Outcomes From a Nurse Practitioner Led Dynamic Compression System Bracing Program for Pectus Carinatum.

BACKGROUND: Dynamic compression system (DCS) is often effective at treating pectus carinatum (PC). However, some patients will fail therapy. This study reports outcomes from a nurse-practitioner led bracing program, and evaluates what factors are predictive of successful therapy. METHODS: We performed a retrospective cohort study involving all patients treated with DCS bracing at our institution between February 2018 and February 2022. Patients with at least three visits were included. The primary outcome was achieving neutral chest. Factors considered potentially predictive included patient age, sex, initial pressure of correction (PIC), and the change in pressure of correction between the first two visits (deltaPC1). A Cox proportional hazards model was used for analysis, and Kaplan-Meier analyses estimated the median time to correction. RESULTS: 283 patients were evaluated. The median age was 14 (IQR 12-15), the majority were male (90.1 %) and white (92.6 %). The median PIC and deltaPC1was 4.13 PSI (IQR 3.17-5.3), and 1.34 PSI (IQR 0.54-2.25), respectively. 117 patients achieved correction. The median estimated time to correction was 7.5 months (95 % CI 5.9-10.1). In the final Cox model, greater deltaPC1 was associated with increased risk of correction (HR: 2.46; 95 % CI 2.03-2.98), and increased PIC was associated with decreased risk of correction up to one year of therapy (0-3 months HR 0.62, 95 % CI 0.50-0.78; 3-12 months HR 0.62; 95 % CI 0.45-0.85). CONCLUSIONS: DCS bracing administered by advanced care providers in collaboration with surgeons can effectively treat PC. The deltaPC1 and PIC are the factors most predictive of successful therapy. LEVEL OF EVIDENCE: Level III.

Modeling of the chest wall response to prolonged bracing in pectus carinatum.

Pectus carinatum is a chest wall deformity that is often treated through the wearing of an external brace. The treatment of the deformity could benefit from a greater understanding of chest wall characteristics under prolonged loading. These characteristics are difficult to model directly but empirical studies can be used to create statistical models. 185 patients from 2018-2020 received bracing treatment. Data on the severity of the deformity, treatment pressures, and time of wear were recorded at the first fitting and all subsequent follow-up visits. This data was analyzed using a statistical mixed effects model to identify significant measures and trends in treatment. These models were designed to help quantify changes in chest wall characteristics through prolonged bracing. Two statistical models were created. The first model predicts the change in the amount of pressure to correct the deformity after bracing for a given time and pressure. The second model predicts the change in pressure response by the body on the brace after bracing for a given time and pressure. These models show a high significance in the amount of pressure and time to the changes in the chest wall response. Initial deformity severity is also significant in changes to the deformity. The statistical models predict general trends in pectus carinatum brace treatment and can assist in creating treatment plans, motivating patient compliance, and can inform the design of future treatment systems.

Design and implementation of a novel patient-centered empowerment approach for pain optimisation in children undergoing major surgery.

BACKGROUND: Paediatric surgery is a stressful experience for patients and caregivers. While standardised protocols are the norm, patient-centred approaches are needed to empower patients/caregivers for an optimal perioperative pain experience. To address this gap, we employed a patient-centred approach using design thinking (DT) methodology to develop insights, map processes, identify opportunities and design solutions for individualised empowerment tools. METHODS: In consultation with DT experts, a multidisciplinary team of stakeholders (healthcare providers, patients who underwent pectus excavatum/scoliosis surgery and their caregivers), were invited to participate in surveys, interviews and focus groups. The project was conducted in two sequential stages each over 24 weeks-involving 7 families in stage 1 and 16 patients/17 caregivers in stage 2. Each stage consisted of three phases: design research (focus groups with key stakeholders to review and apply collective learnings, map processes, stressors, identify influencing factors and opportunities), concept ideation (benchmarking and co-creation of new solutions) and concept refinement. RESULTS: In stage 1, mapping of stress/anxiety peaks identified target intervention times. We identified positive and negative influencers as well as the need for consistent messaging from the healthcare team in our design research. Current educational tools were benchmarked, parent-child engagement dyads determined and healthcare-based technology-based solutions conceived. The 'hero's journey' concept which has been applied to other illness paradigms for motivation successfully the was adapted to describe surgery as a transformative experience. In stage 2, patient and caregiver expectations, distinct personas and responses to perioperative experience were categorised. Educational tools and an empowerment tool kit based on sensorial, thinking, relaxation and activity themes, tailored to parent/child categories were conceptualised. CONCLUSION: DT methodology provided novel family centred insights, enabling design of tailored empowerment toolkits to optimise perioperative experience. Adapting the hero's journey call to adventure may motivate and build resilience among children undergoing surgery.

