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Background: Phenylketonuria (PKU) is an inherited autosomal recessive disorder caused by variants in the PAH gene which encodes for phenylalanine hydroxylase (PAH). PKU, left untreated, can cause intellectual disability, psychiatric symptoms, and behavioral abnormalities. Lifelong management of PKU is challenging, and many adult patients become lost to follow-up, despite recommendations for lifelong management. Method(s): The PKU clinic team is a multidisciplinary team consisting of an APN Director, physician, dieticians, diet tech, genetic counselor, registered nurse, and social worker. After establishing formal guidelines, algorithms were created to determine thresholds for initiating patient outreach based on both age and type of PKU treatment. EMRbased data collection is used to track adherence to both clinic visits and consistent submission of Phe levels. Data was collected and analyzed for roughly 250 PKU patients. Baseline levels for adherence to clinic visits and filter card submission were collected at time of implementation. Data was then reviewed after 18 months, and has been further analyzed for a second 18 months (which correlates with the start of the COVID-19 pandemic) Results: Overall baseline adherence across the population for annual clinic visitswas 72% (144/200). Clinic visit adherence increased to 88% at 18 months, and then was similar at 86% through COVID-19 pandemic. In the adult population, 54% (57/106) were adherent at baseline with clinic follow-up. With implementation compliance increased to 80% initially and was then reported to be 74% during the pandemic. Baseline for all PKU patients showed 81% (161/200) filter card submission within the last 12 months. Submission increased to 91% after 18 months of overdue outreach, and as of October 2021, 85% of all patients had submitted a filter card within the last calendar year. Adult patients specifically showed an increase, with 53% at baseline to 69% after implementation. Conclusion(s): Implementation of an overdue outreach program is successful in re-engaging patients with the PKU clinic and improving adherence to treatment recommendations. We have seen increased patient adherence across all domains and have maintained this improved adherence despite the global COVID-19 pandemic.Copyright © 2022 Elsevier Inc. All rights reserved.
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Introduction: The onset of the SARS-CoV-2 pandemic alerted the Philippine government to impose the enhanced community quarantine (ECQ) as a means to hamper human mobility and interaction and eventually diminish transmission. Due to severe limitations in accessibility to basic needs due to ECQ, the government devised amelioration programs. A year after the declaration of the SARS-CoV-2 pandemic, variants of concern were detected locally. Consequently, there is a necessity to prepare reinstatement of strict non-pharmaceutical interventions while meeting the food-related basic needs of the population. Studies related to food distribution during a strict community quarantine have been lacking. The importance of allocating provisions during extreme pandemic measures should be properly analyzed, especially when attempts had been made by local government units. Methods: This study devised an agent-based model (ABM) to observe the effects of the food relief system in mitigating the disease during Davao City ECQ when two variants are present in two adjacent villages. These relief distribution types are as follows: "regular and sufficient,” "regular but insufficient,” and "irregular” relief type. In total, three barangay scenarios were considered. Results and discussion: For the worst-case scenario, wherein a lot of infections are anticipated, the results show that the "irregular” relief type peaked at the highest number of cases, while the "regular and sufficient” relief type showed little to almost no new cases. The compromise-case scenario showed almost no difference between "regular but insufficient” and "regular and sufficient.” For the best-case scenario, the three relief types showed low average infected cases with almost small variance. The model was then compared, situationally, with Davao City barangays during ECQ and recommended which food relief type applies to the barangays. This could serve as a baseline on how food reliefs could be optimally distributed in cases where barangay conditions differently affect and transmit the SARS-CoV-2 virus of different variants with varying transmission rates within a community. Further development of the model should potentially be useful for decision support not only during pandemics but also in contexts where resource allocation to a community is involved. Copyright © 2023 Yap, Lachica, Paras, Panogalinog, Tubay and Mata.
