Using A Work System Framework to Investigate Pharmacists' Roles in Cystic Fibrosis Management.

OBJECTIVE: Cystic fibrosis (CF) is a genetic disease that requires complex, lifelong treatment regimens to maintain health and reduce disease progression. The aims of this study were 1) to gain the perspectives of multiple health professions to understand medication and well-being challenges of people living with CF; and 2) to apply the Systems Engineering Initiative for Patient Safety (SEIPS) model to further identify opportunities for pharmacists to support people with CF. METHODS: Health care professionals were recruited from a Cystic Fibrosis Center in the Midwest, to participate in audio-recorded semistructured interviews. Topics examined during the interviews included medication education for patients as well as experiences with outpatient, specialty, and community pharmacists. The themes assessed during the pharmacist interviews included support for people living with CF, preferences in conducting medication education, and pharmacist-specific counseling. Interview transcripts were thematically analyzed into categories to determine major themes. Prevalent codes were categorized into 5 major themes guided by the SEIPS model. Interrater reliability was strong (kappa = 0.94). RESULTS: Five major themes were identified: 1) patient tasks; 2) external environment; 3) organizational conditions; 4) patient medication education; and 5) pharmacists' roles and tasks. Professionals identified the importance of the pharmacist on the multidisciplinary CF care team to enhance patient-centered care for people living with CF. CONCLUSIONS: This study highlights health care professionals' views on the unique skillset that pharmacists add to the care team, including a reduction in medication errors, improved adherence, and overall enhanced patient care.

Characterizing medication management and the role of pharmacists in caring for people living with cystic fibrosis: A work system approach.

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive genetic disease requiring complex, lifelong medication regimens. Given the importance of medication in CF treatment, pharmacists are vital CF care team members in the care of people living with CF (PwCF). OBJECTIVES: This study aimed to (1) define patients' CF medication experiences and educational needs and (2) investigate the CF outpatient clinic and community pharmacist's role in addressing patient challenges. METHODS: A work system approach informed by the Systems Engineering Initiative for Patient Safety (SEIPS) model was used to characterize knowledge and perception of CF medication regimens, educational modalities, and pharmacist interactions for PwCF. Semistructured interviews were conducted with adults living with CF at a CF center clinic. Data analyses identified relationships between the themes in the data and 4 SEIPS work system domains: tasks, tools and technology, person, and environment. RESULTS: Thirty PwCF interviews highlighted 4 themes regarding health care experiences: (1) medication use experience, (2) medication education needs, (3) disease experience, and (4) pharmacist and pharmacy interactions. Patients reported complex medication regimens leading to challenges with medication adherence, although the benefit of treatment was recognized. Although a high level of disease-state knowledge was identified among the participants, PwCF desired to learn about CF medication benefits and adverse effects through credible sources using multiple modalities. Many reported a benefit of pharmacist involvement in their care. CONCLUSION: Pharmacists are well-positioned to support PwCF in adherence, medication regimen management, and medication education. Opportunities exist for growth in these supportive roles of a pharmacist in both community and outpatient clinic settings.

Pneumocystis jirovecii Disease: Basis for the Revised EORTC/MSGERC Invasive Fungal Disease Definitions in Individuals Without Human Immunodeficiency Virus.

