Management of Vertebral Defects, Anal Atresia, Cardiac Defects, Tracheoesophageal Fistula or Atresia, Renal Anomalies, and Limb Abnormalities (VACTERL) in a Child With Complex Medical Needs in the Foster Care System: A Pediatric Case Study.

Vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula or atresia, renal anomalies, and limb abnormalities (VACTERL) association is a complex congenital condition characterized by the presence of malformations that affect various organ systems. Most children born with VACTERL association require surgery shortly after birth, often undergoing multiple procedures during infancy, which can lead to a wide range of physical challenges. The unique combination of malformations in these children in addition to having complex care needs that need to be met can result in physical and social difficulties in their daily lives, affecting both their own and their caregivers' quality of life. In some cases, children with complex medical needs are placed in foster care. When children with complex health needs enter the foster care system, there is a risk of overwhelming the caretaker, leading to their needs continuing to be unmet. Pediatricians have a role not only in helping support families but also in knowing what resources are available to meet these needs, which can be dependent on what their communities offer. Pediatricians require current training to navigate their state's foster care system. This training allows pediatricians to effectively collaborate with foster families while also assisting and coordinating complex care to support these families. We present a case of a child with complex health needs placed in the foster care system, facing multiple healthcare challenges, with care delayed due to difficulty attending appointments. Highlighted is the importance of delivering supportive, personalized, and multidisciplinary care to families with children who have complex health needs, including when caretakers are within the foster care system.

The Darker Facets of Systemic Lupus Erythematosus (SLE).

Cerebrovascular events remain a rare but serious feature of systemic lupus erythematosus (SLE). In this report, we see a 25-year-old lady who presented with sudden-onset right-sided weakness and speech disturbances. She was initiated on anti-platelet therapy and glucocorticoids. Her admission was complicated by worsening kidney function due to lupus nephritis. She responded well to immunosuppressant therapy and was discharged following resolution of her symptoms for outpatient specialist follow-up. The rarity of such cases poses a diagnostic and treatment challenge. A language barrier and difficult social circumstances can exacerbate this. However, awareness of neuropsychiatric lupus as a differential diagnosis at the acute assessment of stroke and early involvement of specialist teams, allied health professionals, and safeguarding teams can lead to a successful long-term outcome.

Is Collaborative Care the Future of Medicine? Lessons Learned from the Care of Children with Colorectal Conditions.

The treatment of patients with colorectal disorders requires care from a wide variety of medical and surgical specialties over the course of their lifetime. This is ideally handled by a collaborative center which facilitates the assessment and development of patient care among multiple specialties which can enhance the quality and implementation of treatment plans, improve communication among different specialties, decrease morbidity, and improve patient satisfaction and outcomes. This collaborative approach can serve as a model for other parts of medicine requiring a similar multi-disciplinary and integrated method of care delivery. We describe the process, as well as the lessons learned in developing such a program.

Nationwide Outcomes After Neoadjuvant Chemotherapy for Locally Advanced Sigmoid Colon Cancer-A Propensity Score-Matched Analysis.

BACKGROUND: The role of neoadjuvant chemotherapy (NAC) in advanced sigmoid colon carcinoma remains to be further characterized. Rationale for NAC includes downstaging on final pathology and optimization of microscopically negative margins (R0 resection). We investigated rates of neoadjuvant chemotherapy use in advanced sigmoid colon cancer at academic cancer centers and assessed factors associated with likelihood of NAC administration. METHODS: The National Cancer Database was queried from 2004 to 2017 for patients with clinical T3 or T4, N0-2, M0 sigmoid colon cancer who underwent surgical resection. Those with neoadjuvant radiation or metastatic disease were excluded. The outcomes of patients who did and did not receive neoadjuvant chemotherapy were evaluated for this retrospective cohort study. RESULTS: There were 23,597 patients of whom 364 (1.5%) received NAC. More patients received NAC at academic (41%, P < .001) and high-volume centers (27%, P < .001). Patients with Medicare/Medicaid (39%) and private insurance (52%) were more likely to receive NAC (P < .001). There was a significantly higher rate of N2 to N1 downstaging in the NAC group. Propensity-score matching demonstrated comprehensive community cancer programs (CCCP) were less likely to provide NAC (OR 0.4; 95% CI 0.23, 0.70, P < .001). There was no difference in survival (P = .20), R0 resection (P = .090), or 30-day readmission rates (P = .30) in the NAC cohort compared to the non-NAC cohort. CONCLUSIONS: Access to centers offering multi-disciplinary care with NAC prior to surgical resection is important. This care was associated with academic and high-volume centers and private or government-sponsored insurance. There was no difference in survival between NAC and non-NAC cohort.

