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Nexviadyme is a brand name for Avalglucosidase Alfa. The medicine, its uses, side effects and dosage are the same regardless of brand.
Used for: Nexviadyme (avalglucosidase alfa) is indicated for long-term enzyme replacement therapy for the treatment of patients with Pompe disease (acid α-glucosidase deficiency).
Verbatim from this product's EMA label. Tap a section to expand.
Nexviadyme treatment should be supervised by a physician experienced in the management of patients with Pompe disease or other inherited metabolic or neuromuscular diseases. Posology Patients may be pre-treated with antihistamines, antipyretics, and/or corticosteroids to prevent or reduce allergic reactions.
The recommended dose of avalglucosidase alfa is 20 mg/kg of body weight administered once every 2 weeks. , severe hypersensitivity, anaphylactic reactions, or risk of fluid overload). , severe hypersensitivity, anaphylactic reactions, or risk of fluid overload), consider decreasing the dose to 20 mg/kg every other week.
4). Special populations Elderly patients No dose adjustment is required in patients >65 years. Hepatic impairment The safety and efficacy of avalglucosidase alfa in patients with hepatic impairment have not been evaluated and no specific dose regimen can be recommended for these patients.
Renal impairment No dose adjustment is required in patients with mild renal impairment. The safety and efficacy of avalglucosidase alfa in patients with moderate or severe renal impairment have not been evaluated and no specific dose regimen can be recommended for these patients.
2). Paediatric population (patients 6 months of age and younger) The safety and efficacy of avalglucosidase alfa in children 6 months of age and younger have not yet been established. There are no data available in patients 6 months of age and younger.
Method of administration Nexviadyme vials are for single use only and the medicinal product should be administered as an intravenous infusion. The infusion should be administered incrementally as determined by patient response and comfort.
It is recommended that the infusion begins at an initial rate of 1 mg/kg/hour and is gradually increased every 30 minutes if there are no signs of infusion-associated reactions (IARs), in accordance with Table 1. Vital signs should be obtained at each step, before increasing the infusion rate.
8 mg/kg/hour can be applied. b For IOPD patients who experience lack of improvement a dose increase to 40 mg/kg every other week is recommended. 6 mg/kg/hour can be applied. In the event of anaphylaxis or severe hypersensitivity reaction or severe IARs, administration of Nexviadyme should be immediately discontinued and appropriate medical treatment should be initiated.
7% of patients each were headache, dyspnoea, hypoxia, tongue oedema, nausea, pruritis, urticaria, skin discoloration, chest discomfort, pyrexia, blood pressure increased or decreased, body temperature increased, heart rate increased, and oxygen saturation decreased.
4% in patients. 8% of patients each discontinued the treatment because of the following events considered to be related to Nexviadyme: respiratory distress, chest discomfort, dizziness, cough, nausea, flushing, ocular hyperaemia, urticaria, and erythema.
6%). The pooled safety analysis from 4 clinical studies (EFC14028/COMET, ACT14132/mini-COMET, TDR12857/NEO, and LTS13769/NEO-EXT) included a total of 142 patients (118 adult and 24 paediatric patients (1 paediatric patient directly enrolled in the open-label extension period of Study 1)) treated with Nexviadyme.
ADRs reported in patients treated with Nexviadyme in the pooled analysis of clinical studies are listed in Table 2. Tabulated list of adverse reactions Adverse reactions per System Organ Class, presented by frequency categories: very common (≥1/10), common (≥1/100 to <1/10), uncommon (≥1/1,000 to <1/100), rare (≥1/10,000 to <1/1,000), very rare (<1/10,000) and not known (cannot be estimated from the available data).
