Patient Medication Information: uses, side effects, how to take - Kanuma
What is Kanuma used for?
Kanuma contains the active substance sebelipase alfa. Sebelipase alfa is similar to the naturally occurring enzyme lysosomal acid lipase (LAL), which the body uses to breakdown fats. It is used to treat patients with lysosomal acid lipase deficiency (LAL deficiency).
LAL deficiency is a genetic disease that leads to liver damage, high blood cholesterol, and other complications due to a build-up of certain types of fats (cholesteryl esters and triglycerides).
How does Kanuma work?
Kanuma contains the active substance sebelipase alfa which is similar to the naturally occurring enzyme lysosomal acid lipase (LAL). It works by breaking down the build-up of fat in tissues and organs.
What are the ingredients in Kanuma?
Medicinal ingredients: Sebelipase alfa
Non-medicinal ingredients: Citric acid monohydrate, Human serum albumin, Trisodium citrate dehydrate, Water for injection
Kanuma comes in the following dosage forms:
Kanuma is a concentrated solution to be diluted before use.
Kanuma is supplied in a clear glass vial (Type I) with a butyl rubber stopper, and an aluminum seal with a plastic flip-off cap, containing 10 mL of concentrate.
Do not use Kanuma if:
- Your doctor determined that life-threatening allergic reactions to Kanuma or its ingredients have occurred.
To help avoid side effects and ensure proper use, talk to your healthcare professional before you take Kanuma. Talk about any health conditions or problems you may have, including if you:
- your infant has confirmed multiple-organ failure
you are pregnant or think you may be pregnant or are planning to have a baby
- you are nursing
- you or your child is on a controlled sodium diet
Other warnings you should know about:
Hypersensitivity including anaphylaxis
In clinical trials, Kanuma has caused hypersensitivity, some of which were severe. The
- symptoms include abdominal pain, agitation, fever, chills, diarrhea, dry flaky skin, swelling, high blood pressure, irritability, nausea, swelling of the throat, pale skin, itchy skin rash and vomiting. The majority of these reactions occurred during or within 4 hours of the completion of the infusion.
- Anaphylaxis/life-threatening allergic reaction: If you/your child develop signs and symptoms of a severe reaction including chest discomfort, red eyes, difficulty breathing, generalized and itchy rash, excess blood to parts of the body, swelling of eyelids, runny nose, fast heartbeat, rapid breathing and hives, seek immediate medical attention.
- Tell your doctor if you have had a severe allergic reaction to eggs or egg products, as people with a known history of egg allergies were excluded from clinical trials.
Driving and using machines
Kanuma may have a minor influence on the ability to drive and use machines. Adverse effects of sebelipase alfa include dizziness which could affect the ability to drive or use machines.
Anti-drug antibodies
The development of blood proteins against Kanuma, also called anti-drug antibodies, may occur during the treatment. Talk to your doctor if you feel that Kanuma is no longer working as well for you.
Tell your healthcare professional about all the medicines you take, including any drugs, vitamins, minerals, natural supplements or alternative medicines.
How to take Kanuma:
Your doctor or nurse will give Kanuma to you or child by an infusion (drip) into a vein. The medicine will be diluted before being given to you or your child. Each infusion will take approximately 2 hours. You or your child may be monitored by your doctor or nurse for an additional 2 hours after the infusion.
Usual dose:
The dose you or your child receives is based on your or your child’s body weight. The recommended dose is 1 mg per kg body weight once every other week intravenously. For infants with signs and symptoms of rapidly deteriorating disease, the recommended starting dose is 1 mg/kg or 3 mg/kg once weekly. Dose adjustments may be considered based on how well you or your child responds to treatment.
Overdose:
There is very limited information on overdosage with Kanuma.
| If you think you, or a person you are caring for, have taken too much Kanuma, contact a healthcare professional, hospital emergency department, or regional poison control centre immediately, even if there are no symptoms. |
|---|
Missed Dose:
Contact your physician immediately if you miss a dose of Kanuma. Your physician will decide when you should receive your next dose.
What are possible side effects from using Kanuma?
These are not all the possible side effects you may feel when taking Kanuma. If you experience any side effects not listed here, contact your healthcare professional.
The table below summarizes the common serious side effects that occurred in children and adult patients with LAL-D receiving Kanuma.
Serious side effects and what to do about them
| Symptom / effect | Talk to your healthcare professional | Stop taking drug and get immediate medical help | |||
|---|---|---|---|---|---|
| Only if severe | In all cases | ||||
| VERY COMMON | |||||
| Stomachache | √ | ||||
| COMMON | |||||
| Anaphylactic reaction (severe allergic reaction)a | √ | ||||
| Hypersensitivityb | √ | ||||
a Occurred in 2 patients treated in clinical studies. Based on Preferred Term ‘anaphylactic reaction’ and application of Sampson criteria to identify signs/symptoms consistent with anaphylaxis.
b May include: chills, eczema, swelling of the throat, nausea, itching, and hives.
Other very common (> 1/10) adverse reactions reported in children and adult patients who received Kanuma included dizziness, diarrhea, tiredness and fever.
Other common (> 1/100 to < 1/10) adverse reactions reported in children and adult patients who received Kanuma included fast heartbeat, skin redness, low blood pressure, shortness of breath, stomach bloating, rash, raised rash, chest discomfort, reaction at the infusion site, and body temperature increased.
The table below summarizes most serious common side effects that occurred in infant patients (< 6 months of age) with LAL-D receiving Kanuma.
Serious side effects and what to do about them
| Symptom / effect | Talk to your healthcare professional | Stop taking drug and get immediate medical help | ||
|---|---|---|---|---|
| Only if severe | In all cases | |||
| VERY COMMON | ||||
| Difficulty breathing | √ | |||
| Hypersensitivitya | √ | |||
| Anaphylactic reaction (severe allergic reaction)b | √ | |||
| Diarrhea | √ | |||
| Vomiting | √ | |||
| Body temperature increased | √ | |||
| Fever | √ | |||
| Development of blood proteins | √ | |||
a May include: irritability, agitation, vomiting, urticaria, eczema, pruritus, pallor, and drug hypersensitivity b Occurred in 3 infant patients treated in clinical studies. Based on Preferred Term ‘anaphylactic reaction’ and application of Sampson criteria to identify signs/symptoms consistent with anaphylaxis.
Other very common (> 10%) side effects occurring in infants include: swelling of eyelids, fast heartbeat, rash, raised rash, decreased oxygen in the blood, blood pressure increased, and rapid breathing.
If you have a troublesome symptom or side effect that is not listed here or becomes bad enough to interfere with your daily activities, talk to your healthcare professional.
Reporting Side Effects
You can report any suspected side effects associated with the use of health products to Health Canada by:
NOTE: Contact your health professional if you need information about how to manage your side effects. The Canada Vigilance Program does not provide medical advice. |
Storage:
Store in a refrigerator (2°C to 8°C). Protect from light.
DO NOT FREEZE
Do not use beyond the expiration date stamped on the carton.
Keep out of reach and sight of children.
If you want more information about Kanuma:
- Talk to your healthcare professional
- Find the full product monograph that is prepared for healthcare professionals and includes this Patient Medication Information by visiting the Health Canada website; the manufacturer’s website www.alexion.com, or by calling 1-844-MAP-PAM2 (1-844-627-7262)