Epilepsy and proxy-reported health-related quality of life in children and young people with non-ambulatory cerebral palsy.

AIM: To assess the association between epilepsy characteristics and proxy-reported health-related quality of life (HRQoL) in children and young people with non-ambulatory cerebral palsy (CP) and seizures. METHOD: This was a cross-sectional study of 164 children and young people (74 females, 90 males; mean age 10 years 6 months, range 2-21 years, SD 5 years 5 months). Caregivers completed the Child Health Index of Life with Disabilities (CPCHILD) in an outpatient setting. We utilized univariable linear regression and multivariable modeling to study relationships between variables and CPCHILD scores. RESULTS: Gross Motor Function Classification System levels were 37% IV and 63% V. Sociodemographic factors included the Child Opportunity Index (median 51, interquartile range [IQR] 25-80). A median of 2 (IQR 1-3) antiseizure medications (ASMs) were used, and days with seizures ranged from 0 (30%) to 28 (20%) days in the previous 4 weeks. Total CPCHILD scores decreased 2.3 points for each ASM (95% confidence interval [CI] -4.1 to -0.42). Compared to persons with focal epilepsy, those with generalized epilepsy had lower total CPCHILD scores (-5.7; 95% CI -11 to -0.55). Number of days with seizures was not associated with total CPCHILD scores. INTERPRETATION: Proxy-reported HRQoL was affected by epilepsy-specific features in children and young people with severe CP. WHAT THIS PAPER ADDS: Health-related quality of life (HRQoL) was lower with increasing numbers of antiseizure medications. Overall quality of life (QoL) scores were lower by a similar amount, independent of seizure frequency. HRQoL was lower in persons with recent hospital admissions for epilepsy.

Hospital resource use after hip reconstruction surgery in children with neurological complex chronic conditions.

AIM: To assess how co-occurring conditions influence recovery after hip reconstruction surgery in children with neurological complex chronic conditions (CCCs). METHOD: This was a retrospective analysis of 4058 children age 4 years or older with neurological CCCs who underwent hip reconstructive surgery between 1st January 2015 and 31st December 2018 in 49 children's hospitals. The presence of co-occurring chronic conditions was assessed using the Agency for Healthcare Research Chronic Condition Indicator system. Multivariable, hierarchical regression was used to assess the relationship between co-existing conditions and postoperative hospital length of stay (LOS), cost, and 30-day readmission rate. RESULTS: The most common co-occurring conditions were digestive (60.1%) and respiratory (37.9%). As the number of co-existing conditions increased from one to four or more, median LOS increased 67% (3d [interquartile range {IQR} 2-4d] to 5d [IQR 3-8d]); median hospital cost increased 41% ($20 248 [IQR $14 921-$27 842] to $28 692 [IQR $19 236-$45 887]); and readmission rates increased 250% (5.5-13.9%), p<0.001 for all. Of all specific co-existing chronic conditions, malnutrition was associated with the greatest increase in postoperative hospital resource use. INTERPRETATION: Co-occurring conditions, and malnutrition in particular, are a significant risk factor for prolonged, in-hospital recovery after hip reconstruction surgery in children with a neurological CCC. Further investigation is necessary to assess how improved preoperative optimization of multiple co-occurring conditions may improve postoperative outcomes and resource utilization. WHAT THIS PAPER ADDS: Children with neurological complex chronic conditions (CCCs) often develop hip disorders which require hip reconstruction surgery. Co-occurring conditions are common in children with neuromuscular CCCs. Having four or more chronic conditions was associated with a longer length of stay, increased costs, and higher odds of readmission. Malnutrition was a significant risk factor for prolonged hospitalization after hip reconstruction surgery.

Puberty and menarche in young females with cerebral palsy and intellectual disability: a qualitative study of caregivers' experiences.

AIM: To explore experiences of parents of young females with cerebral palsy (CP) and intellectual disability at the onset of puberty. METHOD: This was a phenomenological qualitative study. We conducted phone interviews of parents of young females with CP and intellectual disability who had been seen in the CP center at a freestanding children's hospital within the prior 2 years. Inclusion criteria were English-speaking parents of young females who had combined diagnoses of CP and intellectual disability. Interviews were coded and analyzed by the research team facilitated by Dedoose software. RESULTS: Nine interviews were conducted with parents of daughters aged 14 to 24 years. All daughters used wheelchairs for mobility and augmentative technology for communication. Despite homogeneity in functional ability, there was marked variation in parental perception of the significance of puberty for their daughters. Families often learned about reproductive health from informal social networks. Although families acknowledged the need for sexual abuse screening, there was little consensus about how to do it, and most denied that their own daughter could ever be abused. INTERPRETATION: Parents of young females with CP and intellectual disability have diverse reproductive health beliefs that health care providers must explore in order to provide appropriate recommendations for management of puberty. WHAT THIS PAPER ADDS: Parents of young females with cerebral palsy (CP) and intellectual disability have diverse reproductive health beliefs. Approaches to menstrual management in this population must be individualized. Families discounted the likelihood of abuse, despite acknowledging their daughters' risk.