A case study of the impact of a dietitian in the multi-disciplinary team within primary care: a service evaluation.

BACKGROUND: Expanding the primary care workforce to alleviate general practitioner (GP) workload, improve access and improve quality of care is a current UK strategy. Evidence suggests dietitians can improve patient outcomes and make cost savings. The present study aimed to evaluate a dietitian working as an expert generalist and first contact practitioner (FCP) in a general practice multi-disciplinary team (MDT) to provide appropriate care to patients and reduce GP workload. METHODS: A dietitian was employed for 6 months at 0.6 full-time equivalents in a group of general practices in Devon, UK. Data were collected on the referral source, patient satisfaction, health outcomes and changes in prescribing data for all patients seen by the dietitian. Focus groups and interviews provided data to understand the experience of introducing a dietitian into the team. RESULTS: This model of service delivery showed the dietitian acting as an expert generalist, a FCP and able to educate the MDT. A range of professionals within the MDT referred patients with a wide range of diagnoses (both paediatric and adults) and the dietitian acted as a FCP for 29% of patients. Saving were made for the optimisation of medicine management. CONCLUSIONS: The dietitian can improve patient-centred care for several patient groups; enhance learning for staff around nutrition and dietary issues; and contribute to more efficient working and cost savings around prescription of nutritional products. This was an evaluation of one service and further research is needed to understand the value dietitians can contribute and the factors supporting effective and efficient working in this context.

Perioperative Management of Emergency Craniotomes in Children With Cyanotic Congenital Heart Disease: A Case Series.

While congenital heart disease is not uncommon, cyanotic congenital heart disease (CCHD) accounts for a minor fraction of them. However, when cyanosis is present, it usually indicates a severe or critical illness. Tetralogy of Fallot (TOF) is one of the common CCHDs, representing 7-10% of all congenital cardiac malformations. Double-outlet right ventricle (DORV) is another CCHD similar to the TOF and associated with decreased pulmonary flow, ventricular septal defect (VSD), and aorta receiving blood from both ventricles. Reduced oxygen arterial saturation and increased viscosity by polycythemia induce focal cerebral ischemia, often in the area supplied by the middle cerebral artery leading to brain abscess. Brain abscesses require craniotomy, which is a major surgery. These patients also often show features of sepsis and increased intracranial pressure. The presence of CCHD further complicates the situation, making perioperative management even more challenging. There are studies in the literature on the management of similar cases, and they report successful management in most of them. However, not all such cases need intensive postoperative management. We present four pediatric cases who had either TOF or DORV and had to undergo craniotomy for brain abscess or ventriculoperitoneal shunt placement. We describe case management and highlight the critical features and cases that require prolonged postoperative critical care management.

Patient driven change: Is collaborative care the future of medicine? Lessons learned from the care of children with colorectal problems.

A basic premise in the care of complex patients is that experience, increased volume of cases, and an integrated, multi-disciplinary approach yields improved outcomes. Is this true using the example of the care of children with colorectal and pelvic reconstructive needs? This review gives a brief historical context on how care for this patient group evolved, delineates the key elements to create a collaborative care model, and describes multiple advances that have been developed, based on the model, which have improved patient care and quality of life. LEVEL OF EVIDENCE: Review.

Usability of mixed reality in awake craniotomy planning.

