Search

Barth syndrome is a rare but grave neuromuscular genetic disorder. Pain in patients with Barth syndrome has not been extensively described in the literature. This pilot study examined the clinical phenotypes of pain in 38 individuals with Barth syndrome three years and older based on data from personal interviews. The prevalence of pain in the study sample was 23.7% on the day of interview (mean intensity 3.6 ± 2.4) and 68% in the previous week (mean intensity 4.5 ± 2.6). Of the patients who reported pain in the previous week, 52.6% felt aching and 36.8% felt tiring-exhausting. Common locations of reported pain were the head, abdomen, and lower back. Treatments were limited to analgesics, with little opioid or alternative treatments utilized. Results suggest that pain is prevalent in individuals with Barth syndrome. More education and research into the characteristics of Barth syndrome related pain will be useful in enhancing our understanding of this condition and in guiding its management.

Background The most common, debilitating morbidity of sickle cell disease (SCD) is vaso-occlusive crisis (VOC) pain. Although guidelines exist for its management, they are generally not well-followed, and research in other pediatric diseases has shown that clinical pathways improve care. The purpose of our study was to determine whether a clinical pathway improves the acute management of sickle cell vaso-occlusive crisis (VOC) pain in the pediatric emergency department (PED). Procedure Pain management practices were prospectively investigated before and after the initiation of a clinical pathway in the PED of an urban, tertiary care center with 50,000 ED visits per year and approximately 200 active sickle cell patients. The pathway included instructions for triage, monitoring, medication administration, and timing of assessments and interventions. Data were eligible from 35 pre-pathway and 33 post-pathway visits. Primary outcome was time interval to administration of first analgesic medication. Statistical analysis was by Student's t-test, using natural-log-transformed data for outcomes with skewed distribution curves. Results Time interval to first analgesic improved from 74 to 42 minutes (P = 0.012) and to first opioid from 94 to 46 minutes (P = 0.013). The percentage of patients who received ketorolac increased from 57% to 82% (P = 0.03). Decrease in time interval to subsequent pain score assessment was not statistically significant (110 to 72 minutes (P = 0.07)), and change in pain score was not different (P = 0.25). Conclusions The use of a clinical pathway for sickle cell VOC in the PED can improve important aspects of pain management and merits further investigation and implementation. Pediatr Blood Cancer 2014;61:693–696. © 2013 Wiley Periodicals, Inc.

Pruritus is prevalent in children with atopic dermatitis and associated with effects on mood, quality of life, sleep, scholastic performance, social and family functioning. In this study a 7-year-old African American female with severe atopic dermatitis, itching and pain refractory to multiple systemic and topical medications was referred for treatment. At baseline, the patient scratched to the point of bleeding, despite maximal doses of anti-histamines, antidepressant and topical therapies. The patient became progressively shy, anxious, and her scholastic performance suffered. A literature review prompted the implementation of a multi-modal program of family cognitive behavioral therapy, imagery, aromatherapy, drawing, and biofeedback. The results were that decreased itch, scratching, pain, and anxiety were seen within the first month. Fewer lesions and episodes of bleeding were observed with almost complete skin clearance by the fourth month. The article concludes that a short-term, integrative program including psychological, complementary and alternative medicine (CAM), and medical therapies may represent a novel, efficacious approach for children suffering from severe atopic dermatitis.

Objective:Using data from a multi-site study of parent–child symptom reporting concordance, this secondary analysis explored the role of parent self-efficacy related to pain management for seriously ill school-age children and adolescents.Method:In the initial study, 50 children and adolescents who were expected to survive 3 years or less were recruited along with their parent/primary caregiver. Parent self-report data were used in this secondary analysis to describe parent self-efficacy for managing their child's pain, caregiver strain, mood states, and perception of the child's pain; to explore relationships among these variables; and to determine predictors of greater self-efficacy.Results:Parents expressed a wide range of self-efficacy levels (Chronic Pain Self-Efficacy Scale; possible range 10–100, mean 76.2, SD 14.7) and higher levels on average than reported previously by family caregivers of adult patients. Caregiver Strain Index scores were markedly high (possible range 0–13, mean 8.1, SD 3.8) and inversely correlated with self-efficacy (r = −0.44, p = 0.001). On the Profile of Mood States parents reported more negative moods (t = 4.0, p t = −5.0, p R2 = 0.51).Significance of results:Findings advance knowledge of parent self-efficacy in managing the pain of a child with life-threatening illness. Results can be used to design supportive interventions enhancing parents’ caregiving roles during their child's last stages of life.

