Evrysdi

EVRYSDI is indicated for the treatment of spinal muscular atrophy (SMA) in pediatric and adult patients.

• Administer once daily with or without food per the table below (
  2.1 Dosing Information

  EVRYSDI is administered orally once daily with or without food. The recommended dosage is determined by age and body weight (see Table 1). EVRYSDI tablets are available for patients prescribed the 5 mg dose.

  Table 1 Adult and Pediatric Dosing Regimen by Age and Body Weight

  Age and Body Weight	Recommended Daily Dosage	Dosage Form
  Less than 2 months of age	0.15 mg/kg	EVRYSDI for Oral Solution
  2 months to less than 2 years of age	0.2 mg/kg
  2 years of age and older weighing less than 20 kg	0.25 mg/kg
  2 years of age and older weighing 20 kg or more	5 mg	EVRYSDI for Oral Solution or EVRYSDI Tablet
  ):

• Swallow EVRYSDI tablet whole with water or dispersed in non-chlorinated drinking water (e.g., filtered water). (
  2.2 Important Administration Instructions

  It is recommended that a healthcare provider discuss with the patient or caregiver how to prepare the prescribed daily dose prior to administration of the first dose

  [see Instructions for Use for EVRYSDI for Oral Solutionand Patient Information].

  EVRYSDI is taken orally once daily with or without food at approximately the same time each day.

  EVRYSDI for Oral Solution

  In infants who are breastfed, EVRYSDI for oral solution can be administered before or after breastfeeding. EVRYSDI cannot be mixed with formula or milk.

  Instruct patients or caregivers to administer the dose using the reusable oral syringe provided.

  EVRYSDI for oral solution must be taken immediately after it is drawn up into the oral syringe. If EVRYSDI is not taken within 5 minutes, EVRYSDI should be discarded from the oral syringe, and a new dose should be prepared.

  Instruct patients to drink water after taking EVRYSDI for oral solution to ensure the drug has been completely swallowed.

  If the patient is unable to swallow and has a nasogastric or gastrostomy tube, EVRYSDI for oral solution can be administered via the tube. The tube should be flushed with water after delivering EVRYSDI for oral solution

  [see Instructions for Use].

  EVRYSDI Tablets

  Swallow EVRYSDI tablets whole with water. Do not chew, cut, or crush the tablets.

  The EVRYSDI tablet can also be dispersed in one teaspoon (5 mL) of room temperature non-chlorinated drinking water (e.g., filtered water). EVRYSDI tablets must not be dispersed in any liquid other than non-chlorinated drinking water. Do not expose the prepared dispersion to sunlight. Swirl the small cup gently for up to 3 minutes until fully mixed (though some particles will remain). Administer the dispersed tablet immediately. To ensure no particles are left in the small cup, refill it with at least one tablespoon (15 mL) of non-chlorinated drinking water, swirl, and administer immediately again.

  EVRYSDI must be taken immediately after it is dispersed in non-chlorinated drinking water. Discard the prepared dispersion if it is not used within 10 minutes of adding non-chlorinated drinking water.

  Do not administer the prepared dispersion via a nasogastric or gastrostomy tube. If administration through a nasogastric or gastrostomy tube is required, EVRYSDI for oral solution should be used.
  )
• Administer EVRYSDI for oral solution with the provided oral syringe. (
  2.2 Important Administration Instructions

  It is recommended that a healthcare provider discuss with the patient or caregiver how to prepare the prescribed daily dose prior to administration of the first dose

  [see Instructions for Use for EVRYSDI for Oral Solutionand Patient Information].

  EVRYSDI is taken orally once daily with or without food at approximately the same time each day.

  EVRYSDI for Oral Solution

  In infants who are breastfed, EVRYSDI for oral solution can be administered before or after breastfeeding. EVRYSDI cannot be mixed with formula or milk.

  Instruct patients or caregivers to administer the dose using the reusable oral syringe provided.

  EVRYSDI for oral solution must be taken immediately after it is drawn up into the oral syringe. If EVRYSDI is not taken within 5 minutes, EVRYSDI should be discarded from the oral syringe, and a new dose should be prepared.

  Instruct patients to drink water after taking EVRYSDI for oral solution to ensure the drug has been completely swallowed.

  If the patient is unable to swallow and has a nasogastric or gastrostomy tube, EVRYSDI for oral solution can be administered via the tube. The tube should be flushed with water after delivering EVRYSDI for oral solution

  [see Instructions for Use].

