Cystic fibrosis is what kind of disease

Parents despair when their child is diagnosed with cystic fibrosis. The disease is incurable and need a lot of strength and courage to deal with the baby. Unfortunately, not all parents are ready for it and young families break up. Modern medicine and the efforts of parents to help extend the life of the child make him socially active during such severe illness.

Forms of cystic fibrosis

A doctor examines a child with cystic fibrosis

Cystic fibrosis is what kind of disease, what is so dangerous? Why often affects children? Cystic fibrosis or cystic fibrosis, a terrible genetic disease that causes a defective gene in the DNA of the parents. The carriers themselves are not aware of the existence of the disease and not get sick. They have up to 5% of the population of the European race. At conception, the genes are passed to the child by both parents. When moms and dads have a predisposition to cystic fibrosis, it is possible that the baby will get sick. The probability of disease about 25% of genes can be transferred without modifications.

What is cystic fibrosis? Is an incurable hereditary disease that causes mutation of the gene responsible for the viscosity of mucus secreted by the body. It disrupted the work of many important systems. When a person is healthy, the glands of the body produce a secret, which helps with normal functioning. It is the mucus that facilitates proper functioning of lungs, digestive organs, genital sphere. In cystic fibrosis, it becomes viscous, can not normally excreted from the body. There are forms of the disease:

  • broncho-pulmonary;
  • intestinal;
  • mixed.

Broncho-pulmonary

Cystic fibrosis is what kind of disease, why it affects the respiratory system? When a person is healthy, the lungs produce mucous secret. During breathing with phlegm from the body removes harmful bacteria, dust particles. In cystic fibrosis creates a viscous mucus plugs, clogs the bronchi. Conclusion the secret is difficult, the bacteria begin to multiply, leading to infections. Genetic disease cystic fibrosis often ends in death because of infections. The danger is even respiratory disease.

Accompany with cystic fibrosis pneumonia and bronchitis, endless cough. Violation of excretion of mucus, obstruction of the bronchi and chronic pneumonia lead with high probability to the occurrence of serious diseases. In cystic fibrosis often:

  • pulmonary insufficiency;
  • pulmonary fibrosis;
  • heart failure;
  • pulmonary hemorrhage;
  • pneumothorax.

The child is undergoing treatment

Intestinal

The removal of mucus plays a huge role in the functioning of the digestive system. Due to the secretion of normal working liver, pancreas, the food is digested. In patients with cystic fibrosis more viscous mucus creates jams that hinder the work of the bodies. Ceases to stand out, the bile, the pancreatic enzymes. The food is not digested, appear constipation, affects the liver, the pancreatitis develops.

In cystic fibrosis in an organism there are processes of putrefaction, which are accompanied by flatulence, foul-smelling stool. In infants is poorly digested breast milk, the stool becomes oily, with an unpleasant odor. Increased appetite and weight gain is not observed. Bowel movements in cystic fibrosis too rapid, possible rectal prolapse. In older adults with the disease have problems with chewing because of reduced flow of saliva.

Combined

Since cystic fibrosis affects the system of the secretions, are rarely found separately disease of lungs or digestive organs. Frequent long-term cough, chronic bronchitis and pneumonia are combined with stool disorders, sudden weight loss. In adults cystic fibrosis is accompanied by infertility. Men disappear spermatozoa in the seminal fluid and in women, due to the increased viscosity of secretions, restricted mobility. When the disease worsens sweating which leads to dehydration, cramping.

A child with cystic fibrosis is engaged in physiotherapy

Symptoms in adults

Cystic fibrosis is what kind of disease, and which symptoms you can identify? In adults the symptoms are:

  • endless coughing;
  • shortness of breath;
  • heart palpitations;
  • thickening of the fingers;
  • the appearance of polyps in the nose;
  • loss of consciousness;
  • suffocation;
  • temperature rise;
  • deformity of the chest;
  • the deterioration of sweating;
  • weight loss;
  • constipation, diarrhea;
  • pain in the abdomen.

For cystic fibrosis is characteristic not only of individual symptoms but also the presence of many diseases. Diseases occur separately or simultaneously, are found in light and heavy form. Frequently observed:

  • pancreatitis;
  • chronic pneumonia;
  • cholecystitis;
  • chronic bronchitis;
  • pulmonary insufficiency;
  • pulmonary fibrosis;
  • sinusitis;
  • diseases of the genitourinary system;
  • infertility;
  • impotence;
  • emphysema;
  • sinusitis.

