查看更多>>摘要：In this commentary, we discuss health disparities, reflecting on our experience in delayed diagnosis of cystic fibrosis based on race and bias in healthcare practitioners. What is Health Disparity, and how is it defined in the literature and in reality? To understand the word in its full context, let us first focus on what is most evident and that is the word "disparity." The HealthyPeople.org website notes, "Although the term disparities is often interpreted to mean racial or ethnic disparities, many dimensions of disparity exist in the United States, particularly in health. If a health outcome is seen to a greater or lesser extent between populations, there is disparity."
Jonathan J. GrantErin J. McDadeJeffery T. ZobellDavid C. Young...
23页
查看更多>>摘要：Care for people with cystic fibrosis (PWCF) is highly complex and requires a multi-disciplinary approach where the pharmacist plays a vital role. The purpose of this manuscript is to serve as a guideline for pharmacists and pharmacy technicians who provide care for PWCF by providing background and current recommendations for the use of cystic fibrosis (CF)-specific medications in both the acute and ambulatory care settings. The article explores current literature surrounding the role of pharmacists and pharmacy technicians, proven pharmacy models to emulate, and phar-macokinetic idiosyncrasies unique to the CF population while also identifying areas of future research. Clinical recommendations for the use of CF-specific medications are broken down by organ system including mechanism of action, adverse events, dosages, and monitoring parameters. The article also includes quick reference tables essential to the acute and chronic medication therapy management of PWCF.
查看更多>>摘要：Airway clearance has been an integral part of cystic fibrosis (CF) care for almost as long as CF has been identified as a condition. From diagnosis as a neonate through to end-of-life care, airway clearance is an everyday aspect of life, adding a considerable treatment burden to the lives of people with CF. There are many different techniques used for airway clearance which have evolved over time with an aim to improve effectiveness, support adherence and, more recently, to consider the impact of burden. A popular thought is whether airway clearance could be replaced by exercise. With new precision therapies in the form of CFTR modulators available, the CF landscape is rapidly changing, raising the question of whether certain treatments are needed at all. Depending on factors such as CFTR mutation, age, and pre-existing lung damage before starting a CFTR modulator, individuals with CF may need different levels of intensity and type of maintenance treatment. Precision medicine is likely to lead to the need for increased precision and individualized management around other maintenance therapies such as airway clearance.
Catherine M. McDonaldVirginia A. StallingsSuzanne H. MichelMegan E. Gabel...
10页
查看更多>>摘要：Achieving a healthy weight balance has been a central focus of care for people who have cystic fibrosis (CF). Over the years, the emphasis has primarily been on promoting weight gain to optimize pulmonary outcomes. With continued improvements in CF care, including highly effective CF modulators available for many people, the CF community is now experiencing a new challenge: addressing the concern that some people are gaining weight excessively. While at this time, we do not know to what extent overweight and obesity will affect health outcomes for people with CF, it is likely that excessive weight gain may have negative health impacts similar to those seen in the general population. In this paper, we review the history of nutritional guidelines for people with CF, as well as more recent trends toward overweight and obesity for some. A multi-disciplinary approach is needed to collaboratively start the oftentimes difficult conversation regarding excessive weight gain, and to identify resources to help people achieve and maintain a healthy weight through diet, exercise, and behavioral modification.
查看更多>>摘要：Noninvasive ventilation (NIV) use was initially reported in cystic fibrosis (CF) in 1991 as a bridge to lung transplantation, and over the decades, the use of NIV has increased in the CF population. Individuals with CF are prone to various physiologic changes as lung function worsens, and they benefit from NIV for advanced lung disease. As life expectancy in CF has been increasing due to advances such as highly effective modulator therapy, people with CF may also benefit from NIV for other diagnosis beyond advanced lung disease. NIV can improve gas exchange, quality of sleep, exercise tolerance, and augment airway clearance in CF. CF providers can readily become comfortable with this therapeutic modality. In this review, we will summarize the physiologic basis for NIV use in CF, describe indications for initiation, and discuss how to order and monitor patients on NIV. We will discuss aspects unique to people with CF and the use of NIV, as well as suggestions on how to reduce risks such as infection. We hope that this serves as a resource for CF providers, in particular those who do not have dedicated training in sleep medicine as we all continue to care for the CF patient population.