Standardization of clinical care pathway leads to sustained decreased length of stay following Nuss pectus repair: A multidisciplinary quality improvement initiative.

BACKGROUND AND PURPOSE: Postoperatively, standardized clinical care pathways (SCCPs) help patients reach necessary milestones for discharge. The objective of this study was to achieve 90% compliance with a pectus specific SCCP within 9 months of implementation. We hypothesized that adherence to a pectus SCCP following the Nuss procedure would decrease postsurgical length of stay (LOS). METHODS: A multidisciplinary team implemented the pectus SCCP, including goals for mobility, lung recruitment, pain control, intake, and output. The full protocol included 42 components, tracked using chart reviews and a patient-directed checklist. The primary process measure was compliance with the pectus SCCP. The primary outcome measure was LOS; secondary outcomes were patient charges, patient satisfaction, and hospital readmission. RESULTS: Total study patients were n = 509: 159 patients pre-intervention, 350 patients post-intervention (80 implementation group; 270 sustain group). SCCP compliance data were collected on 164 patients post-intervention - 80 implementation, 84 sustain. LOS, ED visits, and hospital readmissions were recorded for all 509 patients. Mean LOS decreased from 4.5 days to 3.4 days, with >90% adherence to the pectus SCCP postintervention. There were no readmissions owing to pain despite earlier termination of epidural analgesia. Total patient charges decreased by 30% and patient satisfaction was high. CONCLUSION: Using quality improvement methodology with strict adherence to a pectus SCCP, we had significant reduction in LOS and patient charge without compromising effective postoperative pain management or patient satisfaction. TYPE OF STUDY: Clinical research; quality improvement. LEVEL OF EVIDENCE: V.

Human laminin-111 and laminin-211 protein therapy prevents muscle disease progression in an immunodeficient mouse model of LAMA2-CMD.

BACKGROUND: Laminin-α2-related congenital muscular dystrophy (LAMA2-CMD) is a devastating genetic disease caused by mutations in the LAMA2 gene. These mutations result in progressive muscle wasting and inflammation leading to delayed milestones, and reduced lifespan in affected patients. There is currently no cure or treatment for LAMA2-CMD. Preclinical studies have demonstrated that mouse laminin-111 can serve as an effective protein replacement therapy in a mouse model of LAMA2-CMD. METHODS: In this study, we generated a novel immunocompromised dyW mouse model of LAMA2-CMD to study the role the immune system plays in muscle disease progression. We used this immune-deficient dyW mouse model to test the therapeutic benefits of recombinant human laminin-111 and laminin-211 protein therapy on laminin-α2-deficient muscle disease progression. RESULTS: We show that immunodeficient laminin-α2 null mice demonstrate subtle differences in muscle regeneration compared to immunocompetent animals during early disease stages but overall exhibit a comparable muscle disease progression. We found human laminin-111 and laminin-211 could serve as effective protein replacement strategies with mice showing improvements in muscle pathology and function. We observed that human laminin-111 and laminin-211 exhibit differences on satellite and myoblast cell populations and differentially affect muscle repair. CONCLUSIONS: This study describes the generation of a novel immunodeficient mouse model that allows investigation of the role the immune system plays in LAMA2-CMD. This model can be used to assess the therapeutic potential of heterologous therapies that would elicit an immune response. Using this model, we show that recombinant human laminin-111 can serve as effective protein replacement therapy for the treatment of LAMA2-CMD.

Laminin and Integrin in LAMA2-Related Congenital Muscular Dystrophy: From Disease to Therapeutics.

