scholarly journals Adult cystic fibrosis care in the 21st century

2015 ◽  
Vol 75 (3) ◽  
Author(s):  
E. Garattini ◽  
D. Bilton ◽  
G. Cremona ◽  
M. Hodson
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Life Expectancy ◽  
21St Century ◽  
Autosomal Recessive ◽  
Therapeutic Strategies ◽  
Complex Care ◽  
Inherited Disease ◽  
Appropriate Care

Cystic fibrosis (CF) is the most common autosomal recessive inherited disease of Caucasian populations. As a result of a variety of diagnostic and therapeutic strategies there has been a dramatic increase in the life expectancy of patients with CF in the last decades and 50% of patients are now adults. This review will focus on the disease in adults and the provision of appropriate care. The complex care required to improve the survival and quality of life in the adult patients can best be provided in a dedicated adult cystic fibrosis unit. These units currently exist in many European countries, but more are needed in Italy.

scholarly journals Stigma and cystic fibrosis

2010 ◽  
Vol 18 (1) ◽  
pp. 139-142 ◽  
Author(s):  
Tainá Maues Peluci Pizzignacco ◽  
Débora Falleiros de Mello ◽  
Regina Aparecida Garcia de Lima
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Chronic Disease ◽  
Children And Adolescents ◽  
Treatment Adherence ◽  
Family Relationships ◽  
Life Histories ◽  
Autosomal Recessive ◽  
Family Members

Cystic Fibrosis (CF), also known as Mucoviscidosis, is a chronic disease of autosomal recessive origin and so far incurable. This analysis considers some characteristics of patients and family members that indicate it is a stigmatizing disease. The CF stigma’s impact on the lives of children and adolescents can affect treatment adherence, socialization, family relationships and the formation of their life histories, with direct consequences on their quality of life.

scholarly journals The Changing Face of Cystic Fibrosis and Its Implications for Screening

2020 ◽  
Vol 6 (3) ◽  
pp. 54 ◽  
Author(s):  
Lutz Naehrlich
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Life Expectancy ◽  
Early Diagnosis ◽  
Newborn Screening ◽  
Treatment Options ◽  
Preventive Treatment ◽  
Multidisciplinary Care ◽  
The Face

Early diagnosis, multidisciplinary care, and optimized and preventive treatments have changed the face of cystic fibrosis. Life expectancy has been expanded in the last decades. Formerly a pediatric disease, cystic fibrosis has reached adulthood. Mutation-specific treatments will expand treatment options and give hope for further improvement of quality of life and life expectancy. Newborn screening for CF fits perfectly into these care structures and offers the possibility of preventive treatment even before symptoms occur. Especially in countries without screening, newborn screening will fulfill that promise only with increased awareness and new care structures.

Cystic fibrosis - general review on sinonasal complications and case report

2017 ◽  
Vol 7 (25) ◽  
pp. 33-37
Author(s):  
Claudiu Manea ◽  
Alina Diaconescu
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Case Report ◽  
Life Expectancy ◽  
Multiple Organ ◽  
Lower Airway ◽  
General Review ◽  
Organ Systems ◽  
Sinonasal Disease

Abstract An irreversible disease, cystic fibrosis (CF), is responsible for affecting multiple organ systems containing epithelia. It is well known that the sinonasal disease caused by CF has consequences for the incidence of the lower airway exacerbations, as well as affecting the quality of life of those patients. This review provides an update by evaluating the available literature regarding pathogenesis, management and treatment of cystic fibrosis patients. To gain a better view of the disease and obtain a higher life expectancy, further studies are needed.

scholarly journals Gene Therapy: A Possible Alternative to CFTR Modulators?

