Quality Assurance Standards for Outreach Cleft Lip and Cleft Palate Repair Programs in Low-Resource Settings.

BACKGROUND: Comprehensive cleft care is a multidisciplinary team endeavor. While untreated craniofacial conditions have multiple undue repercussions, cleft care in outreach settings can be fraught with significant perioperative morbidity risks. AIM: Propose updated quality assurance standards addressing logistic and operational considerations essential for the delivery of safe and effective cleft lip and /or palate (CL/P) care in low and middle-income countries (LMICs) settings. METHODS: Based on American Cleft Palate-Craniofacial Association (ACPA) quality standards, published literature, published protocols by Global Smile Foundation (GSF), and the senior author's three-decade experience, updated standards for outreach cleft care were synthesized. RESULTS: Ten axes for safe, effective, and sustainable cleft lip and palate care delivery in underserved settings were generated: 1) site assessment, 2) establishment of community partnerships, 3) team composition and credentialing, 4) team training and mission preparation, 5) implementation of quality assurance guidelines, operative safety checklists, and emergency response protocols, 6) immediate and long-term postoperative care, 7) medical record keeping, 8) outcomes evaluation, 9) education, and 10) capacity building and sustainability. Subsequent analysis further characterized essential components of each of those ten axes to delineate experience derived and evidence-based recommendations. DISCUSSION: Quality assurance guidelines are essential for the safe delivery of comprehensive cleft care to patients with CL/P in any setting. Properly designed surgical outreach programs relying on honest community partnerships can be effectively used as vehicles for local capacity building and the establishment of sustainable cleft care ecosystems.

Comprehensive Cleft Care Delivery in Developing Countries: Impact of Geographic and Demographic Factors.

INTRODUCTION: The authors analyzed the insights of participants and faculty members of Global Smile Foundation's Comprehensive Cleft Care Workshops concerning the barriers and interventions to multidisciplinary cleft care delivery, after stratification based on demographic and geographic factors. METHODS: During 2 simulation-based Comprehensive Cleft Care Workshops organized by Global Smile Foundation, participants and faculty members filled a survey. Surveys included demographic and geographic data and investigated the most relevant barrier to multidisciplinary cleft care and the most significant intervention to deliver comprehensive cleft care in outreach settings, as perceived by participants. RESULTS: The total response rate was 57.8%. Respondents reported that the greatest barrier to comprehensive cleft care was financial, and the most relevant intervention to deliver multidisciplinary cleft care was building multidisciplinary teams. Stratification by age, gender, and geographical area showed no statistical difference in reporting that the greatest barrier to cleft care was financial. However, lack of multidisciplinary teams was the most important barrier according to respondents with less than 5 years of experience (P = 0.03). Stratification by gender, years in practice, specialty, and geographical area showed no statistical difference, with building multidisciplinary teams reported as the most significant intervention. However, increased training was reported as the main intervention to cleft care for those aged less than 30 years old (P = 0.04). CONCLUSIONS: Our study delivers an assessment for barriers facing multidisciplinary cleft care delivery and interventions required to improve cleft care delivery. The authors are hoping that stratification by demographic and geographic factors will help them delineate community-specific road maps to refine cleft care delivery.

Sustainable Cleft Care: A Comprehensive Model Based on the Global Smile Foundation Experience.

INTRODUCTION: Clefts of the lip and palate are leading congenital facial anomalies. Underserved patients with these facial differences lack access to medical care, surgical expertise, prenatal care, or psychological support. Moreover, the disease results in significant economic strains on patients and their families. While surgical outreach programs have attempted to fill this void, significant challenges facing international comprehensive cleft care persist. OBJECTIVE: Propose a path toward international sustainable cleft care based on the Global Smile Foundation experience. RESULTS: International sustainable comprehensive cleft care can be achieved by regulating surgical outreach programs. Regulation of these missions would ensure standardized care and encourage stakeholders to cooperate and adequately allocate funding and resources. Capacity building can be achieved through "diagonal" cleft care delivery models, multidisciplinary workshops, fellowship programs, research and quality assurance, as well as leveraging emerging technologies such as Augmented Reality. CONCLUSION: International comprehensive cleft care requires continuous collaborative efforts between visiting and local teams as well as international and national organizations. Standardizing and regulating current practices as well as promoting capacity building initiatives can contribute to sustainable cleft care.

World Cleft Coalition International Treatment Program Standards.

OBJECTIVE: To present preliminary efforts to establish an internationally agreed set of minimum core practice and best practice guidelines, along with overarching principles to promote safe and comprehensive cleft care globally. DESIGN: Representatives from 6 national and international organizations collaborated to form a World Cleft Coalition. Representatives met monthly/bimonthly to compile standards for safe, comprehensive, and sustainable cleft care. Outcomes were circulated within each organization and to a small subset of external constituents for feedback. RESULTS: A series of overarching principles were established for those involved in International Cleft Treatment Programs, based on the experience of participating organizations. The overarching principles are followed by a structured and detailed Recommended Practice for Ensuring Safe, Comprehensive and Sustainable Cleft Care, which includes minimum core and best practice for the following areas: surgical safety, quality control, patient education, patient selection, patient follow-up, comprehensive care, partnership with the host nations and professionals, training and exchanges for sustainability, and local capacity building. CONCLUSIONS: Outcomes aimed to provide a working document to define core principles for safe comprehensive cleft care, while balancing various levels of resources, geographic locations, appropriately trained health care professional specialists, and training limitations. The study highlights the process and benefits to a collaborative international working group not only to establish best practice but also to solicit and engage others in discussion of their experiences with building and supporting safe, high-quality, comprehensive, sustainable, worldwide cleft care.

Differential regulation of Ca2+ influx by ORAI channels mediates enamel mineralization.

Store-operated Ca2+ entry (SOCE) channels are highly selective Ca2+ channels activated by the endoplasmic reticulum (ER) sensors STIM1 and STIM2. Their direct interaction with the pore-forming plasma membrane ORAI proteins (ORAI1, ORAI2, and ORAI3) leads to sustained Ca2+ fluxes that are critical for many cellular functions. Mutations in the human ORAI1 gene result in immunodeficiency, anhidrotic ectodermal dysplasia, and enamel defects. In our investigation of the role of ORAI proteins in enamel, we identified enamel defects in a patient with an ORAI1 null mutation. Targeted deletion of the Orai1 gene in mice showed enamel defects and reduced SOCE in isolated enamel cells. However, Orai2-/- mice showed normal enamel despite having increased SOCE in the enamel cells. Knockdown experiments in the enamel cell line LS8 suggested that ORAI2 and ORAI3 modulated ORAI1 function, with ORAI1 and ORAI2 being the main contributors to SOCE. ORAI1-deficient LS8 cells showed altered mitochondrial respiration with increased oxygen consumption rate and ATP, which was associated with altered redox status and enhanced ER Ca2+ uptake, likely due to S-glutathionylation of SERCA pumps. Our findings demonstrate an important role of ORAI1 in Ca2+ influx in enamel cells and establish a link between SOCE, mitochondrial function, and redox homeostasis.