Psychosocial Development in Children With Chronic Renal Insufficiency

Psychosocial Development in Children With Chronic Renal Insufficiency Warren E. Grupe, MD, Ira Greifer, MD, Stanley I. Greenspan, MD, Lewis A. Leavitt, MD, and George Wolff, PhD

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URING the past decade, protracted survival of infants and children with chronic renal insufficiency (CRI) has become the expected outcome. 1.2 During the same period , the problems of physical growth and development have attracted considerable interest, centering on the nutritional, hormonal , metabolic , and other factors that contribute to small stature . 3-5 It has been relatively recent that concerns over cognitive and psychologic development have emerged .s' This expand ing interest has been stimulated , perhaps , by a genuine improvement in the prospect for longevity, and by our improved abilities to apply dialytic and transplantation therapy at younger ages. 8-10 Studies of cognitive and psychosocial function in the adult have focused on memory, attention , and general IQ. 11.12 In the adult with chronic renal failure, deficits in function have been defined in short-term visual and auditory memory, sustained and selective attention , speed of decision making, and general performance IQ scores. I 1.12 These deficits improve with the initiation of dialytic therapy and virtually resolve with successful transplantation.!? Performance evaluation in the adult, however, centers on whether or not that which has been attained can be retained. In the developing child , the attention must broaden to include the actual processes of attainment. For example, a failure to attain at one developmental level may seriously alter the pathways for any subsequent attainments . Furthermore, a failure to retain (ie, regression) only serves to magnify the inability to attain. Finally, and possibly less obvious, a child who merely fails to gain, by definition , is falling further and further behind. There is little wonder, therefore, that conventional mental health interFrom the Children 's Hospital . Boston. Massachusetts; Albert Einstein College oj Medicine. Bronx. New York; the Mental Health Study Center. Adelphi. Maryland; the Universityof Wisconsin . Madison; and the Kinderklinik Medical School. Hannover. FRG. Address reprint requests to Warren E. Grupe. MD. the Children 's Hospital . 300 Longwood Ave . Boston. MA 02ll5. © 1986 by the National Kidney Foundation. lnc. 0272-6386/86/0704-ooll $03.00/0 324

ventions, applied at the onset of end-stage renal disease (ESRD), have limited effect, except for the value that sensitive support has on the process of adaptation to illness and procedures. 13 This broadened vulnerability to the influences of chronologie age, maturational phase, and developmental stage is unique to the rapidly changing organism called the child. It seems reasonable, therefore, that those developmental attainments most characteristic for a particular age or size will be the ones most vulnerable to alteration by illness, and that the more protracted the illness, the broader the impact on the developmental sequence. Although these assumptions appear to have innate validity, previous empirical testing has found the overall adaptation of a group of children treated for ESRD to be quite similar to what one might expect had no serious medical problems existed.P'!" Likewise, several studies of children with renal insufficiency from a young age have failed to define significant deviations in attention, problem solving, learning skills, general intellectual capability, or overall achievement. 15 18 Nevertheless, major psychologic and emotional problems do develop at some point in many children , particularly during adolescence when there is poor family support , family disorganization, low income , a vulnerable personality, and a complex medical course. 6.19.20 Although it has been difficult to define the extent to which renal insufficiency or its treatments have contributed to either the risk factors or the maladaptation, it seems reasonable to explore the potential sites where preventative intervention might be centered. INFANT-PARENT INTERACTION

One model for understanding the influence of disease on emotional development has been proposed, whereby the stages of progressive emotional, social, and cognitive maturation can be monitored in infants in much the same fashion as one follows the progressive sequence of motor or sensory developrnent.w-? The normal infant processes the information it receives through physiologic, sensory, and motor experiences, including

