Journal of BehavioraI Medicine, FoL 2, No. 2, 1979

Psychological Development of the Child with Leukemia: A Review Christine Eiser 1

Accepted for publication: February 5, ] 9 79

With improvements in prognosis for children with leukemia, there has been a corresponding increase in degree of adverse psychological effects on the patient and his family. This review considers (1) the methods of coping adopted by parents, siblings, and the patient and (2) the possibility of damage to normal intellectual growth following the type of medical treatment involved. Implications of previous research in terms of practical heIp for the families and theoretical issues regarding normal brain development are discussed. KEY WORDS: children; coping; leukemia.

INTRODUCTION

Leukemia is a rare disease, affecting 360 children in the United Kingdom per annum. With modern medical care survival is possible for an increasing percentage of these children. The purpose of this article is to describe the disease and its treatment and to consider the psychological repercussions for normal family life and the general development of the child. It will be shown that, despite the relatively low incidence of leukemia compared with other diseases, the questions that may be investigated concerning the psychological effects of the disease have much wider implications. Previous work is reviewed under three main headings: the psychological effects of leukemia on parents, siblings, and the patient. Wherever appropriate, This work was supported by a grant from the Medical Research Council, London. ~Department of Psychological Medicine, The Hospital for Sick Children, Great Ormond Street, London WC1N 3JH, England. 141 0160-7715/79/0600-0141$03.00/0 9 1979 Plenum Publishing Corporation

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parallels between the experiences of children with leukemia and children with other diseases are drawn. Research involving the children with leukemia is again considered under three headings. First, given the potentially fatal nature of the disease the question of how the child understands aspects of death and dying becomes pertinent. Second, we consider how children react emotionally to the fact of undergoing medical treatment for several years, and, third, we consider the effects of the disease and its treatment on normal intellectual growth. Leukemia is a form of malignancy which affects the white blood cells. Different types of leukemia have been identified, but the most common form of the disease among children is acute tymphoblastic leukemia. This type accounts for almost 85% of cases in childhood (Pierce et al., 1969). The peak incidence occurs among 2- to 4-year old children, and the disease is rapidly fatal if not treated. Acute lymphoblastic leukemia involves a greatly increased number of primitive lymphocytes. Important symptoms include lassitude, shortness of breath, spontaneous bruising, and proneness to infections. Diagnosis is made by examination of blood and bone marrow. The marrow puncture is repeated throughout treatment to determine its efficiency, relapse being detected earlier by these means than waiting for abnormal cells to appear in the blood. The cause of leukemia is not known. It can be induced by irradiation or X-rays and has occasionally occurred among several children in one area together, suggesting an infective origin. The incidence is so small, however, that other factors must operate to make certain children most susceptible. Leukemia is more common among children with Down's syndrome than might be expected, but there is little evidence of a genetic predisposition. There is only a very slightly increased risk to siblings of affected children. Treatment is aimed at suppressing production of abnormal cells, a process termed "inducing a remission." The child in complete remission should be in good general health with no evidence of leukemia on bone marrow examination. Centers specializing in the treatment of leukemia report that a first remission is possible for over 90% of children with acute lymphoblastic leukemia. Current figures suggest that 80% of children with acute lymphoblastic leukemia survive for 1 year; 30% may expect a 5-year disease-free survival (George et al., 1979). Drug treatment is continued for 2 or 3 years to try and eradicate the disease. There does not seem to be any benefit in extending drug treatment beyond this time. Apart from a short period (1 or 2 weeks) in hospital on diagnosis, long hospital stays are not usual. The child in remission can attend school and participate in all activities, although side effects of the drug treatment may sometimes limit him. In addition, absence from school is likely to occur during attacks of chickenpox or measles, as these can be associated with serious complications for such children. If relapse occurs, subsequent remissions are of shorter duration. Previously, one of the most common forms of relapse involved

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the central nervous system (CNS) and therefore all children are now routinely treated by prophylactic CNS irradiation. This has been successful in reducing the incidence of CNS relapse. There are thought to be no detrimental effects of this treatment on intellectual development, although this has not been satisfactorily investigated so far. It is often followed by a temporary somnolence period some 6 weeks later, when the children may sleep for an extra few hours for a few days or in the more extreme cases for longer periods over several weeks. Temporary alopecia invariably follows CNS irradiation.

