Medications / Drug Access

What is the proper order for taking my medications?

Although there is some recent research to suggest there is no change in effectiveness with timing inhaled mucolytics before or after chest physiotherapy , if you take multiple inhaled medications, we recommend that they can be taken in this order:

1. Bronchodilator (e.g. Ventolin®)
2. Mucolytic (e.g. Pulmozyme® or hypertonic saline)

Ideally, wait 30 minutes

3. Chest physiotherapy
4. Other inhaled medications (e.g. Pulmicort®, Oxeze®), and finally
5. Inhaled antibiotics (e.g. inhaled tobramycin, colistin, Cayston®, Quinsair®)

Antibiotics

Antibiotics are used in CF to reduce the amount of bacteria that are in your lungs. This will help to improve your lung function and reduce the damage to your lungs.

There are three ways of taking antibiotics:

Inhaled, also called nebulized.
Intravenous (IV) or in the vein.
Oral or by mouth.

See below for the various antibiotics divided by ingestion method.

Inhaled Antibiotics

View All Inhaled Antibiotics

Amikacin

Why is this medication prescribed?
Amikacin (Amikin®) belongs to a class of antibiotics called aminoglycosides. In CF, amikacin is used to treat lung infections caused by specific bacteria – for example Pseudomonas aeruginosa.

How does this medication work?
Amikacin can be given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs. The main goal is to improve or maintain lung function.

What is the usual dose?
The inhaled form of amikacin is available as a 500mg/2mL solution kept in a vial that can be stored at room temperature. The usual dose for adults with CF is 250-500mg twice a day via nebulizer.
How should this medication be taken?

Take your amikacin at approximately the same time everyday so that it is easier to remember. Also take the doses as close to 12 hours apart as possible.

To mix your inhaled dose of amikacin:

Remove the lid from the vial(s).
Using a syringe, withdraw your dose (1mL or 2mL) from the vial and add it to the nebulizer.
Using a syringe, withdraw 0.5 – 1mL (2.5mg – 5mg) of undiluted Ventolin® and add it to the nebulizer with the amikacin.

If you are taking other inhaled medication(s), please refer to the order at the top of this page.

Note: you can mix your inhaled amikacin with ventolin or atrovent but do NOT mix it with other inhaled medications (e.g. Pulmozyme®).

After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

What should I do if I miss a dose?
Do not double any dose. If you forget to take a dose, take the missed dose as soon as you remember. If it is almost time for your next dose, skip the missed dose and take the next scheduled dose.

What should I expect from this medication?
You may or may not experience side effects. These side effects may disappear; but, let your doctor know if they are severe or if they persist for more than 2 days. These can include hoarseness, chest tightness or wheezing, but tell us if you have any other side effects as well. Although these side effects are very rare, contact your doctor if you experience ringing of the ears or hearing loss.

Precautions
Remember to tell your doctor if you have ever had a previous reaction to amikacin, any aminoglycoside antibiotic or any inhaled medication in the past. If you are pregnant or think you may be pregnant discuss this with your CF doctor.

Aztreonam (Cayston®)

Why is this medication prescribed?
Inhaled aztreonam (Cayston®) belongs to a class of antibiotics called monobactams. Cayston® is a special, preservative-free formulation of aztreonam for inhalation used to treat or prevent bacterial infections.

How does this medication work?
It is given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs. The main goal is to improve or maintain lung function.

What is the usual dose?
The usual dose is 75 mg three times a day via the Altera nebulizer system.

How should this medication be taken?
Your doctor may prescribe it for one month on, one month off, or continuously. Take inhaled aztreonam (Cayston®) at approximately the same time everyday so that it is easier to remember. Each of the doses should be taken at least 4 hours apart. Cayston® is the only product that should be used with the provided Altera nebulizer system. Do not use any other other medications in the Altera nebulizer system.

To prepare your dose of inhaled aztreonam (Cayston®):

Have one amber vial of Cayston® and one ampoule of diluent ready. Separate diluent ampoules by gently pulling them apart.
Ensure all the powder in the amber vial of Cayston® settles to the bottom by gently tapping the vial. Lift the metal flap on the top of the vial, and then pull the flap across and down to remove the entire metal ring and overcap from the vial. Safetly dispose of the ring and carefully remove the rubber stopper.
Twist off the plastic tip of the diluent ampoule. Squeeze the contents of the ampoule into the amber vial.
Replace the rubber stopper onto the amber vial, and gently swirl the vial until all the powder has dissolved. The liquid should be clear. Cayston® should be used immediately as soon as it is mixed.
Once your Altera nebulizer system is ready, remove the rubber stopper on the amber vial, and pour all of the solution into the medication reservoir. Gently tap the vial to completely empty the vial of all its contents.
If you are taking other inhaled medication(s), please take them in the order listed at the top of this page.

Note:

DO NOT dilute Cayston® or mix it with any other medications.
DO NOT reconstitute more than one dose at a time.
Make sure to store Cayston® in your REFRIGERATOR.
DO NOT FREEZE. If absolutely necessary (i.e. during transportation), Cayston® can be stored at room temperature for up to 28 days away from heat, moisture and light.
DO NOT use Cayston® if it is cloudy, has particles in the solution, if it is expired, or if it has been stored outside of a refrigerator for more than 28 days.
After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

Precautions
Remember to tell your doctor if you have ever had a previous reaction to aztreonam, or any beta-lactam antibiotic or any inhaled medication in the past. As well, if you are pregnant or think you may be pregnant discuss this with your CF doctor.

Patient Assistance Program
Gilead has an assistance program called Cayston PAP. If you have further questions, please ask your social worker, pharmacist or doctor.

Colistin

Why is this medication prescribed?
Colistimethate, or colistin, belongs to a class of antibiotics called polymyxins. It is used to treat or prevent bacterial infections. It can be given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the airways. It can also be given intravenously to treat acute lung infections. The main goal is to improve or maintain lung function.

How does this medication work?
Colistin is given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs.

What is the usual dose?
Colistin is available as a powder in a vial that can be stored at room temperature. The usual dose for adults with CF is 75mg twice a day via nebulizer.

How should this medication be taken?
Take inhaled colistin at approximately the same time everyday so that it is easier to remember. Also take the doses as close to 12 hours apart as possible.

To mix your daily dose of colistin:

Remove the lid from the colistin vial.
Remove the lids from the sterile water and saline vials.
Swab the rubber top of all vials with an alcohol swab before inserting the syringe.
Draw up 2mL of sterile water for injection into a syringe (always use a new syringe with each treatment preparation).
Inject 2mL of sterile water into the colistin vial, while gently rotating and swirling the bottle (to prevent foaming) until all the white powder has dissolved. DO NOT shake vigorously.
Withdraw 1mL (75mg) of the colistin and place it into the nebulizer.
Draw up 2mL of normal saline and add into the nebulizer.
Place the remaining mixed colistin (1mL) into the refrigerator for future use.
Discard unused portion after 24 hrs.

After mixing your own colistin use it within 24 hours. We recommend that every day you label your colistin after mixing it in the morning and then discard any left over after your evening dose.

If you are taking other inhaled medication(s), please take them in this order listed at the top of this page.

Note: DO NOT mix colistin with any other medications. You may use a Bronchodilator such as Ventolin® before taking your inhaled colistin. Remember, DO NOT use any premixed, ready-to-use, or liquid forms of colistin prepared by a pharmacy.

After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

What should I expect from this medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they persist for more than 2 days. These can include hoarseness, chest tightness and wheezing but tell us if you have any other side effects as well.
To minimize these side effects, we recommend you use a bronchodilator (like Ventolin®) before taking your inhaled colistin dose.
Do not be alarmed if you see the colistin foaming in your nebulizer, this commonly happens during nebulizer treatment and cannot be prevented.

Precautions
Remember to tell your doctor if you have ever had a previous reaction to colistin or any inhaled medication in the past. As well, if you are pregnant or think you may be pregnant discuss this with your CF doctor.

TOBI®

Why is this medication prescribed?
TOBI® is a special, preservative free formulation of tobramycin for inhalation. Compared to other forms of inhaled tobramycin, TOBI® is a more concentrated solution, which allows more antibiotic to reach the lungs. Depending on your drug coverage, you may qualify to use TOBI®.

How does this medication work?
TOBI® is given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs. The main goal is to improve or maintain lung function.

What is the usual dose?
TOBI® is packaged with 4 ampoules per pouch. Each ampoule is 5mL. The usual dose for adults with CF is 300mg/5mL twice a day via nebulizer.

How should this medication be taken?
Your doctor may prescribe it for one month on, one month off, or continuously. Take TOBI® at approximately the same time everyday so that it is easier to remember. Also take the doses as close to 12 hours apart as possible.

To prepare your dose of TOBI®:

Gently separate one ampoule and store the remainder in the refrigerator.
Open the TOBI® ampoule by holding the bottom tab with one hand and twisting off the top of the ampoule with the other hand. Be careful not to squeeze the ampoule until you are ready to empty its contents into the nebulizer cup.
Squeeze all the contents of the ampoule into your nebulizer.

If you are taking other inhaled medication(s), please take them in the order at the top of this page.

Note: DO NOT dilute TOBI or mix it with any other medications. Make sure to store TOBI® in your REFRIGERATOR. DO NOT FREEZE. If absolutely necessary (i.e. during transportation), you can store TOBI® in the opened/unopened foil pouches at room temperature for up to 28 days away from heat, moisture and light. Do not store them in the bathroom. TOBI® may darken over time if it is left out of the refrigerator but this does not change its effectiveness. However, DO NOT use your TOBI® if it is cloudy, has particles in the solution, if it is expired, or if it has been stored outside of a refrigerator for more than 28 days.

After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

Precautions
Remember to tell your doctor if you have ever had a previous reaction to tobramycin, any aminoglycoside antibiotic or any inhaled medication in the past.
As well, if you are pregnant or think you may be pregnant discuss this with your CF doctor. The intravenous form of tobramycin is NOT recommended but the INHALED form can be taken.

Patient Assistance Program
Novartis has an assistance program called the TOBI® Co-Pay Program. Your social worker, pharmacist or doctor are also available if you have further questions.

TOBI Podhaler

Why is this medication prescribed?
TOBI® Podhaler is a dry powder formulation and device for inhaled tobramycin. Compared to other forms of inhaled tobramycin, TOBI® Podhaler does not require a nebulizer. Dry powder is filled into capsules which are used with the Podhaler device. Depending on your drug coverage, you may qualify to use the TOBI® Podhaler.

How does this medication work?
TOBI® Podhaler is given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs. The main goal is to improve or maintain lung function.

What is the usual dose?
TOBI® Podhaler is packaged into weekly boxes containing blister strips, an inhaler, and a storage case. Each weekly box contains 7 blister strips, and each blister strip contains eight capsules. Each capsule contains 28 mg of tobramycin. The usual dose for adults with CF is 112 mg (4 capsules) twice a day via nebulizer. Each blister strip contains the typical daily dose, and each box contains enough blister strips for a week.

How should this medication be taken?
Your doctor may prescribe it for one month on, one month off, or continuously. Take TOBI® Podhaler at approximately the same time everyday so that it is easier to remember. Also, take the doses as close to 12 hours apart as possible.

