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2.
J Cyst Fibros ; 2024 Sep 13.
Artigo em Inglês | MEDLINE | ID: mdl-39327190

RESUMO

BACKGROUND: Rapidly emerging clinical trends offer the opportunity to amend guidance on issues pertaining to CF care delivery. A national survey was conducted to gather perspectives on CF care including potential adaptations to the care model to best meet the needs of this population. METHODS: A survey instrument was developed to capture perspectives on CF care. People with CF (pwCF), including those post lung transplant, caregivers and care teams were surveyed. Descriptive statistics were calculated to characterize respondents and responses. RESULTS: In-person, routine visits with the CF care teams were valued by survey respondents. However, reduced in-person visit frequency from the standard three-month interval was supported for individuals in a stable state of health. This was particularly true for pwCF ages two or older and on a modulator. Lung function, pulmonary exacerbation frequency, and transition periods were noted to influence preference for visit frequency. Integrating telehealth with remote monitoring in between visits was broadly supported. For shared care between CF teams and other medical providers (transplant teams and primary care providers (PCP)), good communication, easily accessible health records, and convenient locations were important. CONCLUSIONS: Survey findings support adapting CF care based on individual needs and life transitions. Themes identified can inform future areas of study and resource development to support successful modification of the CF care model and shared decision-making between patients and their care providers.

3.
J Cyst Fibros ; 2024 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-39327194

RESUMO

Interdisciplinary teams care for people with cystic fibrosis (pwCF) at specialized treatment centers. These teams have laid the foundation for the cystic fibrosis (CF) care model responsible for gains in health outcomes and quality of life within the CF community. However, the landscape of CF care is transforming, invigorated by new technologies, accessibility of cystic fibrosis transmembrane conductance regulator (CFTR) therapies, and increased utilization of telemedicine. In light of these advances, it is appropriate to re-evaluate the CF care team structure. This position paper offers guidance for the structure of a CF care center designed to meet the evolving needs of the CF community. Fundamental to the proposed center structure is recognition of pwCF and their families as integral members of their care teams, underpinning the necessity for shared decision making, awareness of social determinants of health, and active partnership between all healthcare professionals involved in the care of pwCF.

4.
Pediatrics ; 153(5)2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38577740

RESUMO

A multidisciplinary committee developed evidence-based guidelines for the management of cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen-positive, inconclusive diagnosis (CRMS/CFSPID). A total of 24 patient, intervention, comparison, and outcome questions were generated based on surveys sent to people with CRMS/CFSPID and clinicians caring for these individuals, previous recommendations, and expert committee input. Four a priori working groups (genetic testing, monitoring, treatment, and psychosocial/communication issues) were used to provide structure to the committee. A systematic review of the evidence was conducted, and found numerous case series and cohort studies, but no randomized clinical trials. A total of 30 recommendations were graded using the US Preventive Services Task Force methodology. Recommendations that received ≥80% consensus among the entire committee were approved. The resulting recommendations were of moderate to low certainty for the majority of the statements because of the low quality of the evidence. Highlights of the recommendations include thorough evaluation with genetic sequencing, deletion/duplication analysis if <2 disease-causing variants were noted in newborn screening; repeat sweat testing until at least age 8 but limiting further laboratory testing, including microbiology, radiology, and pulmonary function testing; minimal use of medications, which when suggested, should lead to shared decision-making with families; and providing communication with emphasis on social determinants of health and shared decision-making to minimize barriers which may affect processing and understanding of this complex designation. Future research will be needed regarding medication use, antibiotic therapy, and the use of chest imaging for monitoring the development of lung disease.


Assuntos
Fibrose Cística , Medicina Baseada em Evidências , Humanos , Fibrose Cística/terapia , Fibrose Cística/genética , Fibrose Cística/diagnóstico , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Testes Genéticos , Triagem Neonatal/métodos
5.
Pediatr Pulmonol ; 58(9): 2469-2477, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37265418

RESUMO

BACKGROUND: Elexacaftor/tezacaftor/ivacaftor (ETI) has been associated with unprecedented clinical improvements, transforming the management of cystic fibrosis (CF). However, side effects with implications for safety and well-being have been reported, including neuropsychiatric changes. This study aimed to better characterize the emerging positive and negative impacts of ETI. METHODS: The Cystic Fibrosis Foundation's Mental Health Advisory Committee distributed a 26-item survey to US CF care teams to assess clinician observations of patient-reported experiences with ETI. Survey responses measured the prevalence of these effects in five domains: (1) positive physical and psychological effects, (2) sleep difficulties, (3) cognitive difficulties, (4) worsening mental health, and (5) concerns about the future and finances. RESULTS: Seventy-five healthcare providers responded from a pediatric, adult, and combined centers. Positive physical effects of ETI and increased optimism were reported in the upper quartiles (50%-100%) and rated as having a significant impact on daily functioning. Sleep and cognitive difficulties were reported in 1%-24%, with slight impacts on functioning, and psychological symptoms (e.g., increased stress, depression, anxiety) and new psychiatric medications were reported in 1%-24%, with moderate impacts. Concerns about the future were reported in 1%-24%, with minimal impacts. CONCLUSION: Across US centers, providers most often observed positive physical effects of ETI. However, a variety of negative side effects were also reported, including sleep disruptions and worsening psychological functioning, which should be systematically monitored by CF teams. These national-level data are a first step in evaluating the prevalence and consequences of these side effects and can directly inform future studies.