BACKGROUND: Pneumocystis jirovecii pneumonia (PCP) causes substantive morbidity in immunocompromised patients. The EORTC/MSGERC convened an expert group to elaborate consensus definitions for Pneumocystis disease for the purpose of interventional clinical trials and epidemiological studies and evaluation of diagnostic tests. METHODS: Definitions were based on the triad of host factors, clinical-radiologic features, and mycologic tests with categorization into probable and proven Pneumocystis disease, and to be applicable to immunocompromised adults and children without human immunodeficiency virus (HIV). Definitions were formulated and their criteria debated and adjusted after public consultation. The definitions were published within the 2019 update of the EORTC/MSGERC Consensus Definitions of Invasive Fungal Disease. Here we detail the scientific rationale behind the disease definitions. RESULTS: The diagnosis of proven PCP is based on clinical and radiologic criteria plus demonstration of P. jirovecii by microscopy using conventional or immunofluorescence staining in tissue or respiratory tract specimens. Probable PCP is defined by the presence of appropriate host factors and clinical-radiologic criteria, plus amplification of P. jirovecii DNA by quantitative real-time polymerase chain reaction (PCR) in respiratory specimens and/or detection of ß-d-glucan in serum provided that another invasive fungal disease and a false-positive result can be ruled out. Extrapulmonary Pneumocystis disease requires demonstration of the organism in affected tissue by microscopy and, preferably, PCR. CONCLUSIONS: These updated definitions of Pneumocystis diseases should prove applicable in clinical, diagnostic, and epidemiologic research in a broad range of immunocompromised patients without HIV.

Difficult to Treat Infections in the Burn Patient.

Background: Unusual infections can lead to complications in more severely burned patients and pose major challenges in treatment. Methods: The published literature of retrospective reviews and case series of the uncommon infections of osteomyelitis, polymicrobial bacteremia, recurrent bacteremia, endocarditis, central nervous system (CNS), and rare fungal infections in burned patients have been summarized and presented. Results: When compared with infections occurring in the non-burn population, these infections in burn patients are more likely to be because of gram-negative bacteria or fungi. Because of hyperdynamic physiology and changes in immunomodulatory response secondary to burns, the clinical presentation of these infections in a patient with major burns differs from that of the non-burn patient and may not be identified until the post-mortem examination. Some of these infections (osteomyelitis, endocarditis, CNS, rare fungal infections) may necessitate surgical intervention in addition to antimicrobial therapy to achieve cure. The presence of the burn and allograft can also present unique challenges for surgical management. Conclusions: These difficult and unusual infections in the severely burned patient necessitate an index of suspicion, appropriate diagnosis, identification and sensitivities of the putative pathogen, effective systemic antimicrobial therapy, and appropriate surgical intervention if recovery is to be achieved.

Long-term Outcomes and Survival in Moderate-severe Portopulmonary Hypertension After Liver Transplant.

BACKGROUND: Portopulmonary hypertension is present in an estimated 5.3% to 8.5% of liver transplant candidates. Untreated, 5-year survival is estimated between 14% and 28%. Moderate-severe disease is a contraindication to liver transplant due to the high perioperative mortality, but patients optimized with pulmonary vasodilator therapy can become eligible for transplant. There is minimal data regarding posttransplant outcomes and ability to discontinue pulmonary vasodilator therapy posttransplant. METHODS: We performed a single-center retrospective analysis to evaluate long-term outcomes of patients with moderate-severe portopulmonary hypertension who were optimized with pulmonary vasodilator therapy, became eligible for liver transplant, and subsequently underwent transplant. We identified 24 patients optimized with pulmonary vasodilator therapy who underwent subsequent liver transplantation and 25 patients who were treated with pulmonary vasodilator therapy alone. RESULTS: In the transplanted cohort, 1-year survival from portopulmonary hypertension diagnosis date: 95.8%, 3-year survival: 90.9%, and 5-year survival: 90.9%. Posttransplant; 1-, 3-, and 5-year survival was 86.9%. Among transplanted patients, 41.6% (10/24) were optimized with nonparenteral therapy. Following transplantation, 100% (14/14) of the surviving patients were able to discontinue parenteral therapy; median time: 7.2 months (interquartile range: 5.1-8.9 mo), while 61.9% (13/21) were able to discontinue pulmonary vasodilator therapy altogether; median time: 13.9 months (interquartile range: 5.1-17.6 mo). CONCLUSIONS: Patients who are optimized with pulmonary vasodilator therapy before liver transplant can have excellent long-term outcomes posttransplant. Oral pulmonary vasodilator therapy can be effective treatment to qualify a patient for transplant, and the majority are able to wean from pulmonary vasodilator therapy entirely posttransplant.

Revision and Update of the Consensus Definitions of Invasive Fungal Disease From the European Organization for Research and Treatment of Cancer and the Mycoses Study Group Education and Research Consortium.

