Pegvaliase-pqpz injection for subcutaneous use was approved by FDA in May 2018 for treating phenylketonuria (PKU), a rare genetic disease in adults.
Pegvaliase-pqpz is a phenylalanine-metabolizing enzyme prescribed for treating adult patients suffering from phenylketonuria (a rare inherited disorder); it acts by decreasing the high concentrations of phenylalanine in the blood.
Pegvaliase-pqpz is recommended mainly for phenylketonuria patients who show uncontrolled phenylalanine concentrations in blood (i.e., more than 600 micromol/L).
Pegvaliase-pqpz should not be used in patients with-
• Allergy to pegvaliase-pqpz
• Pregnancy and breastfeeding
• Children below 18 years of age and patients above 65 years (due to the lack of safety and effectiveness data in these groups)
Caution is required to use the drug in patients with severe arthritis and other joint disorders.
• The recommended initial dose of pegvaliase-pqpz is 2.5 mg taken once a week for four weeks.
• The initial dose should be administered only under the supervision of the physician to manage any unexpected allergic or hypersensitivity reactions.
• The dose of pegvaliase-pqpz injection can be increased gradually based on the patient's tolerability to pegvaliase-pqpz for at least five weeks.
• The maintenance dose of pegvaliase-pqpz injection is 20 mg once daily for 24 weeks.
• The maximum dose of pegvaliase-pqpz injection is 40 mg once a day for 16 weeks.
• The maximum dose should be prescribed only to patients who have been kept on a maintenance dose and have not shown a decrease of either 20% of phenylalanine in blood or a concentration less than or equal to 600 micromol/L.
• If a patient does not show a better response or any improvement after 16 weeks of maximum dose (40mg once daily), treatment should be discontinued and the patient should be further evaluated clinically.
• Dose reduction and advice regarding the intake of dietary protein and phenylalanine must be given to the patient if the phenylalanine concentrations go below 30 micromol/L.
• Initial administration and readministration of pegvaliase-pqpz after an anaphylaxis should be supervised by a healthcare provider for at least a period of 60 minutes after an injection.
• Pegvaliase-pqpz injection comes as a pre-filled syringe intended for single use and should be administered subcutaneously or just under the skin.
• Injection should be inspected for the presence of particulate matter or any discoloration before administration and should be discarded immediately if such things are present.
• The preferred sites for pegvaliase-pqpz injection are front middle thigh, abdomen, buttocks, and back of the upper arm. Inject 2 inches away from the navel area.
• Treatment with pegvaliase-pqpz can be discontinued after 16 weeks of regular treatment if no improvement is seen even with the maximum daily dose of 40 mg.
• The concentration of phenylalanine in blood should be monitored initially for four weeks followed by periodical assessment when a maintenance dosage is established.
• Patients should be counseled about the intake of dietary protein and phenylalanine based on their blood phenylalanine concentrations throughout the treatment.
• Patients can take anti-allergy and fever medications before injecting pegvaliase-pqpz to minimize the hypersensitivity or allergic reactions caused by the injection.
• In case of severe allergic reactions, patients should be administered an epinephrine auto-injection immediately, and this medical emergency should be managed appropriately.
• The patient or the caregiver should be advised to consult the physician if any unexpected side effects occur.
• Gastrointestinal: Nausea, vomiting, pain in the stomach, loose stools
• Nervous system: Headache, dizziness, anxiety or nervousness
• Respiratory system: Throat pain, cough, stuffy nose
• Skin: Injection site reactions (pain, swelling, discoloration), dry skin, rashes, dermatitis, pruritus, hair loss
• Musculoskeletal: Back pain, neck pain, pain in the extremities, muscle stiffness, joint pain or swelling, stiffness of the joints
• If a dose is missed, the patients should be instructed to take the next dose at the next scheduled time and not to take double the dose.
• Before injecting pegvaliase-pqpz, it is necessary to allow the injection to come to room temperature for at least 30 minutes; however, do not heat in a microwave oven or put in boiling water with the intention of warming the injection.
• Never inject pegvaliase-pqpz into scars, moles, birthmarks, and areas of red, tender, bruised, inflamed or tattooed skin.
• Medroxyprogesterone injections should not be used along with pegvaliase-pqpz as severe allergic reactions may occur; therefore should be avoided.
• The prescriber must be informed prior about the patient's medication history of taking prescription or non-prescription drugs and other herbal supplements to avoid dangerous drug interactions.
• Store pegvaliase-pqpz in the refrigerator at 2°C to 8°C.
• Pegvaliase-pqpz injections, if necessary can also be stored at room temperature for about 30 days.
• The date of removal should be mentioned in the injection pack and should be discarded after 30 days if not used.
• Do not freeze or shake the pegvaliase-pqpz injection.
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