Childhood disintegrative disorder

TOPIC. The identification and management of schizophrenia in childhood.

PURPOSE. To provide an overview of what is currently known about childhood schizophrenia.

SOURCES. Published literature and personal observations and experiences.

CONCLUSIONS. Early identification and treatment of childhood schizophrenia are critical, and more research and education on the part of all mental health professionals are needed in order to identify, provide treatment, and/or make referrals for children with this serious mental disorder.

Search terms: Childhood schizophrenia, developmental delays, neurobiological changes, psychoeducation, psychosis

Most nurse generalists are familiar with the diagnosis of schizophrenia in adulthood, through exposure during basic nursing education or through their work experiences. Nurses who work with children and adolescents in a mental health setting may have been involved with the assessment and treatment of a child diagnosed with schizophrenia. The clinical nurse specialist in child and adolescent psychiatric mental health nursing will likely have identified and treated a client with childhood schizophrenia in an inpatient or outpatient setting. The purpose of this article is to provide an overview of what is known about schizophrenia in childhood.

Incidence Prognosis

Schizophrenia is rarely seen in childhood (Remschmidt, Schultz, Martin, Warnke, & Trott, 1994), especially before the age of 12. It is less than one sixteenth as common as the adult-onset type (Harvard, 1997). According to Tolbert (1996), 1 in 10,000 children will develop the disorder. It occurs most often in late adolescence but can strike young children. When this is the case, about 50% of these children will experience serious neuropsychiatric symptoms (Taylor, 1998). Asarnow, Thompson, and Goldstein (1994) found that 61% of children with early onset childhood schizophrenia maintained the same diagnosis throughout adolescence and young adulthood. The Diagnostic and Statistical Manual of Mental Disorders (American Psychiatric Association [APA], 1994) (DSM-IV) points out the poorer prognosis for early onset schizophrenia. The earlier age of onset has been correlated with high social disability (Eggers & Bunk, 1997). Eggers and Bunk found that no gender differences exist between sexes in average age of onset.

Signs and Symptoms

Young schizophrenic children can and do experience psychosis, which often is preceded by behavioral problems, developmental lags, and "soft" neurological signs. There may be language and motor delays well before the development of actual psychosis. About 30% of these children will demonstrate symptoms of pervasive developmental disorder such as posturing, rocking, and arm flapping, and may present as anxious, confused, or disruptive (Harvard, 1997). Children with schizophrenia fail to develop normal interpersonal relationships and problem-solving skills. They do not develop judgment, abstract reasoning, or age-appropriate self-care. There is an alteration in cognitive development, even though there is no intellectual impairment, causing the child to be unable to actualize knowledge (Taylor, 1998).

Studies of childhood schizophrenics prior to the onset of their illness often show a different physical appearance and a negative affect compared to siblings and peers (Litter & Walker, 1993). Social and cognitive development either regresses or does not occur and generally is not regained.

Although children with schizophrenia have hallucinations, diagnosis before preadolescence is sometimes difficult due to the, child's inability to provide details. One study done by the National Institute of Mental Health (NIMH) (Spencer & Campbell, 1994), however, reported that children between the ages of 5.5 and 11.75 had shared their auditory hallucinations in very specific terms. The hallucinations are usually auditory and persecutory or command in nature. Visual hallucinations also may be present (Werry, 1992). Delusions tend to reflect the day-today life of the child and exhibit themes of monsters, ghosts, or animals (Russell, 1994). Formal thought disorder and disruption of speech is seen more often in older children but is difficult to assess and identify in the younger, immature child (Caplan, 1994). While the actual course of a schizophrenic illness for a given child cannot be predicted, the development of the psychosis is usually gradual, without any abrupt onset or sudden break as is common in an adolescent or an adult (Russell). This slow insidious onset of symptoms, known as the "prodromal phase" (APA, 1994), appears early in the child's development and increases in intensity over time.

Etiology

Research has replicated that schizophrenia is related to neurological damage. The brain structure in childhood schizophrenia (Alaghband-Rad, Hamburger, Giedd, Frazier, & Rapoport, 1997) and molecular functioning of those who have schizophrenia is very different from normals in control groups (Andreasan, 1994; Austrian, 1995; Torrey, Bowler, Taylor, & Gottesman, 1994). Longitudinal studies show evidence of progressive ventricular enlargement in people with early onset schizophrenia (Jacobsen & Rapoport, 1998). According to Gordon et al. (1994), children with schizophrenia also have measurable differences in glucose metabolism. In addition, both children and adults with schizophrenia show irregular autonon-tic nervous system arousal and problems with visual tracking of moving objects. While it is not known what causes these neurobiological changes, there is growing evidence that the illness could be passed genetically or through a neurovirus occurring in the second trimester of pregnancy. Twin studies also suggest a genetic childhood schizophrenia link (Torrey et al., 1995).

