What Strensiq is and what it is used for
What is Strensiq
Strensiq is a medicine used to treat the inherited disease hypophosphatasia that started in childhood. It contains the active substance asfotase alfa.
What is hypophosphatasia
Patients with hypophosphatasia have low levels of an enzyme called alkaline phosphatase that is important for various body functions, including the proper hardening of bones and teeth. Patients have problems with bone growth and strength, which can lead to broken bones, bone pain, and difficulty walking, as well as difficulties with breathing and a risk of seizures (fits).
What is Strensiq used for
The active substance in Strensiq can replace the missing enzyme (alkaline phosphatase) in hypophosphatasia. It is used for long-term enzyme replacement treatment to manage symptoms.
What benefits of Strensiq have been shown in clinical studies
Strensiq has shown benefits for patients’ mineralization of the skeleton and growth.
What you need to know before you use Strensiq
Do not use Strensiq
If you are severely allergic to asfotase alfa (see section ‘Warnings and precautions’ below) or any of the other ingredients of this medicine (listed in section 6).
Warnings and precautions
Talk to your doctor before using Strensiq.
Should you experience anaphylactic reaction, or an event with similar symptoms, your doctor will discuss with you the next steps and the possibility to restart Strensiq under medical supervision. Always follow the instructions provided by your doctor.
Other medicines and Strensiq
Tell your doctor or pharmacist if you are using, have recently used or might use any other medicines.
If you need to undergo laboratory tests (giving blood for testing), tell your doctor that you are treated with Strensiq. Strensiq may cause some tests to show wrongly higher or lower results.
Therefore another type of test may need to be used if you are treated with Strensiq.
Pregnancy
Strensiq should not be used during pregnancy. The use of effective birth control during treatment should be considered in women who are able to get pregnant.
Breast-feeding
It is not known whether Strensiq can pass into breast milk. Tell your doctor if you are breast-feeding or plan to do so. Your doctor will then help you decide whether to stop breast-feeding, or whether to stop taking Strensiq, considering the benefit of breast-feeding to the baby and the benefit of Strensiq to the mother.
If you are pregnant or breast-feeding, think you may be pregnant or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.
Driving and using machines
This medicine is not expected to have any effect on the ability to drive or use machines.
Important information about some of the ingredients of Strensiq
This medicine contains less than 1 mmol sodium (23 mg) per vial, which means it is essentially ‘sodium-free’.
How to use Strensiq
Always use this medicine exactly as described in this leaflet or as your doctor, or pharmacist or nurse has told you. Check with your doctor, pharmacist or nurse if you are not sure.
How to use Strensiq will be explained to you by a doctor who is experienced in the management of patients with metabolic or bone related diseases. After being trained by the doctor or specialized nurse, you can inject Strensiq yourself at home.
Dose
The maximum volume per injection should not exceed 1 ml. If more than 1 ml is required, you need to do multiple injections immediately one after the other.
The following applies when you are injecting 3x per week (patient body weight between 3kg and 40kg)
For individuals with body weight 3kg the volume to be injected is 0.15ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 4kg the volume to be injected is 0.20ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 5kg the volume to be injected is 0.25ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 6kg the volume to be injected is 0.30ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 7kg the volume to be injected is 0.35ml; the colour code of the vial to be used is orange.
For individuals with body weight 8kg the volume to be injected is 0.40ml; the colour code of the vial to be used is orange.
For individuals with body weight 9kg the volume to be injected is 0.45ml; the colour code of the vial to be used is orange.
For individuals with body weight 10kg the volume to be injected is 0.50ml; the colour code of the vial to be used is light blue.
For individuals with body weight 11kg the volume to be injected is 0.55ml; the colour code of the vial to be used is light blue.
For individuals with body weight 12kg the volume to be injected is 0.60ml; the colour code of the vial to be used is light blue.
For individuals with body weight 13kg the volume to be injected is 0.65ml; the colour code of the vial to be used is light blue.
For individuals with body weight 14kg the volume to be injected is 0.70ml; the colour code of the vial to be used is light blue.
For individuals with body weight 15kg the volume to be injected is 0.75ml; the colour code of the vial to be used is pink.
For individuals with body weight 16kg the volume to be injected is 0.80ml; the colour code of the vial to be used is pink.
For individuals with body weight 17kg the volume to be injected is 0.85ml; the colour code of the vial to be used is pink.