This pilot study was undertaken to evaluate the hypotheses that there are differences in pediatric pain management (PPM) knowledge across resident specialties, that questions in the form of multiple-choice items could detect such differences, and that resident knowledge of analgesic-related adverse drug events (ADEs) would be greater than knowledge of PPM. Questions were based on two general categories of knowledge within acute pain management in hospitalized children: pediatric pain assessment and treatment, and identification of analgesic-related ADEs. As part of the pilot nature of this study, a convenience sample of 60 residents completed a 10-item PPM knowledge assessment prior to a PPM lecture. Twenty-six were pediatric residents (43%), 19 were orthopedic residents (32%), and 15 were anesthesiology residents (25%). All items had content validity. When controlling for resident year, performance by resident specialty was significantly different between anesthesia and orthopedics (P=0.006) and between anesthesia and pediatrics (P0.001). Resident knowledge of analgesic-related ADEs was not greater than knowledge of PPM. The most difficult topics were opioid equianalgesia, assessment of the cognitively impaired child, and maximal acetaminophen doses. Repeated administration of the PPM knowledge assessment at multiple institutions will allow further evaluation of our initial findings, and with directed educational interventions, provide opportunity for measurement of improvement.

OBJECTIVES Many adolescents and young adults report having chronic pain. Urine drug toxicology (UDT) is not routinely used in the pediatric pain management population, despite more routine use in adults with pain, particularly those prescribed opioids. As a first step toward establishing monitoring practices in pediatric and adolescent pain management, the present study evaluated the role of UDT in conjunction with a standard clinical interview in identifying the rate of adherence to an established analgesic regimen. The study also aimed to assess the use of UDT in identifying possible aberrant behaviors in this population. METHODS Data were acquired from a convenience sample of 50 pediatric and adolescent pain management initial consultations, during which a clinical interview and UDT were conducted. Data were analyzed to determine adherence to an established analgesic prescription regimen, and for identification of aberrant behaviors including concurrent use of illicit substances and prescription medication misuse. Other pertinent demographic and clinical factors were examined as factors in adherence. RESULTS Opioid medications were prescribed for 42% of the sample receiving pain medications, and 22% of the sample was nonadherent to their prescription analgesic regimen. Factors associated with a higher likelihood of nonadherence were an older age and having an opioid prescription. The majority (90%) of those nonadherent to their analgesic regimen displayed some form of aberrant behavior. Among the nonadherent patients, 50% were identified by UDT alone, and 50% were identified by self-report during the clinical encounter. CONCLUSIONS These results highlight the challenges of identifying nonadherence to a prescription regimen among adolescents with chronic pain. In addition, this preliminary work suggests that UDT could be used in conjunction with careful clinical interviewing to substantiate patient report and increase the likelihood of detecting analgesic nonadherence and aberrant behaviors.

Lower-extremity trauma is an uncommon but reported cause of sciatic nerve injury in children and adolescents. Failure to identify sciatic neuropathy after traumatic injury to the lower extremity may lead to the delayed institution of neuropathic pharmacotherapy, electrodiagnostic testing, physical therapy, and increased risk for the development of complex regional pain syndrome. This article presents a case of an adolescent male with neuropathic pain and weakness in the right lower extremity after traumatic injury. Spontaneous recovery of the injured nerve occurred with early institution of pharmacologic and physical therapies. Operative exploration and neurolysis were considered but were not ultimately necessary.

Objectives of this review were to examine definitions and background of palliative care, as well as address whether there is an increased need for palliative care education among neurologists. The review also explores what literature exists regarding palliative care within general neurology and child neurology. A literature review was conducted examining use of palliative care within child neurology. More than 100 articles and textbooks were retrieved and reviewed. Expert guidelines stress the importance of expertise in palliative care among neurologists. Subspecialties written about in child neurology include that of peripheral nervous system disorders, neurodegenerative diseases, and metabolic disorders. Adult and child neurology patients have a great need for improved palliative care services, as they frequently develop cumulative physical and cognitive disabilities over time and cope with decreasing quality of life before reaching the terminal stage of their illness.

Epidermolysis bullosa (EB) describes a spectrum of disease from occasional bullae and callus formation to a debilitating life-threatening condition. In this study, we report the use of intravenous ketamine given orally to an infant with a phenotypically severe form of EB simplex, Dowling-Meara subtype, to achieve analgesia during painful dressing changes.

