A brief report of caregiver needs and resource utilization during pediatric hematopoietic stem cell transplantation.

Hematopoietic stem cell transplantation (HSCT) is used to eradicate disease and restore normal hematopoietic, immunologic, and/or metabolic functioning. HSCT is a complex treatment that is physiologically and psychologically demanding on the recipient, caregiver, and family. The purpose of this study was to identify needs and resources of family caregivers of pediatric HSCT recipients during the first year after transplant. Parental caregivers (n = 161) completed an online survey. The most cited sources of information were the HSCT team (87.7%), books and other print materials (83.1%), and the Internet (81.5%). However, more than half of the respondents reported that finding resources and services was a problem. More than half identified managing the emotional and social impact of the transplant on their child, posttransplant and follow-up care, practical strategies for caregiving, maintaining the family, and taking care of themselves during this first year as important topics to address. Adequately and regularly assessing caregiver and family needs and providing resources to meet those needs, especially during transitions in care, are important components of transplant care.

Neurodevelopmental outcomes of congenital diaphragmatic hernia survivors followed in a multidisciplinary clinic at ages 1 and 3.

PURPOSE: Infants who survive congenital diaphragmatic hernia (CDH) repair may have ongoing medical and neurodevelopmental morbidity after hospital discharge. We evaluated the relationship between medical and neurodevelopmental outcomes of CDH survivors seen in a multidisciplinary clinic at ages 1 and/or 3. METHODS: From January 1997 to December 2004, 69 (61%) of 112 CDH survivors were followed in our CDH clinic at ages 1 and/or 3. Medical issues (cardiac, pulmonary, gastrointestinal) were tabulated at hospital discharge and at follow-up. Neurodevelopmental data were obtained from clinic assessments by a neurodevelopmental pediatrician. Descriptive results were summarized for each cohort. Multivariate analyses were performed to identify predictors of motor problems at age 1. RESULTS: Of the 69 study participants, 64% were male, 75% had left-sided CDH, 17% had cardiac anomalies, and 25% had other congenital malformations. Nearly all required ventilator management (99%) with a median ventilator time of 14 days (range, 1-54 days); 30% required extracorporeal membrane oxygenation. While 87% of patients had medical issues at hospital discharge, 61% and 67% had medical issues at ages 1 and 3, respectively. Pulmonary problems were noted in 34% and 33% of the ages 1 and 3 cohorts, respectively. Motor and language problems were detected in 60% and 18% of the age 1 cohort and 73% and 60% of the age 3 cohort, respectively. Multivariate analysis found ventilator time as the only independent predictor of motor problems at age 1 (odds ratio, 1.12 per day; 95% confidence interval, 1.05-1.20; P < .01). CONCLUSIONS: Young CDH survivors continue to have ongoing medical problems and a high incidence of motor and language problems. Duration of neonatal ventilatory support was a significant predictor of motor problems at age 1. Prospective studies are needed to confirm these findings.

Impact on family of survivors of congenital diaphragmatic hernia repair: a pilot study.

BACKGROUND/PURPOSE: We hypothesized that long-term impact on the family of survivors of congenital diaphragmatic hernia (CDH) would vary based on the child's current clinical problems and clinical severity, family income, insurance, and out-of-pocket expenses. METHODS: Parents of a retrospective cohort of 53 CDH survivors who underwent repair at Children's Hospital Boston in Massachusetts from 1991 through 1999 completed the Impact on Family Scale (IFS), the Child Health Ratings Inventories General Health Module Parent Report, a rating of their child's clinical severity, an inventory of current clinical problems, and family demographics. Statistical tests of the primary outcome, the IFS total score, were performed. RESULTS: Survivors of CDH had a median age of 8 years. Most families had private insurance (78%) and annual income levels higher than the federal poverty level (96%). The IFS total score was correlated with child's clinical severity (r = 0.71, P < .001) and was worse among families of children with current clinical problems (P = .01), families with public insurance (P = .01), and those with out-of-pocket expenses of at least $500 (P = .002). Regression analysis identified the independent effect of clinical severity on family impact (P < .001). CONCLUSION: Family impact is profound and long-standing at a median of 8 years after surgery for a subset of CDH survivors with more severe conditions and current clinical problems.

