Canadian Psychiatric Association Journal Vol. 20
Ottawa, Canada, August 1975
No.5
Somatopsychic Relationships GROWTH HORMONE TREATMENT IN HYPOPITUITARY DWARFS: Longitudinal Psychological Effects*
MARIA KUSALIC, M.D.l CLAIRE FORTIN, L.Ps. 2
This report deals with their psychological evolution in relation to physical growth.
This is one of a series of studies on the psychological effects of medically induced growth in a group of hypopituitary dwarfs treated with Human Growth Hormone (HGH). Before treatment these dwarfs were found to be psychologically immature and hypoactive, without any manifestation of aggressive drives, and had an underlying low self-esteem when compared with the normal population. When compared with a matched group with constitutional growth delay the hypopituitary patients were found to use denial, whereas the control group used well-organized compensatory mechanisms (9). Immaturity was found in both groups. When the hypopituitary dwarfs reached adolescence they lacked the core attributes of masculinity or femininity and manifested some ambiguity or even inversion in their choice of gender role (10). 'This paper was presented to the 10th European Conference of Psychosomatic Research, 1974, Edinburgh, Scotland. Revised manuscript received April 1975. This work was supported in part by Riviere des Prairies Hospital. 1 Research Psychiatrist, Riviere des Prairies Hospital and Psychiatrist. Jewish General Hospital, Montreal, P.Q. 2 Research Psychologist, Department of Psychology, H6pital St. Justine, Montreal, P.Q. tGilles Leboeuf, M.D., Department of Endocrinology, H6pital sr, Justine.
Can. Psychialr. Assoc. J. Vol. 20 (1975)
Method Subjects The group consisted of eleven patients, nine boys and two girls ranging in age from 5 10/12 to 21 5/12 years at the beginning of treatment (Table II). All patients were diagnosed as hypopituitary dwarfs by an endocrinologist] who treated them for more than five years by administration of HGH for six months, alternating with a six-month hormone free period. Long before their hypopituitary insufficiency was recognized, most of them had suffered medical problems - long delivery and often breech presentation (2); slight lag in all aspects of development, both locomotor and verbal; hypomotility and sleeping problems. The severity of feeding difficulties in infancy led the parents to consult a pediatrician and there is evidence that these problems still persist. All patients (except the younger girl) are being treated with synthroid because of lack of TSH, and four of them are also lacking ACTH (patients B,C,F and H). The medical diagnosis was established before four years of age, with a mean of 22 months. Procedure Following the selection by the endocrinologist, the subject and his family were
325
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CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
assessed by a psychiatrist. A psychological evaluation based on a battery of tests followed - the Wechsler Intelligence Scale for Children; the Machover 'Draw-a-Person' test, along with the questionnaire suggested by Machover which probes for associations about the projected drawn figure regarding ambitions, feelings, part of the body preferred, how others consider the drawn person, whether the subject would trade places with him and an additional drawing of the family; the Rorschach; and eight selected cards of the Thematic Apperception Test (I, 3BM, 8BM, II, l3B, 17BM, l8GH, 19) chosen for particular reference to aggression achievement needs and self-representation. In both evaluations special emphasis was placed on: attitude towards dwarfism; expectation of treatment; aggression; oral needs; and parental attitude toward the dwarf. Following these initial evaluations each interviewer wrote an independent report and summary, and then after discussion the researchers arrived at a consensus. The procedure was repeated after each period of treatment.
Results The gain in height ranged from 17.5 to 35.9 em after five years of treatment with a mean of27.2 em. Before treatment the hypopituitary dwarfs were very immature, with an underlying low self-esteem and a lack of overall aggressiveness. After the first treatment the low self-esteem became the main feature and aggressive drives began to manifest themselves mildly in the patients' phantasies; after the second, immaturity remained but was overshadowed by depression which manifested itself most clearly in low self-esteem whereas aggressive drives became evident with the occasional appearance of verbal discharge; after the third, the low self-esteem remained but the depression appeared more in the form of affective withdrawal, and the aggressive drives seemed to grow in direct proportion to changes in a patient's stature; after the fourth, immaturity was still present but became overshadowed by an even lower self-esteem and a state of -helplessness and emotional detachment. Some patients started to regress - 'B' became enuretic, 'C' became enuretic and encopretic, 'H' had delusions, and T developed psychotic depression. After the fifth, the group as a
Vol. 20, No.5
whole remained depressed and apathetic but verbally aggressive. The regression in the patients which began after the fourth treatment became even more prominent, contrary to expectation and in spite of the continuation of treatment for more than five years. The patient 'I' in whom the psychosis emerged after the fourth treatment required repeated admission. The delusional and enuretic patients remained the same and the one who had becomeenuretic and encopretic had to be admitted to an institution. Examining other areas will throw further light on their functioning and they are grouped according to age. Life Style As the age increases peer contact decreases. Group A (one boy, one girl, both aged II) are under-achievers academically. Their playmates are younger children of their size, play is infrequent, they are followers in a group, and do not participate in team sports. Overall their psychological adjustment to linear text growth was the best. Group B (two boys 14 years old and one girl 15 years old), one boy and the girl are under-achievers; their playmates are mostly of their own size - although much younger but they have limited contact with their peers; and they do not participate in groups. The third patient is encopretic and living in total social isolation, he repeated Grade III and tripled Grade V. None of them have hobbies. Group C (all males ranging in age from 20 to 27 years) - three are welfare recipients and do nothing but watch television; three others are attending school and repeating grades; they do not mix at all with others and have no hobbies. Sexual Identity (All groups) All have adequate gender identity but as they grow older role inversion becomes more pronounced; the males manifest passivity, conformity and dependency. There is apparently no overt sexual activity (10).
