Cessation of smoke exposure improves pediatric CF outcomes: Longitudinal analysis of CF Foundation Patient Registry data.

BACKGROUND: Tobacco smoke exposure is a major risk factor for the health of children and adolescents with CF. In this study, we assess whether cessation of smoke exposure is associated with improved outcomes in this population. METHODS: We used annualized and encounter-based data from the U.S. CF Foundation Patient Registry (2006-2018) on all individuals born 1998-2010. The analytical sample included those who ever reported second-hand smoke exposure (daily or weekly), ever lived with a smoker, or ever reported smoking themselves. We used non-linear mixed models for pulmonary exacerbations and linear mixed models for ppFEV1 and BMI as a function of ceased exposure. RESULTS: The sample included 3,633 individuals contributing 19,629 person-years. Cessation of smoke exposure reduced the odds of a pulmonary exacerbation in 12 months by 17% (OR 0.83, p < 0.001) in the first year of cessation, with an additional 6% decrease (OR 0.94, p = 0.003) for each additional year of cessation. Cessation was associated with improvements in ppFEV1 and BMI: 0.7% ppFEV1 increase (p < 0.001) in the first year of cessation and 0.4% increase (p = 0.001) for each additional year of cessation; 1% increase in BMI percentile (p < 0.001) in the first year of cessation plus 0.4% increase (p = 0.009) for each additional year. Three years of cessation reduce the predicted probability of a pulmonary exacerbation in 12 months by 8% and improve ppFEV1 and BMI by 2%. CONCLUSION: Eliminating smoke exposure may reduce pulmonary exacerbations and improve respiratory and nutritional outcomes in children and adolescents with CF. Both smoking cessation and exposure prevention should be prioritized in pediatric CF care.

A Mobile Health Platform for Self-Management of Pediatric Cystic Fibrosis: Qualitative Study of Adaptation to Stakeholder Needs and Integration in Clinical Settings.

BACKGROUND: Cystic fibrosis (CF) is an inherited chronic condition that requires extensive daily care and quarterly clinic visits with a multidisciplinary care team. The limited exchange of information outside of the quarterly clinic visits impedes optimal disease self-management, patient engagement, and shared decision making. OBJECTIVE: The aim of this study is to adapt a mobile health (mHealth) app originally developed in Sweden to the needs of patients, families, and health care providers in a CF center in the United States and to test it as a platform for sharing patient-generated health data with the CF health care team. METHODS: Focus groups with health care providers of patients with CF, adolescents with CF, and caregivers of children with CF were conducted to determine what modifications were necessary. Focus group data were analyzed using a thematic analysis, and emergent themes were ranked according to desirability and technical feasibility. The mHealth platform was then modified to meet the identified needs and preferences, and the flow of patient-generated health data to a secure Research Electronic Data Capture database was tested. Protocols for data management and clinical follow-up were also developed. RESULTS: A total of 5 focus groups with 21 participants were conducted. Recommended modifications pertained to all functionalities of the mHealth platform, including tracking of symptoms, treatments, and activities of daily care; creating and organizing medication lists and setting up reminders; generating reports for the health care team; language and presentation; sharing and privacy; and settings and accounts. Overall, health care providers recommended changes to align the mHealth platform with US standards of care, people with CF and their caregivers requested more tracking functionalities, and both groups suggested the inclusion of a mental health tracker as well as more detailed response options and precise language. Beta testers of the modified platform reported issues related to translatability to US environment and various bugs. CONCLUSIONS: This study demonstrated the importance of identifying the needs and preferences of target users and stakeholders before adopting existing mHealth solutions. All relevant perspectives, including those of clinicians, patients, and caregivers, should be thoroughly considered to meet both end users' needs and evidence-based practice recommendations.

Acute chest syndrome in pediatric sickle cell disease: Associations with racial composition and neighborhood deprivation.

