Patient Information Leaflet

Lamzede10mg powder for solution for infusion

velmanasa alfa

This medicine is subject to additional monitoring, which will speed up the detection of new information about its safety. You can contribute by reporting any adverse effects you may experience. The final part of section4 includes information on how to report these adverse effects.

Read this leaflet carefully before you start using this medicine, because it contains important information for you.

• Keep this leaflet, as you may need to read it again.
• If you have any questions, ask your doctor.

-If you experience any adverse effects, consult your doctor,nurse or pharmacist, even if they are not listed in this leaflet. See section4.

1.What is Lamzede and what it is used for

2.What you need to know before starting to use Lamzede

3.How to use Lamzede

4.Possible adverse effects

5.Storage of Lamzede

6.Contents of the pack and additional information

Lamzede contains the active ingredient velmanasa alfa, which belongs to a group of medications called enzyme replacement therapies.It is used to treat patients with alpha‑manosidosis of mild to moderate severity. It is used to treat the non-neurological symptoms of the disease.

Alpha‑manosidosis is a rare genetic disorder caused by a decrease in an enzyme called alpha‑manosidase, which is needed to break down certain sugar compounds (called "oligosaccharides rich in mannose") in the body.When this enzyme is absent or does not function properly, these sugar compoundsaccumulate inside cells and cause the signs and symptoms of the disease.Among the typical manifestations of the disease are distinctive facial features, mental retardation, difficulty controlling movements, hearing and speech difficulties, frequent infections, skeletal problems, muscle pain, and weakness.

Velmanasa alfa is designed to replace the missing enzyme in patients with alpha‑manosidosis.

No use Lamzede

-if you are allergic to velmanasa alfa or any of the other components of this medication (listed in section6).

Advertencias y precauciones

Consult your doctor before starting to use Lamzede.

Reactions of hypersensitivity may occur with the administration of Lamzede. These reactions usually appear during or shortly after the infusion and may manifest with various symptoms, such as localized or diffuse skin reactions, gastrointestinal symptoms, or swelling of the throat, face, lips, or tongue (see section4 “Posibles efectos adversos”). If the hypersensitivity reaction is severe, it is recommended to immediately stop the administration of Lamzedeand follow the current medical protocol for emergency treatment.Less severe hypersensitivity reactions can be controlled by temporarily interrupting the infusion or reducing the infusion rate; the doctor may consider administering medications used to treat allergies.

If you receive treatment with Lamzede, you may experience an adverse effect during or immediately after the infusion (perfusion) used to administer the medication (see section4“Posibles efectos adversos”). This is known asinfusion-related reactionand may sometimes be severe.

-Your doctor may decide to keep you under observation for one hour or more after the infusion in relation to infusion-related reactions.

-Infusion-related reactions include dizziness, headache, nausea, low blood pressure, fatigue, and fever. If you experience an infusion-related reaction,inform your doctor immediately.

-If you experience an infusion-related reaction, you may be administered additional medications to treat or help prevent future reactions. These medications may include medications used to treat allergies (antihistamines), medications used to treat fever (antipyretics), and medications to control inflammation (corticosteroids).

-If the infusion-related reaction is severe, your doctor will immediately stop the infusion and begin administering appropriate medical treatment.

-If the infusion-related reactions are severe and/or you experience a loss of effect of this medication, your doctor will perform a blood test to check if there are antibodies that may affect the treatment outcome.

-Most of the time, you will be able to continue receiving Lamzede even if you experience an infusion-related reaction.

Antibodies may play a role in hypersensitivity and infusion-related reactions observed with the use of Lamzede. Although 24% of patients presented antibodies against Lamzede during its clinical development, no clear correlation was observed between antibody titers and reduced efficacy or occurrence of hypersensitivity reactions.

Otros medicamentos y Lamzede

Inform your doctor if you are taking, have recently taken, or may need to take any other medication.

Embarazo y lactancia

If you are pregnant or breastfeeding, or if you think you may be pregnant, consult your doctor before using this medication.

You should not takethis medicationduring pregnancyunlessyour doctor indicates that it is clearly necessary. Your doctor will discuss this with you.