Parent-to-Parent Advice on Considering Spinal Fusion in Children with Neuromuscular Scoliosis.

OBJECTIVES: To convey advice from families whose children recently underwent spinal fusion to families whose children are under consideration for initial spinal fusion for neuromuscular scoliosis and to providers who counsel families on this decision. STUDY DESIGN: We interviewed 18 families of children who underwent spinal fusion between August 2017 and January 2019 at a freestanding children's hospital. We conducted phone interviews a median of 65 (IQR 51-77) days after surgery. We audio recorded, transcribed, and coded (line-by-line) interviews using grounded theory by 2 independent reviewers, and discussed among investigators to induce themes associated with surgical decision making and preparation. RESULTS: Six themes emerged about decision making and preparation for spinal fusion: (1) simplify risks and benefits; it is easy to get lost in the details; (2) families prolonging the decision whether or not to pursue spinal fusion surgery may not benefit the child; (3) anticipate anxiety and fear when making a decision about spinal fusion; (4) realize that your child might experience a large amount of pain; (5) anticipate a long recovery and healing process after spinal fusion; and (6) be engaged and advocate for your child throughout the perioperative spinal fusion process. CONCLUSIONS: Parents of children who had recently undergone spinal fusion had strong perceptions about what information to convey to families considering surgery, which may improve communication between future parents and physicians. Further investigation is needed to assess how best to incorporate the wisdom and experiences of parent peers into shared decision making and preparation for spinal fusion in children with neuromuscular scoliosis.

Associations of Coexisting Conditions with Healthcare Spending for Children with Cerebral Palsy.

OBJECTIVE: To determine which coexisting conditions have the strongest associations with healthcare use and spending among children with cerebral palsy (CP). STUDY DESIGN: Retrospective analysis of 16 695 children ages 0-18 years with CP - identified with International Classification of Diseases, Ninth Revision, Clinical Modification codes - using Medicaid from January 1, 2013 to December 31, 2013 from 10 states in the Truven MarketScan Medicaid Database. Using generalized linear models, we assessed which coexisting conditions (including medical technology) identified with Agency for Healthcare Research and Quality's Chronic Condition Indicators had the strongest associations with total healthcare spending across the healthcare continuum. RESULTS: Median per-patient annual Medicaid spending for children with CP was $12 299 (IQR $4826-$35 582). Most spending went to specialty (33.1%) and hospital (26.7%) care. The children had a median 6 (IQR 4-10) coexisting conditions; epilepsy was the most common (38.1%). Children with epilepsy accounted for 59.6% ($364 million) of all CP spending. In multivariable analysis, the coexisting conditions most strongly associated with increased spending were tracheostomy (median additional cost per patient = $56 567 [95%CI $51 386-61 748]) and enterostomy (median additional cost per patient = $25 707 [95%CI $23 753-27 660]). CONCLUSIONS: Highly prevalent in children with CP using Medicaid, coexisting conditions correlate strongly with healthcare spending. Tracheostomy and enterostomy, which indicate significant functional impairments in breathing and digestion, are associated with the highest spending. Families, providers, payers, and legislators may leverage these findings when designing policies positioned to enable the best health and care for children with cerebral palsy.

Health Services and Health Care Needs Fulfilled by Structured Clinical Programs for Children with Medical Complexity.

OBJECTIVE: To describe family-reported health service needs of children with medical complexity (CMC) and to assess which needs are more often addressed in a tertiary care center-based structured clinical program for CMC. STUDY DESIGN: Mailed survey to families of CMC enrolled in a structured-care program providing care coordination and oversight at 1 of 3 children's hospitals. Outcomes included receipt of 14 specific health service needs. Paired t tests compared unmet health care needs prior to and following program enrollment. RESULTS: Four hundred forty-one of 968 (46%) surveys were returned and analyzed. Respondents reported their children had a mean age of 7 (SD 5) years. A majority of respondents reported the child had developmental delay (79%) and feeding difficulties (64%). Of the respondents, 56% regarded the primary care provider as the primary point of contact for medical issues. Respondents reported an increase in meeting all 14 health services needs after enrollment in a tertiary care center-based structured clinical program, including primary care checkups (82% vs 96%), therapies (78% vs 91%), mental health care (34% vs 58%), respite care (56% vs 75%), and referrals (51% vs 83%) (all P < .001). CONCLUSIONS: Tertiary care center-based structured clinical care programs for CMC may address and fulfill a broad range of health service needs that are not met in the primary care setting.

