A national postgraduate nurse practitioner and physician assistant fellowship in cystic fibrosis: An innovative approach to the provider shortage in complex and rare disease.

ABSTRACT: Cystic fibrosis (CF) is a complex life-limiting genetic condition that affects the respiratory, digestive, reproductive system, and sweat glands. Advances in treatment have led to improved survival and quality of life. Today, most persons with CF live to adulthood but require highly specialized care at accredited CF Care Centers. The growing and aging CF population combined with the provider workforce shortage have increased the demand for qualified CF providers. Nurse practitioners (NPs) and physician assistants (PAs) have been providing CF care for decades, but most learned on the job. The Leadership and Education for Advanced Practice Provider (LEAPP) fellowship in CF care aims to address the provider gap, ease transition to practice, and ensure access to specialized care. Unlike other institutional based joint NP/PA fellowships, LEAPP was designed to train providers at various locations across the national CF care center network. The program is innovative in several ways: (1) LEAPP employs a flipped classroom that pairs an online curriculum with case-based virtual discussion with content experts from the CF care network; (2) fellows receive mentored clinical training at their home CF center; (3) LEAPP partnered with a university-based team to ensure best practices and evaluation for adult learners; and (4) LEAPP promotes organizational enculturation through program components of professional mentoring, quality improvement, and leadership. This innovative approach may be suitable for other complex conditions that require highly specialized care, such as sickle cell disease, spina bifida, and solid organ transplant.

Streptococcal Pharyngitis: Rapid Evidence Review.

Group A beta-hemolytic streptococcal pharyngitis is a common infection responsible for more than 6 million office visits in the United States annually. Only 10% of adults seeking care for a sore throat have group A beta-hemolytic streptococcal pharyngitis; however, 60% or more are prescribed antibiotics. Guidelines recommend using clinical decision rules to assess the risk of group A beta-hemolytic streptococcal infection, followed by rapid antigen testing if a diagnosis is unclear, before prescribing antibiotics. Fever, tonsillar exudate, cervical lymphadenitis, and patient ages of 3 to 15 years increase clinical suspicion. A cough is more suggestive of a viral etiology. The limited history used in these decision rules is amenable to virtual visits. After a negative rapid antigen test result, a throat culture is recommended in children and adolescents. Penicillin and amoxicillin are first-line antibiotics, with a recommended course of 10 days; first-generation cephalosporins are recommended for patients with nonanaphylactic allergies to penicillin. There is significant resistance to azithromycin and clarithromycin in some parts of the United States. Steroids are not recommended for symptomatic treatment. Patients with worsening symptoms after appropriate antibiotic initiation or with symptoms lasting 5 days after the start of treatment should be reevaluated. Tonsillectomy is rarely recommended as a preventive measure: seven episodes of streptococcal pharyngitis in 1 year, five episodes in each of the past 2 years, or three episodes in each of the past 3 years are commonly used thresholds for considering surgery.

Quality improvement and rapid PDSA cycles to maintain routine surveillance of pulmonary pathogens during the COVID-19 pandemic in a pediatric cystic fibrosis clinic.

BACKGROUND: The COVID-19 pandemic necessitated immediate transition from in person to telehealth encounters; novel nursing practices were needed to ensure that children with cystic fibrosis (CF) receive care that approximates evidence-based guidelines. LOCAL PROBLEM: The aim was to ensure that as many children as possible received routine surveillance of pulmonary pathogens by a CF culture sputum culture during a pandemic. METHODS: Multiple Plan-Do-Study-Act (PDSA) cycles were utilized to implement practice change over four months. INTERVENTIONS: Cultures were obtained via curbside appointment with a registered nurse (RN) or at the patients' home with mailed equipment. RESULTS: 133 cultures obtained: 50.37% (67) by RN collection curbside and 49.62% (66) by self/caregiver at home. 120 culture swabs or sterile cups were mailed; 55% (66) were returned. Cost of mailing equipment was $760.16. CONCLUSION: Nursing utilization of PDSA cycles developed novel processes that ensured guideline-based care during the initial months of the pandemic.

