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 cystic fibrosis  

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what is the treatment for cystic fibrosis?


chest physiotherapy, daily oral or inhaled antibiotics to counter lung infection, inhaled anti-asthma therapy, corticosteroid , dietary vitamin supplements, especially A and D, pancreatic enzymes, inhalation of a medication called pulmozyme to make the sputum less sticky, medicines to relieve constipation or to improve the activity of the enzyme supplements, insulin for CF-related diabetes, medication for CF-associated liver disease, oxygen to help with breathing, in severe cases, a lung or heart and lung transplant operation, help to overcome fertility problems, counselling to help cope with the psychological aspects.


Just to add more points:
CF is an autosomal recessive disorder of the exocrine glands. The pulmonary manifestations include acute and chronic bronchitis, bronchiectasis, chronic bouts of pneumonia, hemoptysis, and cor pulmonale, which can occur late in the disease. Other common findings include chronic cough, exercise intolerance, recurrent respiratory infections, digital clubbing, increased anteroposterior diameter, and basilar crackles. If the pilocarpine sweat test reveals sodium and chloride levels greater than 80 mEq/L, a diagnosis of CF can be made. The primary goals of treatment include thinning the mucus secretions, keeping the airways open, and treating recurrent infections. Thinning of mucus can be achieved with mucolytics such as N-acetylcysteine. N-Acetylcysteine(Mucomyst) splits the disulfide linkages between these mucoproteins, resulting in a decrease in mucous viscosity. It is indicated as adjuvant therapy in the treatment of abnormal viscid or inspissated mucus secretions in CF, chronic lung disease, post-traumatic chest complications, and atelectasis secondary to mucus obstruction. Inhaled bronchodilators are used to open the airways. Furthermore, prednisone has been shown to increase pulmonary function and increase body weight. The definitive treatment is lung transplantation.


how common CF in USA?


CF is the most common hereditary disease in the USA and Western country (around 1/1000 new birth).

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