American Journal of Kidney Diseases, Vol VII. No 4 (April), 1986: pp 324-328

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those from family and community, to develop a human relationship . This model defines an orderly progression through developmental phases by the infant, who has the capacity to continually reorganize and differentiate experiences at each developmental stage. In sequence, the infant progresses from the initial attempts to maintain emotional and physiologic homeostasis in the midst of multiple sensory stimuli, through attachment to parents, to formal communication, then behavioral organization and role identification leading to the ability to initiate ideas, images, and symbols, followed by the development of language and the ability of cooperative interaction with others. This model -implies that disruption of this process by disease can be expected to produce significant immediate and later aberrations in emotional performance and subsequent progress. For example, a neonate with renal disease, through hospitalization, separation, or treatment, may never gain mastery over an array of abnormal stimuli ; likewise, illness may deny the 3- to 4-month-old the personal interaction required to form human attachment. Interactively, the child's caretakers may be so consumed with anxiety or despondency over the illness that the infant reaching certain milestones finds its human environment unprepared to provide the appropri ate stimuli or return the appropriate response. The result can be parental overprotection that destroys the infant's initiative, or parental unresponsiveness that confounds meaningful interaction . It might be logically assumed that a significant disruption of the sequence through illness, hospitalization, separation, or therapy will either alter the child's progression or abort the final attainment of functional emotional, cognitive, or social competency. However, it is not yet clear that the child with chronic renal disease, or any other chronic illness, follows a developmental course analogous to the normal child or that the chronic illness, per se, increases the risk of abnormal development. It is also not known if the child with CRI follows the same developmental sequences with the same timing, or at a slower pace. Likewise, the presence of compensating, idiosyncratic, or alternative pathways for development have not been excluded in the child with chronic disease. Similarly, the cumulative impact of multiple insults over time on the developmental sequence, and the subsequent effect this may have on attainment, has not been delineated . Finally, if attain-

ment of these initial milestones is an essential foundation for subsequent coping as disease progresses, it could be postulated that infants with chronic renal disease may require even higher attainment than normal children . In this regard , family and interactional factors could be very important factors. COGNITIVE FUNCTION

Whatever the ultimate importance of these early disruptions on the child's ultimate level of attainment, it has been difficult to elucidate abberations in psychosocial and cognitive abilities that can be related to the presence of CRI. As with other chronic illnesses, psychosocial normalcy prevails, or is only subtly impaired, when assessed by standard tests. I H 8 Trachtman et al, evaluating a group of 19 patients with ESRD, found their overall cognitive achievement to be similar to a group of children with chronic asthma. 15 However, the children on hemodialysis were noted to perform less well in tests of general intelligence, mathematics, and memory. 15 The ESRD group, which ranged in age from 10 to 23 years, was not homogeneous, however. Those children whose dialytic therapy began below the age of 12 years scored much lower on tests of intelligence , auditory and visual memory skills, and school achievement; this suggests that the effect on performance may be more dependent on the age of onset rather than to the degree or etiology of the disease . 15 It has not yet been possible to separate the impact of the age at onset from the duration of abnormal renal function. Rotundo et al noted that only three of 23 children whose renal failure appeared in the first year of life had normal neurologic function, while the other 20 had disturbing deficits that included developmental delay , microcephaly, seizures , hypotonia, and dyskinesia.P The severity of these defects introduces the confounding variable of profound organic neurologic dysfunction on attainment. INTERACTIVE BEHAVIOR

In an attempt to evaluate the influence of protracted illness in a defined clinical subset with congenital disease, Wolff et al have prospectively studied a group of 23 patients with infantile nephropathic cystinosis. 24 26 This is a group in which one might expect multiple interruptions in the developmental sequence because of the early

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onset of hospitalization , separation, life threatening metabolic crises, and painful treatments. Yet previous work had shown that this group of patients, despite severe illness and a delay in school entry, demonstrated basically normal intellectual, scholastic, and social development until the middle school years when the necessity for hemodialysis became more prevalent. 25 .26 Nevertheless, with the regular sampling available through a longitudinal study, periods of reversal became apparent. For example, transient developmental delay appeared during episodes of metabolic crisis, while hospitalization magnified the degree of unsuccessful coping. As ESRD developed, there was a decline in school performance and an increase in behavioral problems. The patients' own behavior and accomplishment became entwined with the attitudes and interactive behavior of both the parents and the medical team. Difficulties with behavior or noncompliance with treatment were more noticeable in those instances in which parents exhibited ambivalent attitudes toward rearing and /or toward the treatment program . The ambivalence, in turn, developed early in the course of the disease associated with hospitalization, separation, depression , and a sense of the impending loss of their child . Likewise, the medical team exhibited fear of the life threatening disease and a tendency to avoid emotionally injurious situations. Medical personnel are dependent on the calls for help from patients and parents, who must learn to cope, whereas the physicians need only conceptualize. When asked , patients and parents can recall multiple examples of how the response of medical personnel reinforced dependency or limited advancement. Other external and internal factors also created pressures, particularly in the 12- to 14year-olds , that modified the patients' behavior and attitudes . These pressures included a lack of social acceptance , short stature, teasing by peers, delayed puberty, and the need to adapt to life as a small adult regularly treated as if much younger.

