Gregory S Sawicki

Harvard Medical School | HMS · Department of Pediatrics

Introduction To achieve and maintain adequate weight, people with cystic fibrosis (CF) May often consume energy-dense, nutrient-poor foods high in added sugars and refined carbohydrates; however, little is known about the glycemic and metabolic effects of dietary composition in this patient population. The objective of this pilot study was to inves...

Objective: Effective behavioral nutrition interventions promoting adherence to cystic fibrosis (CF) dietary guidelines and promoting weight gain in children have been demonstrated in research studies but not translated to clinical care. Achieving recommended dietary intake is important as body mass index percentile is positively associated with lun...

Rationale Evaluating approaches to reduce treatment burden is a research priority among people with CF (pwCF) on highly effective modulators including elexacaftor/tezacaftor/ivacaftor (ETI). Objective To evaluate the impact of discontinuing both hypertonic saline (HS) and dornase alfa (DA) versus continuing both therapies among a subgroup of partic...

As adolescents and young adults (AYA) with a range of diagnoses transition from pediatric to adult-focused healthcare systems, developing strategies to maintain health outcomes during this period is critical. A key driver for maintaining optimal outcomes is adherence to recommended care therapies and condition-specific management plans. However, fo...

Background Mobile health (mHealth) interventions have immense potential to support disease self-management for people with complex medical conditions following treatment regimens that involve taking medicine and other self-management activities. However, there is no consensus on what discrete behavior change techniques (BCTs) should be used in an e...

Introductions Cystic fibrosis-related diabetes (CFRD) is associated with pulmonary decline, compromised nutritional status, and earlier mortality. Onset is often insidious, so screening for early detection of glycemic abnormalities is important. Continuous glucose monitoring (CGM) has been validated in people with CF and has been shown to detect ea...

OBJECTIVE Cystic fibrosis–related diabetes (CFRD) affects up to 50% of adults with cystic fibrosis and adds significant morbidity and treatment burden. We evaluated the safety and efficacy of automated insulin delivery with the iLet bionic pancreas (BP) in adults with CFRD in a single-center, open-label, random-order, crossover trial. RESEARCH DES...

In Part 3 of this 3-part series, we focus on the importance of transitioning young adult patients to adult care to ensure their optimal health and well-being into adulthood. The articles in this section bring to light the unique needs of these adolescents and how to better help them and their families navigate this time of transition. Available to...

BACKGROUND People with rare diseases want to use technology to support their disease self-management but are seldom the target population for technology developers. Cystic fibrosis (CF) is a rare, multisystemic medical condition and CF management is complex and burdensome for many people with CF. People with CF have noted that existing apps do not...

Background: The daily treatment regimen for an individual with cystic fibrosis (CF) can take more than 2 h to complete, and chronic treatment adherence rates are low. Developing partnerships between CF clinical researchers and the CF community is essential in developing acceptable, feasible, and effective strategies to improve self-management and...

The cost of cystic fibrosis (CF) care is high with a mean annual expenditure of $131,000 and is driven largely by medications and hospital utilization This article is protected by copyright. All rights reserved.

CFTR modulators have transformed CF but are some of the most expensive medications on the market and are over‐priced compared to the value given. The high price of CFTR modulators leaves people with CF dependent on Vertex's copay assistance programs. Despite record‐breaking profits, Vertex is drastically decreasing its copayment assistance programs...

Background: Elexacaftor-tezacaftor-ivacaftor has been shown to be safe and efficacious in people with cystic fibrosis and at least one F508del allele. Our aim was to identify a novel cystic fibrosis transmembrane conductance regulator (CFTR) modulator combination capable of further increasing CFTR-mediated chloride transport, with the potential fo...

Background Maintaining a healthy weight is a focus of Cystic Fibrosis (CF) care. With the increased use of highly effective CFTR modulators, many people with CF are gaining weight more easily, which may affect eating habits and body image. This study investigates providers’ understanding and current practices surrounding body image disturbance and...

Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to be safe and efficacious in people with cystic fibrosis (CF) with ≥ 1 F508del-CFTR allele in Phase 3 clinical trials. ELX/TEZ/IVA treatment led to improved lung function, with increases in percent predicted forced expiratory volume in 1 second (ppFEV1) and Cystic Fibrosis Questionnaire-Revi...

Background: Study 661-110 (EXTEND) is a phase 3, open-label, three-part rollover study designed to assess the long-term safety and efficacy of tezacaftor/ivacaftor (TEZ/IVA) in participants aged ≥12 years homozygous for F508del (F/F) or heterozygous for F508del and a residual function mutation (F/RF). TEZ/IVA was shown to be safe and efficacious f...