PURPOSE: This study aimed to describe our institutional use of a commercially available mixed reality viewer within a multi-disciplinary planning workflow for awake craniotomy surgery and to report an assessment of its usability. MATERIALS AND METHODS: Three Tesla MRI scans, including 32-direction diffusion tensor sequences, were reconstructed with BrainLab Elements auto-segmentation software. Magic Leap mixed reality viewer headsets were registered to a shared virtual viewing space to display image reconstructions. System Usability Scale was used to assess the usability of the mixed reality system. RESULTS: The awake craniotomy planning workflow utilises the mixed reality viewer to facilitate a stepwise discussion through four progressive anatomical layers; the skin, cerebral cortex, subcortical white matter tracts and tumour with surrounding vasculature. At each stage relevant members of the multi-disciplinary team review key operative considerations, including patient positioning, cortical and subcortical speech mapping protocols and surgical approaches to the tumour.The mixed reality system was used for multi-disciplinary awake craniotomy planning in 10 consecutive procedures over a 5-month period. Ten participants (2 Anaesthetists, 5 Neurosurgical trainees, 2 Speech therapists, 1 Neuropsychologist) completed System Usability Scale assessments, reporting a mean score of 71.5. Feedback highlighted the benefit of being able to rehearse important steps in the procedure, including patient positioning and anaesthetic access and visualising the testing protocol for cortical and subcortical speech mapping. CONCLUSIONS: This study supports the use of mixed reality for multidisciplinary planning for awake craniotomy surgery, with an acceptable degree of usability of the interface. We highlight the need to consider the requirements of non-technical, non-neurosurgical team members when involving mixed reality activities.

Pharmacist knowledge of gout management: impact of an educational intervention.

BACKGROUND: Pharmacists play a key role in community gout education. We investigated pharmacist knowledge of gout management and developed an educational intervention which was assessed in a cohort of Irish pharmacists. METHODS: A ten-question questionnaire about gout management was developed to assess pharmacists' knowledge. A 14 min 26 s video educational intervention was co-designed by a rheumatologist, a pharmacist, and designer of pharmacy education resources. The effectiveness of this pharmacy-specific intervention was assessed using the same questionnaire in 53 pharmacists (25 in the intervention group; 28 in the control group). Contingency tables were used to analyse differences between groups. RESULTS: There were 173 pharmacist respondents to the initial survey; 35.3% answered that first-line therapy for gout involves a combination of a xanthine oxidase inhibitor (e.g., allopurinol) combined with a prophylactic agent (e.g., colchicine), and 28.9% of respondents answered that colchicine prophylaxis should be used when initiating urate-lowering therapy. Following the educational intervention, pharmacist's knowledge about gout management increased across many domains, including serum urate targets when using urate-lowering therapy (p = 0.006), use of colchicine prophylaxis (p = 0.011), and duration of colchicine use (p < 0.001). CONCLUSION: Gout management recommendations can be impeded if translation into pharmacy practice is neglected. Pharmacists are a valuable information resource for patients. Co-designing a brief education intervention with pharmacists is an effective, low-cost way to increase pharmacist knowledge on the management of gout.

The Diagnosis of Common Variable Immunodeficiency After Multisystem Dysfunction.

Common variable immunodeficiency (CVID) is a primary immunodeficiency caused by the lack of B cell differentiation into plasma cells, thereby leading to decreased serum immunoglobulins. Patients with this condition are predisposed to recurrent infections and are more likely to develop certain cancers and autoimmune diseases. We report the case of a 53-year-old female suffering from recurrent pulmonary infections and a history of non-Hodgkin lymphoma (NHL) who had a poor response to the measles, mumps, and rubella (MMR) and varicella vaccines as a child, and was infected with coronavirus disease 2019 (COVID-19) twice in 2020. Testing of her antibody titers in order to determine suitability for Streptococcus pneumoniae (S. pneumoniae) vaccination found an overall decrease in major immunoglobulin classes (IgG, IgM, and IgA) and B cells with normal morphology. The diagnosis of CVID was made, and prompt treatment with intravenous immunoglobulins (IVIG) brought her IgG levels up from 282 to 680 mg/dL within three months. This case highlights the importance for providers to keep immunological dysfunction on their differentials for patients with atypical presentations involving multiple organ systems.