IMPACT OF THE DISEASE ON THE FAMILY Parents

When a child is found to have leukemia, the family as a whole suffers extreme degrees of stress. During periods of remission family members are encouraged to lead as normal a life as possible. Parents are aware, however, that there is a continual threat that the child may quite unexpectedly relapse and the disease prove fatal. Such long-term stress requires that the parents adopt appropriate strategies for coping with the child. There is a need to balance, on the one hand, the possibility of death while, on the other, maintaining confidence in everyday aspects of their own and their children's lives. There has so far been no systematic study of the development and maintenance of such coping strategies throughout the course of a child's illness, although previous workers investigating this problem for families of children with chronic diseases have invariably been impressed by the degree of coping resources displayed (Burton, 1974). Our knowledge of the family dynamics when a child has leukemia is based almost exclusively on retrospective reports made by parents, often following the child's death. Despite this reservation, many aspects of the child's illness may be expected to be so traumatic that parents will have clear recollections of their emotions. This is certainly true of parents' recall of their reactions on being informed of the diagnosis. The emotions invariably reported are, quite naturally, of shock, grief, or disbelief (Friedman et al., 1963; Futterman and Hoffman, 1973; Natterson and Knudson, 1960). For many parents there follows a period of intense activity, during which time they search for as much information about the disease as possible. Parents question themselves as to whether they sought medical help quickly enough or were in any way responsible for the child's illness. Natterson and Knudson (1960) reported that assurances from medical personnel as to the unknown etiology of the disease did much to alleviate parental guilt feelings. Although some authors regard an active search for in-

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formation to be indicative of an adaptive, healthy attitude to the illness, it is not the response adopted by all parents. McCarthy (1975) reported that some parents preferred to be given the minimum of information, related only to everyday aspects "of the child's medication and care. The difference may in part be culture based, McCarthy's work being the only one based on an English rather than an American sample. During periods of remission most parents attempt to lead as normal a life as possible. They acknowledge, however, the urge to overindulge the sick child (Futterman and Hoffman, 1973). Despite parents' claims that they are able to carry on as usual, Natterson and Knudson (1960) found that they were likely to report changes in their work habits and social lives that indicated they were not entirely successful. The difficulty is always that of striking a balance between "carrying on as usual" and not appearing hard-hearted. Most authors report that as the child approaches the terminal stage of the illness, parents enter a stage of anticipatory mourning (Richmond and Waisman, 1955), a process in which the inevitability of death is acknowledged and emotional bonds begin to be relinquished. Futterman and Hoffman (1973) describe a number of clearly defined stages in parental emotions throughout the entire course of the disease. The first stage involves simply the acknowledgment of death. This is followed by a period of grieving, which fluctuates in relation to the child's state of health, and a stage of reconciling the value of the child's life in relation to his impending death. Intermittent clinging and distancing characterize a period of detachment from the child, and finally the parents develop a relatively fixed representation of the child which will endure beyond his death (memorialization). Using projective techniques, Orbach et al. (1955) found evidence of anticipatory mourning among mothers before the child showed signs of physical deterioration. Futterman and Hoffman (1973) argue that this process is essentially adaptive. They found very few instances of severe psychopathology among members of the 23 families they studied. Of a total of 45 interviews, 13 were conducted after the death of the child and a further eight while the child was in medical relapse. Stehbens and Lascari (1974) again stressed the immense coping resources of parents; 37 of 46 interviewed after the child's death were reported to be "fully recovered" within 6 months. Similar results were reported by Natterson and Knudson (1960) and Friedman et al. (1963). The only exception to these findings is that reported by Binger et al. (1969). They interviewed the parents and siblings of 20 children who had all died earlier. Some degree of psychological disturbance was reported in most. of these families. In part, such findings are dependent on definitions of coping and disturbance adopted, and the availability of psychosocial care. The work of Binger and his colleagues appear to have been undertaken in a spirit of willingness and prior acceptance of the need for such services to families in these circumstances.