August, 1975
GROWTH HORMONE IN HYPOPITUITARY DWARFS
Fig. I
Fig. 2
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328
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CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
Attitudes Toward Treatment All have unrealistic hopes of obtaining normal stature. There is no apparent relationship between gain in Height Age (HA) and initial psychosocial adjustment - those who manifest the poorest adjustment were equally disturbed above and below the medium gain in HA. However, there was a relationship between HA and Chronological Age (CA) before treatment and subsequent to social adjustment. Those with the lowest original HA (and second and third lowest CA) became enuretic during the course of treatment and remained so to the end; those with the highest HA (and second and third highest CA) became psychotic during treatment and remained so (see Table II).
TABLE I DESCRIPTION OF POPULAnON
Case Identification No.
Name
Sex
I.Q.
A B C D E F G H I
L.L. D.F.
F M
P.C.
M F
99 86 93 122 95 87 96 96 120 114 83
Y.M.
J K
Families Out of eleven families nine were found to have a high degree of pathology. All except two of the dwarfs live with their natural parents; these two were both adopted (one by their oldest sister and another by a cousin). Seven families are from rural Canada, mostly Quebec, the other four from Montreal. They belong to Groups IV and V of Hollingshead's Scale (7) except two who belong to the white collar group. From a dynamic point of view all families except two were assessed as inadequate because of the presence of paren-
Before Treatment
M
L.M. S.J. J.G. F.M. P.L.
M M M M
R.L.
M
G.S.
M
Chronological Age
5 6 8 9 II 13
15 15 16 17 21
10/12 3/12 10/12 7{12 4/12 3{12 7/12 7/12 4/12 6/12 6/12
tal schism and because of ambivalent, over-protective covertly rejecting parental attitudes toward the dwarfed patients, which increased at the end of treatment. There was also an unusually high degree of individual pathology: eight out of twentytwo parents have marked psychiatric disorder. It is interesting to note that the two youngest patients have the least pathology in the family. This could be a coincidence due to the small size of the group or to the rather early initiation of treatment at the age of five.
TABLE II EFFECT OF TREATMENT ON HEIGHT AND HEIGHT AGE* (HA)
Before Treatment Pts A B C D E F G H I J K
CA
10{12 3/12 10/12 7/12 4/12 fl2 7{12 7/12 4/12 17 6/12 21 6/12
5 6 8 9 II 13 15 15 16
HA
2 3 3 4 6 5 5 9 10 7 8
6{12 9/12 6/12 11/12 5{12 1/12 7/12 9{12 2/12 9/12 6/12
After Treatment Change in Gap GapHA
3 2 5 4 4 7
10
4{12 6/12 4/12 6/12 11fl2 2/12
1{12 6 6 2/12 9 9/12 10
HA
8 8 10 10 12 9 II 13 16 II 12
3/12 6/12 4/12 6{12 3{12 2/12 6/12 6/12 3/12
GapHA
2 2 3 4 4 8 8 6 3 8 7
7{12 9/12 10/12 3{12 4{12 7/12 8{12 10/12 6/12 6{12 9/12
ofHA
+0 -0 +1 +0 +0 -I +I -0 +2 +I +2
9/12 3/12 6/12 3/12 7/12 5/12 4/12 9/12 8/12 3/12 3/12
* Height Age = corresponding to height plotted on 50th percentile Burgess Chart/Engelbach Endocrinological data and measurement were made available through the courtesy of Dr. Gilles Leboeuf
GainHA
5 4 6 4 5 4 5 3 6 3 3
0/12 9/12 6/12 7/12 7{12 5/12 8/12 8/12 4/12 9/12 9/12
Gaincm
36.2 27.3 35.9 29.2 34.3 23.5 28.9 17.5 28.3 18.7 19.7
August, 1975
329
GROWTH HORMONE IN HYPOPITUITARY DWARFS
Discussion Here described is a group of vulnerable children with very high risk of psychological and physical ailments as well as the effects of endocrine deficiency on the nervous system. From an early age they were unusual and posed a serious adaptation problem for their families. The secretion of HGH depends upon the " . . . wide variety of stressful stimuli, both physical and psychological" (3). It has been proven in Deprivational dwarfism (5) or Psychosocial dwarfism (12, 14, 15) that slow growth rhythm parallels adverse family and social circumstances along with HGH secretion. In a more favourable environment the syndrome is reversible. To what extent already decreased hypopituitary function is further diminished by the adverse family situation of dwarfs is very difficult to assess. Green, Schur and Lipkowitz (6) describe a dwarf patient with impairment of the ego apparatus along with "deficient endowment", making him less able to cope with daily social requirements. In the patients in this study the ego weakness left them vulnerable when coping with the stress of growing and of having to abandon previous secondary gains (9). The same ego weakness makes them prone to regression and depression. These patients have never been pleased with their bodies and hoped for miraculous TABLE