Developing and delivering high quality surgery into community in LMIC: Experience in Kerala, India.

The road to the delivery of high-quality cleft surgery into communities in the LMIC is beset with numerous hurdles. These include the availability of trained health care personnel, the availability of infrastructure in health care, parent awareness of the need for timely interventions in these children, presence of affordable treatment centres with adequate means of transport to and from these facilities for patients coming from the interior rural locations, but most importantly, the financial considerations that are the underlying limitations in most of these above mentioned considerations. We would like to approach this problem by describing the evolution of our own centre over several decades. Our centre, The Charles Pinto Centre for Cleft lip and palate at the Jubilee Mission Hospital is located in Thrissur, in the beautiful state of Kerala. In the late 1950s, Dr HS Adenwalla who had trained under experts in the art of Cleft Lip and Palate Surgery in Mumbai joined the hospital and transformed it through sheer dedication and meticulous clinical expertise into a major hub for treatment and also training in this speciality. In this endeavour he was greatly helped by non governmental organizations of which Smile Train deserves special mention in view of its model of functioning, empowering local partners by adequate funding for treatment, training and establishment of infrastructure, while ensuring strict adherence to safety standards. We believe that it is essential that there should be trained and dedicated health care personnel who are willing to treat such patients without an eye on the monetary benefits accrued, and also enough funding either from the government or from the NGOs to provide equipment and infrastructure for safe, high quality and accessible treatment and the establishment of multidisciplinary management. Lastly, the patients should also be made aware of the need for timely treatment and long-term follow- up.

Adult North Star Network (ANSN): Consensus Guideline For The Standard Of Care Of Adults With Duchenne Muscular Dystrophy.

There are growing numbers of adults with Duchenne Muscular Dystrophy living well into their fourth decade. These patients have complex medical needs that to date have not been addressed in the International standards of care. We sought to create a consensus based standard of care through a series of multi-disciplinary workshops with specialists from a wide range of clinical areas: Neurology, Cardiology, Respiratory Medicine, Gastroenterology, Endocrinology, Palliative Care Medicine, Rehabilitation, Renal, Anaesthetics and Clinical Psychology. Detailed reports of evidence reviewed and the consensus building process were produced following each workshop and condensed into this final document which was approved by all members of the Adult North Star Network including service users. The aim of this document is to provide a framework to improve clinical services and multi-disciplinary care for adults living with Duchenne Muscular Dystrophy.

Identifying errors and safety considerations in patients undergoing thrombolysis for acute ischemic stroke.

BACKGROUND & PURPOSE: Alteplase is the standard of care for early pharmacologic thrombolysis after acute ischemic stroke (AIS). Alteplase is also considered a high-alert medication and is fraught with potential for error. We sought to describe the difference in medication error rates in in patients receiving alteplase for acute ischemic stroke from regional hospitals compared to patients receiving alteplase at the Comprehensive Stroke Center. METHODS: This was a retrospective cohort comparison of patients who were greater than 18 years old that received intravenous alteplase for the treatment of AIS from June 2015 to June 2018. Several institution specific databases were utilized to obtain pertinent data. A standardized taxonomy was utilized to classify medication errors. Patients were excluded if they received any fibrinolytic other than alteplase or if alteplase was used for a non-stroke indication. Two cohorts (from regional hospitals or the Comprehensive Stroke Center (CSC)) were compared. RESULTS: A total of 676 patients received alteplase during the study period (34% from the CSC and 66% from regional hospitals). There were 133 (19.8%) errors identified. Ten errors (1.6%) occurred at the CSC and 123 (18.2%) errors occurred at regional hospitals. More patients who had an error with alteplase administration (12.7%) experienced a hemorrhagic conversion compared to those with no error in administration (7.2%, p= 0.04). CONCLUSION: The error rate of alteplase infusion for ischemic stroke is high, particularly in patients from referring centers. Errors may be associated with adverse events. Further education and administration safeguards should be implemented to decrease the risk of medication errors.

Implementation of Person-Centered Care: A Feasibility Study Using the WE-CARE Roadmap.

