Cystic fibrosis (CF) or pancreatic fibrosis, is a disease that is inherited, which causes the excess production of thick, sticky mucus, that builds up in the lungs and digestive tract. It gives rise to chronic lung disease, problems with digestion, and male infertility. It is caused by a defective gene that initiates the body to produce abnormally thick, sticky mucus that congests the lungs. This mucus traps bacteria in tiny passages, which can cause life-threatening infections. When a person is affected repeatedly with infections and blockages, irreversible lung damage may take place.
- The cystic fibrosis gene was identified in 1989. This has enabled the development of carrier tests, better treatment, and improved control of the condition.
- CF is among the most common autosomal recessive genetic diseases in North America. For a person to inherit it, he must be passed two defective CF genes, one from each parent. Millions of Americans who carry only one defective gene are called 'carriers', and do not have any symptoms.
- Approximately 1 in 29 Caucasian Americans have the cystic fibrosis gene. This deadly disease affects more Caucasians (people of Northern or Central European descent) in the United States. It is less common among other races and ethnic groups, such as African-Americans and Asian-Americans.
- A majority of children with this problem are diagnosed by age 2. However, children with a milder version of the illness may not be diagnosed until 18 years or older. CF is one of the most widely occurring chronic lung disease in children and young adults, and may result in early death.
- The excess mucus causes symptoms of a frequent or lingering cough, repeated lung infections such as pneumonia or sinusitis, breathing difficulties, and digestion problems such as severe constipation or frequent trips to the toilet.
- While pancreatic fibrosis causes pancreatic complications in 85% of the patients, lung function remains the critical factor in prognosis and survival. These complications extend to impairment of the pancreas, which prevents the release of enzymes needed for the digestion of food. The result of this is often seen in a person who eats well but does not put on any weight.
- Treatment is administered to deal with lung problems and intestinal cum nutritional problems. Lung and sinus infection are treated with antibiotics. Inhaled medicines are used to open up airways, and DNA enzyme replacement therapy is used to thin the mucus and make it easier to cough up.
- Intestinal and nutritional problems are alleviated through replacement of the pancreatic enzymes that are missing, a special diet, and vitamin supplements.
- CF patients need to visit the clinic regularly, may be administered antibiotic treatments, and may require hospitalization on a regular basis. Most children suffering from pancreatic fibrosis require daily physiotherapy and medication, among other treatments. Adults with CF also need intensive chest physiotherapy everyday to loosen the build-up of mucus in the lungs.
- In earlier times, CF meant having an extremely short lifespan. However, research and advanced treatments have resulted in an increased life expectancy of up to 38 years. This limit is also being constantly extended, and it is expected that babies now born with CF should live into their fifties, subject to various conditions. However, an increased life expectancy also means new and additional complications such as pancreatic fibrosis related diabetes and osteoporosis.
A chance of survival and a decent quality of life depends on early detection and a comprehensive treatment plan.