Introduction: Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. Approximately 5% of people with CF have residual function (RF) CFTR mutations that result in partially retained CFTR activity. Published literature on disease trajectory among those with RF mutations i...

Background Reducing treatment burden is a priority for people with cystic fibrosis, whose health has benefited from using new modulators that substantially increase CFTR protein function. The SIMPLIFY study aimed to assess the effects of discontinuing nebulised hypertonic saline or dornase alfa in individuals using the CFTR modulator elexacaftor pl...

Background As survival and health improve in people with cystic fibrosis (CF), more women with CF (wwCF) are considering their sexual and reproductive health (SRH). This study compared SRH experiences, behaviors, and care utilization of wwCF to the general population and defined CF-impacted considerations and care preferences. Methods We surveyed...

Objectives: The objective of this work was to study the performance of two available home spirometers used by people with Cystic Fibrosis (CF) over a short-term period and to assess user experience. Study design: This was a prospective observational study. Participants age 6 years and older were recruited to participate if they could complete ac...

Introduction: There is evidence for increased risk of eating disorders in individuals with diet-treated chronic illnesses, however, data in patients with Cystic Fibrosis (CF) is less clear. No studies have evaluated avoidant/restrictive food intake disorder (ARFID) in the CF population. We investigated the prevalence of eating disorders, including...

Pediatric Telehealth Best Practices goes beyond AAP policy to offer straightforward strategies for establishing and sustaining a successful telehealth program. Its contents are curated from AAP published content, including guidance from the Section on Telehealth Care as well as journals and books. Available for purchase at https://shop.aap.org/pedi...

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Introduction: There is evidence for increased risk of eating disorders in individuals with diet-treated chronic illnesses, however, data in patients with Cystic Fibrosis (CF) is less clear. No studies have evaluated avoidant/restrictive food intake disorder (ARFID) in the CF population. We investigated the prevalence of disordered eating, including...

Objective: More people with cystic fibrosis (pwCF) are reaching adulthood and considering their reproductive futures. Unfortunately, many pwCF report gaps in their reproductive healthcare. We describe measures of stakeholder engagement in developing a reproductive goals decision aid for women with CF called MyVoice:CF. Methods: Stakeholders reviewe...

Background As people with cystic fibrosis (CF) are living longer, men with CF increasingly face both general and disease-specific sexual and reproductive health (SRH) concerns. This study explored the SRH experiences and preferences of men with CF in health care in the era of widespread use of highly effective CF modulator therapies. Methods We re...

Background Two previous Phase 3 studies (“parent studies”) showed that tezacaftor/ivacaftor was generally safe and efficacious for up to 24 weeks in children 6 through 11 years of age with cystic fibrosis (CF) and F508del/F508del (F/F) or F508del/residual function (F/RF) genotypes. We assessed the safety and efficacy of tezacaftor/ivacaftor in an o...

Measures of body fat and lean mass may better predict important clinical outcomes in patients with cystic fibrosis (CF) than body mass index (BMI). Little is known about how diet quality and exercise may impact body composition in these patients. Dual X-ray absorptiometry (DXA) body composition, 24-h dietary recall, and physical activity were asses...

Background New modulator therapies have markedly improved the health of people with cystic fibrosis (CF), allowing an increased focus on quality-of-life improvements for men with CF, including those related to sexual and reproductive health (SRH). This study explored CF providers’ attitudes and experiences with addressing men's health in CF. Metho...

Objective: The objective of this study was to describe in detail the trends of the Transition Readiness Assessment Questionnaire (TRAQ-20) with respect to patient age and gender among a cohort of 655 youth with inflammatory bowel disease (IBD) to enhance the tool's utility in clinical and research settings. Materials and methods: All participant...

Background Chronic care delivery models faced unprecedented financial pressures, with a reduction of in-person visits and adoption of telehealth during the COVID-19 pandemic. We sought to understand the reported financial impact of pandemic-related changes to the cystic fibrosis (CF) care model. Methods The U.S. CF Foundation State of Care surveys...

Background Cystic fibrosis (CF) care programs in the United States rapidly adopted telehealth during the COVID-19 pandemic. Understanding factors that promote or impede telehealth will inform planning for future telehealth-enabled care models. Methods Adult, pediatric, and affiliate CF care programs in the United States (n = 287) were surveyed twi...

Background Cystic fibrosis (CF) programs and people with CF (PwCF) employed various monitoring methods for virtual care during the COVID-19 pandemic. This paper characterizes experiences with remote monitoring across the U.S. CF community. Methods The CF Foundation (CFF) sponsored distribution of home spirometers (April 2020 to May 2021), surveys...