Assuntos
Fibrose Cística , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Adulto , Humanos , Criança , Fibrose Cística/tratamento farmacológico , Pessoal de Saúde , Regulador de Condutância Transmembrana em Fibrose Cística , Aminofenóis/efeitos adversos , Benzodioxóis/efeitos adversos , Mutação
6.
Pediatr Pulmonol ; 58(6): 1768-1776, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37014155

RESUMO

OBJECTIVES: The CF Foundation sponsored competitive awards for Mental Health Coordinators (MHCs) from 2016 to 2018 to implement the international guidelines for mental health screening and treatment in US CF centers. Longitudinal surveys evaluated success in implementing these guidelines using the Consolidated Framework for Implementation Research (CFIR). METHODS: MHCs completed annual surveys assessing implementation from preparation/basic implementation (e.g., using recommended screeners) to full implementation/sustainability (e.g., providing evidence-based treatments). Points were assigned to questions through consensus, with higher scores assigned to more complex tasks. Linear regression and mixed effects models were used to: (1) examine differences in centers and MHC characteristics, (2) identify predictors of success, (3) model the longitudinal trajectory of implementation scores. RESULTS: A total of 122 MHCs (88.4% responded): Cohort 1, N = 80; Cohort 2, N = 30; and Cohort 3, N = 12. No differences in center characteristics were found. Significant improvements in implementation were observed across centers over time. Years of experience on a CF team was the only significant predictor of success; those with 1-5 years or longer reported the highest implementation scores. Change over time was predicted by >5 years of experience. CONCLUSIONS: Implementation of the mental health guidelines was highly successful over time. Funding for MHCs with dedicated time was critical. Longitudinal modeling indicated that CF centers with diverse characteristics could implement them, supported by evidence from the CF Patient Registry showing nearly universal uptake of mental health screening in the United States. Years of experience predicted better implementation, suggesting that education and training of MHCs and retention of experienced providers are critical to success.


Assuntos
Fibrose Cística , Humanos , Estados Unidos , Fibrose Cística/diagnóstico , Fibrose Cística/terapia , Fibrose Cística/psicologia , Saúde Mental , Programas de Rastreamento , Inquéritos e Questionários , Estudos Longitudinais
7.
J Cyst Fibros ; 20 Suppl 3: 31-38, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34930540

RESUMO

BACKGROUND: Depression and anxiety are two to four times more prevalent in people with CF (pwCF) than the general population. COVID-19 may exacerbate mental health challenges, increasing demand for psychological services, while decreasing their availability. We assessed the impact of the pandemic on depression and anxiety in pwCF, including how COVID-19 affected the frequency of mental health screening and the types of services provided. METHODS: A 38-item internet survey, completed in June 2020, assessed how COVID-19 affected: 1) the mental health clinician's role and screening processes; 2) barriers to screening and resource needs; 3) impact of COVID-19 on depression and anxiety, and 4) positive outcomes and confidence in sustaining mental health screening and treatment, including telehealth services, after the pandemic. RESULTS: Responses were obtained from 131 of the 289 US CF programs. Overall, 60% of programs (n=79) continued mental health screening and treatment, although less frequently; 50% provided individual tele-mental health interventions, and 9% provided telehealth group therapy. Clinically elevated depression symptoms (PHQ-9≥10; moderate to severe), were found in 12% of 785 pwCF, with 3.1% endorsing suicidal ideation. Similarly, elevated anxiety (moderate to severe; GAD-7≥10) was found in 13% of pwCF (n=779). CONCLUSIONS: The COVID-19 pandemic created an opportunity to implement innovative solutions to disruptions in mental health screening and treatment in CF programs. We found that pwCF had increased access to psychological interventions during the pandemic via telehealth, supporting the continued integration of tele-mental health screening and treatment into CF care.


