7.3 treatment

Pharmacotherapy in CF targets various aspects of the disease, including

the underlying genetic defect,

respiratory complications,

and nutritional deficiencies.

pharmacological managment

CFTR modulators

antibiotics

mucolyics

pancreatic enzyme replacement therapy

CFTR modulators (look at anatomy genetics of CF)

improve the function of defective proteins for patients with specific CFTR mutations

antibiotic therapy is used for chronic pulmonary infections which are a significant concern in CF managment

inhaled antibiotics

oral/IV antibiotics

inhaled antibiotics

For individuals with chronic P. aeruginosa infection, sustained treatment with an inhaled antibiotic is recommended to reduce bacterial load and frequency of exacerbations.

oral + IV antibiotics

Used during exacerbations or for eradication of specific bacteria, tailored based on sputum culture results.

Mucolytics are used manage the thick, sticky mucus characteristic of CF, mucolytic agents are employed:

• Dornase alfa:

• Hypertonic saline:

• Dornase alfa:

• A recombinant human deoxyribonuclease that breaks down DNA in the sputum, reducing its viscosity and aiding in airway clearance.

• Hypertonic saline:

• Inhalation of hypertonic saline draws water into the airways, thinning mucus and facilitating its removal.

Pancreatic Enzyme Replacement Therapy (PERT)

Due to pancreatic insufficiency, many individuals with CF require PERT to aid digestion and nutrient absorption. This therapy involves taking pancreatic enzyme supplements with meals and snacks to ensure adequate nutrition.

Non-Pharmacological Management

Nutritional Support

Given the impact of CF on the digestive system, nutritional management is vital. This includes vitamin supplementation, particularly fat-soluble vitamins (A, D, E, and K), and dietary interventions to ensure adequate caloric intake and nutrient absorption. Dietitians within the MDT play a key role in developing personalized nutrition plans.