Changes in Clinical Markers During A Short-Term Transfer Program of Adult Cystic Fibrosis Patients from Pediatric to Adult Care

Background: Transition from child-oriented to adult-oriented health care in Cystic Fibrosis (CF) has become more important over recent decades as the survival of people with this disease has increased. The transition process usually begins in adolescence, with full transfer completed in early adulthood. Objective: This study investigated the impact of a short-term transfer program on clinical markers in an adult CF cohort still being managed by pediatricians. Methods: Clinically relevant data from the year before (T-1), the time of Transfer (T) and the year after the transfer (T+1) were analysed retrospectively. Results: 39 patients (median age 29.0 years; 64% male) were transferred between February and December 2016. Lung function had declined significantly in the year before transfer (in % predicted: Forced Expiratory Volume in 1 second (FEV), 62.8 vs. 57.7, p <0.05; Forced Vital Capacity (FVC), 79.9 vs. 71.1, p<0.05), but remained stable in the year after transfer (in % predicted: FEV: 56.3; FVC 68.2). BMI was stable over the whole observational period. There was no relevant change in chronic lung infection with P. aeruginosa, Methicillin-Resistant Staphylococcus aureus (MRSA) and Burkholderia sp. during the observation period. The number of patient contacts increased significantly in the year after versus the year before transfer (inpatient: 1.51 vs. 2.51, p<0.05; outpatient: 2.67 vs. 3.41, p<0.05). Conclusions: Our data show that, within the framework of a structured transfer process, it is possible to transfer a large number of adult CF patients, outside a classic transition program, from a pediatric to an adult CF center in a short period of time, without any relevant changes in clinical markers and, stability.

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The Impact of Special Allocation Fund (DAK) for Health on Achievements of Health Development Performance in Indonesia

Economies ◽

10.3390/economies8030072 ◽

2020 ◽

Vol 8 (3) ◽

pp. 72

Author(s):

Agnes Putri Apriliani ◽

Khoirunurrofik Khoirunurrofik

Keyword(s):

Fixed Effects ◽

Structural Breaks ◽

Health Financing ◽

Health Care Facilities ◽

Future Research ◽

Fixed Effects Model ◽

Short Term ◽

Health Development ◽

Short Period ◽

The Impact

In the implementation of decentralization, the Special Allocation Fund (DAK) for health is given to certain areas of Indonesia to support health financing. The performance of this financing, along with national health development priorities’ achievements, is illustrated through the indicators of coverage of deliveries in health care facilities (PF) and coverage of first neonatal visits (KN1). Yearly increases in the health DAK budget have not been accompanied by increases in these coverages, and there are still significant disparities between regions. Using secondary data at the district/city level for 2014–2017, this study aims to investigate the impact of health DAK on coverage of PF and KN1. The analytical method deployed is linear regression of panel data using a fixed-effects model. The results show that in the short term, health DAK has a positive but insignificant effect on PF and KN1 coverage. However, health DAK has a positive and significant impact on PF coverage in the second year. Impact on KN1 coverage is unfeasible, even over a period of two years. These results indicate that the processes of planning, budgeting, and administering of health DAK require improvement so that benefits can be felt in the short term through better innovations in health programs. Nevertheless, given that our findings are based on a short period of study, the results from such analyses should consequently be treated with the utmost caution Therefore, future research should target a longer period of data collection to detect more trusty lagged effects and structural breaks of a policy intervention.

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A need-adapted transition program after pediatric kidney transplantation

Journal of Transition Medicine ◽

10.1515/jtm-2018-0004 ◽

2019 ◽

Vol 1 (1) ◽

Cited By ~ 1

Author(s):

Susanne Rieger ◽

Dirk Bethe ◽

Angela Bagorda ◽

Dorothea Treiber ◽

Jörg Beimler ◽

...

