Cystic fibrosis: what is that disease, the symptoms and treatment in adults, diagnosis

What is cystic fibrosis and how to treat it in adults?

Cystic fibrosis is a genetic disease that affects the glands of internal secretion. In addition to respiratory disease covers the intestinal tract and lungs, and also pancreas and liver. The reason for the formation of cystic fibrosis has not been fully elucidated, but it is based on a genetic predisposition. During the progression of the disease is the disruption of chloride, which cease to move inside the body and cause the formation of thick secretions between the cells in the pancreas and the mucous membranes.

The disease is dangerous to humans, as has the mark on all the internal organs of the patient. Until recently it was thought that cystic fibrosis is only a children’s disease, but researchers found that the disease can appear at any time regardless of age. In the complex treatment of the duration of life increases several times. If you are diagnosed with the disease, first we need to know what is the disease cystic fibrosis, and therapies to reduce symptoms.

Characteristics of the disease

Cystic fibrosis is a chronic disease in which there is disruption of the internal organs. The disease causes the formation of a viscous and sticky fluid that with the passage of the disease builds up between the cells of the body and causes the formation of the inflammatory process.

For reference! On average, cystic fibrosis diagnosed in one patient from 3 thousand babies.

Cystic fibrosis is dangerous for the patient’s death. But with the progression of medicine, in the treatment of inflammation included a new antibacterial therapy and anti-inflammatory drugs. Thus, the average life expectancy of the patient is much increased. In time to begin treatment, people should know how is cystic fibrosis and its main features.

Cystic fibrosis is not a contagious disease. It is transmitted by inheritance, affecting the seventh gene of the patient. Cystic fibrosis code of ICD-10 has a common sign Е84.

A mutation in the gene cystic fibrosis leads to disruption of protein synthesis. Due to the dysfunction of the chromosome, the accumulation of chloride ions between cells of exocrine. Due to the attraction of moisture from the body, between the cells of a sticky mucus. Over time it turns into a viscous substance and causes malfunction of internal organs.

Inflammation manifests itself in the form of lesions of the pulmonary system, disorders of the sweat glands, liver and kidneys. Often cystic fibrosis may cause infertility and chronic diseases of the bronchi.

Accumulated viscous mucus becomes the cause of accumulation of filth and dust in the lungs, and aggravating factor of inflammation in the bronchi. Because of the dysfunction of hollow tubes in the respiratory system, the patient often suffers from bacterial and viral diseases. Violation of the protective functions of the bodies leads to a decrease in the immune system and inability to fight pathogens.

What are the symptoms?

Symptoms of cystic fibrosis in adults are manifested in different ways and largely depend on the degree of inflammation. Regardless of the immune system and way of life of the patient, the first cystic fibrosis affects the respiratory tract, and then leads to dysfunction of the gastrointestinal tract.

The clinical picture is marked in the following way:

  • in the first few months the patient is suffering from dry cough;
  • dryness of the nasal passages and mouth;
  • frequent sneezing reflex;
  • the mucus accumulation in the bronchi;
  • constant fatigue and lethargy;
  • sleep disturbance and loss of appetite;
  • in the last phases of development of cystic fibrosis there is a great loss in weight;
  • if the child got sick, parents notice the violation of physical development;
  • in the early stages, the body temperature normal or slightly higher than normal, but with the progression it greatly increases;
  • shortness of breath;
  • severe shortness of breath;
  • swelling of the face and mucous membranes in the nose;
  • loss of interest to everything around him.

For reference! Symptoms of cystic fibrosis depend on the degree of inflammation. Not always, the patient will notice the onset of the disease in the early stages.

The main symptom of inflammation is continuous and severe cough. In the early stages it can occurs without expectoration, but with the progression of the disease is accompanied by strong deterioration of health. Cough impact on sleep, causing insomnia. In the future, it causes discoloration of the face.

In addition to the basic clinical picture, patients were seen a strong increase in temperature, itching, chills, headache, nausea, and vomiting. Another symptom of cystic fibrosis is a violation of the structure of the thorax.

Diagnosis of cystic fibrosis

To diagnose cystic fibrosis, the patient must undergo a series of examinations, which shall be handed over analyses of urine, feces, blood, phlegm. After collecting the anamnesis, the patient goes to a test for detecting the amount of chloride ions in the sweat secretion.

For reference! Before this test, the patient is administered the drug pilocarpine.

For accurate diagnosis the patient should take the test at least three times. If all the indicators the chlorine level above 60 mmol/l, the patient put an accurate diagnosis.

Complex therapy

Treatment of chronic diseases in the respiratory tract and the gastrointestinal tract takes a long time. In the process of therapy, it is important not to skip the medication and monitor the patient’s state of health. It is clear that to get rid of the inflammation completely impossible, but the doctors need to minimize the symptoms and improve the patient’s condition. In addition, it is necessary to reduce the risk of complications and progression of disease.

In the course of the complex treatment includes the following medications:

  • With the development of cystic fibrosis in the nasal cavity accumulates dirt, dust and other harmful particles. To get rid of pathogens the patient needs daily to rinse the nasal passages with the help of firstgov sea or ocean water. The best preparations in this area are: «Dolphin», «Physiomer», «Aqualor», «Marimer», «Humer», «Aqua Maris», «no-Sol».
  • After the reorganization in the nasal passages, type a vasoconstrictor. It will allow you to restore airway function, reduce swelling of tissues and improve the General condition of the patient. Usually, doctors prescribe the following drops: «Glycine», «Otrivin», «Naphazoline», «Nazivin», «Rinorus, «ksimelin», «Llanos».
  • When applying drops for constriction of the blood vessels, don’t forget to moisturize nasal passages with the help of drugs based on essential oils. For this usually use eye drops, «Pensol» or lubricate nasal passages with olive oil.
  • In the complex treatment included a course of antibiotics. They reduce the severity of symptoms and eliminate pathogens in the bronchi. To achieve these goals is assigned to the following drugs – «Amoxiclav», «Amoxicillin», «Arlet», «Suprax», «Pance», «Iksim», «Macroben», «Cephalexin» and others.
  • To provide anti-inflammatory, desensitizing agents, and anti-toxic action on the organism, the patient is prescribed corticosteroids. To use synthetic drugs only after consulting your doctor. Usually doctors prescribe certain medicines, among which the best are «Alloderm», «Alklometazon», «Prednisolone».

When acute course of cystic fibrosis, the patient is prescribed oxygen therapy. Because of persistent cough, patient can’t breathe, which causes a number of negative processes in the body. To saturate tissues with oxygen, the patient should undergo pulmonary, oxygen mesotherapy or periodical hypobaric hypoxic stimulation.

In addition, the patient must take vitamins and minerals, as well as to monitor General health. With the progression of inflammation you should always be under the supervision of a physician, and in a period of remission, it is important to strengthen the immune system.

To improve the patient’s condition, treatment includes physical therapy. The most effective physiotherapy treatments described here.

Conclusion

The treatment of cystic fibrosis takes several months. After remission, the patient should not relax, because at this time, it is important to strengthen the immune system and be ready for new attacks. To recover the body at this time should take decoction of medicinal herbs and spend more time in the fresh air.

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