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Alpelisib (Vijoice, Piqray)

These instructions are for Children’s Hospital of Philadelphia (CHOP) patients prescribed Alpelisib.

Why does my child need this medicine?

Your child has been diagnosed with a condition that is caused by a mutation (change) in the PIK3CA gene.
The medicine that your provider has prescribed is Alpelisib.
Alpelisib helps treat vascular malformations and overgrowth syndromes caused by changes in the PIK3CA gene

Your child has a complicated vascular anomaly that needs medical treatment.
They have been diagnosed with PROS (PIK3CA- Related Overgrowth Syndrome). This is a group of conditions caused by a PIK3CA gene mutation. PROS includes diagnoses of CLOVES, KTS, FAVA, M-CM, and many others. Currently, there are no other treatments available that we believe will be as helpful to your child’s condition as Alpelisib.
Alpelisib is also used to treat complicated venous malformations which can be caused by PIK3CA or TIE2 mutations.

Alpelisib was first approved as Piqray medication to treat advanced or metastatic breast cancer when there is a PIK3CA mutation.
It was FDA approved in 2021 as Vijoice for use in pediatric patients with PIK3CA Related Overgrowth Syndrome (PROS)

Alpelisib helps blood vessels (mainly veins and lymphatics) mature to function more normally.

Alpelisib comes as an oral tablet. Your child will take it by mouth. It may be crushed.
Give it every day, at the same time, with food.
Your child’s dose (number of tablets) will be based on your child’s age, tolerance, and extent of disease.
Your child’s dose will come from a specialty pharmacy that is contracted with your insurance. This information will be relayed to you by our team and the pharmacy.
If a dose is missed, you may administer the dose as soon as remembered and the next dose is at least 12 hours away.

Do not allow your child to drink grapefruit juice while they are taking this medicine. Grapefruit juice could potentially interact with Alpelisib and cause it to not work as well.
Talk to your healthcare provider about all other medicines your child is taking, including prescription, over-the-counter medicines, and dietary supplements.
Wear gloves when handling this medicine.
Wear gloves when handling your child’s soiled diapers and urine while your child is taking this medicine.
Wash your hands after handling this medicine or handling your child’s diapers or urine while taking this medicine.
If your child is unable to tolerate this medicine, contact your healthcare provider.

Your child will initially be seen by your doctor once a month for the first 3 months after starting Alpelisib.
Based on your child’s condition, visits may change to every 3 months.
Your child will be getting blood work at every visit. Some of the blood tests include:
- Complete blood count (CBC): counts your child’s white cells, red cells, and platelets
- Complete metabolic panel (CMP): to check electrolytes and blood proteins
- Kidney and liver function tests: to see how well the kidneys and liver are working
- Pancreas enzyme tests: to see if the pancreas is inflamed
- Blood sugar tests: to see if there is any change in sugar levels while on the medicine
Cardiac tests are needed throughout the time your child is on this medication. These tests let the doctor know how well your child is tolerating the Alpelisib.
- EKG: Will look at your child’s cardiac rhythm. This is first done initially at 3 months and then every 6-12 months.
- ECHO: This will look at your child’s heart function. This is done initially and at other time intervals if needed.

Some side effects may go away quickly while others may last longer. Tell your provider about any side effects your child has been experiencing.

Call your healthcare provider right away if your child has any of the side effects below:

Reviewed December 2023 by Stephanie Adams, RN, BSN and Michael D. Fox, MD, MEd

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