Signs of cystic fibrosis in children

A pediatrician examines a baby

The disease from the first days of baby’s life is characterized by constant coughing. Viscous mucus clogs the respiratory system, interferes with normal breathing, can not be expectoration. Are there traffic jams contribute to the development of pathogenic microbes. Infections, cepacia in cystic fibrosis result in toddlers bronchitis, pneumonia. Children lag behind in the development of the body, shortness of breath, fainting. On the intellectual development of the disease is not reflected.

When feeding babies because of the violation of production of enzymes processed into breast milk. Baby constantly wants to eat, and the weight does not increase. Even worse is manifested cystic fibrosis in newborns during breastfeeding. The disease is accompanied by:

  • violation of the chair;
  • increased flatulence;
  • bloating;
  • feces with an unpleasant odor;
  • weight loss;
  • vomiting;
  • paleness of the skin;
  • the appearance on the skin of salt crystals.

Diagnosis of disease

Analysis of cystic fibrosis

A first analysis of cystic fibrosis do in the hospital. It is mandatory for all newborns. After the birth on the fourth day, take a blood sample from the heel and determine the level of pancreatic enzyme. If performance improves, do the sweat test is the main analysis that confirms the disease. A woman during pregnancy can make DNA analysis for detection of cystic fibrosis. For this, the eighth through the tenth week of pregnancy, make a fence of amniotic fluid. Screening is required when the family is already sick.

Cystic fibrosis newborns diagnosed by the contents in the feces of fatty acids, chymotrypsin, an enzyme of the pancreatic juice. The growth of the baby diagnosis is performed in order to clarify the degree of organ damage in cystic fibrosis. In disease occasionally do:

  • chest x-ray;
  • chest x-rays;
  • spirometric measurements;
  • checking of hemoglobin;
  • the sputum cultures;
  • bronchoscopy;
  • measure blood oxygen saturation.

Sweat test for cystic fibrosis

This study is the most accurate in the diagnosis of the disease. The child was subcutaneously administered a special drug, Pilocarpine, which causes heavy sweating. Sterile cloth and collect the extracted liquid. In the analysis of cystic fibrosis determine the number of salts in the sample. Have a healthy baby content should not exceed 60 mmol/L.

Treatment of cystic fibrosis

Laboratory studies

Modern medicine does not know the way of complete cure of cystic fibrosis. Only medical observation, attention from relatives and his own desire is able to extend the life of the patient. Important is the exclusion of the possibility of contagious diseases. If you experience need surgery. When the disease periodically conduct tests, exercise therapy, diet. The main tasks of the fight against the disease is:

  • thinning mucus to make breathing easier;
  • to fight off infections;
  • regulation of the digestive system.

Great importance in the treatment plays a positive patient attitude, the desire to be socially active. Disease recommend daily sessions of breathing exercises, exercises that promote the excretion of mucus. In cystic fibrosis welcome sports. Useful are:

  • stretching exercises;
  • Jogging;
  • swimming;
  • active walking;
  • Cycling;
  • horse riding.

Diet

Because patients with cystic fibrosis often have increased appetite, but at the same time have a small body weight need to adjust the power. Special dietary restrictions no. Food should be nutritious, it is impossible to eliminate from the diet sugar, be sure to replenish lost salt. Food must be fractional. The diet must include:

  • meat;
  • eggs;
  • dairy products;
  • vegetables;
  • fruits;
  • nuts;
  • dried fruits;
  • cereals;
  • all the oil;
  • fish;
  • vitamin complexes.

Physiotherapy in cystic fibrosis

Medicines

Treatment of cystic fibrosis, prescription drugs and determining dosages should only deal with the doctor. Three important areas impact on the disease:

  • To fight off infections. Timely treatment with antibiotics helps to avoid death. Used in inhalations, injections, tablets, Amikacin, Oxacillin, Meronem – depending on the seeded bacteria.
  • Liquefaction of sputum. To cope with this task mucolytics – Fluimucil, Pulmozyme, Ambroxol.
  • Regulation of digestion with pancreatic enzymes and fat-soluble vitamins – A, E, K, d

Video: how to overcome cystic fibrosis and to continue to live

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