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CYSTIC FIBROSIS
- 1. DR ASHRAF AHMAD PULMONOLOGIST KAASH – Taif 2015
- 2. INTRODUCTION is a multisystem disease affecting the digestive system, sweat glands, and the reproductive tract, but progressive lung disease continues to be the major cause of morbidity and mortality. Patients with CF have abnormal transport of chloride and sodium across the respiratory epithelium, resulting in thickened, viscous airway secretions
- 3. GENETICS CF is caused by mutations in a single large gene on chromosome 7 that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) protein . Clinical disease requires disease-causing mutations in both copies of the CFTR gene.
- 6. PATHOGENESIS CFTR malfunction is associated with low water content in secretions from the respiratory, pancreatic, and biliary epithelium, which causes the secretions to be viscous and difficult to clear. GIT: the abnormal bile and pancreatic secretions cause maldigestion and malabsorption, progressive liver and pancreatic disease, rectal prolapse, and intestinal obstruction (distal intestinal obstruction syndrome or intussusception).
- 7. CONT. Lung: the abnormal secretions cause chronic airway obstruction and reduce bacteriocidal killing, leading to progressive pulmonary colonization with pathogenic bacteria, and to the formation of bacterial biofilms. Chronic infection causes an inflammatory response and tissue destruction, causing bronchiectasis. Infection with P. aeruginosa is particularly favored in patients with CF, due to abnormally decreased oxygen tension within the hyperviscous mucous layer.
- 8. EPIDEMIOLOGY CF occurs in approximately 1:3000 Caucasians, 1:9200 Hispanics, 1:10,900 Native Americans, 1:15,000 African Americans and 1:30,000 Asian Americans .
- 15. TTT OF LUNG DISEASE
- 16. CFTR MODULATORS (Ivacaftor ) is a small molecular weight oral drug that is specifically designed to treat patients who have a G551D mutation in at least one of their CFTR genes. We recommend ivacaftor for all CF patients 6 years of age and older who carry at least one copy of the G551D CFTR mutation. Ivacaftor, 150 mg PO every 12 hours, should be taken with fat-containing foods.
- 17. ANTIBIOTICS Chronic treatment with oral antibiotics is not encouraged because the benefits have not outweighed the problems associated with antibiotic resistance with 2 exceptions: Azithromycin : its benefits may be due to its antiinflammatory and/or antibacterial properties. Nebulized antibiotics directed against Pseudomonas aeruginosa (eg, tobramycin and aztreonam) appears to improve lung function and is recommended for many patients.
- 18. BRONCHODILATORS There is clinical evidence that chronic use of beta-2- adrenergic receptor agonist therapy increases expiratory flow rates in patients with CF . This has led to the recommendation that clinicians prescribe albuterol every four to six hours, or a long- acting beta-agonist (salmeterol or formoterol) every 12 hours .
- 19. AGENTS TO PROMOTE AIRWAY SECRETION CLEARANCE Inhaled DNase I (dornase alfa) : decrease the viscosity of purulent CF sputum by cleaving long strands of denatured DNA that are released by degenerating neutrophils. Inhaled hypertonic saline :hydrate inspissated mucus that is present in the airways of patients with CF. CHEST PHYSIOTHERAPY
- 20. ANTI-INFLAMATORY THERAPY Macrolide antibiotics ; studies concluded that 6m of treatment with azithromycin improves respiratory function in patients with CF and reduces the frequency of pulmonary exacerbations . Systemic glucocorticoids Chronic administration : the CF Foundation recommends against the routine chronic use of oral corticosteroids for CF in the absence of asthma or ABPA, because of the associated adverse effects. Short-term treatment for acute pulmonary exacerbations : only for those with predominant asthma symptoms
- 21. Cont. inhaled glucocorticoids: Although it seems reasonable to continue prescribing aerosolized glucocorticoids to CF patients who have definite signs and symptoms of asthma or ABPA there is insufficient evidence to warrant broader use. We agree with the guidelines committee of the CF Foundation, which recommends against their routine use .