Children with medical complexity: creating a framework for care based on the International Classification of Functioning, Disability and Health.

Children with medical complexity (CMC) comprise a growing sector of the pediatric population. They are characterized by having severe chronic multiple system conditions, functional limitations, and family needs. While CMC comprise 0.4% to 0.7% of children, they have high healthcare costs and resource utilization, and are thus a key population of interest in health reform efforts. Despite the growth in the development of complex care delivery models, there is little available information to guide the training of diverse professionals in the provision of care that CMC and their families need. We propose a new framework for analysis and discussion based on the components of the International Classification of Function, Disability and Health, with augmentation of further areas of common need identified in this unique population of children. This framework can be used to delineate necessary professional team areas of expertise, guide improvement in care and provider training, and, potentially, to reduce cost as best practices for care are developed and researched.

Examining the Role of Sublingual Atropine for the Treatment of Sialorrhea in Patients with Neurodevelopmental Disabilities: A Retrospective Review.

Sialorrhea is common in children with neurodevelopmental disabilities (NDD) and is reported in >40% of children with cerebral palsy (CP). It causes a range of complications, including significant respiratory morbidity. This single-center retrospective chart review aims to document sublingual atropine (SLA) utilization to guide further study in establishing its role in secretion management for children with NDD. A chart review was completed for patients with NDD ≤ 22 years of age treated with SLA at a free-standing children's hospital between 1 January 2016 and 1 June 2021. Descriptive statistics were generated to summarize findings. In total, 190 patients were identified, of which 178 met inclusion criteria. The average starting dose for SLA was 1.5 mg/day, or 0.09 mg/kg/day when adjusted for patient weight. Eighty-nine (50%) patients were prescribed SLA first line for secretion management while 85 (48%) patients tried glycopyrrolate prior to SLA. SLA was used after salivary Botox, ablation, and/or surgery in 16 (9%) patients. This study investigates SLA as a potential pharmacologic agent to treat sialorrhea in children with NDD. We identify a range of prescribing patterns regarding dosing, schedule, and place in therapy, highlighting the need for further evidence to support and guide its safe and efficacious use.

Reduced Postdischarge Incidents After Implementation of a Hospital-to-Home Transition Intervention for Children With Medical Complexity.

OBJECTIVES: Prior research suggests that errors occur frequently for patients with medical complexity during the hospital-to-home transition. Less is known about effective postdischarge communication strategies for this population. We aimed to assess rates of 30-day (1) postdischarge incidents and (2) readmissions and emergency department (ED) visits before and after implementing a hospital-to-home intervention. METHODS: We conducted a prospective intervention study of children with medical complexity discharged at a children's hospital from April 2018 to March 2020. A multistakeholder team developed a bundled intervention incorporating the I-PASS handoff framework including a postdischarge telephone call, restructured discharge summary, and handoff communication to outpatient providers. The primary outcome measure was rate of postdischarge incidents collected via electronic medical record review and family surveys. Secondary outcomes were 30-day readmissions and ED visits. RESULTS: There were 199 total incidents and the most common were medication related (60%), equipment issues (15%), and delays in scheduling/provision of services (11%). The I-PASS intervention was associated with a 36.4% decrease in the rate of incidents per discharge (1.51 versus 0.95, P = 0.003). There were fewer nonharmful errors and quality issues after intervention (1.27 versus 0.85 per discharge, P = 0.02). The 30-day ED visit rate was significantly lower after intervention (12.6% versus 3.4%, per 100 discharges, P = 0.05). Thirty-day readmissions were 15.8% versus 10.2% postintervention (P = 0.32). CONCLUSIONS: A postdischarge communication intervention for patients with medical complexity was associated with fewer postdischarge incidents and reduced 30-day ED visits. Standardized postdischarge communication may play an important role in improving quality and safety in the transition from hospital-to-home for vulnerable populations.

Preoperative hematocrit and platelet count are associated with blood loss during spinal fusion for children with neuromuscular scoliosis.

