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Research paper thumbnail of Evolution of nutritional management in children with cystic fibrosis – a narrative review

Journal of human nutrition and dietetics, Apr 25, 2024

Research paper thumbnail of Author response for "Evolution of nutritional management in children with cystic fibrosis – a narrative review

Research paper thumbnail of Epidemiology and management of nontuberculous mycobacterial disease in people with cystic fibrosis, the Netherlands

Journal of Cystic Fibrosis

Research paper thumbnail of Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis

Journal of Cystic Fibrosis

Research paper thumbnail of WS16-3 Newborn screening for cystic fibrosis in the Netherlands: effects on clinical outcome at age six

Journal of Cystic Fibrosis, 2019

(NBS) programs has allowed a better monitoring of the disease incidence, which appears lower than... more (NBS) programs has allowed a better monitoring of the disease incidence, which appears lower than in the past. This study assesses time trends in incidence of CF over a 43-year period in an area where CF is frequent (Brittany, France) and where NBS is implemented since 30 years. Methods: This study enrolled patients with classical form of CF born in Brittany between January 1 st 1975 and December 31 st 2017 (n = 554). Patients born before the set up of NBS in 1989 were registered through active enquiries and combination of data sources. Time trends in incidence were examined using Poisson regression and expressed using the average percent change (APC) over time or between two periods of interest. Results: The overall incidence of CF was estimated at 1/2811 live births over the study period. Poisson regression showed that the incidence rate decreased significantly over the whole period (annual APC: −1.8%, 95% CI: [−2.5%; −1.2%], p < 0.0001). The incidence rates dropped from 1/1983 over the first 5-y. period (1975-79) to 1/5046 over the last one (2013-17), what corresponded to a decline of 60% between these two periods (APC = −60.7%, p < 0.0001). The average number of patients born each year declined from 18.6 to 6.8 over these two periods. A clear breakpoint was observed in the incidence rate at the end of the 1980s (p < 0.0001) but the incidence rate continued to decrease significantly since implementation of NBS in 1989 (annual APC = −1.5%, 95% CI: [−2.8%; −0.2%], p = 0.0242). It declined from 1/2693 over the 1989-94 period to 1/5046 nowadays (APC = −46.6%, p = 0.0031). Conclusion: This study highlights how the incidence of CF has evolved over time in an area where CF is frequent. It reports a clear drop in incidence that results from a complex mixture of factors and that coincides with the availability of prenatal diagnosis. It also shows that incidence continues to decline since implementation of NBS 30 years ago.

Research paper thumbnail of Development and outcomes of a patient driven cystic fibrosis quality of care improvement project

Journal of Cystic Fibrosis

Research paper thumbnail of Cystic Fibrosis en fysiotherapie

Research paper thumbnail of P053 Long-term clinical effects of ivacaftor (Kalydeco): real-life data from the Dutch cystic fibrosis Registry

Journal of Cystic Fibrosis

Research paper thumbnail of European Cystic Fibrosis Society Standards of Care: Best Practice guidelines

Journal of Cystic Fibrosis Official Journal of the European Cystic Fibrosis Society, 2014

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades... more Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in Table 1. We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.

Research paper thumbnail of WS03.1 Positive effects of a coaching program for parents after the diagnosis of CF following newborn screening

Journal of Cystic Fibrosis, 2015

Objectives Parents are emotionally severely disrupted after their child is diagnosed with CF foll... more Objectives Parents are emotionally severely disrupted after their child is diagnosed with CF following Newborn Screening. The Dutch CF Foundation (NCFS) started a coaching program for parents to assist them to regain control of their lives. Methods After the diagnosis, parents were offered a maximum of 4 individual sessions at home and one group session with other parents and an experienced family coach. At the start of the coaching program and at the end, both parents filled in questionnaires related to the perceived health status of their child (TAPQOL), anxiety and distress of the parents, and satisfaction about the coaching program. If applicable, paired non-parametric testing was performed for differences between the start and the end of the program. Results 48 parents participated in the program. Mean age of the child at start was 28 weeks. Almost all parents completed 3 or 4 individual sessions. Parental perception of the health status of their child improved significantly over time. Anxiety and distress of the parents significantly decreased. The coaching program was rated good or very good by 80% concerning information, by 90% concerning the coach, and by 75% concerning the content (with only lower scores for the amount of sessions, which were preferred to be higher). 36 parents participated in the group session. 90% rated this session as good or very good. 90% of the parents has reached their predefined goals and almost all parents advised to continue the coaching project in the home situation for future parents. Conclusion A coaching program for parents after the diagnosis CF is feasible and useful. Other groups also might benefit from a coaching program.