Laminin-α2-related congenital muscular dystrophy (LAMA2-CMD) is a devastating neuromuscular disease caused by mutations in the LAMA2 gene. These mutations result in the complete absence or truncated expression of the laminin-α2 chain. The α2-chain is a major component of the laminin-211 and laminin-221 isoforms, the predominant laminin isoforms in healthy adult skeletal muscle. Mutations in this chain result in progressive skeletal muscle degeneration as early as neonatally. Laminin-211/221 is a ligand for muscle cell receptors integrin-α7ß1 and α-dystroglycan. LAMA2 mutations are correlated with integrin-α7ß1 disruption in skeletal muscle. In this review, we will summarize laminin-211/221 interactions with integrin-α7ß1 in LAMA2-CMD muscle. Additionally, we will summarize recent developments using upregulation of laminin-111 in the sarcolemma of laminin-α2-deficient muscle. We will discuss potential mechanisms of action by which laminin-111 is able to prevent myopathy. These published studies demonstrate that laminin-111 is a disease modifier of LAMA2-CMD through different methods of delivery. Together, these studies show the potential for laminin-111 therapy as a novel paradigm for the treatment of LAMA2-CMD.

Surgical site infection related to use of elastomeric pumps in pectus excavatum repair. Lessons learned from root cause analysis.

BACKGROUND: Pectus excavatum repair (PEX) is among the most painful thoracic procedures performed. Continuous peripheral nerve blockade (CPNB) is known to be efficacious in optimizing pain control while limiting narcotic use in adult thoracic procedures. It was introduced in May 2015 as a bridge to oral pain control in children undergoing PEX. Consequently, the surgical site infection (SSI) rate increased from 2.7% to 27.7%. METHODS: SSI surveillance followed national guidelines. The abrupt increase prompted root cause analysis and cessation of CPNB use. A dynamic systems model of SSI in PEX was developed. Statistical analysis compared SSI outcomes with and without CPNB. RESULTS: From May 2015 to June 2015, 21 PEX were performed; 11 with CPNB. 6 SSIs were observed. Use of CPNB significantly (p=0.008) increased SSI incidence. Haller index, number of bars, usage of Fiberwire®, methicillin resistant S. aureus colonization and length of stay did not differ. Root cause analysis revealed the proximity of CPNB catheters to the wound, the use of CPNB with implanted hardware and a delayed utilization of CPNB catheters to be of concern. CONCLUSION: Introduction of CPNB coincided with a significant increase in SSI. Further study is needed to assess the safety of CPNB in pediatric PEX. LEVEL OF EVIDENCE: Level III treatment study.

Standardization and improvement of care for pediatric patients with perforated appendicitis.

BACKGROUND/PURPOSE: Reduction of treatment variation and application of evidenced based care are increasingly important in the current care environment. Utilizing formal quality improvement methods, an evidenced based guideline was implemented at our institution. METHODS: A guideline was established regarding timing of surgery (immediate vs interval appendectomy) and duration of antibiotics. Twelve months of baseline data were collected prior to implementation. The guideline dictates immediate appendectomy (IA) and postoperative antibiotic therapy until discharge (regular diet, clinically improved, normal complete blood count (CBC)). Data was collected prospectively during hospitalization and at 30days postdischarge. Control charts document adherence to the overall guideline, IA, antibiotic guideline, and readmission for complications. RESULTS: Guideline implementation resulted in an increase in IA (79% vs 94%), decrease in the use of IV antibiotics post discharge (25% to 4%), no change in overall LOS, no change in postoperative abscess formation, and slight decrease in 30day readmission. Charges were decreased. CONCLUSION: Implementation of an evidenced based guideline resulted in significant practice change for managing perforated appendicitis. The changes suggest more efficient care without compromising patient outcome. Utilization of quality improvement methods allows for implementing and tracking the change as well as creating a platform for future improvement.

The material of practice: the Canadian Nursing History Collection.

The Canadian Nursing History Collection is a special holding of over 1500 artifacts at the Canadian Museum of Civilization and the Canadian War Museum. The most significant of its kind, the collection includes uniforms, pins, diaries, instrument kits, and military medals. Researchers and the public will access the collection through an on-line catalogue, a major exhibition, and a book. The material culture of nursing represented by this collection provides nursing historians with a whole new body of evidence for insights into nursing history. The sample of hospital uniforms, from 1900 to the present, for example, raises new and theoretical approaches.