Cystic fibrosis: what it is and how it presents itself
Cystic fibrosis is a hereditary disease that affects the respiratory and digestive systems. The disease is transmitted by a recessive gene, so for a person to be affected, both parents must be carriers of this gene
Cystic fibrosis causes thicker-than-normal mucus production, which, in turn, leads to obstructions in the two systems; in the respiratory system, the mucus obstructs the airways, resulting in lung infections, and in the digestive system, it obstructs the bile ducts and impairs digestion.
By attacking the digestive system, cystic fibrosis inevitably also affects the functioning of the liver, pancreas, colon and all organs involved in digestion, making it increasingly difficult to absorb nutrients and eliminate waste from the body.
CHILD HEALTH: LEARN MORE ABOUT MEDICHILD BY VISITING THE BOOTH AT EMERGENCY EXPO
Typical symptoms of cystic fibrosis are:
- pale, fatty stools
- weight loss
- poor growth (due to malabsorption of nutrients)
- sweating more salty than normal
- frequent dehydration.
Symptoms pertaining to the respiratory system are generally the presence of nasal polyps, pneumonia, bronchitis, thick mucus production and shortness of breath.
Other symptoms may be diabetes, gallstones, pancreatitis, liver disease.
Examinations and treatment of cystic fibrosis
Cystic fibrosis can be discovered through various examinations.
The first is undoubtedly DNA testing, which can establish whether the patient has the defective gene that causes the disease.
Other tests are pancreatic function tests, fat and trypsin tests in the faeces, lung function tests, and a chest X-ray.
Unfortunately, there is no cure for cystic fibrosis, so any therapy is mainly based on improving the patient’s symptoms and quality of life.
Early diagnosis allows an appropriate treatment programme to be conducted on the patient, which can improve quality of life and life expectancy.
Pharmacological therapy is essentially based on antibiotics to treat respiratory infections and the administration of pancreatic enzymes to replace missing enzymes.
Associated with this therapy, supplementary vitamins and bronchodilators are often prescribed to combat airway obstruction.
Research is investigating new methods of correcting the hereditary defect at source; genetic engineering is attempting to ‘replace’ the diseased gene with a healthy one in cystic fibrosis patients.
Prognosis
The prognosis is not always very favourable; in general death occurs around the age of 40 from pulmonary complications such as pneumonia, pneumothorax, chronic lung failure.
Read Also:
Emergency Live Even More…Live: Download The New Free App Of Your Newspaper For IOS And Android
Cystic Fibrosis: Symptoms, Signs, Diagnosis, Tests
Severe Asthma: Drug Proves Effective In Children Who Do Not Respond To Treatment
Nasal Cannula For Oxygen Therapy: What It Is, How It Is Made, When To Use It
Oxygen-Ozone Therapy: For Which Pathologies Is It Indicated?
Hyperbaric Oxygen In The Wound Healing Process