AIM: To assess the relationship of preoperative hematology laboratory results with intraoperative estimated blood loss and transfusion volumes during posterior spinal fusion for pediatric neuromuscular scoliosis. METHODS: Retrospective chart review of 179 children with neuromuscular scoliosis undergoing spinal fusion at a tertiary children's hospital between 2012 and 2017. The main outcome measure was estimated blood loss. Secondary outcomes were volumes of packed red blood cells, fresh frozen plasma, and platelets transfused intraoperatively. Independent variables were preoperative blood counts, coagulation studies, and demographic and surgical characteristics. Relationships between estimated blood loss, transfusion volumes, and independent variables were assessed using bivariable analyses. Classification and Regression Trees were used to identify variables most strongly correlated with outcomes. RESULTS: In bivariable analyses, increased estimated blood loss was significantly associated with higher preoperative hematocrit and lower preoperative platelet count but not with abnormal coagulation studies. Preoperative laboratory results were not associated with intraoperative transfusion volumes. In Classification and Regression Trees analysis, binary splits associated with the largest increase in estimated blood loss were hematocrit ≥44% vs. <44% and platelets ≥308 vs. <308 × 109/L. CONCLUSIONS: Preoperative blood counts may identify patients at risk of increased bleeding, though do not predict intraoperative transfusion requirements. Abnormal coagulation studies often prompted preoperative intervention but were not associated with increased intraoperative bleeding or transfusion needs.

Women with cerebral palsy: A qualitative study about their experiences with sexual and reproductive health education and services.

PURPOSE: To explore the recalled experiences of women with CP regarding sexual health education and services they received. METHODS: Semi-structured interviews and focus groups were conducted at four academic tertiary hospitals with 33 adult women with CP. Templates were used to ask about four key content domains: appointment planning (including planning for a gynecologic exam), accessibility of services, experiences with providers, and recommendations for improvement. Sessions were transcribed verbatim and analyzed to generate a coding dictionary. Blinded coding was carried out for each transcript, with duplicate coding used to confirm identified themes. Iterative analysis was used to identify and consolidate coding and key themes. RESULTS: Similar barriers were discussed at the four sites, including lack of accessible exam tables, hospital staff unfamiliar with physical disabilities, and assumptions that women with CP are not sexually active. Many described the sexual education they received as brief, omitted, or mistimed. Self-advocacy was crucial, and recommended strategies ranged from pre-gynecologic exam medication to visit checklists. CONCLUSION: Reproductive health education for young women with CP is frequently inadequate. Medical professionals lack relevant knowledge and awareness; medical facilities lack necessary infrastructure. Recommendations for improvements are made.

Core Curricular Priorities in the Care of Children With Medical Complexity: A North American Modified Delphi Study.

BACKGROUND: Previous studies of pediatric residents have identified educational gaps in caring for children with medical complexity. Training opportunities in complex care vary across residency programs. Defining core curricular topics in complex care is a priority in medical education. OBJECTIVE: To identify core topics to include in a standard complex care curriculum for pediatric residents. METHODS: An initial topic list was generated through literature review and proceedings of national meetings. Expert panelists were identified based on experience in complex care and residency education. A modified Delphi method was used to determine group consensus by asking participants to rate the importance of complex care curricular topics for pediatric residents. Consensus was defined as >70% of experts identifying a topic as essential. There was a predetermined maximum of 3 iterative, electronic survey rounds, with feedback provided to participants between each round. RESULTS: Sixteen experts participated. Response rate was 100% for all rounds. Experts were from the United States (44%) and Canada (56%); most were affiliated with an academic medical center (96%) involved in both inpatient and outpatient care (69%). Eleven topics were identified as essential across 3 rounds: feeding difficulties, pain/irritability, transition, feeding tube management, difficult discussions, team management/care coordination, dysmotility, aspiration, safety/emergency planning, neuromuscular/skeletal issues, and advocacy. Essential topics were organized according to the International Classification of Functioning, Disability and Health. CONCLUSIONS: Eleven curricular priorities in complex care were identified across multiple domains of the International Classification of Functioning, Disability and Health framework, serving as a guide for standardized curriculum development for future pediatricians.

Team Approach: The Perioperative Management of Reconstructive Hip Surgery for the Non-Ambulatory Child with Cerebral Palsy and Spastic Hip Disease.