Due to the small patient population, an adverse reaction reported in 2 patients is classified as common. Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness. Table 2 – Adverse reactions occurring in patients treated with Nexviadyme in a pooled analysis of clinical studies (N=142) System organ class Frequency Preferred term Infections and infestations Uncommon Conjunctivitis Immune Disorders Very common Common Hypersensitivity Anaphylaxis Nervous system disorders Very common Common Common Common Common Uncommon Headache Dizziness Tremor Somnolence Burning sensation Paraesthesia Eye Disorders Common Common Common Common Uncommon Ocular hyperaemia Conjunctival hyperaemia Eye pruritus Eyelid oedema Lacrimation increased Cardiac Disorders Common Uncommon Tachycardia Ventricular extrasystoles Vascular Disorders Common Common Common Common Common Common Hypertension Flushing Hypotension Cyanosis Hot flush Pallor Respiratory, thoracic, and mediastinal disorders Common Common Common Common Common Uncommon Uncommon Cough Dyspnoea Respiratory distress Throat irritation Oropharyngeal pain Tachypnoea Laryngeal oedema Gastrointestinal disorders Very common Common Common Common Common Common Common Nausea Diarrhoea Vomiting Lip swelling Swollen tongue Abdominal pain Abdominal pain upper 8 System organ class Frequency Preferred term Common Uncommon Uncommon Uncommon Dyspepsia Hypoaesthesia oral Paraesthesia oral Dysphagia Skin and subcutaneous tissue disorders Very common Very common Common Common Common Common Common Common Common Uncommon Uncommon Pruritus Rash Urticaria Erythema Palmer erythema Hyperhidrosis Rash erythematous Rash pruritic Skin plaque Angioedema Skin discolouration Musculoskeletal and connective tissue disorders Common Common Common Common Muscle spasms Myalgia Pain in extremity Flank pain General disorders and administration site conditions Common Common Common Common Common Common Common Common Common Common Common Common Uncommon Uncommon Uncommon Uncommon Uncommon Uncommon Uncommon Uncommon Uncommon Fatigue Chills Chest discomfort Pain Influenza-like illness Infusion site pain Pyrexia Asthenia Face oedema Feeling cold Feeling hot Sluggishness Facial pain Hyperthermia Infusion site extravasation Infusion site joint pain Infusion site rash Infusion site reaction Infusion site urticaria Localized oedema Peripheral swelling Investigation Common Common Common Uncommon Uncommon Uncommon Uncommon Blood pressure increased Oxygen saturation decreased Body temperature increase Heart rate increased Breath sounds abnormal Complement factor increased Immune complex level increased Table 2 includes treatment related adverse events that are considered biologically plausibly related to avalglucosidase alfa based on the alglucosidase alfa SmPC.
Traceability In order to improve the traceability of biological medicinal products, the name and the batch number of the administered product should be clearly recorded. 8). Appropriate medical support measures, including cardiopulmonary resuscitation equipment especially for patients with cardiac hypertrophy and patients with significantly compromised respiratory function, should be readily available when Nexviadyme is administered.
If severe hypersensitivity or anaphylaxis occur, Nexviadyme should be discontinued immediately, and appropriate medical treatment should be initiated. The risks and benefits of re-administering Nexviadyme following anaphylaxis or severe hypersensitivity reaction should be considered.
Some patients have been re-challenged using slower infusion rates at a dose lower than the recommended 5 dose. In patients with severe hypersensitivity, desensitization procedure to Nexviadyme may be considered. If the decision is made to re-administer the medicinal product, extreme caution should be exercised, with appropriate resuscitation measures available.
Once a patient tolerates the infusion, the dose may be increased to reach the approved dose. If mild or moderate hypersensitivity reactions occur, the infusion rate may be slowed or temporarily stopped. 8). Patients with an acute underlying illness at the time of Nexviadyme infusion appear to be at greater risk for IARs.
Patients with advanced Pompe disease may have compromised cardiac and respiratory function, which may predispose them to a higher risk of severe complications from IARs. Antihistamines, antipyretics, and/or corticosteroids can be given to prevent or reduce IARs.
However, IARs may still occur in patients after receiving pre-treatment. If severe IARs occur, immediate discontinuation of the administration of Nexviadyme should be considered and appropriate medical treatment should be initiated.
1 when re-challenge was unsuccessful. 8)
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4). Symptoms may persist despite temporarily stopping the infusion; therefore, the treating physician should wait at least 30 minutes for symptoms of the reactions to resolve before deciding to stop the infusion for the remainder of the day.
If symptoms subside, infusion rate should be resumed for 30 minutes at half the rate, or less, of the rate at which the reactions occurred, followed by an increase in infusion rate by 50% for 15 to 30 minutes. If symptoms do not recur, the infusion rate should be increased to the rate at which the reactions occurred and consider continuing to increase the rate in a stepwise manner until the maximum rate is achieved.