Keyword(s):

Renal Transplant ◽

Transition Process ◽

Transition Program ◽

Renal Transplant Recipients ◽

Transplant Recipients ◽

Adult Care ◽

Increased Risk ◽

Pediatric Renal Transplant ◽

The Individual ◽

The Impact

AbstractA successful transition of renal transplant recipients from pediatric to adult care requires a structured, need-adapted and multidisciplinary approach to preserve renal graft function during this critical period of life. In this article we present our clinical protocol for transition from pediatric to adult care, which we developed on the basis of the International Society of Nephrology (ISN)/International Pediatric Nephrology Association (IPNA) consensus guidelines influenced by our own experience. This transition program was established in our center in July 2017. The entire transition process is structured and accompanied by a transition key worker (social worker). From 12 years of age we train pediatric renal transplant recipients in medical knowledge, self-management skills and networking with self-help groups. The training is adapted to the individual patient‘s intellectual ability, lasts about 10 years and takes place with increasing intensity. Repeatedly we perform standardized informational interviews and check patient’s knowledge of transplant-related topics. Psychosocial and educational issues are evaluated concomitantly. The actual transfer takes place in a pediatric-adult-transition clinic. Relevant medical and psychosocial aspects are discussed and the future treatment regimen is established. The date of transfer is adapted to the individual patient’s need; it varies between 18 and 24 years of age. In periods of increased risk for non-adherence the transfer is postponed to intensify the efforts for training and assistance. After transfer a standardized evaluation of each individual patient takes place focusing on medical and psychosocial issues and on satisfaction with the transition process. Collection of these data is still in progress and will be analyzed systematically at a later stage in order to evaluate the impact of this new transition program on the stability of transplant function. That analysis might serve as a basis for negotiations about refunding with health insurance companies.

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Lung function over the life course of paediatric and adult patients with cystic fibrosis from a large multi-centre registry

Scientific Reports ◽

10.1038/s41598-020-74502-1 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Arul Earnest ◽

Farhad Salimi ◽

Claire E. Wainwright ◽

Scott C. Bell ◽

Rasa Ruseckaite ◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Function ◽

Mixed Model ◽

Linear Mixed Model ◽

Pancreatic Insufficiency ◽

Forced Expiratory Volume ◽

Rate Of Change ◽

Data Registry ◽

Mortality Outcome ◽

The Impact

Abstract A key measure of lung function in people with Cystic Fibrosis (CF) is Forced Expiratory Volume in the first second FEV1 percent predicted (FEV1pp). This study aimed to address challenges in identifying predictors of FEV1pp, specifically dealing with non-linearity and the censoring effect of death. Data was obtained from a large multi-centre Australian Cystic Fibrosis Data Registry (ACFDR). A linear mixed model was used to study FEV1pp as the endpoint. There were 3655 patients (52.4% male) included in our study. Restricted cubic splines were used to fit the non-linear relationship between age of visit and FEV1pp. The following predictors were found to be significant in the multivariate model: age of patient at visit, BMI z-score, age interaction with lung transplantation, insulin dependent diabetes, cirrhosis/portal hypertension, pancreatic insufficiency, Pseudomonas aeruginosa infection and baseline variability in FEV1pp. Those with P. aeruginosa infection had a lower mean difference in FEV1pp of 4.7 units, p < 0.001 compared to those who did not have the infection. Joint modelling with mortality outcome did not materially affect our findings. These models will prove useful for to study the impact of CFTR modulator therapies on rate of change of lung function among patients with CF.

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Using different methods to process forced expiratory volume in one second (FEV1) data can impact on the interpretation of FEV1 as an outcome measure to understand the performance of an adult cystic fibrosis centre: A retrospective chart review

F1000Research ◽

10.12688/f1000research.14981.1 ◽

2018 ◽

Vol 7 ◽

pp. 691

Author(s):

Zhe Hui Hoo ◽

Muhaned S.A. El-Gheryani ◽

Rachael Curley ◽

Martin J. Wildman

Keyword(s):