Research paper thumbnail of A Coaching Program for Parents with a Child, Diagnosed with CF after NBS, Feasibility and Possible Effects

Psychology and Behavioral Science International Journal

Research paper thumbnail of Intestinal organoids and personalized medicine in cystic fibrosis

Current Opinion in Pulmonary Medicine, 2016

New therapeutics have been introduced for cystic fibrosis that modulate cystic fibrosis transmemb... more New therapeutics have been introduced for cystic fibrosis that modulate cystic fibrosis transmembrane conductance regulator (CFTR) function in a mutation-specific fashion. Despite CFTR genotype-based stratification of treatments, treatment efficacy is variable between study participants suggesting that individual factors further contribute to drug efficacy. Moreover, these treatments are licensed for a limited amount of CFTR mutations, and study participants with rare mutations that can potentially benefit from available treatments may be missed. New approaches that better support the identification of responders to CFTR modulators are, therefore, needed. We, here, review how a patient-oriented research collaboration between basic and clinical scientists and a national cystic fibrosis patient organization led to the development of a CFTR-dependent assay using primary stem cell cultures termed intestinal organoids that can measure the individual efficacy of CFTR modulators in a preclinical laboratory setting. Early observations suggest that drug responses in organoids reflect drug responses in vivo. We particularly focus on the importance of patient-oriented research collaborations, and how such a collaboration helped to develop a personalized medicine approach for CFTR modulators. Intestinal organoids and biobanks thereof may be used to select optimal, individually tailored treatments for current and future (combinations of) CFTR modulators with only limited patient discomfort.

Research paper thumbnail of Mini-guts in a dish: Perspectives of adult Cystic Fibrosis (CF) patients and parents of young CF patients on organoid technology

Journal of Cystic Fibrosis

Research paper thumbnail of Insights into the cystic fibrosis care in Eastern Europe: Results of survey

Journal of Cystic Fibrosis

Research paper thumbnail of Aligning patients' needs and research priorities towards a comprehensive CF research program

Journal of Cystic Fibrosis

Research paper thumbnail of Standardy opieki Europejskiego Towarzystwa Mukowiscydozy: wytyczne i najlepsze praktyki

Research paper thumbnail of Patient participation in research funding: an overview of when, why and how amongst Dutch health funds

Research Involvement and Engagement

Background Patient participation in decision-making on health-related research has gained ground.... more Background Patient participation in decision-making on health-related research has gained ground. Nineteen Dutch health-related research-funding organisations (HFs) have taken up the challenge to include patients in their funding process. A ‘Patient participation (PP) advisory team’ was set-up, with HF-representatives and patient advocates, who together initiated this study. We provide an overview of when, why, and how PP activities take place in HFs’ funding processes, share main challenges and identify possible solutions. Methods A qualitative research design was used. Data was gathered by questionnaires (n = 14) and semi-structured interviews (n = 18) with HF employees responsible for patient participation, followed by a workshop (n = 27) with involved employees of HFs and key players in PP from national patient organisations and research organisations. A descriptive analysis was used for the questionnaire. A semi-directed content analysis was used for the interviews and the work...

Research paper thumbnail of Responsible use of organoids in precision medicine: the need for active participant involvement

Research paper thumbnail of Farewell to Han van der Laag

Journal of Cystic Fibrosis, 2005

Research paper thumbnail of European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis

Journal of Cystic Fibrosis, 2014

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades... more Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in . We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.