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Too much of the work conducted with parents of children with leukemia has been based on interviews with parents after the child's death. Important questions relating to how attitudes and behavior change as a function of the child's health and reaction to treatment have not, therefore, been investigated. Many parents report a periodic onset of depressive symptoms associated with the child's hospital appointments; the effects of cyclical emotional upheaval of this kind on the family cannot be assessed by one-time, retrospective interview studies. Authors have generally failed to acknowledge the interaction between coping styles of different members of the family. The only exception to this criticism is the study by Murstein (1960), who found that a father's reactions were as dependent on the psychological well-being of his wife as on the physical condition of the child. Reports that parents are fully recovered or back to normal following the death o f a child from leukemia, (e.g., Stehbens and Lascari, 1974) ignore the inevitability of change following grief and bereavement (Morris, 1974). The picture of a return following a child's death to a similar way of life to that during his lifetime is clearly superficial. Interview studies of the type reported are subject to at least two limitations. First, they are often conducted by mental health professionals who have not previously been involved with the families and who cannot necessarily expect to command the degree of empathy essential to enable parents to give more than the socially acceptable responses. More important, the interview can take place at varying intervals of time from the child's death. This will not only be crucial in determining the content of the parents' reports but also will affect their reliability or validity. In the study by Stehbens and Lascari (1974), for example, parents were interviewed 6 months to 3 years after the death of their child, and no account of this time difference was made in discussing the results. Among those initially invited to take part in the study, 20% refused, raising the possibility of nonrepresentativeness of the sample. Finally, it is worth noting that only half the families interviewed found the meeting helpful, and two found it very disturbing.

Siblings

Children suffering from leukemia are likely to attract special care and attention from all adults who know of their illness and such increases in attention are probably at the expense of other children in the family. In this respect the siblings of children with leukemia are likely to react similarly to siblings of children with other chronic diseases. Despite the increasing recognition of this problem there have still been few controlled studies on the effects on the psychological development of siblings of chronically sick children. Maladjustment in behavioral development has been reported among siblings of mentally retarded

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children (Farber, 1959, 1960), children with spina bifida (Tew and Lawrence, 1973), and children with Down's syndrome (Gath, 1973). Once again, there has been no work on the reactions o f siblings o f children with leukemia who are in remission. They have been the subject o f study only from the point of view o f their reactions to the child's death. Lewis (1967), in a study of 33 Australian families, found that serious disturbance requiring medical consultation or lasting more than 1 year occurred among the siblings of 17 children who had died. A further five families reported transient problems among siblings. One or more o f the siblings in half of the 20 families studied by Binger e t al. (1969) showed difficulties adapting to the death of the child. Disorders reported by parents included poor school performance, school phobia, depression, enuresis, headaches, and severe abdominal pains. Less severe disturbances were reported by Stehbens and Lascari (1974). Transient behavior disturbances only were found, and 70% of siblings were reported to be "back to normal" within 1 week of the child's death. Given t h e essential nature o f an adequate grieving process, it would have been useful to know how these children responded at a later date, and whether adverse repercussions o f this rather abrupt return to normal followed. McCarthy (1975) could find no consistent pattern of behavior among siblings. He did note, however, that a few became more "grownup," particularly in their sense o f protectiveness and responsibility. The reaction o f a child to the death of a sibling is likely to be dependent on many factors: the determinants of children's responses to the death of a sibling we found to include: the nature of the death; the age and characteristics of the child who died; the child's degree of actual involvement in his sibling's death; the child's pre-existing relationship to the dead sibling; the immediate impact of the death upon the parents; the parents' handling of the initial reactions of the surviving child; the reactions of the community; the impact of the death upon the family structure; the availability to the child and parents of various "substitutes"; the parents' enduring reactions to the child's death; major concurrent stresses upon the child and his family, and the developmental level of the surviving child at the time of death, including not only psychosexual development, but ego development with particular emphasis upon cognitive capacity to understand death. The effects upon the child obviously are not static, undergoing constant developmental transformation and evolution. (Cain et al., 1964) It is likely that all these factors as well as o t h e r s - identification with dead child and self-esteem - will determine the child's reaction throughout the whole course o f a sibling's illness. In many ways, where a child with leukemia has a long medical remission, the family situation may be particularly complicated for siblings to handle. Not only may the parents be seen to favor the sick child, but also there is likely to be no apparent reason for their behavior. It is unfortunate that we have so little information about the child's reaction to the death o f a sibling, but an even greater pity that we have virtually no information about how siblings are affected during periods of remission.

