Kathryn E. Gustafson

Adaptation to chronic childhood illness.

Introduces a multidimensional framework for examining the development and adaptation of children with chronic illness, encompassing biomedical, psychological, and socio-ecological factors. Illnesses discussed are asthma, cancer, cystic fibrosis, diabetes, sickle cell disease and spina bifida.

Early Developmental Outcome in Children With Hypoplastic Left Heart Syndrome and Related Anomalies The Single Ventricle Reconstruction Trial

Background— Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary bypass. Methods and Results— In the Single Ventricle Reconstruction trial of the Norwood procedure with modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery shunt, 14-month neurodevelopmental outcome was assessed by use of the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. We used multivariable regression to identify risk factors for adverse outcome. Among 373 transplant-free survivors, 321 (86%) returned at age 14.3±1.1 (mean±SD) months. Mean PDI (74±19) and MDI (89±18) scores were lower than normative means (each P<0.001). Neither PDI nor MDI score was associated with type of Norwood shunt. Independent predictors of lower PDI score (R2=26%) were clinical center (P=0.003), birth weight <2.5 kg (P=0.023), longer Norwood hospitalization (P<0.001), and more complications between Norwood procedure discharge and age 12 months (P<0.001). Independent risk factors for lower MDI score (R2=34%) included center (P<0.001), birth weight <2.5 kg (P=0.04), genetic syndrome/anomalies (P=0.04), lower maternal education (P=0.04), longer mechanical ventilation after the Norwood procedure (P<0.001), and more complications after Norwood discharge to age 12 months (P<0.001). We found no significant relationship of PDI or MDI score to perfusion type, other aspects of vital organ support (eg, hematocrit, pH strategy), or cardiac anatomy. Conclusions— Neurodevelopmental impairment in Norwood survivors is more highly associated with innate patient factors and overall morbidity in the first year than with intraoperative management strategies. Improved outcomes are likely to require interventions that occur outside the operating room. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.

Nursery neurobiologic risk score: levels of risk and relationships with nonmedical factors.

ABSTRACT. This study compares the Neurobiologic Risk Score (NBRS) with developmental outcome in 199 infants ≤1500 g birth weight to determine levels of risk and to investigate the relative contributions of the NBRS and nonmedical factors to developmental outcome. The NBRS correlated significantly (p < .0001) with the Bayley Mental (MDI) and Psychomotor (PDI) Indexes, and neurologic examination score (NS) at 6, 15, and 24 months. Three risk groups were identified: low, NBRS ≤4; intermediate, NBRS 5 to 7; and high, NBRS ≥8 with an incidence of major handicaps at 24 months of 7%, 32%, and 50%, respectively. Of eight factors considered, the NBRS accounted for the greatest variance: MDI, 14 to 27%; PDI, 25 to 29%; NS, 34 to 42%. Additional increments of variance were contributed by gender (MDI, PDI, NS), maternal intelligence and race (MDI), and maternal education (PDI). The NBRS is a useful tool for identifying risk for developmental abnormalities due to neonatal medical events. J Dev Behav Pediatr 14:375–380, 1993. Index terms: very low birth weight infants, developmental outcome, developmental follow-up, biologic risk, prematurity.

Methylphenidate in Neuropsychological Sequelae of Radiotherapy and Chemotherapy of Childhood Brain Tumors and Leukemia

or chemotherapy-induced encephalopathy. We report 12 children with brain tumor or acute lymphocytic leukemia who had an adequate trial of methylphenidate. Ten had sustained benefit, with improvement in attention, academic achievement, language skills, memory, and behavior. Children with previous radiotherapy to brain, with or without additional chemotherapy, were considered for a trial of methylphenidate therapy if they had experienced a decline in academic performance, attentional difficulties, behavioral lability, or other decreases in mental ability. They represent a complex series of patients, with diverse intracranial tumors (which by themselves may affect behavior and mentation) and complex treatments, including surgery, high-dose limited-field or whole-brain radiotherapy, and often, chemotherapy with multiple agents. Four were considered hyperactive and/or learning disabled before the diagnosis of a neoplasm; whether this was an early manifestation of the tumor is unknown. Results were assessed from the reports of parents and teachers and from direct observation, after at least 6

Developmental outcome of very low birth weight infants as a function of biological risk and psychosocial risk.

The relative contribution of biological and psychosocial risk factors to developmental outcome of 102 very low birth weight infants (<1500 g) was delineated through 24 months corrected age. Biological risk, assessed by the Neurobiologic Risk Score (NBRS), accounted for significant amounts of variance in Bayley Mental Developmental Index (MDI) and Psychomotor Developmental Index (PDI) at the 6-, 15-, and 24-month assessment points. Psychosocial risk, reflected in maternal appraisals of daily stress, accounted for a significant increment in cognitive outcome (MDI), over and above that accounted for by the NBRS, at each assessment point. Cognitive functioning at each assessment point differed as a function of biological risk and psychosocial risk status. The findings are discussed in terms of maternal stress as a marker of, and salient intervention target for, caregiving environments that can maximize or minimize the effects of biological vulnerability. J Dev Behav Pediatr 15:232–238, 1994. Index terms: very low birth weight infants, developmental outcome, biological risk, psychosocial risk.

