Cystic fibrosis is a form of hereditary disorder which severely damages the digestive system, lungs along with other vital organs of the body. This condition affects explicitly the cells that secrete sweat, mucus, or digestive juices. These secretions are smooth and thin usually, however, in people suffering from cystic fibrosis, there is a mutated gene that makes the secretions thick and sticky. Patients suffering from cystic fibrosis have secretions that block the ducts, passageways, and secretory tubes, especially in organs like pancreas or lungs.
The signs and symptoms of cystic fibrosis vary from person to person, depending on the condition of the disease. Most of these symptoms worsen with time or might improve with time, depending on the severity. Some people do not experience symptoms even till their adolescence. Individuals with cystic fibrosis have higher levels of salt within their sweat. Other symptoms are either related to the digestive or respiratory system. Respiratory symptoms include persistent cough, breathlessness, coughing, lung infections, exercise intolerance, stuffy nose, or inflamed nasal airways. The digestive symptoms linked with cystic fibrosis affect the transport of digestive juices from the pancreas to the small intestine, which in turn prevents the absorption of nutrients in the diet. The symptoms related digestive tract include abnormal growth and weight gain, greasy or foul-smelling stools, intestinal blockage, or severe constipation.
The causes of cystic fibrosis include a defect in the genes which regulate the transport of salt inside and outside the cells resulting in thick and sticky secretions of the mucus in different systems i.e., respiratory, digestive or reproductive systems. These genes are known as CFTR or Cystic fibrosis transmembrane conductance regulator gene.
The risk factors linked with cystic fibrosis include specific races like Northern European people have an increased tendency to develop this condition. Other than this, family history also matters a lot in the genetic transmission of cystic fibrosis.
The complications linked with cystic fibrosis include:
- Bronchiectasis where the airways get damaged leading to difficulty in the transport of air inside and outside the lungs
- Chronic lung infections due to thick mucus, which serves as the home to fungal and bacterial species.
- Nasal polyps, which occurs due to swelling and inflammation of the airway.
- Hemoptysis or bloody cough due to the thinning of the airways
- Pneumothorax where the air fills up in spaces surrounding the lungs and chest wall leading to breathlessness and chest pain.
- Respiratory failure
- Nutritional deficiencies, diabetes, blockage of the bile duct, intestinal obstruction, osteoporosis, dehydration, electrolyte imbalance, and many others.
For early diagnosis of cystic fibrosis, doctors recommend screening and diagnostic tests in the newborns as wells as older children and adults. These tests include a blood sample, genetic tests, and chest X-rays, sputum tests, pulmonary function tests, CT scans, and sweat tests.
As far as treatment is concerned, there is no such cure for treating cystic fibrosis. Various pharmacological and non-pharmacological measures can be adopted for relieving the symptoms linked with cystic fibrosis. For pharmacological measures, consult the doctor for a proper treatment plan. However, for home care, you need to follow the simple protocol to prevent yourself against lung infection, loosen the mucus from the respiratory tract, get yourself treated for intestinal blockage, consume adequate nutrition’s in your diet, and follow a healthy lifestyle. Try washing your hands frequently, eliminate smoking, perform exercises, attend the appointments regularly, and support each other.
Until Next Time,
Team Doctor ASKY!