To prepare your dose of TOBI® Podhaler:

Wash and dry your hands before using the Podhaler.
Remove the Podhaler from its case by holding the base and twisting off the top of the case in a counter-clockwise direction.
Hold the body of the inhaler, unscrew and remove the mouthpiece from the inhaler body. Place the mouthpiece aside on a clean, dry surface.
Separate the morning and evening doses from the blister strip. Then peel back the foil from the blister strip to reveal one capsule, and remove it from the blister card.
Insert the capsule into the inhaler chamber. Replace the mouthpiece and screw it on firmly until it stops. Do not overtighten.
Hold the inhaler with the mouthpiece down, and press the blue button firmly with your thumb as far as it will go. Then release the button. This breaks the capsule open in the Podhaler. The powder is ready for inhalation now.
After inhalation, inspect the used capsule to make sure there is no powder left. If there is still some powder and the capsule looks punctured, place it back into the Podhaler chamber. Do not press the blue button again, and resume inhalation. However, if the capsule appears to be unpunctured, place it back into the Podhaler, repeat step 6, and resume inhalation.

If you are taking other inhaled medication(s), please take them in the order listed at the top of this page

Note: Store TOBI® Podhaler at room temperature and protect from moisture.

What should I do if I miss a dose?
Do NOT double any dose. If you forget to take a dose, take the missed dose as soon as you remember. If it is almost time for your next dose, skip the missed dose and take the next scheduled dose.

Precautions
Remember to tell your doctor if you have ever had a previous reaction to tobramycin, an aminoglycoside antibiotic or any inhaled medication in the past. As well, if you are pregnant or think you may be pregnant discuss this with your CF doctor. The intravenous form of tobramycin is NOT recommended but the INHALED form can be taken.

Patient Assistance Program
Novartis has an assistance program called the TOBI® Co-Pay Program. Your social worker, pharmacist or doctor are available if you have further questions.

Inhaled Tobramycin

Why is this medication prescribed?
Tobramycin belongs to a class of antibiotics called aminoglycosides. Tobramycin is used to treat or prevent bacterial infections.

How does this medication work?
It can be given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs. The main goal is to improve or maintain lung function.

What is the usual dose?
The inhaled form of tobramycin is available as an 80mg/2mL solution in a vial that can be stored at room temperature. The usual dose for adults is 80mg to 160mg twice a day via nebulizer.

How should this medication be taken?
Take your tobramycin at approximately the same time everyday so that it is easier to remember. Also take the doses as close to 12 hours apart as possible.

To mix your inhaled dose of tobramycin:

Remove the lid from the vial(s).
Carefully pour all the medication (2mL or 4mL) from the vial into the nebulizer.
Using a syringe, withdraw 0.5 – 1mL (2.5mg – 5mg) of undiluted Ventolin® and add it to the nebulizer with the tobramycin.

If you are taking other inhaled medication(s), please take them in the order listed at the top of this page.

Note: you can mix your inhaled tobramycin with ventolin or atrovent but do NOT mix it with other inhaled medications (e.g. Pulmozyme®).

After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

What should I expect from this medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they persist for more than 2 days. These can include hoarseness, chest tightness or wheezing, but tell us if you have any other side effects as well. Although these side effects are very rare, contact your doctor if you experience ringing of the ears or hearing loss.

Precautions
Remember to tell your doctor if you have ever had a previous reaction to tobramycin, any aminoglycoside antibiotic or any inhaled medication in the past. As well, if you are pregnant or think you may be pregnant discuss this with your CF doctor. The intravenous form of tobramycin is NOT recommended but the inhaled form can be taken.

Qunisair® CONTENT REQUIRED
Vancomycin CONTENT REQUIRED

Amikacin

How does this medication work?
Amikacin can be given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs. The main goal is to improve or maintain lung function.

How should this medication be taken?
Take your amikacin at approximately the same time everyday so that it is easier to remember. Also take the doses as close to 12 hours apart as possible.

To mix your inhaled dose of amikacin:

Remove the lid from the vial(s).
Using a syringe, withdraw your dose (1mL or 2mL) from the vial and add it to the nebulizer.
Using a syringe, withdraw 0.5 – 1mL (2.5mg – 5mg) of undiluted Ventolin® and add it to the nebulizer with the amikacin.

If you are taking other inhaled medication(s), please refer to the order at the top of this page.

Note: you can mix your inhaled amikacin with ventolin or atrovent but do NOT mix it with other inhaled medications (e.g. Pulmozyme®).

After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

Aztreonam (Cayston®)

How does this medication work?
It is given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs. The main goal is to improve or maintain lung function.

What is the usual dose?
The usual dose is 75 mg three times a day via the Altera nebulizer system.

How should this medication be taken?
Your doctor may prescribe it for one month on, one month off, or continuously. Take inhaled aztreonam (Cayston®) at approximately the same time everyday so that it is easier to remember. Each of the doses should be taken at least 4 hours apart. To prepare and take inhaled aztreonam (Cayston®) please also refer to Preparing & Taking Your Treatment in the Treatment Best Practices section here. Cayston® is the only product that should be used with the provided Altera nebulizer system. Do not use any other other medications in the Altera nebulizer system.

To prepare your dose of inhaled aztreonam (Cayston®):

Have one amber vial of Cayston® and one ampoule of diluent ready. Separate diluent ampoules by gently pulling them apart.
Ensure all the powder in the amber vial of Cayston® settles to the bottom by gently tapping the vial. Lift the metal flap on the top of the vial, and then pull the flap across and down to remove the entire metal ring and overcap from the vial. Safetly dispose of the ring and carefully remove the rubber stopper.
Twist off the plastic tip of the diluent ampoule. Squeeze the contents of the ampoule into the amber vial.
Replace the rubber stopper onto the amber vial, and gently swirl the vial until all the powder has dissolved. The liquid should be clear. Cayston® should be used immediately as soon as it is mixed.
Once your Altera nebulizer system is ready, remove the rubber stopper on the amber vial, and pour all of the solution into the medication reservoir. Gently tap the vial to completely empty the vial of all its contents.
If you are taking other inhaled medication(s), please take them in the order listed at the top of this page.

Note:

DO NOT dilute Cayston® or mix it with any other medications.
DO NOT reconstitute more than one dose at a time.
Make sure to store Cayston® in your REFRIGERATOR.
DO NOT FREEZE. If absolutely necessary (i.e. during transportation), Cayston® can be stored at room temperature for up to 28 days away from heat, moisture and light.
DO NOT use Cayston® if it is cloudy, has particles in the solution, if it is expired, or if it has been stored outside of a refrigerator for more than 28 days.
After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

Patient Assistance Program
Gilead has an assistance program called Cayston PAP. For more information, click here. If you have further questions, please ask your social worker, pharmacist or doctor.

Colistin

How does this medication work?
Colistin is given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs.

What is the usual dose?
Colistin is available as a powder in a vial that can be stored at room temperature. The usual dose for adults with CF is 75mg twice a day via nebulizer.

How should this medication be taken?
Take inhaled colistin at approximately the same time everyday so that it is easier to remember. Also take the doses as close to 12 hours apart as possible.

To mix your daily dose of colistin:

Remove the lid from the colistin vial.
Remove the lids from the sterile water and saline vials.
Swab the rubber top of all vials with an alcohol swab before inserting the syringe.
Draw up 2mL of sterile water for injection into a syringe (always use a new syringe with each treatment preparation).
Inject 2mL of sterile water into the colistin vial, while gently rotating and swirling the bottle (to prevent foaming) until all the white powder has dissolved. DO NOT shake vigorously.
Withdraw 1mL (75mg) of the colistin and place it into the nebulizer.
Draw up 2mL of normal saline and add into the nebulizer.
Place the remaining mixed colistin (1mL) into the refrigerator for future use.
Discard unused portion after 24 hrs.

After mixing your own colistin use it within 24 hours. We recommend that every day you label your colistin after mixing it in the morning and then discard any left over after your evening dose.

If you are taking other inhaled medication(s), please take them in this order listed at the top of this page.

After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

TOBI®

How does this medication work?
TOBI® is given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs. The main goal is to improve or maintain lung function.

What is the usual dose?
TOBI® is packaged with 4 ampoules per pouch. Each ampoule is 5mL. The usual dose for adults with CF is 300mg/5mL twice a day via nebulizer.

To prepare your dose of TOBI®:

Gently separate one ampoule and store the remainder in the refrigerator.
Open the TOBI® ampoule by holding the bottom tab with one hand and twisting off the top of the ampoule with the other hand. Be careful not to squeeze the ampoule until you are ready to empty its contents into the nebulizer cup.
Squeeze all the contents of the ampoule into your nebulizer.

If you are taking other inhaled medication(s), please take them in the order at the top of this page.

After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

Patient Assistance Program
Novartis has an assistance program called the TOBI® Co-Pay Program. To learn more about this program, click here. Your social worker, pharmacist or doctor are also available if you have further questions.

TOBI Podhaler

To prepare your dose of TOBI® Podhaler:

Wash and dry your hands before using the Podhaler.
Remove the Podhaler from its case by holding the base and twisting off the top of the case in a counter-clockwise direction.
Hold the body of the inhaler, unscrew and remove the mouthpiece from the inhaler body. Place the mouthpiece aside on a clean, dry surface.
Separate the morning and evening doses from the blister strip. Then peel back the foil from the blister strip to reveal one capsule, and remove it from the blister card.
Insert the capsule into the inhaler chamber. Replace the mouthpiece and screw it on firmly until it stops. Do not overtighten.
Hold the inhaler with the mouthpiece down, and press the blue button firmly with your thumb as far as it will go. Then release the button. This breaks the capsule open in the Podhaler. The powder is ready for inhalation now.
After inhalation, inspect the used capsule to make sure there is no powder left. If there is still some powder and the capsule looks punctured, place it back into the Podhaler chamber. Do not press the blue button again, and resume inhalation. However, if the capsule appears to be unpunctured, place it back into the Podhaler, repeat step 6, and resume inhalation.

If you are taking other inhaled medication(s), please take them in the order listed at the top of this page

Note: Store TOBI® Podhaler at room temperature and protect from moisture.

Patient Assistance Program
Novartis has an assistance program called the TOBI® Co-Pay Program. For more information and to see if you are eligible, click here. Your social worker, pharmacist or doctor are also available if you have further questions.

Inhaled Tobramycin

Why is this medication prescribed?
Tobramycin belongs to a class of antibiotics called aminoglycosides. Tobramycin is used to treat or prevent bacterial infections.

How does this medication work?
It can be given by inhalation to decrease the amount of bacteria (Pseudomonas aeruginosa) in the lungs. The main goal is to improve or maintain lung function.

How should this medication be taken?
Take your tobramycin at approximately the same time everyday so that it is easier to remember. Also take the doses as close to 12 hours apart as possible.

To mix your inhaled dose of tobramycin:

Remove the lid from the vial(s).
Carefully pour all the medication (2mL or 4mL) from the vial into the nebulizer.
Using a syringe, withdraw 0.5 – 1mL (2.5mg – 5mg) of undiluted Ventolin® and add it to the nebulizer with the tobramycin.

If you are taking other inhaled medication(s), please take them in the order listed at the top of this page.

Note: you can mix your inhaled tobramycin with ventolin or atrovent but do NOT mix it with other inhaled medications (e.g. Pulmozyme®).

After use, rinse your nebulizer well with warm water to prevent build up that can clog your nebulizer.

What should I expect from this medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they persist for more than 2 days. These can include hoarseness, chest tightness or wheezing, but tell us if you have any other side effects as well. Although these side effects are very rare, contact your doctor if you experience ringing of the ears or hearing loss.

Precautions
Remember to tell your doctor if you have ever had a previous reaction to tobramycin, any aminoglycoside antibiotic or any inhaled medication in the past. As well, if you are pregnant or think you may be pregnant discuss this with your CF doctor. The intravenous form of tobramycin is NOT recommended but the inhaled form can be taken.

