Sensory processing in children with Paediatric Acute-onset Neuropsychiatric Syndrome.

INTRODUCTION: Previous research indicates that children with Paediatric Acute-onset Neuropsychiatric Syndrome (PANS) experience sensory reactivity differences that impact occupational performance. The purpose of this study was to determine whether there are differences in sensory reactivity in these children across two different time points; during exacerbation and during remission, using the Sensory Processing Measure (SPM) Home-Form. The study also sought to investigate whether children with PANS experience sensory differences during remission periods, when compared with SPM Home-Form norms. METHODS: A two-period bidirectional case-crossover design was used, and an online assessment was conducted to measure sensory reactivity. Parents of children aged 4.6 to 13.1 years with a diagnosis of PANS were recruited from various sites across Australia, USA, England, Ireland, Scotland, Canada, and New Zealand. The SPM Home-Form was used to measure sensory reactivity at two time points, when PANS was in remission (T-R) and in exacerbation (T-E). Study entry was permitted at either T-E or T-R. Participant exacerbation status was monitored over a maximum 12-month period, and a follow-up SPM Home-Form was sent when a change in exacerbation status was indicated. A linear mixed model was used to assess the difference between SPM Home-Form norm-referenced scores during exacerbation and remission. RESULTS: The study included 82 participants, with 80 providing data at study entry, and 27 providing data at follow-up. Results showed a statistically significant decline in performance across the SPM Home-Form domains of Hearing, Social Participation, Planning and Ideas, and Total Sensory Systems T-scores during exacerbation when compared with remission data. Results also demonstrated atypical sensory reactivity across Vision, Hearing, Touch, Balance and Motion, and Total Sensory Systems domains during periods of remission compared with SPM Home-Form norms. CONCLUSION: This study found that children with PANS experience significant sensory reactivity differences during exacerbation and remission across multiple sensory domains, with a decline in performance during exacerbation. Where there are occupational performance challenges, occupational therapists should consider administering sensory assessments so that effective intervention plans addressing the unique sensory reactivity needs of children with PANS can be developed.

Caregiver Burden, Stress, and Relationship Cohesion Among Self-Identified Caregivers of Children with Pediatric Acute-Onset Neuropsychiatric Syndrome.

Objective: Children with Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) experience sudden onset neuropsychiatric symptoms after infection or other triggers. Symptoms range from mild to severe, potentially lasting days, weeks, months, or longer. Exacerbation-related functional decline presents in many aspects of daily life, generally accompanied by family stress and caregiver burden. We sought to investigate the relationship between severity of PANS symptoms and caregiver burden/stress and the relationship between severity of PANS symptoms and degree of caregiver/child cohesion. Methods: This cross-sectional online study surveyed caregivers recruited from PANS-related social media support sites. The Pediatric Acute Neuropsychiatric Symptom Scale - Parent Version (PNSS) measured current severity. Caregiver Burden Inventory (CBI) and Caregiver Self-Assessment Questionnaire (CSAQ) assessed caregiver burden/stress. Inclusion of Other in the Self (IOS) scale determined caregiver-perceived current and desired cohesion with their child(ren) with PANS. Results: Of the 216 respondents 79.6% exceeded CBI threshold indicating need for respite in adult care receiver populations. On the CSAQ, 72.9% expressed high distress, 80.5% reported feeling overwhelmed, and 58.1% reported crying spells, meeting cutoffs for support/respite used in adult care receiver populations. Most caregivers reported not having the desired degree of cohesion with their child on the IOS (85.5%). Parents of children with more severe PNSS symptoms fared significantly worse on all measures (CBI: H = 57.83; CSAQ: F = 29.26; IOS: H = 38.04; p < 0.001 for all). Content analysis of comments revealed five themes: (1) severe caregiver and/or family emotional distress and trauma; (2) caregivers wondering what happened to their child; (3) lack of awareness and support among health and education professionals; (4) relationship strain with family, friends, and significant others; and (5) financial and/or legal struggles because of their child's diagnosis. Conclusion: There is strong need for support and respite for children with PANS and their families. Long-term effects including posttraumatic stress symptoms among family members should be studied.

Factors Associated with Symptom Persistence in PANS: Part II-Presenting Features, Medical Comorbidities, and IVIG Treatment History.