4 Home infusion Infusion of Nexviadyme at home may be considered for patients who are tolerating their infusions well and have no history of moderate or severe IARs for a few months. The decision to have a patient move to home infusion should be made after evaluation and upon recommendation by the treating physician.
A patient’s underlying co-morbidities and ability to adhere to the home infusion requirements need to be taken into account when evaluating the patient for eligibility to receive home infusion. The following criteria should be considered: • The patient must have no ongoing concurrent condition that, in the opinion of the physician, may affect patient’s ability to tolerate the infusion.
• The patient is considered medically stable. A comprehensive evaluation must be completed before the initiation of home infusion. • The patient must have received Nexviadyme infusions supervised by a physician with expertise in management of Pompe patients for a few months that could be in a hospital or in another appropriate setting of outpatient care.
Documentation of a pattern of well-tolerated infusions with no IARs, or mild IARs that have been controlled with premedication, is a prerequisite for the initiation of home infusion. • The patient must be willing and able to comply with home infusion procedures.
• Home infusion infrastructure, resources, and procedures, including training, must be established and available to the healthcare professional. The healthcare professional should be available at all times during the home infusion and a specified time after infusion, depending on patient’s tolerance prior to starting home infusion.
4). Subsequent infusions may need to occur in a hospital or in an appropriate setting of […]
In a comparative study, EFC14028/COMET, 100 LOPD (late-onset Pompe disease) patients aged 16 to 78 naïve to enzyme replacement therapy were treated either with 20 mg/kg of Nexviadyme (n=51) or 20 mg/kg of alglucosidase alfa (n=49).
1% of those treated with alglucosidase alfa. 2% patients receiving alglucosidase alfa in the study permanently discontinued treatment due to adverse reactions; none of the patients from the Nexviadyme group permanently discontinued the treatment.
The most frequently reported ADRs (>5%) in patients treated with Nexviadyme were headache, nausea, pruritus, urticaria, and fatigue. 9 The 95 patients who entered open-label extension period of EFC14028/COMET consisted of 51 patients who continued treatment with Nexviadyme and 44 patients who switched from alglucosidase alfa to Nexviadyme.
8%) patients who switched to Nexviadyme. The most frequently reported adverse reactions (>5%) by patients continuing Nexviadyme treatment throughout the study were nausea, chills, […]
The benefits and risks of re- administering Nexviadyme following severe IARs should be considered. Some patients have been re- challenged using slower infusion rates at a dose lower than the recommended dose. Once a patient tolerates the infusion, the dose may be increased to reach the approved dose.
8). 8). IARs and hypersensitivity reactions may occur independent of the development of ADA. The majority of IARs and hypersensitivity reactions were mild or moderate and were managed with standard clinical practices. 8). ADA testing may be considered if patients do not respond to therapy.
Adverse-event-driven immunologic testing, including IgG and IgE ADA, may be considered for patients who have risk for allergic reaction or previous anaphylactic reaction to alglucosidase alfa. Contact your local Sanofi representative or Sanofi EU Medical Services for information on the Sanofi Speciality Care testing services.
Risk of acute cardiorespiratory failure Caution should be exercised when administering Nexviadyme to patients susceptible to fluid volume overload or patients with acute underlying respiratory illness or compromised cardiac and/or respiratory function for whom fluid restriction is indicated.
These patients may be at risk of serious exacerbation of their cardiac or respiratory status during infusion. Appropriate medical support and monitoring measures should be readily available during Nexviadyme infusion, and some patients may require prolonged observation times that should be based on the individual needs of the patient.
6 Cardiac arrhythmia and sudden death during general anaesthesia for central venous catheter placement Caution should be used when administering general anaesthesia for the placement of a central venous catheter or for other surgical procedures in patients with IOPD with cardiac hypertrophy.
Cardiac arrhythmia, including ventricular fibrillation, ventricular tachycardia, and bradycardia, resulting in cardiac arrest or death, or requiring cardiac resuscitation or defibrillation, have been associated with the use of general anaesthesia in IOPD patients with cardiac hypertrophy.