disturbance in the boy's personality was illustrated when he once came to the hospital dressed as a Cub Scout during National Scout Week. He talked at length as if still a member, not disclosing that actually he had dropped out because of inability to meet the social and recreational demands of his agemates. The mother worried about his tendency to lie and make up boastful stories, not recognizing this as subterfuge of psychodynamic significance. She also saw the boy as making mountains out of molehills, like his father, and considered his behavior immature in many ways. He related poorly with his agemates. When classmates told their friends that he was 6 or 7, he would not correct them to say that his real age was 10. His school achievement was
March 1966
subject to great fluctuations, with a general tendency to poor concentration and underachievement. In interviews, it was evident that the personality mechanisms of neglect of the cognitive field, euphemism, and compensatory confabulation were much in use.
Maturational lag and personality disturbance. F r o m Table I, the frequencies of psychomaturation levels are as follows: adequate, 7; deficient, 2; distorted, 4; and deficient-distorted 4. A distorted level of psychomaturity means that a psychopathologic mechanism was in evidence. I n no instance was the psychopathology grossly severe. There were no cases of distortion severe enough to be called unequivocally psychotic. I n the larger clinic population of cases not receiving investigative growth hormone therapy, however, psychosis was not unknown. One girl became frankly paranoid as a y o u n g woman, with delusions of pregnancy that were in part wish-fulfilling and in part persecutory. This patient as an infant had exhibited an extreme sibling rivalry when her sister and only sibling arrived, and, as a child, had exhibited a particularly vivid fantasy life in which she dramatized and symbolized the problems of her dwarfism and family life. T w o cases were severely deficient in psychomaturity, their retardation complicated by congenital central nervous system deficit in one case, and by autism a n d / o r congenital mental deficiency in the other. Examination of the psychodynamic mechanisms listed in Table I shows that developmental lag was conspicuous in 6 patients. Except in 2 children (Nos. 8 and 11), developmental lag overlapped with some form of neglect or constriction of the cognitive field such as evading, disowning, denying, or retreating from the problems consequent on dwarfism (Nos. 2, 3, 7, and 13). This inhibitive, dissociative type of mechanism was p r i m a r y in 7 patients (Nos. 1, 3, 5, 7, 12, 13, and 17), 3 of w h o m showed the symptom of elective mutism. T h e dissociative mechanism was subsidiary and evidenced as compensatory or boastful confabulation, euphemism, discounting, or explaining away, in 4
Volume 68 Number 3
other cases (Nos. 2, 6, 9, and 10). Thus, in 11 of the 17 cases, there was some form of ostrich, head-in-sand policy of disengaging from a problem by not facing up to it. O f the remaining 6 cases) 2 (Nos. 8 and 11) were complicated by low IQ, which left only 4 (Nos. 4, 14, 15, and 16) who showed primary evidence of dealing with their problem of being a dwarf by some maneuver of direct, active mastery. One of these was an overconforming boy who was an indecisive daydreamer; one was realistically bitter about the indignities of his stature; one was an intellectual prodigy with all his selfrespect invested in his intellect; and the other, the most normally adjusted of the group, was a moderate underachiever at school who concentrated on social rather than academic development. The readjustment syndi'ome: Reaction to HGH treatment and growth. From the right-hand column of Table I, the children's attitudes toward treatment with H G H can be categorized as in Table II. Those who administer treatment of any type rather readily assume that patients will place the same value on its outcome as they themselves do. This assumption is most readily upheld when a patient has undergone a sudden change or deviation from his familiar state of health, especially if he suffers pain. The assumption m a y be quite in error
Psychology of dwarfism
38 7
when the patient's ill health represents a chronic state of disability to which he has been long accustomed. In this instance, therapeutic intervention brings about changes in the corporeal self and the expectancies of life experience. Such rapid change toward normalcy may be as difficult to adjust to as a deforming injury or other rapid departure from normalcy. In fact, any m a j o r change, whether therapeutic and positive, or noxious and negative, is followed by what deserves to be designated as the readjustment syndrome. The demands of readjustment m a y be so taxing that the patient rejects treatment and desires to return to the familiarity and relative safety of the status quo. One child (W. A.) encountered such difficulties of readjustment that she frankly resisted hospitalization, claiming she was happy being small and did not want to grow. After 4 months of treatment, she wanted to quit. It was only after a year, with the results of H G H beginning to show, that she acquiesced to the idea of growing taller. Within 6 more months, however, she had, she said, grown to be 4 feet and did not want to be any taller. H e r disenchantment with growth fairly transparently related to a hidden motivation to recapture her lost status as the favored little one and only child in her grandparents' home. Because of her cleft lip and frailness, the grandparents had taken her
