Inherited disease cause thick, sticky mucus to form in the lungs, pancreas and other organs. The gene responsible is CFTR, it retains chloride in body. It is also known as mucoviscidosis.
The mucus can block airway, causing lung damage and make it hard to breathe. In pancreas, it clogs the pathway that participates in digestive system. Presently there is no cure.
Early symptoms: failure to gain weight, no bowel movement in the first 24-48 hrs, salty tasty skin, pale/foul smelling stool with mucus, recurrent episodes of pneumonia
Cause of lung infections in patients with CF: Pseudomona aeruginosa, Haemophilus influenza, Staph aureus (MSSA, MRSA), MAC, ABPA - allergic broncho pulmonary aspergillosis
Diagnostic test: sweat gland chloride test
Inhaled antibiotics
Prevent and treat lung and sinus infection
Pseudomonas aeruginosa, S. aureus, H. influenzae are the most common organism cause lung infection.
Symptoms: cough followed by smelly greenish sputum.
Aeuruginosa requires 2 IV abx: AMG, beta lactam, quinolone; dose is larger than normal, concentration need to be obtained in lung
If several inhalants: take bronchodilator first, chest physiotherapy next, then other inhaled med, finally TOBI
Tobramycin (TOBI, Nebcin): ready to use ampule, store in fridge but can be kept R.T 28 days, use with nebulizer and compressor. Clean nebulizer every other treatment day, disinfect by boiling.
Require reconstitution w/ 1ml diluent, use immediately
SE: bronchospasm, fever
Promote mucus clearance
Acetylcycstein (Mucomyst)
A mucolytic agent also used for APAP toxicity.
3-5ml of 20% soln TID-QID, dilute with NS or sterile water
Given via nebulizer, may damage lung tissue and lead to bronchitis.
Pulmozyme does not, and is the preferred agent
Dornase alfa (Pulmozyme), DNA enzyme produced from Chinese hamster. It cleaves DNA and reduces mucous viscosity.
5mg via nebulizer QD x 6 mos. Store ampule in refrigerator and protect from light
Bronchodilator: given prior to chest percussion
Pancreatic enzyme
Thick mucus blocks pancreatic enzyme reaching GI tract. Greasy, foul-smelling stool is manifestation of pancreatic insufficiency.
Enzymes are given w/ meals with 1/2 dose with snack. The capsule can be open and sprinkled over a low pH food. Do not mix w/ milk-based food as they have a higher pH.