Approaches to neurodevelopmental assessment in congenital diaphragmatic hernia survivors.

BACKGROUND: Infants with congenital diaphragmatic hernia require complex surgical care and may have neurodevelopmental morbidity. We examined the performance of reports of motor functioning in 25 congenital diaphragmatic hernia survivors using the parent-completed Developmental Profile-II and a clinical evaluation by a neurodevelopmental pediatrician (MD) measured against the Bayley motor scale. METHODS: Bayley motor scores were dichotomized as normal or abnormal. Sensitivity and specificity were calculated for each test. RESULTS: The median age at assessment was 25 months. Bayley motor scores were abnormal in 77% of infants tested (10/13). The MD examinations detected motor problems in 92% (12/13). Sensitivity and specificity of the MD examination were 1.0 and 0.33, respectively. Developmental Profile-II physical scores were abnormal in 15% (2/13); sensitivity and specificity were 0.2 and 1.0, respectively. CONCLUSIONS: The high rate of abnormal motor findings in this study supports the need for ongoing screening and evaluation. The sensitivity of MD examinations was excellent, but hypotonia findings were not universally corroborated by the Bayley. Although specificity of parent-reported motor findings was high, parents underreported abnormal motor findings. Parental reports of neurodevelopmental problems should be heeded, and physicians should perform screening motor examinations. Bayley assessments may be warranted to determine the functional implications of observed abnormalities.

Long-term functional impact of congenital diaphragmatic hernia repair on children.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) is a malformation requiring neonatal surgical repair with in-hospital survival rates above 90%. We examined the long-term functional impact of CDH repair on a cross-sectional cohort of survivors. METHODS: A cohort of 53 CDH families participated in this study. Functional impact was evaluated with parent report of the Functional Status IIR and the Child Health Ratings Inventories General Health Module. Parents also provided a clinical severity score, the child's medical history, and family demographic information. The primary outcome was the effect of medical morbidity on the Functional Status IIR total score. RESULTS: Congenital diaphragmatic hernia survivors had a median age of 8 years; 50% were in third grade or above. Sixty-six percent had major medical issues at hospital discharge, whereas 48% had current clinical problems. Functional Status IIR total score was strongly correlated with child's clinical severity (r = -0.65; P < .0001) and was lower among children with ongoing medical morbidity, denoting worse functioning (P = .01). Child Health Ratings Inventories General Health Module scores followed a similar pattern. CONCLUSIONS: A subset of long-term CDH survivors continues to have ongoing clinical problems a median of 8 years after surgery, translating to lower functional status. Affected children and their families may benefit from prospective identification and ongoing interventions.

Telling children and adolescents about their cancer diagnosis: Cross-cultural comparisons between pediatric oncologists in the US and Japan.

PURPOSE: Over the last 50 years, direct communication about cancer with adults has shifted from an approach of not telling to one of telling. Less is known about communication practices with children. The purpose of this study is to (1) describe patterns of communication at diagnosis between pediatric oncologists and children with cancer and (2) compare cultural differences in these practices in the US and Japan. METHODS: This 2003 survey, developed in English and translated into Japanese was mailed to members of the American Society of Pediatric Hematology/Oncology and the two Japanese Societies of Pediatric Hematology and Oncology; there were 350 US and 362 Japanese respondents. Descriptive statistics and logistic regressions were performed. RESULTS: US physicians had a consistent pattern of telling children (65% always told the child; less than 1% rarely or never told). Japanese physicians had greater variability in their patterns of telling (with only 9.5% always telling, 34.5% rarely or never telling). Direct communication with the child was influenced by personal attitudes, patient factors, and work culture in both countries. Many more variables emerged as influencing Japanese physicians' communication practices than for US physicians. US physicians were influenced by their own sense of responsibility for telling, while Japanese physicians were more influenced by personal attitudes, patient factors, and work culture. CONCLUSIONS: US and Japanese physicians differed when communicating directly with the child about his or her cancer. The impact of these practices on children and their parents should be explored and the parent and child's perspectives elicited. This information will help facilitate culturally sensitive patient and family centered communication.