BACKGROUND: Acute chest syndrome (ACS) is the leading cause of death for children with sickle cell disease (SCD). Recurrent ACS has detrimental effects on pulmonary health and health care costs. Neighborhood characteristics affect the outcomes of many pediatric chronic diseases, but their role in SCD is not well studied. In this study, we investigated the effects of area-level socioeconomic deprivation and racial composition on the recurrence of ACS. STUDY DESIGN: We performed a retrospective cross-sectional analysis of clinical data from a large pediatric SCD center. Patients' residential addresses were geocoded and linked to a composite area deprivation index (ADI) and percent African American population at the level of Census block groups. The association of recurrent ACS with neighborhood characteristics was evaluated using logistic regression analysis. RESULTS: The sample included 709 children with SCD. Residence in a socioeconomically deprived neighborhood was associated with 27% less risk of recurrent ACS, and residence in a predominantly African American neighborhood was associated with 41% less risk of ACS recurrence. The racial composition explained the protective effect of living in a high-deprivation area after adjusting for sociodemographic and clinical covariates. Demographic and clinical factors associated with recurrent ACS included older age, male gender, asthma, hydroxyurea use, and chronic transfusion therapy. CONCLUSIONS: This is the first study to report a protective effect of residing in a predominantly African American community for ACS recurrence. Further prospective studies are needed to confirm the association and to understand the mechanisms of such relationship.

Tobacco smoke exposure limits the therapeutic benefit of tezacaftor/ivacaftor in pediatric patients with cystic fibrosis.

OBJECTIVES: Tobacco smoke exposure reduces CFTR functional expression in vitro and contributes to acquired CFTR dysfunction. We investigated whether it also inhibits the clinical benefit of CFTR modulators, focusing on tezacaftor/ivacaftor, approved in February 2018 for individuals with CF age ≥12 years. METHODS: A retrospective longitudinal analysis of encounter-based data from the CF Foundation Patient Registry (2016-2018) compared the slope of change in lung function (GLI FEV1% predicted) before and after tezacaftor/ivacaftor initiation in smoke-exposed vs unexposed age-eligible pediatric patients. Tobacco smoke exposure (Ever/Never) was determined from caregiver self-report. Statistical analyses used hierarchical linear mixed modeling and fixed effects regression modeling. RESULTS: The sample included 6,653 individuals with a total of 105,539 person-period observations. Tezacaftor/ivacaftor was prescribed to 19% (1,251) of individuals, mean age 17 years, mean baseline ppFEV1 83%, 28% smoke-exposed. Tezacaftor/ivacaftor users who were smoke-exposed had a lower baseline ppFEV1 and experienced a greater lung function decline. Over two years, the difference in ppFEV1 by smoke exposure among tezacaftor/ivacaftor users increased by 1.2% (7.6% to 8.8%, p<0.001). In both mixed effects and fixed effects regression models, tezacaftor/ivacaftor use was associated with improved ppFEV1 among unexposed individuals (1.2% and 1.7%, respectively; p<0.001 for both) but provided no benefit among smoke-exposed counterparts (0.3%, p = 0.5 and 0.6%, p = 0.07, respectively). CONCLUSION: Tobacco smoke exposure nullifies the therapeutic benefit of tezacaftor/ivacaftor among individuals with CF aged 12-20 years old. To maximize the therapeutic opportunity of CFTR modulators, every effort must be taken to eliminate smoke exposure in CF.

Tobacco smoke exposure in pediatric cystic fibrosis: A qualitative study of clinician and caregiver perspectives on smoking cessation.

