scholarly journals Disease disclosure in individuals with cystic fibrosis: Association with psychosocial and health outcomes

2016 ◽  
Vol 15 (5) ◽  
pp. 696-702 ◽  
Author(s):  
Adrienne P. Borschuk ◽  
Robin S. Everhart ◽  
Michelle N. Eakin ◽  
Devin Rand-Giovannetti ◽  
Belinda Borrelli ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Health Outcomes

scholarly journals Health Disparities among adults cared for at an urban cystic fibrosis program

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Emily DiMango ◽  
Kaitlyn Simpson ◽  
Elizabeth Menten ◽  
Claire Keating ◽  
Weijia Fan ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
New York ◽  
New York City ◽  
Health Disparities ◽  
Pulmonary Function ◽  
Health Outcomes ◽  
York City ◽  
Median Income ◽  
Cross Sectional ◽  
Caucasian Patients

Abstract Background Evidence is conflicting regarding differential health outcomes in racial and ethnic minorities with cystic fibrosis (CF), a rare genetic disease affecting approximately 28,000 Americans. We performed a cross-sectional analysis of health outcomes in Black/Latinx patients compared with non-Hispanic Caucasian patients cared for in a CF center in New York City. Adult patients enrolled in the CF Foundation Patient Registry at the Columbia University Adult CF Program and seen at least once during 2019 were included. Health metrics were compared between Black/Latinx and non-Hispanic Caucasian patients. Results 262 patients were eligible. 39 patients (15%) identified as Black/Latinx or non-Hispanic Caucasian. Descriptive statistics are reported with mean (standard deviation). Current age was 35.9 (13.3) years for non-Hispanic Caucasian and 32.0 (9.3) years for Black/Latinx patients (p = 0.087). Age of diagnosis did not differ between groups; 9.56 (15.96) years versus 11.59 (15.8) years for non-Hispanic Caucasian versus Black/Latinx respectively (p = 0.464). Pulmonary function, measured as mean forced expiratory volume in one second (FEV1) was 70.6 (22.5) percent predicted in non-Hispanic Caucasian versus 59.50 (27.9) percent predicted in Black/Latinx patients (p = 0.010). Number of visits to the CF clinic were similar between groups. When controlled for age, gender, co-morbidities, median income, and insurance status, there was a continued association between minority status and lower FEV1. Conclusions Minorities with CF have significantly lower pulmonary function, the major marker of survival, than non-Hispanic Caucasians, even when controlled for a variety of demographic and socioeconomic factors that are known to affect health status in CF. Significant health disparities based on race and ethnicity exist at a single CF center in New York City, despite apparent similarities in access to guideline based care at an accredited CF Center. This data confirms the importance of design of culturally appropriate preventative and management strategies to better understand how to direct interventions to this vulnerable population with a rare disease.

Health Outcomes Associated With Transition From Pediatric to Adult Cystic Fibrosis Care

PEDIATRICS ◽  
2013 ◽  
Vol 132 (5) ◽  
pp. 847-853 ◽  
Author(s):  
Lisa Tuchman ◽  
Michael Schwartz
Keyword(s):  
Cystic Fibrosis ◽  
Health Outcomes

scholarly journals Associations of psychosocial factors with health outcomes among youth with cystic fibrosis

2009 ◽  
Vol 44 (1) ◽  
pp. 46-53 ◽  
Author(s):  
Joän M. Patterson ◽  
Melanie Wall ◽  
Jerica Berge ◽  
Carlos Milla
Keyword(s):  
Cystic Fibrosis ◽  
Health Outcomes ◽  
Psychosocial Factors

scholarly journals Modeling long-term health outcomes of patients with cystic fibrosis homozygous for F508del-CFTR treated with lumacaftor/ivacaftor

2019 ◽  
Vol 13 ◽  
pp. 175346661882018 ◽  
Author(s):  
Jaime L. Rubin ◽  
Lasair O’Callaghan ◽  
Christopher Pelligra ◽  
Michael W. Konstan ◽  
Alexandra Ward ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Health Outcomes ◽  
Median Survival ◽  
Patient Simulation ◽  
Simulation Models ◽  
Patient Characteristics ◽  
Model Parameters ◽  
Term Survival ◽  
Lifetime Treatment