Developmental Outcome of Very Low Birth Weight Infants at Four Years of Age as a Function of Biological Risk and Psychosocial Risk

The continuing contribution of early biological and psychosocial risk factors to developmental outcome of 55 very low birth weight infants (≤ 1500 g) was assessed at 4 years of age. Biological risk, assessed by the Neurobiologic Risk Score, accounted for significant portions of the variance in the perceptual-performance (17%) and motor (35%) dimensions of the McCarthy Scales of Childrens Abilities. Psychosocial risk, reflected in maternal appraisals of daily stress during the newborn period, did not account for a significant portion of variance in any of the McCarthy Scales. Maternal education level, however, another measure of psychosocial risk, accounted for significant portions of variance (from 6% to 34%) on each of the McCarthy Scales. Movement from low neurobiologic risk status to poor outcome status at 4 years of age was associated with a number of psychosocial variables, including maternal education and early levels of maternal daily stress. The findings are discussed in terms of early markers for very low birth weight infants who require careful follow-up and of potential intervention targets to promote developmental outcome.

Psychological adjustment of children with sickle cell disease: Stability and change over a 10-month period.

Rates of poor psychological adjustment of children with sickle cell disease remained relatively constant over initial and follow-up assessment points. However, there was relatively little stability in the classification of the adjustment of individuals, low congruence in specific behavior problem patterns and diagnoses in accordance with the Diagnostic and Statistical Manual of Mental Disorders (3rd ed.; American Psychiatric Association, 1980), and less stability in child adjustment by child report than by mother report. With initial levels of adjustment controlled, childrens strategies for coping with pain accounted for a significant increment in child-reported symptoms (19%) and mother-reported internalizing behavior problems (8%) at follow-up beyond the contribution of illness and demographic parameters and follow-up interval. The findings suggest that childrens coping strategies are a salient intervention target for enhancing adjustment.

Illness specific patterns of psychological adjustment and cognitive adaptational processes in children with cystic fibrosis and sickle cell disease.

Illness-specific patterns of adjustment and cognitive adaptational process were identified in children (7-12 years of age) with cystic fibrosis (CF, n = 40) or sickle cell disease (SCD, n = 40). Anxiety diagnoses were most frequent for both illness subgroups but children with CF had a higher rate of oppositional disorder (27.5%) than did children with SCD (2.5%). Significant portions of the variance in adjustment were accounted for by stress appraisal (19%), expectations of efficacy (9%) and health locus of control (9%) for children with CF and by stress appraisal (21%) and self-worth (12%) for children with SCD. The interaction of general and specific illness tasks and adaptational process with developmental tasks in delineating intervention opportunities is discussed.

Psychological problems associated with drug therapy in childhood asthma

Ten studies designed to evaluate the psychological and behavioral effects of the antiasthma agent theophylline in children have been carried out to date. In this review, we evaluate the strengths and weaknesses of those investigations and discuss whether theophylline is responsible for learning disabilities or behavioral disorders such as hyperactivity. We also discuss the similarities between studies of theophylline and those of other xanthines, such as caffeine, to suggest that some persons appear to have a heightened response to these substances and would benefit from closer monitoring, not only of serum drug levels but also of their consumption of food and beverages that contain caffeine. In addition, we describe strategies for examining whether a functional relationship exists between theophylline use and behavioral and psychological changes in children.

Change in the Psychological Adjustment of Children with Cystic Fibrosis or Sickle Cell Disease and Their Mothers

Objectives: This study has three interrelated objectives: (1) to track the adjustment of children and adolescents with sickle cell disease (SCD) or cystic fibrosis (CF) and their mothers through a third assessment point 2 years after the initial assessment; (2) to determine whether the adaptational processes of the transactional stress and coping model associated with adjustment at the initial assessment continue to be associated with adjustment 2 years later; and (3) to determine whether the pattern of association of adaptational processes with adjustments differs by illness subgroup. Methods: The study samples included 59 children with CF and 50 children with SCD and their mothers. Measures were obtained on maternal adjustment and appraisals of daily and illness stress, coping methods, and family functioning. Child measures included child-reported and mother-reported child adjustment and child perceptions of self-worth and health locus of control and pain coping methods. Results: Consistency in adjustment classification was only 31–32% for child self-report, 66% for mother-reported child behavior problems, and 56–77% for mother self-reported adjustment for the CF and SCD groups, respectively. Support was provided for the association of adaptational processes with maternal adjustment and with the adjustment of children with SCD but not for children with CF. Conclusion: The stability of adjustment has implications for prevention and treatment intervention and subsequent research steps. Intervention efforts should be focused on the relatively small subgroups of children with chronic illnesses and their mothers with consistently poor adjustment.