Quinsair®

Why is this medication prescribed?

Quinsair is an inhaled antibiotic that is usually prescribed for patients with CF that have Pseudomonas aeruginosa in their lungs. Inhaled antibiotics like Quinsair are prescribed to improve or maintain lung function and reduce exacerbations.

How does this medication work?

The active ingredient in Quinsair is an antibiotic called levofloxacin. Levofloxacin is from the fluoroquinolone family of antibiotics that are highly effective against certain types of bacteria like Pseudomonas. When levofloxacin is inhaled, it works to kill the bacteria in the lungs which helps improve lung function and reduce the risk of having a pulmonary exacerbation.

What is the usual dose?

The usual dose is 240mg administered twice daily.

How should this medication be taken?

Quinsair is administered by nebulization using the eFlow nebulizer system and Zirela nebulizer handset.

To prepare your dose of inhaled Quinsair:

Empty the entire contents of one ampule of Quinsair into the Zirela nebulizer medication reservoir. Follow the directions for your eFlow nebulizer system and inhale the entire dose of medication; this should take about 5 minutes.

What should I do if I miss a dose?

Take a missed dose as soon as you think about it. If it is less than 8 hours until your next dose, skip the missed dose and go back to your normal time. Do not take two doses at the same time or extra doses.

What should I expect from this medication? (i.e. side effects)

You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they persist for more than 2 days. These can include increased cough or sputum production but tell us if you have any other side effects as well. Some patients may experience an unpleasant taste with Quinsair; eating chocolate or a candy prior to your dose may help.

Precautions

Remember to tell your doctor if you have ever had a previous reaction to levofloxacin, or any fluoroquinolone antibiotic or any inhaled medication in the past. As well, if you are pregnant or think you may be pregnant discuss this with your CF doctor.

Patient Assistance Program

There is a patient assistance program provided by the manufacturer to assist with drug reimbursement. Contact your pharmacist, social worker or doctor to discuss.

Vancomycin

Why is this medication prescribed?

Inhaled vancomycin is usually prescribed for CF patients that grow Staphylococcus aureus in their lungs. Inhaled vancomycin can help improve or maintain lung function and reduce the number of pulmonary exacerbations.

How does this medication work?

Vancomycin is an antibiotic in the glycopeptide family. It is used to treat infections caused by certain types of bacteria like Staphylococcus aureus and methicillin-resistant Staphylococcus aureus (MRSA) in particular. When vancomycin is inhaled, it works to kill the bacteria in the lungs which helps improve lung function and reduce the risk of having a pulmonary exacerbation.

What is the usual dose?

The usual dose for adults is 250 mg or 500 mg twice daily.

How should this medication be taken?

Inhaled vancomycin should be used with a nebulizer. Check with your respiratory therapist to confirm that your nebulizer and handset is compatible with vancomycin. Take inhaled vancomycin at approximately the same time each day with doses about 12 hours apart. Vancomycin should not be mixed with other drugs in the nebulizer.

To mix your inhaled dose:

Remove the lid from the vancomycin vial.
Remove the lids from the sterile water and normal saline ampoules/vials..
Swab the rubber top of all vials with an alcohol swab.
Draw up 5 mL of sterile water using a needle and syringe.
Carefully insert the needle with syringe into the middle of the rubber top of the vancomycin vial, and inject all the sterile water (5mL) into the vial.
Gently roll to dissolve all the powder.
Using a needle and syringe, withdraw 2.5 mL (250 mg) or 5ml (500mg) of vancomycin and add it to the nebulizer
Draw up 2 mL of normal saline (0.9%) and add it to the nebulizer.
Mixed vancomycin vials can be kept for 24 hours at room temperature or for 96 hours (4 days) in the refrigerator. Discard any unused portions of mixed vials after these durations.

What should I do if I miss a dose?

Take a missed dose as soon as you think about it. If it is close to the time for your next dose, skip the missed dose and go back to your regular time. Do not take two doses at the same time or extra doses to make up for the missed dose.

What should I expect from this medication? (i.e. side effects)

All drugs may cause side effects. However, many people have no side effects or only have minor side effects. Sometimes inhaled vancomycin can cause hoarseness of voice, chest tightness or wheeze. Call your doctor or get medical help if any of these side effects or any other side effects bother you or do not go away. These are not all of the side effects that may occur. If you have questions about side effects, call your doctor to discuss.

Precautions

If you are pregnant or think you might be pregnant, discuss this with your doctor before starting any antibiotics. Intravenous or inhaled vancomycin can be taken during pregnancy if the benefits outweigh the potential risks.
Let your doctor know if you have ever had a reaction to vancomycin or any inhaled medication.
If you have never tried inhaled vancomycin before, lung function tests will be done before and after your first dose to make sure you don’t have a reaction.
The vials are labelled for intravenous use but they can be given by inhalation.
Ensure your doctor and pharmacist is aware of all the other prescription and over-the-counter medications, herbals, or supplements you are taking to help prevent any drug conflicts.

To prepare and take your inhaled antibiotics, please also refer to “Preparing & taking your treatment” in the Treatment Best Practices section.

Oral Antibiotics

Oral antibiotics may be prescribed for home use if an individual is experiencing a mild exacerbation, or as part of antibiotic combination therapy for more severe exacerbations. Oral antibiotics may also be used as part of chronic therapy. The type of antibiotic as well as the dose will vary depending on the type of bacteria in your lungs, but your doctor will determine which antibiotic combination is best for you.

View All Oral Antibiotics

Azithromycin

Why Is This Medication Prescribed?
Azithromycin (Zithromax®) is an antibiotic that belongs to the macrolide class of medications. In CF, azithromycin is used in chronic therapy as an anti-inflammatory drug and a mucolytic.

How Does This Medication Work?
Azithromycin decreases the inflammation in your lungs and helps you thin and clear your mucous.

What Is The Usual Dose?
Azithromycin is available in a tablet form. A typical adult dose is 250-500 mg by mouth once daily.

How Should This Medication Be Taken?
You may take azithromycin with or without food. You may take this drug with food if it upsets your stomach. Store the tablet at room temperature away from heat, moisture and direct light. Continue taking azithromycin as it is part of chronic treatment. Do not stop unless your doctor tells you to.

What Should I Do If I Miss A Dose?
Do not double any dose. If you forget to take a dose, take the missed dose as soon as you remember. If it is almost time for your next dose, skip the missed dose and take the next scheduled dose.

Precautions
Tell your doctor of any drug allergies and previous allergic reactions with drugs before using azithromycin. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. Avoid taking antacids that contain magnesium or aluminum while you are taking azithromycin as they may reduce the absorption of azithromycin.

Ciprofloxacin

Why Is This Medication Prescribed?
Ciprofloxacin (Cipro®) is an antibiotic that belongs to the fluroquinolone class of medications. In CF, ciprofloxacin is used to treat lung infections caused by specific bacteria – for example Pseudomonas aeruginosa.

How Does This Medication Work?
Ciprofloxacin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Ciprofloxacin is available in a tablet form. The usual dose for an adult with CF is 750 mg twice daily by mouth.

How Should This Medication Be Taken?
You may take ciprofloxacin with or without food. You may take this drug with food if it upsets your stomach. Store the tablet at room temperature away from heat, moisture and direct light. Finish taking all of your medication even if you start to feel better after taking a few doses. Do not stop unless your doctor tells you to.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include nausea, diarrhea, vomiting, abdominal pain, or headache, but tell us if you have any other side effects as well. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, numbness, tingling or a yellow tinge in your skin or eyes. Ciprofloxacin can cause your skin to become more sensitive to sunlight. Limit the amount of time you spend in the sun until you know how this drug affects you. When you are in the sun, keep your body covered with clothing and use sunblock (preparations with zinc oxide) on exposed skin.

Precautions
Tell your doctor of any drug allergies and previous allergic reactions with drugs before using ciprofloxacin. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. Ciprofloxacin can interact with certain medications if they are taken at the same time. Examples are antacids, calcium, iron or zinc containing products. Avoid taking ciprofloxacin at the same time as these products. Take ciprofloxacin 2 hours before or 6 hours after these products.

Cloxacillin

Why Is This Medication Prescribed?
Cloxacillin (Apo-cloxi®) is an antibiotic that belongs to the penicillin class of medications. In CF, cloxacillin is used to treat lung infections caused by specific bacteria – for example Staphylococcus aureus.

How Does This Medication Work?
Cloxacillin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Cloxacillin is available in a tablet form. The usual dose for an adult with CF is 500 mg every 6 hours or 1 gram twice daily by mouth.

How Should This Medication Be Taken?
Take this medication on an empty stomach, at least one hour before or two hours after a meal. Finish taking all of your medication even if you start to feel better after taking a few doses. Do not stop unless your doctor tells you to.

Precautions
Tell your doctor of any drug allergies and previous allergic reactions with drugs before using cloxacillin. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics.

Doxycycline

Why Is This Medication Prescribed?
Doxycycline (Vibramycin®) is an oral antibiotic that belongs to the tetracycline class of medications. In CF, doxycycline is used to treat lung infections caused by specific bacteria – for example, Burkholderia cepacia.

How Does This Medication Work?
Doxycycline works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Doxycycline is available as a capsule or tablet. A typical adult dose is 100mg twice a day by mouth.

How Should This Medication Be Taken?
To prevent the medication from being stuck in the esophagus, drink a small amount of water before and a full glass of water after each dose. Take doxycycline while sitting or standing up and do not lie down immediately afterwards. You can take this medicine with food if it upsets your stomach. Finish taking all of your medication even if you start to feel better after taking a few doses. Do not stop unless your doctor tells you to. Doxycycline should be stored at room temperature away from heat, moisture and direct light.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These can include nausea, diarrhea, loss of appetite, or sore mouth or gums, but tell us if you have any other side effects as well. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, trouble swallowing, or unusual bleeding or bruising. Doxycycline can cause your skin to become more sensitive to sunlight. Limit the amount of time you spend in the sun until you know how this drug affects you. When you are in the sun, keep your body covered with clothing and use sunblock (preparations with zinc oxide) on exposed skin.

Precautions
Tell your doctor of any drug allergies and previous allergic reactions with drugs before using doxycycline. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. Doxycycline can interact with certain medications if they are taken at the same time.

If you are taking:

Antacids – take doxycycline 1 hour before or two hours after
Iron – take doxycycline 2 hours before or 3 hours after
Calcium supplements (including calcium found in milk and dairy products) – take doxycycline 1 hour before or 2 hours after
Laxatives containing magnesium – take doxycycline 1 hour before or 2 hours after

Keflex

Content coming soon

Levofloxacin

Why Is This Medication Prescribed?
Levofloxacin (Levaquin®) is an antibiotic that belongs to the fluroquinolone class of medications. In CF, ciprofloxacin is used to treat lung infections caused by specific bacteria – for example Burkholderia cepacia.

How Does This Medication Work?
Levofloxacin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Levofloxacin is available in a tablet form. The usual dose for an adult with CF is 750 mg once daily by mouth.

How Should This Medication Be Taken?
You may take levofloxacin with or without food. You may take this drug with non-dairy food if it upsets your stomach. Store the tablet at room temperature away from heat, moisture and direct light. Finish taking all of your medication even if you start to feel better after taking a few doses. Do not stop unless your doctor tells you to.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include nausea, diarrhea, vomiting, abdominal pain, or headache, but tell us if you have any other side effects as well. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, numbness, reduced urine output, tingling, or a yellow tinge in your skin or eyes. Levofloxacin can cause your skin to become more sensitive to sunlight. Limit the amount of time you spend in the sun until you know how this drug affects you. When you are in the sun, keep your body covered with clothing and use sunblock (preparations with zinc oxide) on exposed skin.

