Getting started with ARIKAYCE

Welcome to the video tutorial for using your Lamira® Nebulizer System for ARIKAYCE® (amikacin liposome inhalation suspension).

ARIKAYCE is a prescription medicine used to treat adults with refractory (difficult to treat) Mycobacterium avium complex (MAC) lung disease as part of a combination antibacterial drug treatment plan (regimen).

It is not known if ARIKAYCE is safe and effective in children younger than 18 years of age.

This product was approved by FDA using the Limited Population pathway. This means FDA has approved this drug for a limited and specific patient population, and studies on the drug may have only answered focused questions about its safety and effectiveness.

ARIKAYCE can cause serious side effects, including:

• inflammation of the lungs. These respiratory problems may be symptoms of allergic inflammation of the lungs and often come with fever, wheezing, coughing, shortness of breath, and fast breathing
• coughing up of blood (hemoptysis). Coughing up blood is a serious and common side effect of ARIKAYCE
• severe breathing problems. Severe breathing problems can be symptoms of bronchospasm. Bronchospasm is a serious and common side effect of ARIKAYCE. Bronchospasm symptoms include shortness of breath, difficult or labored breathing, wheezing, and coughing or chest tightness
• worsening of chronic obstructive pulmonary disease (COPD). This is a serious and common side effect of ARIKAYCE

While using ARIKAYCE, these side effects may become serious enough that treatment in a hospital is needed.

Please stay tuned for the full Safety Information at the end of this video.

Today, I invite you to watch and learn as I use the Lamira Nebulizer System for ARIKAYCE.

Before using your Lamira Nebulizer System, carefully read the entire Instructions for Use. Take special note of all safety precautions including those marked Danger and Warning, as these are not fully covered in this video. If you do not understand any part of the Instructions for Use, talk to your doctor or contact Arikares Support.

The Lamira Nebulizer Handset is for your use only. Do not share your handset with other people. The Handset is specifically designed for ARIKAYCE, so you should never use other medicine in the Handset. These instructions, along with this video, provide fully detailed information about each step for treating yourself with ARIKAYCE. Feel free to watch the video as many times as you need. Now let's get started!

GETTING STARTED

To prepare, have your ARIKAYCE 28-day drug kit and Lamira Nebulizer System package ready. You will also need clear liquid soap for cleaning the Handset, and distilled water for disinfecting the Handset.

Your ARIKAYCE 28-day drug kit will contain: 1 ARIKAYCE Quick Start Guide, 1 Instructions for Use insert, 1 Full Prescribing Information and Medication Guide insert, 1 Lamira Handset (the Handset includes the Medication Reservoir, the Aerosol Chamber, the Medication Cap, the Blue Valve, and the Mouthpiece; the Handset is to be used for 28 days), 4 Lamira Aerosol Heads (replace the Aerosol Head with a new one, after 7 uses), and 28 once-daily vials of ARIKAYCE.

Then, check your Lamira Nebulizer System package to make sure you have all the items you'll need: the Carrying Case, the AC Power Supply, 4 AA batteries, the Connection Cord, the Controller, a spare Aerosol Head, and a spare Handset. Your Lamira can use either AA batteries or the A/C Power Supply.

Today, I'll show you how to use the Lamira with the A/C Power Supply.

You should store your ARIKAYCE medicine in the refrigerator until the expiration date on the vial. Do not freeze your medicine.

Remove your ARIKAYCE from the refrigerator at least 45 minutes before you use it. This way, it is at room temperature when you are ready to take your treatment.

CLEANING AND DISINFECTING BEFORE FIRST USE

When you get your Handset and Aerosol Head, they will not be sterile. It's important that you clean and disinfect your Handset and Aerosol Head before you use them for the first time. Cleaning and disinfecting your Handset and Aerosol Head is important to reduce the risk of infection, illness, and contamination. Here, let me show you how to do it.

Open the plastic bag with the Handset parts. Remove the Aerosol Head from its packaging, being careful to only touch the plastic outer ring. Do not touch the center silver part of the Aerosol Head. Gently pull up on the tab to open the Handset and remove the Blue Valve. Prepare a clean tub or bowl by adding a few drops of clear liquid dish soap and warm tap water to it. Do not use white or antibacterial liquid dish soaps. Rinse all the parts of your handset including the Medication Cap and Seal, the Blue Valve, the Reservoir and Chamber, the Mouthpiece, and the Aerosol Head. Pay special attention to rinsing the Aerosol Head and rinse each side of it for 10 seconds. Thorough rinsing of both sides of the Aerosol Head helps to prevent clogging and keeps it working properly. Do not put the Handset or the Aerosol Head in the microwave oven. Do not try to clean the Handset or Aerosol Head in a dishwasher, and do not try to clean the Handset or the Aerosol Head with brushes or abrasives. Cover the Handset parts and Aerosol Head in the warm soapy water and soak for 5 minutes, shaking them periodically. When 5 minutes are up, rinse all the parts thoroughly under warm running tap water to remove all the soap. Check each part - if anything looks dirty, then let it soak again for another 5 minutes. Once all the parts are cleaned and free of soap, you can move onto the next step - disinfecting the Handset.