Research paper thumbnail of Evolution of nutritional management in children with cystic fibrosis – a narrative review

Journal of human nutrition and dietetics, Apr 25, 2024

Research paper thumbnail of Author response for "Evolution of nutritional management in children with cystic fibrosis – a narrative review

Research paper thumbnail of Epidemiology and management of nontuberculous mycobacterial disease in people with cystic fibrosis, the Netherlands

Journal of Cystic Fibrosis

Research paper thumbnail of Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis

Journal of Cystic Fibrosis

Research paper thumbnail of WS16-3 Newborn screening for cystic fibrosis in the Netherlands: effects on clinical outcome at age six

Journal of Cystic Fibrosis, 2019

(NBS) programs has allowed a better monitoring of the disease incidence, which appears lower than... more (NBS) programs has allowed a better monitoring of the disease incidence, which appears lower than in the past. This study assesses time trends in incidence of CF over a 43-year period in an area where CF is frequent (Brittany, France) and where NBS is implemented since 30 years. Methods: This study enrolled patients with classical form of CF born in Brittany between January 1 st 1975 and December 31 st 2017 (n = 554). Patients born before the set up of NBS in 1989 were registered through active enquiries and combination of data sources. Time trends in incidence were examined using Poisson regression and expressed using the average percent change (APC) over time or between two periods of interest. Results: The overall incidence of CF was estimated at 1/2811 live births over the study period. Poisson regression showed that the incidence rate decreased significantly over the whole period (annual APC: −1.8%, 95% CI: [−2.5%; −1.2%], p < 0.0001). The incidence rates dropped from 1/1983 over the first 5-y. period (1975-79) to 1/5046 over the last one (2013-17), what corresponded to a decline of 60% between these two periods (APC = −60.7%, p < 0.0001). The average number of patients born each year declined from 18.6 to 6.8 over these two periods. A clear breakpoint was observed in the incidence rate at the end of the 1980s (p < 0.0001) but the incidence rate continued to decrease significantly since implementation of NBS in 1989 (annual APC = −1.5%, 95% CI: [−2.8%; −0.2%], p = 0.0242). It declined from 1/2693 over the 1989-94 period to 1/5046 nowadays (APC = −46.6%, p = 0.0031). Conclusion: This study highlights how the incidence of CF has evolved over time in an area where CF is frequent. It reports a clear drop in incidence that results from a complex mixture of factors and that coincides with the availability of prenatal diagnosis. It also shows that incidence continues to decline since implementation of NBS 30 years ago.

Research paper thumbnail of Development and outcomes of a patient driven cystic fibrosis quality of care improvement project

Journal of Cystic Fibrosis

Research paper thumbnail of Cystic Fibrosis en fysiotherapie

Research paper thumbnail of P053 Long-term clinical effects of ivacaftor (Kalydeco): real-life data from the Dutch cystic fibrosis Registry

Journal of Cystic Fibrosis

Research paper thumbnail of European Cystic Fibrosis Society Standards of Care: Best Practice guidelines

Journal of Cystic Fibrosis Official Journal of the European Cystic Fibrosis Society, 2014

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades... more Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in Table 1. We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.

Research paper thumbnail of WS03.1 Positive effects of a coaching program for parents after the diagnosis of CF following newborn screening

Journal of Cystic Fibrosis, 2015

Objectives Parents are emotionally severely disrupted after their child is diagnosed with CF foll... more Objectives Parents are emotionally severely disrupted after their child is diagnosed with CF following Newborn Screening. The Dutch CF Foundation (NCFS) started a coaching program for parents to assist them to regain control of their lives. Methods After the diagnosis, parents were offered a maximum of 4 individual sessions at home and one group session with other parents and an experienced family coach. At the start of the coaching program and at the end, both parents filled in questionnaires related to the perceived health status of their child (TAPQOL), anxiety and distress of the parents, and satisfaction about the coaching program. If applicable, paired non-parametric testing was performed for differences between the start and the end of the program. Results 48 parents participated in the program. Mean age of the child at start was 28 weeks. Almost all parents completed 3 or 4 individual sessions. Parental perception of the health status of their child improved significantly over time. Anxiety and distress of the parents significantly decreased. The coaching program was rated good or very good by 80% concerning information, by 90% concerning the coach, and by 75% concerning the content (with only lower scores for the amount of sessions, which were preferred to be higher). 36 parents participated in the group session. 90% rated this session as good or very good. 90% of the parents has reached their predefined goals and almost all parents advised to continue the coaching project in the home situation for future parents. Conclusion A coaching program for parents after the diagnosis CF is feasible and useful. Other groups also might benefit from a coaching program.