Cerebral palsy (CP) is the leading cause of physical disability in children. Although physical disability is the hallmark of CP, children with CP also often have complex medical comorbidities. Spastic hip subluxation is the second most common orthopaedic deformity in children with CP, and the incidence increases with the severity of CP. Hip surveillance should be implemented in children with CP to monitor hip stability over time. A reconstructive surgical procedure is recommended when the migration percentage is >40%. Perioperative care of children undergoing a hip reconstructive surgical procedure is a multidisciplinary endeavor requiring the expertise of professionals with different backgrounds. The core team should comprise orthopaedic surgeons, nurses, nurse practitioners, pediatricians, nurse case managers, anesthesiologists, and physical therapists. Additional team members include nutritionists, clinical pharmacists, social workers, child life therapists, and consulting specialty services. This review describes the team approach to the perioperative care of non-ambulatory children with spastic hip subluxation undergoing a reconstructive hip surgical procedure, utilizing a case scenario of a 7-year old girl with CP and complex associated medical comorbidities.

Visiting Jack: Mixed Methods Evaluation of a Virtual Home Visit Curriculum With a Child With Medical Complexity.

BACKGROUND: There are limited training opportunities for pediatricians in caring for children with medical complexity (CMC) in the home and community. Prior studies have described a lack of comfort caring for CMC among pediatric residents. OBJECTIVE: 1) To evaluate the impact of participation in a virtual home visit curriculum on pediatric residents' confidence, knowledge, and application of knowledge in complex care; 2) to explore changes in perspectives relating to the care of CMC after participation in the curriculum. METHODS: This was a prospective pre-post intervention study in 2019 with first-year pediatric residents, using quantitative and qualitative methods. The intervention, co-created with a family partner, was an online video-based curriculum followed by an in-person seminar. Pre- and postassessments were compared using paired t tests. Follow-up interviews and focus groups were performed 5 to 8 weeks after training. Transcripts were analyzed using inductive thematic analysis. RESULTS: Twenty-four residents (100%) participated. Residents reported increased confidence in all aspects of complex care presented in the curriculum, with significant increase in knowledge and application of knowledge (all P < .001). Twelve residents (50%) participated in a follow-up interview or focus group. Four themes were identified: 1) recognizing prior attitudes toward complexity, 2) new mental framework for complex care at home, 3) drivers of behavior change, and 4) commitment to change practice. CONCLUSIONS: Participation in this curriculum was associated with increased confidence, knowledge, and application of knowledge in complex care outside of the hospital. Qualitative findings align with transformative learning theory, lending insight into effective approaches to complex care training.

Parent Perspectives on Short-Term Recovery After Spinal Fusion Surgery in Children With Neuromuscular Scoliosis.

Family perspectives on short-term recovery after spinal fusion for neuromuscular scoliosis are essential for improving patient outcomes. Semistructured interviews were conducted with 18 families of children within 3 months after spinal fusion performed August 2017 to January 2019 at a children's hospital. Interviews were recorded, transcribed, and coded line-by-line by 2 independent reviewers using grounded theory to identify themes. Five themes emerged among families when reflecting back on the postoperative recovery: (1) communicating and making shared decisions regarding postoperative care in a patient- and family-centered manner, (2) setting hospital discharge goals and being ready for discharge, (3) planning for transportation from hospital to home, (4) acquiring supports for caregiving at home after discharge, and (5) anticipating a long recovery at home. Important family perceptions were elicited about the recovery of children from spinal fusion for neuromuscular scoliosis that will inform better perioperative planning for clinicians, future patients, and their families.

Pediatric complex care and surgery comanagement: Preparation for spinal fusion.

The aim of this study is to assess the impact of preoperative comanagement with complex care pediatricians (CCP) on children with neuromuscular scoliosis undergoing spinal fusion. We performed chart review of 79 children aged 5-21 years undergoing spinal fusion 1/2014-6/2016 at a children's hospital, with abstraction of clinical documentation from preoperative health evaluations performed regularly by anesthesiologists and irregularly by a CCP. Preoperative referrals to specialists, labs, tests, and care plans needed last minute for surgical clearance were measured. The mean age at surgery was 14 (SD 3) years; cerebral palsy (64%) was the most common neuromuscular condition. Thirty-nine children (49%) had a preoperative CCP evaluation a median 63 days (interquartile range (IQR) 33-156) before the preanesthesia visit. Children with CCP evaluation had more organ systems affected by coexisting conditions than children without an evaluation (median 11 (IQR 9-12) vs. 8 (IQR 5-11); p < .001). The rate of last-minute care coordination activities required for surgical clearance was lower for children with versus without CCP evaluation (1.8 vs. 3.6). A lower percentage of children with CCP evaluation required last-minute development of new preoperative plans (26% vs. 50%, p = .002). Children with CCP involvement were better prepared for surgery, requiring fewer last-minute care coordination activities for surgical clearance.