See why thousands of patients choose our centers as their preferred destination for ongoing care, including Pombiliti Infusion treatments.
Pombiliti is a biologic therapy that is indicated to treat:
- Adults with late-onset Pompe disease weighing 88 pounds (40 kg) or more and who are not improving on their current enzyme replacement therapy (ERT)
What is Pombiliti?
What Is Pombiliti?
Pombiliti (cipaglucosidase alfa-atga) and Opfolda (miglustatare) are prescription medicines used in combination for the treatment of adults with late-onset Pompe disease weighing 88 pounds (40 kg) or more and who are not improving on their current enzyme
replacement therapy (ERT).
How Pombiliti Works
Patients with late-onset Pompe disease (LOPD) lack or have a deficiency of the enzyme that breaks down glycogen in muscle cells, causing the glycogen to build up and result in muscle damage.
Pombiliti replaces the missing or deficient enzyme. It is taken up by muscle cells, where it helps break down the excess glycogen that accumulates and causes muscle damage.
Pombiliti Infusion Dosage & Administration
Pombiliti Infusion Dosage and Administration
Like other LOPD treatments, POMBILITI + OPFOLDA therapy is given every 2 weeks. For OPFOLDA to become optimally absorbed and functional inside your bloodstream, it must be taken while you are fasting.
POMBILITI must be taken in combination with OPFOLDA. Because POMBILITI + OPFOLDA were designed to work exclusively with each other, they must be taken at the right time under the right circumstances. Neither should be taken with another Pompe treatment.
Dosage and Frequency
POMBILITI + OPFOLDA dosing is determined by your actual body weight. You will take 3 or 4 OPFOLDA capsules depending on your weight.* Your doctor may recommend taking medicine such as an antihistamine, antipyretic (fever reducer), or corticosteroid before your POMBILITI infusion, especially if you took a medication with your previous ERT. Always take your medication exactly as directed by your healthcare provider.
Administering Pombiliti via IV Infusion
- The nurse prepares the patient’s arm by using rubbing alcohol to sterilize the location that is being used to insert the IV needle.
- Following needle insertion, the IV line is held in place with tape.
- The nurse attaches the patient’s IV line to the line coming down from the bag of medication.
Every half-an-hour, the patient’s nurse takes his or her vitals.
Pombiliti Side Effects
Pombiliti Infusion Side Effects
As with all medications, side effects are possible.
Common side effects include:
- Headache
- Fatigue
- Diarrhea
- Nausea
- Joint pain
- Dizziness
- Muscle pain
- Itching
- Vomiting
- Shortness of breath
- Rash or hives
- Tingling sensation
Preparing for Your Pombiliti Infusion
Follow these steps to prepare for your Pombiliti Infusion:
Consult Your Healthcare Provider
- Medical History Review: Ensure your healthcare provider has a complete and up-to-date medical history, including any allergies, current medications, and past reactions to Infusion.
- Pre-Infusion Testing: Complete any required blood tests or other pre-Infusion tests as recommended by your healthcare provider.
Pre-Infusion Medications
- Prescription Pre-Medications: Your Pombiliti Infusion may require pre-medications to reduce the risk of a reaction or adverse event. Your referring provider will include any pre-medications when sending in your prescription order.
- Instructions: Follow your healthcare provider’s instructions regarding these medications, including the timing and dosage. Take these medications about 30-60 minutes before the Infusion as directed.
Hydration and Nutrition
- Hydrate: Drink plenty of water in the days leading up to your Infusion to ensure you are well-hydrated.
- Eat Light: Have a light meal before your Infusion to help maintain your energy levels during the procedure.
Prepare for Comfort
- Clothing: Wear loose, comfortable clothing with easy access to your arms for the Infusion site.
- Entertainment: Bring something to keep you occupied, such as a book, tablet, or music, as your treatment or observation period may take several hours.
Plan for Transportation
- Arrange a Ride: Depending on the medication and your reaction to the Infusion, you might not feel well enough to drive yourself home. Consider arranging for someone to drive you or use a ride service.
By following these steps, you can help ensure that your Pombiliti Infusion is as safe and comfortable as possible. Always follow the specific instructions provided by your healthcare team.
How to Get a Referral for Pombiliti
Every Pombiliti Infusion referral must be accompanied by a physician’s order for treatment.
Patient information should include:
- Name
- Current address
- Home and mobile phone number
- Insurance information
Information the physician must include on the patient’s order for therapy:
- Patient’s name and DOB
- Order date
- Diagnosis
- Physician’s name and NPI#
- Office address, phone, and fax numbers
- Provider’s signature
Clinical documentation from the physician should include information to support a diagnosis for one or more of the following:
- Adults with late-onset Pompe disease weighing 88 pounds (40 kg) or more and who are not improving on their current enzyme replacement therapy (ERT)
If you or someone you care about has one of the conditions that Pombiliti treats, IVX Health would be honored to provide your care. To find an IVX Health center near you, please click here.
Patients choose IVX Health for ongoing Pombiliti treatments because:
- ALL private suites
- Comfortable recliners, with chairs for guests
- Evening & weekend appointments
- Friendly clinical staff deliver personal service
- Big screen TVs, high-speed WiFi, free snacks
Interested in moving your care to IVX Health?
Interested?
IVX Health would love to learn more about your care needs. If you’re interested in switching your infusion services – or if you just want to learn more – let us know.