Table II. Patients' attitudes to induced growth Initial attitude
NO. O[ children
Reaction to growth
No. of children
Ambivalent with definite rationalization for not being treated and changed
Ambivalent Antagonistic Favorable
1 2 1
Cooperative, but with reservations focalized around fear of being hurt by needles
Favorable
3
Noncommittal but cooperative
Noncommittal Favorable
2 1
Cooperative and realistic
Favorable
2
Eager, with reservations as defence against possible failure
Favorable
2
Enthusiastic, wanting a growth miracle
Favorable
1
Too young to communicate
Too young to communicate
2
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Money and Pollitt
at birth and raised her for 9 years. Then her mother capriciously decided to repossess her. Her siblings were strangers to her. Her suicidally depressed father openly rejected her because of her appearance. It was at this juncture, at the age of nearly 10, that promise of growth became a threat to her, rather than a hope. Despite the girl's difficulty with readjustment, therapy with H G H was continued. The fear and possibility of failure counterbalancing the longing for therapeutic success may pose a readjustment problem, as it did for the boy, S. C. Despite his willingness for treatment, he complained of many mys~ terious bodily aches and pains in response to hospitalization and, at home, the injection needle. He often felt too unwell to go to school. He and his parents had always given special attention to academic excellence, in view of high I Q versus his statural defect. Gradually, his achievement level showed signs of dropping off with his attendance. Then, rapidly, at the age of 11, as it appeared that he may not obtain a good growth response from H G H , he developed a major, acute school phobia, with extremes of hiding in the pilings under the house and of locking himself in the bathroom to avoid school. He wrote: "I haven't gone to school yet . . . I don't know what to do. I don't want to go, and it's too hard; I can't go. The years are going much faster than my growth. I thought I could do it, but when the day comes, I can't." A month after this letter, the phobia broke suddenly, at which time it had become clear that H G H was having some effect. During the ensuing 2 years, general progress continued to be satisfactory. Even when difficulties of readjustment are not great, they are not nonexistent and present a challenge. One boy of nearly 13 years (B. J.) was able to express quite explicitly his philosophy of active striving as in his statement of advice to a new H G H candidate: "Well, I'd tell him to have his life, so if he's having this chance, this real good chance, ybu'd have to make the best of it . . . . You'd have to be a better person-to kind of have a goal that he, er, that he
March 1966
knows he will reach. And, er, that he'll be a, he'll be good---I mean, if he's receiving this, if he's receiving the growth hormone, he has to kind of turn out to be something good . . . . That's kind of necessary for me, I don't know about him." In general, problems of readjustment are best handled by combining pharmacologic treatment with supportive psychotherapy. They can be avoided altogether if hormonal treatments are begun very early in life, before the dwarf has been required to make an adjustment to life as a dwarf. The cardinal rule here, as elsewhere in medicine, is that prevention is better than cure. DISCUSSION
Statural smallness has two determining influences on the personality and psychologic development of a dwarf. First, because it induces other people to baby the dwarf instead of treating him according to his size, it places a special developmental burden on him not to lag in psychomaturation. Second, his smallness confers on him an index of recognizance--an animadversion index--so that, without any effort on his part, he becomes extremely widely known in his community and school. This notoriety places another burden of sorts on the dwarf, either as an instrument of mortification to him, on account of its origins, or as an instrument of popularity and friendship. If mortified by his size, a dwarf tends also to manifest personality mechanisms of retreat that belong in the general category of dissociation, denial, and inhibition with neglect or constriction of the cognitive field and, possibly, with disorders of judgment, confabulation, and fantasy. If able to capitalize on his size as an animadversion index, a dwarf still has the problem of eliciting from people responses suited to his age and not his size. Simultaneously, he has to make concessions because he cannot completely replicate the behavior of others his age. Almost inevitably, he can resolve these disparities only by accepting to some degree a role as mascot and a formalized joking relationship. In this role he must be good-natured and
