An overview of the management of cystic fibrosis

Pediatric Pulmonology, 2007

Journal of Cystic Fibrosis, 2018

Journal of the American Academy of Nurse Practitioners, 2012

2009

In the UK nineteen million people, approximately one third of the population, live with a chronic illness. (DoH 2005).This trend is rising and with increasing life expectancy will continue to do so. The cystic fibrosis population of eight thousand in the UK forms part of this group (CF Trust 2008) The term “expert patient” is currently a very topical one, with health professionals acknowledging that patients with chronic conditions are often more knowledgeable about their disease than they are. This is almost always the case in CF as most people with CF have a diagnosis within the first two years of life and thereafter spend the rest of their lives living with and managing the disease. This experience of living with a disease inevitably brings knowledge and other skills such as resourcefulness and decision making.

Journal of Cystic Fibrosis, 2005

ACTA MEDICA IRANICA, 2019

Cystic fibrosis (CF), as a fatal genetic condition, is associated with high morbidity and mortality rates. In Iran, limited studies exist on this disease. This study aimed to compare the demographic, clinical and paraclinical data of CF patients. This cross-sectional study was conducted in 2014-2015 on 174 CF patients referred to the Tehran Children Medical Center hospital, which is the main referral center for CF. For each patient, the forced expiratory volume in one second (FEV1) was measured, and the comparative demographic, clinical, and laboratory data of patients were recorded. Overall, 59% of studied patients were boys (n=102) and 41% were girls (n=72). The mean patient age (and standard deviations) was 7.1±5.7 years, with a range of 10 days to 28 years. In 67% of cases, the disease was diagnosed before their first birthday. The patients in this study were classified based on the FEV1 into mild (62%), moderate (33%) and severe (5%), indicating the degree of pulmonary complications. Cultures of respiratory secretions were positive for Pseudomonas aeruginosa and Staphylococcus aureus, in 23% and 16% of cases, respectively. In total, 61% of patients (n=83) were assigned to receive oral azithromycin for prophylaxis. Gastroesophageal reflux (reflux) was the most common gastrointestinal complication (35%). Regarding the complex nature of CF and the necessity of constant monitoring of patients during the lifespan , the comparative demographic, clinical and laboratory analysis of patients and registering and standardization of patients' data can be a major step in the better understanding of the disease, and thereby increasing the quality of life and life expectancy in the affected population.

Journal of Cystic Fibrosis, 2014

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.

Journal of Cystic Fibrosis, 2009

This is the third article related to a review of the literature based on data from national cystic fibrosis (CF) patient registries up to June 2008 and covering a total of 115 published studies. It focuses on several topics: CF incidence, genotype/phenotype correlation, microbiology, pregnancy/ paternity, clinical complications, lung transplantation, and others.

Oman Medical Journal, 2015

ystic Fibrosis (CF) is an autosomal recessive disease and occurs in the Caucasian population with a prevalence of 1:2500 1 making it one of the most common genetic disorders in this population. CF affects mucus-producing organs and presents predominantly with respiratory and gastrointestinal symptoms. The disease is clinically characterized by chronic airways infection and inflammation, resulting in lung fibrosis and progressive loss of pulmonary function. Lung disease is believed to be the leading cause of 90% of deaths in patients with CF. 3 A failure to thrive is a result of pancreatic insufficiency and subsequent malabsorption of nutrients, resulting in reduced weight gain and difficulties in maintaining body weight. Medical care concepts have advanced in Europe and Northern America, where specialized CF centers provide holistic CF care. The advanced care concepts, developed over the last four decades, have contributed to an increase in life expectancy from only six months in 1959 to around 30 years by 2003. Where optimum CF care is available the predicted median survival is now more than 50 years. 9-11 Some of these advances in CF care are yet to be realized in Oman. The current average life expectancy for people with CF in the North Al Batinah region was recently reported as only 10.5 years. 12 In addition, there is limited research available on the perceived impact of CF on daily life from the patients perspective, nor is there research on the cultural/ethnic and socioeconomic factors that influence attitudes to health and disease with respect to CF care in Oman. A central part of CF care is the prevention, diagnosis, and treatment of acute pulmonary exacerbations (PEx). They are clinically characterized original article