Objective: Individuals with Pediatric Acute Onset Neuropsychiatric Syndrome (PANS) experience neuropsychiatric symptoms following an infection or other trigger. Although PANS is typically described as relapsing-remitting, a large community-based 2017 study revealed a range of courses. The present study examined clinical predictors of symptom persistence, measured as % days symptom-free, in this same sample. Methods: A 146-question online survey gathered histories (infections and other triggers, medical and developmental comorbidities), symptomatology, interventions, and outcomes (including school functioning) of PANS patients. Multivariate analyses were applied to examine associations between these variables and % days symptom-free across the disease course. Results: Among the 646 subjects included, significant relationships were found between greater symptom persistence and higher rates of medical comorbidities (especially rashes, headaches, chronic sinusitis, frequent diarrhea, and immune deficiencies), developmental diagnoses, and respondent-perceived developmental lags. Subjects with greater symptom persistence were significantly more likely to report PANS exacerbations associated with infections in close contacts, vaccinations, environmental triggers, and exacerbations of comorbidities and were more likely to report PANS recurrences triggered by Epstein Barr Virus, mycoplasma, and sinus infections. More persistent PANS was also associated with significantly higher frequencies of certain symptoms (sleep disturbance, urinary incontinence, muscle pain, brain fog, sensory defensiveness, irritability, and aggression-related symptoms), less effectiveness of intravenous immunoglobulin in combating symptoms, and more difficulty attending school. Conclusions: Our results suggest high symptom persistence in PANS to be associated with more pervasive medical and neuropsychiatric symptoms. Differences in symptom persistence are associated with both intrinsic (e.g., immune competence) and extrinsic (e.g., infections, treatment) factors. Because extrinsic factors are potentially modifiable, it is critical that providers be aware of current guidelines on PANS evaluation and treatment.

Factors Associated with Symptom Persistence in PANS: Part I-Access to Care.

Objective: Pediatric acute-onset neuropsychiatric syndrome (PANS) presents with abrupt neuropsychiatric symptoms, often after an immunologic trigger. A 2017 survey of 698 subjects found diagnostic delays to be associated with recurrences, suggesting that timely care impacts course. This secondary analysis explores the impact of barriers to care on symptom persistence. Methods: A 146-question online survey gathered history, symptomatology, intervention, and outcome data from subjects with PANS. Multivariate analyses examined associations between symptom persistence over the entire reported disease course, measured as % days symptom-free over reporting periods averaging approximately 4 years, and access-to-care history, reflected in availability of medical expertise and affordability of care. The impacts of time from symptom onset to treatment and effectiveness of initial antibiotics were also examined. Results: Among the 646 subjects analyzed, greater symptom persistence was associated with longer intervals between symptom onset and treatment (F = 4.43, p = 0.002). Thirty-four percent of subjects with the least symptom persistence (>75% symptom-free days), versus 13% of those with the most (symptoms every day), had been diagnosed by the first practitioner seen (likelihood ratio [L-R] χ2 = 36.55, p < 0.0001, for comparison across all groups). Diagnosis and treatment had not been impeded by lack of access to expertise for 52% of subjects with the least persistent symptoms, versus 22% of those with the most (L-R χ2 = 22.47, p < 0.0001). Affordability had not impacted diagnosis and treatment for 76% of subjects with the least persistent symptoms, versus 42% of those with the most (L-R χ2 = 27.83, p < 0.0001). The subjects whose PANS symptoms resolved with antibiotic treatment of the inciting infection experienced less symptom persistence than others (χ2 = 23.27, p = 0.0001). More persistently symptomatic subjects were more likely to have discontinued intravenous immunoglobulin (IVIG) treatment for access-to-care reasons. Conclusions: Unimpeded access to care for PANS is associated with more symptom-free days over reporting periods averaging approximately 4 years. Difficulty reaching expert providers, missed opportunities for diagnoses, and financial limitations may worsen outcomes. Practitioners, particularly primary providers, should adhere to published diagnostic and treatment guidelines promptly upon presentation.

Validation of the Interprofessional Collaborative Competency Attainment Survey (ICCAS) retrospective pre-test measures.

