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Cystic Fibrosis- PFC Ofori Addo


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Cystic Fibrosis- PFC Ofori Addo

  1. 1. Cystic FibrosisPresented by Ofori-Addo, Eugene
  2. 2. The Disease – Cystic Fibrosis  Cystic fibrosis is known to be an inherited autosomal (pattern of inherited genetic disease) chronic disorder mostly seen in children that affects the lungs and the digestive system.  It is also known to be most common cause of chronic lung disease mostly seen in children and younger adults.  Also known to be the most fatal hereditary disorder that affects numerous Caucasians in the United States  (Medline Plus, 2013, Cystic Fibrosis Foundation)
  3. 3. Statistics  As the most common Chronic lung disease in children and some adults, this disorder affects about-  30, 000 children and young adults  Where about 1200 children are diagnosed every year  About 70% of these patients are diagnosed with the disease at age 2  And about bout 40% of the population being 18 years of age and older.  Life span of patients with Cystic Fibrosis is about 37 years (Cystic Fibrosis Foundation, 2014)  * Respiratory failure is mostly the major consequence of Cystic Fibrosis and usually what causes death amongst CF Patients Population Children age 2- 70% Young Adults - 40%
  4. 4. Causes of Cystic Fibrosis  This is an inherited diseases complication so its is genetically transmitted  From parents  through Genes  2 copies of genes are needed to signify inheritance of the disease
  5. 5. Pathophysiology  Cystic Fibrosis becomes present when there is a  Defective gene i.e. related to protein that is involved in chloride ion transport  Patients with the diseases usually develop unusual thick sticky mucus plugging that clogs the lungs and causes obstruction which leads to life- threatening lung infections and also obstruction in the pancreas.  Cystic Fibrosis Explained  JPQEY
  6. 6. Symptoms  Cystic fibrosis have several symptoms which include -  A persistent cough that produces thick spit (sputum) and mucus  Wheezing  Shortness of breath  Meconium ileus which appears at birth  Salty-tasting skin which is usually greasy feeling, bulky and has a bad smell  Frequent lung infections
  7. 7. Diagnostic Testing  Genetic Study (analysis)  When a new born child has signs and symptoms confirmed, diagnosis of CF can usually be done by using Blood Test  Since it is an inherited diseases, family members should be checked for possible diagnosis  The Sweat test  This test is a very accurate way of diagnosing CF.  Test measures Sodium and or chloride amounts present in patient’s sweat. (test not reliable in newborns)
  8. 8. Other common diagnosis  When a patient presents Cystic Fibrosis, they are usually seen to have these further complications-  Inability for effective airway clearance  Inability to digest food and absorb nutrients which causes imbalanced nutrition
  9. 9. Cystic Fibrosis – X-RAY  Young man with a history of cystic fibrosis has hyperinflation and predominantly upper lobe bronchiectasis.  Bronchiectasis is a condition in which damage to the airways causes them to widen and become flabby and scarred.  A chest x ray can show areas of abnormal lung and thickened, irregular airway walls.
  10. 10. Therapy and Management  Planning and intervention usually include-  Provision of respiratory therapy treatments  Chest physiotherapy to clear secretions  Administering medications  Meeting nutritional needs  Providing fat soluble vitamins like A, D, E, K to prevent deficiency  Providing psychosocial support
  11. 11. Treatments  These help and aim at relieving symptoms and complications of CF  Aerosolized antibiotics  Mucus-thinning drugs that thin out secretions and makes it easier to cough up  Use of bronchodilators  As the purpose of relaxing smooth muscles in the airways  Bronchial airway drainage  Postural drainage
  12. 12. Drugs Contd..
  13. 13. Treatment contd..  Enzymes taken orally for better nutrition  Should contain high calorie diet since CF patients are usually malnutritioned due to pancreatic enzymes needed to digest are not available  Lung transplant at its worst Significant when there is severe breathing problems that has lead to life-threatening infections of the lungs  Pain relievers such as  Ibuprofen- may help slow down lung deterioration
  14. 14. Questions? What do you call a nun with a lung infection and general mucus build-up? Sister Fibrosis.