Robert J. Graham

Chronic Ventilator Need in the Community: A 2005 Pediatric Census of Massachusetts

OBJECTIVES. The purpose of this study was to describe the population of children with chronic mechanical ventilation in Massachusetts and their patterns of medical care. PATIENTS AND METHODS. Investigators surveyed all of the Massachusetts home ventilator clinics, pediatric pulmonary services, hospital-based pediatric services for special health care needs, insurers, home care vendors, nursing agencies, the Massachusetts Department of Public Health, selected individual providers, and rehabilitation and long-term care facilities providing services to children with chronic respiratory support needs. Support was defined as daily use of noninvasive, negative-pressure, or invasive/transtracheal ventilators. Subsequent matching of demographic data, including date of birth, zip code, and gender supported maximal census yield without duplications. Geographic information systems were used to create distribution maps and estimate distances between children with chronic mechanical ventilator needs and key resources. RESULTS. A total of 197 children were identified as requiring chronic mechanical respiratory support in Massachusetts in 2005, which was a nearly threefold increase in this population in the 15-year interval since the last census. Congenital or perinatal-acquired neurologic or neuromuscular disorders constituted the majority of primary diagnoses (n = 107 [54%]). Chronic lung disease attributed to prematurity represented only 7% of the sample. CONCLUSIONS. Children receiving chronic mechanical respiratory support are a growing population. The shift in underlying diagnoses from pulmonary disease to neurogenic respiratory insufficiency has implications for hospital and community-based providers from all disciplines in extending services to the home setting. Barriers encountered when performing this study, however, reflect an overall lack of coordination among the many individuals and agencies involved in their care. Coordinated and centralized care efforts require a clear and managed flow of information; census reports such as this one are only the beginning. Direct needs assessments and quality-of-life surveys from families are needed to design and implement programmatic changes and advocacy efforts.

Chronic conditions among children admitted to U.S. pediatric intensive care units: Their prevalence and impact on risk for mortality and prolonged length of stay*

Objective:To estimate the prevalence of chronic conditions among children admitted to U.S. pediatric intensive care units and to assess whether patients with complex chronic conditions experience pediatric intensive care unit mortality and prolonged length of stay risk beyond that predicted by commonly used severity-of-illness risk-adjustment models. Design, Setting, and Patients:Retrospective cohort analysis of 52,791 pediatric admissions to 54 U.S. pediatric intensive care units that participated in the Virtual Pediatric Intensive Care Unit Systems database in 2008. Measurements:Hierarchical logistic regression models, clustered by pediatric intensive care unit site, for pediatric intensive care unit mortality and length of stay >15 days. Standardized mortality ratios adjusted for severity-of-illness score alone and with complex chronic conditions. Main Results:Fifty-three percent of pediatric intensive care unit admissions had complex chronic conditions, and 18.5% had noncomplex chronic conditions. The prevalence of these conditions and their organ system subcategories varied considerably across sites. The majority of complex chronic condition subcategories were associated with significantly greater odds of pediatric intensive care unit mortality (odds ratios 1.25–2.9, all p values < .02) compared to having a noncomplex chronic condition or no chronic condition, after controlling for age, gender, trauma, and severity-of-illness. Only respiratory, gastrointestinal, and rheumatologic/orthopedic/psychiatric complex chronic conditions were not associated with increased odds of pediatric intensive care unit mortality. All subcategories were significantly associated with prolonged length of stay. All noncomplex chronic condition subcategories were either not associated or were negatively associated with pediatric intensive care unit mortality, and most were not associated with prolonged length of stay, compared to having no chronic conditions. Among this group of pediatric intensive care units, adding complex chronic conditions to risk-adjustment models led to greater model accuracy but did not substantially change unit-level standardized mortality ratios. Conclusions:Children with complex chronic conditions were at greater risk for pediatric intensive care unit mortality and prolonged length of stay than those with no chronic conditions, but the magnitude of risk varied across subcategories. Inclusion of complex chronic conditions into models of pediatric intensive care unit mortality improved model accuracy but had little impact on standardized mortality ratios.