Precautions
Tell your doctor of any drug allergies and previous allergic reactions with drugs before using levofloxacin. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. Levofloxacin can interact with certain medications if they are taken at the same time. Examples are antacids, calcium, iron or zinc containing products. Avoid taking levofloxacin at the same time as these prodcuts. Take levofloxacin 2 hours before or 2 hours after these products.

Minocycline

Why Is This Medication Prescribed?
Minocycline (Minocin®) is an oral antibiotic that belongs to the tetracycline class of medications. In CF, minocycline is used to treat lung infections caused by specific bacteria – for example Burkholderia cepacia.

How Does This Medication Work?
Minocycline works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Minocycline is available as a capsule or tablet. A typical adult dose is 100mg twice a day by mouth.

How Should This Medication Be Taken?
To prevent the medication from being stuck in the esophagus, drink a small amount of water before and a full glass of water after each dose. Take minocycline while sitting or standing up and do not lie down immediately afterwards. You can take this medicine with food if it upsets your stomach. Finish taking all of your medication even if you start to feel better after taking a few doses. Do not stop unless your doctor tells you to. Minocycline should be stored at room temperature away from heat, moisture and direct light.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include nausea, diarrhea, loss of appetite, or sore mouth or gums, but tell us if you have any other side effects as well. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, trouble swallowing, or unusual bleeding or bruising. Minocycline can cause your skin to become more sensitive to sunlight. Limit the amount of time you spend in the sun until you know how this drug affects you. When you are in the sun, keep your body covered with clothing and use sunblock (preparations with zinc oxide) on exposed skin.

Precautions
Tell your doctor of any drug allergies and previous allergic reactions with drugs before using minocycline. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. Minocycline can interact with certain medications if they are taken together at the same time. If you are taking:

Antacids – take minocycline 1 hour before or two hours after
Iron – take minocycline 2 hours before or 3 hours after
Calcium supplements (including calcium found in milk and dairy products) – take minocycline 1 hour before or 2 hours after
Laxatives containing magnesium – take minocycline 1 hour before or 2 hours after

Septra®

Why Is This Medication Prescribed?
Septra® (sulfamethoxazole/trimethoprim) is a combination of 2 antibiotics, sulfamethoxazole, a ‘sulfa’ antibiotic, and trimethoprim. In CF, Septra® is used to treat lung infections caused by specific bacteria – for example Stenotrophomonas maltophilia or Burkholderia cepacia.

How Does This Medication Work?
Septra® works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Septra® is available in tablet, liquid or injectable form. A typical CF adult dose is 2 Double Strength (DS) tablets (800mg/160mg) by mouth twice a day.

How Should This Medication Be Taken?
This medication can be taken with or without food. If you experience stomach upset you can take it with food. It is important to drink at least 1.5L (8 glasses) of fluid a day during treatment. Finish taking all of your medication even if you start to feel better after taking a few doses. Do not stop unless your doctor tells you to stop. Store this medicine at room temperature away from heat, moisture and direct light.

Precautions
Tell your doctor of any drug allergies and previous allergic reactions with drugs before using Septra®. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. Septra® can cause your skin to become more sensitive to sunlight. Limit the amount of time you spend in the sun until you know how this drug affects you. While in the sun, keep your body covered with clothing and use sunscreen on exposed skin.

Azithromycin

How Does This Medication Work?
Azithromycin decreases the inflammation in your lungs and helps you thin and clear your mucous.

What Is The Usual Dose?
Azithromycin is available in a tablet form. A typical adult dose is 250-500 mg by mouth once daily.

Ciprofloxacin

How Does This Medication Work?
Ciprofloxacin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Ciprofloxacin is available in a tablet form. The usual dose for an adult with CF is 750 mg twice daily by mouth.

Cloxacillin

How Does This Medication Work?
Cloxacillin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Cloxacillin is available in a tablet form. The usual dose for an adult with CF is 500 mg every 6 hours or 1 gram twice daily by mouth.

Doxycycline

How Does This Medication Work?
Doxycycline works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Doxycycline is available as a capsule or tablet. A typical adult dose is 100mg twice a day by mouth.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These can include nausea, diarrhea, loss of appetite, or sore mouth or gums, but tell us if you have any other side effects as well. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, trouble swallowing, or unusual bleeding or bruising. Doxycycline can cause your skin to become more sensitive to sunlight. Limit the amount of time you spend in the sun until you know how this drug affects you. When you are in the sun, keep your body covered with clothing and use sunblock (preparations with zinc oxide) on exposed skin.

If you are taking:

Antacids – take doxycycline 1 hour before or two hours after
Iron – take doxycycline 2 hours before or 3 hours after
Calcium supplements (including calcium found in milk and dairy products) – take doxycycline 1 hour before or 2 hours after
Laxatives containing magnesium – take doxycycline 1 hour before or 2 hours after

Keflex

Why is this medication prescribed?

Cephalexin is usually prescribed to treat pulmonary exacerbations in patients that grow Staphylococcus aureus in their lungs. Cephalexin can also be prescribed for a variety of other infections such as skin infections, pharyngitis and urinary tract infections. In addition, cephalexin can also treat other types of bacteria including H. influenzae and S. pneumoniae.

How does this medication work?

Cephalexin is an antibiotic in the beta-lactam family. It is used to treat infections caused by certain types of bacteria like Staphylococcus aureus. When used to treat CF pulmonary exacerbations, cephalexin works by helping to kill the bacteria infecting the lungs.

What is the usual dose?

When used in CF to treat pulmonary exacerbations the usual dose is 1 gram four times daily.

How should this medication be taken?

Cephalexin is a tablet that is taken by mouth with or without food. If the medication is causing an upset stomach, it can be taken with food. Try to space out the doses of cephalexin evenly throughout the day.

What should I do if I miss a dose?

Take a missed dose as soon as you remember. If it is close to the time for your next dose, skip the missed dose and go back to your normal time. Do not take 2 doses at the same time or extra doses.

What should I expect from this medication? (i.e. side effects)

You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include nausea, vomiting, or diarrhea, but tell us if you have any other side effects as well. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, or severe diarrhea.

Precautions

Tell your doctor of any drug allergies and previous allergic reactions with drugs before using cephalexin. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics.

Levofloxacin

How Does This Medication Work?
Levofloxacin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Levofloxacin is available in a tablet form. The usual dose for an adult with CF is 750 mg once daily by mouth.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include nausea, diarrhea, vomiting, abdominal pain, or headache, but tell us if you have any other side effects as well. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, numbness, reduced urine output, tingling, or a yellow tinge in your skin or eyes. Levofloxacin can cause your skin to become more sensitive to sunlight. Limit the amount of time you spend in the sun until you know how this drug affects you. When you are in the sun, keep your body covered with clothing and use sunblock (preparations with zinc oxide) on exposed skin.

Minocycline

How Does This Medication Work?
Minocycline works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Minocycline is available as a capsule or tablet. A typical adult dose is 100mg twice a day by mouth.

How Should This Medication Be Taken?
To prevent the medication from being stuck in the esophagus, drink a small amount of water before and a full glass of water after each dose. Take minocycline while sitting or standing up and do not lie down immediately afterwards. You can take this medicine with food if it upsets your stomach. Finish taking all of your medication even if you start to feel better after taking a few doses. Do not stop unless your doctor tells you to. Minocycline should be stored at room temperature away from heat, moisture and direct light.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include nausea, diarrhea, loss of appetite, or sore mouth or gums, but tell us if you have any other side effects as well. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, trouble swallowing, or unusual bleeding or bruising. Minocycline can cause your skin to become more sensitive to sunlight. Limit the amount of time you spend in the sun until you know how this drug affects you. When you are in the sun, keep your body covered with clothing and use sunblock (preparations with zinc oxide) on exposed skin.

Antacids – take minocycline 1 hour before or two hours after
Iron – take minocycline 2 hours before or 3 hours after
Calcium supplements (including calcium found in milk and dairy products) – take minocycline 1 hour before or 2 hours after
Laxatives containing magnesium – take minocycline 1 hour before or 2 hours after

Septra®

How Does This Medication Work?
Septra® works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
Septra® is available in tablet, liquid or injectable form. A typical CF adult dose is 2 Double Strength (DS) tablets (800mg/160mg) by mouth twice a day.

IV Antibiotics

Intravenous (or IV) antibiotics are given in hospital during an exacerbation, but may also be prescribed for home use. IV antibiotics are mixed into fluid and go directly into your vein. High doses of IV antibiotics are used to help kill bacteria and reduce the number in your lungs. The type of antibiotic as well as the dose will vary depending on the type of bacteria in your lungs. Your doctor will determine which antibiotic or antibiotic combination is best for you.

View All IV Antibiotics

Amikacin

Why Is This Medication Prescribed?
Amikacin (Amikin®) is an antibiotic that belongs to a class of medications called aminoglycosides. In CF, amikacin is used to treat lung infections caused by specific bacteria – for example Pseudomonas aeruginosa.

How Does This Medication Work?
Amikacin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual dose for an adult is 300-500mg twice a day by IV infusion.

How Should This Medication Be Taken?
Amikacin is a medication that needs careful checking. If there is too little in your body, the drug will not fight the infection; if there is too much, it can harm your kidneys and your hearing. Your doctor will order blood to be drawn at the start of your treatment to make sure your amikacin levels are safe. The treatment usually lasts 10-14 days, however, your doctor may decide that you need a longer treatment. It is possible that your treatment could be finished at home. If you finish therapy at home, amikacin should be given by someone trained to administer IV medications. Sometimes you or a family member can be taught to administer IV antibiotics. If the IV bags are to be kept in the fridge, take the IV bag for the next dose out of the fridge one hour before the dose is due and let it warm to room temperature. You must complete your treatment of antibiotics. Do not stop unless your doctor tells you to.

Precautions
If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. Your doctor or your team will measure your amikacin levels more frequently if you are dehydrated or are receiving long term treatment.

Aztreonam

Why Is This Medication Prescribed?
Aztreonam (Azactam®) is an intravenous (IV) antibiotic. It belongs to a class of antibiotics called monobactams. In CF, aztreonam is used to treat lung infections caused by specific bacteria – for example Pseudomonas aeruginosa.

How Does This Medication Work?
Aztreonam works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual dose for adults with CF is 2 grams every 8 hours by IV infusion.

How Should This Medication Be Taken?
It is important that you receive aztreonam on a regular schedule because the drug works best when there is a constant amount in the blood. The treatment usually lasts 10 to 14 days, however, your doctor may decide that you require a longer treatment. It is possible that your treatment could be finished at home. If you finish therapy at home, aztreonam should be given by someone trained to administer IV medications. Sometimes you or a family member can be taught to administer IV antibiotics. If the IV bags are to be kept in the fridge, take the IV bag for the next dose out of the fridge 1 hour before the dose is due and let it warm to room temperature. You must complete your treatment of antibiotics. Do not stop unless your doctor tells you to.

Precautions
Tell your doctor of any drug allergies and previous allergic reactions with drugs before using aztreonam. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics.

Ceftazidime

Why Is This Medication Prescribed?
Ceftazidime (Fortaz®) is an intravenous (IV) antibiotic. It belongs to a class of drugs called cephalosporins which are related to the penicillin antibiotics. In CF, ceftazidime is used to treat lung infections caused by specific bacteria – for example, Pseudomonas aeruginosa.

How Does This Medication Work?
Ceftazidime works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual dose for adults is 2 to 3 grams every 6 or 8 hours by IV infusion.