  EVRYSDI Tablets

  Swallow EVRYSDI tablets whole with water. Do not chew, cut, or crush the tablets.

  The EVRYSDI tablet can also be dispersed in one teaspoon (5 mL) of room temperature non-chlorinated drinking water (e.g., filtered water). EVRYSDI tablets must not be dispersed in any liquid other than non-chlorinated drinking water. Do not expose the prepared dispersion to sunlight. Swirl the small cup gently for up to 3 minutes until fully mixed (though some particles will remain). Administer the dispersed tablet immediately. To ensure no particles are left in the small cup, refill it with at least one tablespoon (15 mL) of non-chlorinated drinking water, swirl, and administer immediately again.

  EVRYSDI must be taken immediately after it is dispersed in non-chlorinated drinking water. Discard the prepared dispersion if it is not used within 10 minutes of adding non-chlorinated drinking water.

  Do not administer the prepared dispersion via a nasogastric or gastrostomy tube. If administration through a nasogastric or gastrostomy tube is required, EVRYSDI for oral solution should be used.
  )
• EVRYSDI for oral solution must be constituted by a healthcare provider prior to dispensing. (
  2.4 Preparation of Powder for Oral Solution by Healthcare Provider

  EVRYSDI powder must be constituted to the oral solution by a pharmacist or other healthcare provider prior to dispensing to the patient.

  Preparation of the EVRYSDI Oral Solution 0.75 mg/mL

  The EVRYSDI " Instructions for Constitution" booklet contains more detailed instructions on the preparation of the oral solution

  [see Instructions for Constitution]

  .

  Caution should be exercised when handling EVRYSDI powder for oral solution

  .

  Avoid inhalation and direct contact with skin or mucous membranes with the dry powder and the constituted solution. If such contact occurs, wash thoroughly with soap and water; rinse eyes with water. Wear disposable gloves during the preparation and cleanup procedure.

  • Gently tap the bottom of the closed glass bottle to loosen the powder.
  • Remove the cap. Do not throw away the cap.
  • Carefully pour 79 mL of purified water into the EVRYSDI bottle to yield the 0.75 mg/mL oral solution. Do not mix EVRYSDI with formula or milk.
  • Insert the press-in bottle adapter into the bottle opening by pushing it down against the bottle lip. Ensure it is completely pressed against the bottle lip.
  • Re-cap the bottle tightly and shake well for 15 seconds. Wait for 10 minutes. You should have obtained a clear solution. If not, shake well again for another 15 seconds or until you have obtained a clear solution.
  • Write the date of expiration of the constituted oral solution (calculated as 64 days after constitution) and the lot number on the bottle label. Peel off the part of the bottle label that has the expiration date of the powder.
  • Put the bottle back in its original carton.
  • Select the appropriate oral syringes (1 mL, 6 mL, or 12 mL) based on the patient's dosage and remove the other oral syringes from the carton.
  • Dispense with the " Instructions for Use" and FDA-approved patient labeling. Alert patients to read the important handling information described in the Instructions for Use.

  Storage

  Keep the constituted oral solution of EVRYSDI in the original amber bottle to protect from light. Store in a refrigerator at 2°C to 8°C (36°F to 46°F). Do not freeze. Discard any unused portion 64 days after constitution. Keep the bottle in an upright position with the cap tightly closed. If refrigeration is not available, EVRYSDI can be kept at room temperature up to 40°C (up to 104°F) for a combined total of 5 days. EVRYSDI can be removed from, and returned to, a refrigerator. The total combined time out of refrigeration should not exceed 5 days.
  )
• See Full Prescribing Information for important preparation and administration instructions. (
  2.2 Important Administration Instructions

  It is recommended that a healthcare provider discuss with the patient or caregiver how to prepare the prescribed daily dose prior to administration of the first dose

  [see Instructions for Use for EVRYSDI for Oral Solutionand Patient Information].

  EVRYSDI is taken orally once daily with or without food at approximately the same time each day.

  EVRYSDI for Oral Solution

  In infants who are breastfed, EVRYSDI for oral solution can be administered before or after breastfeeding. EVRYSDI cannot be mixed with formula or milk.

  Instruct patients or caregivers to administer the dose using the reusable oral syringe provided.

  EVRYSDI for oral solution must be taken immediately after it is drawn up into the oral syringe. If EVRYSDI is not taken within 5 minutes, EVRYSDI should be discarded from the oral syringe, and a new dose should be prepared.

