Assessment of comorbid symptoms in pediatric autonomic dysfunction.

PURPOSE: Pediatric patients with autonomic dysfunction and orthostatic intolerance (OI) often present with co-existing symptoms and signs that might or might not directly relate to the autonomic nervous system. Our objective was to identify validated screening instruments to characterize these comorbidities and their impact on youth functioning. METHODS: The Pediatric Assembly of the American Autonomic Society reviewed the current state of practice for identifying symptom comorbidities in youth with OI. The assembly includes physicians, physician-scientists, scientists, advanced practice providers, psychologists, and a statistician with expertise in pediatric disorders of OI. A total of 26 representatives from the various specialties engaged in iterative meetings to: (1) identify and then develop consensus on the symptoms to be assessed, (2) establish committees to review the literature for screening measures by member expertise, and (3) delineate the specific criteria for systematically evaluating the measures and for making measure recommendations by symptom domains. RESULTS: We review the measures evaluated and recommend one measure per system/concern so that assessment results from unrelated clinical centers are comparable. We have created a repository to apprise investigators of validated, vetted assessment tools to enhance comparisons across cohorts of youth with autonomic dysfunction and OI. CONCLUSION: This effort can facilitate collaboration among clinical settings to advance the science and clinical treatment of these youth. This effort is essential to improving management of these vulnerable patients as well as to comparing research findings from different centers.

Creating a data dictionary for pediatric autonomic disorders.

PURPOSE: Whether evaluating patients clinically, documenting care in the electronic health record, performing research, or communicating with administrative agencies, the use of a common set of terms and definitions is vital to ensure appropriate use of language. At a 2017 meeting of the Pediatric Section of the American Autonomic Society, it was determined that an autonomic data dictionary comprising aspects of evaluation and management of pediatric patients with autonomic disorders would be an important resource for multiple stakeholders. METHODS: Our group created the list of terms for the dictionary. Definitions were prioritized to be obtained from established sources with which to harmonize. Some definitions needed mild modification from original sources. The next tier of sources included published consensus statements, followed by Internet sources. In the absence of appropriate sources, we created a definition. RESULTS: A total of 589 terms were listed and defined in the dictionary. Terms were organized by Signs/Symptoms, Triggers, Co-morbid Disorders, Family History, Medications, Medical Devices, Physical Examination Findings, Testing, and Diagnoses. CONCLUSION: Creation of this data dictionary becomes the foundation of future clinical care and investigative research in pediatric autonomic disorders, and can be used as a building block for a subsequent adult autonomic data dictionary.

Self-instructional CPR training for parents of high risk infants.

OBJECTIVE: Premature infants (PRE) and infants with congenital heart disease (CHD) are at high risk for respiratory or cardiac arrest in their first year. Bystander cardiopulmonary resuscitation (CPR) is a major predictor of resuscitation outcome. The purpose of this study was to assess the usefulness of a self-instructional DVD kit (Infant CPRAnytime) for families of high-risk infants. We hypothesized that comfort level of performing CPR would increase, parents would share the kit with others, and review it during the year. METHODS: Parents of PRE (<35 weeks or <2500 g) or CHD infants received a self-instructional CPR kit. One parent completed a questionnaire, reviewed the DVD, and practiced CPR before discharge. They were asked to share the kit with other care providers, practice CPR every 3 months, and complete questionnaires at 4 and 12 months. RESULTS: We enrolled 311 subjects: 238 PRE and 73 CHD. Comfort level performing CPR increased from 2.8 at baseline to 3.5 at 12 months (p=0.023). The kit was shared with 3.1 additional persons and reviewed by the parent 1.8 times over 12 months. Eight emergency rescue events were reported: choking (3) and CPR (5). All events requiring CPR were in infants with CHD. Six infants survived with reported good or stable neurologic status. CONCLUSIONS: Self-instructional tools provide an excellent method of CPR training for parents of high risk infants. Caregiver comfort increased over 12 months and parents continued to review the kit during the first year. An additional 3.1 persons used the kit for CPR training.