Prescribing, Dosing, and Administering VILTEPSO

00:00: This video guide will demonstrate how to prepare and administer

VILTEPSO (viltolarsen) injection to appropriate patients.

 

00:09: VILTEPSO is indicated for the treatment of Duchenne muscular dystrophy (DMD) in patients who have a confirmed mutation of the DMD gene that is amenable to exon 53 skipping.

 

00:22: This indication is approved under accelerated approval based on an increase in dystrophin production in skeletal muscle observed in patients treated with VILTEPSO. Continued approval for this indication may be contingent upon verification and description of clinical benefit in a confirmatory trial.

 

00:41: The Important Safety Information for VILTEPSO will be reviewed during this video. You can visit www.viltepso.com to view and download the
full Prescribing Information for VILTEPSO
(viltolarsen) injection.

 

00:58: Treatment with VILTEPSO is always provided by a qualified healthcare professional but may
be given in a choice of treatment locations, including the patient’s home or at a treatment center such as a specialty practice, a free-standing infusion center, or a hospital outpatient facility.

 

01:17: VILTEPSO is given as a weekly intravenous infusion. The appropriate dose of VILTEPSO is calculated based upon patient weight, at a recommended weekly dosage of 80 mg/kg.

 

1:29: VILTEPSO is infused over 60 minutes by a healthcare professional. If a dose of VILTEPSO is missed, it should be administered as soon as possible after the scheduled time.

 

01:40: Let’s take a look at how VILTEPSO is supplied, stored, and handled.

 

01:45: VILTEPSO is provided as a solution in single-dose glass vials containing 250 mg/5 mL, 50 mg/mL, viltolarsen for intravenous infusion only.

 

2:00: The solution is clear and
colorless. VILTEPSO vials should be stored at 2 degrees Celsius to 8 degrees Celsius, that’s 36 degrees Fahrenheit to 46 degrees Fahrenheit, until they are ready to be prepared. Do not freeze the vials.

 

02:18: VILTEPSO contains no preservatives. Infusion should begin as soon as
possible, but no more than 5 hours after preparation, and be completed within 6 hours of preparation, allowing for 1 hour of infusion time.

 

02:33: Depending on the dose, VILTEPSO may be diluted with 0.9% sodium chloride solution,
USP, as we’ll demonstrate in a few minutes.

 

02:44: If used within 5 hours of preparation, the diluted solution should be stored at 20 degrees Celsius to 26 degrees Celsius, or 68 degrees Fahrenheit to 79 degrees Fahrenheit.

 

02:57: However, if immediate use is not possible, the solution may be stored for up to 24 hours at 2 degrees Celsius to 8 degrees Celsius, 36 degrees Fahrenheit to 46 degrees Fahrenheit. Do not freeze diluted solution.

 

03:12: So how do you determine the correct dose for your patient?

 

03:17: Each vial contains 250 mg/5 mL, or 50 mg/mL, of VILTEPSO. The proper dose of VILTEPSO is personalized to
the patient’s weight in kg and is
based on

a recommended dosage regimen of 80 mg/kg/week.

 

03:36: It is infused intravenously once per week. You will need to weigh the patient during an earlier
appointment in order to calculate dosing so that the proper amount of VILTEPSO can be ordered for an infusion at a later date.

 

03:52: It is also important to weigh the patient at the time of infusion to make sure nothing has changed. If the patient has gained or lost weight, you should recalculate to ensure the
patient receives the proper dose.

 

04:04: You can also get the proper dose by referring to the quick guide for the dosing of VILTEPSO, available from your VILTEPSO representative, but let’s go

through the calculations so you have a better understanding of how we arrive at the right dose.

 

04:19: Let’s look at an example. This example is for demonstration purposes

only. VILTEPSO dose is personalized based on the patient’s weight at the time of infusion. We’ll calculate the weekly dose of VILTEPSO for a
patient who weighs 35 kg, about 77 pounds.

 

04:38: The first thing we’ll do
is determine the total amount of VILTEPSO that is needed based on the patient’s weight.

 

04:44: Then, we’ll need to determine the number of 5 mL vials we’ll need to order for the patient in this example. The first step is to weigh the patient.

 

04:53: In this example, our patient weighs 35 kg. Next, calculate the patient’s dose
in mg based on the recommended dosage of 80 mg/kg.

 

05:05: So, 35 kg X 80 mg/kg. That is
35 X 80 = 2800 mg needed per
infusion.

 

05:16: Now, we know that the product concentration of VILTEPSO is 50 mg/mL, so we divide our
2800 total mg required by 50 to get the total mL needed.
2800/50=56. So, 56 mL of VILTEPSO is the required dose for this infusion.

 

 

06:12: In this example, 12 vials
of VILTEPSO should be ordered. The
extra 0.8 of a vial will not be used and will be discarded.

 

06:22: Now that you understand how to calculate the proper infusion
dose, let’s talk about how to prepare VILTEPSO for infusion.

 

06:29: It’s good practice to wait until the patient arrives for the infusion before preparing VILTEPSO. That way you can be sure you have prepared the

proper dose and you will not have wasted VILTEPSO if the patient does not show up for their appointment.