You must disinfect your Handset parts and Aerosol Head before you use them for the first time. To do this, fill a clean pot with enough distilled water to completely cover all the handset parts including the Aerosol Head. Heat the water to a boil in a clean pot. Then place the Handset parts, including the Aerosol Head, into the boiling water. Boil for a full 5 minutes. It may be helpful to use a timer. Then, air dry the Handset parts and the Aerosol Head on a lint-free towel. After they're completely dry you can wrap them in a lint-free towel for storage to make sure the Blue Valve is not damaged. Reassemble the parts only just before taking your treatment.

ASSEMBLING THE HANDSET

Gather all your supplies in a comfortable well-lit room where you feel relaxed. I prefer my bedroom, but some people prefer the living room. Be sure you have a clean, flat, stable surface to work on, and an accessible outlet if you plan on using the A/C power supply.

Once you're settled, connect the power supply. Place the Controller on a clean, flat, stable surface. Push the round end of the A/C Power Supply plug into the plug inlet port on the underside of the gray Battery Door. Do not try to insert the A/C Power Supply into the front of the Controller. Then plug the A/C Power Supply into a wall outlet.

To reduce risk of electrocution, do not place or store the Lamira Nebulizer System near or into water or other liquid such as bathtub or sink. Do not use while bathing. If the Lamira Nebulizer System does fall into water or other liquid, do not reach for it. Unplug it right away. Pick up the Lamira only after it has been unplugged.

Now that your Handset parts and Aerosol Head are cleaned, disinfected, and dry, they are ready for use. Wash and dry your hands, then you can assemble your Handset. Open the Handset by gently pulling up on the tab of the Medication Reservoir. This releases the Aerosol Chamber so you can open it. Then, insert the Blue Valve on top of the Aerosol Chamber, making sure the two valve flaps are positioned down and the valve flaps are not bent. Do not push the Blue Valve inside the Aerosol Chamber. It should be placed on top of the Aerosol Chamber.

Next, insert the Aerosol Head. Remember, when handling the Aerosol Head, carefully grasp the 2 flexible plastic tabs on each side, and never touch the silver part at any time during assembly. Hold the Aerosol Head so that the text is facing toward you and is at the top of the Aerosol Head. Squeeze the 2 flexible plastic tabs together while inserting the metal arms and flexible plastic tabs into the Medication Reservoir. You should feel the flexible plastic tabs grab as you insert it. Close your Handset by pushing the Aerosol Chamber together with the Medication Reservoir until you hear a snap and there are no gaps along the Blue Valve edge. If you don't hear a snap, open the Handset and check that the Blue Valve is seated properly. Attach the Mouthpiece to your Handset, making sure the Blue Flap is facing up and is pressed in the slot on the Mouthpiece. Make sure the Mouthpiece stays attached to the Handset during treatment.

Lastly, attach the Connection Cord to the Handset and the Controller. Align the bottom of the Connector with the bottom of the Handset and push the Connection Cord upward against the rear underside of the Handset until you hear the parts snap together. Then push the round end of the Connection Cord into the plug inlet port located under the digital display. Place the Controller with the attached Handset on a clean, flat, stable surface.

Only use adapters and accessories that are made for the Lamira. Never substitute the Handset for any other eFlow® Technology Handset, and never use the Lamira Aerosol Head in any other eFlow® Technology Handset.

TAKING ARIKAYCE

Before taking ARIKAYCE, here's some important information you should know:

If you use a bronchodilator, use it first before using ARIKAYCE. Refer to your bronchodilator leaflet for information. The nebulizer for ARIKAYCE should only be used for taking ARIKAYCE. Each vial of ARIKAYCE is for single use only. Do not use ARIKAYCE with any other type of Handset or Aerosol Head than the one provided in the carton. Do not put other medicines in the Lamira Nebulizer Handset. Do not drink the liquid in the vial. Do not use ARIKAYCE if the expiration date has expired.

Remove your ARIKAYCE from the refrigerator at least 45 minutes before you use it. This way, it is at room temperature when you are ready to take your treatment.

Get your ARIKAYCE ready before adding it to the Lamira Nebulizer Handset, by shaking the vial well for at least 10 to 15 seconds, until the medicine looks the same throughout and well mixed. Open your vial by lifting the cap from the vial, then throw away the cap. Grip the metal ring on top of the vial, then pull it down gently until one side breaks away from the vial but do not pull the tab completely off. Pull the metal band from around the vial top in a circular motion until it comes off completely. Throw away the metal band. Then, carefully remove the rubber stopper by pulling it upward.

Make sure your Handset is placed on a clean, flat, stable surface. Then pour the vial of ARIKAYCE into the Medication Reservoir. Do not use more than one vial per treatment.