BACKGROUND: Invasive fungal diseases (IFDs) remain important causes of morbidity and mortality. The consensus definitions of the Infectious Diseases Group of the European Organization for Research and Treatment of Cancer and the Mycoses Study Group have been of immense value to researchers who conduct clinical trials of antifungals, assess diagnostic tests, and undertake epidemiologic studies. However, their utility has not extended beyond patients with cancer or recipients of stem cell or solid organ transplants. With newer diagnostic techniques available, it was clear that an update of these definitions was essential. METHODS: To achieve this, 10 working groups looked closely at imaging, laboratory diagnosis, and special populations at risk of IFD. A final version of the manuscript was agreed upon after the groups' findings were presented at a scientific symposium and after a 3-month period for public comment. There were several rounds of discussion before a final version of the manuscript was approved. RESULTS: There is no change in the classifications of "proven," "probable," and "possible" IFD, although the definition of "probable" has been expanded and the scope of the category "possible" has been diminished. The category of proven IFD can apply to any patient, regardless of whether the patient is immunocompromised. The probable and possible categories are proposed for immunocompromised patients only, except for endemic mycoses. CONCLUSIONS: These updated definitions of IFDs should prove applicable in clinical, diagnostic, and epidemiologic research of a broader range of patients at high-risk.

HIV PrEP in the Military: Experience at a Tertiary Care Military Medical Center.

Objectives: We evaluated human immunodeficiency virus (HIV) pre-exposure prophylaxis (PrEP) administration at the Walter Reed National Military Medical Center (WRNMMC), which serves a geographic area at high risk of HIV infection. Methods: Medical records were reviewed for all patients initiating PrEP at WRNMMC from November 1, 2013, to March 30, 2016. Demographic, laboratory, clinical, and risk exposure characteristics and outcomes were described. Results: One hundred fifty-nine patients received PrEP; 133 (84%) patients were active duty, 95 (60%) patients were over 28 yr old. The majority were non-Hispanic Whites (n = 87, 55%). The median men who have sex with men (MSM) risk index score was 18.0 (IQR 12.0-22.0); 20 patients scored less than 10. One hundred and thirty-one (82%) patients remained on PrEP through the evaluation period. Patients mainly discontinued PrEP for service-related or toxicity reasons. Incident STIs occurred in 31 (19%) patients. No cases of HIV seroconversion were observed. Conclusions: In this first description of PrEP utilization in a U.S. military health care system, a significant number of patients were non-Hispanic Whites, well-educated, were older, or were otherwise at low risk for HIV acquisition. Further effort is needed to enhance PrEP use among the higher risk young African-American MSM population, and further studies are needed to determine the cost-effectiveness of PrEP in individuals who are not categorized as high risk.

Role of pirfenidone in the management of pulmonary fibrosis.

Pulmonary fibrosis is associated with a number of specific forms of interstitial lung disease (ILD) and can lead to progressive decline in lung function, poor quality of life, and, ultimately, early death. Idiopathic pulmonary fibrosis (IPF), the most common fibrotic ILD, affects up to 1 in 200 elderly individuals and has a median survival that ranges from 3 to 5 years following initial diagnosis. IPF has not been shown to respond to immunomodulatory therapies, but recent trials with novel antifibrotic agents have demonstrated lessening of lung function decline over time. Pirfenidone has been shown to significantly slow decline in forced vital capacity (FVC) over time and prolong progression-free survival, which led to its licensing by the United States Food and Drug Administration (FDA) in 2014 for the treatment of patients with IPF. However, pirfenidone has been associated with significant side effects, and patients treated with pirfenidone must be carefully monitored. We review recent and ongoing clinical research and experience with pirfenidone as a pharmacologic therapy for patients with IPF, provide a suggested approach to incorporate pirfenidone into a treatment algorithm for patients with IPF, and examine the potential of pirfenidone as a treatment for non-IPF forms of ILD accompanied by progressive pulmonary fibrosis.