Cystic fibrosis: early diagnosis - the key to recovery

Cystic fibrosis - a life-threatening disorder that causes severe lung damage and nutritional deficiencies. The hereditary disease cystic fibrosis affects the cells responsible for the production of mucus, sweat and digestive juices, which is why these secretions become abnormally thick and sticky. Rather than perform the function of lubricants, such fluids clog the channels and ducts, particularly in the lungs and the pancreas.

Cystic fibrosis is the most common among people originating from northern Europe; it occurs in one of 3000 newborns. In the past, the majority of these patients died in adolescence. Today, patients with cystic fibrosis often live to 50 years or longer.

Symptoms

Symptoms of cystic fibrosis can vary greatly in different patients and even in the same patient at different ages. Most of the symptoms associated with the work of the lungs and the digestive system:

• Persistent cough;
• Wheezing;
• Frequent lung infections;
• Repeated sinus infection;
• Particularly strong, unpleasant odor stools;
• Slow weight gain and growth retardation in the child;
• Frequent bloating due to constipation.

When the child has these symptoms should immediately seek medical attention.

Causes

Patients with cystic fibrosis have a defective gene that causes a mutation of the protein responsible for the delivery of salt (sodium chloride) in the cell and its withdrawal from the cells. As a result of the respiratory tract, digestive tract and reproductive system produces thick, sticky mucus.

The defective gene that causes cystic fibrosis is a recessive. Sick child only if it receives two copies of the gene, one from each parent. Having only one copy, he does not fall ill with cystic fibrosis, but can pass the gene to their children.

Complications

Cystic fibrosis can cause many complications, such as:

• Bronchiectasis. Cystic fibrosis is one of the main causes of bronchiectasis, disorders in which the airways widen, become flabby, and covered with scar tissue.
• Chronic infections. The thick mucus in the lungs and sinuses provide an excellent breeding ground for bacteria. The majority of people with cystic fibrosis almost always has any lung infection or sinus.
• The collapse of the lung. Recurrent lung infections increase the risk of lung collapse.
• Nasal polyps. Because of swelling and inflammation of the nasal cavity increases the likelihood of developing polyps - single or multiple growths inside the nose.
• Respiratory failure. Over time, cystic fibrosis can damage lung tissue so badly that she could not cope with their tasks. Respiratory failure usually develops gradually - as long as the state does not become life-threatening.

Complications of the digestive system

• Nutritional deficiencies. The thick mucus blocks the ducts, which come from the digestive enzymes the pancreas into the gut. None of these enzymes in the body can not absorb proteins, fats or fat-soluble vitamins - A, D, E and K.
• Diabetes. Cystic fibrosis significantly increases the risk of developing diabetes.

In addition, complications of cystic fibrosis may be blocking the bile duct, prolapse of the rectum, bowel obstruction, irregularities in the reproductive system, osteoporosis Osteoporosis - he threatens you?   and electrolyte imbalance.

For the diagnosis of cystic fibrosis carry out various laboratory tests and tests using medical imaging techniques. Always tested pulmonary function.

Treatment

In the treatment of patients with cystic fibrosis can be used the following drugs:

• Antibiotics are used to treat and prevent bacterial infections in cystic fibrosis.
• Preparations thinning the mucus easier to cough mucus, improving lung function.
• Bronchodilators such as albuterol, open the airways by relaxing the muscles around the bronchial tubes.

Patients with severe forms of cystic fibrosis and life-threatening complications of lung transplantation may be necessary. It may also be necessary if the patient develops resistance to many antibiotics used for treatment of pulmonary infections. Lung transplantation - a very complex operation, which can lead to serious complications.

Home Treatment

To successfully control the development of cystic fibrosis patients need to take action to improve their health in the home.

Since cystic fibrosis pancreatic enzymes Diabetes and pancreas - the things you need to know   not penetrate in sufficient amounts in the intestine, the nutrients of the food are not absorbed properly, which could lead to a shortage of nutrients. Therefore, patients need to take supplements of pancreatic enzymes and fat-soluble vitamins. In addition, the daily calories for patients with cystic fibrosis may be more than the standard rate.

Patients should drink plenty of fluids, as this facilitates expectoration.

In addition to conventional vaccines, which make all the children suffering from cystic fibrosis necessarily need a vaccine for pneumococcal disease, as well as annual flu shots The flu shot - does it make sense to do it? . Cystic fibrosis affects the immune system is not, but for various diseases, most people tolerate well, it can cause severe complications.

Regular exercise contribute to the conclusion of sputum, as well as strengthen the heart and lungs. In addition, many patients with cystic fibrosis sports are an excellent way to improve self-esteem - it makes them feel his own inferiority, limited capacity, which often arises in connection with the disease.

Smoking, both active and passive, is particularly dangerous for patients with cystic fibrosis. If your child is diagnosed with cystic fibrosis, did not allow anyone to smoke in your home or in your car; if possible do not bring the child to the places where smoking is allowed.

Psychological support is very important for patients with cystic fibrosis. Strong emotions Emotions and culture: how to decipher the emotional code Mainly anger and fear are very common among these patients and may manifest itself most clearly in adolescence. Parents, it is important to be sensitive to the psychological aspects of the disease. If necessary, seek the help of a psychologist - can be useful both individual and family counseling.