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Patient

~ i l d ' s Awareness o f Death and Dying The potentially fatal nature of leukemia means that parents and nursing staff are especially concerned with the child's understanding of his disease and its implications. The youngest patients are usually told that they have "something wrong with their blood," and most centers advise parents to give the child of 10 years or more a reasonable account of his illness. Even so, the extent to which children are informed about the disease, or how well they understand the implications, is not generally known. There is unlikely to be a single answer to this question, but there is not yet any global information available as to the outcome for children depending on their degree of knowledge. The main concern of researchers has been on the child's understanding of the possible fatal nature of the disease. Here there are at least some guidelines from research on the healthy child's understanding of death. Early work by Nagy (1948), for example, was taken as evidence that children under 10 years were not fully able to understand the implication of death. However, more recent work has shown that children younger than this are able and willing to talk about their ideas and fantasies regarding death. Several authors have attributed this openness to increased exposure to problems of death and dying brought to the child by television (Tallmer et al., 1974). Using projective measures and interviews, Tallmer et al. found that death awareness was greater among working-class than middle-class children. Childer and Wimmer (1971) found that the idea of the universal nature of death increased steadily with age. However, death as a final irreversible process appeared harder to understand, with some 10-year-olds still finding this a difficult idea to grasp. Koocher (1975) asked 6- to 15-year-olds to answer four questions: "What makes things die?"; "How can you make dead things come back to life?"; "When will you die?"; and "What will happen then?" His findings gave several practical implications. Real explanations of death, he argues, should be simple and direct, and draw as far as possible on the child's experiences. Distortions in the child's understanding can readily be dispelled by having the child repeat back the information. Only 50% of children included in this study discussed death in terms of how others might react. Instead they focused on details of the funeral and what it might be like in heaven. The child appears to master the concept of death by focusing on specific, practical details. Fear of death certainly seems to be very important to the child with leukemia. Natterson and Knudson (1960) reported that this was especially applicable for children over 10 years, with preschoolers showing greatest anxiety about separation from their mother and 6- to 10-year-olds being most fearful about medical procedures. Although most researchers are now agreed that the

