What it is and what it is used for

for Lamzede contains the active substance velmanase alfa which belongs to a group of medicines known as enzyme replacement therapies. It is used to treat patients with mild to moderate alpha-mannosidosis disease. It is given for the treatment of non-neurological symptoms of the disease. Alpha-mannosidosis disease is a rare genetic disorder caused by a lack of an enzyme named alpha-mannosidase, which is needed to break down certain sugar compounds (called 'mannose‐rich oligosaccharides') in the body. When this enzyme is missing or does not work properly, these sugar compounds build up inside cells and cause the signs and symptoms of the disease. The typical manifestations of the disease include distinctive facial features, mental retardation, difficulty in controlling movements, difficulties in hearing and speaking, frequent infections, skeletal problems, muscle pain and weakness. Velmanase alfa is designed to replace the missing enzyme, in patients with alpha-mannosidosis disease.

What you need to know before you take it

e Lamzede Do not use Lamzede: if you are allergic to velmanase alfa or any of the other ingredients of this medicine (listed in section 6). Warnings and precautions Talk to your doctor before Lamzede is used. Hypersensitivity reactions may occur with the administration of Lamzede. These reactions usually appear during or soon after the infusion and may manifest with several symptoms, such as localised or diffuse skin reactions, gastrointestinal symptoms or swelling of the throat, face, lips or tongue (see section 4 "Possible side effects"). If the hypersensitivity reaction is severe, immediate discontinuation of Lamzede is recommended and current medical standards for emergency treatment are to be followed. Less severe hypersensitivity reactions may be managed by temporary interruption of the infusion or by slowing down infusion rate; administration of medicines used to treat allergy may be considered by the physician. If you are treated with Lamzede, you may experience a side effect during or immediately following the drip (infusion) used to give the medicine (see section 4 "Possible side effects"). This is known as an infusion-related reaction and can sometimes be severe. Your doctor may decide to keep you under observation for one hour or longer after the infusion in relation to the infusion related reactions. Infusion-related reactions include dizziness, headache, nausea, low or high blood pressure, tiredness and fever. If you experience an infusion-related reaction, you must tell your doctor immediately. If you have an infusion-related reaction you may be given additional medicines to treat or help prevent future reactions. These medicines may include medicines used to treat allergies (antihistamines), medicines used to treat fever (antipyretics) and medicines to control inflammation (corticosteroids). If the infusion-related reaction is severe, your doctor will stop the infusion immediately and start giving you appropriate medical treatment. If the infusion-related reactions are severe and/or there is a loss of effect from this medicine, your doctor will perform a blood test to check for antibodies that might affect the outcome of your treatment. Most of the time you can still be given 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 developed antibodies against Lamzede during its clinical development, no clear correlation was found between antibody titres and reduction in efficacy or occurrence of hypersensitivity reactions. Other medicines and Lamzede Tell your doctor if you are taking, have recently taken or might take any other medicines. Pregnancy and breast-feeding If you are pregnant or breast-feeding, think you may be pregnant or are planning to have a baby, ask your doctor for advice before this medicine is used. You should not take this medicine during pregnancy unless your doctor states it is clearly necessary. Your doctor will discuss that with you. It is not known whether velmanase alfa passes into breast milk. Lamzede can be used during breast-feeding since the velmanase alfa will not be absorbed by a breast-fed child. Driving and using machines Lamzede has no or negligible influence on the ability to drive and use machines. Lamzede contains sodium This medicine contains less than 1 mmol sodium (23 mg) per dose unit, that is to say essentially 'sodium-free'.

How to take it

Lamzede This medicine is only to be used under the supervision of a doctor experienced in the treatment of alphamannosidosis or other similar diseases and should only be given by a healthcare professional. Lamzede is only used under the supervision of a doctor who is knowledgeable in the treatment of Alpha Mannosidosis disease. Your doctor may advise that you can be treated at home provided you meet certain criteria. Please contact your doctor if you would like to be treated at home. Dose The recommended dose of Lamzede is 1 mg/kg of body weight given once every week. Use in children and adolescents Lamzede may be given to children and adolescents at the same dose and frequency as in adults. Administration Lamzede is supplied in a vial as a powder for infusion which will be made up with water for injections before being given. Once it has been made up, the medicine will be given by infusion pump (drip) into a vein over a period of at least 50 minutes under your doctor's supervision. If you have any further questions on the use of this medicine, ask your doctor, nurse or pharmacist.