2021 ◽  
Vol 12 ◽  
Author(s):  
J. Mercier ◽  
M. Ruffin ◽  
H. Corvol ◽  
L. Guillot
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Gene Therapy ◽  
Life Expectancy ◽  
New Technologies ◽  
Gene Replacement ◽  
New Drugs ◽  
Cftr Protein ◽  
The 1960S

Cystic fibrosis (CF) is a rare genetic disease that affects several organs, but lung disease is the major cause of morbidity and mortality. The gene responsible for CF, the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene, has been discovered in 1989. Since then, gene therapy i.e., defective gene replacement by a functional one, remained the ultimate goal but unfortunately, it has not yet been achieved. However, patients care and symptomatic treatments considerably increased CF patients’ life expectancy ranging from 5 years old in the 1960s to 40 today. In the last decade, research works on CFTR protein structure and activity led to the development of new drugs which, by readdressing CFTR to the plasma membrane (correctors) or by enhancing its transport activity (potentiators), allow, alone or in combination, an improvement of CF patients’ lung function and quality of life. While expected, it is not yet known whether taking these drugs from an early age and for years will improve the quality of life of CF patients in the long term and further increase their life expectancy. Besides, these molecules are not available (specific variants of CFTR) or accessible (national health policies) for all patients and there is still no curative treatment. Another alternative that could benefit from new technologies, such as gene therapy, is therefore still attractive, although it is not yet offered to patients. Faced with the development of new CFTR correctors and potentiators, the question arises as to whether there is still a place for gene therapy and this is discussed in this perspective.

scholarly journals Understanding the relevance of pain, depression and anxiety-like manifestations in the management of cystic fibrosis

2015 ◽  
Vol 67 (3) ◽  
pp. 1067-1070
Author(s):  
Evelina Moraru ◽  
Ramona Diaconu ◽  
Emil Anton ◽  
Laura Bozomitu ◽  
Carmen Anton ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Risk Factors ◽  
Cystic Fibrosis ◽  
Genetic Disease ◽  
Autosomal Recessive ◽  
Clinical Manifestations ◽  
Original Data ◽  
Epidemiological Studies ◽  
Depression And Anxiety

Cystic fibrosis (CF) is the most frequent autosomal recessive genetic disease in Caucasians. Although epidemiological studies continue to provide data and information that could lead to a better understanding of the clinical manifestations of the disease, there are still many controversies regarding the importance of some risk factors, such as pain, depression or anxiety in the pathogenesis of this disorder and its management. In the present study, we focused on the relevance of these 3 factors in the management of CF and to their related mechanisms in this disorder. Our original data showed that pain, depression and anxiety, determined by using a specific quality of life (KINDL) questionnaire, are important in understanding this disorder, the effectiveness of treatment and could also represent important factors for improving treatment. The screening and treatment of these specific manifestations in CF patients is recommended and holds promise for improving the outcome of patients with CF.

Cancer pain management

2014 ◽  
Vol 155 (3) ◽  
pp. 93-99
Author(s):  
Péter Heigl
Keyword(s):  
Quality Of Life ◽  
Pain Management ◽  
Pain Relief ◽  
Life Expectancy ◽  
Cancer Pain ◽  
Cancer Pain Management ◽  
Medical Activity ◽  
Short Summary ◽  
Adequate Quality

Pain is a significant and alarming symptom of cancer seriously affecting the activity and quality of life of patients. Recent research proved that inadequate analgesia shortens life expectancy. Therefore, pain relief is not only a possibility but a professional, ethical and moral commitment to relieve patients from suffering, as well as ensure their adequate quality of life and human dignity. Proper pain relief can be achieved with medical therapy in most of the cases and the pharmacological alternatives are available in Hungary. Yet medical activity regarding pain relief is far from the desired. This paper gives a short summary of the guidelines on medical pain management focusing particularly on the use of opioids. Orv. Hetil., 2014, 155(3), 93–99.

“Position Paper” Timely Insulinisation in Type 2 Diabetes Patients

2012 ◽  
Vol 19 (4) ◽  
pp. 441-444
Author(s):  
László Barkai ◽  
Nicolae Hâncu ◽  
György Jermendy ◽  
Maya Konstantinova ◽  
Radu Lichiardopol ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Type 2 Diabetes ◽  
Life Expectancy ◽  
Insulin Therapy ◽  
Metabolic Control ◽  
Current Medical ◽  
Position Paper ◽  
Medical Evidence

AbstractThe objective of this position paper is to review the current medical evidence andguidelines regarding the treatment of type 2 diabetes (T2DM) and to issue medicalrecommendations strengthening the timely use of insulin in patients with T2DMuncontrolled on noninsulin therapy. When noninsulin therapy fails to achieve or tomaintain HbA1c targets, insulin therapy is required. Timely insulin therapy couldprovide proper metabolic control that might prevent complications, lead toimprovement of life expectancy and quality of life.

