Patient-reported symptoms and functioning as indicators of mortality in advanced cystic fibrosis: A new tool for referral and selection for lung transplantation

Solé, Amparó; Pérez, Irene Lacruz; Vázquez, Isabel; Pastor, A.; Escrivá, Juan; Sales, G.; Hervás, David; Glanville, Allan R.; Quittner, Alexandra L.

doi:10.1016/j.healun.2016.01.1233

Cited by 23 publications

(15 citation statements)

References 32 publications

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“…following correction for multiple comparisons; these potential associations will be important to clarify in future studies using larger samples, particularly given that low scores on the Physical Functioning scale of the CFQ-R predict long-term decline and mortality in CF [13].…”

Section: Discussionmentioning

confidence: 99%

Screening for ADHD in adults with cystic fibrosis: Prevalence, health-related quality of life, and adherence

Georgiopoulos

Friedman

Porter

et al. 2018

Journal of Cystic Fibrosis

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The highly specific screening tool ASRS-v1.1 can ascertain previously undetected ADHD symptoms in adults with CF. ADHD was substantially more prevalent than expected in this population. Elevated ASRS-v1.1 screens correlated with poorer Health-Related Quality of Life (HRQoL) in some domains, but not with BMI, FEV1%pred, or self-reported CF treatment adherence. Additional research will elucidate the impact of ADHD and its treatment on HRQoL, CF self-care and health outcomes.

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Section: Discussionmentioning

confidence: 99%

Screening for ADHD in adults with cystic fibrosis: Prevalence, health-related quality of life, and adherence

Georgiopoulos

Friedman

Porter

et al. 2018

Journal of Cystic Fibrosis

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“…Markers of shortened survival include low FEV 1 , 6-min walk test (6MWT) distance b400 m, room air hypoxemia (SpO 2 b 88% or P a O 2 b 55 mmHg, at rest or with exertion; at sea level), hypercarbia (P a CO 2 N 50 mmHg, confirmed on arterial blood gas), pulmonary hypertension (PA systolic pressure N 50 mmHg on echocardiogram or evidence of right ventricular dysfunction in the absence of a tricuspid regurgitant jet), BMI b18 kg/m 2 for adults (or BMI less than 5th percentile for children), increased frequency of pulmonary exacerbations (N2 exacerbations per year requiring IV antibiotics or one exacerbation requiring positive pressure ventilation), massive hemoptysis, or pneumothorax [1,[12][13][14][15][16][17][18][19]25,43,44]. Additionally, a low physical functioning score on the CFQ-R questionnaire has been associated with reduced survival and, while there was no consensus recommendation for use of CFQ-R in routine clinical practice, a Physical score b 30 could be considered alongside other markers of shortened survival in the decision to refer for transplant [44,45].…”

Section: Ormentioning

confidence: 99%

Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines

Ramos

Smith

McKone

et al. 2019

Journal of Cystic Fibrosis

179

145

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Objective: Provide recommendations to the cystic fibrosis (CF) community to facilitate timely referral for lung transplantation for individuals with CF. Methods: The CF Foundation organized a multidisciplinary committee to develop CF Lung Transplant Referral Consensus Guidelines. Three workgroups were formed: timing for transplant referral; modifiable barriers to transplant; and transition to transplant care. A focus group of lung transplant recipients with CF and spouses of CF recipients informed guideline development. Results: The committee formulated 21 recommendation statements based on literature review, committee member practices, focus group insights, and in response to public comment. Critical approaches to optimizing access to lung transplant include early discussion of this treatment option, assessment for modifiable barriers to transplant, and open communication between the CF and lung transplant centers. Conclusions: These guidelines will help CF providers counsel their patients and may reduce the number of individuals with CF who die without consideration for lung transplant.

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“… 49 Authors suggested that it could predict survival 50 and be a determinant for lung transplantation. 51 Content validity was acceptable. 25 52 …”

Section: Resultsmentioning

confidence: 95%

A systematic review of patient-reported outcome measures (PROMs) in cystic fibrosis

2020

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BackgroundTo determine patient-reported outcome measures (PROMs) which may be suitable for incorporation into the Australian Cystic Fibrosis Data Registry (ACFDR) by identifying PROMs administered in adult and paediatric cystic fibrosis (CF) populations in the last decade.MethodsWe searched MEDLINE, EMBASE, Scopus, CINAHL, PsycINFO and Cochrane Library databases for studies published between January 2009 and February 2019 describing the use of PROMs to measure health-related quality of life (HRQoL) in adult and paediatric patients with CF. Validation studies, observational studies and qualitative studies were included. The search was conducted on 13 February 2019. The COnsensus-based Standards for the selection of health Measurement INstruments Risk of Bias Checklist was used to assess the methodological quality of included studies.ResultsTwenty-seven different PROMs were identified. The most commonly used PROMs were designed specifically for CF. Equal numbers of studies were conducted on adult (32%, n=31), paediatric (35%, n=34) and both (27%, n=26) populations. No PROMs were used within a clinical registry setting previously. The two most widely used PROMs, the Cystic Fibrosis Questionnaire—Revised (CFQ-R) and the Cystic Fibrosis Quality of Life Questionnaire (CFQoL), demonstrated good psychometric properties and acceptability in English-speaking populations.DiscussionWe found that although PROMs are widely used in CF, there is a lack of reporting on the efficacy of methods and timepoints of administration. We identified the CFQ-R and CFQoL as the most suitable for incorporation in the ACFDR as they captured significant effects of CF on HRQoL and were reliable and valid in CF populations. These PROMs will be used in a further qualitative study assessing patients’ with CF and clinicians’ perspectives toward the acceptability and feasibility of incorporating a PROM in the ACFDR.PROSPERO registration numberCRD42019126931.

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Patient-reported symptoms and functioning as indicators of mortality in advanced cystic fibrosis: A new tool for referral and selection for lung transplantation

Cited by 23 publications

References 32 publications

Screening for ADHD in adults with cystic fibrosis: Prevalence, health-related quality of life, and adherence

Screening for ADHD in adults with cystic fibrosis: Prevalence, health-related quality of life, and adherence

Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines

A systematic review of patient-reported outcome measures (PROMs) in cystic fibrosis

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