Cystic Fibrosis ◽

Data Processing ◽

Outcome Measure ◽

Forced Expiratory Volume ◽

P Value ◽

Processing Methods ◽

The Impact ◽

Height Data ◽

Median Change ◽

Data Processing Methods

Background: Forced expiratory volume in one second (FEV1) is an important cystic fibrosis (CF) prognostic marker and an established endpoint for CF clinical trials. FEV1 is also used in observation studies, e.g. to compare different centre’s outcomes. We wished to evaluate whether different methods of processing FEV1 data can impact on a centre’s outcome. Methods: This is a single-centre retrospective analysis of routinely collected data from 2013-2016 which included 208 adults with CF. Year-to-year %FEV1 change was calculated by subtracting best %FEV1 at Year 1 from Year 2 (i.e. negative values indicate %FEV1 decline), and compared using Friedman test. Three methods were used to process %FEV1 data. First, %FEV1 calculated with Knudson equation was extracted directly from spirometer machines. Second, FEV1 volume were extracted then converted to %FEV1 using clean height data and Knudson equation. Third, FEV1 volume were extracted then converted to %FEV1 using clean height data and GLI equation. In addition, %FEV1 decline calculated using GLI equation was adjusted for baseline %FEV1 to understand the impact of case-mix adjustment. Results: There was a trend of reduction in %FEV1 decline with all three data processing methods but the magnitude of %FEV1 decline differed. Median change in %FEV1 for 2013-2014, 2014-2015 and 2015-2016 was –2.0, –1.0 and 0.0 respectively using %FEV1 in Knudson equation whereas the median change was –1.1, –0.9 and –0.3 respectively using %FEV1 in the GLI equation. A statistically significant p-value (0.016) was only obtained when using %FEV1 in Knudson equation extracted directly from spirometer machines. Conclusions: Although the trend of reduction in %FEV1 decline was robust, different data processing methods yielded varying results when %FEV1 decline was compared using a standard related group non-parametric statistical test. Observational studies with %FEV1 decline as an outcome measure should carefully consider and clearly specify the data processing methods used.

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Cricothyroidotomy: The Impact of Antecedent Endotracheal Intubation

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348948209100423 ◽

1982 ◽

Vol 91 (4) ◽

pp. 437-439 ◽

Cited By ~ 21

Author(s):

Ernest A. Weymuller ◽

Charles W. Cummings

Keyword(s):

Airway Management ◽

Surgical Intervention ◽

Current Debate ◽

Short Term ◽

Airway Control ◽

Prolonged Intubation ◽

Underlying Factor ◽

Short Period ◽

Life Threatening ◽

The Impact

In light of the current debate regarding cricothyroidotomy, we elected to study the procedure at our institution. Cricothyroidotomy was instituted whenever tracheotomy was necessary for airway management. After a fairly short period of time, some significant complications of cricothyroidotomy were apparent and the study was aborted prior to achieving statistically significant results. The report reviews 15 consecutive cricothyroidotomies. Five (33%) developed significant complications requiring surgical intervention. Of the five, two had life-threatening airway obstruction. The major underlying factor in patients who developed complications was prolonged intubation prior to the institution of cricothyroidotomy. The study suggests that cricothyroidotomy should not be performed after prolonged intubation. The issue of primary cricothyroidotomy for short-term airway control remains unanswered.

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Efficacy of Glutathione for Patients With Cystic Fibrosis: A Meta-analysis of Randomized-Controlled Studies

American Journal of Rhinology and Allergy ◽

10.1177/1945892419878315 ◽

2019 ◽

Vol 34 (1) ◽

pp. 115-121 ◽

Cited By ~ 2

Author(s):

Jinqiu Zhao ◽

Wenxiang Huang ◽

Shujun Zhang ◽

Jing Xu ◽

Wei Xue ◽

...

Keyword(s):

Cystic Fibrosis ◽

Pulmonary Function ◽

Positive Impact ◽

Meta Analysis ◽

Forced Expiratory Volume ◽

Cochrane Library ◽

Control Group ◽

Randomized Controlled ◽

Randomized Controlled Studies ◽

The Impact

Introduction The impact of glutathione on pulmonary function remains elusive for patients with cystic fibrosis. The aim of this systematic review and meta-analysis is to explore the influence of glutathione versus placebo on pulmonary function of cystic fibrosis. Methods We search PubMed, EMbase, Web of science, EBSCO, and Cochrane library databases through May 2019, and randomized-controlled trials (RCTs) regarding the effect of glutathione on pulmonary function of cystic fibrosis are included in this meta-analysis. Results Four RCTs are included. Compared with control group in patients with cystic fibrosis, glutathione treatment shows positive impact on forced expiratory volume 1 second (FEV1) (mean difference [MD] = 0.19; 95% confidence interval (CI), 0.10–0.28; P < .0001) and body mass index (MD = 0.27; 95% CI, 0.02–0.51; P = .03), but has no obvious influence on 6-minute walk test (standard MD = 0.28; 95% CI, −0.08 to 0.64; P = .13), number of exacerbations (MD = −0.10; 95% CI, −0.34 to 0.15; P = .43), abdominal pain or distal intestinal obstruction (risk ratios [RR] = 0.78; 95% CI, 0.32–1.90; P = .58), or hemoptysis (RR = 1.87; 95% CI, 0.43–8.26; P = .41). Conclusions Glutathione treatment provides some benefits to improve pulmonary function of patients with cystic fibrosis, as evidenced by the increase in FEV1.