How Should This Medication Be Taken?
It is important that you receive ceftazidime on a regular schedule because the drug works best when there is a constant amount in the blood. The treatment usually lasts 10 to 14 days, however, your doctor may decide that you require a longer treatment. It is possible that your treatment could be finished at home. If you finish therapy at home, ceftazidime should be given by someone trained to administer IV medications. Sometimes you or a family member can be taught to administer IV antibiotics. If the IV bags are to be kept in the fridge, take the IV bag for the next dose out of the fridge one hour before the dose is due and let it warm to room temperature. You must complete your treatment of antibiotics. Do not stop unless your doctor tells you to.

Precautions
If you are allergic to penicillin or other antibiotics, contact your doctor. If you have a history of gastrointestinal diseases, contact your doctor before taking ceftazidime. If you had seizures in the past, inform your doctor. If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics.

Chloramphenicol

Why Is This Medication Prescribed?
Chloramphenicol (Chloromycetin®) is a broad-spectrum intravenous (IV) antibiotic. In CF, chloramphenicol is used to treat lung infections caused by specific bacteria – for example Pseudomonas aeruginosa.

How Does This Medication Work?
Chloramphenicol works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual adult dose in CF is 500mg every 6 hours by IV infusion.

How Should This Medication Be Taken?
It is important that you receive chloramphenicol on a regular schedule because the drug works best when there is a constant amount in the blood. The treatment usually lasts 10 to 14 days, however, your doctor may decide that you require a longer treatment. It is possible that your treatment could be finished at home. If you finish therapy at home, chloramphenicol should be given by someone trained to administer IV medications. Sometimes you or a family member can be taught to administer IV antibiotics. If the IV bags are to be kept in the fridge, take the IV bag for the next dose out of the fridge one hour before the dose is due and let it warm to room temperature. You must complete your treatment of antibiotics. Do not stop unless your doctor tells you to.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include nausea, diarrhea, headache, blurred vision, and pain at the site of the injection, but tell us if you have any other side effects as well. There is a rare but possibly serious side effect from choloramphenicol called aplastic anemia. Blood tests will have to be done several times a week to monitor for this side effect. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, or swelling of the face, throat or lips.

Precautions
If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics.

Colistin

Why Is This Medication Prescribed?
Colistin (Coly-Mycin®) is an intravenous antibiotic that belongs to a class of medications called polymyxins. In CF, colistin is used to treat lung infections caused by specific bacteria – for example Pseudomonas aeruginosa.

How Does This Medication Work?
Colistin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual colistin dose for adults with CF is 75mg twice a day by IV.

How Should This Medication Be Taken?
It is important that you receive colistin on a regular schedule as the drug works best when there is a constant amount of drug in the body. The treatment usually lasts 10-14 days, however, your doctor may decide that you need a longer treatment. It is possible that your treatment could be finished at home. If you finish therapy at home, colistin should be given by someone trained to administer IV medications. Sometimes you or a family member can be taught to administer IV antibiotics. If the IV bags are to be kept in the fridge, take the IV bag for the next dose out of the fridge one hour before the dose is due and let it warm to room temperature. You must complete your treatment of antibiotics. Do not stop unless your doctor tells you to.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include mild nausea, diarrhea, and pain at the site of the injection, but tell us if you have any other side effects as well. Watch for and report any generalized pain or numbness, tingling of hands and feet, itchiness, vertigo, dizziness or slurred speech. If you notice decreased urine output please notify your doctor. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, or swelling of the face, throat or lips.

Precautions
If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. If you are dehydrated or receive a long treatment or high dosage of colistin you may have an increased risk of toxicity.

Meropenem

Why Is This Medication Prescribed?
Meropenem (Merrem®) is a broad-spectrum intravenous (IV) antibiotic that belongs to a class of antibiotics called carbapenems. In CF, meropenem is used to treat lung infections caused by specific bacteria – for example Pseudomonas aeruginosa.

How Does This Medication Work?
Meropenem works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual meropenem dose for adults with CF is 2 grams every eight hours by IV infusion.

How Should This Medication Be Taken?
It is important that you receive meropenem on a regular schedule because the drug works best when there is a constant amount in the blood. The treatment usually lasts 10 to 14 days, however, your doctor may decide that you require a longer treatment. It is possible that your treatment could be finished at home. If you finish therapy at home, meropenem should be given by someone trained to administer IV medications. Sometimes you or a family member can be taught to administer IV antibiotics. If the IV bags are to be kept in the fridge, take the IV bag for the next dose out of the fridge one hour before the dose is due and let it warm to room temperature. You must complete your treatment of antibiotics. Do not stop unless your doctor tells you to.

Precautions
If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. Meropenem has been shown to interact with probenecid and valproic acid. It is important that your doctor or pharmacist knows what other medications you are taking.

Tazocin®

Why Is This Medication Prescribed?
Tazocin® (Piperacillin/Tazobactam) is a broad-spectrum intravenous (IV) antibiotic that is a combination of piperacillin and tazobactam. Piperacillin belongs to a class of antibiotics called penicillins. In CF, Tazocin® is used to treat lung infections caused by specific bacteria – for example Pseudomonas aeruginosa.

How Does This Medication Work?
Tazocin® works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual dose for adults with CF is 4.5 grams every 6 hours by IV infusion.

How Should This Medication Be Taken?
It is important that you receive Tazocin® on a regular schedule because the drug works best when there is a constant amount in the blood. The treatment usually lasts 10 to 14 days, however, your doctor may decide that you require a longer treatment. It is possible that your treatment could be finished at home. If you finish therapy at home, Tazocin® should be given by someone trained to administer IV medications. Sometimes you or a family member can be taught to administer IV antibiotics. If the IV bags are to be kept in the fridge, take the IV bag for the next dose out of the fridge one hour before the dose is due and let it warm to room temperature. You must complete your treatment of antibiotics. Do not stop unless your doctor tells you to.

Precautions
If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics.

Tigecycline

Content coming soon

Tobramycin

Why Is This Medication Prescribed?
Tobramycin is an intravenous antibiotic that belongs to a class of medications called aminoglycosides. In CF, tobramycin is used to treat lung infections caused by specific bacteria – for example Pseudomonas aeruginosa.

How Does This Medication Work?
Tobramycin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The amount of medication given depends on your weight and will be adjusted based on your blood levels. How often the medication is given will be determined by your doctor.

How Should This Medication Be Taken?
Tobramycin is a medication that needs careful checking. If there is too little in your body, the drug will not fight the infection; if there is too much, it can harm the kidneys and your hearing. Your doctor will order blood to be drawn at the start of your treatment to make sure your tobramycin levels are safe. The treatment usually lasts 10-14 days, however, your doctor may decide that you need a longer treatment. It is possible that your treatment could be finished at home. If you finish therapy at home, tobramycin should be given by someone trained to administer IV medications. Sometimes you or a family member can be taught to administer IV antibiotics. If the IV bags are to be kept in the fridge, take the IV bag for the next dose out of the fridge one hour before the dose is due and let it warm to room temperature. You must complete your treatment of antibiotics. Do not stop unless your doctor tells you to.

Precautions
If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics. If you are dehydrated or receiving a long treatment you may have an increased risk of toxicity.

Vancomycin

Content coming soon

Amikacin

How Does This Medication Work?
Amikacin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual dose for an adult is 300-500mg twice a day by IV infusion.

Aztreonam

How Does This Medication Work?
Aztreonam works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual dose for adults with CF is 2 grams every 8 hours by IV infusion.

Ceftazidime

How Does This Medication Work?
Ceftazidime works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual dose for adults is 2 to 3 grams every 6 or 8 hours by IV infusion.

Chloramphenicol

How Does This Medication Work?
Chloramphenicol works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual adult dose in CF is 500mg every 6 hours by IV infusion.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include nausea, diarrhea, headache, blurred vision, and pain at the site of the injection, but tell us if you have any other side effects as well. There is a rare but possibly serious side effect from choloramphenicol called aplastic anemia. Blood tests will have to be done several times a week to monitor for this side effect. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, or swelling of the face, throat or lips.

Precautions
If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics.

Colistin

How Does This Medication Work?
Colistin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual colistin dose for adults with CF is 75mg twice a day by IV.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they last for more than 2 days. These include mild nausea, diarrhea, and pain at the site of the injection, but tell us if you have any other side effects as well. Watch for and report any generalized pain or numbness, tingling of hands and feet, itchiness, vertigo, dizziness or slurred speech. If you notice decreased urine output please notify your doctor. Contact your doctor as soon as possible if you experience rash, hives, difficulty breathing, or swelling of the face, throat or lips.

Meropenem

How Does This Medication Work?
Meropenem works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual meropenem dose for adults with CF is 2 grams every eight hours by IV infusion.

Tazocin®

How Does This Medication Work?
Tazocin® works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The usual dose for adults with CF is 4.5 grams every 6 hours by IV infusion.

Precautions
If you are pregnant or think you might be pregnant, talk to your doctor before starting any antibiotics.

Tigecycline

Content coming soon.

Tobramycin

How Does This Medication Work?
Tobramycin works by decreasing the amount of bacteria in the lungs.

What Is The Usual Dose?
The amount of medication given depends on your weight and will be adjusted based on your blood levels. How often the medication is given will be determined by your doctor.

Vancomycin

Why is this medication prescribed?

Intravenous vancomycin is usually prescribed to treat pulmonary exacerbations in patients that grow Staphylococcus aureus in their lungs.

How does this medication work?

Vancomycin is an antibiotic in the glycopeptide family. It is used to treat infections caused by certain types of bacteria like Staphylococcus aureus and methicillin-resistant Staphylococcus aureus (MRSA) in particular. When vancomycin is given intravenously, it works to kill the bacteria causing an infection or exacerbation in the lungs.

What is the usual dose?

The dose of intravenous vancomycin can vary widely between patients and may change during a course of therapy. Vancomycin is dosed based on patient weight, kidney function and may be adjusted based on drug levels obtained during treatment. Typical doses are between 0.5 and 2 grams given two to three times daily.

How should this medication be taken?

Vancomycin is given by slow infusion into a vein. For patients receiving vancomycin at home, it can be administered through a CADD pump that remains attached to the patient all day and automatically administers the medication when it is due. Alternatively patients may choose to administer vancomycin by intermittent infusion in which the patient prepares and hangs a bag of vancomycin at specific times during the day. Discuss which option might work best for you with your CF clinic team.

What should I do if I miss a dose?

It is important that vancomycin is given exactly when it is due. If you miss a dose call the clinic to find out what to do.

What should I expect from this medication? (i.e. side effects)

If vancomycin is administered too quickly it can cause flushing, itchiness and a rash on the upper torso. If this happens stop the infusion and discuss with your CF clinic team. This type of reaction usually resolves if the infusion rate is slowed.

Vancomycin carries a risk of kidney dysfunction. Therefore while you are taking intravenous vancomycin you will have regular bloodwork to monitor your kidney function and the levels of vancomycin in your body.

Precautions

If you are pregnant or think you might be pregnant, discuss this with your doctor before starting any antibiotics. Intravenous or inhaled vancomycin can be taken during pregnancy if the benefits outweigh the potential risks.

Antibiotic Desensitization

Allergic reactions to antibiotics are more common in individuals with CF than in the general population due to frequent use of antibiotics in CF. The most common reactions are rashes, itchiness and hives; however, in rare cases anaphylaxis (a severe life-threatening allergic reaction usually occurring within minutes with low blood pressure, narrowing of your throat and swelling of your tongue which will make breathing difficult, nausea, vomiting, dizziness and fainting) can occur. If you experience one of these common reactions to an antibiotic you doctor will likely try to find an alternative antibiotic. However, if these antibiotics are needed in your treatment the team may refer you to an allergist. The allergist will use skin-testing to determine what type of allergy you have. If you have a specific type of allergic sensitivity known as an IgE mediated reaction, it is possible for you to be desensitized to that antibiotic.