Volume 68 Number 3
cheerful about all the activities and participations from which his stature disqualifies him, perhaps even playing the comedian and' clown. There does not seem to be much place, in the psychology of dwarfism, for personality mechanisms that pertain to aggressive and destructive mastery. In the present sample of cases, there were no instances of temper tantrums, none of destructive acting out as in firesetting or stealing, and none of assault or social delinquency. In searching for an explanation for this absence of aggressive or destructive acting out, one readily thinks of a direct effect of the handicap of size: T o be excessively small relative to one's agemates precludes the possibility of being the victor in direct attack. Nonetheless, in nondwarfed children, aggression is vented on opponents of unequal size, even the parents, from time to time. Thus, the absence of such aggression in dwarfism seems to be more than fortuitous. One thinks of the proverbial cheerfulness of achondroplastic dwarfs and wonders whether in dwarfism of other etiology there may be some built-in inhibition of aggression. An alternate explanation may be sought in terms of the fact that a dwarf spends a long time being baby-sized and evoking from people responses geared toward the nurturance and succorance of helpless little living things. It is well known to animal ethologists that the perceptual appearance of the young of many species triggers various innate releasing mechanisms of parental behavior in adults of the species. In humans, there seems to be an analogous mechanism with respect to the size of another member of the species. Thus, it is possible that the majority of dwarfs are weak in aggressive and destructive responses because they were sheltered throughout a long, critical period of diminutive childhood size from threatening stimuli which would evoke aggressiveness and destructiveness. In this respect it is interesting to consider that, in the traditional role of court jester, the dwarf was able to be critical and hostile in a way that would have cost a normal-sized
Psychology o[ dwarfism
389
courtier his head. The dynamic here may well have been that the ruler felt safe and unthreatened by someone so much his miniature in stature and muscular strength. One is reminded of adult male squirrel monkeys 3 who display threateningly to one another in a power struggle, or to themselves in a mirror, but not to females or juveniles. SUMMARY
Seventeen dwarfs were studied psychologically either prior to, or at the time of initiating investigative therapy with human growth hormone ( H G H ) . Longitudinal study ranged from as long as 10 years to 8 months. The maximum duration of H G H therapy was 3 ~ years. In all cases, the degree of psychomaturation achieved bore a complementary relationship to the success of adults, notably the parents, in treating the dwarf according to his age instead of his size. Two cases were complicated by low IQ. When psychomaturational lag was complicated by psychopathologic mechanisms of the personality, they were related to retreat, inhibition, dissociation, and neglect or constriction of the cognitive field. There was an absence of psychopathologic personality mechanisms related to aggression and acting out. Psychosis was absent in the present sample, though frank paranoid delusion was observed once in another sample of untreated patients. Treatment with H G H necessitates a major reorientation to the self and toward life that qualifies as a readjustment syndrome. In severe cases, the difficulties of readjustment bring about rejection of treatment. In such cases, discontinuance of medication is not recommended, but supportive psychotherapy is advisable. Problems of readjustment are minimal when therapy is begun early in life - - t h e earlier the better. The authors are grateful t o Dr. Robert M. Blizzard, director of the Pediatric Endocrine Clinic, and Dr. Donald Cheek, director of the Growth Study, for making available their find-. ings on patients; to Dr. Philip Drash who has succeeded Dr. Pollitt as psychologist on the Growth Study; to Mrs. Jean McCloskey for her
390
Money and Pollitt
discerning and insightful ward reports on the patient behavior; and to Mrs. Viola Lewis for checking tabulations. REFERENCES
1. Bayer, L. M., and Bayley, N.: Growth diagnosis, Chicago, 1959, University of Chicago Press. 2. Brasel, J. A., Wright, J. C., Wilkins, L. and Blizzard, R.: An evaluation of seventy-five patients with hypopituitarism beginning in childhood, Am. J. Med. 38: 484, 1965.