Psychosocial well-being and quality of life in siblings of children with congenital heart disease: A systematic review

2021 ◽  
pp. 136749352110129
Author(s):  
Alice S Schamong ◽  
Hannah Liebermann-Jordanidis ◽  
Konrad Brockmeier ◽  
Elisabeth Sticker ◽  
Elke Kalbe
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Associated Factors ◽  
Congenital Heart ◽  
Negative Impact ◽  
Well Being

Congenital heart disease (CHD) is a major global health problem. Until recently, the siblings of this group did not receive much attention. This review, conducted from November 2019 to October 2020, aims to summarize knowledge about psychosocial well-being and quality of life (QoL), associated factors, and interventions for siblings of children with CHD. Systematic searches were conducted in PubMed, PsycINFO, PsycARTICLES, Web of Science via EBSCOhost, and CENTRAL. Twelve articles were included. Results showed that psychosocial well-being was impaired in 14% to 40% of siblings. Negative impact of illness was highest for CHD siblings compared to siblings of children with cancer, cystic fibrosis, or diabetes. QoL was impaired in up to one-third. Siblings of children with CHD and cancer rated their QoL lower than those of siblings of children with cystic fibrosis or type-1 diabetes. Associated factors were sibling age, gender, socioeconomic status, miscarriage, previous sibling death, visibility of illness, and severity of condition. Only one of two interventions focused on siblings of CHD children. Although data are scarce and inhomogeneous, it indicates that siblings of CHD children suffer from lower psychosocial well-being and QoL than siblings of children with other chronic conditions. Interventions to improve their situation should be developed.

scholarly journals Prediction of all-cause mortality from 24 month trajectories in patient-reported psychological, clinical and quality of life outcomes in uveal melanoma patients

2021 ◽  
Author(s):  
Stephen L. Brown ◽  
Peter L. Fisher ◽  
Laura Hope-Stone ◽  
Heinrich Heimann ◽  
Rumana Hussain ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Life Expectancy ◽  
Uveal Melanoma ◽  
Cox Regression ◽  
Multivariate Analyses ◽  
Tumor Diameter ◽  
Control Variables ◽  
Patient Reported ◽  
All Cause Mortality

AbstractA number of patient-reported outcomes (PROs) predict increased mortality after primary cancer treatment. Studies, though, are sometimes affected by methodological limitations. They often use control variables that poorly predict life expectancy, examine only one or two PROs thus not controlling potential confounding by unmeasured PROs, and observe PROs at only a single point in time. To predict all-cause mortality, this study used control variables affording good estimates of life expectancy, conducted multivariate analyses of multiple PROs to identify independent predictors, and monitored PROs two years after diagnosis. We recruited a consecutive sample of 824 patients with uveal melanoma between April 2008 and December 2014. PROs were variables shown to predict mortality in previous studies; anxiety, depression, visual and ocular symptoms, visual function impairment, worry about cancer recurrence, and physical, emotional, social and functional quality of life (QoL), measured 6, 12 and 24 months after diagnosis. We conducted Cox regression analyses with a census date of December 2018. Covariates were age, gender, marital and employment status, self-reported co-morbidities, tumor diameter and thickness, treatment modality and chromosome 3 mutation status, the latter a genetic mutation strongly associated with mortality. Single predictor analyses (with covariates), showed 6-month depression and poorer functional QoL predicting mortality, as did 6–12 month increases in anxiety and 6–12 month decreases in physical and functional QoL. Multivariate analyses using all PROs showed independent prediction by 6-month depression and decreasing QoL over 6–12 months and 12–24 months. Elevated depression scores six months post-diagnosis constituted an increased mortality risk. Early intervention for depressive symptoms may reduce mortality.

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