 

06:43: When the patient arrives, check their weight. If the patient’s weight has changed, this may affect the amount of VILTEPSO you will use.

 

06:53: Remove VILTEPSO from the refrigerator and check
the expiration date on all the vials. Do not use any vials after
the expiration date has been
reached. Allow the vials to warm to room temperature.

 

07:08: Mix the contents of each vial by gently inverting 2 to 3 times. Do not shake the vials.

 

07:16: Also, make sure to visually inspect each vial of VILTEPSO. VILTEPSO is a clear and colorless solution. Do not use if the solution in the vials is discolored or particulate matter is present.

 

07:31: The instructions for preparing the infusion solution differ depending on whether you are using less than 20 vials, or 20 or more vials.

 

07:41: First, we’ll go over the process for preparation when you are using less than 20 vials. Let’s take the 35 kg patient as an example.

 

07:51: In the previous section,
we determined that a 35 kg patient would require 11.2 vials of VILTEPSO. That comes to 56 mL of VILTEPSO.

 

08:04: This is important. When the number of vials is less than 20, the VILTEPSO infusion is always done with a volume of 100 mL.

 

08:14: Since a VILTEPSO infusion uses
a 100 mL bag of 0.9 sodium chloride solution, USP, you must withdraw 56
mL of the saline solution from the bag to make room for the 56 mL of VILTEPSO you will be injecting into the bag.

 

08:32: This keeps the total solution volume at 100 mL. Remember to clean the port of the 100 mL IV bag with
an alcohol swab before removing or injecting solution. Now, clean the top of the vials with an alcohol swab.

 

08:49: Then, withdraw the proper amount of VILTEPSO from the vials based on the recommended weight-based dosing and inject into the infusion bag so that the total volume in the bag is 100 mL.

 

09:03: Do not mix or dilute with other drugs. Slow dispensing is important to minimize foaming.

 

09:10: When complete, you should have used the contents of 11 vials, plus 20% of the 12th vial. Visually inspect the infusion bag containing the solution
for particulates.

 

09:23: Gently invert the infusion bag to ensure equal distribution of product.
Do not shake. Vials of VILTEPSO are single-use vials and do
not contain preservatives. Any unused solution in the vials must be discarded.

 

09:40: When preparing for an infusion that will use 20 or more vials of VILTEPSO, you will begin with an empty 100 mL infusion bag. That’s because 25 mL vials is equal
to 100 mL of
VILTEPSO, which is the indicated infusion volume.

 

09:58: Of course, if you need to use more than 20 vials, the total volume will be greater than 100 mL, which is
okay. The preparation is similar to the previous instructions.

 

10:09: Withdraw the proper amount of VILTEPSO from the vials. Inject slowly into the infusion bag to minimize foaming and do not mix or dilute with other drugs.

 

10:21: When complete, visually inspect the infusion bag for particulates and do not shake the bag, and remember, any unused solution in the vials must
be discarded.

 

 

10:43: VILTEPSO should be intravenously infused using a peripheral or central venous catheter over a period of 60 minutes. Filtration of VILTEPSO is not required.

 

10:54: Do not administer as
an intravenous push or bolus and do
not infuse VILTEPSO concomitantly with other agents in the same access line.

 

11:04: If an infusion reaction occurs, interrupt or slow the rate of infusion and use appropriate medical management. Observe the patient during the infusion and for an appropriate time following infusion.

 

11:18: When the infusion is complete, flush the patient’s intravenous access line with 0.9% sodium chloride, and discard all materials in an appropriate manner.

 

11:30: Important Safety Information for VILTEPSO injection. Warnings and Precautions. Kidney toxicity
was observed in animals
who received viltolarsen.

 

11:40 Although kidney toxicity was
not observed in the clinical studies with VILTEPSO, the clinical experience with VILTEPSO is limited, and kidney
toxicity, including potentially fatal glomerulonephritis, has
been observed after administration
of some antisense oligonucleotides.

 

11:59: Kidney function should
be monitored in patients taking VILTEPSO. Serum creatinine may not be a reliable measure of kidney function in DMD patients.

 

12:10: Serum cystatin C, urine dipstick, and urine protein-to-creatinine ratio should be measured before starting VILTEPSO. Consider also
measuring glomerular filtration rate before starting VILTEPSO.

 

12:23: During treatment, monitor urine dipstick every month, and serum cystatin C and urine protein-to-creatinine ratio every three months.

 

12:33: Urine should be free of excreted VILTEPSO for monitoring of urine protein. Obtain urine either prior to VILTEPSO infusion, or at least 48
hours after the most recent infusion.

 

12:46: Alternatively, use a laboratory test that does not use the reagent pyrogallol red, which has the potential to generate a false positive result due to cross reaction with any VILTEPSO in the urine.

 

12:59: If a persistent increase in serum cystatin C or proteinuria is detected, refer to a pediatric nephrologist for further evaluation.

 

13:10: Adverse reactions. The most common adverse reactions include upper respiratory tract infection, injection site reaction, cough, and pyrexia.

 

13:21: Please see additional Important Safety Information throughout. For more information about VILTEPSO, see full Prescribing Information at www.viltepso.com/prescribing-information.