Overview of Inflammation Treatment

The airways of patients with cystic fibrosis (CF) are in constant danger of becoming inflamed. Airborne pathogens such as allergens and pollutants are the leading cause of inflammation. Viral or bacterial infections can also cause the airways to tighten, making breathing more difficult, and reduce a CF patient’s ability to perform mucus clearance maneuvers. It is believed that the lungs of cystic fibrosis patients are prone to inflammation as a result of the malfunction of the CFTR protein.

Repeated infection, especially as a result of mucoid Pseudomonas aeruginosa (PA) can result in lasting damage to lung tissue. PA has also been observed to cause a hyperimmune inflammatory response. In other words, the body reacts to PA even more strongly than it does to other bacterial infections.

Lung inflammation is present even in patients who are not exhibiting signs of active infection. Again, this is due to the body’s inability to utilize the chloride ion transport across cells. Studies conducted on newborns with cystic fibrosis have hypothesized that at birth the lungs are still normal. However, with each subsequent infection, damage to the airways becomes cumulative as the airways continue to narrow. In order to avoid the lasting effects of inflammation, it is important for cystic fibrosis patients to work with their physicians to determine an appropriate preventive maintenance routine.

One of the prevailing treatments to control inflammation includes the use of high dose ibuprofen. Typically this is effective for younger patients who have not yet experienced many pulmonary exacerbations. Other therapies include:

1. Corticosteroids[1]
2. Macrolide antibiotics[2]
3. NSAIDs (non-steroidal anti-inflammatory drugs)[3]
4. Defensins and cathelicidins[4]
5. Leukotriene receptor antagonists[5]
6. Alpha-1-antitrypsin and secretory leukoprotease inhibitor (SLPI)[6]

For more information, talk to your CF specialist or pulmonologist about whether preventive anti-inflammatory agents is appropriate for you or your child.

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[1] Medications (eg, prednisone) that are related to cortisone, a naturally occurring hormone. Corticosteroids lessen inflammation, swelling, and pain. In some cases, corticosteroids are injected directly into a joint; however, they can have serious side effects - such as damage to bones and cartilage - especially when used over long periods of time.

www.ehealthmd.com/library/osteoarthritis/OSA_glossary.html

[2] The macrolides are a group of drugs (typically antibiotics) whose activity stems from the presence of a macrolide ring, a large lactone ring to which one or more deoxy sugars, usually cladinose and desosamine, are attached. The lactone ring can be either 14, 15 or 16-membered. Macrolides belong to the polyketide class of natural products.
en.wikipedia.org/wiki/Macrolides

[3] Non-steroidal anti-inflammatory drugs, usually abbreviated to NSAIDs, are drugs with analgesic, antipyretic and anti-inflammatory effects - they reduce pain, fever and inflammation. The term "non-steroidal" is used to distinguish these drugs from steroids, which (amongst a broad range of other effects) have a similar eicosanoid depressing anti-inflammatory action. NSAIDs are sometimes also referred to as non-steroidal anti-inflammatory agents/analgesics (NSAIAs). ...
en.wikipedia.org/wiki/NSAID

[4] Similar to antimicrobial peptides. These peptides are excellent candidates for development as novel therapeutic agents and complements to conventional antibiotic therapy because in contrast to conventional antibiotics they do not appear to induce antibiotic resistance while they generally have a broad range of activity, are bacteriocidal as opposed to bacteriostatic and require a short contact time to induce killing. A number of naturally occurring peptides and their derivatives have been developed as novel anti-infective therapies for conditions as diverse as oral mucositis, lung infections associated with cystic fibrosis (CF) and topical skin infections. Source: wikipedia

[5] Leukotriene receptor antagonists, such as montelukast sodium (e.g. Singulair tablets) or zafirlukast (e.g. Accolate tablets), treat asthma via a totally different pathway to other available medicines. They work by blocking substances in your lungs called leukotrienes, which cause narrowing and swelling of the airways. Blocking leukotrienes can improve asthma symptoms, including night-time symptoms, and can help prevent asthma attacks. http://www.mydr.com.au/default.asp?article=2700

[6] Acid-stable proteinase inhibitor (elastase, cathespin) against proteinases liberated from liberated from cervical mucus disintegrating leukocytes; from sputum in bronchial infections. source: http://cancerweb.ncl.ac.uk/cgi-bin/omd?antileukoprotease