March 1966
3. MacLean, P. D. : Mirror display in the squirrel monkey, Science 146: 950, 1964. 4. Pollitt, E., and Money, J.: Studies in the psychology of dwarfism. I. Intelligence quotient and school achievement, J. PEDIAT. 64: 415, 1964. 5. Wright, J. C., Brasel, J. A., Aceto, T., Jr., Finkelstein, J., Kenny, F., SpMding, J., and Blizzard, R.: Studies with humml growth hormone (HGH). An attempt to correlate acute metabolic responses with linear growth during prolonged therapy, Am. J. Med. 38: 499, 1965.
381
Studies in doepsychology of d vmfism II. Personality maturation and response to growth hormone treatment in hypopituitary dwarfs
Seventeen dwarfs, who were treated with human growth hormone, were investigated psychologically. The degree of psychomaturation achieved tended to parallel the degree to which the dwarf had been treated socially according to age and not size. Psychopathology, when present, was of the inhibitive, dissociative type, with constriction o[ the cognitive field; aggression and acting out were conspicuously absent. Psychosis was absent. The expectancies and consequences of treatment, including the increased growth, constitute a readjustment syndrome which is helped by psychologic counseling.
John Money, Ph.D.,* and Ernesto Pollitt B A L T I M O R t ~ , ]VID.
A L L W H O COlVIE in contact with a dwarf, whether professional people, parents, relatives, or strangers, find that impulsively and intuitively they gear their reactions and expectancies of the dwarf first to his size and not his age. It is only on second thought, or through habituation, that allowance is made for age and social maturity. The effect of silhouette and visual gestalt in determining social response is apparent even on viewing a photograph of a dwarf (Fig. 1). The effect is equally apparent in reverse, that is, From the Departments of Psychiatry and Pediatrics, The Johns Hopkins University School of Medicine. Supported in research by Research Grant No. HD-00325 and Research Career Development Award No. HD-K3-18,635, The National Institute of Child Health and Human Development, United States Public Health Service. *Address, Phlpps 400, The Johns Hopkins Hospital, Baltimore, Md. 21205.
in one's social response to viewing a child with precocious physique and sexual maturation. In another context, the silhouette effect also applies to the sexual differentiation of one's response, as is evident when dealing with hermaphroditic ambiguity, or with transvestism and impersonation. All dwarfs are obliged to deal with some degree of babying in their social-behavioral and personality development as a result of the silhouette effect. One expects a complementary relationship between being babied and responding immaturely. This relationship is also a matter of degree; that is, the more a dwarf is babied, the greater is the immaturity or lag in social-behavioral and personality development.
PURPOSE The purpose of this paper is to r e p o r t that parental attitudes and expectancies, on
382
Money and Pollitt
March 1966
Fig. 1. Two 17-year-old boys: one of average height and one dwarfed. The dwarfed patient (J.W.) had gained 1~ inches during 8 months of treatment at the time of photography.
the one hand, and the dwarfed child's psychomaturational (social-behavioral and personality) level, on the other, complement one another; to report the various phenomena of personality disturbance encountered when psychom~ituration becomes distorted; and to report the readjustment reactions of the patients to the growth that results from
treatment with H G H (human growth hormone or Somatotropin). This paper is the second 4 from a longitudinal study of dwarfed children receiving investigative therapy with H G H in the pediatric growth study at T h e Johns Hopkins Hospital. The sample comprises 17 of the 21 dwarfed
Volume 68 Number 3
children who were selected between late 1960 and mid 1964 for a trial test with human growth hormone and subsequent investigative therapy. The 17 cases were diagnosed as: isolated growth hormone deficiency, 3; hypopituitarism, 6; panhypopituitarism, 5; and undiagnosed, with positive H G H response, 3. Three children were disqualified on account of diagnosis (gonadal dysgenesis, 2; primordial dwarfism, 1) and one eligible patient was lost as a result of scheduling falltires. PROCEDURES
Endocrine. Initially, the patients were hospitalized for approximately a 4 week period for special tests and evaluation. Additional, briefer, evaluative admissions followed at 4 or 8 month intervals, dependent on the investigative cycle of therapy. The medication for the 17 patients varied slightly in dosage. Generally, however, the hormone was administered in a dosage of 2 mg. a day for a 2 week period, followed by a 2 week interval without medication for an 8 month period. A 4 month period followed, during which no medication was received. Variations in the procedure were dependent on the patients' response to growth hormone. A detailed report of H G H treatment on 11 of the 17 patients considered in this paper is given elsewhere. 2, 5 In most cases a normal rate of growth per annum was achieved, the m a x i m u m rate for the first year being around 6 inches. There was a tendency toward diminished effectiveness of treatment after an initial spurt of catch-up growth, so that the patients m a y mature as short adults. Psychologic. All 17 patients were interviewed in the psychohormonal research unit. One boy was followed for as long as 10 years, 6 of which were prior to H G H treatment. The follow-up intervals were yearly, or longer, except during the active period of the H G H program during which all patients returned to the hospital more often. Only one boy was followed as briefly as 8 months, that is, the interval between initial evaluation and first posttreatment return.