change by treatment; not only to grow taller but also to become larger, this was never achieved. Secord and Jourard (I 6) point out " . . . the body and the self tend to be cathected to the same degrees" and elsewhere (I7) that " . . . small size [is associated] with weak or negative feelings" in the form of low self-esteem. The older the patient and the longer he is exposed to this devastating feeling, the less able he is to adjust to change because his personality is formed on the basis of such a poor body cathexis. Each change is perceived as a symbolic loss or as a disruption of a precarious balance between the "libidinal or aggressive cathexis of the self-image" (II). The weak ego is broken down under the pressure of these repetitive blows to selfesteem - each successive period of treatment is another blow. Palmer states: "The child who . . . regards his own body as unacceptable may be so concerned with his identity as to be unable to function effectively in any way" (12) - these are the pathetic figures of dwarfs watching television all day and living on welfare. Akiskal and McKinney (I) state that "Depression is equated with chronic frustration stemming from environmental stresses that are beyond the coping ability of the individual who views himself as being helpless and finds relief in the rewards of the 'sick role'." The depression is a consequence of underlying immaturity in III
FAMILIES
(pts. A, B)
GROUP A:
Adequate families
GROUP B & C
Father Unpredictably absent from home
Mother
Depressed
(pt. D)
In and out of jail
Conversion and dissociative attacks
(pt. C)
Alcoholic and paralyzed by stroke Alcoholic Alcoholic
(pt. E) Died in mental hospital
(pt. I) (pt. G)
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CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
hypopituitary dwarfs, and seems to be common and predisposing to withdrawal and retreat, inhibition and neglect or constriction of the cognitive field (4). The depressive picture at the beginning of treatment was linked to a low quality of self-esteem (8) but later in the course of treatment the helplessness and emotional detachment dominated the clinical picture. But the dwarf suffers even more deeply from a lack of sense of identity. According to Palmer (13) the" ... sense of identity lies at the core of the ego and involves all ego function ... the role assigned to him, his affective evaluation of these identities. Thus identity may be considered as that part of the ego which looks at itself, which considers how the ego is looked at by others, and in which the sense of pride or guilt is centered." Therefore it is not surprising that their sense of identity is so fragile and confused since they are greatly constricted in their ego functions as perceived by themselves and by others. In this group of eleven patients marked emotional, medical, and family problems were observed. Treatments with HGH helped them to grow taller, but their longlasting emotional and family problems persisted and jeopardized their adjustment to growth. Conclusion No correlation was found between the amount of growth and pathology presented at the end of treatment, which was mainly related to the personality organization before treatment. A positive correlation was observed between chronological age at the beginning of treatment and subsequent adjustment - the younger the child was when the treatment began the more successful the treatment. This was attributed to their lesser and shorter awareness of their dwarf situation and to the earlier stage of personality formation. When regression occurred in the older patients it was of a psychotic type, but this was not observed in any of the other patients. A suggestive parallel relationship was found between HA (and CA) and lack of ACTH before treatment and psychosocial
Vol. 20, No.5
regression during treatment. It was noted that girls responded better to treatment with respect to psychosocial adjustment. References