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Introduction: Phenylketonuria (PKU) is an inherited autosomal recessive disorder caused by variants in the PAH gene which encodes for phenylalanine hydroxylase (PAH). PAH deficiency leads to phenylalanine (Phe) accumulation, which untreated can cause intellectual disability, microcephaly, delayed speech, seizures, psychiatric symptoms, and behavioral abnormalities. Early detection of elevated Phe through newborn screening allows for rapid initiation of a Phe-restricted diet to prevent severe neurological outcomes;however, suboptimal Phe control throughout the lifespan is associated with increased rates of psychiatric illness and deficits in executive function even in early treated patients. Lifelong management of PKU is challenging, and it is well documented that many adult patients become lost to follow-up, despite the American College of Medical Genetics recommendation for lifelong management. Here we describe and evaluate efforts to improve follow-up care for patients with PKU of all ages at one center through formalization of clinic guidelines and creation of an overdue outreach program. Methods: The PKU clinic team is a multidisciplinary team consisting of an APN Director, physician, dieticians, diet tech, genetic counselor, registered nurse, and social worker. Regular meetings were scheduled with all clinic staff members to review PKU treatment guidelines, recommended lab monitoring, and visit frequency. After establishing formal guidelines, algorithms were created to determine thresholds for initiating patient outreach based on both age and type of PKU treatment. EMR-based data collection is used to track adherence to both clinic visits and consistent submission of Phe levels. Data was collected and analyzed for Lurie Children’s PKU program, which consists of roughly 250 patients. Baseline levels for adherence to clinic visits and filter card submission were collected at time of implementation. Data was then collected and analyzed initially after 18 months, and has been further analyzed for a second 18 months (which correlates with the start of the COVID-19 pandemic). Results: Overall baseline adherence across the PKU patient population for annual clinic visits was 72% (144/200). Clinic visit adherence increased to 88% at 18 months, and then was essentially unchanged at 86% through COVID-19 pandemic. In the pediatric patient population, annual clinic follow-up adherence was 92% (79/86) at baseline, which increased to 98% with implementation and maintained 98% during the pandemic. In the adult patient population, 54% (57/106) were adherent at baseline with clinic follow-up. With implementation compliance increased to 80% initially and was then reported to be 74% during the pandemic. Baseline for all PKU patients showed 81% (161/200) filter card submission within the last 12 months. Submission increased to 91% after 18 months of overdue outreach, and as of October 2021, 85% of all patients had submitted a filter card within the last calendar year. Adult patients specifically showed an increase, with 53% at baseline to 69% after implementation. Hyperphenylalaninemia (hyperphe) patients over the age of 2 showed an 18% (5/28) submission at baseline. With the overdue outreach program, this increased to 31% of patients initially and has further increased to 39%. Clinic visits for patients greater than 7 years old rose from 13% (3/24) initially to 57% and has further increased to 67%. Conclusion: Implementation of a coordinated overdue outreach program is successful in re-engaging patients with the PKU clinic and improving adherence to treatment recommendations. We have seen increased patient adherence across all domains, and have maintained this improved adherence despite the global COVID-19 pandemic. We believe that integrating overdue outreach guidelines into clinical practice is a replicable model for PKU clinics.
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Background. Monoclonal antibody (Mab) infusions have reduced hospitalization and mortality among higher risk patients with mild to moderate COVID-19 symptoms. Using an interdisciplinary team approach, we created a clinical team to proactively screen and outreach patients with COVID-19 to equitably offer Mab. Methods. From December 28, 2020 - May 3, 2021, a clinical team consisting of an Infectious disease pharmacist and physician, reviewed each outpatient with a positive SARS-CoV-2 PCR test at the Atlanta VA Healthcare System (AVAHCS) daily. The clinical team used the published Emergency Use Authorization criteria to determine eligibility. Eligible patients were prioritized using the Veterans Health Administration (VACO) Index for COVID-19 Mortality, which estimates the risk of 30-day mortality after COVID-19 infection using pre-COVID-19 health status (Figure 1). Eligible patients were contacted via telephone to confirm eligibility and obtain verbal consent. We performed SARS-CoV-2 IgG antibody tests when possible prior to Mab infusion, but results did not preclude Mab receipt. Telehealth follow-up occurred at 1- and 7-days post infusion. Overview of the elements of the VACO index, part 2 of 2. Results. In total, 1,346 COVID-19 patients were identified;86 (6%) patients were eligible, and 48/86 (55%) received Mab infusions (Figure 2). The median time from symptom-onset to positive COVID-19 PCR test result was 6 days (0-9) and the median time from positive COVID-19 PCR test result to Mab infusion was 2 days (0-8). SARS-CoV-2 IgG antibodies were detected in 4 of 24 (17%) patients tested. The most common comorbidities were hypertension (73%) and diabetes, (42%) (Table). Five (10%) patients required hospitalization for worsening COVID-19 symptoms post infusion. No deaths occurred. Conclusion. This approach of combining laboratory surveillance and active screening minimized delay in symptoms onset to Mab infusion, thereby optimizing outpatient treatment of COVID-19 disease. Our approach successfully treated a more diverse patient population compared to clinical trials. Mab infusions overall was well tolerated with few hospitalizations and no deaths in this cohort.