Psychology o/ dwarfism
383
A minimum of two interview hours per patient per follow-up was established; the interviews were longer for the initial workup, and longer when special problems required attention. Using both direct and oblique questioning techniques, the interviews were oriented to obtain information about the patients' attitudes, feelings, dreams, thoughts, and experiences with respect to their size, physical and recreational activities, social and romantic acceptance, academic and family life, and their experience with growth hormone. The majority of interviews, or a portion of them, were recorded and transcribed. Otherwise, at the conclusion of the interview, a note was dictated for inclusion in the patient's history. In all cases an attempt was made to interview the parents or other responsible relatives. In 6 cases, only the mother was seen, once, only the father. In 9 cases, both parents were seen and in one case, neither, because the child traveled alone from a distant state. In addition to the interviews with patients and parents, observations were made of the children's behavior and way of relating to their peers, parents, and ward personnel during the period of hospitalization. These observations were carried out by the head nurse of the pediatric clinical research unit. The school authorities sent information about behavior, social and physical activities, and study habits of 13 children. From the foregoing data, statements relative to the following topics were excerpted as they pertained first, to the child and second, to the parents: (1) short stature, (2) physical capabilities, (3) social relations, and (4) H G H treatment. A one-page summary of parent-child complementarity (or its absence) was formulated, concluding with a notation on prominent personality mechanisms in evidence and an estimate of psychomaturation as adequate or deficient for age, or as in part adequate but in part "distorted" or disturbed by psychopathologic mechanisms; or as deficient and dis-_ totted.
3 84
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March 1966
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FINDINGS Psychomaturation: Parent-child complementarity. I n all 17 cases (except No. 111 25 84 whose work-up and treatment was undertaken with the parents in absentia) it was possible to see that the child's psychom a t u r i t y level (Table I) complemented and was complemented by the rearing practices, attitudes, and expectancies of the parents. This correspondence is readily illustrated in representative psychologic summaries, of which, owing to space limitations, only one is here reproduced. It is a case of severe maturational lag complicated by neurotic mechanisms. B. D., No. 91 02 74. This l l-year-old child was first seen at the age of 8 years and followed for 3 years. The informants were the patient, mother, and father. When the boy was 9, the father was first interviewed and said that neither he nor his wife had been fooled by the boy's short stature. They had treated him according to his age. For example, if the boy were to fight, they would not fight for him, but left him to defend himself. The father actually participated little in the handling of the 2 children, as he considered himself too ill-tempered and not as competent as his wife. The wife concurred in this opinion stating that her husband was too dependent on her, relied on her excessively, and made mountains out of molehills. The marriage and family life had been very stormy. The husband had a long psychiatric history, marked by volatile fluctuations of mood, with one period of hospitalization for 3 months. The presence of maturational lag and neurotic disturbance in the boy's personality was illustrated when he once came to the hospital dressed as a Cub Scout during National Scout Week. He talked at length as if still a member, not disclosing that actually he had dropped out because of inability to meet the social and recreational demands of his agemates. The mother worried about his tendency to lie and make up boastful stories, not recognizing this as subterfuge of psychodynamic significance. She also saw the boy as making mountains out of molehills, like his father, and considered his behavior immature in many ways. He related poorly with his agemates. When classmates told their friends that he was 6 or 7, he would not correct them to say that his real age was 10. His school achievement was
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subject to great fluctuations, with a general tendency to poor concentration and underachievement. In interviews, it was evident that the personality mechanisms of neglect of the cognitive field, euphemism, and compensatory confabulation were much in use.