Predictors of Clinical Outcomes and Hospital Resource Use of Children After Tracheotomy

OBJECTIVES: The objectives are to describe health outcomes and hospital resource use of children after tracheotomy and identify patient characteristics that correlate with outcomes and hospital resource use. PATIENTS AND METHODS: A retrospective analysis of 917 children aged 0 to 18 years undergoing tracheotomy from 36 childrens hospitals in 2002 with follow-up through 2007. Children were identified from ICD-9-CM tracheotomy procedure codes. Comorbid conditions (neurologic impairment [NI], chronic lung disease, upper airway anomaly, prematurity, and trauma) were identified with ICD-9-CM diagnostic codes. Patient characteristics were compared with in-hospital mortality, decannulation, and hospital resource use by using generalized estimating equations. RESULTS: Forty-eight percent of children were ≤6 months old at tracheotomy placement. Chronic lung disease (56%), NI (48%), and upper airway anomaly (47%) were the most common underlying comorbid conditions. During hospitalization for tracheotomy placement, children with an upper airway anomaly experienced less mortality (3.3% vs 11.7%; P < .001) than children without an upper airway anomaly. Five years after tracheotomy, children with NI experienced greater mortality (8.8% vs 3.5%; P ≤ .01), less decannulation (5.0% vs 11.0%; P ≤ .01), and more total number of days in the hospital (mean [SE]: 39.5 [4.0] vs 25.6 [2.6] days; P ≤ .01) than children without NI. These findings remained significant (P < .01) in multivariate analysis after controlling for other significant cofactors. CONCLUSIONS: Children with upper airway anomaly experienced less mortality, and children with NI experienced higher mortality rates and greater hospital resource use after tracheotomy. Additional research is needed to explore additional factors that may influence health outcomes in children with tracheotomy.

Integrating palliative care into chronic care for children with severe neurodevelopmental disabilities.

BACKGROUND Children with severe neurodevelopmental disabilities and complex medical conditions are a growing and unique segment of the pediatric population. The increasing use of life sustaining technologies has provided the chance at an extended life and increasing inclusion within the broader community. Families work to overcome personal and professional biases, clinical uncertainties, and pragmatic obstacles to improve quality of life. Little attention, however, has been paid to the unique challenges of caring for a dying child affected with neurodevelopmental delay. DISCUSSION In this paper we outline several specific barriers to the provision of excellent end-of-life care for these children and their families. We also outline our approach for overcoming these barriers. SUMMARY The benefit of comprehensive palliative care in select pediatric populations has been demonstrated. Extending and tailoring those service to meet the unique needs of children with severe disabilities is the next logical step in that continuum. Ultimately, acknowledging that the lives of children with neurodevelopmental disabilities and their families have a unique quality will permit the human face of medical care to keep pace with technologic advances. Appreciation of the value of a perhaps incomparable quality of life will allow for a better quality of dying for children with severe neurodevelopmental disabilities.

Evidence-based guideline summary: Evaluation, diagnosis, and management of congenital muscular dystrophy: Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Issues Review Panel of the American Association of Neuromuscular & Electrodiagnostic Medicine.

Objective: To delineate optimal diagnostic and therapeutic approaches to congenital muscular dystrophy (CMD) through a systematic review and analysis of the currently available literature. Methods: Relevant, peer-reviewed research articles were identified using a literature search of the MEDLINE, EMBASE, and Scopus databases. Diagnostic and therapeutic data from these articles were extracted and analyzed in accordance with the American Academy of Neurology classification of evidence schemes for diagnostic, prognostic, and therapeutic studies. Recommendations were linked to the strength of the evidence, other related literature, and general principles of care. Results: The geographic and ethnic backgrounds, clinical features, brain imaging studies, muscle imaging studies, and muscle biopsies of children with suspected CMD help predict subtype-specific diagnoses. Genetic testing can confirm some subtype-specific diagnoses, but not all causative genes for CMD have been described. Seizures and respiratory complications occur in specific subtypes. There is insufficient evidence to determine the efficacy of various treatment interventions to optimize respiratory, orthopedic, and nutritional outcomes, and more data are needed regarding complications. Recommendations: Multidisciplinary care by experienced teams is important for diagnosing and promoting the health of children with CMD. Accurate assessment of clinical presentations and genetic data will help in identifying the correct subtype-specific diagnosis in many cases. Multiorgan system complications occur frequently; surveillance and prompt interventions are likely to be beneficial for affected children. More research is needed to fill gaps in knowledge regarding this category of muscular dystrophies.

