Personality Development in Children with Growth Hormone Deficiency

Diane Rotnem, A.C.S. W., Myron Genel, M.D., Raymond L. Hintz, M.D., and Donald J. Cohen, M.D.

Abstract. Personality and social development of 14 children with hypopituitary short stature, and age- and sex-matched controls were assessed by interview, observation, and projective testing. Children with growth hormone deficiency suffer from pervasive disturbances in identity formation, distorted body image, difficulties in the expression of aggression, and feelings of incompetence. The children's depression, emotional immaturity, and general personality difficulties are related to the way in which short stature elicits behavior appropriate to younger children; the child's sense of guilt for the short stature; parental overprotectiveness; increasing disparity in size from peers; and perhaps to basic endocrinological effects on central nervous system maturation.

General physical appearance shapes the social interactions of both animals and man (Darwin, 1872; Hess, 1970). Even relatively minor atypicalities may momentarily alter the quality of a social encounter (Goffman, 1959; 1974). In childhood, when one's sense of self-worth and personal identity are established mainly on the basis of the reactions of others, major differences in appearance may lead to enduring and profound personality difficulties (Burlingham, 1972; Erikson, 1959; Winnicott, 1965). The emotional scars of many children with severe rashes, facial anomalies, and orDiane Rotnem is Pediatric Research Social Worker, Department of Pediatrics and the Children's Clinical Research Center, Yale-New Haven Hospital. Dr. Genel is Associate Professor of Pediatrics and Program Director, Children's Clinical Research Center, Yale University School of Medicine. Dr. Hintz is Assistant Professor of Pediatrics, and Head of the Division o/Pediatric Endocrinology, Stanford University. Dr. Cohen is Associate Professor of Pediatrics, Psychiatry, and P.I}chology, and Associate Program Director for Ps}chosocial Research, Children's Clinical Research Center, Yale Univeni(y School of Medicine. This investigation was supported in part by the Children's Clinical Research Center Grant RR 00125, General Clinical Research Centers Program, Division of Research Resources, Nationallnstitutes of Health, Public Health Sen-ice Research Grant HD-03008; and the Grant Foundation o/New York. The studies were made possible by the collaboration of the nursing staff of the Children's Clinical Research Center under the direction of Mary Carey, R.N. Dr. Haskell Cohen gave permission for reproduction of TED test pictures. Reprints may be requested from Dr. Cohen at the Yale Child Study Center, 333 Cedar Street, New Haven, CT 06510.

412

Children with Growth Hormone Deficiency

413

thopedic deformities, for example, are painfully evident to any experienced clinician (Cohen, 1974, 1975). This study explores the ways in which psychological endowment, self-perception, and social treatment interact in the emergence of personality in children with hypopituitary dwarfism. HYPOPITUITARY DWARFISM

Hypopituitarism with impaired secretion of growth hormone is a relatively rare cause of short stature in children, accounting for 10 to 15 percent of children referred to pediatric endocrinology centers for evaluation of impaired growth (Brasel and Blizzard, 1974). Generally of unknown etiology (idiopathic), growth hormone secretion deficiency is often accom panied by deficiency of other tropic pituitary hormones (ACTH, TSH, or gonadotropins) and is rarely of familial origin. Hypopituitarism may also be of organic etiology, most commonly secondary to craniopharyngiomas and other tumors of the hypothalamic-pituitary area, and occasionally, inflammatory lesions, such as histocytosis X. Children with idiopathic hypopituitarism are generally of normal size at birth. One third demonstrate growth failure within the first year of life and 50 percent are significantly below the 3rd percentile by 2 years of age. Without specific hormone replacement, afAicted children grow less than 2 inches per year and often fail to achieve a height greater than 5 feet. Although diminuitive, these children appear perfectly normal, except for their characteristically immature features, and have normal intellectual capacity (Drash et aI., 1968; Money and Pollitt, 1968; Pollitt and Money, 1964). Parental concern genet'ally arises during nursery school or in the early school years. However, it is unusual for a child to receive a medical diagnosis before age 5 or 6 years unless other associated hormonal deficiencies are present. Since the isolation of growth hormone (Li and Papkoff, 1956), specific therapy has been available; however, replacement therapy has been restricted to a small number of children because of the scarcity of HGH and the absence of a suitable synthetic substitute. The hypopituitary child may have profound psychological problems, including social isolation, infantilism, negative self-concept, and difficulties in displaying aggression (Drash et aI., 1968; Schwartz and Sturges, 1973). These disturbances may be traced, in large part, to the impact of the child's appearance in shaping social interactions with family and others. Children with short stature are different from those with other chronic diseases who are

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Diane Rotnem et al.

frequently exposed to pain, medication, and hospitalization (Freud, 1952; Green and Solnit, 1964; McCollum, 1975; Mattsson, 1~72). Systematic study of personality changes with HGH replacement offers the greatest promise of unraveling the complex interactions between hormonal deficiency, personality, and social interaction. However, such investigation is complicated by several sources of variance: initial personality differences; varying patterns of family adaptation; age at start of treatment; response to treatment; and variability in growth hormone preparation. In the present study, longitudinal observation and use of each child as his or her own control reduce some of these sources of variance. In this paper, we report social and psychological observations on 14 children with hypopituitary short stature before the institution of HGH replacement. Future reports will describe the response of these children and their families to HGH treatment.

DESCRIPTION OF STUDY

Subjects

Fourteen patients with documented growth hormone deficiency (ages 6;6 to 16;9 years; mean 10 years, 8 months; SO 4;5 years), 2 females and 12 males, were invited to participate in the personality study. Consent was obtained from subjects and their parents (or guardians) who came from predominantly middle-income families. Growth hormone deficiency had been previously diagnosed in all on the basis of impaired HGH response to insulin-induced hypoglycemia and to either L-Dopa stimulation or arginine infusion (Frazier, 1974). Height age was calculated by extrapolating to the age at which the height represented the 50th percentile on the Boston Children's Hospital growth grids, and all patients were under the 3rd percentile. Bone age was assessed by X-rays of the hand and wrist (Greulich and Pyle, 1959). Thyroid function ,,'as assessed by measurement of total thyroxine and thyroxine-binding capacity (Seligson and Seligson, 1972). TSH determination, and by RAI uptake before and after TSH stimulation. In ;) patients (F., J\'., and 0.) TSH response to infusion of thyrotropic releasing factor (TRF), 7 mcg.lkg. LV., was also assessed (Foley et aI., 1972). Pituitary-adrenal function was determined by plasma cortisol response to insulin-induced hypoglycemia and by urine hydroxycorticoid and compound S ,'esponse to metyrapone inhibition (300 mg.lm 2 q. 4h.). In patients over 13 years of age, gonadotropin seC1'etion ,vas

41;)

Children with Growth Hormone Deficiency

assessed by radioimmunoassay determination of random serum LH and FSH (Bio-Science Laboratories, Van Nuys, California). Fourteen patients wel"e considered to have idiopathic hypopituitarism, 9 with presumed isolated HGH deficiency (table I) and 5 Tahle 1 Suhjects \\i I h Isolal('d HCH Dcfici('nn

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with coexisting deficiency of other anterior pituitary hormones (table 2). One patient, 0 .. had combined anterior and postel"ior pituitary deficiency secondaq to a hypothalamic lesion of unknown etiology. One other patient, D., also had a diagnosis of incontinenTable

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