Maturational lag and personality disturbance. F r o m Table I, the frequencies of psychomaturation levels are as follows: adequate, 7; deficient, 2; distorted, 4; and deficient-distorted 4. A distorted level of psychomaturity means that a psychopathologic mechanism was in evidence. I n no instance was the psychopathology grossly severe. There were no cases of distortion severe enough to be called unequivocally psychotic. I n the larger clinic population of cases not receiving investigative growth hormone therapy, however, psychosis was not unknown. One girl became frankly paranoid as a y o u n g woman, with delusions of pregnancy that were in part wish-fulfilling and in part persecutory. This patient as an infant had exhibited an extreme sibling rivalry when her sister and only sibling arrived, and, as a child, had exhibited a particularly vivid fantasy life in which she dramatized and symbolized the problems of her dwarfism and family life. T w o cases were severely deficient in psychomaturity, their retardation complicated by congenital central nervous system deficit in one case, and by autism a n d / o r congenital mental deficiency in the other. Examination of the psychodynamic mechanisms listed in Table I shows that developmental lag was conspicuous in 6 patients. Except in 2 children (Nos. 8 and 11), developmental lag overlapped with some form of neglect or constriction of the cognitive field such as evading, disowning, denying, or retreating from the problems consequent on dwarfism (Nos. 2, 3, 7, and 13). This inhibitive, dissociative type of mechanism was p r i m a r y in 7 patients (Nos. 1, 3, 5, 7, 12, 13, and 17), 3 of w h o m showed the symptom of elective mutism. T h e dissociative mechanism was subsidiary and evidenced as compensatory or boastful confabulation, euphemism, discounting, or explaining away, in 4
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other cases (Nos. 2, 6, 9, and 10). Thus, in 11 of the 17 cases, there was some form of ostrich, head-in-sand policy of disengaging from a problem by not facing up to it. O f the remaining 6 cases) 2 (Nos. 8 and 11) were complicated by low IQ, which left only 4 (Nos. 4, 14, 15, and 16) who showed primary evidence of dealing with their problem of being a dwarf by some maneuver of direct, active mastery. One of these was an overconforming boy who was an indecisive daydreamer; one was realistically bitter about the indignities of his stature; one was an intellectual prodigy with all his selfrespect invested in his intellect; and the other, the most normally adjusted of the group, was a moderate underachiever at school who concentrated on social rather than academic development. The readjustment syndi'ome: Reaction to HGH treatment and growth. From the right-hand column of Table I, the children's attitudes toward treatment with H G H can be categorized as in Table II. Those who administer treatment of any type rather readily assume that patients will place the same value on its outcome as they themselves do. This assumption is most readily upheld when a patient has undergone a sudden change or deviation from his familiar state of health, especially if he suffers pain. The assumption m a y be quite in error
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when the patient's ill health represents a chronic state of disability to which he has been long accustomed. In this instance, therapeutic intervention brings about changes in the corporeal self and the expectancies of life experience. Such rapid change toward normalcy may be as difficult to adjust to as a deforming injury or other rapid departure from normalcy. In fact, any m a j o r change, whether therapeutic and positive, or noxious and negative, is followed by what deserves to be designated as the readjustment syndrome. The demands of readjustment m a y be so taxing that the patient rejects treatment and desires to return to the familiarity and relative safety of the status quo. One child (W. A.) encountered such difficulties of readjustment that she frankly resisted hospitalization, claiming she was happy being small and did not want to grow. After 4 months of treatment, she wanted to quit. It was only after a year, with the results of H G H beginning to show, that she acquiesced to the idea of growing taller. Within 6 more months, however, she had, she said, grown to be 4 feet and did not want to be any taller. H e r disenchantment with growth fairly transparently related to a hidden motivation to recapture her lost status as the favored little one and only child in her grandparents' home. Because of her cleft lip and frailness, the grandparents had taken her
Table II. Patients' attitudes to induced growth Initial attitude
NO. O[ children
Reaction to growth
No. of children
Ambivalent with definite rationalization for not being treated and changed
Ambivalent Antagonistic Favorable
1 2 1