4. Possible side effects Like all medicines, this medicine can cause side effects, although not everybody gets them. Most side effects occur during the infusion or shortly after ("infusion-related reaction", see section 2 "Warnings and precautions"). While under treatment with Lamzede, you may experience some of the following reactions: Serious side effects Common side effects (may affect up to 1 in 10 people) loss of consciousness (fainting, which may be preceded by feeling dizzy, lightheaded or confused) acute renal insufficiency (kidney problems which can be recognised from fluid retention, swelling in legs, ankles or feet, drowsiness, shortness of breath or fatigue) hypersensitivity and serious allergic reaction (symptoms including localised or diffuse skin itching, dizziness, difficulty breathing, chest pain, chills, fever, gastrointestinal symptoms such as nausea, vomiting, diarrhoea or intestinal pain, swelling of the throat, face, lips or tongue) If you experience any side effect like these, please tell your doctor immediately. Other side effects Very common side effects (may affect more than 1 in 10 people) diarrhoea weight increase fever/increased body temperature Common side effects (may affect up to 1 in 10 people) low heart beat (bradycardia) blue skin and lips (cyanosis) psychotic behaviour (mental illness with hallucinations, difficulty in thinking clearly and understanding reality, anxiety), initial difficulty in sleeping confused state, fainting, tremor, dizziness, headache intestinal (abdominal) pain, irritation of the stomach caused by digestive acids (reflux gastritis), nausea, vomiting pain at the site the infusion is given, chills, feeling hot, malaise, tiredness (fatigue) skin rashes (urticaria), increased sweating (hyperhidrosis) nosebleed joint pain, back pain, joint stiffness, muscle pain, pain in extremity (hands, feet) eye irritation, eye swelling (eyelid oedema), eye redness increased appetite

Possible side effects

• frequency not known (frequency cannot be estimated from the available data) infection of the inner wall of the sac around the heart (endocarditis) furuncle infection caused by a bacteria called Staphylococcus decreased appetite agitation, stool soiling, nervousness inability to coordinate muscle movements somnolence increased lacrimation deafness aortic valve incompetence (a condition in which the aortic valve does not close tightly) fast and/or rapid heart beat low blood pressure vascular fragility oropharyngeal pain wheezing painful swallowing reddening of the skin joint swelling, joint warmth weakness symptoms of a mild infusion related reaction such as paleness, lack of interest and energy and reduced muscle strength or tension Reporting of side effects If you get any side effects, talk to your doctor. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via: Yellow Card Scheme, Website: www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store By reporting side effects you can help provide more information on the safety of this medicine.

How to store it

Lamzede 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 label and the carton after 'EXP'. The expiry date refers to the last day of that month. Chiesi item

