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Saturday, November 12, 2016

Cystic fibrosis



There is no denying the fact that Cystic fibrosis is a hereditary disease in which mucus that the body produces is too thick and gummy, which affects the work of both the lungs and the pancreas, creating both breathing and digestive problems. It may also affect the male reproductive system and sweat glands. Symptoms of cystic fibrosis vary widely, and include slow growth in children, a chronic cough, sometimes producing phlegm, rectal prolapse, greasy stools, breathing difficulties and nasal passage inflammation, as stated by Mayo Clinic. Cystic fibrosis affects the digestive and respiratory systems, producing symptoms primarily in those tracts. Sometimes, cystic fibrosis symptoms affect other areas of life, such as limiting the capacity to exercise in affected individuals.
Symptoms of acute pulmonary edema, which results from the accumulation of too much fluid in the lungs, include a cough with bloody phlegm, extreme shortness of breath that aggravates while lying down, anxiety and a suffocating sensation, states Mayo Clinic. People with chronic pulmonary edema experience unusual shortness of breath when physically active, waking up in the middle of sleep with a breathless sensation, and difficulty breathing when lying down or performing strenuous tasks.  Most children with cystic fibrosis stay in good health until adulthood. Younger adults often graduate from college and work, but as they grow older, many cystic fibrosis patients experience lung disease that leaves them unable to work. Death is often attributed to lung complications, especially chronic infections.
Doctors typically treat patients with drugs to thin the mucus, making it easier to cough up. Prescribed antibiotics help with infections. Patients learn to clear mucus from their lungs once or more a day. Sometimes doctors prescribe pancreatic enzymes to help with the digestive issues. Patients often follow a special diet and are encouraged to exercise several times a week. People who have cystic fibrosis inherited defective genes from both parents, while those who inherited the gene from only one parent are carriers of the condition but do not develop cystic fibrosis, states Mayo Clinic. White people of Northern European descent are most likely to suffer from cystic fibrosis, though people of other races may also be born with the condition. People suffering from cystic fibrosis may see changes in their symptoms over time, in some cases experiencing an improvement and in others an increase in severity.

In view of the above it is evident that the people with cystic fibrosis display symptoms including repeated lung infections, a persistent cough with thick mucus, inflamed nasal passages, wheezing and shortness of breath, notes Mayo Clinic. Because thick mucus may block the tubes between the pancreas and small intestine, people with cystic fibrosis may be unable to absorb the nutrients in food and can suffer from severe constipation, poor weight gain and intestinal blockage. Complications from cystic fibrosis can lead to secondary conditions such as respiratory failure, chronic infections, distal intestinal obstruction syndrome and osteoporosis.

Treatments for cystic fibrosis include medications, physical therapy for the chest, pulmonary rehabilitation and surgery. While cystic fibrosis does not have a cure, treatment can reduce the complications and ameliorate the symptoms.


Cystic fibrosis is a genetic disorder that affects nearly 70,000 people throughout the world. It is a hereditary disease caused by a defective gene that produces a thick, sticky mucus that clogs the lungs. It also obstructs the natural functions of the pancreas, preventing the body from breaking absorbing nutrients.  Cystic fibrosis is a life-threatening disease that is most commonly found in people of Northern European descent. It occurs less frequently in African Americans, Hispanics and Native Americans and is rarely found in people of Asian and Middle Eastern origin. In past decades, children diagnosed with cystic fibrosis rarely survived until school age.
Now, with advances in medical technology, cystic fibrosis patients often live to adulthood. Approximately 1,000 new cases of cystic fibrosis are diagnosed every year, and most diagnoses occur before 2 years of age. The symptoms of cystic fibrosis include wheezing, shortness of breath and poorly functioning lungs, slow growth and inefficient weight gain, frequent wheezing and coughing, as well as salty tasting skin and recurring lung infections.
The defective gene at the center of cystic fibrosis affects cells that normally produce thin, slippery lubricating liquid, such as digestive juices and saliva. Normally, these liquids are slippery and thin, whereas cystic fibrosis causes them to become thick and sticky, clogging up and obstructing the normal processes with which these liquids are supposed to function.
Most children with cystic fibrosis stay in good health until adulthood, MedlinePlus explains. Younger adults often graduate from college and work, but as they grow older, many cystic fibrosis patients experience lung disease that leaves them unable to work. Death is often attributed to lung complications, especially chronic infections.
Doctors typically treat patients with drugs to thin the mucus, making it easier to cough up, Medline Plus says. Prescribed antibiotics help with infections. Patients learn to clear mucus from their lungs once or more a day. Sometimes doctors prescribe pancreatic enzymes to help with the digestive issues. Patients often follow a special diet and are encouraged to exercise several times a week.

Cystic fibrosis is one of several conditions that can keep the pancreas from making or releasing enough digestive enzymes into the intestinal tract for food digestion, so supplementation is necessary at times. Generally, patients take the medication orally with snacks and meals. The dosage for artificial pancreatic enzyme varies based on weight, diet and overall medical condition. It is important to hydrate well while on this medication. Taking the medicine every time one eats food maximizes the benefits. Some patients require a special diet in addition to the supplementation, and following the doctor's instructions yields the best overall results.

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