See below for more information on what a desensitization is, as well as an outline of the process here at St. Michael’s.

What is desensitization?

Desensitization is a process where the patient is given a small quantity of an allergen and the amount is slowly increased. This tricks the immune system into accepting a medication to which it would normally overreact to.

Antibiotic desensitizations

Let’s say you are allergic to Meropenem. The desensitization would start with 1/10,000 of a typical dose. We would roughly double the dose at 15 minute intervals. Throughout this process you and the team will monitor for any reactions (ie rash or itching). If you’ve had no reactions then you’ve tolerated the desensitization and now you can receive normal doses safely.

The desensitization only lasts as long as you continue to receive the antibiotic on a regular schedule. If several doses of an antibiotic are missed or if you’ve finished treatment with that antibiotic then your immune system becomes re-sensitized and you could be at risk for allergic reaction the next time you receive the antibiotic. That’s why if you need to be treated with this antibiotic again in the future, you would have to undergo the desensitization process again.

Based on a study that looked at 95 desensitization procedures at St. Michael’s Hospital over a 10 year period, 87 (92%) were successfully completed. The most common symptoms were itchiness and rash and no patients had anaphylactic reactions.

What if you need to be desensitized?

Before the day that you are booked for desensitization you will require an additional IV access site for the antibiotic to be given.

On the day of your desensitization, you will be transferred to the intensive care unit where you will be monitoring during the desensitization by a nurse. Once there, the nurse will prepare the equipment to monitor your vital signs like heart rate, blood pressure, and breathing rate. He or she will also check your IV access line and prepare the doses of antibiotic to be given to you.

The whole desensitization process will take about 8-10 hours. After your first dose you will receive increasing concentrations of the antibiotic throughout the process. Your nurse will ask you if you are experiencing any reactions and your vital signs will be monitored after every dose throughout the procedure. If at any time during the procedure you notice either the same symptoms as your original reaction or any new allergic symptoms, please let your nurse know immediately. These could include rash, hives, itchiness, tachycardia (fast heart rate), hypotension (low blood pressure), bronchospasm (airway constriction), wheals, or angioedema (swelling under the skin).

Once the desensitization is finished, you will return to your regular room and you will continue to receive the full dose of the antibiotic on a regular schedule. Your nurses will give you the antibiotic at specific times because we don’t want you to develop any breakthrough allergic symptoms. There is less risk of you experiencing an allergic reaction if we keep a more consistent level of antibiotic, but please let your team know if you do experience any allergic symptoms at any time during your treatment.

Other Inhaled Medications

Bronchodilators increase the size of air passages to the lungs by relaxing the muscles that surround your air passages. This allows you to breathe easier. Some examples of common bronchodilators are salbutamol (Ventolin®), salmeterol (Serevent®) or formoterol (Oxeze®). Bronchodilators also allow your other medications (such as Pulmozyme® – see ‘mucus-busters’ below – or inhaled antibiotics – see above) to reach deep into your lungs so that you can obtain maximum benefit from the medication.

Some inhaled medications such as Advair® contain both a bronchodilator and a steroid. For example the Advair Diskus contains fluticasone (an inhaled corticosteroid) and salmeterol (a bronchodilator). For more information on the Diskus, visit the Canadian Lung Association website for more information.

Inhaled corticosteroids such as budesonide (Pulmicort®) and fluticasone (Flovent®) work in the lungs to reduce inflammation. This will help you to be able to breathe easier and will also allow your other medications to reach deeper into your lungs.

If you are using a nebulizer, please refer to Preparing & Taking Your Treatment in the Treatment Best Practices section on this page to prepare your dose of bronchodilators or steroids.

If you are taking other inhaled medication(s), please take them in the order listed at the top of this page.

The Mucous Busters: Mucolytics

Thick and sticky lung secretions or mucous (also called sputum) builds up and blocks the airways of people with CF. Drugs that either break up particles in the mucus or help make the mucous thinner are called mucolytics. Along with daily chest physiotherapy, they are used to help you cough up sputum and secretions more effectively.

Although there are many different types of mucolytics, the most effective and commonly used ones are Pulmozyme® and hypertonic saline. It is important to remember that although they both help with mucus clearance, they work in different ways. There are patients who may be on both of these medications at the same time.

Pulmozyme® (Dornase Alfa)

Why Is This Medication Prescribed?
Pulmozyme® is used in cystic fibrosis to make your sputum thinner. The sputum of people with cystic fibrosis can get thicker when cells that normally fight lung infections get trapped and break down. This breakdown of cells causes there to be extra deoxyribonucleic acid (also known as extracellular DNA) in the sputum, making it extra thick.

How Does This Medication Work?
Pulmozyme® works by breaking down DNA in the sputum, thus making sputum less thick. With proper chest physiotherapy, sputum can be cleared more easily.

What Is The Usual Dose?
Pulmozyme® is packaged with 6 ampoules per foil pouch. Each ampoule is 2.5mL. The usual dose for adults is 2.5mg (1 ampoule of 2.5mL) daily.

How Should This Medication Be Taken?
Take Pulmozyme® at approximately the same time everyday so that it is easier to remember. It should be taken before physiotherapy session or activity. Do not take it in the evening before bed.

To prepare your dose of Pulmozyme®, refer to Preparing & Taking Your Treatment in the Treatment Best Practices section on this page and:

Gently separate one ampoule and store the remainder in the foil pouch in the refrigerator.
Open the Pulmozyme® ampoule by holding the bottom tab with one hand and twisting off the top of the ampoule with the other hand. Be careful not to squeeze the ampoule until you are ready to empty its contents into the nebulizer cup.
Squeeze all the contents of the ampoule into your nebulizer.

If you are taking other inhaled medication(s), please take them in the order listed at the top of this page.

Note: DO NOT dilute or mix Pulmozyme® with other medications. Pulmozyme® must be stored in the REFRIGERATOR and protected from b light. DO NOT freeze. It should be kept refrigerated during transport. Pulmozyme® solution should be discarded if it is cloudy or discolored. It does not contain preservatives and, once opened, the entire contents of the ampoule must be used or discarded.

After use, rinse your nebulizer well with warm water before your next treatment to prevent build up that can clog your nebulizer. For more thorough cleaning, see Cleaning and Disinfecting Your Equipment.

What Should I Do If I Miss A Dose?
IF you miss a dose take it as soon as you remember but DO NOT double up or take extra doses of your Pulmozyme® to make up for the missed one! If it is late in the day, and you don’t plan to do anymore physiotherapy, skip that dose and take your dose the next day at the regular time.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they persist for more than 2 days. These include hoarseness, voice changes, chest tightness, wheezing, rash, increased cough, or sore throat, but tell us if you have any other side effects as well! To minimize these side effects, Ventolin® can be taken before your Pulmozyme® dose.

Precautions
Remember to tell your doctor if you have ever had a reaction to Pulmozyme® or any other inhaled medication in the past. If you are pregnant or think you may be pregnant, discuss this with your CF doctor before starting Pulmozyme®.

Patient Assistance Program
Roche has an assistance program called the Pulmozyme EasySTART. For more information and to see if you are eligible, click here. You can also ask your social worker, pharmacist or doctor if you have further questions.

Hypertonic Saline

Why Is This Medication Prescribed?
Hypertonic saline belongs to a class of drugs called mucolytics. Thick and sticky mucous can build up and block airways in the lungs. Using hypertonic saline may improve or help preserve lung function. It may also help decrease the number of respiratory tract infections.

How Does This Medication Work?
Using Hypertonic Saline puts more salt and water into your lungs and makes sputum less thick. Thinner sputum is easier to cough up.

What Is The Usual Dose?
5% Hypertonic Saline: The usual dose for adults is 5mL daily or twice daily. 7% Hypertonic Saline: The usual dose for adults is 4mL (1 ampoule) once or twice daily.

How Should This Medication Be Taken?
To prepare and take hypertonic saline, refer to Preparing & Taking Your Treatment in the Treatment Best Practices section on this page.

To prepare your dose (from a 5% bag):

Wipe insertion site of Hypertonic Saline bag with alcohol (swab).
Insert needle into port, and withdraw 5mL of Hypertonic Saline solution.
Expel all the contents of the syringe into your nebulizer.

To prepare your dose (from 7% ampoule):

Open the Hypertonic Saline ampoule by holding the bottom tab with one hand and twisting off the top of the ampoule with the other hand. Be careful not to squeeze the ampoule until you are ready to empty its contents into the nebulizer cup.
Squeeze all the contents of the ampoule into your nebulizer.

Take Hypertonic Saline at about the same time everyday so that it is easier to remember. It should be taken before physiotherapy session or activity. Do not take it in the evening before bed.

If you are taking other inhaled medication(s), please take them in the order listed at the top of this page.

Note: DO NOT dilute or mix hypertonic saline with other medications. Hypertonic Saline should be stored in the REFRIGERATOR in between uses. DO NOT freeze.

5% Hypertonic Saline bag should be thrown away if the solution is cloudy or discoloured. It contains no preservative and, once opened, should only be used for 1 week. Please change the Hypertonic Saline bag, needle, and syringe every week. To help you remember when to change the bag, label the bag with the date it was opened on.

7% Hypertonic Saline ampoule should be discarded if the contents are cloudy or have changed colour . Do not use past the date stamped on the ampoule.

What Should I Do If I Miss A Dose?
If you miss a dose take it as soon as you remember but DO NOT double your dose of Hypertonic Saline.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear but let your doctor know if they are severe or if they persist for more than 2 days. These include hoarseness, voice changes, chest tightness, wheezing, increased cough, or sore throat, but tell us if you have any other side effects as well!

Precautions
Remember to tell your doctor if you have ever had a reaction to Hypertonic Saline or any other inhaled medication in the past.

Systemic Corticosteroids

Corticosteroids are widely used for treating a variety of conditions in which tissues becomes inflamed. In CF, corticosteroids may be used to treat lung inflammation and mucous plugging. They are used for various periods of time in individuals who have significant shortness of breath and wheezing that accompanies narrowed airways.

Corticosteroids can be used in a condition called allergic bronchopulmonary aspergillosis. Prednisone (see below) is the treatment of choice for ABPA and helps to reduce symptoms of wheezing, chest tightness and shortness of breath.

Corticosteroids are also used in transplant patients to prevent the immune system from rejecting the transplanted organ.

Prednisone

Why Is This Medication Prescribed?
Prednisone is a medication that belongs to the class of medications called corticosteroids. It is similar to a hormone produced naturally by the body called cortisol, which plays a role in the body’s response to stress. Prednisone is also used in individuals who have had a transplant to prevent the immune system from rejecting the transplanted organ.

How Does This Medication Work?
Prednisone works by decreasing inflammation in your airways and lungs.

What Is The Usual Dose?
Prednisone has variable dosing and your doctor will decide what the appropriate dosage is. It is usually given by mouth once daily but can be given twice daily.

How Should This Medication Be Taken?
Because this medication may cause stomach upset it is best taken with food or milk. Alcoholic beverages may increase stomach burning or upset. If prednisone is prescribed once daily it is best taken first things in the morning with breakfast. If prescribed twice daily then it is best taken with breakfast and dinner.