Recognizing Vaccine-Preventable Diseases and Managing Outbreaks.

Outbreaks of vaccine-preventable diseases are becoming more common in the United States. Outbreaks of some diseases, such as measles, can be attributed to decreasing vaccination rates. Clinicians need to be aware of the vulnerabilities in their communities. Detection of an outbreak requires familiarity with signs, symptoms, and laboratory findings for these now unusual diseases. Clinicians also need to work with public health officials to identify, treat, and limit the spread of these infections. This article describes the populations most at risk from illnesses associated with sporadic outbreaks, with information on diagnosis, treatment, and ways to limit the spread of infection.

Management of Fever in Infants and Young Children.

Despite dramatic reductions in the rates of bacteremia and meningitis since the 1980s, febrile illness in children younger than 36 months continues to be a concern with potentially serious consequences. Factors that suggest serious infection include age younger than one month, poor arousability, petechial rash, delayed capillary refill, increased respiratory effort, and overall physician assessment. Urinary tract infections are the most common serious bacterial infection in children younger than three years, so evaluation for such infections should be performed in those with unexplained fever. Abnormal white blood cell counts have poor sensitivity for invasive bacterial infections; procalcitonin and C-reactive protein levels, when available, are more informative. Chest radiography is rarely recommended for children older than 28 days in the absence of localizing signs. Lumbar puncture is not recommended for children older than three months without localizing signs; it may also be considered for those from one to three months of age with abnormal laboratory test results. Protocols such as Step-by-Step, Laboratory Score, or the Rochester algorithms may be helpful in identifying low-risk patients. Rapid influenza testing and tests for coronavirus disease 2019 (COVID-19) may be of value when those diseases are circulating. When empiric treatment is appropriate, suggested antibiotics include ceftriaxone or cefotaxime for infants one to three months of age and ampicillin with gentamicin or with cefotaxime for neonates. For children three months to three years of age, azithromycin or amoxicillin is recommended if pneumonia is suspected; for urinary infections, suggested antibiotics are cefixime, amoxicillin/clavulanate, or trimethoprim/sulfamethoxazole. Choice of antibiotics should reflect local patterns of microbial resistance.

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Evaluation of fever in infants and young children.

Febrile illness in children younger than 36 months is common and has potentially serious consequences. With the widespread use of immunizations against Streptococcus pneumoniae and Haemophilus influenzae type b, the epidemiology of bacterial infections causing fever has changed. Although an extensive diagnostic evaluation is still recommended for neonates, lumbar puncture and chest radiography are no longer recommended for older children with fever but no other indications. With an increase in the incidence of urinary tract infections in children, urine testing is important in those with unexplained fever. Signs of a serious bacterial infection include cyanosis, poor peripheral circulation, petechial rash, and inconsolability. Parental and physician concern have also been validated as indications of serious illness. Rapid testing for influenza and other viruses may help reduce the need for more invasive studies. Hospitalization and antibiotics are encouraged for infants and young children who are thought to have a serious bacterial infection. Suggested empiric antibiotics include ampicillin and gentamicin for neonates; ceftriaxone and cefotaxime for young infants; and cefixime, amoxicillin, or azithromycin for older infants.

Pro-inflammatory cytokines and lipopolysaccharide induce changes in cell morphology, and upregulation of ERK1/2, iNOS and sPLA2-IIA expression in astrocytes and microglia.