It is known that adopted children who have a biological parent with schizophrenia, and who are in an adopfive home with no pathology in the adoptive parents, maintain the same risk for development of schizophrenia. This suggests that environment is not the cause of the disorder (Hales & Hales, 1995).

In addition to genetic, neurochemical, and neuroanatomical theories of etiology, a number of psychosocial theories exist. According to Johnson (1997), the intrapsychic theory of the etiology of schizophrenia is based on the premise that the personality of the schizophrenic individual is predisposed to break down under high levels of stress. This concept is based on the work of Arieti (1980) and supports the idea that pathological symptoms develop due to characteristics of anxiety, hypersensitivity, and social detachment. The child who becomes schizophrenic has been sensitized to the negative characteristics of a parent and incorporates these feelings into his or her own distorted self-image (Arieti, 1976).

A vulnerability model postulates that under the stress of biological and psychosocial factors, the person will succumb to schizophrenia (Wasylenki, 1992). The stressors include the neurological dysfunction of schizophrenia and the resulting increase in dopamine activity in the mesolimbic dopamine system, and the psychobiological, environmental, and interpersonal stressors. In the presence of moderators, such as perceived support, antipsychotic medications, and symptom management skills, the client can be protected from the effects of stressors (O'Conner, 1994).

In the 1960s and 1970s, family communication patterns were implicated in the etiology of schizophrenia. Communication of family members was seen as indirect, unclear, incongruent, and interfering with normal growth (Satir, 1972). The theory of "double-bind communication" was believed to be at the core of schizophrenia, and children were thought to be victims of conflicting messages (Bateson, Jackson, Haley, & Weakland, 1968). These families were seen as "fused" or "undifferentiated ego masses" (Bowen, 1971). The member with the highest degree of "fusion" was seen as the one most vulnerable to schizophrenia. In light of recent knowledge focusing on biological theories, these family communication theories have been largely discounted (Johnson, 1997).

Differential Diagnosis

Children can be diagnosed with childhood schizophrenia using the unmodified DSM-III, DSM-III-R, and DSM-IV criteria (Jacobsen & Rapoport, 1998). There are now fairly clear distinctions between childhood schizophrenia and autistic and pervasive developmental disorders (PPD). Essentially, in autistic disorder onset is before age 3 and, in contrast to childhood schizophrenia, mental retardation is common (Tolbert, 1996).

The pervasive developmental disorders each have criteria that differ from childhood schizophrenia and are not usually accompanied by psychosis. While this is the subject of considerable research, it has been found that children with PPD have no period of normal development. Childhood schizophrenics show at least a subtle regression from a previous level of functioning.

Twenty-five percent of all autistic children have seizures, unlike children with schizophrenia (Young, Newcom, & Leven, 1989). In any case, conditions such as Rett's disorder, Asperger's disorder, and childhood disintegrative disorder must be ruled out (Tolbert, 1996). The clinician must exclude organic conditions, such as metabolic disorders, delirium, epilepsy, and neurodegenerative disorders (Clark & Lewis, 1998). Obsessive compulsive disorders and psychotic mood disorders also must be excluded (Gordon et al., 1994; Kafantasis, 1996; McClellan, Werry, & Ham, 1993; Rapoport & Ismond, 1996; Werry, McClellan, Andrews, & Ham, 1994). Since both the diagnosis and treatment of childhood schizophrenia carry serious long-term risks, a thorough assessment is essential before a treatment plan is established and implemented (McClellan & Werry, 1994; Werry & Taylor, 1994). Whenever possible there should be a period of observation while the child remains drug free. This may be not be possible due to the child's mental distress or behavior, but it is the best approach. The differential diagnosis of childhood schizophrenia encompasses most of general psychiatry and medicine (Clark & Lewis).

Table 1 identifies the critical areas of assessment that should be done to determine the diagnosis of childhood schizophrenia. It should be noted that the child mental exam is very salient and should be done in a calm, unhurried manner over several sessions if needed. Since the DSM-IV symptoms required for diagnosis of schizophrenia can be present in children as young as 4 or 5, the assessment must take into account developmental issues.

