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Understanding and Managing Cystic Fibrosis

Understanding and Managing Cystic Fibrosis

Cystic fibrosis, also known as mucoviscidosis is a genetic disorder where the autosomal recessive gene is affected. The disease affects the lungs, heart, pancreas, liver and intestines. It is characterized by abnormal transport of sodium and chloride through epithelium, making the secretions thick and viscous.

cystic fibrosis affected organs

The name refers to scarring (fibrosis) and cyst formation within the pancreas that was first documented in 1930s. Due to the abnormal secretions, lung infections are frequent and breathing difficulty is the most serious symptom. Other cystic fibrosis symptoms are poor growth, enlarged heart, sinus infections, respiratory pulmonary diseases, digestive problems, infertility and abnormal pancreas function.

The first indication of cystic fibrosis are salty skin, poor growth and weight gain despite normal food intake, thick sticky mucus, frequent respiratory infections, persistent cough and shortness of breath.

Lungs and sinus infections result from clogging because of the thick sticky mucus, reduce ciliary mobility and inflammation.

Inflammation and repeated infections cause structural changes in the lungs leading to many symptoms; in early stages, constant coughing, increase phlegm production and decrease resistance to effort and physical exercise. In later stages, when structural changes in the lungs and airways occur, there are breathing difficulties and difficulty in getting enough oxygen to the blood (hypoxia). Coughing up blood, recurrent pneumonia, high blood pressure in the lungs, respiratory failure, heart failure, are other serious symptoms.

The para nasal passages and sinuses may also be affected due to the thick mucus and sinuses infections are common, together with their symptomatology, headache, facial pain, fever, nasal drainage. Nasal polyps are also very common to develop as a result of multiple sinus infections. Cardio-respiratory complications are the most cause of death in people with cystic fibrosis.

The lungs of persons with cystic fibrosis are infected and colonised with bacteria from an early age. These types of bacteria and their characteristics change and develop in time in persons with cystic fibrosis, because they have a perfect environment in the thick viscous mucus and they develop resistance to commonly used antibiotics. More often these types of flourishing bacteria are spread between different individuals with cystic fibrosis. People with cystic fibrosis have to be aware of this danger and avoid spreading these dangerous strains of bacteria among other cystic fibrosis sufferers. In healthcare facilities, people with cystic fibrosis should be isolated from one another and strict hygiene should be maintained to avoid spreading strains of bacteria among them.

Beside uncommon strains of bacteria, different types of fungi and / or yeasts can chronically colonised the airways, some of them found almost exclusively in patients with cystic fibrosis. Defective mucociliary clearance favorites the growth of these fungi / yeasts colonies, together with prolonged antibiotic and corticosteroid therapies that also favorite their growth.

The respiratory complications can be reduced using salt therapy. Hypertonic saline solution is used in hospitals to help in clearing the thick stagnant mucus and avoid infection. The long-term salt aerosol exposure, in home salt therapy, provides exposure during the night sleep, when the micro particles of salt slowly deposit on the respiratory mucosa. It helps to make the thick mucus more fluid, unclogs the airways and reduces the inflammation. Salt is known to have bactericide properties and helps in fighting bacterial infections, beside its mucolytic and anti inflammatory effect. Using a home salt therapy device will create a seashore microenvironment in your own house.