Cooperative, but with reservations focalized around fear of being hurt by needles
Favorable
3
Noncommittal but cooperative
Noncommittal Favorable
2 1
Cooperative and realistic
Favorable
2
Eager, with reservations as defence against possible failure
Favorable
2
Enthusiastic, wanting a growth miracle
Favorable
1
Too young to communicate
Too young to communicate
2
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at birth and raised her for 9 years. Then her mother capriciously decided to repossess her. Her siblings were strangers to her. Her suicidally depressed father openly rejected her because of her appearance. It was at this juncture, at the age of nearly 10, that promise of growth became a threat to her, rather than a hope. Despite the girl's difficulty with readjustment, therapy with H G H was continued. The fear and possibility of failure counterbalancing the longing for therapeutic success may pose a readjustment problem, as it did for the boy, S. C. Despite his willingness for treatment, he complained of many mys~ terious bodily aches and pains in response to hospitalization and, at home, the injection needle. He often felt too unwell to go to school. He and his parents had always given special attention to academic excellence, in view of high I Q versus his statural defect. Gradually, his achievement level showed signs of dropping off with his attendance. Then, rapidly, at the age of 11, as it appeared that he may not obtain a good growth response from H G H , he developed a major, acute school phobia, with extremes of hiding in the pilings under the house and of locking himself in the bathroom to avoid school. He wrote: "I haven't gone to school yet . . . I don't know what to do. I don't want to go, and it's too hard; I can't go. The years are going much faster than my growth. I thought I could do it, but when the day comes, I can't." A month after this letter, the phobia broke suddenly, at which time it had become clear that H G H was having some effect. During the ensuing 2 years, general progress continued to be satisfactory. Even when difficulties of readjustment are not great, they are not nonexistent and present a challenge. One boy of nearly 13 years (B. J.) was able to express quite explicitly his philosophy of active striving as in his statement of advice to a new H G H candidate: "Well, I'd tell him to have his life, so if he's having this chance, this real good chance, ybu'd have to make the best of it . . . . You'd have to be a better person-to kind of have a goal that he, er, that he
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knows he will reach. And, er, that he'll be a, he'll be good---I mean, if he's receiving this, if he's receiving the growth hormone, he has to kind of turn out to be something good . . . . That's kind of necessary for me, I don't know about him." In general, problems of readjustment are best handled by combining pharmacologic treatment with supportive psychotherapy. They can be avoided altogether if hormonal treatments are begun very early in life, before the dwarf has been required to make an adjustment to life as a dwarf. The cardinal rule here, as elsewhere in medicine, is that prevention is better than cure. DISCUSSION
Statural smallness has two determining influences on the personality and psychologic development of a dwarf. First, because it induces other people to baby the dwarf instead of treating him according to his size, it places a special developmental burden on him not to lag in psychomaturation. Second, his smallness confers on him an index of recognizance--an animadversion index--so that, without any effort on his part, he becomes extremely widely known in his community and school. This notoriety places another burden of sorts on the dwarf, either as an instrument of mortification to him, on account of its origins, or as an instrument of popularity and friendship. If mortified by his size, a dwarf tends also to manifest personality mechanisms of retreat that belong in the general category of dissociation, denial, and inhibition with neglect or constriction of the cognitive field and, possibly, with disorders of judgment, confabulation, and fantasy. If able to capitalize on his size as an animadversion index, a dwarf still has the problem of eliciting from people responses suited to his age and not his size. Simultaneously, he has to make concessions because he cannot completely replicate the behavior of others his age. Almost inevitably, he can resolve these disparities only by accepting to some degree a role as mascot and a formalized joking relationship. In this role he must be good-natured and