METHODOLOGY Since developmental difficulties may not become evident until several years have elapsed, and even then only through the use of .regular interviews, home visits , and outreach, it would be of great value if appropriate and effic ient testing procedures were available that could identify problems early enough for successful intervention.

However, the predictive value of even the formal and standard vehicles we use to evaluate cognitive performance are affected by biologic, psychosocial , and methodologic variables.?? "Intelligence" has multiple components that are generally accessed by means of behavioral assessment. 28 Learning and cognition include such components as attention, memory, sensory processes, sensory motor coordination, and language. Each component may be differentially affected by disease or other biologic events . Furthermore, the developmental process is transactional, ie, the child and the environment are continually interacting with each other, one producing changes in the other. Thus, psychosocial variables, like pathophysiologic variables, can differentially effect the measured performances. For example, by 48 months of age, social economic class has already had an effect on the developmental scores. Likewise, data have been presented indicating that a parent's perception of a child changes the parents' response to a change in an infant 's social signals. s? Important to medical personnel is the indication that it made no difference to the parent's responsiveness whether the perception was self-generated or artificially induced by an experiment. This perception is then reflected in their infant's own response to a new situation and the child 's cognitive function measured at a later time . To label an infant, therefore, can change a parent's perception, alter both parents' attentiveness , and apparently modify their infant's cognitive development. Thus , the contribution of social forces (such as home environment, family structure, parental perception, and selfesteem) to the measurement of cognition requires elucidation in any circumstance relating cognition to a chronic disease process . Finally, methodologic problems with assessment can be inferred from data that failed to show a strong relationship between infant test scores and measures of cognitive performance in later childhood .P'>? In the dynamics of normal development , there may be significant qualitative shifts in intellectual functioning that occur during childhood . Performance of skills that develop at different ages may be only mode stly related , which may be due, in part, to the behavioral repertoire that is accessible by current testing procedures. Nevertheless, there may be genuine discontinuity between various cognitive functions that on the surface appear to be related . However, it may also

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be that current testing procedures in the infant are not adequate to examine those functions that are truly related to subsequent cognitive capabilities . CONCLUSIONS

Several conclusions emerge, although each would benefit from further investigation . Although the studies are incomplete, there is more evidence than not that infants and children with eRI are not at a greater risk or lesser risk of psychosocial or cognitive dysfunction than children with other chronic illnesses . The extent of the impact of their illness on developmental outcome may be a function of the age at which the disease , becomes apparent, although the importance of the duration of the pathophysiologic process is yet to be determined. It is probable that the behavioral capabilitie s that characteristically emerge during a particular developmental period are the ones most

vulnerable to the assault of illness. Although it appears intuitively valid that aberrations in the sequence or the manner by which new behavior is acquired is detrimental, no clear relation can be yet established. The measurement of important developmental functions is particularly difficult in the young child . There is certainly a poor relationship between test results during infancy and subsequent function, and no assurance that current testing procedures are sensitive enough to evaluate function that is truly related to that which, later on, we call cognition . Finally, attention centered on the interactive components of the child's development may prove quite fruitful, since emotional, social, and cognitive growth in the infant appears transactional and, in the presence of chronic illness, acutely sensitive to the actions , attitudes , and behaviors of those who care for the infant, parents and physicians alike .

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GRUPE ET AL 28. Schnell RR : Standardized psychological testing , in Levine M, Carey W, Crocker A , Gross R (eds) : Developmental-Behavioral Pediatrics. Philadelphia, Saunders, 1983, pp 1004-1021 29 . Donovan W, Leavitt LA : Early cognitive development as a funct ion of maternal behavioral and phys iologic responsiveness . Child Dev 49 :1251 -1254, 1978 30 . Ross LE , Leavitt LA : Process research: Its use in prevention and intervention with high-ri sk children, in Tjossem TD (ed) : Intervention Strategies for High Risk Infants and Young Children. Baltimore, University Park Press, 1976, pp 107-117