r-., T. W.: Depressive disorders: toward a unified hypothesis, Science, J82: 4107, 1973. 2. Bierich, J. R.: On the aetiology of hypopituitary dwarfism in growth and growth hormone, A. Pecile, E. E. Miller, ExcerptaMedica, Amsterdam, 1972. 3. Brown, G. M. and Reichlin, S.: Psychological and neural regulation of growth hormone secretion, Psychosom. Med., 34: I, 1972. 4. Drash, W. P., Greenberg, E. N. and Money, J.: Intelligence and Personality in four Syndromes of Dwarfism in Human Growth-Body Composition, Cell Growth, Energy and Intelligence. Ed. Donald Clark, Philadelphia, Lea & Febiger, 1968. 5. Gardner, 1. L.: Deprivational dwarfism, Sci. Am., 227: I, 1972. 6. Green, S., Schur, H. and Lipkowitz, M.: Study of a Dwarf, Psychoanal. Study Child., XLV: 236,1959. 7. Hollingshead, A. and Redlich, F. C.: Social Class and Mental Illness , New York, John Wiley, 1958. 8. Kusalic, M., Fortin, C. and Gauthier, Y.: -Psychodynamic aspects of dwarfism: response to growth hormone treatment, Can. Psychiatr. Assoc.J., 17: 1,29-34,1972. 9. Kusalic, M. and Fortin, C.: Comparison of the Psychodynamics of Hypopituitary and Constitutional Dwarf Children. In V World Congress of Psychiatry, Abstracts No. 77 J , p. 381, Ciba-Geigy, Roche, Sandoz and Wander, 1971. 10. Kusalic, M., Fortin, C. and Gauthier, Y: Gender role in hypopituitary dwarfism. Presented at the Canadian Psychiatric Association Meeting, Vancouver, June 1973. II. Mendelson, M. E.: Psychoanalytic Concepts of Depression, Springfield, III. Charles C. Thomas, 1960. 12. Money, J., Wolff, G. and Annecillo, c. Pain agnosia and self injury in the syndrome of reversible somatotropin deficiency (psychosocial dwarfism), J. Autism Child. Schizophr. 2: 127, 1972. 13. Palmer, J.: Psychological Assessment of Children, New York, London, John Wiley and Sons, 1970. 14. Powell, G. F., Brasel, 1. A. and Blizzard, R. D.: Emotional deprivation and growth I. Akiskal, S. H. and McKinney,
August, 1975
GROWTH HORMONE IN HYPOPITUITARY DWARFS
retardation simulating idiopathic hypopituitarism. Part I clinical evaluation of the syndrome, N. Engl. J. Med. 276: 1271, 1967. 15. Reinehart, J. and Drash, A., Psychosocial dwarfism: environmentally induced recovery,Psychosom. Med. 31: 165, 1969. 16. Secord, F. P. and Jourard, M. S.: The appraisal of body cathexis and the self, J. Consult. Clin. Psychol., 17: 5,1953. 17. Secord, F. P. and Jourard, M. S.: Body size and body-cathexis, J. Consult. Clin. Psychol., 18: 3,1954.
Resume Cette etude longitudinale de 11 patients, de 5 a 21 ans , souffrant de nanisme hypophysaire et traites a I'horrnone de croissance, rapporte l' evol ution psychologique de ces sujets apres cinq annees de traitement. L'attitude vis-a-vis le nanisme, l'agressivite, l'espoir investi dans le traitement et l'attitude des parents envers le sujet sont les variables psychologiques retenues pour observation a travers une entrevue psychiatrique et une evaluation de la personnalite a l'aide de tests projectifs effectuees avant le debut du traitement et apres chaque periode de 6 mois d'administration de l'hormone. Le gain en taille varie entre 17,5 et 35,9 em apres 5 ans. Initialement, les sujets etaient decrits comme immatures avec une
331
tres pauvre estime de soi sous-jacente et une absence de toute manifestation agressive. Deja, apres le premier traitement, une reaction depressive prend le pas sur l'immaturite et quelques fantaisies agressives apparaissent. Mais au terme de 5 ans de traitement hormonal, malgre une croissance physique reduite relativement a leurs expectatives, le tableau clinique apparait assez sombre: 4 des 11 sujets (dont un a ete hospitalise pour depression psychotique) presentent des troubles graves de la personnalite. En conclusion, les auteurs n'ont pas trouve de relation entre l'indice de croissance et la pathologie observee a la fin du traitement hormonal; cette pathologie est plutot liee a l'organisation deficiente de la personnalite au depart. Par ailleurs, il semble que plus l'enfant est jeune plus l'effet du traitement est positif, sur Ie plan personnalite a tout Ie moins; ceci est impute au fait que la personnalite a cet age n'est pas encore cristallisee. Chez les patients plus ages on a observe une regression massive de type psychotique. On observe aussi une relation parallele possible entre I' age statural, l' absence de secretion d' ACHT avant Ie traitement et la regression psychosocia1e en cours de traitement. A signaler aussi, les deux seules filles du petit groupe etudie semblent mieux repondre au traitement en terme d'une meilleure adaptation psychosociale.