The passage of velmanasa alfa into breast milk is unknown. Lamzede can be used during breastfeeding, as the infant will not absorb the velmanasa alfa.

Conducción y uso de máquinas

The influence of Lamzede on the ability to drive and use machines is negligible or insignificant.

Lamzede contains sodium

This medication contains less than 1mmol of sodium (23mg) per dose unit; it is essentially “sodium-free”.

This medication should only be used under the supervision of a doctor with experience in the treatment of alpha-manosidosis or similar diseases and should only be administered by a healthcare professional.

Lamzede should only be used under the supervision of a doctor with experience in the treatment of alpha-manosidosis. Your doctor may advise you to receive the treatment at home if you meet certain criteria. Contact your doctor if you wish to receive the treatment at home.

Dose

The recommended dose of Lamzede is 1 mg/kg of body weight administered once a week.

Use in children and adolescents

Lamzede can be administered to children and adolescents at the same dose and frequency as adults.

Administration

Lamzede is supplied in a vial in the form of a powder for infusion that will be reconstituted with water for injection before administration.

Once reconstituted, the medication will be administered via an infusion pump (drip) in a vein over a period of at least 50 minutes under the supervision of your doctor.

If you have any other questions about the use of this medication, ask your doctor, nurse, or pharmacist.

Like all medicines, this medicine can cause side effects, although not everyone will experience them. Most side effects occur during infusion or shortly after it ("infusion-related reaction", see section2 "Warnings and Precautions").

While you are being treated with Lamzede, you may experience some of the following reactions:

Severe side effects

Side effectsfrequent(may affect up to 1 in 10people)

-loss of consciousness (fainting, which may be preceded by dizziness, drowsiness, or confusion)

-acute kidney injury (kidney problems that may be recognized by fluid retention, swelling of the legs, ankles, or feet, drowsiness, feeling of lack of air or fatigue)

-severe hypersensitivity and allergic reaction (its symptoms include localized or diffuse skin itching, dizziness, difficulty breathing, chest pain, chills, fever, gastrointestinal symptoms such as nausea, vomiting, diarrhea, or abdominal pain, swelling of the throat, face, lips, or tongue)

If you experience any side effect similar to those described, inform your doctor immediately.

Other side effects

Side effectsvery frequent(may affect more than 1 in 10people)

-diarrhea

-weight gain

-fever/raised body temperature

Side effectsfrequent(may affect up to 1 in 10people)

-slow heart rate (bradycardia)

-blue discoloration of the skin and lips (cyanosis)

-psychotic behavior (mental illness with hallucinations, difficulty thinking clearly, and understanding reality, anxiety), difficulty falling asleep

-confusion, fainting, tremor, dizziness, headache

-abdominal pain (intestinal pain), stomach irritation caused by digestive acids (gastritis by reflux), nausea, vomiting

-pain at the infusion site, chills, feeling of heat, general discomfort, fatigue

-skin rashes (urticaria), increased sweating (hyperhidrosis)

-nasal bleeding

-joint pain, back pain, joint stiffness, muscle pain, pain in the extremities (hands, feet)

-eye irritation, swollen eyelids (edema palpebral), red eyes

-increased appetite

Side effects ofunknown frequency(cannot be estimated from available data)

-infection of the inner wall of the sac surrounding the heart (endocarditis)

-boil

-bacterial infection caused byStaphylococcus

-decreased appetite

-agitation, fecal incontinence, nervousness

-difficulty coordinating muscle movements

-drowsiness

-increased tear secretion

-hearing loss

-aortic valve incompetence (disorder in which the aortic valve does not close hermetically)

-rapid heart rate

-low blood pressure

-vascular fragility

-oral and pharyngeal pain

-whistling sounds ("pops") when breathing

-difficulty swallowing

-skin redness

-swelling in the joints, heat in the joints

-weakness

Reporting side effects

If you experience any type of side effect, consult your doctor,even if it is a possible side effect that does not appear in this leaflet. You can also report them directly through the Spanish System for Pharmacovigilance of Medicines for Human Usehttps://www.notificaram.es. By reporting side effects, you can contribute to providing more information on the safety of this medicine.