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fear of death is a major concern for the child over 10 years, regardless of whether or not he is informed of the diagnosis (Spinetta e t a L , 1973)the conclusions of Natterson and Knudson (1960) regarding the younger child have been questioned. Their information was based on parental interviews and direct observation of the child's behavior. Although children may be very frightened about medical procedures, it does not exclude the possibility that they are also aware of the seriousness of their condition. Employing projective and indirect techniques to probe all the child's fears, researchers have reported that the 6- to 10-year-old child can be aware of the fatal aspect of his disease and can verbalize these fears in some situations. Waechter (1971) compared four groups of 6- to 10-year-olds: 1. Children with a fatal illness (including leukemia, neoplastic diseases, and cystic fibrosis). 2. Children with a nonfatal, chronic illness. 3. Children with a brief illness. 4. Normal healthy children. Children with a fatal illness were much more anxious than children with a chronic disease (as measured by a General Anxiety Scale for Children). Each child was also shown eight pictures and asked to tell a story about each one. Those with a fatal illness were more likely to relate stories with overtly expressed death themes and to express more concerns about body mutilation or separation. Again using a storytelling task and an anxiety questionnaire, Spinetta et al. (1973) compared 25 children aged 6-10 years old with leukemia with 25 children suffering from chronic, nonfatal diseases. Although children with leukemia did not make overt reference to death, they did show greater awareness of threat to body integrity and functioning, and also expressed greater anxiety regarding hospital and home, than children with chronic diseases. Studying the same groups of children, Spinetta et al. (1974) investigated how these heightened levels of anxiety and fear might affect the interpersonal relationships of children with fatal diseases. Each child was given dolls representing significant others (mother, father, doctor, nurse) and asked to place the dolls wherever he liked in a three-dimensional model of a hospital room. Children with leukemia placed the dolls at greater distances from the dolls representing themselves than did those with chronic conditions. Spinetta e t al. (1974) argued that the child copes with a fatal condition by progressively distancing himself from other people in anticipation of his own death. Heightened anxiety among children with leukemia compared to those with chronic diseases also applied when the children were treated on an outpatient basis rather than in hospital. Spinetta and Maloney (1975) found that leukemia patients became more anxious about outpatient attendances, while

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children with chronic diseases became less anxious, as the illness became of longer duration. This may be taken to imply that leukemia patients become aware of the nature of their illness despite the fact that it is rarely discussed openly, in contrast to children with chronic conditions (Burton, 1974). The study of children's drawings further supports the view that children are more aware of their condition than appears from their speech (Bach, 1974). Vernick (1973) has argued that indirect approaches of this kind are employed only because of the researcher's anxiety in discussing the subject of death, rather than the inability of the child to communicate in any other way. Rainbault and Royer (1969) concluded from their study of chronically sick children that, from 5 years of age, children can readily and realistically speak about their fears related to death and dying. Certainly the practical problems involved in assessing children's awareness of their condition by the use of indirect, projective techniques are considerable. The child may be given a free situation in which to express himself, but the interpretation of this expression is likely to reflect the researcher's bias (Mischel, 1968). It is unfortunate that the question of how the child with leukemia views his condition is largely based on techniques which generally have little reliability or face validity. A greater variety of research techniques is called for. Methods based on repertory grid or semantic differential techniques would be a simple extension of the previous work, the latter having already been shown to be a useful tool in assessing the psychological effects of cancer among adult patients (Kennedy et al., 1976). Emotional AdJustment

Children with chronic diseases have generally been found to show varying degrees of emotional and social maladjustment (Diller, 1972). The main cause of this maladjustment has often been attributed to a specific characteristic of the disease, rather than the general factor of being ill. Thus Gayton and Friedman (1975) concluded that frequent hospitalizations and progressive deterioration of the child are of major importance in determining the degree of maladjustment among children with cystic fibrosis. Diabetics must cope particularly with the need for special diets, and become preoccupied with urine examinations and injections (Knowles, 1971). For children on hemodialysis the main worries are associated with rejection of the kidney (Bernstein, 1971). In a study of 46 patients with spina bifida Dorner (1976) found that they had many anxieties related to most aspects of their social lives. In contrast to these findings, Korsch et al. (1973) reported that of 35 patients followed between 1 and 5 years after kidney transplantation, most showed a satisfactory recovery. All but two of 19 older children were at university or had appropriate jobs; 11 of the 16 younger patients were in appropriate schools. The children did not show a higher incidence of personality disturbance