Developmental Issues Related to Assessment

Very young children have no clear boundaries and may not be able to verbally describe their experiences. The clinician may have to use play therapy as the assessment technique. It should be kept in mind that young children are concrete in thought with limited social experiences (Piaget, 1962). They often use private speech or talk to themselves aloud. According to Piaget, the immature child does not differentiate between words or symbols and what words represent. Fantasy and imaginative play emerge by the second year and continue for 3 to 4 years until the child becomes interested in peer games (Papalia & Olds, 1992). Animism (the attribution of life to objects that are not alive) is common due to normal egocentrism. Children may even get confused about things that move such as the wind or trees. Even as the child gets older and toward concrete operations (ages 5-7), the issue of creativity may come into play. Guilford (1959) cites the presence of both convergent thought or single right answers (traditional) and divergent thought (unusual). Both are normal, along with various fears that occur in childhood. The clinician must carefully assess the child, keeping in mind these normal developmental processes.

Treatment and Management

It is now clear that the longer psychosis continues untreated the worse the eventual long-term prognosis becomes, so early intervention is important (Birchwood, McGorry & Jackson, 1997; Wyatt, 1995). The treatment in the case of childhood schizophrenia needs to be multimodal and possibly include pharmacotherapeutics, family interventions, cognitive therapy, and environmental interventions (McClellan & Werry, 1994; Parry-Jones, 1991; Remschmidt, 1993; Tolbert, 1996). According to Jaffa (1995), children may be managed in both inpatient and outpatient settings.

An inpatient unit should be a child environment, staffed with a full multidisciplinary team, including educational professionals. Inpatient care is most likely to be needed during the first acute episode in order to do a complete diagnostic assessment and start acute treatments. Families also can be worked with and readily engaged with psychoeducational approaches to be continued after discharge. The goal is to maintain the child in the community with outpatient follow-up care (Werry & Taylor, 1994). Some children have such a slow insidious onset with no acute behavioral problems that they can be managed on an outpatient basis, if there is agreement by all parties to the necessary assessments and interventions. This prevents disruption of family relationships and social contacts (Tolbert, 1996).

Standard antipsychotic drugs appear to be effective for schizophrenic children, and the atypical drug dozapine is helpful for at least 50% of children who do not respond to the typical drugs (Jacobsen & Rapoport, 1998) such as haloperidol, which is a popular choice for acuteonset episodes. Promising newer neuroleptic drugs such as risperidone provide high potency and a rapid onset of action. When using neuroleptic therapy, many clinicians find it helpful to get a baseline score on the Abnormal Involuntary Movement Scale (AIMS) (Psychopharmacology Research Branch, NIMH, 1976) before using an antipsychotic drug on a child, given the high risk of extrapyramidal symptoms (Tolbert, 1996).

Psychotherapy is also an important part of the treatment and management of childhood schizophrenia. All members of the mental health team must educate youngsters and families about the illness. Parents need special help learning to deal with their desires for a "normal" child. Children with schizophrenia need to develop coping skills, and school personnel need to help maximize the benefits of educational mainstreaming.

Nursing Implications

The role of the nurse in the treatment of childhood schizophrenia depends on both the environmental setting and the preparation of the practitioner. It is important to note the paucity of literature on specific psychiatr)ic nursing interventions for schizophrenic children. When inpatient care is indicated, the child is usually treated in a structured child/adolescent therapeutic milieu in an acute, residential, or day-treatment program (Colson, 1998). The three main goals, according to Critchley (1991), are to provide physical and psychological security, to promote normal growth and development, and to ameliorate the psychiatric disorder. The nurse works toward the achievement of these goals as directed by the Standards of Child and Adolescent Mental Health Nursing Practice (ANA, 1985) and the combined Adult and Child and Adolescent PsychiatricMental Nursing Standards (ANA, 1994) in cooperation with the treatment team. Most programs include the use of pharmacological interventions and behavior modification strategies incorporating activities of daily living, peer, staff and family interactions, as well as school and recreational activities.

Since the hospitalized schizophrenic child or adolescent client is often psychotic, the nurse intervenes in the personal system of the child to help him/her process information from the environment. When the child's reality is distorted due to misperceptions of the environment, the nurse must continually clarify these perceptions and correct them (King, 1981). Thought-disordered youngsters may require constant reminders of time and space boundaries, such as mealtimes and locations. Visual charts and schedules may help them in this process. In addition, peers may need to be directed to ignore behaviors on the part of the child that are the result of disordered thinking (Pearson, 1992).