Addiction to painkillers or other substances in pediatric and adolescent cases of noncancer chronic pain is an understudied phenomenon, even amidst documented increases in rates of prescription opioid use and misuse. Case studies can inform the training of clinicians in ethically negotiating a balance between optimizing analgesia and mitigating risk of aberrant drug-taking behaviors. This report discusses an 18-year-old woman with idiopathic scoliosis and clinical depression secondary to undertreated refractory chronic back pain who underwent surgery to correct pseudoarthrosis after a prior spinal instrumentation operation. This intervention in conjunction with a course of patient-controlled analgesia, hydromorphone, and outpatient tramadol, naproxen, methadone, and gabapentin was successful in addressing her long-standing lumbar pain. The patient, however, continued to complain to her pain management team of postsurgical discomfort and insisted on being prescribed Ultracet™ (acetaminophentramadol) rather than generic tramadol. The patient’s eventual disclosure of severe withdrawal discomfort and history of covert abuse of Ultracet ™ is discussed with respect to key warning signs, gaps, and contingencies in the screening, surgical, and pain management processes.Keywords: adolescent, chronic pain, prescription opioid abuse, tramadolDOI:10.5055/jom.2013.0163

The most common, debilitating morbidity of sickle cell disease (SCD) is vaso-occlusive crisis (VOC) pain. Although guidelines exist for its management, they are generally not well-followed, and research in other pediatric diseases has shown that clinical pathways improve care. The purpose of our study was to determine whether a clinical pathway improves the acute management of sickle cell vaso-occlusive crisis (VOC) pain in the pediatric emergency department (PED).Pain management practices were prospectively investigated before and after the initiation of a clinical pathway in the PED of an urban, tertiary care center with 50,000 ED visits per year and approximately 200 active sickle cell patients. The pathway included instructions for triage, monitoring, medication administration, and timing of assessments and interventions. Data were eligible from 35 pre-pathway and 33 post-pathway visits. Primary outcome was time interval to administration of first analgesic medication. Statistical analysis was by Student's t-test, using natural-log-transformed data for outcomes with skewed distribution curves.Time interval to first analgesic improved from 74 to 42 minutes (P = 0.012) and to first opioid from 94 to 46 minutes (P = 0.013). The percentage of patients who received ketorolac increased from 57% to 82% (P = 0.03). Decrease in time interval to subsequent pain score assessment was not statistically significant (110 to 72 minutes (P = 0.07)), and change in pain score was not different (P = 0.25).The use of a clinical pathway for sickle cell VOC in the PED can improve important aspects of pain management and merits further investigation and implementation.

Withdrawal syndromes following administration of sedative and analgesic infusions represent a significant morbidity that is described increasingly in the pediatric critical care literature. However, there are few descriptions of pentobarbital withdrawal symptoms and their treatment in the pediatric population. We describe an infant receiving multiple sedative and analgesic medications, including pentobarbital, who was resistant to our institution's standard weaning protocol, but whose severe withdrawal symptoms responded dramatically to phenobarbital loading and maintenance.

Objectives: To characterize the opioid prescribing and monitoring practices of providers for chronic nonmalignant pain (CNP) and subacute postoperative pain (SAPOP) in adolescents.Design: Web-based cross-sectional self-report survey.Setting: Free-standing pediatric tertiary academic center.Participants: A total of 183 physicians and nurse practitioners were eligible. Of 115 (62.8 percent) participants who responded, 108 (93.9 percent) completed the survey.Main outcome measures: Self-reported frequency of opioid prescription for SAPOP and CNP conditions and frequency of associated monitoring practices.Results: For 10 of the 13 pain conditions included, some participants endorsed “monthly or more opioid prescriptions” while others endorsed “opioids do not represent appropriate management.” Opioid prescribing is present for almost all pain conditions but is substantially more common for nonacute vaso-occlusive-related sickle cell disease, scoliosis correction, and video-assisted pectus excavatumrelated pains. When compared with the reference group, CNP with no identifiable pathology, the odds ratio (OR) of an opioid being prescribed for CNP states with identifiable pathology was not significantly higher. The OR for SAPOP was significantly higher (p < 0.0001). None of the opioid prescribers reported collecting urine toxicology before or during opioid therapy.Conclusions: This survey identifies a diversity of self-reported clinician opioid prescribing practices for adolescents with CNP and SAPOP. Urine collection for drug toxicology screening is not utilized by opioid prescribers. Surveys of similar clinician practice behaviors at other institutions are warranted to replicate this finding and to establish common clinical practice for usage and monitoring of opioids in conditions where guidelines do not yet exist.