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LEAFLET LAMZEDE 10mg UK

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Store and transport refrigerated (2°C ‐ 8°C). Do not freeze. Store in the original package in order to protect from light. After reconstitution, the medicine should be used immediately. If not used immediately, the reconstituted solution may be stored up to 24 hours at 2°C to 8°C. This medicine must not be used if the reconstituted solution contains opaque particles or is discoloured. 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 Lamzede contains The active substance is velmanase alfa. One vial contains 10 mg of velmanase alfa. After reconstitution, one mL of the solution contains 2 mg of velmanase alfa (10 mg / 5 mL). The other ingredients are: disodium phosphate dihydrate, sodium dihydrogen phosphate dihydrate (see section 2 "Lamzede contains sodium"), mannitol (E421) and glycine. What Lamzede looks like and contents of the pack Lamzede is a white to off‐white powder for solution for infusion, supplied in a glass vial. Each carton contains 1, 5 or 10 vials. Not all pack sizes may be marketed. Marketing Authorisation Holder Chiesi Limited 333 Styal Road Manchester M22 5LG UK Manufacturer Chiesi Farmaceutici S.p.A. Via San Leonardo, 96 43122 Parma Italy This leaflet was last revised in March 2026. 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 Medicines and Healthcare products Regulatory Agency will review any new information on this medicine every year and this leaflet will be updated as necessary. Is this leaflet hard to see or read? Phone 0161 488 5555 for help. ___________________________________________________________________ The following information is intended for healthcare professionals only. Lamzede requires reconstitution and is intended for intravenous infusion only. Each vial is for single use only. Instructions for reconstitution and administration Lamzede should be reconstituted and administrated by a healthcare professional. Aseptic technique is to be used during preparation. Filter needles must 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 1 mg/kg is determined using the following calculation: Patient's weight (kg) x dose (mg/kg) = Patient dose (in mg). Patient dose (in mg) divided by 10 mg/vial (content of one vial) = number of vials to reconstitute. If the number of calculated vials includes a fraction, it should be rounded up to the next whole number. Approximately 30 minutes prior to reconstitution, the required number of vials should be removed from the refrigerator. The vials should reach ambient temperature (between 15°C and 25°C) prior to reconstitution. Each vial is reconstituted by slowly injecting 5 mL of water for injections to the inside of the wall of each vial. Each mL of reconstituted solution contains 2 mg of velmanase alfa. Only the volume corresponding to the recommended dose should be administered. Example: Patient's weight (44 kg) x dose (1 mg/kg) = Patient dose (44 mg). 44 mg divided by 10 mg/vial = 4.4 vials, therefore, 5 vials should be reconstituted. From the total reconstituted volume, only 22 mL (corresponding to 44 mg) should be administered. b) The powder should be reconstituted in the vial by a slow drop-wise addition of the water for injections down the inside of the vial and not directly onto the lyophilized powder. Forcefully ejecting the water for injections from the syringe onto the powder should be avoided to minimise foaming. The reconstituted vials should stand on the table for about 5-10 minutes. Thereafter each vial should be tilted and rolled gently for 15-20 seconds to enhance the dissolution process. The vial should not be inverted, swirled, or shaken. c) An immediate visual inspection of the solution for particulate matter and discoloration should be performed after reconstitution. The solution should be clear and not used if opaque particles are observed or if the solution is discoloured. Due to the nature of the medicinal product, the reconstituted solution may occasionally contain some proteinaceous particles in form of thin white strands or translucent fibers which will be removed by the in-line filter during infusion (see point e). d) The reconstituted solution is to be slowly withdrawn from each vial with caution to avoid foaming in the syringe. If the volume of the solution exceeds one syringe capacity, the required number of syringes should be prepared in order to replace the syringe quickly during the infusion. e) The reconstituted solution should be administered using an infusion set equipped with a pump and an in-line low protein-binding 0.22 μm filter. The total volume of infusion is determined by the patient's weight and should be administrated over a minimum of 50 minutes. It is recommended to use always the same dilution (2 mg/ml). For patients weighing less than 18 kg, and receiving less than 9 mL reconstituted solution, the infusion rate should calculated so that the infusion time is ≥50 minutes. The maximum infusion rate is 25 mL/hour. The infusion time can be calculated from the following table: Patient weight (kg)

Dose (mL)

Maximum infusion rate (mL/h)

Minimum infusion time (min)

Patient weight (kg)

Dose (mL)

Maximum infusion rate (mL/h)

Minimum infusion time (min)

5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52

2.5 3 3.5 4 4.5 5 5.5 6 6.5 7 7.5 8 8.5 9 9.5 10 10.5 11 11.5 12 12.5 13 13.5 14 14.5 15 15.5 16 16.5 17 17.5 18 18.5 19 19.5 20 20.5 21 21.5 22 22.5 23 23.5 24 24.5 25 25.5 26

3 3.6 4.2 4.8 5.4 6 6.6 7.2 7.8 8.4 9 9.6 10.2 10.8 11.4 12 12.6 13.2 13.8 14.4 15 15.6 16.2 16.8 17.4 18 18.6 19.2 19.8 20.4 21 21.6 22.2 22.8 23.4 24 24.6 25 25 25 25 25 25 25 25 25 25 25

50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 50 52 53 54 55 56 58 59 60 61 62

53 54 55 56 57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99

26.5 27 27.5 28 28.5 29 29.5 30 30.5 31 31.5 32 32.5 33 33.5 34 34.5 35 35.5 36 36.5 37 37.5 38 38.5 39 39.5 40 40.5 41 41.5 42 42.5 43 43.5 44 44.5 45 45.5 46 46.5 47 47.5 48 48.5 49 49.5

25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25 25

64 65 67 67 68 70 71 72 73 74 76 77 78 79 80 82 83 84 85 86 88 89 90 91 92 94 95 96 97 98 100 101 102 103 104 106 107 108 109 110 112 113 114 115 116 118 119

f) When the last syringe is empty, the dose syringe is replaced with a 20 mL syringe filled with sodium chloride 9 mg/mL (0.9%) solution for injection. A volume of 10 mL sodium chloride solution should be administered through the infusion system to infuse the remaining fraction of Lamzede in the line to the patient. Disposal Any unused medicinal product or waste material should be disposed of in accordance with local requirements.

CP0064-8

Chiesi item

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