BACKGROUND: Activation of glial cells, including astrocytes and microglia, has been implicated in the inflammatory responses underlying brain injury and neurodegenerative diseases including Alzheimer's and Parkinson's diseases. Although cultured astrocytes and microglia are capable of responding to pro-inflammatory cytokines and lipopolysaccharide (LPS) in the induction and release of inflammatory factors, no detailed analysis has been carried out to compare the induction of iNOS and sPLA2-IIA. In this study, we investigated the effects of cytokines (TNF-alpha, IL-1beta, and IFN-gamma) and LPS + IFN-gamma to induce temporal changes in cell morphology and induction of p-ERK1/2, iNOS and sPLA2-IIA expression in immortalized rat (HAPI) and mouse (BV-2) microglial cells, immortalized rat astrocytes (DITNC), and primary microglia and astrocytes. METHODS/RESULTS: Cytokines (TNF-alpha, IL-1beta, and IFN-gamma) and LPS + IFN-gamma induced a time-dependent increase in fine processes (filopodia) in microglial cells but not in astrocytes. Filopodia production was attributed to IFN-gamma and was dependent on ERK1/2 activation. Cytokines induced an early (15 min) and a delayed phase (1 ~ 4 h) increase in p-ERK1/2 expression in microglial cells, and the delayed phase increase corresponded to the increase in filopodia production. In general, microglial cells are more active in responding to cytokines and LPS than astrocytes in the induction of NO. Although IFN-gamma and LPS could individually induce NO, additive production was observed when IFN-gamma was added together with LPS. On the other hand, while TNF-alpha, IL-1beta, and LPS could individually induce sPLA2-IIA mRNA and protein expression, this induction process does not require IFN-gamma. Interestingly, neither rat immortalized nor primary microglial cells were capable of responding to cytokines and LPS in the induction of sPLA2-IIA expression. CONCLUSION: These results demonstrated the utility of BV-2 and HAPI cells as models for investigation on cytokine and LPS induction of iNOS, and DITNC astrocytes for induction of sPLA2-IIA. In addition, results further demonstrated that cytokine-induced sPLA2-IIA is attributed mainly to astrocytes and not microglial cells.

Complementary and alternative medicine in US family medicine practices: a pilot qualitative study.

CONTEXT: The growth of complementary and alternative medicine (CAM) has led some family medicine practices to include CAM. Acupuncture or herbal medicine, for example, may be offered at such practices. When a practice incorporates both CAM and conventional treatments, its goals and values may differ from those found in traditional primary care. Little is known about the development of these integrated practices, which may be expected to become more widespread. OBJECTIVE: To identify some of the concepts and challenges shaping family medicine practices that incorporate CAM. DESIGN: Comparative case study. METHOD: Multi-method assessment process including participant observation, key informant interviews, semi-structured depth interviews, and observation of patient-provider encounters. SETTING: Four family medicine/CAM practices in the mid-Atlantic region of the United States. RESULTS: Key themes that influence these practices' organization include dimensions of health, the selection of therapies used, the practices' approach to evidence, their perspective on the amount of time spent with patients, and their adaptations to financial concerns. Each practice emphasized long patient visits. In each, physicians had expertise that enabled them to draw on both conventional medicine and CAM. CONCLUSION: Successful incorporation of CAM modalities within a family medicine framework requires adaptation not only at the practice level but also by individual physicians.

Hyperlipidemia guideline adherence and association with patient gender.

BACKGROUND: Gender disparities in cardiovascular disease (CVD) management have become increasingly apparent in recent years. Previous research has focused on inpatient disparities, but little is known about how patient gender affects assessment, treatment, and management of patients for hyperlipidemia and cardiovascular risk in primary care settings. Patients with coronary artery disease (CAD) and hyperlipidemia are at high risk for cardiovascular and cerebrovascular morbidity. We sought to examine the effect of patient gender on assessment, treatment, and target maintenance of hyperlipidemia among patients with CAD in a primary care setting. METHODS: Chart abstraction was done for 715 patients with CAD in 55 family practices in New Jersey and eastern Pennsylvania as part of the Using Learning Teams for Reflective Adaptation (ULTRA) project. Hyperlipidemia assessment, treatment, and target adherence scores were determined for those at-risk patients based on National Heart, Lung, and Blood Institute (NHLBI) recommended National Cholesterol Education Program (NCEP) ATP III guidelines. Generalized linear models were used to determine the association of hyperlipidemia guideline adherence with patient gender, using comorbidities and age as confounders. RESULTS: After controlling for comorbidities and age, women were less likely to be assessed for lipids (p = 0.0462). There was no difference in treatment (p = 0.1074) or target laboratory values (p = 0.3949). CONCLUSIONS: Women with CAD are less often assessed for lipids than men in primary care practices. More intensive efforts may be necessary to educate physicians and patients about cardiovascular risk for women.