Establishing the effectiveness of IPE experiences is essential, and current assessment measures may be subject to various types of bias. The Interprofessional Collaborative Competency Attainment Survey (ICCAS) is administered after the learning experience as retrospective pretest and posttest measures of perceived collaborative practice skills. Because the ICCAS does not have an actual pretest, there are potential risks of recall bias and social desirability bias. To address these concerns, Jackson (2017) proposed conducting a true ICCAS pretest before the learning experience and examining the differences between the true and retrospective pretest scores and the differences between these pretest and posttest scores. This study design was implemented for two in-person Interprofessional forums conducted in the Fall (N = 745) and following Spring (N = 599) semesters. Students from 11 (Fall) and 12 (Spring) health professions education programs were included. True ICCAS pretest scores did not significantly differ from the retrospective pretest scores in either sample, although the effect sizes for pretest to posttest gains were slightly smaller for the true pretest scores. These results support and provide confidence for using the standard ICCAS administration methodology by demonstrating the administration methodology is not adversely impacted by recall or social desirability biases.

Assessing the assessment: a comparison of the IPASS and ICCAS to measure change in interprofessional competencies following a large-scale interprofessional forum.

Measuring the effectiveness of interprofessional education (IPE) experiences is essential but challenging. Surveying learners before (pretest) and after (posttest) an IPE experience may result in minimal change due to response shift bias. Retrospective pretest/posttest assessment may ameliorate response shift bias but may also result in inflated change scores due to social desirability bias. We studied a cohort of 675 students from 12 health professions who completed the Interprofessional Attitudes Scale (IPAS) within the 4 weeks before (pretest) and the 3 weeks after (posttest) an IPE forum and completed the Interprofessional Collaborative Competency Attainment Survey (ICCAS) within the 3 weeks after the forum as a retrospective pretest/posttest. We found higher scores on the pretest IPAS than the retrospective pretest ICCAS and greater change in scores on the ICCAS than the IPAS, indicating potential response shift and social desirability biases. Furthermore, we found few significant correlations between change scores in subscales of the two tests, but a high number of strong and significant correlation among the ICCAS subscale change scores. Our results indicate the timing of pretest administration may impact change scores or that subscales of IPAS may be more unique than those of ICCAS. These findings suggest that educators should consider the potential impact of response shift and social desirability biases when interpreting results of the IPAS and ICCAS in response to an IPE learning experience.

Treatment of Pediatric Acute-Onset Neuropsychiatric Disorder in a Large Survey Population.

OBJECTIVE: The goal of this study was to investigate treatment histories and outcomes in a large community sample of youth with Pediatric Acute-onset Neuropsychiatric Syndrome (PANS), and, where appropriate, to examine the impact of immune deficiency on treatment outcomes. METHODS: A comprehensive internet-based survey was completed by parents or guardians of youth who had received physician diagnoses of PANS, or by young adults (age 18+) who had themselves been diagnosed by a physician (N = 698). Data regarding the treatment histories of these patients, including the variety of medical and psychological treatments employed and the caregiver- or self-reported response to each, are presented. RESULTS: The PANS patients in this study had commonly been treated with antibiotic (N = 675), anti-inflammatory (N = 437), and/or psychotropic therapy (N = 378). Response to antibiotic treatment was best when treatment was relatively aggressive, with broad-spectrum antibiotics and courses of >30 days generally producing the best results (i.e., up to 52% of patients achieving a "very effective" response). For immune-deficient patients (caregiver-reported laboratory studies below normal limits; N = 108), use of broad-spectrum antibiotics appeared to be particularly desirable. Anti-inflammatory therapies, including over-the-counter medications such as ibuprofen, were at least "somewhat effective" for most patients. Intravenous immunoglobulin (IVIG) had been used to treat PANS in 193 (28%) of the patients and was at least "somewhat effective" for 89%, although for 18% of these, the effect was not sustained. The highest rate of sustained response to IVIG treatment was seen in immune-deficient patients who received doses of at least 0.8 g/kg IVIG on a regular basis. Psychotropic medications, most commonly SSRIs (38% reported a trial), were commonly employed, but were often ineffective (e.g., 44% found SSRIs "somewhat" to "very effective"). Many patients (N = 473) had received some form of psychotherapy with some benefit, with cognitive behavioral therapy found to be at least somewhat effective in a majority of those treated with this modality. CONCLUSION: Among the PANS patients represented in this study, relatively aggressive treatment courses targeted at eradicating infection and modulating the inflammatory response appeared to provide the best caregiver-reported therapeutic results, and to be generally well tolerated. Given its relative efficacy and tolerability, treatment targeting the inflammatory response may represent an underutilized approach in this population. The results of this study should be considered in light of the limitations inherent in a self-selected and administered online survey.