Pigmies placed on the shoulders ofgiants see more than the giants themselves. Lucan A.D. 39-65
Ottawa, Canada, August 1975
No.5
Somatopsychic Relationships GROWTH HORMONE TREATMENT IN HYPOPITUITARY DWARFS: Longitudinal Psychological Effects*
MARIA KUSALIC, M.D.l CLAIRE FORTIN, L.Ps. 2
This report deals with their psychological evolution in relation to physical growth.
This is one of a series of studies on the psychological effects of medically induced growth in a group of hypopituitary dwarfs treated with Human Growth Hormone (HGH). Before treatment these dwarfs were found to be psychologically immature and hypoactive, without any manifestation of aggressive drives, and had an underlying low self-esteem when compared with the normal population. When compared with a matched group with constitutional growth delay the hypopituitary patients were found to use denial, whereas the control group used well-organized compensatory mechanisms (9). Immaturity was found in both groups. When the hypopituitary dwarfs reached adolescence they lacked the core attributes of masculinity or femininity and manifested some ambiguity or even inversion in their choice of gender role (10). 'This paper was presented to the 10th European Conference of Psychosomatic Research, 1974, Edinburgh, Scotland. Revised manuscript received April 1975. This work was supported in part by Riviere des Prairies Hospital. 1 Research Psychiatrist, Riviere des Prairies Hospital and Psychiatrist. Jewish General Hospital, Montreal, P.Q. 2 Research Psychologist, Department of Psychology, H6pital St. Justine, Montreal, P.Q. tGilles Leboeuf, M.D., Department of Endocrinology, H6pital sr, Justine.
Can. Psychialr. Assoc. J. Vol. 20 (1975)
Method Subjects The group consisted of eleven patients, nine boys and two girls ranging in age from 5 10/12 to 21 5/12 years at the beginning of treatment (Table II). All patients were diagnosed as hypopituitary dwarfs by an endocrinologist] who treated them for more than five years by administration of HGH for six months, alternating with a six-month hormone free period. Long before their hypopituitary insufficiency was recognized, most of them had suffered medical problems - long delivery and often breech presentation (2); slight lag in all aspects of development, both locomotor and verbal; hypomotility and sleeping problems. The severity of feeding difficulties in infancy led the parents to consult a pediatrician and there is evidence that these problems still persist. All patients (except the younger girl) are being treated with synthroid because of lack of TSH, and four of them are also lacking ACTH (patients B,C,F and H). The medical diagnosis was established before four years of age, with a mean of 22 months. Procedure Following the selection by the endocrinologist, the subject and his family were
325
326
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
assessed by a psychiatrist. A psychological evaluation based on a battery of tests followed - the Wechsler Intelligence Scale for Children; the Machover 'Draw-a-Person' test, along with the questionnaire suggested by Machover which probes for associations about the projected drawn figure regarding ambitions, feelings, part of the body preferred, how others consider the drawn person, whether the subject would trade places with him and an additional drawing of the family; the Rorschach; and eight selected cards of the Thematic Apperception Test (I, 3BM, 8BM, II, l3B, 17BM, l8GH, 19) chosen for particular reference to aggression achievement needs and self-representation. In both evaluations special emphasis was placed on: attitude towards dwarfism; expectation of treatment; aggression; oral needs; and parental attitude toward the dwarf. Following these initial evaluations each interviewer wrote an independent report and summary, and then after discussion the researchers arrived at a consensus. The procedure was repeated after each period of treatment.
Results The gain in height ranged from 17.5 to 35.9 em after five years of treatment with a mean of27.2 em. Before treatment the hypopituitary dwarfs were very immature, with an underlying low self-esteem and a lack of overall aggressiveness. After the first treatment the low self-esteem became the main feature and aggressive drives began to manifest themselves mildly in the patients' phantasies; after the second, immaturity remained but was overshadowed by depression which manifested itself most clearly in low self-esteem whereas aggressive drives became evident with the occasional appearance of verbal discharge; after the third, the low self-esteem remained but the depression appeared more in the form of affective withdrawal, and the aggressive drives seemed to grow in direct proportion to changes in a patient's stature; after the fourth, immaturity was still present but became overshadowed by an even lower self-esteem and a state of -helplessness and emotional detachment. Some patients started to regress - 'B' became enuretic, 'C' became enuretic and encopretic, 'H' had delusions, and T developed psychotic depression. After the fifth, the group as a
Vol. 20, No.5
whole remained depressed and apathetic but verbally aggressive. The regression in the patients which began after the fourth treatment became even more prominent, contrary to expectation and in spite of the continuation of treatment for more than five years. The patient 'I' in whom the psychosis emerged after the fourth treatment required repeated admission. The delusional and enuretic patients remained the same and the one who had becomeenuretic and encopretic had to be admitted to an institution. Examining other areas will throw further light on their functioning and they are grouped according to age. Life Style As the age increases peer contact decreases. Group A (one boy, one girl, both aged II) are under-achievers academically. Their playmates are younger children of their size, play is infrequent, they are followers in a group, and do not participate in team sports. Overall their psychological adjustment to linear text growth was the best. Group B (two boys 14 years old and one girl 15 years old), one boy and the girl are under-achievers; their playmates are mostly of their own size - although much younger but they have limited contact with their peers; and they do not participate in groups. The third patient is encopretic and living in total social isolation, he repeated Grade III and tripled Grade V. None of them have hobbies. Group C (all males ranging in age from 20 to 27 years) - three are welfare recipients and do nothing but watch television; three others are attending school and repeating grades; they do not mix at all with others and have no hobbies. Sexual Identity (All groups) All have adequate gender identity but as they grow older role inversion becomes more pronounced; the males manifest passivity, conformity and dependency. There is apparently no overt sexual activity (10).