BACKGROUND: Person-Centered Care (PCC) is a promising approach towards improved quality of care and cost containment within health systems. It has been evaluated in Sweden and England. This feasibility study examines initial PCC implementation in a rehabilitation hospital for children in Poland. METHODS: The WE-CARE Roadmap of enablers was used to guide implementation of PCC for patients with moderate scoliosis. A multi-disciplinary team of professionals were trained in the PCC approach and the hospital Information Technology (IT) system was modified to enhance PCC data capture. Semi-structured interviews were conducted with the nine health care professionals involved in the pilot study and three patients/parents receiving care. Transcribed data were analyzed via content analysis. RESULTS: 51 patients and their families were treated via a PCC approach. High proportions of new PCC data fields were completed by the professionals. The professionals were able to implement the three core PCC routines and perceived benefits using the PCC approach. Patients and their families also perceived improved quality care. The WE-CARE framework enablers facilitated PCC implementation in this setting. CONCLUSIONS: This feasibility pilot study indicates that the Gothenburg PCC approach can be successfully transferred to a rehabilitation hospital in Poland with favorable perceptions of implementation by both professionals and patients/their families.

Identification of Risk Factors for Coexisting Sinusitis and Inflammatory Bowel Disease.

BACKGROUND: This study aimed to analyze the association of coexisting sinusitis and IBD, establish significant factors involved in their development, and enable further biological correlation between these two diseases. METHODS: The IBD and Sinusitis Study at UChicago Medicine (TISSUe) is a retrospective, single-center study. We reviewed patients to confirm IBD and chronic sinusitis diagnoses. Case-control propensity score matching was performed using matched controls with IBD only or sinusitis only. Statistical methods included Chi-squared test and Wilcoxon rank sum test. Logistic regression analysis was performed, and factors were considered significant if p<0.05. RESULTS: Stratifying 214 patients with coexisting IBD and sinusitis, 176 patients had IBD first and 38 patients had sinusitis first. Multivariable analysis of factors associated with subsequent disease with matched controls determined that duration of disease, UC, steroid exposure ever, and younger age of IBD diagnosis were associated with subsequent sinusitis in patients with IBD; steroid exposure ever and duration of sinusitis were significantly associated with subsequent IBD in patients with sinusitis. CONCLUSIONS: This study suggests that IBD maintenance therapies are not associated with increased risk of sinusitis, as proposed by adverse events in clinical trial data; rather, UC diagnosis and duration of disease may be more influential in sinusitis development. While further studies are necessary, this study also demonstrates that sinusitis precedes IBD in some patients, probing its biological association with IBD and possible classification as an extraintestinal manifestation.

The Oncology and Sexuality, Intimacy, and Survivorship Program Model: An Integrated, Multi-disciplinary Model of Sexual Health Care within Oncology.

Cancer-related sexual dysfunction is documented as one of the most distressing and long-lasting survivorship concerns of cancer patients. Canadian cancer patients routinely report sexuality concerns and difficulty getting help. In response to this gap in care, clinical practice guidelines were recently published in the Journal of Clinical Oncology. A sweeping trend is the creation of specialized clinics for patients' sexual health concerns. However, this much-needed attempt to address this service gap can be difficult to sustain without addressing the cancer care system from a broader perspective. Herein, we describe the implementation of a tiered systemic model of cancer-related sexual health programming in a tertiary cancer center. This program follows the Permission, Limited Information, Specific Suggestions, Intensive Therapy (PLISSIT) model, used previously for guiding individual practitioners. Visually, the model resembles a pyramid. The top 2 levels, corresponding to Intensive Therapy and Specific Suggestions, are comprised of group-based interventions for common cancer-related sexual concerns and a multi-disciplinary clinic for patients with complex concerns. The bottom 2 levels, corresponding to Permission and Limited Information, consist of patient education and provider education and consultation services. We describe lessons learned during the development and implementation of this program, including the necessity for group-based services to prevent inundation of referrals to the specialized clinic, and the observation that creating specialized resources also increased the likelihood that providers would inquire about patients' sexual concerns. Such lessons suggest that successful sexual health programming requires services from a systemic approach to increase sustainability.