查看更多>>摘要：Highly effective modulator therapy (HEMT) for cystic fibrosis (CF) has been touted as one of the greatest advances to date in CF care. As these therapies are now available for many older children and adults with CF, marked improvement of their nutritional status, pulmonary and gastrointestinal symptoms has been observed. However, most infants and younger children are not current candidates for HEMT due to age and/or cystic fibrosis transmembrane conductance regulator (CFTR) mutation. For these young children, it is essential to provide rigorous monitoring and care to avoid potential disease sequelae while awaiting HEMT availability. The following article highlights recent advances in the care of infants and young children with CF with regard to surveillance and treatment of nutritional, pulmonary, and gastrointestinal disorders. Recent clinical trials in this population are also reviewed.
Natalie E. WestTraci M. KazmerskiJennifer L Taylor-CousarVin Tangpricha...
12页
查看更多>>摘要：With improved therapies, people with cystic fibrosis are living longer and healthier lives and increasingly have questions surrounding their sexual and reproductive health. This article will summarize the important issues of which providers should be aware during the lifespan of people with cystic fibrosis, including puberty, adulthood, and menopause. A wide range of sexual and reproductive health topics are addressed such as puberty, transgender and gender nonbinary identities, contraception, sexually transmitted infections, hypogonadism, sexual functioning, cyclical hemoptysis, and urinary incontinence. We discuss gaps in knowledge and current evidence as well as management strategies to optimize care. Our goal is to support providers to enable them to give comprehensive care throughout the lifespan of people with cystic fibrosis.
查看更多>>摘要：Caring for people with cystic fibrosis has changed considerably since the first description of the disorder and continues to evolve in the era of highly effective modulator therapy. These new treatment advancements are resulting in improved health outcomes in an ever-growing adult population with improved long-term survival. This study explores potential comorbidities and mental health implications associated with increased longevity and survivorship. It also considers the need for further evolution in patient-centered care with an expanded healthcare team in a more virtually connected world.
Kimberly A. McBennettPamela B. DavisMichael W. Konstan
8页
查看更多>>摘要：Since the first description of cystic fibrosis in 1938, there have been significant advances in both quality of life and longevity for people living with this disease. In this article we describe the milestones of the last 80 years and what we perceive to be the remaining barriers to normalcy for this population. Based on data from 2019, the Cystic Fibrosis Foundation Registry Report from the United States calculated the predicted median survival age of a child born that year with cystic fibrosis to be 48.4 years, a remarkable achievement. Summarized data from the registries of the United Kingdom, Canada, Belgium, Europe, Australia, France and Ireland show a range of median survival age from 44 to 53 years, and median age at death ranging from 29 to 35.6 years. The greater longevity for patients with Cystic Fibrosis (CF) is not only a therapeutic triumph, but also a therapeutic challenge, as diseases and complications of aging now must be factored into the care of patients with CF. This article and this volume (in much greater depth than this article can provide) will address how the CF field achieved this greatly improved survival expectation, which factors have promoted it and which have not yet been appropriately addressed, and what the rapidly developing therapeutic strategies hold for the future. Since there is variability around the world in the timing and application of various advances, in this article we will concentrate on milestones in the United States.
Traci M. KazmerskiNatalie E. WestRaksha JainAhmet Uluer...
14页
查看更多>>摘要：As people with cystic fibrosis (CF) live longer and healthier lives, increasing numbers are considering the full range of reproductive options for their futures, including parenthood, pregnancy, or pregnancy prevention. As the face of CF changes, the CF care model must adapt to meet the reproductive health needs of both parents and nonparents with CF. This article summarizes the reproductive goals and family-building concerns faced by people with CF, including fertility, pregnancy, and alternative paths to parenthood, the impact of parenthood on mental and physical health, and important future research.
NSTL
Wiley