Information on Cystic Fibrosis from the National Institutes of Health
Cystic fibrosis is an inherited disease in which the secretary glands secrete mucus that is too thick. In order to inherit the disease, a person must have two copies of a mutated protein called CTFR. Symptoms are often noted shortly after birth, including: salty taste to the skin, recurrent respiratory infections, pneumonia, digestive problems, and failure to gain weight. Although there is no cure, modern therapies allow many cystic fibrosis sufferers to live into their forties and fifties. Over time, complications arising from the symptoms of cystic fibrosis damage the body, so it is essential to control the symptoms as much as possible. Treatment includes exercise, a nutritional program, medications, and respiratory therapy. More at the National Institutes of Health.
Cystic Fibrosis Information at Stanford University
Cystic Fibrosis (CF) is one of the most common genetic (inherited) diseases in America. It is also one of the most serious. It mainly affects the lungs and the digestive systems in the body, causing breathing problems and problems digesting foods. It is a chronic disease that currently has no cure. Read more at the Cystic Fibrosis Center, Stanford University.
Information on Cystic Fibrosis from the Cystic Fibrosis Foundation
Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that...Read more at the Cystic Fibrosis Foundation.
Research advances in cystic fibrosis
Knowledge about cystic fibrosis has come a long way in the past few decades. Improved animal models are teaching scientists a lot about how cystic fibrosis develops and revealing new therapeutic targets. Modern advances in medical technology have opened the possibility for more effective treatments of cystic fibrosis in the future. View the Factsheet from the NIH.
Podcast: Living with Cystic Fibrosis
My name is Tiana and I am 11. I’ve been visiting Great Ormond Street Hospital for 11 years. I come to the hospital because I have a condition called cystic fibrosis which needs to be treated regularly. Listen to my podcast at childrenfirst.com (NHS/UK) Great Ormond Street Hospital.
Video: Recurrent chest infections in a child can be a symptom of cystic fibrosis
Children with frequent chest infections that may not go away completely should be examined by their doctor. If additional symptoms are present, such as abnormally large or foul smelling stools, inadequate growth, and poor weight gain, the child should be examined for cystic fibrosis. Tests commonly used in cystic fibrosis diagnosis include wearing an absorbent patch to measure the amount of present in the sweat and using x-rays to determine if abnormal chest secretions are present. View the video at the National Library of Medicine.
The role of genetic counselors in cystic fibrosis education
Genetic counselors share information about how cystic fibrosis is inherited, how parents and children can be screened, and what the symptoms and prognosis of the disease are. View the major recommendations at the National Guideline Clearinghouse.
Treating bronchiectasis in the cystic fibrosis patient
Bronchiectasis is a condition in which the airways are chronically damaged and become permanently widened. This condition may result from the recurrent infections experienced by an individual with cystic fibrosis. The appropriate treatment of this condition depends upon many compounding factors. View the major recommendations at the National Guideline Clearinghouse.
Using pulmonary rehabilitation to treat cystic fibrosis
Pulmonary rehabilitation may be performed in the patient with cystic fibrosis. The treatment plan is designed with the patient's individual needs in mind and may integrate therapeutic practices from many medical disciplines. The overall goal is to optimize organ function, reduce symptoms, and stabilize or reverse complications of the illness. View the major recommendations at the National Guideline Clearinghouse.
Measuring lung function in children with cystic fibrosis
In patients with cystic fibrosis, the physician may wish to measure lung and airway function. There are many ways to measure how well the respiratory system is working. Each methodology has pros and cons. View the major recommendations at the National Guideline Clearinghouse.
Abstract: Long-term exposure to cystic fibrosis medications can lead to kidney injury over time
Thanks to improved treatment options cystic fibrosis patients are living longer than ever. With increased lifespan comes complications from long-term treatment. In particular, prolonged exposure to the immunosupressants used following lung transplantation and certain antibiotics used to treat respiratory infections can harm the kidneys. Clinicians should attempt to minimize these toxicities for the overall benefit of the patient. View the abstract in Pubmed.
Abstract: Delaying chronic infection with Pseudomonas aeruginosa improves patient outcome
Chronic infection with Pseudomonas aeruginosa is a major cause of illness and death in patients with cystic fibrosis. A recent study demonstrates that strategies aimed at preventing infection early in life or aggressively eliminating it with antibiotics following the first infection can significantly delay the chronic infection of most individuals. View the abstract in Pubmed.
Abstract: The importance of nutrition in cystic fibrosis prognosis
Respiratory problems are hallmark of cystic fibrosis. However, the first signs of the disease are usually abnormal gastrointestinal function and decreased ability to absorb nutrients from foods. Patients with the most severe nutritional inadequacies should be identified as early as possible, as interventions intended to increase nutrient and calorie intake greatly improve patient outcome. View the abstract in Pubmed.
Abstract: The cause of chronic inflammation in cystic fibrosis
The protein CTFR is mutated in patients with cystic fibrosis, causing it to accumulate within an incorrect compartment of cells. This alerts the cell that something is abnormal, so it reacts by producing inflammatory mediators. Thus, in patients with cystic fibrosis, the lungs are continuously inflamed, even in the absence of infection. View the free full text in Pubmed.
Abstract: Lung transplantation in cystic fibrosis patients
Cystic fibrosis is one of the leading causes of lung transplantation in the U.S. Optimal outcomes are realized if other symptoms of the disease are followed closely and managed. View the abstract in Pubmed.
Abstract: The severity of cystic fibrosis is associated with the specific mutation found in an individual's CTFR gene
Defects in the cystic fibrosis gene, CTFR, are known to decrease production of the CTFR protein. Different mutations in the CTFR gene have been observed in cystic fibrosis patients. The specific mutation present determines how much of the CTFR protein will be made. Lower levels of CTFR protein are associated with more severe disease. View the abstract in Pubmed.
Abstract: Cystic fibrosis patients who experience negative reactions to antibiotics can be desensitized to the drugs quickly
Due to the frequency of respiratory infections in cystic fibrosis, patients are repeatedly administered antibiotic therapies. In some individuals, this leads to a hypersensitivity to a particular antibiotic. A new protocol has been developed that successfully and quickly desensitizes patients to antibiotics. View the abstract in Pubmed.
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Diagram of the lungs
Crystal structure of part of the CTFR protein
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Psuedomonas aerugonosa, a common chronic infection acquired by cystic fibrosis patients
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