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than either physically well children or a group with chronic disease. Studying children with cystic fibrosis, asthma, diabetes, or reduced hearing ability, Tavormina et al. (1976) also reported that their general emotional adjustment was good. On standardized measures of personality (e.g., locus of control and selfconcept), these children scored within the normal range. Differences in the health of the children probably account for most of these discrepancies; Tavorm i n a et al. (1976) note that their sample all had "mild" levels of disability. Clearly, the tests used and criteria of abnormality adopted also account for some of the differences in previous reports. Most research indicates, however, that the effects of chronic diseases in childhood tend toward adverse emotional and social adjustment. There is some indication that this also applies to children with leukemia. Meadows and Evans (1976) assessed 23 children at least 5 years after diagnosis. Only nine were reported to show no abnormal psychiatric signs. For the remainder the degree of pathology was greater among children treated by CNS irradiation than among those who were treated by drugs alone. The high level of abnormalities found by Meadows and Evans may be partly accounted for by the fact that the children had all been undergoing treatment for at least 5 years; most patients being treated for leukemia under more recent protocols would not continue chemotherapy beyond 2 or 3 years. A higher incidence of school phobia (10%) than among normal children (1.7%; Kennedy, 1965)has also been reported for leukemic children (Lansky et al., 1975). Despite the indications from previous literature suggesting the possibility of abnormal emotional development among children with leukemia, few studies have been undertaken with this group. The length and intensity of treatment involved would lead us to predict a degree of emotional maladjustment among these children. At the same time, previous work suggests that part of the abnormalities in development observed in other groups of chronically sick children may be attributed to specifics of the illness, i.e., special diets and the need to self-administer injections for diabetics or frequent physiotherapy at home in the case of cystics. Children with leukemia are required only to take drugs on a regular basis; the relative lack of intrusion into other aspects of their daily life may counter many of the disturbing aspects of their disease. There remains an urgent need to document the emotional responses of these children, partly so that counseling and rehabilitation of the families can be more systematic and partly in comparing the responses of leukemic children with those with other chronic diseases to isolate those aspects of disease in childhood which have the most serious repercussions for normal psychological development. Although for purposes of review the emotional reactions of the child have been considered separately from those of his family, the two are intimately connected. Tropauer et al. (1970) have shown that the coping responses of the child with a chronic disease reflect the style of his parents. The general psychological development of the child with leukemia needs to be considered in

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relation not only to characteristics of his own personality but also to those of his family.

In tellectual D e v e l o p m e n t

There is little research to suggest that children with chronic diseases are of lower intellectual ability than normal children. Indeed, there may be compensatory processes at work, so that, in recognizing their physical inferiority to others, sick children may be determined to compete at an academic level (Burton, 1974). Children who were severely disabled with spina bifida did score lower on IQ tests than normal, but with less severe disabilities little effect on IQ was found (Tew, 1977). Falkman (1977)reported that a group of cystics had IQs within a normal range, with only four of a sample of 39 having scores below 85. Children with diabetes were also found to be of normal intellect (Ack et al., 1961), although there was a tendency for children acquiring the disease before 5 years of age to have lower scores than those acquiring the disease after this age. Ack et al. (1961) attributed this difference to biochemical changes taking place which were particularly damaging if they occurred in the immature, i.e., preschool, brain. Aside from the possible effects of chronic disease on intellect, there are additional reasons to suspect that the intellectual development of children with leukemia may be at risk. It is not definitely known how CNS irradiation may affect brain development and subsequent intellectual skills. One possibility is that CNS irradiation, at the given dosage, does not damage the brain itself but does reduce the effectiveness of the blood-brain barrier. Subsequent problems may then result from the passage of antileukemic drugs to the brain via the damaged blood-brain barrier (Price and Jamieson, 1975). By comparing children who had been treated by CNS irradiation with those who had not, Soni et al. (1975) reported that there were no adverse effects on intellect. They reported two studies. In the first they compared 14 children who had been treated by CNS irradiation for leukemia with 19 children who had been treated for solid tumors by irradiation to other sites of the body. In the second study they compared five children with leukemia and treated by CNS irradiation with nine children also with leukemia but who were treated only by drugs. In neither study did they find evidence of reduced intellectual skills among the children treated by CNS irradiation. However, with repeated testings over a 4-year period, Soni et al. (1975) noted that children with solid tumors improved their scores on intelligence tests, presumably as the result of practice effects and as they improved physically. No such increase in test scores was noted for the leukemic children. Soni et al. (1975) therefore questioned whether CNS irradition may limit the child's intellectual skills by reducing his capacity to learn from previous experience.

