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WHAT IS ADA-SCID?

People with ADA-SCID have a deficiency in the ADA enzyme. ADA stands for adenosine deaminase, and SCID stands for severe combined immunodeficiency. Those are the medical terms—but what do they actually mean? 

Here’s what goes on inside the body of someone with ADA-SCID:

Every day, cells divide in your body. This is a normal part of life for all people.

The dividing process creates leftover material that your body doesn’t need. ADA helps to remove this leftover material.

But in ADA-SCID, ADA doesn’t work correctly, so it can’t remove the leftover material. As that material builds up, it leads to deoxyadenosine nucleotide (dAXP), a toxic material. 

dAXP damages and removes immune cells called lymphocytes—key tools that your body needs to fight off infections.

DOWNLOAD A FACT SHEET ABOUT ADA-SCID

When ADA can’t do its job, neither can the immune system. This leads to common signs of ADA-SCID:

Signs and symtpoms

aSome lung issues in ADA-SCID, as well as in other types of severe combined immune deficiency (SCID), may be caused by problems with the metabolic system, not just infections.

If left untreated, ADA-SCID usually becomes life-threatening before the age of 2 years.

IT’S CRUCIAL FOR DOCTORS TO DIAGNOSE ADA-SCID AND START TREATMENT AS EARLY AS POSSIBLE.

DIAGNOSING ADA-SCID

Learn the signs and symptoms of ADA-SCID and the role of newborn screening in diagnosis.

Important Safety Information

Warnings and Precautions:

  • If you have lower than normal platelet counts, you may be at increased risk of bleeding at the injection site. Your doctor should not prescribe Revcovi if you have severely low blood platelet counts.
  • There may be a delay in improvement of your immune system’s function after starting Revcovi treatment. During this delay, it is important to be protected from exposure to infections until your immune system improves.

What are the possible side effects of Revcovi?

  • Cough and vomiting were the most common side effects reported by patients receiving Revcovi.
  • Based on reports of side effects of other enzyme replacement therapies used to treat ADA-SCID, which are similar to Revcovi, patients taking Revcovi may also experience:
    • Blood and blood cell–related problems: red blood cells being destroyed faster than they can be made, resulting in anemia; and higher or lower than normal number of platelets in the blood
    • Skin-related problems: redness and itching at the injection site
    • Cancers of the immune system called lymphomas

What types of monitoring will be needed while I am taking Revcovi?

  • Continuous therapy and adherence to the recommended drug schedule is important for the success of the treatment.
  • While you are being treated with Revcovi, your doctor will need to do blood tests to monitor the levels of ADA activity and metabolites and overall immune function.
  • This monitoring will ensure you are maintaining appropriate levels for therapeutic benefit. If an ongoing decline in your blood’s measured adenosine deaminase (ADA) activity occurs, your immune function and clinical status will be monitored closely and precautions will be taken to help reduce your risk of infection.

What is known about taking Revcovi during pregnancy?

  • If you are pregnant or planning to become pregnant, or breastfeeding or planning to breastfeed, please talk to your doctor.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

Indication

Revcovi® (elapegademase-lvlr) is indicated for the treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) in pediatric and adult patients.

Please see the Full Prescribing Information.

Important Safety Information

Warnings and Precautions:

  • If you have lower than normal platelet counts, you may be at increased risk of bleeding at the injection site. Your doctor should not prescribe Revcovi if you have severely low blood platelet counts.
  • There may be a delay in improvement of your immune system’s function after starting Revcovi treatment. During this delay, it is important to be protected from exposure to infections until your immune system improves.

What are the possible side effects of Revcovi?

  • Cough and vomiting were the most common side effects reported by patients receiving Revcovi.
  • Based on reports of side effects of other enzyme replacement therapies used to treat ADA-SCID, which are similar to Revcovi, patients taking Revcovi may also experience:
    • Blood and blood cell–related problems: red blood cells being destroyed faster than they can be made, resulting in anemia; and higher or lower than normal number of platelets in the blood
    • Skin-related problems: redness and itching at the injection site
    • Cancers of the immune system called lymphomas

What types of monitoring will be needed while I am taking Revcovi?

  • Continuous therapy and adherence to the recommended drug schedule is important for the success of the treatment.
  • While you are being treated with Revcovi, your doctor will need to do blood tests to monitor the levels of ADA activity and metabolites and overall immune function.
  • This monitoring will ensure you are maintaining appropriate levels for therapeutic benefit. If an ongoing decline in your blood’s measured adenosine deaminase (ADA) activity occurs, your immune function and clinical status will be monitored closely and precautions will be taken to help reduce your risk of infection.

What is known about taking Revcovi during pregnancy?

  • If you are pregnant or planning to become pregnant, or breastfeeding or planning to breastfeed, please talk to your doctor.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

Indication

Revcovi® (elapegademase-lvlr) is indicated for the treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) in pediatric and adult patients.

Please see the Full Prescribing Information.

References available upon request.

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