Assuntos
Ansiedade , COVID-19 , Fibrose Cística , Depressão , Saúde Mental , Intervenção Psicossocial , Telemedicina , Ansiedade/diagnóstico , Ansiedade/fisiopatologia , Ansiedade/terapia , COVID-19/epidemiologia , COVID-19/prevenção & controle , COVID-19/psicologia , Fibrose Cística/epidemiologia , Fibrose Cística/psicologia , Fibrose Cística/terapia , Atenção à Saúde/métodos , Atenção à Saúde/tendências , Depressão/diagnóstico , Depressão/fisiopatologia , Depressão/terapia , Acessibilidade aos Serviços de Saúde/normas , Acessibilidade aos Serviços de Saúde/estatística & dados numéricos , Humanos , Programas de Rastreamento/métodos , Avaliação das Necessidades , Intervenção Psicossocial/métodos , Intervenção Psicossocial/tendências , Sistemas de Apoio Psicossocial , SARS-CoV-2 , Inquéritos e Questionários , Telemedicina/métodos , Telemedicina/organização & administração , Estados Unidos/epidemiologia
8.
Pediatr Pulmonol ; 55(11): 2995-3004, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32649006

RESUMO

BACKGROUND: A large-scale epidemiological study of 6088 individuals with cystic fibrosis (CF) and 4102 caregivers in nine countries documented elevated symptoms of depression and anxiety, leading to international guidelines for annual screening and follow-up. To facilitate national implementation, 84 CF programs funded a mental health coordinators (MHC). Implementation was evaluated after 1 year using the consolidated framework for implementation research (CFIR) to identify facilitators and barriers. METHODS: A 45-item internet survey was developed to assess relevant CFIR implementation steps. Surveys were completed in 2016. It assessed five domains tailored to study aims: (a) Intervention characteristics, (b) outer setting, (c) inner setting, (d) characteristics of individuals, and (e) process of implementation. RESULTS: Response rate was 88%, with pediatric and adult programs equally represented. A majority of MHCs were social workers (54.1%) and psychologists (41.9%); 41% had joined the team in the past year. Facilitators across the five domains included universal uptake of screening tools, greater awareness and detection of psychological symptoms, reduced stigma, and positive feedback from patients and families. Barriers included limited staff time, space, and logistics. DISCUSSION: This is the largest systematic effort to integrate mental health screening and treatment into the care of individuals with a serious, chronic illness and their caregivers. MHCs implementing screening, interpretation and follow-up reported positive results, and significant barriers. This national implementation effort demonstrated that depression and anxiety can be efficiently evaluated and treated in a complex, chronic disease. Future efforts include recommending the addition of screening scores to national CF Registries and examining their effects on health outcomes.


Assuntos
Fibrose Cística/psicologia , Programas de Rastreamento/métodos , Saúde Mental , Adulto , Instituições de Assistência Ambulatorial , Cuidadores , Criança , Humanos , Avaliação de Programas e Projetos de Saúde , Inquéritos e Questionários , Estados Unidos
9.
Health Soc Work ; 45(3): 202-210, 2020 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-32382728

RESUMO

To maximize health, individuals with cystic fibrosis (CF) follow a complex, burdensome daily care regimen. Managing CF is associated with a range of significant biopsychosocial challenges and places individuals with CF, and their caregivers, at greater risk for developing anxiety and depression. To promote wellness and address the potential barriers that affect management of this complex chronic illness, many individuals would benefit from treatment from a behavioral health provider. Social workers within multidisciplinary CF care teams are well positioned to respond to this need, and an expanding number of social workers serving as behavioral health providers in the community will be sought as a resource to provide treatment to this population. This article serves as a primer for social workers to maximize knowledge of the psychosocial and potential behavioral health needs of individuals with CF across the life span. To best support individuals with CF, authors describe the disease-specific manifestations and outline the numerous potential clinical targets for social work to promote wellness. The article concludes by highlighting the importance of communication with the medical team and considerations for effective collaborative care.


Assuntos
Continuidade da Assistência ao Paciente , Fibrose Cística/terapia , Promoção da Saúde , Serviços de Saúde Mental , Assistentes Sociais/psicologia , Cooperação e Adesão ao Tratamento , Ansiedade/psicologia , Fibrose Cística/psicologia , Depressão/psicologia , Humanos , Qualidade de Vida/psicologia
10.
J Patient Exp ; 7(6): 1748-1754, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33457639

RESUMO

We sought to evaluate the feasibility, acceptability, and benefits of a virtual one-to-one peer support program for people with cystic fibrosis and their family members through a retrospective program evaluation. This peer support program was developed in collaboration with patients, health care providers, and CF Foundation program staff. Mentees were paired with a trained peer mentor for 3-month mentoring via video, phone, email, or text. We found that the peer support program was feasible and acceptable. Success factors include a range of positive benefits including practical support as well as social and emotional support. Two-thirds of mentees reported at least 4 different benefits. Mentors reported multiple benefits after providing support through mentoring. Our program evaluation demonstrates that virtual peer support based on informal sharing of life experiences is an achievable way to provide social support and enhance health and well-being in chronic disease management.