For individuals with body weight 18kg the volume to be injected is 0.90ml; the colour code of the vial to be used is pink.
For individuals with body weight 19kg the volume to be injected is 0.95ml; the colour code of the vial to be used is pink.
For individuals with body weight 20kg the volume to be injected is 1ml; the colour code of the vial to be used is pink.
For individuals with body weight 25kg the volume to be injected is 0.50ml; the colour code of the vial to be used is green.
For individuals with body weight 30kg the volume to be injected is 0.60ml; the colour code of the vial to be used is green.
For individuals with body weight 35kg the volume to be injected is 0.70ml; the colour code of the vial to be used is green.
For individuals with body weight 40kg the volume to be injected is 0.80ml; the colour code of the vial to be used is green.
The following applies when you are injecting 6 x per week (patient body weight between 6kg and 100kg).
For individuals with body weight 6kg the volume to be injected is 0.15ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 7kg the volume to be injected is 0.18ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 8kg the volume to be injected is 0.20ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 9kg the volume to be injected is 0.23ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 10kg the volume to be injected is 0.25ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 11kg the volume to be injected is 0.28ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 12kg the volume to be injected is 0.30ml; the colour code of the vial to be used is dark blue.
For individuals with body weight 13kg the volume to be injected is 0.33ml; the colour code of the vial to be used is orange.
For individuals with body weight 14kg the volume to be injected is 0.35ml; the colour code of the vial to be used is orange.
For individuals with body weight 15kg the volume to be injected is 0.38ml; the colour code of the vial to be used is orange.
For individuals with body weight 16kg the volume to be injected is 0.40ml; the colour code of the vial to be used is orange.
For individuals with body weight 17kg the volume to be injected is 0.43ml; the colour code of the vial to be used is orange.
For individuals with body weight 18kg the volume to be injected is 0.45ml; the colour code of the vial to be used is orange.
For individuals with body weight 19kg the volume to be injected is 0.48ml; the colour code of the vial to be used is light blue.
For individuals with body weight 20kg the volume to be injected is 0.50ml; the colour code of the vial to be used is light blue.
For individuals with body weight 25kg the volume to be injected is 0.63ml; the colour code of the vial to be used is light blue.
For individuals with body weight 30kg the volume to be injected is 0.75ml; the colour code of the vial to be used is pink.
For individuals with body weight 35kg the volume to be injected is 0.88ml; the colour code of the vial to be used is pink.
For individuals with body weight 40kg the volume to be injected is 1 ml; the colour code of the vial to be used is pink.
For individuals with body weight 50kg the volume to be injected is 0.50ml; the colour code of the vial to be used is green.
For individuals with body weight 60kg the volume to be injected is 0.60ml; the colour code of the vial to be used is green.
For individuals with body weight 70kg the volume to be injected is 0.70ml; the colour code of the vial to be used is green.
For individuals with body weight 80kg the volume to be injected is 0.80ml; the colour code of the vial to be used is green.
For individuals with body weight 90kg the volume to be injected is 0.90ml; the colour code of the vial to be used is green (x2).
For individuals with body weight 100kg the volume to be injected is 1 ml; the colour code of the vial to be used is green (x2).
Injection recommendations
Before injecting Strensiq, please read the following instructions carefully
How to inject Strensiq:
Step 1: Preparing the Strensiq dose
Step 2: Withdrawing Strensiq solution from the vial
Step 3: Placing the needle for injection on the syringe
Control visually that the volume contained into the syringe is correct.The volume per injection should not exceed 1 ml. If it is the case, multiple injections should be done at different sites.
You are now ready to inject the correct dose.
Step 4: Injecting Strensiq
If you need a second injection for your prescribed dose, get another Strensiq vial and repeat steps 1 through 4.
Step 5: Disposing of supplies
Please collect your syringes, vials and needle in a sharps container. Your doctor, pharmacist or nurse will advise you on how you can obtain a sharps container.
If you use more Strensiq than you should
If you suspect that you have been accidently administered a higher dose of Strensiq than prescribed, please contact your doctor for advice.
If you forget to use Strensiq
Do not inject a double dose to make up for a forgotten dose and contact your doctor for advice.
If you have any further questions on the use of this medicine, ask your doctor, pharmacist or nurse.
Possible side effects
Like all medicines, this medicine can cause side effects, although not everybody gets them.