According to Violand and Williams (1994), negative reactions occur among family members when a child is in crisis and has to be hospitalized. Parents feel out of control and need help to dispel feelings of helplessness. Parents must be taught they are an integral part of treatment. Regardless of the level of care, nurses play a key role in the support and psychoeducation of family members of the schizophrenic child. Parents need assistance with parenting skills and management of behavior, which nurses can model. They need education not only on their child's illness, but also on the neuroleptic medications and how to administer them, how to monitor for side effects, and when and whom to call when problems arise. Families can be referred by nurses to community support groups such as the National Alliance for the Mentally Ill (NAMI), which has chapters in most communities throughout the United States. With the movement of much of psychiatric care toward the community setting, nurses are becoming an important part of the delivery of mental health services to children in the home.

Advanced practice nurses and clinical specialists are setting up school-based primary prevention and treatment programs for children and adolescents (Lamb & Puskar, 1991). Nontraditional settings provide opportunities for nurses to work with children in homeless shelters and battered women's shelters (Gilbert, 1988). Clinical specialists may work in community mental health clinics as therapists or advanced practitioners. These settings provide the nurse with a front-line opportunity to do primary prevention, case finding, referrals, and treatment of children with schizophrenia and their families.

Psychiatric nurses can intervene effectively in social systems by assessing the attitudes held by those within the system about thought-disordered schizophrenic children and adolescents. When misconceptions are identified, the nurse has the important task of educating the individuals involved. Securing needed services for a child may simply require an explanation by the nurse that the child is not dangerous. If the child is in a special education program, teachers will be prepared to meet the child's needs, but the mainstream educator may need assistance and education (Pearson, 1992).

Problems With Management and Treatment

There are numerous problems related to the management and treatment of childhood schizophrenia. The young are more prone to acute dystonic reactions, which generally occur within 72 hours after the start of the drug or with of an increase of dosage. This can be a very frightening and painful experience for the child, the family, and caretakers, even with the availability of medications to reduce or counteract such symptoms. In addition, errors are sometimes made when drugs are used to treat children with schizophrenia. This can be in the form of overdose, failure to appreciate the slow time frame of antipsychotic action leading to premature cessation of the medication, or switching to another drug. Polypharmacy adds to the problem, as does failure to systematically monitor for side effects resulting in resistance from clients and noncompliance. The other unfortunate practice on the part of some clinicians is total reliance on medication instead of a multidisciplinary, multimodal approach (McClellan & Werry, 1991). It should be noted that a 4- to 6-week trial may be required to establish the effectiveness of drug therapy This is becoming more and more difficult in a hospital setting under managed care scrutiny (Tolbert, 1996).

Werry (1996) cites a number of problem areas related to childhood schizophrenia. One is a lack of interest among child and adolescent psychiatrists in the illness. Another is that young people often are dealt with by adult facilities-a denial of their basic rights, since few adult units have schools or know how to deal with or understand the developmental needs involved. In addition, there is still much confusion about childhood schizophrenia, resulting in misdiagnosis and inappropriate treatment.

To some clinicians, another problem area revolves around the reliance on the DSM-IV (APA, 1994) for the diagnosis of childhood schizophrenia. It requires that the signs and symptoms be present for at least 6 months prior to the diagnosis of childhood schizophrenia. This means that only the most persistent cases will be diagnosed (Volkner, 1996), and they have the poorer prognosis (Werry, 1992). The 1-month criterion of the International Classification of Diseases (ICD-10) (World Health Organization, 1992) is much less restrictive and may be the most useful clinically (Mason, Harrison, Croudace, Glazebrook, & Medley, 1997).

The actual identification and treatment of children's mental health problems by primary care providers are a problem. Overall, primary care providers appear to underidentify mental health problems among children and adolescents, especially if the impairments are not severe (Richardson, Keller, Selby-Harrington, & Parish, 1996). This unfortunate state of affairs serves to increase costs even more, since problems not recognized early tend to be less amenable to short-term interventions.

To add to all these difficulties, budget cuts in response to managed care directives have caused the modification of inpatient psychiatric care for children and adolescents to brief treatment. Staffing also has been reduced. These changes mean that psychiatric nurses and other members of the mental health team must work with more acutely ill children in a much shorter period of time, making it difficult to establish therapeutic alliances with children and their families and to make lasting behavioral changes (Delaney, 1992).

Conclusions

The early, accurate identification and treatment of childhood schizophrenia are important goals for the mental health community and the community at large. More research is needed related to this serious neuropsychiatric disorder of childhood, its etiology, and how it might be prevented. In addition, nurses and all mental health professionals need to become more aware of this serious childhood mental illness in order to identify, treat, and/or make the necessary referrals.

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Louise T Lambert, EdD, RN, CS, LPC, is Assistant Professor, School of Nursing, University of Louisiana at Monroe, Monroe, LA.

Author contact: nulambert@ulm.edu, with a copy to the Editor: Poster@uta.edu

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