August, 1975
GROWTH HORMONE IN HYPOPITUITARY DWARFS
Fig. I
Fig. 2
327
328
Vol. 20, No.5
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
Attitudes Toward Treatment All have unrealistic hopes of obtaining normal stature. There is no apparent relationship between gain in Height Age (HA) and initial psychosocial adjustment - those who manifest the poorest adjustment were equally disturbed above and below the medium gain in HA. However, there was a relationship between HA and Chronological Age (CA) before treatment and subsequent to social adjustment. Those with the lowest original HA (and second and third lowest CA) became enuretic during the course of treatment and remained so to the end; those with the highest HA (and second and third highest CA) became psychotic during treatment and remained so (see Table II).
TABLE I DESCRIPTION OF POPULAnON
Case Identification No.
Name
Sex
I.Q.
A B C D E F G H I
L.L. D.F.
F M
P.C.
M F
99 86 93 122 95 87 96 96 120 114 83
Y.M.
J K
Families Out of eleven families nine were found to have a high degree of pathology. All except two of the dwarfs live with their natural parents; these two were both adopted (one by their oldest sister and another by a cousin). Seven families are from rural Canada, mostly Quebec, the other four from Montreal. They belong to Groups IV and V of Hollingshead's Scale (7) except two who belong to the white collar group. From a dynamic point of view all families except two were assessed as inadequate because of the presence of paren-
Before Treatment
M
L.M. S.J. J.G. F.M. P.L.
M M M M
R.L.
M
G.S.
M
Chronological Age
5 6 8 9 II 13
15 15 16 17 21
10/12 3/12 10/12 7{12 4/12 3{12 7/12 7/12 4/12 6/12 6/12
tal schism and because of ambivalent, over-protective covertly rejecting parental attitudes toward the dwarfed patients, which increased at the end of treatment. There was also an unusually high degree of individual pathology: eight out of twentytwo parents have marked psychiatric disorder. It is interesting to note that the two youngest patients have the least pathology in the family. This could be a coincidence due to the small size of the group or to the rather early initiation of treatment at the age of five.
TABLE II EFFECT OF TREATMENT ON HEIGHT AND HEIGHT AGE* (HA)
Before Treatment Pts A B C D E F G H I J K
CA
10{12 3/12 10/12 7/12 4/12 fl2 7{12 7/12 4/12 17 6/12 21 6/12
5 6 8 9 II 13 15 15 16
HA
2 3 3 4 6 5 5 9 10 7 8
6{12 9/12 6/12 11/12 5{12 1/12 7/12 9{12 2/12 9/12 6/12
After Treatment Change in Gap GapHA
3 2 5 4 4 7
10
4{12 6/12 4/12 6/12 11fl2 2/12
1{12 6 6 2/12 9 9/12 10
HA
8 8 10 10 12 9 II 13 16 II 12
3/12 6/12 4/12 6{12 3{12 2/12 6/12 6/12 3/12
GapHA
2 2 3 4 4 8 8 6 3 8 7
7{12 9/12 10/12 3{12 4{12 7/12 8{12 10/12 6/12 6{12 9/12
ofHA
+0 -0 +1 +0 +0 -I +I -0 +2 +I +2
9/12 3/12 6/12 3/12 7/12 5/12 4/12 9/12 8/12 3/12 3/12
* Height Age = corresponding to height plotted on 50th percentile Burgess Chart/Engelbach Endocrinological data and measurement were made available through the courtesy of Dr. Gilles Leboeuf
GainHA
5 4 6 4 5 4 5 3 6 3 3
0/12 9/12 6/12 7/12 7{12 5/12 8/12 8/12 4/12 9/12 9/12
Gaincm
36.2 27.3 35.9 29.2 34.3 23.5 28.9 17.5 28.3 18.7 19.7
August, 1975
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GROWTH HORMONE IN HYPOPITUITARY DWARFS
Discussion Here described is a group of vulnerable children with very high risk of psychological and physical ailments as well as the effects of endocrine deficiency on the nervous system. From an early age they were unusual and posed a serious adaptation problem for their families. The secretion of HGH depends upon the " . . . wide variety of stressful stimuli, both physical and psychological" (3). It has been proven in Deprivational dwarfism (5) or Psychosocial dwarfism (12, 14, 15) that slow growth rhythm parallels adverse family and social circumstances along with HGH secretion. In a more favourable environment the syndrome is reversible. To what extent already decreased hypopituitary function is further diminished by the adverse family situation of dwarfs is very difficult to assess. Green, Schur and Lipkowitz (6) describe a dwarf patient with impairment of the ego apparatus along with "deficient endowment", making him less able to cope with daily social requirements. In the patients in this study the ego weakness left them vulnerable when coping with the stress of growing and of having to abandon previous secondary gains (9). The same ego weakness makes them prone to regression and depression. These patients have never been pleased with their bodies and hoped for miraculous TABLE