Background: Lumacaftor/ivacaftor combination therapy is efficacious and generally safe for patients with cystic fibrosis (CF) homozygous for the F508del-CF transmembrane conductance regulator (CFTR) mutation. However, long-term survival benefits of lumacaftor/ivacaftor (LUM/IVA) cannot yet be quantified. Simulation models can provide predictions about long-term health outcomes. In this study, we aimed to project long-term health outcomes of LUM/IVA plus standard care (SC) in patients with CF homozygous for F508del-CFTR. Methods: This modeling study was an individual patient simulation in US patients aged ⩾6 years with CF, homozygous for F508del-CFTR. The primary outcome was projected survival among (a) a cohort of patients who ever initiated LUM/IVA, accounting for treatment discontinuations, and (b) a cohort of patients who remain on continuous LUM/IVA. Patient characteristics and model parameters were derived from clinical trials: VX14-809-109, VX13-809-011B, TRAFFIC/TRANSPORT, and PROGRESS; published literature; and the US CF Foundation Patient Registry. Results: Lumacaftor/ivacaftor + SC is expected to increase median survival by 6.1 years versus SC alone, accounting for treatment discontinuations. The incremental median predicted survival versus SC assuming initiation of LUM/IVA at ages 6, 12, 18, and 25 years was 17.7, 12.6, 8.0, and 3.8 years, respectively. Assuming lifetime treatment with LUM/IVA, incremental median survival was predicted to be 7.8 years longer in the LUM/IVA + SC cohort. Initiating LUM/IVA at ages 6, 12, 18, and 25 years and assuming lifetime treatment resulted in incremental median predicted survival of 23.4, 18.2, 11.0, and 4.8 years, respectively. Conclusions: Lumacaftor/ivacaftor is projected to increase survival for patients with CF. Initiation at an early age and treatment persistence result in further increments in projected survival.

WS16.3 Relationship between physical activity and long-term health outcomes in adults with cystic fibrosis

2020 ◽  
Vol 19 ◽  
pp. S27
Author(s):  
T. Dwyer ◽  
R. Dentice ◽  
J. Marouvo ◽  
P. Bye ◽  
J. Alison
Keyword(s):  
Physical Activity ◽  
Cystic Fibrosis ◽  
Health Outcomes

scholarly journals Pseudomonas aeruginosaAlginate Overproduction Promotes Coexistence withStaphylococcus aureusin a Model of Cystic Fibrosis Respiratory Infection

mBio ◽  
2017 ◽  
Vol 8 (2) ◽  
Author(s):  
Dominique H. Limoli ◽  
Gregory B. Whitfield ◽  
Tomoe Kitao ◽  
Melissa L. Ivey ◽  
Michael R. Davis ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Health Outcomes ◽  
Community Dynamics ◽  
Antimicrobial Agents ◽  
Respiratory Infections ◽  
Chronic Infections ◽  
Microbial Community Dynamics ◽  
Secreted Factors ◽  
Patient Health

ABSTRACTWhile complex intra- and interspecies microbial community dynamics are apparent during chronic infections and likely alter patient health outcomes, our understanding of these interactions is currently limited. For example,Pseudomonas aeruginosaandStaphylococcus aureusare often found to coinfect the lungs of patients with cystic fibrosis (CF), yet these organisms compete under laboratory conditions. Recent observations that coinfection correlates with decreased health outcomes necessitate we develop a greater understanding of these interbacterial interactions. In this study, we tested the hypothesis thatP. aeruginosaand/orS. aureusadopts phenotypes that allow coexistence during infection. We compared competitive interactions ofP. aeruginosaandS. aureusisolates from mono- or coinfected CF patients employingin vitrococulture models.P. aeruginosaisolates from monoinfected patients were more competitive towardS. aureusthanP. aeruginosaisolates from coinfected patients. We also observed that the least competitiveP. aeruginosaisolates possessed a mucoid phenotype. Mucoidy occurs upon constitutive activation of the sigma factor AlgT/U, which regulates synthesis of the polysaccharide alginate and dozens of other secreted factors, including some previously described to killS. aureus. Here, we show that production of alginate in mucoid strains is sufficient to inhibit anti-S. aureusactivity independent of activation of the AlgT regulon. Alginate reduces production of siderophores, 2-heptyl-4-hydroxyquinolone-N-oxide (HQNO), and rhamnolipids—each required for efficient killing ofS. aureus. These studies demonstrate alginate overproduction may be an important factor drivingP. aeruginosacoinfection withS. aureus.IMPORTANCENumerous deep-sequencing studies have revealed the microbial communities present during respiratory infections in cystic fibrosis (CF) patients are diverse, complex, and dynamic. We now face the challenge of determining the influence of these community dynamics on patient health outcomes and identifying candidate targets to modulate these interactions. We make progress toward this goal by determining that the polysaccharide alginate produced by mucoid strains ofP. aeruginosais sufficient to inhibit multiple secreted antimicrobial agents produced by this organism. Importantly, these secreted factors are required to outcompeteS. aureus, when the microbes are grown in coculture; thus we propose a mechanism whereby mucoidP. aeruginosacan coexist withS. aureus. Finally, the approach used here can serve as a platform to investigate the interactions among other CF pathogens.

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