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The Impact of Short-Term Dietary Restriction on Stem Cell Function

Innovation in Aging ◽

10.1093/geroni/igab046.443 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. 116-117

Author(s):

Archana Unnikrishnan

Keyword(s):

Stem Cells ◽

Stem Cell ◽

Dietary Restriction ◽

Cell Function ◽

Aging Effects ◽

Short Term ◽

Regenerative Capacity ◽

Short Period ◽

The Impact ◽

Old Mice

Abstract Stem cells play a critical role in the maintenance of tissue function and their proliferative/regenerative capacity is essential to this role. Because stem cells persist over the lifespan of an animal, they are susceptible to gradual accumulation of age-associated damage, resulting in the loss of regenerative function that can impair organ function. Understanding the mechanism(s) that regulates stem cell function is essential for retarding the aging process, and stem cells are attractive targets for aging interventions. Dietary restriction (DR), the most robust anti-aging intervention to-date, has been shown to enhance the activity and integrity of stem cells in a variety of tissues (e.g., muscle, bone marrow, and intestine), and it is believed that effect of DR on stem cells plays an important role in the anti-aging action of DR. For example, DR has been shown to preserve and increase the number of intestinal stem cells (ISCs) and enhance their regenerative capacity in young animals. Data from my lab shows that ISCs from old mice have limited proliferation activity and form few if any organoids in vitro (a surrogate for a fully functional crypt) and that ISCs isolated from old mice on life-long DR show an improved ability to form organoids. While it is well accepted that life-long DR increases lifespan and has anti-aging effects an important aspect of DR that has been largely overlooked is that DR implemented only for a short time early in life can increase lifespan of rodents even when rodents are fed ad libitum the remainder of their life. In line with this, we recently found that ISCs from old mice fed DR for only a short-period resulted in a dramatic increase in ability of the ISCs to form organoids. This is the first evidence that short-term DR administrated late in life can rescue the loss in ISC function that occurs with age.

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The impact of 12 months treatment with ivacaftor on Scottish paediatric patients with cystic fibrosis with the G551D mutation: a review

Archives of Disease in Childhood ◽

10.1136/archdischild-2015-310420 ◽

2016 ◽

Vol 103 (1) ◽

pp. 68-70 ◽

Cited By ~ 7

Author(s):

Carol Dryden ◽

Jane Wilkinson ◽

David Young ◽

Richard John Brooker

Keyword(s):

Body Mass Index ◽

Cystic Fibrosis ◽

Published Data ◽

Oral Antibiotics ◽

Cataract Formation ◽

Short Term ◽

Sweat Chloride ◽

Paediatric Patients ◽

Intravenous Antibiotics ◽

The Impact

We reviewed the impact of ivacaftor on Scottish paediatric patients with cystic fibrosis ≥6 years of age after 12 months of treatment. Statistically significant improvements in FEV1 and body mass index and a reduction in sweat chloride, all comparable with previously published data were observed. The findings also suggested reduced use of intravenous antibiotics and oral antibiotics. No significant adverse effects were observed but a possible association with cataract formation could not be excluded. This review suggests that, in the short term at least, ivacaftor is effective and safe in paediatric patients ≥6 years of age with G551D.

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Impact of Achromobacter xylosoxidans isolation on the respiratory function of adult patients with cystic fibrosis

ERJ Open Research ◽

10.1183/23120541.00051-2019 ◽

2019 ◽

Vol 5 (4) ◽

pp. 00051-2019 ◽

Cited By ~ 1

Author(s):

Macha Tetart ◽

Frederic Wallet ◽

Maeva Kyheng ◽

Sylvie Leroy ◽

Thierry Perez ◽

...