Delayed-Onset Seizure and Cardiac Arrest After Amitriptyline Overdose, Treated With Intravenous Lipid Emulsion Therapy

In recent years, intravenous lipid emulsion (ILE) therapy has emerged as a new rescue antidote for treatment of certain toxicities, including cyclic antidepressants, and as the primary treatment of toxic manifestations after local anesthetic exposure. We present a case of a 13-year-old girl who developed delayed seizures and cardiac arrest after amitriptyline ingestion. As part of the treatment, she was treated with ILE therapy. The patients laboratories were not interpretable for several hours after the lipid emulsion. The patient developed pancreatitis after the ILE therapy. This case is unique; not only is it one of the first reported cases of lipid emulsion being used in a pediatric patient, but in that the patient developed delayed toxicity and iatrogenic harm from the ILE.

Experiencing the pediatric intensive care unit: perspective from parents of children with severe antecedent disabilities.

Objective:To describe the experience of pediatric intensive care hospitalization from the perspective of a parents of children with severe, antecedent disability. Design:Qualitative analysis of data obtained through semistructured interviews. Setting:Pediatric intensive care unit (PICU) in a children’s hospital. Subjects:Parents of eight children admitted to the PICU with severe antecedent disabilities. Interventions:None. Measurements and Main Results:Data were collected between May and July 2006. Parents were interviewed during, or just after, PICU admission until data saturation was achieved. Seven major themes emerged from analysis: 1) know my child’s baseline; 2) integrate and bridge multiple services; 3) disconnect between role of parent at home versus parent in the PICU; 4) a PICU admission does not equate with respite; 5) high stakes learning environment; 6) heterogeneity within group; and 7) lack of fit within the acute care model. The need for stellar communication and mutuality within the parent-professional relationship were common conceptual threads through all interviews. Conclusions:Children with severe antecedent disabilities and their parents are inextricably linked with critical care services. Study results provide data that can be used to help clinicians better understand the perspectives of these parents and also help guide interventions to improve care and support. Although communication is essential, the structure and processes of critical care including rounding format, continuity in management, and mutual participation models should be tested and tailored to better meet parental needs and expectations.

Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care

Spinal muscular atrophy (SMA) is a severe neuromuscular disorder due to a defect in the survival motor neuron 1 (SMN1) gene. Its incidence is approximately 1 in 11,000 live births. In 2007, an International Conference on the Standard of Care for SMA published a consensus statement on SMA standard of care that has been widely used throughout the world. Here we report a two-part update of the topics covered in the previous recommendations. In part 1 we present the methods used to achieve these recommendations, and an update on diagnosis, rehabilitation, orthopedic and spinal management; and nutritional, swallowing and gastrointestinal management. Pulmonary management, acute care, other organ involvement, ethical issues, medications, and the impact of new treatments for SMA are discussed in part 2.

Pediatric Neurocritical Care: A Neurology Consultation Model and Implication for Education and Training

Pediatric neurocritical care is developing specialization within pediatric intensive care and pediatric neurology practice, and the evolving clinical expertise has relevance to training and education in both fields. We describe a model of service using a Neurology Consulting Team in the intensive care unit setting. Medical records were reviewed from a 32-month cohort of Neurology Consulting Team referrals. Six hundred eighty-nine (19%) of 3719 patients admitted to the intensive care unit were assessed by the team. The most common diagnostic categories were seizures, neurosurgical, cerebrovascular, or central nervous system infection. Fifty-seven percent (350 of 615 patients) required mechanical ventilation. Cohort mortality was 7% vs 2% for the general intensive care population (P < 0.01). The team provided 4592 initial and subsequent consultations; on average there were five to six new consultations per week. Each patient had a median of two (interquartile range, 1 to 6) consultations during admission. Three quarters of the cohort required neurodiagnostic investigation (1625 tests), with each patient undergoing a median of two (range, 0 to 3) studies. Taken together, the subset of pediatric intensive care unit patients undergoing neurology consultation, investigation, and management represents a significant practice experience for trainees, which has implications for future curriculum development in both pediatric critical care medicine and pediatric neurology.

Anesthesia and perioperative medical management of children with spinal muscular atrophy

Objective:  To describe the perioperative medical care, anesthetic considerations, and the risk of postanesthetic respiratory failure in patients with pediatric spinal muscular atrophy (SMA).