Lungs are normal at birth, but breathing problems can develop later. Thick bronchial secretions eventually block the small airways, which become inflamed as a result.
As the disease progresses, the bronchial walls thicken, the airways become filled with polluted secretions, and a collapse of healthy areas is created. All of these changes reduce the ability of the lungs to transfer oxygen to the blood.
About half of children with CF come to the doctor for the first time because they do not stop coughing and wheezing and suffer from respiratory infections. The cough is the most noticeable symptom. Over time the thorax acquires a barrel shape characteristic of chronic lung (pancreatic-chest) diseases. Also, the lack of oxygen supply to remote areas such as the skin creates finger clubbing and bluishness.
Balance of salt and water in the airways
Over time an irreversible dilation of the airways occurs, and this phenomenon is called bronchiectasis. Cysts (pus pockets) are formed an excessive inflammatory response. The cysts then undergo fibrosis, and pulmonary exacerbation occurs to the point of respiratory failure. Therefore early treatment is essential to prevent irreversible changes in health.
In the second decade of their lives, children develop slowly, their puberty slows down, and physical endurance decreases.
Adolescents and adults may have complications such as pneumothorax, bloody cough (hemoptysis). Recurrent pneumonia (the typical cause is the bacterium Pseudomonas aerogenosae) causes gradual destruction of the lungs to the point of pulmonary failure.
In 80% of patients, the blockage of the pancreatic ducts, which carry digestive enzymes to the intestine, causes a lack of absorption of fats and proteins. Lack of absorption manifests in the baby in multiple ejaculations, the stool is large in volume, smelly and greasy, and more than once, he has a swollen abdomen. Growth is slow even though appetite is normal or increased. The baby is thin, and his muscles are relaxed. Sometimes the initial manifestation of the disease in the first days of life is intestinal obstruction by the newborn’s feces (meconium ileus); this form of neonatal obstruction occurs in 17% of newborns with cystic fibrosis. , It blocks the intestines. There is poor absorption of fat-soluble vitamins A, D, EK.In adulthood, there is progressive destruction of the pancreas and damage to the internal (endocrine) glands that cause diabetes.T viscous secretions in the bile ducts can cause liver dysfunction and prolonged jaundice in the newborn. The adult will have a liver dysfunction and inflammation of the liver and eventually cirrhosis in a small percentage of patients.
Sometimes dehydration is the first manifestation of the disease. There is an increased salt secretion in the sweat, which is expressed in salty sweat. This disorder can lead to recurrent episodes of fluid and salt imbalance to the point of dehydration.