Attach the Medication Cap by lining up the tabs on the Medication Cap with the tab slots on the Medication Reservoir. Turn the Medication Cap clockwise until it stops. As the Medication Cap is turned, the inner cap of the Medication Cap should rise.

To begin your treatment, sit in a relaxed, upright position. Press and hold down the ON/OFF button for 2 to 3 seconds. You will hear one beep, the status light will turn green, and the START SCREEN will appear on the LCD. Your Lamira is now on.

After treatment begins, the TREATMENT SCREEN will replace the START SCREEN and aerosol mist will begin to flow. Holding the Handset level, insert the Mouthpiece into your mouth. Place it on top of your bottom lip and tongue and close your lips around it but do not cover the blue flap.

Take slow, deep breaths then breathe normally in and out through the Mouthpiece until your treatment is complete. Your treatment should take about 14 minutes, but could take up to 20 minutes. Keeping the Handset level throughout your treatment is important so it stays on.

If the Handset is held at an angle over 45 degrees, it will sound two beeps (and LED flashes) and shut off after 30 seconds. If that occurs, just hold the Handset level and press the ON/OFF button to start your treatment again.

During your treatment, you can pause the device by pressing the ON/OFF button for 1 second. To resume your treatment, press the ON/OFF button for a full second.

At the end of your treatment: The Lamira will beep 2 times, the dose complete checkmark will appear briefly on the screen, then the Controller will automatically shut off. Always check the Medication Reservoir by removing the Medication Cap to make sure you have completed your dose. If more than a few drops remain, replace the Medication Cap and press the ON/OFF button and complete your dose.

CLEANING AND DISINFECTING AFTER EACH USE

Once you've completed your treatment, remember to rinse and clean the Handset and Aerosol Head immediately after each use. Cleaning and disinfecting your Handset and Aerosol Head is important to reduce the risk of infection, illness, and contamination.

Disconnect your Handset from the Connection Cord. Remove the Medication Cap by turning counterclockwise and pulling straight up. Remove the Mouthpiece from the Aerosol chamber by pulling it straight off. Be sure the Blue Flap is still attached to the Mouthpiece. Gently pull up on the tab to open the Handset and remove the Blue Valve. Being careful to touch only the plastic outer ring of the Aerosol Head, press the 2 flexible plastic tabs on the side of the Aerosol Head towards each other and remove it. Do not touch the center silver part of the Aerosol Head. After the Aerosol Head has been used 7 times, throw it away and use a new one after it has been cleaned and disinfected.

Before you rinse your Handset, gently wipe away any drops of medicine from the Medication Reservoir, Aerosol Chamber, and Mouthpiece. This reduces the risk of antibiotics being added to water systems. Use only plain, dry paper towels or wipes. Do not use towels or wipes that have any chemicals added to them such as alcohol, lotion, or baby wipes. Be careful not to harm the parts. Do not wipe the Aerosol Head. Throw away the paper towels by disposing them in the trash with solid waste.

Prepare a clean tub or bowl by adding a few drops of clear liquid dish soap and warm tap water to it. Do not use white or antibacterial liquid dish soaps. Rinse each of the parts under warm running tap water for about 10 seconds, then immerse them in the warm soapy water. Pay close attention to rinsing the Aerosol Head and rinse each side of it for 10 seconds. Thorough rinsing of both sides of the Aerosol Head helps to prevent clogging and keeps it working properly. Cover the Handset parts and Aerosol Head in the warm soapy water and soak for 5 minutes, shaking them periodically. When 5 minutes are up, rinse all the parts thoroughly under warm running tap water to remove all the soap. Check each part - if anything looks dirty, then let it soak again for another 5 minutes. Once all the parts are cleaned and free of soap, you can move onto the next step - disinfecting the Handset.

You must disinfect your Handset parts and Aerosol Head after every use by boiling them in distilled water. Make sure the pot has enough distilled water to completely cover all the Handset parts including the Aerosol Head. Heat the water to a boil in a clean pot. Then place the Handset parts, including the Aerosol Head, into the boiling water. Boil for a full 5 minutes. It may be helpful to use a timer. Then, air dry the Handset parts and Aerosol Head on a lint-free towel.

After they're completely dry, you can wrap them in a lint-free towel for storage to make sure the Blue Valve is not damaged. Reassemble the parts only just before taking your next treatment.

You can clean the outside of the Controller and the Connection Cord as needed with a soft, clean, damp cloth. To reduce the risk of electric shock, disconnect all connections before cleaning. Do not wash the Controller, Connection Cord, or A/C Power Supply. Do not place the Controller unit under water or allow liquid to get inside the Controller.

I hope you found this video helpful. For frequently asked questions or any troubleshooting, refer to the Instructions for Use that comes with your ARIKAYCE.

Call this number with any questions or concerns you may have. There is always someone to help you along with every step of your treatment journey.