Keep this medication out of the sight and reach of children.

Do not use this medication after the expiration date that appears on the label and on the box after “EXP/CAD”. The expiration date is the last day of the month indicated.

Store and transport refrigerated (between 2°C and 8°C). Do not freeze.

Store in the original packaging to protect it from light.

After reconstitution, the medication must be used immediately. If not used immediately, the reconstituted solution can be stored for a maximum of 24 hours at between 2°C and 8°C.

This medication should not be used if the reconstituted solution contains opaque particles or has changed color.

Medications should not be disposed of through drains or trash. Ask your pharmacist how to dispose of empty containers and unused medications. This will help protect the environment.

Composition of Lamzede

-The active principle is velmanasa alpha.

One vial contains 10mg of velmanasa alpha.

After reconstitution, 1ml of the solution contains 2mg of velmanasa alpha (10mg/5ml).

-The other components are: dihydrogen phosphate sodium dihydrate, dihydrogen phosphate sodium dihydrate (see section2 “Lamzede contains sodium”), mannitol (E 421) and glycine.

Appearance of the product and contents of the pack

Lamzede is a white to off-white powder for solution for infusion, supplied in a glass vial.

Each pack contains 1, 5 or 10vials.

Only some pack sizes may be marketed.

Marketing Authorization Holder

Chiesi Farmaceutici S.p.A.

Via Palermo 26/A

43122 Parma

Italy

Responsible for manufacturing

Chiesi Farmaceutici S.p.A.

Via San Leonardo, 96

43122 Parma

Italy

Further information on this medicinal product can be obtained by contacting the local representative of the marketing authorization holder:

Last update of the leaflet: January 2023

This medicinal product has been authorised under «exceptional circumstances». This authorisation means that due to the rarity of this disease, it has not been possible to obtain a complete information on this medicinal product..

The European Medicines Agency will review annually any new information available on this medicinal product and this leaflet will be updated as necessary.

Other sources of information

Further information on this medicinal product is available on the website of the European Medicines Agency:http://www.ema.europa.eu.There are also links to other websites on rare diseases and orphan medicinal products.

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This information is intended for healthcare professionals only.

Lamzede requires reconstitution and is only for intravenous infusion.

Each vial is for single use only.

Instructions for reconstitution and administration

The reconstitution and administration of Lamzede should be performed by a healthcare professional.

Aseptic technique should be used during preparation. Needles with filters should not be used during preparation.

a)The number of vials to be used should be calculated based on the individual patient's weight. The recommended dose of 1mg/kg is determined by the following calculation:

• Patient weight (kg) × dose (mg/kg) = Patient dose (in mg).
• Patient dose (in mg) divided by 10mg/vial (content of one vial) = number of vials to be reconstituted. If the calculated number of vials includes a fraction, it should be rounded up to the next whole number.
• About 30minutes before reconstitution, the required number of vials should be removed from the refrigerator. The vials should reach room temperature (between 15°C and 25°C) before reconstitution.

Each vial is reconstituted by slowly injecting 5ml of water for injection into the inner wall of each vial. Each millilitre of reconstituted solution contains 2mg of velmanasa alpha. Only the corresponding volume to the recommended dose should be administered.

Example:

• Patient weight (44kg)×dose (1mg/kg) = Patient dose (44mg).
• 44mg divided by 10mg/vial = 4.4vials; therefore, 5vials should be reconstituted.
• From the total reconstituted volume, only 22ml (corresponding to 44mg) should be administered.

b)The powder should be reconstituted within the vial by slowly adding water for injection into the inner wall of the vial and not directly onto the lyophilised powder. The vials should be avoided to be ejected with force from the syringe onto the powder to reduce foam formation to a minimum. The reconstituted vials should be left to stand in a vertical position on the table for about 5‑10minutes. Then, each vial should be tilted and gently rolled for 15‑20seconds to enhance the dissolution process. The vial should not be inverted, removed or agitated.

c)After reconstitution, an immediate visual inspection of the solution should be performed to check for particles in suspension or colour changes. The solution should be transparent andshould not be used if particles are observed or if the colour has changed.Due to the nature of the medicinal product, the reconstituted solution may occasionally contain some proteinaceous particles in the form of fine white threads or translucent fibres, which will be eliminated by the integrated filter during infusion (see point e).

d)The reconstituted solution should be slowly withdrawn from each vial with caution to avoid foam formation in the syringe. If the volume of the solution exceeds the capacity of a syringe, the required number of syringes should be prepared to rapidly replace the syringe during infusion.

e)The reconstituted solution should be administered using an infusion device equipped with a pump and an integrated filter with a low protein binding of 0.22µm.

