Cystic fibrosis medical therapy
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]
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Overview
Medical treatments for patients with cystic fibrosis include mucolytic agents (dornase alfa, N-acetyl-L-cysteine), airway surface rehydration (hypertonic saline, osmotic agents), anti-infective agents (for prophylaxis, eradication of early infection and suppression of chronic infection), anti-inflammatory agents (NSAIDs, inhaled corticosteroids, LTB4 receptor antagonists and Azithromycin) and potentiators of CFTR protein defect.
Medical Therapy
- Treatment for cystic fibrosis has targeted following consequences of the defect such as GI and pulmonary mucus plugging and infection.[1]
- Medical treatments for patients with cystic fibrosis are include:[2][3][4]
| Medical treatment in patients with Cystic fibrosis | ||
|---|---|---|
| Category | Approaches | Explanation |
| Mucolytic agents | Dornase alfa | Cleave the extracellular DNA and aid airway clearance |
| N-acetyl-L-cysteine | Also increase levels of the intracellular antioxidant glutathione (GSH) that protect against the neutrophil-driven tissue damage | |
| Airway surface rehydration | Hypertonic saline | As it may cause bronchoconstriction, it is commonly used with an bronchodilator |
| Osmotic agents | Mannitol is a nonabsorbable sugar alcohol which provides an osmotic gradient on the airway surface | |
| Correction of ion transport | ||
| Anti-infective agents | Prophylaxis | Anti-staphylococcal antibiotics (such as flucloxacillin) until ~3 years of age is recommended to reduce the incidence of methicillin-susceptible S. aureus (MSSA) |
| Eradication of early infection | If P. aeruginosa not detected and treated aggressively, this gram-negative, opportunistic bacterium will become chronic.
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| Suppression of chronic infection | The most commonly used nebulized antibiotics against P. aeruginosa are tobramycin, colistin and aztreonam. | |
| Acute exacerbations | Pulmonary exacerbations are treated with oral or IV antibiotics depending on severity. | |
| Anti-Inflammatory agents | Nonsteroidal anti-inflammatory agents (NSAIDs) | Ibuprofen showed some benefit in young patients with mild disease in high doses. |
| Inhaled corticosteroids | ||
| LTB4 receptor antagonists | Leukotriene B4 (LTB4) is produced by macrophages and PMNs in response to infection and plays a significant role in inflammatory response. | |
| Azithromycin | ||
| CFTR protein defect | Potentiators | Enhance the activity of the CFTR channel if it is correctly located.
The most significant advance in the treatment of CF over the last few years has been the development of ivacaftor (ivacaftor increases the time the CFTR channel is open) |
| Correctors and combination therapy | lumicaftor/ivacaftor | |
References
- ↑ Edmondson C, Davies JC (May 2016). "Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications". Ther Adv Chronic Dis. 7 (3): 170–83. doi:10.1177/2040622316641352. PMC 4907071. PMID 27347364.
- ↑ Ratjen FA (2009). "Cystic fibrosis: pathogenesis and future treatment strategies". Respir Care. 54 (5): 595–605. PMID 19393104.
- ↑ Edmondson C, Davies JC (2016). "Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications". Ther Adv Chronic Dis. 7 (3): 170–83. doi:10.1177/2040622316641352. PMC 4907071. PMID 27347364.
- ↑ Konstan MW, Ratjen F (2012). "Effect of dornase alfa on inflammation and lung function: potential role in the early treatment of cystic fibrosis". J. Cyst. Fibros. 11 (2): 78–83. doi:10.1016/j.jcf.2011.10.003. PMC 4090757. PMID 22093951.