It is devastating to a parent when their child is diagnosed with cystic fibrosis. They may feel guilt because this disease is an inherited medical condition. A parent may not know they are a CF gene carrier. If both parents are carriers there is a 25 percent chance one of their children will be born with this condition. There is a 50 percent chance one or more of their children will be carriers, and a 25 percent chance the child will not be diagnosed with cystic fibrosis.

A disease that affects over 30,000 children and adults in the United States. Many people are carriers and may not know it. The disease affects the mucus producing glands of the body. This disease also affects the respiratory, digestive, and reproductive organs as well as the sweat glands in the body. The normal, thin, liquid lining the passageways to the digestive and respiratory areas of the body becomes blocked when that normal lining turns thick and begins to block the passageways to these parts of the body.

Fibrosis is the most common hereditary disease in Caucasians in the United States. Nearly ten million Caucasians in America are carriers of the mutant gene that causes the disease and are unaware of that fact. Caucasians have a higher risk of getting cystic fibrosis although the disease has been detected in all ethnic groups. Breaking the statistics down so we can understand them is this; one in 31 Americans are carriers of the disease and have no symptoms to identify them as carrying the defective gene.

The lifespan of a cystic fibrosis patient is between 24 and 30 years but with newer technologies and drug treatments many are living longer. New medications and drainage procedures may increase the life span of a child born with this condition. Previously, it was rare for a child with cystic fibrosis to grow to adulthood. The cause of death for life threatening condition patient will normally be respiratory failure. The causes include respiratory tract infections or breathing distress and couples with enlargement of the right side of the heart.

There currently is no cure for this disease. There are treatments that can ease the symptoms of the disease and prolong the life span of a cystic fibrosis patient. Treatments may include antibiotics to fight infection that settles in the bronchial tubes and lungs, daily breathing and respiratory treatment. Patients are urged to chest physiotherapy that can be done manually or with a special machine. Manual chest physiotherapy can be controlled breathing for 30 to 45 minutes. You can also put the patient in a position that allows the mucus to drain from the lungs while the chest or back is clapped and vibrated. This allows the thick mucus to break away from the airways and be passed from the body. This procedure should be done over different parts of the back and chest because the mucus can settle in different areas of the lung. Mechanical devices are also available to perform the same function.

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By: Juliet Taylor

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Cystic fibrosis (CF) is the most common hereditary chronic disease of the mucus and sweat glands. It largely affects the lungs, pancreas, sex organs, intestines, liver, and sinuses. The condition is due to a defective gene that produces an irregularly shaped form of a cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is a type of protein that is essential to the body.

People having cystic fibrosis do not have enough CFTR for their cells to work normally because their bodies quickly destroy the mutant CFTR. Majority of Cystic Fibrosis cases result from the lack of an essential amino acid building block in the CFTR, causing it to fold into its characteristic and functional three-dimensional structure abnormally. A protein that fails to fold into the intended shape usually produces inactive proteins with different characteristics. This is how CF affects the mucus and sweat glands.

Normally, mucus is watery, thus keeping the linings of certain organs are moist and keeps them from drying out or getting infected. But in the case of CF, the abnormally shaped CFTR causes mucus to become thick and sticky. The mucus tends to build up in your lungs and block the airways. The condition is then a potent venue for bacteria to grow causing serious lung infections, which will eventually damage the lungs. The most common cause of death of CF patients is respiratory failure.

In the same way, mucus build up can block ducts in the pancreas. With this, digestive enzymes produced by the pancreas, will not be able to reach the small intestine. These pancreatic enzymes are responsible for breaking down dietary nutrients in order for the body to efficiently absorb fats and proteins. Moreover, the abnormally shaped protein or CFTR can induce your body to withdraw large amounts of salt, upsetting the mineral balance in your body.

A specific treatment plan is required for each particular CF condition. For cystic fibrosis affecting the lungs, regular chest physiotherapy, an average of two time daily, is very important. This procedure helps clear away the thick mucus in the airways.

Exercise is also beneficial. It helps regulate blood and oxygen circulation as well as encourage muscle function in the body. Inhalers and oxygen are also helpful in this lung disease for it can open up the airways.

Antibiotics and antifungals are standard part of the treatment and are usually taken for long periods. It helps with the inflammation and pain. The dosage and type of the medications would depend on the type of bacteria infecting the body and the severity of the disease. Also, others may decide to take proteolytic enzyme supplements such as serrapeptase or treatments to help in clearing inflammation and mucus.

For people with CF that primarily affecting the digestie system, they must take enzyme supplement. People with such disease will have difficulty in absorbing certain nutrients due to the decreased digestive enzymes. Thus, taking enzyme supplements such as lipase alongside a diet high in fat and carbohydrates is a standard treatment.

By: Paul S Fitzgerald

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Cystic fibrosis is one of the most common genetic disorders. The genes on each chromosome control specific functions of the human body. The gene on chromosome 7 produces a protein called cystic fibrosis transmembrane regulator. Mutation in the DNA level of chromosome 7 leads to the absence of this protein which leads to cystic fibrosis.

Cystic fibrosis is a recessive disease. It means that both copies of the gene must be defective. An affected person will have abnormal cystic fibrosis transmembrane regulator gene on each chromosome 7. Therefore both biological parents must have an abnormal gene. One abnormal copy is inherited from each parent. A person with one normal gene and one abnormal gene is called a cystic fibrosis carrier. Cystic fibrosis carriers do not show any symptoms but they may pass the abnormal gene to their children. Therefore the parents can be either cystic fibrosis carriers or affected themselves.

Cystic fibrosis affects the production and function of cystic fibrosis transmembrane regulator. This disturbs the chloride transfer across cell membranes. As a result, chloride irons build up in the cells of the lungs and other organs. Water stays inside the cells to dilute the chloride and the normal secretions of the organs become thick. Mucus in the exocrine glands becomes thick and sticky. This results in blocks in the pancreas, lungs, liver and other glands. This block leads to cyst formation in glands. This results in cystic fibrosis. Cystic fibrosis causes frequent respiratory infections.

Diagnosis of cystic fibrosis is confirmed by a sweat test or DNA testing. Cystic fibrosis causes the sweat glands to secrete excessive salt. The most common symptoms of cystic fibrosis are coughing and chronic breathing difficulties. This causes repeated lung infections. The lungs and the digestive system are more affected by cystic fibrosis. The symptoms of cystic fibrosis include chronic cough, chest infection, small growth in nose, diabetes, weight loss, pancreatitis, and diarrhea. The treatment of cystic fibrosis includes oral or inhaled antibiotics, corticosteroid tablets, dietary vitamins, anti-asthma therapy and medication.

By: Kent Pinkerton

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