Phenomenologic analysis of initial consults provided during the first year of a new Pediatric Advanced Care Team (PACT) program provides essential understanding of the experience and inform program direction and future clinical research. Parents bring to the consult a desire to remain experts in their children’s lives yet experience vulnerability as they seek assistance in making critical decisions often under conditions of disquieting uncertainty. Dynamic communication efforts involving the referring providers, PACT team members, and family are a key influence in facilitating consults’ stated goals and in establishing the integrated palliative paradigm in a tertiary care environment. Validation was provided for a new research infrastructure that will function concurrently with the PACT clinical program in this rapidly evolving field.

BackgroundThere are well-established deficiencies in residents' knowledge of acute-pain assessment and treatment in hospitalized children.MethodsAmong residents in 3 specialties (anesthesiology, orthopedics, and pediatrics), we investigated whether a pediatric pain management (PPM) curriculum that offered a lecture combined with a demonstration of how to use the OUCH card would yield higher performance on a subsequent PPM knowledge assessment. The OUCH card was created as a portable reference tool for trainees to provide analgesic dosing information, pain-assessment tools, and treatment of opioid-induced adverse effects. There was an initial convenience sample of 60 residents randomized to Form A or B of the pretest. From this, 39 residents (15 anesthesiology, 13 orthopedic, 11 pediatric) completed a PPM knowledge posttest approximately 4 weeks after the pretest, PPM lecture, and OUCH card instruction.ResultsUsing a repeated measure design, the interaction of resident specialty and pretest to posttest scores was significant (P = .01) along with the covariate of residency year (P = .026).ConclusionsThese preliminary data based on a convenience sample of residents suggest that PPM training along with use of the OUCH card may help to reduce knowledge differences among residents. Faculty whose clinical practice includes children with acute pain should consider including learning or performance aids like the OUCH card in education and clinical care for its potential benefit in resident learning.

HERE’S THE CASE A 15-year-old girl from the Caribbean presented with high-grade pleomorphic osteogenic sarcoma of her distal right femur and multiple bilateral pulmonary nodules. After two courses of high-dose methotrexate, cisplatin, and doxorubicin, a limb-sparing procedure was performed. The margins were negative for tumor, and the tumor necrosis was approximately 75%. Chemotherapy continued, bilateral thoracotomies were performed, and ifosfamide and etoposide were added to the patient’s regimen. Imaging studies showed no evidence of disease, and therapy was continued in her home country. She returned with a left-sided foot drop

Abstract 2660 Background: Sickle cell disease causes considerable morbidity and mortality. Three guidelines exist for the management of vaso-occlusive pain, although studies have shown that these guidelines are not well followed and that there is considerable variation in care. Investigations of clinical pathways for pediatric sickle cell pain have not been published to our knowledge, but research in other pediatric conditions has shown that the implementation of clinical pathways can improve medical care. We initiated an investigation of the effects of a pain management pathway for sickle cell vaso-occlusive crisis in the emergency department (ED) with the hypothesis that the introduction of a clinical pathway would improve the efficiency and efficacy of acute pain management. Methods: We conducted a prospective, cohort study from February 2009 to March 2010 in an urban, tertiary care ED. We collected data from patients aged 3–18 years old with sickle cell disease who presented to the ED with vaso-occlusive crisis (VOC) pain. Baseline data was collected for five months prior to the introduction of the clinical pathway, followed by a four-week time interval in which the ED physicians and nurses were in-serviced on the pathway, followed by six months in which data was collected with the clinical pathway in place. Our pathway, which is in checklist format with instructions for triage, monitoring, medication administration, and timing of assessments and interventions, was developed by representatives from the divisions of Pediatric Hematology, Pediatric Emergency Medicine, and Pediatric Pain Medicine, Symptom Management and Palliative Care and based upon current standard-of-care guidelines. Results: Over the eleven month study period, 68 patients were enrolled. Random chart audits revealed a 75% capture rate. Significant improvement was demonstrated in time interval to first analgesic from 74 minutes to 42 minutes (p value 0.02), time interval to first opioid from 94 minutes to 46 minutes (p value < 0.01), and time interval to subsequent assessment of pain score from 110 minutes to 72 minutes (p value 0.02). The percentage of patients who received ketorolac also increased from 57% to 82% (p value 0.03). Change in pain scores was not significantly different, nor was admission rate. Conclusions: Implementation of a pain management pathway for sickle cell VOC led to an improvement in the time interval to administration of first analgesic and time interval to pain re-assessment, bringing these aspects of patient management closer to accepted guidelines. Whether improvement can be made in admission rates, change in pain ratings, and patient satisfaction will require further study. Disclosures: No relevant conflicts of interest to declare.