11.
J Cyst Fibros ; 14(4): 497-506, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25577127

RESUMO

BACKGROUND: Vitamin D deficiency is common in CF. Whether vitamin D affects pulmonary function in CF is unknown. METHODS: Data were abstracted from clinically stable CF patients who had pulmonary function studies and serum 25-hydroxyvitamin D [25(OH)D, ng/ml] levels drawn within 2 months of each other. Findings were adjusted for multiple variables known to affect pulmonary function in CF. RESULTS: Enrollees totaled 597. Overall mean 25(OH)D level was 29.6±12.8 ng/ml (SD). Serum 25(OH)D levels showed a significant correlation with forced expiratory volume in 1s (FEV1) % predicted (r=0.20, p<0.0001) and forced vital capacity % predicted (r=0.13, p=0.0019). Multivariate analysis revealed that serum 25(OH)D remained an independent predictor of FEV1 % predicted even after controlling for multiple other factors known to affect CF lung function. CONCLUSIONS: Serum 25(OH)D levels are significantly associated with pulmonary function in CF. Further study is required to determine whether this association is causal.


Assuntos
Fibrose Cística/complicações , Fibrose Cística/fisiopatologia , Deficiência de Vitamina D/complicações , Deficiência de Vitamina D/fisiopatologia , Adolescente , Adulto , Criança , Fibrose Cística/sangue , Feminino , Volume Expiratório Forçado/fisiologia , Humanos , Masculino , Estudos Retrospectivos , Capacidade Vital/fisiologia , Vitamina D/análogos & derivados , Vitamina D/sangue , Deficiência de Vitamina D/sangue , Adulto Jovem
12.
Ther Adv Respir Dis ; 8(2): 39-47, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24594976

RESUMO

Nonadherence to inhaled therapies is a major problem in the treatment of cystic fibrosis that can influence lung function and health outcomes. Potential contributors to nonadherence have been identified, including demographic and psychosocial factors, time and convenience of inhaled therapy, and treatment beliefs. Additional research is clearly needed to clarify the contributors and to determine which interventions and technological advances will enhance adherence to inhaled therapies in patients with cystic fibrosis. Nurses and allied health professionals are ideally positioned to assist patients and families with adherence to inhaled therapies through monitoring, communication, and education about the available therapies and their proper use. This review briefly summarizes the available evidence about contributors to nonadherence, potential interventions, novel delivery devices for inhaled therapies, and opportunities for additional research.


Assuntos
Fibrose Cística/tratamento farmacológico , Adesão à Medicação , Medicamentos para o Sistema Respiratório/administração & dosagem , Administração por Inalação , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Nebulizadores e Vaporizadores , Educação de Pacientes como Assunto , Medicamentos para o Sistema Respiratório/efeitos adversos , Resultado do Tratamento
13.
Am J Nurs ; 110(8): 30-7; quiz 38-9, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20671493

RESUMO

OBJECTIVE: A survey was conducted to estimate how many adult patients with cystic fibrosis who receive care at centers accredited by the Cystic Fibrosis Foundation (CFF) also have biological children with the disease. METHODS: A six-item questionnaire was sent to 144 CFF-accredited care centers and satellites. After one month, a reminder e-mail was sent to the nonresponding centers. RESULTS: A total of 119 responses were received. Forty (34%) of the responding centers reported having at least one and as many as five patients with cystic fibrosis who also had biological children with the disease. A total of 66 such patients were identified. Of these, 39 (59%) were newly diagnosed themselves as a consequence of prenatal testing or a child's diagnosis. CONCLUSION: Despite some study limitations, these findings show that a number of people with cystic fibrosis are having children who also have the disease. Moreover, many parents are learning that they have cystic fibrosis only after their child is diagnosed. Further research is needed to better estimate both the proportion of adults with cystic fibrosis who are having children with the disease and the proportion late-diagnosed, as well as to explore the psychosocial needs of these patients and their children. KEYWORDS: cystic fibrosis, cystic fibrosis research, family planning, genetic testing, carrier testing, newborn screening.


Assuntos
Fibrose Cística/prevenção & controle , Saúde da Família , Testes Genéticos , Adulto , Fibrose Cística/epidemiologia , Fibrose Cística/genética , Fibrose Cística/enfermagem , Diagnóstico Tardio/prevenção & controle , Feminino , Triagem de Portadores Genéticos , Inquéritos Epidemiológicos , Humanos , Recém-Nascido , Triagem Neonatal , Gravidez , Diagnóstico Pré-Natal , Apoio Social , Estados Unidos/epidemiologia
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