How do they stay healthy anyway?

PREVENTATIVE THERAPIES:

   -vaccinations: influenza and pneumococcal: reduce viral infections
   -unfortunately... set limitations for proximity to other CF patients reduces exchange of infection



TREATMENT RECOMMENDATIONS:

1. CHEST PHYSIOTHERAPY:

COUGH IT OUT!

there are different methods of shaking up the chest walls to break free that THICK, STICKY MUCUS from the chest walls

-Chest percussion and drainage: physical slapping on with hands on different parts of the chest

Details from Cystic Fibrosis Foundation: You can Percuss to!

-external percussion vest (does the slapping for you and makes you an incredible singer)

-airway oscillating devices

if > 6 years old...

Other therapies:

NEWEST and COOLEST fighting at the CORE of the problem! 

Ivafactor:  (a CFTR modulator) 150 mg every 12 hours by mouth
              -restores function of the mutant CF protein at the base!
              -improves chloride movement
              -reduces pulmonary exacerbations
              -improves cough clearance!  (helps COUGH IT OUT!)

  **only works on specific mutations so genotyping is needed


Nebulizers:  inhaled medications

      mucolytics: Mucus busters
      hypertonic saline: draws water into airways to thin mucus and COUGH IT OUT!
      bronchodilators: open up airways before chest physiotherapy


Antibiotic Therapies:  dependent on the type of bacterial infection, give after Chest Physiotherapy

                    *popular antibiotics for popular infections

AZITHROMYCIN: an anti-inflammatory and antibiotic 
                            -research shows chronic usage improved chronic cough and lung function!

for P. aeruginosa bacteria
      -Tobramycin or gentamycin (aminoglycides) 
      -"Cillins": penicillin, amoxicillin, Cephalosporin, Carbopenams (beta-lactams) 

Intravenous antibiotics for treating exacerbations of cystic fibrosis 

A TABLE OF ANTIBIOTICS

ANTI-INFLAMMATORY:

chronic high does ibuprofen: new research proven helpful if CF lung function > 60%

      
Other choices for more severe CF:

-supplemental oxygen
-BiPAP: to help with difficulty expanding chest walls (positive pressure ventilation)
-Lung Transplant: contraindicated for patients < 12 y.o.
                            -none for infection with Burkholderia gladioli


MULTISYSTEM TREATMENTS:

may also need to treat for CF-related:
    -nutritional deficiencies: provide enzymes, tube feeding
    -diabetes: insulin management
    -osteoporosis: exercise or calcium supplements


References:

Cystic Fibrosis Foundation - Home. (n.d.). Retrieved February 14, 2015, from http://www.cff.org

Simon, R. (2014, December 18). Cystic fibrosis: Overview of the treatment of lung disease. Retrieved February 14, 2015, from http://www.uptodate.com/contents/cystic-fibrosis-overview-of-the-treatment-of-lung-disease?source=search_result&search=cystic fibrosis treatment&selectedTitle=1~150

Seattle Children's Hospital Policies and Procedures: Cystic Fibrosis Pathway