change by treatment; not only to grow taller but also to become larger, this was never achieved. Secord and Jourard (I 6) point out " . . . the body and the self tend to be cathected to the same degrees" and elsewhere (I7) that " . . . small size [is associated] with weak or negative feelings" in the form of low self-esteem. The older the patient and the longer he is exposed to this devastating feeling, the less able he is to adjust to change because his personality is formed on the basis of such a poor body cathexis. Each change is perceived as a symbolic loss or as a disruption of a precarious balance between the "libidinal or aggressive cathexis of the self-image" (II). The weak ego is broken down under the pressure of these repetitive blows to selfesteem - each successive period of treatment is another blow. Palmer states: "The child who . . . regards his own body as unacceptable may be so concerned with his identity as to be unable to function effectively in any way" (12) - these are the pathetic figures of dwarfs watching television all day and living on welfare. Akiskal and McKinney (I) state that "Depression is equated with chronic frustration stemming from environmental stresses that are beyond the coping ability of the individual who views himself as being helpless and finds relief in the rewards of the 'sick role'." The depression is a consequence of underlying immaturity in III
FAMILIES
(pts. A, B)
GROUP A:
Adequate families
GROUP B & C
Father Unpredictably absent from home
Mother
Depressed
(pt. D)
In and out of jail
Conversion and dissociative attacks
(pt. C)
Alcoholic and paralyzed by stroke Alcoholic Alcoholic
(pt. E) Died in mental hospital
(pt. I) (pt. G)
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CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
hypopituitary dwarfs, and seems to be common and predisposing to withdrawal and retreat, inhibition and neglect or constriction of the cognitive field (4). The depressive picture at the beginning of treatment was linked to a low quality of self-esteem (8) but later in the course of treatment the helplessness and emotional detachment dominated the clinical picture. But the dwarf suffers even more deeply from a lack of sense of identity. According to Palmer (13) the" ... sense of identity lies at the core of the ego and involves all ego function ... the role assigned to him, his affective evaluation of these identities. Thus identity may be considered as that part of the ego which looks at itself, which considers how the ego is looked at by others, and in which the sense of pride or guilt is centered." Therefore it is not surprising that their sense of identity is so fragile and confused since they are greatly constricted in their ego functions as perceived by themselves and by others. In this group of eleven patients marked emotional, medical, and family problems were observed. Treatments with HGH helped them to grow taller, but their longlasting emotional and family problems persisted and jeopardized their adjustment to growth. Conclusion No correlation was found between the amount of growth and pathology presented at the end of treatment, which was mainly related to the personality organization before treatment. A positive correlation was observed between chronological age at the beginning of treatment and subsequent adjustment - the younger the child was when the treatment began the more successful the treatment. This was attributed to their lesser and shorter awareness of their dwarf situation and to the earlier stage of personality formation. When regression occurred in the older patients it was of a psychotic type, but this was not observed in any of the other patients. A suggestive parallel relationship was found between HA (and CA) and lack of ACTH before treatment and psychosocial
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regression during treatment. It was noted that girls responded better to treatment with respect to psychosocial adjustment. References
