What Is Cystic Fibrosis? What Causes It?

In the United States more than 30,000 people suffer from cystic fibrosis (CF). Doctors diagnose about 1,000 new cases each year

CF affects cells in the body that produce mucus, sweat, digestive juice. Normally, these are very thin and slippery, so you can operate the body system smoothly. But if you have CF, they will be as thick as glue. This will block the whole pipe and pipe body

Eventually mucus accumulates in the respiratory tract. This makes breathing difficult. Mucus confines bacteria and causes infection. It can also cause serious lung injuries such as cysts (fluid filled sacs) and fibrosis (scar tissue). CF is named like this

It is not infectious. It is caused by a mutation (change) in a single gene called cystic fibrosis transmembrane conductance regulator (CFTR). It controls the flow of salt and liquid in and out of the cell. If the CFTR gene does not function properly, the whole body forms viscous mucus.

If you inherit only one, there are no symptoms. But you will become a carrier of this disease. This means that you may give it to your own children one day.

About 10 million Americans are CF carriers. Every time two CF carriers have children, children are born with 25% (1/4) chance.

The CF is not damaged in any part of the body of the lungs. This disease also affects the following organs:

Pancreas: Mucus that occurs because CF is occluding the pancreatic duct. This will prevent digestive enzymes (protein degrading food) from reaching the intestines. Therefore, it is difficult for your body to absorb the nutrients you need. This can also lead to diabetes for a long time.

Liver: When the tube which removes bile is clogged, the liver inflamed and cause severe scarring (liver cirrhosis).

Small intestine: Since the decomposition of strongly acidic food from the stomach is a difficult subject, the inner wall of the small intestine corrodes

Large intestine: A thick secretion (liquid) in the stomach thickens feces (feces). This can cause blockage. In some cases, the intestine may begin to break like an accordion (called 'intestinal nesting').

Bladder: Chronic cough may weaken bladder muscles. Approximately 65% ​​of CF women are suffering from so-called "stress incontinence". This means that if you cough, sneeze, laugh or lift, urine will leak out. It is more common in women, but even men can own it.

Kidneys: Some with CF have kidney stones. These small and hard mineral deposits can cause nausea, vomiting, pain. Ignoring it may cause kidney infection

Reproductive organs: Excess mucus affects fertility of both men and women. Most men with CF have a sperm or a tube leading to a so-called "vascular dementia". Women with CF have very thick cervical mucus, which makes sperm more difficult to fertilize eggs.

Other parts of the body: CF may also cause bone thinning (osteoporosis) and muscle weakness. Since it disrupts the balance of minerals in the blood, it can also cause hypotension, fatigue, rapid heart rate and general weakness.

CF is a serious condition requiring daily care, but many treatments have been improved. People with CF have much longer average life expectancy and improved quality of life.

Cystic fibrosis requires routine care, but people with this disease can usually work at school and work, and in the past few decades more people with cystic fibrosis The quality of life is good. Improvements in screening and therapy mean that people with cystic fibrosis can now live on average about 30 years of age, and some people can live in their 40s and 50s. Screening for cystic fibrosis neonates is currently available in all states in the United States. As a result, you can diagnose the condition within the first month before symptoms appear. It is important for people born before neonatal screening to understand the signs and symptoms of cystic fibrosis

Cystic fibrosis is an autosomal recessive disorder - that is, two defective genes (one for each parent) are needed to develop the disease. The gene thought to cause cystic fibrosis is called cystic fibrosis transmembrane conductance regulator or CFTR. This gene is on chromosome 7. The severity and extent of this disease vary greatly among patients with cystic fibrosis. Typically, this condition results in chloride transport defects through epithelial cells and an increase in the viscosity of bodily secretions, particularly from the respiratory tract (ie, lungs, throat) and pancreas. As a result, the patient is prone to pancreatic dysfunction and recurrent thoracic infection