Therapeutic adherence to oral medication regimens by adolescents with cancer. II. Clinical and psychologic correlates.

The focus of our study was to identify specific demographic, treatment, and psychologic factors that may differentiate patients who complied with cancer therapy from those who did not. In a companion study, bioassay techniques revealed frequent nonadherence to oral medication regimens among 50 adolescents and young adults with acute lymphoblastic leukemia or Hodgkin disease. In this sample, compliers and noncompliers did not differ with respect to either demographic variables (age, gender) or treatment variables (treatment duration, continuity, complexity, or parental involvement). Psychologic variables were assessed in 34 patients. Patients who adhered poorly to the therapeutic regimen had significantly less developed concepts of the illness (specifically, concepts of causality and prognosis), less perceived vulnerability (especially as related to subclinical disease), higher levels of denial as a psychologic defense, and less cohesive future orientation. The perceived health locus of control was not a significant factor. The results suggest that patients construct their own subjective view of the illness and its treatment, which then has implications for adherence to medication regimens.

Therapeutic adherence to oral medication regimens by adolescents with cancer. I. Laboratory assessment.

Outpatient adherence to oral medication regimens was evaluated in 50 adolescents and young adults with cancer: 21 patients with acute lymphoblastic leukemia or Hodgkin disease who were to take prednisone as a component of their chemotherapy regimen, and 29 patients with Hodgkin disease whose chemotherapy had been discontinued and who were to take penicillin for postsplenectomy prophylaxis. Of the 21 adolescent patients with acute lymphoblastic leukemia or Hodgkin disease who were to take prednisone as outpatients, 11 patients were found to be nonadherent to treatment (52%). Among the 29 adolescents for whom penicillin was prescribed for postsplenectomy prophylaxis, nonadherence was detected in 14 patients (48%). If a method is available, laboratory assessment to determine adherence should be performed in adolescents and young adults receiving long-term therapy, particularly if one wishes to draw valid conclusions regarding efficacy of various treatment protocols.

Children reared in a reverse isolation environment: effects on cognitive and emotional development.

Cognitive and emotional aspects of development in four infants reared in a reverse isolation environment because of congenital severe combined immunodeficiency disease were studied by psychological test performance and formal observation. The children were studied while they were inpatients and following their discharge after successful medical treatment was accomplished. Treatment time in reverse isolation varied from 10 to 52 months. Deficient self-generated activity, including motor and motor-based cognitive skills such as expressive language, were observed in two of the four children. Deficits were also observed to be at least moderately reversible either upon discharge or in relation to an inpatient intervention program. Case material is discussed with reference to severe disruption of oral feeding experience, quality of parental involvement, and sensory isolation inherent in the environment.

Language recovery following isolation for severe combined immunodeficiency disease.

Children with congenital severe combined immunodeficiency disease (SCID) lack an immune system and require continuous reverse isolation to protect them from exogenously transmitted infection. In our setting, protected environments consist of laminar flow rooms in which everything is sterilized (food, clothing, toys and so on). Staff and family wear complete surgical garb, preventing skin contact, observation of mouth movement and olfaction of typical human odours. Case studies of language development in children reared from infancy in protected environments have cited instances of mental retardation or language delay with some improvements after intervention, although the degree of language exposure is not well documented . We report here the case of a male patient, successfully treated for SCID, who lived in reverse isolation from 9 months to 4 yr 4 months of age and who has demonstrated nearly complete recovery of language usage. Our study illustrates the resilience of human intellectual function following a condition of early severe deprivation.

Immediate and long-term posttherapy neuropsychologic performance in children with acute lymphoblastic leukemia treated without central nervous system radiation.

Intellectual and neuropsychologic test performance was studied in 41 children with acute lymphoblastic leukemia within one year of completing multidrug therapy in which intrathecal methotrexate was the sole agent of central nervous system prophylaxis. To control for possible effects of intensive systemic chemotherapy and the disruptive psychosocial effects of treatment on psychometric test function, 33 children with embryonal rhabdomyosarcoma who had no CNS disease or treatment were similarly studied. All patients studied had been in continuous complete remission since diagnosis. Within each diagnostic group, patients were placed in a younger (less than 8 years) or an older (more than 8 years) group depending on age at the time of testing. No significant difference was found in intellectual function between the young ALL and ERMS groups or between the total ALL and ERMS groups. Further five-year follow-up of 12 of the youngest ALL patients showed no significant long-term change in intellectual or other specific neuropsychologic functions. Children with ALL who remained in continuous complete remission from diagnosis and who were treated with prophylactic intrathecal methotrexate and no CNS radiation did not have global or specific neuropsychologic impairment.

Comparison of morbidity in children requiring abdominal radiation and chemotherapy, with and without total parenteral nutrition.

We evaluated the effectiveness of total parenteral nutrition and placing the "bowel at rest," as compared to that of ad libitum food intake, on nutritional status and tolerance to combined chemotherapy and radiotherapy in a randomized, prospective trial in children with previously untreated malignancy requiring abdominal and pelvic irradiation and chemotherapy. Administration of TPN was found to be safe and efficacious in maintaining the children in good nutritional status during combined therapy; one-third of the control patients became malnourished and required TPN. There was no beneficial effect of "bowel at rest" and TPN on the ability of patients to tolerate combined therapies in terms of decreased toxicity; however, use of TPN was associated with improved adherence to chemotherapy schedules. Following termination of TPN or ad libitum food intake, and while receiving chemotherapy, the majority of the children who had previously received TPN lost significant weight. To date there has been no difference in mortality rate between the control and TPN groups. Although we conclude that TPN per se had little beneficial effect beyond that of maintaining good nutritional status, every child undergoing intensive combined therapy should have early and periodic assessments of nutritional status, so that the early signs of malnutrition can be detected, and the adverse effects of malnutrition can be prevented by nutritional replenishment, by TPN, or by other methods.