  Instruct patients to drink water after taking EVRYSDI for oral solution to ensure the drug has been completely swallowed.

  If the patient is unable to swallow and has a nasogastric or gastrostomy tube, EVRYSDI for oral solution can be administered via the tube. The tube should be flushed with water after delivering EVRYSDI for oral solution

  [see Instructions for Use].

  EVRYSDI Tablets

  Swallow EVRYSDI tablets whole with water. Do not chew, cut, or crush the tablets.

  The EVRYSDI tablet can also be dispersed in one teaspoon (5 mL) of room temperature non-chlorinated drinking water (e.g., filtered water). EVRYSDI tablets must not be dispersed in any liquid other than non-chlorinated drinking water. Do not expose the prepared dispersion to sunlight. Swirl the small cup gently for up to 3 minutes until fully mixed (though some particles will remain). Administer the dispersed tablet immediately. To ensure no particles are left in the small cup, refill it with at least one tablespoon (15 mL) of non-chlorinated drinking water, swirl, and administer immediately again.

  EVRYSDI must be taken immediately after it is dispersed in non-chlorinated drinking water. Discard the prepared dispersion if it is not used within 10 minutes of adding non-chlorinated drinking water.

  Do not administer the prepared dispersion via a nasogastric or gastrostomy tube. If administration through a nasogastric or gastrostomy tube is required, EVRYSDI for oral solution should be used.
  ,
  2.4 Preparation of Powder for Oral Solution by Healthcare Provider

  EVRYSDI powder must be constituted to the oral solution by a pharmacist or other healthcare provider prior to dispensing to the patient.

  Preparation of the EVRYSDI Oral Solution 0.75 mg/mL

  The EVRYSDI " Instructions for Constitution" booklet contains more detailed instructions on the preparation of the oral solution

  [see Instructions for Constitution]

  .

  Caution should be exercised when handling EVRYSDI powder for oral solution

  .

  Avoid inhalation and direct contact with skin or mucous membranes with the dry powder and the constituted solution. If such contact occurs, wash thoroughly with soap and water; rinse eyes with water. Wear disposable gloves during the preparation and cleanup procedure.

  • Gently tap the bottom of the closed glass bottle to loosen the powder.
  • Remove the cap. Do not throw away the cap.
  • Carefully pour 79 mL of purified water into the EVRYSDI bottle to yield the 0.75 mg/mL oral solution. Do not mix EVRYSDI with formula or milk.
  • Insert the press-in bottle adapter into the bottle opening by pushing it down against the bottle lip. Ensure it is completely pressed against the bottle lip.
  • Re-cap the bottle tightly and shake well for 15 seconds. Wait for 10 minutes. You should have obtained a clear solution. If not, shake well again for another 15 seconds or until you have obtained a clear solution.
  • Write the date of expiration of the constituted oral solution (calculated as 64 days after constitution) and the lot number on the bottle label. Peel off the part of the bottle label that has the expiration date of the powder.
  • Put the bottle back in its original carton.
  • Select the appropriate oral syringes (1 mL, 6 mL, or 12 mL) based on the patient's dosage and remove the other oral syringes from the carton.
  • Dispense with the " Instructions for Use" and FDA-approved patient labeling. Alert patients to read the important handling information described in the Instructions for Use.

  Storage

  Keep the constituted oral solution of EVRYSDI in the original amber bottle to protect from light. Store in a refrigerator at 2°C to 8°C (36°F to 46°F). Do not freeze. Discard any unused portion 64 days after constitution. Keep the bottle in an upright position with the cap tightly closed. If refrigeration is not available, EVRYSDI can be kept at room temperature up to 40°C (up to 104°F) for a combined total of 5 days. EVRYSDI can be removed from, and returned to, a refrigerator. The total combined time out of refrigeration should not exceed 5 days.
  )

EVRYSDI for oral solution: 60 mg as a light yellow, pale yellow, yellow, greyish yellow, greenish yellow, or light green powder for constitution. Following constitution, the volume of the greenish yellow to yellow solution is 80 mL, providing 60 mg/80 mL (0.75 mg/mL) risdiplam.

EVRYSDI tablet: 5 mg as a pale yellow film-coated tablet, round and curved, with EVR debossed on one side.

Pregnancy: Based on animal data, may cause fetal harm. (