What Should I Do If I Miss A Dose?
If you forget to take a dose, take the missed dose as soon as you remember. If it is almost time for your next dose, skip the missed dose and take the next scheduled dose. Do not double the dose.

What Should I Expect From This Medication?
Some side effects may occur or you may not experience any. Let your doctor know if the side effects are severe or if they persist for more than 2 days. Possible side effects include:

signs of infection such as cough, fever, sore throat and mouth ulcers
continuing or excessive heartburn or stomach upset
bone or joint pain
muscle cramps or weakness
increased thirst or urination
eye pain or blurred vision
mood changes
slow wound healing
cosmetic effects such as round/puffing of the face, weight gain, hair growth and acne

Precautions
This drug may increase your blood sugars. Diabetic individuals should check their blood sugars regularly and report any unusual levels to their doctor.
Do not stop using this medication without first checking with your doctor, as the dose is sometimes tapered before stopping the drug completely. Your body will need time to adjust to the declining levels of drug in your system. The length of time taken to taper the drug depends on the dose you were on and how long you have been on it.

CFTR Modulator Drugs

Ivacaftor (Kalydeco)

Why is this medication prescribed?
Ivacaftor (Kalydeco) is a CFTR modulator. CFTR modulators are a new type of medication that works directly on the defective CFTR protein to facilitate the movement of chloride and bicarbonate and thus correct the problem with the excessively thick mucous seen in CF.

How does this medication work?
The CFTR protein is a channel that has a gate that opens and closes. Certain mutations cause the gate to be closed or not fully open and these are called ‘gating mutations’. Ivacaftor works specifically on gating mutations to allow the gate to fully open so chloride and bicarbonate can pass more efficiently across the cell membrane.

What is the usual dose?
This medication is in pill form. The usual dose is 150 mg tablet taken orally every 12 hours.

How should this medication be taken?
The medication should be taken with fat containing foods to aid absorption

What should I do if I miss a dose?
If a dose of ivacaftor is missed within 6 hours of the time it is usually taken, then you can take the medication as soon as possible with fat containing foods. If more than 6 hours have passed since ivacaftor is usually taken, the missed dose should not be taken and you should resume the usual dosing schedule when it is due next.

What should I expect from this medication?
This medication may or may not cause side effects.

Precautions
Contact your doctor if you experience a rash or other unusual side effects.

Lumacaftor / Ivacaftor (Orkambi)

Why is this medication prescribed?
Lumacaftor/Ivacaftor (Orkambi) is a CFTR modulator which contains two different drugs. CFTR modulators are a new type of medication that works directly on the defective CFTR protein to facilitate the movement of chloride and bicarbonate and thus correct the problem with the excessively thick mucous seen in CF. This medication is prescribed to people who have two copies of the deltaF508 CF mutation.

How does this medication work?
The CFTR protein is normally located in the cell membrane and acts as a channel that has a gate that opens and closes. Lumacaftor/ivacaftor helps to move the protein to the right location in the cell membrane and ensure that the gate is open so it can function more efficiently.

What is the usual dose?
This medication is in pill form. The usual dose is two tablets (each containing lumacaftor 200mg/ivacaftor 125 mg) taken orally every 12 hours.

How should this medication be taken?
The medication should be taken with fat containing foods to aid absorption

What should I do if I miss a dose?
If a dose is missed within 6 hours of the time it is usually taken, then you can take the medication as soon as possible with fat containing foods. If more than 6 hours have passed since lumacaftor/ivacaftor is usually taken, the missed dose should not be taken and you should resume the usual dosing schedule when it is due next.

What should I expect from this medication?
This medication may or may not cause side effects. Some people have found this medication was associated with increased shortness of breath, diarrhea, runny nose, rash.

Precautions
Contact your doctor if you experience any unusual side effects. Lumacaftor/ivacaftor can interact with other medications and you should review all your medications with your CF doctor before taking this medication.

Tezacaftor / Ivacaftor (Symdeko)

Why is this medication prescribed?
Tezacaftor/Ivacaftor (Symdeko) is a CFTR modulator which contains two different drugs. CFTR modulators are a new type of medication that works directly on the defective CFTR protein to facilitate the movement of chloride and bicarbonate and thus correct the problem with the excessively thick mucous seen in CF.

How does this medication work?
The CFTR protein is normally located in the cell membrane and acts as a channel that has a gate that opens and closes. Tezacaftor/ivacaftor helps to move the protein to the right location in the cell membrane and ensure that the gate is open so it can function more efficiently.

What is the usual dose?
This medication is in pill form. The usual dose is one tablet of tezacaftor 100mg/ivacaftor 150 mg taken in the morning and one tablet of ivacaftor 150mg taken in the evening approximately 12 hours apart.

How should this medication be taken?
The medication should be taken with fat containing foods to aid absorption

What should I do if I miss a dose?
If a dose is missed within 6 hours of the time it is usually taken, then you can take the medication as soon as possible with fat containing foods. If more than 6 hours have passed since the missed morning or evening dose, the missed dose should not be taken and you should resume the usual dosing schedule when it is due next.

What should I expect from this medication?
This medication may or may not cause side effects. Some people have found this medication was associated with dizziness, headache, runny nose.

Precautions
Contact your doctor if you experience any unusual side effects.

Elexacaftor / Tezacaftor / Ivacaftor (Trikafta)

Why is this medication prescribed?
Elexacaftor/tezacaftor/ivacaftor (Trikafta) is a CFTR modulator also known as triple combination therapy because it contains three different drugs. CFTR modulators are a new type of medication that works directly on the defective CFTR protein to facilitate the movement of chloride and bicarbonate and thus correct the problem with the excessively thick mucous seen in CF.

How does this medication work?
The CFTR protein is normally located in the cell membrane and acts as a channel that has a gate that opens and closes. Elexacaftor/tezacaftor/ivacaftor helps to move the protein to the right location in the cell membrane and ensure that the gate is open so it can function more efficiently.

What is the usual dose?
This medication is in pill form. The usual dose is two tablets of elexacaftor 100 mg/tezacaftor 50mg/ivacaftor 75 mg taken in the morning and one tablet of ivacaftor 150mg taken in the evening approximately 12 hours apart.

How should this medication be taken?
The medication should be taken with fat-containing foods to aid absorption. Avoid taking trikafta with foods and drinks that contain grapefruit because they may affect the amount of trikafta in your body.

What should I do if I miss a dose?
If you miss your morning dose: If it has been 6 hours or less since the morning dose is usually taken, then take the missed dose with fat-containing foods as soon as possible. Then take the next dose at the usual time with fat-containing foods. If it has been more than 6 hours since the morning dose is usually taken then take the missed dose as soon as possible but do NOT take the evening dose and resume regular dosing schedule the next day.
If you miss your evening dose: If it has been 6 hours or less since the evening dose is usually taken, take the missed dose with fat-containing food as soon as possible. Then take the next dose at the usual time. If it has been more than 6 hours since the evening dose is usually taken, do NOT take the missed dose. Take the next morning dose at the usual time with fat-containing food.

What should I expect from this medication?
This medication may or may not cause side effects. Some people have found this medication was associated with headache, runny nose, stomach pain, or rash. Some medications can interact with Trikafta therefore talk to your CF team about all the medications you are taking before you start the medication.

Precautions
Contact your doctor if you experience any unusual side effects such as abdominal pain, yellowing of the skin or whites of the eyes, nausea or vomiting.

Find more information about dietary changes required when taking CFTR modulators in the last tab of “Maintaining a healthy weight“.

Biologic Therapy for Asthma

Benralizumab (Fasenra)

Why Is This Medication Prescribed?
Benralizumab (Fasenra) is a biologic medication prescribed for the treatment of severe asthma or allergic bronchopulmonary aspergillosis (ABPA). It may be used in patients with cystic fibrosis who continue to have asthma type symptoms (ex. chest tightness, wheezing) despite treatment with other asthma medications.

How Does This Medication Work?
Benralizumab works by inhibiting a protein called interleukin-5 (IL-5). IL-5 is a type of chemical messenger that activates eosinophils, which are cells that are part of the inflammatory response that is responsible for asthma and ABPA. To benefit from Benralizumab, patients need to have a high level of eosinophils in their blood.

What Is The Usual Dose?
The recommended dose of Benralizumab is 30mg every 4 weeks for the first 3 doses, and then once every 8 weeks thereafter.

How Should This Medication Be Taken?
Benralizumab is administered by a single subcutaneous injection into the thigh or abdomen. This can be done at a clinic or at home after some training.

What Should I Do If I Miss A Dose?
Patients who miss a dose of Benralizumab should administer their next dose as soon as possible and/or check with your doctor or clinic.

What Should I Expect From This Medication?
Improvement in your asthma symptoms is usually noticeable after about 4 weeks. You should continue taking your other asthma medications while on Benralizumab. The most common side effects are headaches, joint pain, fever, sore throat, and muscle pain.

Precautions
Tell your doctor if you have previously had a severe allergic reaction to Benralizumab. Call your doctor or go to the emergency room if you experience hives, swelling of the tongue, face, or mouth, difficulty breathing, or loss of consciousness. Benralizumab should not be used to treat acute asthma symptoms.

Dupilumab (Dupixent)

Why Is This Medication Prescribed?
Dupilumab (Dupixent) is a biologic medication prescribed for the treatment of severe asthma or allergic bronchopulmonary aspergillosis (ABPA). It may be used in patients with cystic fibrosis who continue to have asthma type symptoms (ex. chest tightness, wheezing) despite treatment with other asthma medications.

How Does This Medication Work?
Dupilumab works by inhibiting two proteins called interleukin-4 (IL-4) and interleukin-13 (IL-13). These proteins are important parts of the inflammatory response that is responsible for asthma and ABPA.

What Is The Usual Dose?
The recommended dose of Dupilumab is 400mg initially followed by 200mg every other week or an initial dose of 600mg followed by 300mg every other week for more severe asthma.

How Should This Medication Be Taken?
Dupilumab is administered by two subcutaneous injections into the thigh or abdomen initially, and 1 injection for the following doses. This can be done at a clinic or at home after some training.

What Should I Do If I Miss A Dose?
Patients who miss a dose of Dupilumab can administer it within 7 days of the original schedule. If it has been longer than 7 days, patients should skip the missed dose and wait until their next scheduled dose.

What Should I Expect From This Medication?
Improvement in lung function from Dupilumab can occur in as little as 2 weeks. You should continue taking your other asthma medications while on Dupilumab. The most common side effects are sore throat, injection site reactions, and a high count of eosinophils in the blood (eosinophilia).

Precautions
Tell your doctor if you have previously had a severe allergic reaction to Dupilumab. Call your doctor or go to the emergency room if you experience hives, swelling of the tongue, face, or mouth, difficulty breathing, or loss of consciousness. Dupilumab should not be used to treat acute asthma symptoms.

Omalizumab (Xolair)

Why Is This Medication Prescribed?
Omalizumab (Xolair) is a biologic medication prescribed for the treatment of severe asthma or allergic bronchopulmonary aspergillosis (ABPA). It may be used in patients with cystic fibrosis who continue to have asthma type symptoms (ex. chest tightness, wheezing) despite treatment with other asthma medications.

How Does This Medication Work?
Omalizumab works by blocking IgE antibodies, which are part of the inflammatory response that is responsible for asthma and ABPA.

What Is The Usual Dose?
Dosing for Omalizumab is based on a patient’s weight and levels of IgE antibodies in the blood. Typically, a dose of 150-375mg is used every 2 to 4 weeks.

How Should This Medication Be Taken?
Omalizumab is administered by subcutaneous injection. This can be done at a clinic or at home after some training. Your prescribed dose may require more than one injection at a time.