Research paper thumbnail of A Coaching Program for Parents with a Child, Diagnosed with CF after NBS, Feasibility and Possible Effects

Psychology and Behavioral Science International Journal

Research paper thumbnail of Intestinal organoids and personalized medicine in cystic fibrosis

Current Opinion in Pulmonary Medicine, 2016

New therapeutics have been introduced for cystic fibrosis that modulate cystic fibrosis transmemb... more New therapeutics have been introduced for cystic fibrosis that modulate cystic fibrosis transmembrane conductance regulator (CFTR) function in a mutation-specific fashion. Despite CFTR genotype-based stratification of treatments, treatment efficacy is variable between study participants suggesting that individual factors further contribute to drug efficacy. Moreover, these treatments are licensed for a limited amount of CFTR mutations, and study participants with rare mutations that can potentially benefit from available treatments may be missed. New approaches that better support the identification of responders to CFTR modulators are, therefore, needed. We, here, review how a patient-oriented research collaboration between basic and clinical scientists and a national cystic fibrosis patient organization led to the development of a CFTR-dependent assay using primary stem cell cultures termed intestinal organoids that can measure the individual efficacy of CFTR modulators in a preclinical laboratory setting. Early observations suggest that drug responses in organoids reflect drug responses in vivo. We particularly focus on the importance of patient-oriented research collaborations, and how such a collaboration helped to develop a personalized medicine approach for CFTR modulators. Intestinal organoids and biobanks thereof may be used to select optimal, individually tailored treatments for current and future (combinations of) CFTR modulators with only limited patient discomfort.

Research paper thumbnail of Mini-guts in a dish: Perspectives of adult Cystic Fibrosis (CF) patients and parents of young CF patients on organoid technology

Journal of Cystic Fibrosis

Research paper thumbnail of Insights into the cystic fibrosis care in Eastern Europe: Results of survey

Journal of Cystic Fibrosis

Research paper thumbnail of Aligning patients' needs and research priorities towards a comprehensive CF research program

Journal of Cystic Fibrosis

Research paper thumbnail of Standardy opieki Europejskiego Towarzystwa Mukowiscydozy: wytyczne i najlepsze praktyki

Research paper thumbnail of Patient participation in research funding: an overview of when, why and how amongst Dutch health funds

Research Involvement and Engagement

Background Patient participation in decision-making on health-related research has gained ground.... more Background Patient participation in decision-making on health-related research has gained ground. Nineteen Dutch health-related research-funding organisations (HFs) have taken up the challenge to include patients in their funding process. A ‘Patient participation (PP) advisory team’ was set-up, with HF-representatives and patient advocates, who together initiated this study. We provide an overview of when, why, and how PP activities take place in HFs’ funding processes, share main challenges and identify possible solutions. Methods A qualitative research design was used. Data was gathered by questionnaires (n = 14) and semi-structured interviews (n = 18) with HF employees responsible for patient participation, followed by a workshop (n = 27) with involved employees of HFs and key players in PP from national patient organisations and research organisations. A descriptive analysis was used for the questionnaire. A semi-directed content analysis was used for the interviews and the work...

Research paper thumbnail of Responsible use of organoids in precision medicine: the need for active participant involvement

Research paper thumbnail of Farewell to Han van der Laag

Journal of Cystic Fibrosis, 2005

Research paper thumbnail of European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis

Journal of Cystic Fibrosis, 2014

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades... more Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in . We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.