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Overview: Avlayah is used to treat certain symptoms of Hunter syndrome in children. It works by helping the body break down certain sugars to keep them from building up and causing damage. Common side effects include reactions during or after the infusion, infections like colds or ear infections, fever, rash, and stomach problems such as vomiting or diarrhea. Serious allergic reactions are possible. Get help right away if you have trouble breathing, swelling of your lips or tongue, dizziness, unusual tiredness, or changes in your urine.

Uses

What is Avlayah used for?

Avlayah (tividenofusp alfa-eknm) is commonly used for treating brain and nervous system problems in children with Hunter syndrome, also called mucopolysaccharidosis II (MPS II). It is a rare inherited condition that mainly affects boys.

What to Know About Avlayah for Hunter Syndrome

Avlayah may also be used for other conditions as determined by your health care provider.

How does Avlayah work (mechanism of action)?

In people with Hunter syndrome, the body is lacking or does not properly make an enzyme needed to break down certain sugars. As a result, these sugars build up and cause damage in different parts of the body. Avlayah works by replacing this enzyme, which may help the body break down and clear the buildup.

How is Avlayah supplied (dosage forms)?

Avlayah is available in an injectable form that is given by a health care provider. 

How should I store Avlayah?

Avlayah is usually given by a health care provider in a hospital or clinic. If you will store Avlayah at home, follow the instructions given by your health care provider.

Side Effects

What are the most common side effects of Avlayah?

The most common side effects of Avlayah are listed below. Tell your health care provider if you have any of these side effects that bother you.

  • Infusion reactions (see below)
  • Infection in the nose, throat, or ears, or common cold symptoms, such as runny or stuffy nose
  • Fever
  • Anemia (see below)
  • Cough
  • Vomiting
  • Diarrhea
  • Rash
  • Falls
  • Headache

There may be other side effects of Avlayah that are not listed here. Contact your health care provider if you think you are having a side effect of a medicine. In the U.S., you can report side effects to the FDA at www.fda.gov/medwatch or by calling 800-FDA-1088 (800-332-1088). In Canada, you can report side effects to Health Canada at www.health.gc.ca/medeffect or by calling 866-234-2345.

What are the serious side effects of Avlayah?

While less common, the most serious side effects of Avlayah are described below, along with what to do if they happen.

Severe Allergic Reactions. Avlayah may cause allergic reactions, which can be serious or even life-threatening. Allergic reactions can happen at any time during treatment with Avlayah, including when you first start treatment and after you have been on Avlayah for a while. Your health care provider may give you medicines before you receive Avlayah to help prevent allergic reactions. Get help right away if you have any of the following symptoms of a serious allergic reaction.

  • Breathing problems or wheezing
  • Racing heart
  • Fever or general ill feeling
  • Swollen lymph nodes
  • Swelling of the face, lips, mouth, tongue, or throat
  • Trouble swallowing or throat tightness
  • Itching or skin rash
  • Bumps on the skin called hives that can be red, pink, white, or brown depending on your skin tone
  • Nausea or vomiting
  • Dizziness, feeling lightheaded, or fainting
  • Stomach cramps
  • Joint pain

Infusion Reactions. Serious reactions can happen while you are receiving Avlayah or within 24 hours after you receive the medicine. Your health care provider may give you medicines before you receive Avlayah to help prevent infusion reactions. Your infusion may need to be slowed or stopped if you have a reaction during treatment. Tell your health care provider right away if you have any of the following symptoms during or after your infusion.

  • Fever or chills
  • Rash, hives, or swelling of your face or lips
  • Trouble breathing, wheezing, or cough
  • Flushing, which may cause warmth, sweating, or reddening of the face and neck
  • Fast heartbeat
  • Feeling dizzy or lightheaded
  • Nausea, vomiting, diarrhea, or stomach pain
  • Headache or irritability

Anemia. Avlayah may cause anemia, which is when you have low levels of red blood cells. Your risk may be higher if your red blood cells are low before you start Avlayah. Keep all appointments for blood tests before starting and while using Avlayah. Your health care provider will let you know if you should take an iron supplement to manage anemia. Tell your health care provider if you have any of the following symptoms of anemia.

  • Unusual weakness or tiredness
  • Cold hands and feet
  • Fast or abnormal heartbeat
  • Pale or yellowish skin 
  • Dizziness, lightheadedness, or feeling like you are about to pass out
  • Shortness of breath

Kidney Problems. A serious kidney problem called membranous nephropathy has been reported during treatment with Avlayah. Your health care provider may do blood or urine tests to check your kidneys during treatment. Tell your health care provider right away if you have any of the following symptoms.

  • Swelling in your legs, ankles, or feet
  • Foamy or bubbly urine
  • Changes in how much you pee
  • Unusual tiredness

Warnings & Precautions

Who should not use Avlayah?

Allergies to Ingredients. People who are allergic to any of the following should not use Avlayah.

  • Avlayah
  • Tividenofusp alfa
  • Any of the ingredients in the specific product dispensed

Your pharmacist can tell you all of the ingredients in Avlayah.

What should I know about Avlayah before using it?

Avlayah is usually given as an infusion into a vein in a hospital or clinic. If you are able to safely tolerate the medicine, it may be given as a home infusion by a health care provider. Your health care provider will decide if this option is right for you.

It is important that you keep all of your appointments, including lab tests, while using Avlayah. This helps your health care provider check for side effects.

What should I tell my health care provider before using Avlayah?

Tell your health care provider about all of your health conditions and any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using. This will help them know if Avlayah is right for you.

In particular, make sure that you discuss any of the following.

Pregnancy. It is not known if or how Avlayah could affect pregnancy or harm a fetus. Tell your health care provider if you are pregnant or plan to become pregnant.

Breastfeeding. It is not known if Avlayah passes into breast milk. Tell your health care provider if you are breastfeeding or plan to breastfeed. Your health care provider will let you know if you should use Avlayah while breastfeeding.

How can I get more information about Avlayah?

For more information about Avlayah, you can visit the drugmaker’s website at www.avlayah.com or call them at 844-365-4365.

Interactions

Does Avlayah interact with foods or drinks?

There are no known interactions between Avlayah and foods or drinks.

It is unknown if drinking alcohol will affect Avlayah.

Does Avlayah interact with other medicines (drug interactions)?

Always tell your health care provider about any prescription or over-the-counter (OTC) medicines, vitamins/minerals, herbal products, and other supplements you are using.

In particular, make sure that you discuss if you are using any other enzyme replacement medicines.

This may not be a complete list of medicines that can interact with Avlayah. Always check with your health care provider.

Does interact with other drugs you are taking?

Enter your medication into the WebMD interaction checker

Overdose/Missed Dose

What should I do if I accidentally use too much Avlayah?

If you or someone else has used too much Avlayah, get medical help right away, call 911, or contact a Poison Control center at 800-222-1222.

What should I do if I miss a dose of Avlayah?

If you miss a dose of Avlayah or miss an appointment to get your dose, contact your health care provider as soon as possible.