Cystic fibrosis is a very serious disorder that is fatal if not treated properly. A defective gene causes thin body secretions, such as lung mucus, digestive juices, sweat and reproductive secretions, to become thick and sticky. Serious and life-threatening problems may arise due to this thickening. There is no permanent cure for cystic fibrosis yet, but symptomatic treatment is given.
When the lungs and airways are choked, the cystic fibrosis patient coughs and produces very thick sputum. He is short of breath and develops wheezing. Polyps may grow in the nasal passages. The frequency of sinus, chest infections, pneumonia and bronchitis increase.
As the digestive juices do not reach the intestine, due to blocked ducts from the pancreas and liver, the fats and protein are not digested. The stool is bulky, greasy and foul smelling. The cystic fibrosis patient may have excessive appetite but is undernourished and underweight, as the food is not digested properly for absorption. A trypsin test may indicate whether sufficient enzymes from the pancreas are available for digestion.
The sweat may taste salty. When we kiss a child with cystic fibrosis we can taste this excessive salt taste. To diagnose cystic fibrosis positively we measure this saltiness in sweat. Technicians coat an odorless chemical on a small area of the skin in the arm, and stimulate that area with electric current to produce copious sweat. This sweat should contain about 40 m mol/l in a normal person. Above 60 m mol/l of salt in a sweat test will confirm the diagnosis of cystic fibrosis. With cystic fibrosis patients the duct that connects the testes and vas deferens may get blocked. Most cystic fibrosis patients are sterile.
Treatment is mainly to ease the symptoms and lead a normal life in spite of the malady. Bronchodilators and mechanical vibrators keep the lungs and airways clear. Enzyme and vitamin supplements keep the digestive system near normal. With correct lifestyle management Americans are living to their 30s and 40s with cystic fibrosis.
Cystic Fibrosis And Symptoms
Cystic fibrosis is a recessive disease and affects the lungs.
The primary aim of medical and scientific research on cystic fibrosis is to understand, treat and cure cystic fibrosis. An inherited disease, cystic fibrosis is thought to affect about 30,000 Americans and is the most common, life-shortening genetic disease known. Cystic fibrosis is a life-threatening disease caused by a defective gene and affecting about 30,000 children in America.
Researchers from the Beth Israel Deaconess Medical Centre, the University of Massachusetts and Massachusetts General Hospital took tissue samples from 38 patients with cystic fibrosis.
It was found they had extremely high levels of arachidonic acid (AA) and abnormally low levels of docosahexaenoic acid (DHA).
People who did not have CF did not have the fatty acids imbalance.
Researchers believe that many of the symptoms of cystic fibrosis follow the same pattern: mutated gene produces a mutated glycoconjugate resulting in a defective cellular component. There are approximately ten million symptomless carriers of the defective cystic fibrosis gene in America.
They say too much of one acid and too little of another means patients' bodies are more prone to inflammation.
In the New England Journal of Medicine, they suggest Omega-3 oils, found in fish, could help correct the imbalance.
Each week three young people in the UK die from the disease, which is caused by the faulty CFTR gene.
CF causes an abnormally thick, sticky mucus to be produced in the body, causing chronic inflammation of the lungs leading to life-threatening infections.
The average life expectancy for a person with CF is around 31.
To diagnose cystic fibrosis, the laboratory carries out a sweat test. When the lungs and airways are choked, the cystic fibrosis patient coughs and produces very thick sputum. The authors concluded that forced ionisation of the indoor air represents a natural and efficient treatment for respiratory diseases in patients with cystic fibrosis.
When the cystic fibrosisTR is not normal, the regulation of salt through the membranes becomes defective. In the respiratory system the thin mucus lining becomes thick and sticky. As the digestive juices do not reach the intestine, due to blocked ducts from the pancreas and liver, the fats and protein are not digested.
Dr Steven Freedman of the gastroenterology division at Beth Israel Deaconess Medical Center, who led the research, said: "Since 1989, we have known that the defective CFTR gene is responsible for CF.
"But we didn't understand how this defective gene leads to the symptoms of the disease.
"This new study sheds light on what may be happening and provides a link between CFTR function and fatty acid metabolism."
He added: "It is known that high amounts of AA and low amounts of DHA would predispose to inflammation.
"This discovery may help explain why there is an excessive inflammatory response among CF patients.
"This is the basis for why Omega-3 fish oils, found in cold-water fish as well as supplements, reduce inflammation since they increase levels of DHA and suppress AA."
'No diet change'
Dr Adam Jaffe, head of the CF Research Group at London's Institute of Child Health, told BBC News Online the research was interesting but not conclusive.
"Patients shouldn't change their diet based on spurious associations between fatty acids and inflammation.
"But I would not be against them adding supplements to their diets."
Tom O'Connor, an expert on Omega-3, shares a wealth of information on his website
Both Kent Pinkerton & Tom O`Connor are contributors for EditorialToday. The above articles have been edited for relevancy and timeliness. All write-ups, reviews, tips and guides published by EditorialToday.com and its partners or affiliates are for informational purposes only. They should not be used for any legal or any other type of advice. We do not endorse any author, contributor, writer or article posted by our team.
Kent Pinkerton has sinced written about articles on various topics from Careers and Job Hunting, Family Concerns and Cyst. provides detailed information on Cystic Fibrosis, Cystic Fibrosis Symptoms, Causes Of Cystic Fibrosis, Cystic Fibrosis Treatments and more.. Kent Pinkerton's top article generates over 18100 views. to your Favourites.
Tom O`Connor has sinced written about articles on various topics from Cyst, Asthma. Tom O`Connor an adept and reality study on health and life. Tom O`Connor's top article generates over 1600 views. to your Favourites.
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