r-., T. W.: Depressive disorders: toward a unified hypothesis, Science, J82: 4107, 1973. 2. Bierich, J. R.: On the aetiology of hypopituitary dwarfism in growth and growth hormone, A. Pecile, E. E. Miller, ExcerptaMedica, Amsterdam, 1972. 3. Brown, G. M. and Reichlin, S.: Psychological and neural regulation of growth hormone secretion, Psychosom. Med., 34: I, 1972. 4. Drash, W. P., Greenberg, E. N. and Money, J.: Intelligence and Personality in four Syndromes of Dwarfism in Human Growth-Body Composition, Cell Growth, Energy and Intelligence. Ed. Donald Clark, Philadelphia, Lea & Febiger, 1968. 5. Gardner, 1. L.: Deprivational dwarfism, Sci. Am., 227: I, 1972. 6. Green, S., Schur, H. and Lipkowitz, M.: Study of a Dwarf, Psychoanal. Study Child., XLV: 236,1959. 7. Hollingshead, A. and Redlich, F. C.: Social Class and Mental Illness , New York, John Wiley, 1958. 8. Kusalic, M., Fortin, C. and Gauthier, Y.: -Psychodynamic aspects of dwarfism: response to growth hormone treatment, Can. Psychiatr. Assoc.J., 17: 1,29-34,1972. 9. Kusalic, M. and Fortin, C.: Comparison of the Psychodynamics of Hypopituitary and Constitutional Dwarf Children. In V World Congress of Psychiatry, Abstracts No. 77 J , p. 381, Ciba-Geigy, Roche, Sandoz and Wander, 1971. 10. Kusalic, M., Fortin, C. and Gauthier, Y: Gender role in hypopituitary dwarfism. Presented at the Canadian Psychiatric Association Meeting, Vancouver, June 1973. II. Mendelson, M. E.: Psychoanalytic Concepts of Depression, Springfield, III. Charles C. Thomas, 1960. 12. Money, J., Wolff, G. and Annecillo, c. Pain agnosia and self injury in the syndrome of reversible somatotropin deficiency (psychosocial dwarfism), J. Autism Child. Schizophr. 2: 127, 1972. 13. Palmer, J.: Psychological Assessment of Children, New York, London, John Wiley and Sons, 1970. 14. Powell, G. F., Brasel, 1. A. and Blizzard, R. D.: Emotional deprivation and growth I. Akiskal, S. H. and McKinney,
August, 1975
GROWTH HORMONE IN HYPOPITUITARY DWARFS
retardation simulating idiopathic hypopituitarism. Part I clinical evaluation of the syndrome, N. Engl. J. Med. 276: 1271, 1967. 15. Reinehart, J. and Drash, A., Psychosocial dwarfism: environmentally induced recovery,Psychosom. Med. 31: 165, 1969. 16. Secord, F. P. and Jourard, M. S.: The appraisal of body cathexis and the self, J. Consult. Clin. Psychol., 17: 5,1953. 17. Secord, F. P. and Jourard, M. S.: Body size and body-cathexis, J. Consult. Clin. Psychol., 18: 3,1954.
Resume Cette etude longitudinale de 11 patients, de 5 a 21 ans , souffrant de nanisme hypophysaire et traites a I'horrnone de croissance, rapporte l' evol ution psychologique de ces sujets apres cinq annees de traitement. L'attitude vis-a-vis le nanisme, l'agressivite, l'espoir investi dans le traitement et l'attitude des parents envers le sujet sont les variables psychologiques retenues pour observation a travers une entrevue psychiatrique et une evaluation de la personnalite a l'aide de tests projectifs effectuees avant le debut du traitement et apres chaque periode de 6 mois d'administration de l'hormone. Le gain en taille varie entre 17,5 et 35,9 em apres 5 ans. Initialement, les sujets etaient decrits comme immatures avec une
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tres pauvre estime de soi sous-jacente et une absence de toute manifestation agressive. Deja, apres le premier traitement, une reaction depressive prend le pas sur l'immaturite et quelques fantaisies agressives apparaissent. Mais au terme de 5 ans de traitement hormonal, malgre une croissance physique reduite relativement a leurs expectatives, le tableau clinique apparait assez sombre: 4 des 11 sujets (dont un a ete hospitalise pour depression psychotique) presentent des troubles graves de la personnalite. En conclusion, les auteurs n'ont pas trouve de relation entre l'indice de croissance et la pathologie observee a la fin du traitement hormonal; cette pathologie est plutot liee a l'organisation deficiente de la personnalite au depart. Par ailleurs, il semble que plus l'enfant est jeune plus l'effet du traitement est positif, sur Ie plan personnalite a tout Ie moins; ceci est impute au fait que la personnalite a cet age n'est pas encore cristallisee. Chez les patients plus ages on a observe une regression massive de type psychotique. On observe aussi une relation parallele possible entre I' age statural, l' absence de secretion d' ACHT avant Ie traitement et la regression psychosocia1e en cours de traitement. A signaler aussi, les deux seules filles du petit groupe etudie semblent mieux repondre au traitement en terme d'une meilleure adaptation psychosociale.
Pigmies placed on the shoulders ofgiants see more than the giants themselves. Lucan A.D. 39-65