Building a Parkinson-Network-Experiences from Germany.

Parkinson's disease is a complex neurodegenerative disease that can be best treated with a multi-disciplinary care approach. Building care networks has been shown as a useful tool to facilitate the integration of care services and improve outcomes for patients and care providers. However, experiences and practices relating to building a network are very limited in the field of Parkinson's disease. This paper portrays existing Parkinson networks in Germany. With the help of a standardized template, description of networks and their building-blocks, so-called modules, were collected from all over Germany. Modules were rated in terms of their expected benefit and the required effort when implementing them, with the help of an expert survey. The rating showed that some modules were perceived as more important than others, but all modules were recognized as beneficial for patients and care providers. Overall, the German experience shows that building a Parkinson network facilitates the integration of care and provides a benefit to all stakeholders involved.

A double dilemma: treatment of stage IV fetal twin-twin transfusion syndrome in the setting of maternal recurrent venous thromoembolism: a case report.

BACKGROUND: Fetal conditions can pose significant challenges in the management of pregnancies complicated by pre-existing maternal medical conditions. CASE PRESENTATION: We report a case of a 34-year-old woman with Stage IV Twin Twin Transfusion syndrome in the presence of maternal recurrent complex venous thromboembolic disease. Following a previous pregnancy loss, complicated by a third episode of thromboembolic disease, an inferior vena cava filter was placed. One month later, a pregnancy was confirmed and subsequently identified as a monochorionic twin pregnancy. Twin-Twin Transfusion syndrome was identified at 18 weeks' gestation and progressed rapidly to Quintero Stage IV. In consultation with a multi-disciplinary international team, fetoscopic laser photocoagulation was performed. The pregnancy progressed to delivery of female infants at 33 weeks gestation, who have achieved all developmental milestones at 2 years of age. CONCLUSIONS: We describe the multi-disciplinary effort to optimise the maternal condition to allow fetoscopic laser photocoagulation and continued management of the maternal and fetal conditions to a successful pregnancy outcome.

Waiting it out: consultation delays prolong in-patient length of stay.

BACKGROUND: Decreasing delays for hospitalised patients results in improved hospital efficiency, increased quality of care and decreased healthcare expenditures. Delays in subspecialty consultations and procedures can cause increased length of stay due to reasons outside of necessary medical care. OBJECTIVE: To quantify, describe and record reasons for delays in consultations and procedures for patients on the general medicine wards. METHODOLOGY: We conducted weekly audits of all admitted patients on five Internal Medicine teams over 8 weeks. A survey was reviewed with attending physicians and residents on five internal medicine teams to identify patients with a delay due to consultation or procedure, quantify length of delay and record reason for delay. RESULTS: During the study period, 316 patients were reviewed and 48 were identified as experiencing a total of 53 delays due to consultations or procedures. The average delay was 1.8 days for a combined total of 83 days. Top reasons for delays included scheduling, late response to page and a busy service. The frequency in length of consult delays vary among different specialties. The highest frequency of delays was clustered in procedure-heavy specialties. CONCLUSION: This report highlights the importance of reviewing system barriers that lead to delayed service in hospitals. Addressing these delays could lead to reductions in length of stay for inpatients.

How Can We Improve Disease Education in People with Gout?

PURPOSE OF REVIEW: Gout management is currently suboptimal despite excellent available therapy. Gout patient education has been shown to enhance medication adherence and self-management, but needs improvement. We explored the literature on gout patient education including gaps in gout patient knowledge; use of written materials; in-person individual and group sessions; education via nurses, pharmacists, or multi-disciplinary groups; and use of phone, web-based, mobile health app, and text messaging educational efforts. RECENT FINDINGS: Nurse-led interventions have shown significant improvement in reaching urate goals. Pharmacist-led programs have likewise succeeded, but to a lesser degree. A multi-disciplinary approach has shown feasibility. Needs-assessments, patient questionnaires, and psychosocial evaluations can enhance targeted education. An interactive and patient-centered approach can enhance gout educational interventions. Optimal programs will assess for and address educational needs related to knowledge gaps, health literacy, race, gender, socio-economic status, and level of social support.