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Two studies by Eiser (Eiser and Lansdown, 1977;Eiser, 1978a) again suggest that the intellectual ability of children with leukemia may be reduced in some cases. Eiser and Lansdown (1977) studied 15 children. All were well, having completed treatment after 2 or 3 years, and remained in remission but no longer on drugs. They were divided into two groups: a younger group of nine children who were all under 5 years old on diagnosis and on testing were aged between 5.5 and 7.6 years, and an older group who were all over 5 years of age on diagnosis and on testing were aged between 8.3 and 9.5 years. The younger group was assessed using the McCarthy (1970) scales of general ability and the older group using the Wechsler (1949) scales (WISC). The scores of each child were compared with the age-related norms provided for the scales and with the scores obtained by one other healthy child, matched individually for age, sex, and social class. The scores of all the children with leukemia were within the normal range. Children who were over 5 years old on diagnosis scored as well as their matched controls on all tests used. However, those under 5 years old on diagnosis did not always score as well as their matched controls on all tests used. The McCarthy scales are composed of five subscales, described as measuring prknarily Verbal, Perceptual-Performance, Quantitative, Memory, and Motor skills. Most of the items on these scales, with the exception of some tasks of gross motor skills, can then be combined to yield a general measure of ability (General Cognitive Index, GCI). Children with leukemia scored as well as their matched controls on the Verbal scale, and in terms of reading ability. However, they obtained lower scores on the general index of ability and on the Quantitative, Memory, and Motor subscales. Despite the small sample size and any criticism that could be made of the McCarthy scales (Eiser, 1978b), this work indicates that children treated for leukemia before 5 years of age may be at risk in terms of subsequent intellectual growth and that the degree of risk may be specific to some tasks while general verbal and reading ability remains unaffected. However, the design of the study was such that it was not possible to isolate any specific cause of the reduced intellectual skills. In an attempt to compare the intellectual skills of children treated for leukemia as a function of their medical treatment, Eiser (1978a) studied 26 children. Again all were well and had completed all treatment at least 2 years earlier. The children were divided into three groups: 1. Seven who had never had CNS irradiation. 2. Ten who had prophylactic CNS irradiation at least 6 months after diagnosis. 3. Nine who had prophylactic CNS irradiation within 2monthsofdiagnosis. (This group is typical of the majority of children being treated for leukemia under the most recent protocols.) Children were assessed primarily using the Wechsler Scales of Ability (revised version, Wechsler, 1974).

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Children in the first two groups had scores well within the average range. Children in the third group scored below the expected mean (mean Full Scale IQ = 88.6). On the Verbal subtests of the WISC, they scored significantly lower than the other two groups on tests of verbal reasoning (similarities) and arithmetic. Their scores on the Performance scale IQ and all subtests of this scale were again significantly below those of the other two groups. The fact that children in the no irradiation group achieved higher scores than those undergoing prophylactic CNS irradiation on diagnosis implicates CNS irradiation as a possible cause of this difference. However, the groups necessarily differed in other aspects of their treatment and in the length of time since treatment was completed. Nevertheless, although the results are based on small numbers, it may be tentatively concluded that children treated for leukemia under current protocols appear to function intellectually at a level lower than might be expected.