The total infusion volume should be determined based on the patient's weight and should be administered over a minimum of 50minutes. It is recommended to use the same dilution (2mg/ml) always. In patients weighing less than 18kg, who are to receive less than 9ml of reconstituted solution, the infusion rate should be calculated so that the infusion time is ≥50minutes.The maximum infusion rate is 25ml/hour. The infusion time can be calculated using the following table:

Patient weight (kg)	Dose (ml)	Maximum infusion rate (ml/h)	Minimum infusion time (minutes)		Patient weight (kg)	Dose (ml)	Maximum infusion rate (ml/h)	Minimum infusion time (minutes)
5	2.5	3	50		53	26.5	25	64
6	3	3.6	50		54	27	25	65
7	3.5	4.2	50		55	27.5	25	67
8	4	4.8	50		56	28	25	67
9	4.5	5.4	50		57	28.5	25	68
10	5	6	50		58	29	25	70
11	5.5	6.6	50		59	29.5	25	71
12	6	7.2	50		60	30	25	72
13	6.5	7.8	50		61	30.5	25	73
14	7	8.4	50		62	31	25	74
15	7.5	9	50		63	31.5	25	76
16	8	9.6	50		64	32	25	77
17	8.5	10.2	50		65	32.5	25	78
18	9	10.8	50		66	33	25	79
19	9.5	11.4	50		67	33.5	25	80
20	10	12	50		68	34	25	82
21	10.5	12.6	50		69	34.5	25	83
22	11	13.2	50		70	35	25	84
23	11.5	13.8	50		71	35.5	25	85
24	12	14.4	50		72	36	25	86
25	12.5	15	50		73	36.5	25	88
26	13	15.6	50		74	37	25	89
27	13.5	16.2	50		75	37.5	25	90
28	14	16.8	50		76	38	25	91
29	14.5	17.4	50		77	38.5	25	92
30	15	18	50		78	39	25	94
31	15.5	18.6	50		79	39.5	25	95
32	16	19.2	50		80	40	25	96
33	16.5	19.8	50		81	40.5	25	97
34	17	20.4	50		82	41	25	98
35	17.5	21	50		83	41.5	25	100
36	18	21.6	50		84	42	25	101
37	18.5	22.2	50		85	42.5	25	102
38	19	22.8	50		86	43	25	103
39	19.5	23.4	50		87	43.5	25	104
40	20	24	50		88	44	25	106
41	20.5	24.6	50		89	44.5	25	107
42	21	25	50		90	45	25	108
43	21.5	25	52		91	45.5	25	109
44	22	25	53		92	46	25	110
45	22.5	25	54		93	46.5	25	112
46	23	25	55		94	47	25	113
47	23.5	25	56		95	47.5	25	114
48	24	25	58		96	48	25	115
49	24.5	25	Country of registration Испания Active substance Velmanasa alfa Prescription required Yes Manufacturer Chiesi Farmaceutici S.P.A. Composition Fosfato disodico dihidratado hidrogenado (3,1 MG mg mg), Dihidrogenofosfato de sodio dihidrato (0,115 MG mg mg), Manitol (e-421) (227,5 MG mg mg) This information is for reference only and does not constitute medical advice. Always consult a licensed doctor before taking any medication. Oladoctor is not responsible for medical decisions based on this content. Talk to a doctor online Have questions about this medication or your symptoms? Connect with a licensed doctor for guidance and personalized care. 5.01 review Анна Морет Дерматология18 years of experience Анна Морет — врач-дерматолог и дерматовенеролог с международной сертификацией. Специализируется на дерматологии взрослых и детей, венерологии, эстетическом уходе за кожей и общей медицине. 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