What Is Replagal Used For?

Replagal is a specialized medication designed to treat Fabry Disease through enzyme replacement therapy.

Primary Uses

Replagal is used to treat Fabry Disease, a genetic condition where the body doesn’t produce enough of a specific enzyme called α-galactosidase A.

How It Works

Replagal contains agalsidase alfa ghu, which is a form of the human enzyme α-galactosidase A. It works by replacing the enzyme that your body is missing or not producing enough of due to Fabry Disease. The enzyme is produced by switching on the gene for α-galactosidase A in cells, then removed and made into a sterile concentrate for infusion.

How to Take Replagal

Replagal is given as an intravenous infusion in a hospital or clinical setting under medical supervision.

Dosage Instructions

The usual dose is 0.2 mg for every kg you weigh (about 14 mg or 4 vials for an average 70 kg person). The medication is mixed with 0.9% sodium chloride solution and infused into a vein in your arm over 40 minutes. Infusions are given every two weeks. Your treatment will be supervised by a doctor who specializes in treating Fabry Disease. You may need long-term treatment with Replagal.

What If You Miss a Dose?

If you miss an infusion, the enzyme levels that Replagal is meant to replace will remain low. Contact your doctor immediately – they will decide when you need your next infusion. If you stop using Replagal, your enzyme levels will stay low and Fabry Disease symptoms won’t be treated.

Side Effects

Like all medications, this medicine can cause side effects. Most are mild and temporary, but some require medical attention.

Common Side Effects

These side effects occur relatively frequently and usually don’t require emergency care:

• allergic reaction
• headache or dizziness
• nausea
• fatigue
• diarrhea
• cough
• vomiting
• pain or swelling of joints
• back or limb pain
• fever
• flu-like symptoms
• tingling or numbness in fingers or toes
• abdominal pain or discomfort
• chest pain
• palpitations
• muscle pain
• unusual weakness
• feeling cold
• rash
• swelling of hands or feet
• ears ringing
• tremor or shakes
• flushing
• change in taste of food
• increased tear secretion
• prolonged sleep or difficulty staying awake
• increased or low blood pressure
• chest tightness
• hoarseness or sore throat
• runny nose
• acne
• red, itchy, or mottled skin
• rash at infusion site
• feeling hot
• general pain or discomfort
• generally feeling unwell

Infusion-Related Reactions

About 13.7% of patients experience reactions during or after Replagal infusion. Most are mild and include chills, headache, nausea, fever, facial flushing, and tiredness. These typically occur 2-4 months after starting treatment and decrease over time, though they may begin more than 1 year after starting. More serious reactions with fever, chills, fast heart rate, hives, vomiting, and throat/tongue swelling have been reported uncommonly. Your doctor may temporarily stop the infusion and treat symptoms with antihistamines or corticosteroids if needed.

Important Warnings

Before starting Replagal, it’s important to discuss your medical history and current medications with your doctor.

Who Should Not Take Replagal

Do not use Replagal if you are allergic to agalsidase alfa ghu or any of the ingredients listed in the medication. Always check the ingredients to make sure you can use this medicine safely.

Medical Conditions to Discuss

Tell your doctor if you think you’re allergic to any ingredients in Replagal, have previously used Replagal and had unusual reactions like skin rash or flu-like symptoms, or take any medicines for other conditions. Also inform your doctor about any previous allergic reactions to injections.

Pregnancy and Breastfeeding

Check with your doctor if you are pregnant, planning to become pregnant, breastfeeding, or planning to breastfeed. Your doctor will discuss the risks and benefits of using Replagal during pregnancy or while breastfeeding.

Drug Interactions

Some medications may interfere with Replagal and affect how it works. Always tell your doctor about all medicines you’re taking.

• chloroquine (used to prevent or treat malaria)
• amiodarone (used to treat life-threatening heart rhythm problems)
• benoquin (used to lighten skin in people with vitiligo)
• gentamicin (used to treat serious bacterial infections)

Alcohol & Driving

Be cautious about activities requiring alertness until you know how Replagal affects you.

Alcohol

No specific alcohol interactions are mentioned, but discuss alcohol use with your doctor.

Driving and Operating Machinery

Be careful before you drive or use any machines or tools until you know how Replagal affects you. Replagal is not expected to affect your ability to drive a car or operate machinery, but side effects like dizziness or fatigue could impact your alertness.

Storage

Store Replagal at 2-8°C (in a refrigerator). Do not freeze. The medication is usually kept in the hospital pharmacy where you receive treatment, and the infusion is prepared individually for you. Any unused solution is discarded. The infusion should be given immediately after preparation unless your doctor instructs otherwise, as Replagal contains no preservatives to prevent bacterial growth. Keep away from children and don’t use after the expiry date or if the package is damaged.

Overdose Information

Since Replagal is given by healthcare professionals under supervision, overdose is unlikely. However, if you suspect an overdose has occurred, seek immediate medical attention. Contact the Poisons Information Centre (13 11 26), your doctor, or go to the nearest hospital emergency department, even if you don’t feel unwell.

Frequently Asked Questions