OBJECTIVE: Tobacco smoke exposure has negative impacts on the lung health of children with cystic fibrosis (CF), yet evidence-based strategies for smoking cessation have not been tested with or tailored to CF caregivers. This qualitative study identified barriers and facilitators of smoking cessation in this population and outlined potential interventional approaches. METHODS: We conducted semi-structured interviews with CF familial caregivers who were current or former smokers, and with members of the CF care team. We asked about experiences, practices, and prerequisites for a successful program. Interviews were recorded, transcribed verbatim, and coded by two investigators. Analysis used a thematic approach guided by the PRECEDE model, which identifies predisposing (intrapersonal), reinforcing (interpersonal), and enabling (structural) factors relevant to health behaviors and programs. RESULTS: Seventeen interviews were conducted-eight with familial caregivers and nine with CF team members. Whereas caregivers provided greater insight into internal difficulties and motivators to quit smoking, clinicians offered more extensive input on barriers and solutions related to the clinical environment. Based on study recommendations, a successful tobacco cessation program should include (a) family education about the harms of smoke exposure for children with CF; (b) screening for exposure, ideally with biochemical verification; (c) access to trained tobacco counselors; (d) affordable pharmacotherapy; and (e) outpatient follow-up of those undergoing tobacco treatment. CONCLUSION: This qualitative study revealed intrapersonal, interpersonal, and structural barriers to eliminating tobacco smoke exposure in children with CF, outlined opportunities to address these barriers, and made recommendations for a comprehensive tobacco cessation strategy.

Training Needs of Clinical and Research Professionals to Optimize Minority Recruitment and Retention in Cancer Clinical Trials.

The study of disparities in minority recruitment to cancer clinical trials has focused primarily on inquiries among minority patient populations. However, clinical trial recruitment is complex and requires a broader appreciation of the multiple factors that influence minority participation. One area that has received little attention is minority recruitment training for professionals who assume various roles in the clinical trial recruitment process. Therefore, we assessed the perspectives of cancer center clinical and research personnel on their training and education needs toward minority recruitment for cancer clinical trials. Ninety-one qualitative interviews were conducted at five U.S. cancer centers among four stakeholder groups: cancer center leaders, principal investigators, referring clinicians, and research staff. Interviews were recorded and transcribed. Qualitative analyses focused on response data related to training for minority recruitment for cancer clinical trials. Four prominent themes were identified: (1) Research personnel are not currently being trained to focus on recruitment and retention of minority populations; (2) Training for minority recruitment and retention provides for a specific focus on factors influencing minority research participation; (3) Training on cultural awareness may help to bridge cultural gaps between potential minority participants and research professionals; (4) Views differ regarding the importance of research personnel training designed to focus on recruitment of minority populations. There is a lack of systematic training for minority recruitment. Many stakeholders acknowledged the benefits of minority recruitment training and welcomed training that focuses on increasing cultural awareness to increase the participation of minorities in cancer clinical trials.

The Role of Perceived Discrimination in Obesity Among African Americans.

INTRODUCTION: African Americans, especially those in the South, suffer a disproportionate burden of obesity and are at high risk for perceived discrimination (PD). This study investigates the association between PD and weight status among African Americans and clarifies the role of perceived stress and health behaviors in this relationship. METHODS: Data came from the Jackson Heart Study, Examination 1 (2000-2004; analyses conducted in 2016 using Stata, version 14). African Americans from Jackson, Mississippi, aged 21-95 years were recruited (N=5,301). Weight status was measured using anthropometric data with BMI; waist circumference (in centimeters); and obesity class (I, II, III). Survey instruments were used to measure PD, perceived global stress, and health behaviors. Multivariate regression was used to model weight status outcomes as a function of PD, perceived stress, and health behaviors. RESULTS: After controlling for sociodemographic factors and health status, perceived everyday discrimination was associated with higher BMI (b=0.33, p<0.01); higher waist circumference (b=0.70, p<0.01); and higher relative risk of Class III obesity versus non-obesity (relative risk ratio, 1.18; p<0.001). Global perceived stress was linked to higher BMI (b=0.42, p<0.05) and higher waist circumference (b=1.18; p<0.01) and partially mediated the relationships between PD and these weight status outcomes. Health behaviors led to suppression rather than mediation between PD and weight status and between stress and weight status. CONCLUSIONS: PD and perceived stress are potential risk factors for higher weight status. They should be considered as a part of a comprehensive approach to reduce obesity among African Americans.