If you are not sure what the side effects below are, ask your doctor to explain them to you.
The most serious side effects seen in patients receiving asfotase alfa have been allergic reactions including life threatening allergic reactions requiring medical treatment similar to anaphylaxis. This side effect is common [may affect up to 1 in 10 people]). Patients who experienced these serious allergic reactions had difficulty breathing, choking sensation, nausea, swelling around the eyes, and dizziness. The reactions occurred within minutes after using asfotase alfa and can occur in patients who were using asfotase alfa for more than one year. If you experience any of these symptoms, discontinue Strensiq and seek medical help immediately.
Additionally, other allergic reactions (hypersensitivity) which may appear as redness (erythema), fever (pyrexia), rash, itchiness (pruritis), irritability, feeling sick (nausea), throwing up (vomiting), pain, chills (rigor), numbness of the mouth (hypoaesthesia oral), headache, blushing (flushing), fast beating of the heart (tachycardia), and cough may occur commonly. If you experience any of these symptoms, discontinue Strensiq and seek medical help immediately.
Very Common: may affect more than 1 in 10 people
Reactions at the injection site during the injection of the medicine or during the hours following the injection (which can lead to redness, discolorations, itching, pain, fatty lumps or decreased fatty tissue on the surface of the skin, skin hypopigmentation, and/or swelling)
Fever (pyrexia)
Irritability
Skin redness (erythema)
Pain in hands and feet (pain in extremity)
Bruise (contusion)
Headache
Common: may affect up to 1 in 10 people
Stretched skin, skin discolouration
Feeling sick (nausea)
Numbness of the mouth (hypoaesthesia oral)
Aching muscles (myalgia)
Scar
Increased tendency to bruise
Hot flush
Infection of skin at injection site (injection site cellulitis)
Reduced levels of calcium in the blood (hypocalcaemia)
Kidney stones (nephrolithiasis)
Reporting of side effects
If you get any side effects, talk to your doctor or pharmacist or nurse. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the national reporting system listed below:
Ireland
HPRA Pharmacovigilance
Website: www.hpra.ie
United Kingdom (Northern Ireland)
Yellow Card Scheme
Website: www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store.
Adverse events should also be reported to Alexion Pharma UK Ltd on uk.adverseevents@alexion.com, Freephone (Ireland): 1 800 936 544, (UK Northern Ireland): 0800321 3902.
By reporting side effects you can help provide more information on the safety of this medicine.
How to store Strensiq
Keep this medicine out of the sight and reach of children.
Do not use this medicine after the expiry date which is stated on the carton and the vial label after EXP. The expiry date refers to the last day of that month.
Store in a refrigerator (2°C-8°C).
Do not freeze.
Store in the original package in order to protect from light.
After opening the vial, the product should be used immediately (within 3 hours maximum at room temperature, between 23°C and 27°C).
Do not throw away any medicines via wastewater or household waste. Ask your pharmacist how to throw away medicines you no longer use. These measures will help protect the environment.
Contents of the pack and other information
What Strensiq contains
The active substance is asfotase alfa. Each ml of solution contains 100 mg of asfotase alfa.
Each vial of 0.8 ml solution (100 mg/ml) contains 80 mg of asfotase alfa.
The other ingredients are sodium chloride, sodium phosphate monobasic monohydrate, sodium phosphate dibasic heptahydrate and water for injections.
What Strensiq looks like and contents of the pack
Strensiq is presented as a clear, slightly opalescent or opalescent, colourless to slightly yellow aqueous solution for injection in vials containing 0.8 ml of solution. A few small translucent or white particles may be present.
Pack sizes of 1 or 12 vials.
Not all pack sizes may be marketed in your country.
Marketing Authorisation Holder
Alexion Europe SAS
103-105 rue Anatole France
92300 Levallois-Perret
France
Manufacturer
Alexion Pharma International Operations Limited
College Business and Technology Park, Blanchardstown
Dublin 15
Ireland
This leaflet was last revised in January 2023.
This medicine has been authorised under ‘exceptional circumstances’.
This means that because of the rarity of this disease it has been impossible to get complete information on this medicine.
The European Medicines Agency will review any new information on this medicine every year and this leaflet will be updated as necessary.
Other sources of information
Detailed information on this medicine is available on the European Medicines Agency web site: http://www.ema.europa.eu. There are also links to other websites about rare diseases and treatments.