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Drug Therapies

Oral Products

Kalydeco (ivacaftor)
1 tablet or packet mixed with fat-containing food every 12 hours

Orkambi (lumacaftor/ivacaftor)
2 tablets with fat-containing food every 12 hours

Inhaled Products

Pulmozyme (dornase alfa)
1 ampule once daily via nebulizer

Cayston (aztreonam)
1 reconstituted single-use vial 3 times daily via nebulizer for 28 days

Tobi/Bethkis/Kitabis (tobramycin)
1 ampule every 12 hours via nebulizer for 28 days

Oral Products

Kalydeco (ivacaftor)

Description

Kalydeco is a CF transmembrane conductance regulator (CFTR) potentiator indicated for the treatment of CF in patients age 2 years and older who have one mutation in the CFTR gene that is responsive to ivacaftor based on clinical and/or in vitro assay data. Kalydeco belongs to a class of drugs known as Ion Channel Modulators.

Dosing

Kalydeco is taken every 12 hours by mouth in 150 mg tablets or as 50 mg and 75 mg packets. Kalydeco should be taken with fat-containing food (eggs, peanut butter, whole milk).

Side Effects

Body aches, chest pain, chills, cough, troubled breathing, ear congestion, fever, headache, loss of voice, nasal congestion, sore throat, dark urine, light-colored stools, loss of appetite, nausea/vomiting, yellow eyes or skin.

Orkambi (lumacaftor/ivacaftor)

Description

Orkambi is a CFTR potentiator which combines lumacaftor and ivacaftor and is indicated for the treatment of CF in patients age 6 years and older who are homozygous for the F508del mutation in the CFTR gene. If the patient’s genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of the F508del mutation on both alleles of the CFTR gene.

Dosing

Orkambi is taken every 12 hours by mouth in 100 mg-125 mg or 200 mg-125 mg tablets. Orkambi should be taken with fat containing food (eggs, peanut butter, whole milk).

Side Effects

Labored breathing, tightness in the chest, blurred vision, dark urine, dizziness, headache, loss of appetite, nausea/vomiting, nervousness, pain in the upper stomach, pounding in the ears, yellow eyes or skin.

Inhaled Products

Pulmozyme (dornase alfa)

Description

Pulmozyme is indicated for daily administration along with standard therapies for the management of CF patients to improve pulmonary function. In CF patients with an FVC ≥ 40% of predicted, daily administration of Pulmozyme has also been shown to reduce the risk of respiratory tract infections requiring injectable antibiotics.

Dosing

Pulmozyme is inhaled as 1 ampule once daily via nebulizer. It should not be combined with other nebulizer medications. Store Pulmozyme in the refrigerator, do not leave at room temperature for longer than 24 hours.

Side Effects

Body aches, chest pain, congestion, cough, sore throat, fever, hoarseness, runny nose, trouble breathing/swallowing, voice changes.

Cayston (aztreonam)

Description

Cayston is indicated to improve respiratory symptoms in CF patients with Pseudomonas aeruginosa. Safety and effectiveness have not been established in pediatric patients below the age of 7 years, patients with FEV1 75% predicted, or patients colonized with Burkholderia cepacia. If on a bronchodilator or other inhaler medication take those prior to administering antibiotics.

Dosing

Cayston is inhaled as 1 single use vial reconstituted with 1 mL of sterile diluent 3 times daily. Cayston treatment is to be given as 28 days on, then 28 days off. Continue with treatment even if you feel better, unless instructed to stop by your doctor.

Side Effects

Chest discomfort, cough, difficulty breathing, fever.

Tobi/Bethkis/Kitabis (tobramycin)

Description

There are numerous products which contain tobramycin. Each is indicated for the treatment of CF patients 6 years of age and older who have Pseudomonas aeruginosa. None of the products are recommended for patients who are colonized with Burkholderia cepacia. Safety and efficacy of Tobi and Kitabis have not been demonstrated in patients with FEV1 <25% or >75% predicted. Bethkis is not to be used in patients with FEV1 <40% or >80% predicted.

Dosing

Tobi, Bethkis, and Kitabis should be taken as 1 ampule inhaled via nebulizer every 12 hours. Treatment is to be given as 28 days on, then 28 days off. Continue with treatment even if you feel better, unless instructed to stop by your doctor.

Side Effects

Bloody nose, difficulty breathing, discoloration of sputum, fever, runny nose, sneezing, voice changes, hearing loss, kidney problems, bronchospasms.