CONCLUSIONS Perhaps because of the relatively low incidence of leukemia compared with other diseases, children suffering from it are a unique group. However, the type of medical treatment and length of time involved mean that their psychological development can be considered in relation to several questions of general interest: the effects of chronic disease on family life and parental coping mechanisms, the effects of chronic disease on the emotional adjustment of the child, and the effects of CNS irradiation on mental development. The study of this group of children is therefore relevant to questions of wider interest than simply documenting human coping resources to a specific and rare disease. Previous work has been somewhat limited, however, by an underlying assumption that leukemia is necessarily and inevitably a fatal disease. For this reason, research has been heavily weighted toward studying the effects of the disease on the family after the child's death or considering the child's understanding of death and dying. This bias could be excused if any practical considerations had been forthcoming in terms of advising or counseling families of children who had reached terminal stages, but it has not. Many children beginning treatment for leukemia at specialist centers can expect 2-3 years' disease-free survival. It is this group of children with such a life expectancy that have been most neglected in previous work, but who are the more important. In terms of the impact of the disease on family life, there is a need to study the dynamics of the home situation in relation to changes in medical treatment and the child's prognosis over time. This clearly calls for a longitudinal assessment of parents' perceptions and attitudes throughout the treatment: their understanding of the condition and additional external factors that influence their lives. Much has been written about the advantages and disadvantages of

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informing cancer patients of their disease and prognosis (Mclntosh, 1974), but little is known about how parents' understanding of their child's disease influences their child-rearing practices. The existence of misunderstandings about the nature of the disease, concerned with questions of heritability, infection, and so on, is known at a general level, and is likely to have serious consequences in terms of the parents' attitude toward the child and degree to which he is allowed to lead a normal life. The interaction between beliefs about the disease and child-rearing practices is, however, a neglected issue. Researchers in the past have been content simply to describe the incidence and type of parental beliefs, rather than describe how these beliefs affect the child. Along with other authors (Grave, 1974), we acknowledge the immense stresses placed on siblings of children with leukemia and the need to understand how such stresses affect normal emotional and intellectual development. Again, previous work has generally been limited to studying the child's reaction to the death of a sibling. There is little systematic evidence regarding changes in school performance and behavior throughout the long treatment. The detrimental effects of a long illness of the child's father on academic performance (Douglas et al., 1968) suggest that siblings may well deteriorate academically when a child has leukemia. Parents generally acknowledge that siblings do suffer in that they have less time to spend with other children and find it difficult not to make allowances for the sick child (Binger, 1973). It would be relatively simple to investigate behavioral changes in siblings which might reasonably stem from these differences in parental behavior, but little attempt has so far been made to do so. The main thrust of previous research concerned with the leukemia patient has been with questions involving the child's understanding of death and dying. We have argued that such a bias is no longer necessary, given the improved prognosis for many children. More important, where work of this kind continues, it is necessary that it be linked with practial considerations for dealing with these children; guidance as to how to talk to dying children has so far been limited to suggestions made by Koocher (1975), for example, working with healthy children. The biggest gaps have so far been in the areas of emotional adjustment and intellectual development of children with leukemia. There appear to be several reasons from work with other groups of chronically sick children why emotional disturbance might be predicted, and yet there has been little attempt to investigate this. Neither has there been any attempt to integrate work on the emotional responses of the family in general with those of the child. It is also essential to monitor the intellectual development of these children, in part so that appropriate remedial action may be taken if necessary and also because of the insights into process of brain development that are possible following the effects of CNS irradiation and intellectual recovery. Although we have outlined the main areas of interest in current research, it is possible to identify additional questions for study. These include describing

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the emotional problems o f medical staff who treat children for this disease. Some reports o f the distancing o f medical personnel from dying children have already been made, b u t additional work concerned with young nursing staff and the cumulative effects of working with children with cancer would be useful. Long-term follow-up o f families who do lose a child from leukemia is also a neglected issue, previous work being limited on the whole to foUow-ups after only a matter o f months. As the survival rate among children with cancers continues to improve, the questions we have outlined are likely to become o f increasing importance. Many questions will have repercussions not only for the patients themselves but also for their children. It is necessary to balance, on the one hand, the need to monitor all aspects o f the development o f children undergoing radical treatment for cancers with, on the other hand, the need to recognize the rights o f survivors to privacy and the readoption o f a normal life.

ACKNOWLEDGMENTS The author would like to thank Professor P. Graham and Dr. J. Chessells for comments on an earlier version o f this article.

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