Impact of PANS and PANDAS Exacerbations on Occupational Performance: A Mixed-Methods Study.

OBJECTIVE: Pediatric acute-onset neuropsychiatric syndrome (PANS) includes pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections and involves sudden-onset neuropsychiatric symptoms, including obsessions, compulsions, sensory difficulties, and dysgraphia after infection or other triggers. Our objective was to identify problems with areas of occupation, body functions, and performance skills during exacerbations. METHOD: In this online retrospective study, based on the Occupational Therapy Practice Framework: Domain and Process (2nd ed.), we surveyed parents of 111 children with PANS. RESULTS: Activities of daily living, math, handwriting, extracurricular activities, free play, organized sports, community and family social participation, higher level thinking, attention, memory, sequencing, emotional coping, and energy and drive were commonly affected during exacerbations. During exacerbations, children often required assistance and adaptation to remain functional or were unable to function at a typical level. CONCLUSION: Children with PANS present with pervasive occupational performance needs during exacerbation. Children and families may benefit from accommodations to maximize function during this turbulent period.

A Survey of Pediatric Acute-Onset Neuropsychiatric Syndrome Characteristics and Course.

OBJECTIVE: To date, studies in the area of pediatric acute-onset neuropsychiatric syndrome (PANS; including pediatric autoimmune neuropsychiatric disorder associated with streptococcal infection [PANDAS] and pediatric infection-triggered neuropsychiatric disorder [PITAND]) have been relatively small and hence unable to comprehensively address questions of disease heterogeneity (e.g., by age, gender), comorbidities, and progression. In this study, we investigated an internet survey sample to more fully characterize the phenotypic traits; medical, family, and developmental history; functional challenges; and clinical course associated with PANS. METHODS: Six hundred and ninety-eight patients with clinical diagnoses of PANS were included in this study. Participants, who included parents and legal guardians (for minors) or the PANS patients themselves (for those ages 18 and older), were asked to complete a 146-question survey designed to ascertain medical, developmental, and family history; PANS symptomatology; medical and nonmedical interventions for PANS; PANS course; PANS outcomes; and access to PANS care. RESULTS: Our results agree with previous findings concerning the core symptoms of PANS as well as its male predominance (65% in this survey) and infection-triggered onset, thus validating the study population. Infection was implicated as the primary inciting factor in 65% of patients; 54% of patients reported an association with group A streptococcus specifically. The results of this survey also revealed new findings, including a surprisingly strong impact of gender and pubertal status on symptom course and chronicity, a high rate of medical comorbidity suggesting generalized immune dysfunction, a profound impact of PANS episodes on functional status, and a role for early resolution of infection through antibiotic treatment in disease course. CONCLUSIONS: This study serves as the first survey of its size to provide insight into the global clinical picture and range of phenotypes of PANS patients. Significant results included the impact of gender and pubertal status on phenotype, affirmation of the role of the immune system in PANS pathology, and the role of timely resolution of infection in clinical outcomes. Understanding how PANS presents in a broad population-based sample, within the limitations of a self-selected and administered online survey, is an important step toward improving diagnosis, creating more targeted treatment options, educating the clinical and research community, and generating hypotheses for future prospective research.

Integrating public health and allied health education through a core curriculum: an action research approach.

This paper describes the use of an action research model to effect curricular change--specifically, to develop a core curriculum that prepares public health and health professions students to meet emerging needs in today's health care environment. The action research process is based on a series of steps wherein the problem is identified, data are collected and interpreted, action is taken, and action is reflected upon. These steps are then repeated. Consensus building proceeds as participants engage in continual analysis and implementation of changes, followed by more analysis. This process led to the emergence of three core content focus areas: Population, Wellness and Disability; Evidence-Based Practice; and Communication and Professionalism. "Focus Area Working Groups" were established to further delineate each content area. These groups initially developed 62 learning objectives across the three focus areas. Those objectives were subsequently distilled and refined, resulting in 25 "Core Essentials" that now define the core curriculum and serve as content guides rather than prescriptive learning objectives. The action research model proved to be beneficial in helping faculty from diverse health disciplines build consensus as they identified common professional and interprofessional learning needs.