What Should I Do If I Miss A Dose?
Patients who miss a dose of Omalizumab should administer their next dose as soon as possible and/or check with your doctor or clinic.

What Should I Expect From This Medication?
It can take several weeks before benefits are noticeable so patients usually need to take Omalizumab for 3 to 4 months before we can determine if the drug is right for them. Those who respond well to the medication are generally continued on it long-term. Omalizumab is well-tolerated and serious side-effects such as a severe allergic reaction are very rare. The most common side-effects include headache, swelling of the inside of the nose, and joint pain.

Precautions
Tell your doctor if you have previously had a severe allergic reaction to Omalizumab. Call your doctor or go to the emergency room if you experience hives, swelling of the tongue, difficulty breathing, or loss of consciousness. Extra caution should be used in women who are breastfeeding or pregnant.

Mepolizumab (Nucala)

Why Is This Medication Prescribed?
Mepolizumab (Nucala) is a biologic medication prescribed for the treatment of severe asthma or allergic bronchopulmonary aspergillosis (ABPA). It may be used in patients with cystic fibrosis who continue to have asthma type symptoms (ex. chest tightness, wheezing) despite treatment with other asthma medications.

How Does This Medication Work?
Mepolizumab works by inhibiting a protein called interleukin-5 (IL-5). IL-5 is a type of chemical messenger that activates eosinophils, which are cells that are part of the inflammatory response that is responsible for asthma and ABPA. To benefit from Mepolizumab, patients need to have a high level of eosinophils in their blood.

What Is The Usual Dose?
The recommended dose of Mepolizumab is 100mg every 4 weeks in adults.

How Should This Medication Be Taken?
Mepolizumab is administered by a single subcutaneous injection into the thigh or abdomen. This can be done at a clinic or at home after some training.

What Should I Do If I Miss A Dose?
Patients who miss a dose of Mepolizumab should administer their next dose as soon as possible and/or check with your doctor or clinic.

What Should I Expect From This Medication?
Improvement of symptoms once starting Mepolizumab can occur within as little as 48 hours, although it can take up to 4 weeks to see the full benefit. You should continue taking your other asthma medications while on Mepolizumab. The most common side effects are headaches, joint pain, skin reactions such as a rash, fatigue, and muscle pain.

Precautions
Tell your doctor if you have previously had a severe allergic reaction to Mepolizumab. Call your doctor or go to the emergency room if you experience hives, swelling of the tongue, face, or mouth, difficulty breathing, or loss of consciousness. Use of Mepolizumab may increase the chance of getting a herpes zoster infection (shingles). Mepolizumab should not be used to treat acute asthma symptoms.

Tezapelumab (Tezspire)

Coming Soon

Medications in Pregnancy

If you are planning to get pregnant or have just found out you are, you can discuss with your CF team which medications you should continue taking and which ones may need to be stopped. The same applies for those who will be breastfeeding. Many medications can be potentially harmful to your baby and therefore we recommend that you speak to your pharmacist and your CF team if you have any questions about the medications you are taking or are about to start taking.

Complementary or Alternative Medicine

There is increasing need to study complementary and alternative medicine (CAM) because we cannot ignore the benefits we see all around us. But due to limited information and evidence surrounding CAM, we should continue to be cautious and weigh the risks and benefits.

Anything we may take – be it conventional medication, CAM, or even food – can have an effect on our bodies. This can be a beneficial effect, or it can be a side effect – it all depends on how you look at it. So, if a product says it has “no side effects”, we should wonder if it has any good effects at all!

In cystic fibrosis, one of our main concerns is the risk of herbal-drug interactions. This does not mean that an herb and a drug combined produce something toxic. Instead, the interaction may be that the herbal product may cause either an increase or decrease in the amount of the other drug in the body. Even if there are no reports in the literature, there’s still a chance there may be an interaction. Continue to monitor your overall health and keep your health care team in the loop.

Probiotics & Florastor®

Probiotics are live microorganisms (yeast or bacteria) that may improve the microbial balance of your gut and prevent diarrhea. Most probiotics are similar to the bacteria that are found naturally in your gut.

Why do you want to replace the bacteria/yeast in your gut?

The antibiotics that you are taking to kill the bacteria in your lungs also kill the bacteria and yeast that are normally found in your gut. In many patients this can lead to diarrhea – specifically called antibiotic associated diarrhea (AAD). By replacing the bacteria in your gut that have been killed with good bacteria/yeast you may experience fewer episodes of diarrhea and less cramping or bloating.

The two most common probiotics used in CF patients are Lactobacillus species and Saccharomyces boulardii (Florastor®).

Florastor®

Why Is This Medication Prescribed?
Florastor® (Saccharomyces boulardii) is a probiotic that contains live yeast that may improve the microbial balance of your gut and prevent diarrhea.

How Does This Medication Work?
Florastor® works by replacing the normal, healthy bacteria in your gut that is destroyed by antibiotic therapy.

What Is The Usual Dose?
Florastor® is available in a capsule form. A typical adult dose can vary from 1 capsule once a day to several capsules several times a day. Your CF doctor will determine the best dose for you.

How Should This Medication Be Taken?
You may take Florastor® with or without food. Store the tablet at room temperature away from heat, moisture and direct light.

What Should I Expect From This Medication?
You may or may not experience side effects. These side effects may disappear; but, let your doctor know if they are severe or if they last for more than 2 days. A small number of patients experience flatulence or thirst while using Florastor®.

Costs & Drug Coverage Information

There are many medications used in the treatment of cystic fibrosis so it is understandable that the cost can be an issue. Even with government funding and insurance coverage, sometimes we are not able to get the medications we need.

Find out the common cost and coverage issues related to cystic fibrosis medications and what your health care team, social worker, and pharmacist can do behind the scenes.

CF Drug Coverage In Ontario

Ontario's CF Special Drug Program

If you are cared for by the team at St. Michael’s Toronto Adult CF clinic, and are a resident of Ontario, then you have drug access and coverage (funding) for certain CF medications through the Ontario Special Drugs Program. This program does not have any deductibles or co-payments so you will not have to pay for any of the medications that are covered through the program (the list of covered medications is here). These covered medications are dispensed by the out-patient pharmacy at St. Michael’s Hospital, the Prescription Care Centre.

Ontario Drug Benefit (ODB) Program

Some CF medications and many non-CF medications can be accessed through the province’s drug benefit program. A list of over 4,400 drugs are accessible to individuals over 65 years of age, those that qualify for the Ontario Disability Program (OSDP), or those enrolled in the Trillium Drug Program (TDP).

OHIP+

On January 1, 2018, individuals age 24 and under will have access to all medications on the Ontario Drug Benefit Formulary and any medications specially requested through the Exceptional Access Program (EAP), at no cost and with no deductibles.

Private Insurance Plans

Alternatively, you may have some or all of your drug costs covered by your (or your parents’) employer through a private drug insurance plan. It is important to know the details of insurance coverage for example do you have a yearly or lifetime maximum on drug costs, what percentage of the costs are covered by your insurance company among other things. You can check whether your plan covers specific drugs by providing the insurance company with the drug identification number, also called the DIN number. Keep in mind, the insurance company may have additional forms and eligibility criteria for specific medications.

Private Insurance Numbers

If you have private insurance, you may want to contact your private insurance company to determine the extent of your coverage. If you have less than 100% coverage by your private insurance company, you may still be eligible for Trillium. Below are some common private insurance companies and their telephone numbers.

Standard Life: 1-(888)-841-6633
Manulife: 1-(877) 268-3763
ClaimSecure: 1-(800)-461-6579
Desjardins: 1-(866)-344-5195
Great-West Life: 1-(800)-724-3402
Empire Financial Group: 1-(416)-868-6518
Green Shield Canada: 1-(888)-711-1119

Patient Assistance Programs

Patient Assistance Programs are in place to help you to pay for your medications.

TOBI Co-Pay

Novartis has an assistance program called the TOBI® Co-Pay Program that helps patients without full insurance coverage. If you have private drug insurance or have government assistance (ie. Trillium or the Ontario Disability Support Program), you may be eligible under the TOBI® Co-Pay Program to have most or all of your deductible paid for. For more information and to see if you are eligible, ask your social worker, pharmacist or doctor.

Pulmozyme EASYSTART

Roche has an assistance program called the EasySTART Program that can (i) help patients without full insurance coverage and (ii) bridges therapy until coverage details can be finalized. If you have private drug insurance or have government assistance (ie. Trillium or disability), you are eligible under the EasySTART program to start Pulmozyme therapy with little delay. If you are not fully covered for Pulmozyme, the EasySTART program will also have most or all of your deductible paid for. We work with EasySTART representatives, who will contact you to organize the delivery of your Pulmozyme and find ways to maximize coverage of your Pulmozyme.

Cayston Patient Assistance Program (PAP)

Gilead has an assistance program called Cayston PAP that helps patients (i) without full insurance coverage or (ii) in special clinical circumstances. We work with your private insurance company and Cayston PAP representatives to maximize coverage of your Cayston. For more information and to see if you are eligible, ask your social worker, pharmacist or doctor.

The Village Program

Vertex has created The Village Program which will assist patients to navigate the process required so that private insurance companies cover the cost of modulators such as Trikafta. For more information on this program, speak to your CF team.

CF Drug Coverage

If you are currently a non-Ontario resident who is relocating to Ontario, or moving for your CF care and/or lung transplant, there are a couple of things to consider when it comes to drug coverage.

First, when an individual moves from one Canadian province to Ontario, there is a three-month waiting period before he or she can become eligible for coverage in Ontario. During this time, the individual is covered by the provincial plan of your home province. You should make an application for Ontario coverage and an OHIP number immediately upon your arrival to Ontario. Also, consider which maintenance medications you will need and have your current CF team order you enough medication for at least a 3 month period. You can then bring that medication with you to Ontario or have it mailed to you from your pharmacy in your home province.

Second, it is common that new medications may need to be started during your three-month waiting period. Some have reciprocal billing such that your medications will be covered by the Ontario Drug Benefit program which will bill your home province for you. Other provinces require that before you leave your home province, you must receive a letter from the Ministry of Health that you are to submit to the pharmacy each time you pay for medication. This arrangement requires you to pay for medications up front, and then your home province will reimburse you on a monthly or quarterly basis, all depending on the arrangement they make with you. Before you move, please inquire with your home province which arrangement exists, and let our team know on your first visit.

For more information, please speak to your physician, pharmacist, or social worker.

Medications / Drug Access

On this page

What is the proper order for taking my medications?

Antibiotics

Inhaled Antibiotics

Amikacin

Aztreonam (Cayston®)

Colistin

TOBI®

TOBI Podhaler

Inhaled Tobramycin

To prepare and take your inhaled antibiotics, please also refer to “Preparing & taking your treatment” in the Treatment Best Practices section.

Oral Antibiotics

Azithromycin

Ciprofloxacin

Cloxacillin

Doxycycline

Keflex

Levofloxacin

Minocycline

Septra®

IV Antibiotics

Amikacin

Aztreonam

Ceftazidime

Chloramphenicol

Colistin

Meropenem

Tazocin®

Tigecycline

Tobramycin

Vancomycin

Antibiotic Desensitization

Other Inhaled Medications

The Mucous Busters: Mucolytics

Systemic Corticosteroids

CFTR Modulator Drugs

Biologic Therapy for Asthma

Medications in Pregnancy

Complementary or Alternative Medicine

Florastor®

Costs & Drug Coverage Information

CF Drug Coverage In Ontario

Private Insurance Numbers

Patient Assistance Programs

CF Drug Coverage