Keyword(s):

Cystic Fibrosis ◽

Respiratory Function ◽

Forced Expiratory Volume ◽

Adult Patients ◽

Achromobacter Xylosoxidans ◽

First Year ◽

Long Term Effects ◽

Lung Isolation ◽

The Impact

BackgroundThe prevalence of Achromobacter xylosoxidans lung isolation in cystic fibrosis (CF) patients has increased, but the impact on lung function is controversial. The aim of this study was to evaluate the long-term effects of A. xylosoxidans isolation on respiratory function of adult patients with CF in the first 3 years after identification of A. xylosoxidans isolation.MethodsThis was a case–control retrospective study performed at a single CF centre in Lille, France. Data for 36 patients with CF who had at least one sputum culture positive for A. xylosoxidans (Ax+) were evaluated and compared with control CF patients uninfected by A. xylosoxidans (Ax−). Respiratory function and exacerbation frequency were evaluated between 1 year prior to and 3 years after A. xylosoxidans isolation.ResultsCompared with the Ax− group, the Ax+ group had a lower forced expiratory volume in 1 s (FEV1) at baseline (median (interquartile range): 55.2% (50.6–59.8%) versus 73.8% (67.2–80.4%); p=0.005), a greater decline in FEV1 (±se) in the first year after A. xylosoxidans identification (−153.6±16.1 mL·year−1versus −63.8±18.5 mL·year−1; p=0.0003), and more exacerbations in the first 3 years after A. xylosoxidans identification (9 (7–12) versus 7 (5–10); p=0.03). Ax+ patients co-colonised with Pseudomonas aeruginosa (n=27, 75%) had a greater FEV1 decline (p=0.003) and more exacerbations in the year after A. xylosoxidans identification (p=0.037) compared with patients colonised with A. xylosoxidans alone. Patients with chronic A. xylosoxidans isolation (n=23, 64%) had more exacerbations than intermittently colonised patients in the 3 years after A. xylosoxidans identification (p=0.012).ConclusionA. xylosoxidans isolation is associated with a decline in respiratory function in patients with CF. Chronic A. xylosoxidans isolation and P. aeruginosa co-isolation may be markers of more severe respiratory disease in Ax+ patients.

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Practitioner Due Diligence: Real-World Lumacaftor/Ivacaftor Use

The Journal of Pediatric Pharmacology and Therapeutics ◽

10.5863/1551-6776-25.5.431 ◽

2020 ◽

Vol 25 (5) ◽

pp. 431-436

Author(s):

Emily M. Stephan ◽

Christopher J. Nemastil ◽

Ann Salvator ◽

Susan Gemma ◽

Clarissa J. Dilaveris ◽

...

Keyword(s):

Health Care ◽

Body Mass Index ◽

Cystic Fibrosis ◽

Health Care Utilization ◽

Body Mass ◽

Real World ◽

Forced Expiratory Volume ◽

Emergency Department Visits ◽

Care Utilization ◽

The Impact

OBJECTIVE Previous trials evaluated the efficacy of lumacaftor/ivacaftor in Phe508del homozygotes. These trials are limited by manufacturer sponsorship and were conducted under strict protocol. Additionally, this therapy is costly and does not allow for reduction in daily cystic fibrosis therapies. This study assessed the efficacy of lumacaftor/ivacaftor therapy and its effect on health care utilization in a real-world setting. METHODS Retrospective chart review comparing the first 12 months of therapy to the 24 months prior was conducted to evaluate the impact of lumacaftor/ivacaftor on pulmonary function following a streamlined process for therapy introduction. The impact on body mass index and healthcare utilization were also evaluated. The following measurements were assessed: percent predicted forced expiratory volume in 1 second, body mass index and z-scores, number of admissions, length of stay, number of emergency department visits. RESULTS Mean ppFEV1 was improved for the first 12 months on lumacaftor/ivacaftor treatment when compared with the 24 months prior: 78.8 (95% CI: 72.6, 84.9) vs 76.2 (95% CI: 70.1, 82.3) (p = 0.03). Body mass index significantly improved (patients ≥20 years), but improvement in BMI z-score (patients <20 years) was not significant. Number of admissions and LOS were significantly decreased, but ED visits were not. CONCLUSIONS Lumacaftor/ivacaftor is effective for improving ppFEV1 and BMI and for reducing health care utilization. However, this small reduction does not overcome the financial cost of treatment. Long-term outcomes and use must be studied to determine the overall effect of this therapy on cystic fibrosis interventions and their costs.

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