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cheerful about all the activities and participations from which his stature disqualifies him, perhaps even playing the comedian and' clown. There does not seem to be much place, in the psychology of dwarfism, for personality mechanisms that pertain to aggressive and destructive mastery. In the present sample of cases, there were no instances of temper tantrums, none of destructive acting out as in firesetting or stealing, and none of assault or social delinquency. In searching for an explanation for this absence of aggressive or destructive acting out, one readily thinks of a direct effect of the handicap of size: T o be excessively small relative to one's agemates precludes the possibility of being the victor in direct attack. Nonetheless, in nondwarfed children, aggression is vented on opponents of unequal size, even the parents, from time to time. Thus, the absence of such aggression in dwarfism seems to be more than fortuitous. One thinks of the proverbial cheerfulness of achondroplastic dwarfs and wonders whether in dwarfism of other etiology there may be some built-in inhibition of aggression. An alternate explanation may be sought in terms of the fact that a dwarf spends a long time being baby-sized and evoking from people responses geared toward the nurturance and succorance of helpless little living things. It is well known to animal ethologists that the perceptual appearance of the young of many species triggers various innate releasing mechanisms of parental behavior in adults of the species. In humans, there seems to be an analogous mechanism with respect to the size of another member of the species. Thus, it is possible that the majority of dwarfs are weak in aggressive and destructive responses because they were sheltered throughout a long, critical period of diminutive childhood size from threatening stimuli which would evoke aggressiveness and destructiveness. In this respect it is interesting to consider that, in the traditional role of court jester, the dwarf was able to be critical and hostile in a way that would have cost a normal-sized
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courtier his head. The dynamic here may well have been that the ruler felt safe and unthreatened by someone so much his miniature in stature and muscular strength. One is reminded of adult male squirrel monkeys 3 who display threateningly to one another in a power struggle, or to themselves in a mirror, but not to females or juveniles. SUMMARY
Seventeen dwarfs were studied psychologically either prior to, or at the time of initiating investigative therapy with human growth hormone ( H G H ) . Longitudinal study ranged from as long as 10 years to 8 months. The maximum duration of H G H therapy was 3 ~ years. In all cases, the degree of psychomaturation achieved bore a complementary relationship to the success of adults, notably the parents, in treating the dwarf according to his age instead of his size. Two cases were complicated by low IQ. When psychomaturational lag was complicated by psychopathologic mechanisms of the personality, they were related to retreat, inhibition, dissociation, and neglect or constriction of the cognitive field. There was an absence of psychopathologic personality mechanisms related to aggression and acting out. Psychosis was absent in the present sample, though frank paranoid delusion was observed once in another sample of untreated patients. Treatment with H G H necessitates a major reorientation to the self and toward life that qualifies as a readjustment syndrome. In severe cases, the difficulties of readjustment bring about rejection of treatment. In such cases, discontinuance of medication is not recommended, but supportive psychotherapy is advisable. Problems of readjustment are minimal when therapy is begun early in life - - t h e earlier the better. The authors are grateful t o Dr. Robert M. Blizzard, director of the Pediatric Endocrine Clinic, and Dr. Donald Cheek, director of the Growth Study, for making available their find-. ings on patients; to Dr. Philip Drash who has succeeded Dr. Pollitt as psychologist on the Growth Study; to Mrs. Jean McCloskey for her
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discerning and insightful ward reports on the patient behavior; and to Mrs. Viola Lewis for checking tabulations. REFERENCES
1. Bayer, L. M., and Bayley, N.: Growth diagnosis, Chicago, 1959, University of Chicago Press. 2. Brasel, J. A., Wright, J. C., Wilkins, L. and Blizzard, R.: An evaluation of seventy-five patients with hypopituitarism beginning in childhood, Am. J. Med. 38: 484, 1965.
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3. MacLean, P. D. : Mirror display in the squirrel monkey, Science 146: 950, 1964. 4. Pollitt, E., and Money, J.: Studies in the psychology of dwarfism. I. Intelligence quotient and school achievement, J. PEDIAT. 64: 415, 1964. 5. Wright, J. C., Brasel, J. A., Aceto, T., Jr., Finkelstein, J., Kenny, F., SpMding, J., and Blizzard, R.: Studies with humml growth hormone (HGH). An attempt to correlate acute metabolic responses with linear growth during prolonged therapy, Am. J. Med. 38: 499, 1965.