Cystic Fibrosis Overview - CHRONIC Pulmonary Treatments

• Reduce APE (acute exacerbations) and preserve lung function! 

Goals of CF pulmonary treatment 

Chronic vs Acute Pulmonary Exacerbations

Two types of treatment options for CF

• Anti-obstructive treatment

• Anti-inflammatory treatment

• Aerosolized antibiotics

Chronic treatment options for CF include:

Chronic medications AND systemic antibiotics

APE (acute exacerbation) pulmonary treatment options for CF include

Supportive/symptom treatments and CFTR modulators 

Majority of patients with CF will be treated with_____ 

Airway clearance therapies (ACT)

When someone has an Airway obstruction with CF, you treat them chronically with non-pharm by: 

Hypertonic Saline OR Recombinant human DNAse (can include non-pharm + pharmaceuticals options)

When someone has an Airway obstruction with CF, you treat them chronically with pharm by: 

All patients with CF - to clear sputum, maintain lung function, and QOL.

GOAL:

Loosen thick mucus so it can be removed by coughing 

Non-Pharmacologic Airway clearance therapies (ACT) for obstruction are recommended for ______

1. Dornase Alfa - (for 6+ yo with mild/asx to severe lung disease)

2. Hypertonic Saline (improve lung function, QOL, reduce exacerbations) 

3. Azithromycin (chronic persistent PsA to improve lung function/reduce exacerbations)

4. Inhaled Tobramycin (chronic persistent PsA to improve lung function/reduce exacerbations)

Chronic medications for maintenance of lung health (supportive meds) - all for reducing exacerbations

• Increases airway surface liquid volume WHICH improves mucociliary clearance (thins mucus)

Inhaled Hypertonic Saline MOA

• Improves lung function

• Reduces APE

Inhaled Hypertonic saline benefits

• Bronchospasm (pretreat with albuterol if occurs)

Recommendations for use:

• All adults and children > 6 years

• 7% nebulized BID

inhaled hypertonic saline Adverse effects AND recommendations for use

Pre-school age; Lung clearance index

Small airway changes can occur as early as ______ , and post-guideline literature shows statistical significance in decrease of _____ with Hypertonic Saline

• Improves outcomes in infants, cost effective/easy use

• Tolerable AND Safe

benefits of 7% HTS in infants

• Works as recombinant DNAse

• Causes extracellular DNA break down - which causes DECREASED SPUTUM VISCOSITY! (thinner mucus)

Dornase Alfa MOA

Improves long function and reduces exacerbations

Dornase Alfa benefits

• use in 6yo +

• 2.5 mg inhalation daily

Dornase Alfa recommendations

1. Albuterol (use first)

2. HTS/Dornase Alfa (use second)

3. ACT (3rd)

4. Antibiotics (use last)

Order of using inhaled medications for CF treatment

ONLY when comorbid/concurrent asthma is suspected - Asthma is difficult to dx in CF patients

When should Anti-inflammatory corticosteroids be used in the treatment of CF?

Azithromycin

An alternative anti-inflammatory treatment for CF that is NOT steroids is

• Anti-inflammatory effect

• May. prevent biofilm production in PsA (bacteria)

Azithromycin MOA

Bacterial resistance (to non-tuberculosis mycobacterium) 

Potential Azithromycin adverse effects

• Use In 6yo+ WITH/WITHOUT chronic PsA

• Use in patients with NO LIVER DISEASE or B.cepacia/non-tuberc. Mycobacterium infection

• 250-500 mg daily dosing

Azithromycin recommendations for use

Pseudomonas Aeruginosa (PsA)

The major pulmonary predictor of CF mortality/morbidity that is NOT lung function, but a “BUG”!!!