What is Naglazyme and how does it work?
- Naglazyme (galsulfase) is an enzyme used to treat some of the symptoms of a genetic condition called Maroteaux-Lamy syndrome (mucopolysaccharidosis).
- Naglazyme improves walking and stair-climbing capacity. Naglazyme is indicated for patients with Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome).
What are the side effects of Naglazyme?
What is the dosage for Naglazyme?
- The recommended dosage regimen of Naglazyme is 1 mg per kg of body weight administered once weekly as an intravenous infusion.
- Pretreatment with antihistamines with or without antipyretics is recommended 30 to 60 minutes prior to the start of the infusion.
- The total volume of the infusion should be delivered over a period of time of no less than 4 hours.
- Naglazyme should be diluted with 0.9% Sodium Chloride Injection, USP, to a final volume of 250 mL and delivered by controlled intravenous infusion using an infusion pump.
- The initial infusion rate should be 6 mL per hour for the first hour.
- If the infusion is well tolerated, the rate of infusion may be increased to 80 mL per hour for the remaining 3 hours.
- The infusion time can be extended up to 20 hours if infusion reactions occur.
- For patients 20 kg and under or those who are susceptible to fluid volume overload, physicians may consider diluting Naglazyme in a volume of 100 mL.
- The infusion rate (mL per hour) should be decreased so that the total infusion duration remains no less than 4 hours.
- Each vial of Naglazyme provides 5 mg of galsulfase (expressed as protein content) in 5 mL of solution and is intended for single use only.
- Do not use the vial more than one time.
- The concentrated solution for infusion must be diluted with 0.9% Sodium Chloride Injection, USP, using aseptic techniques.
- Prepare Naglazyme using low-protein-binding containers and administer the diluted Naglazyme solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 µm in-line filter.
- There is no information on the compatibility of diluted Naglazyme with glass containers.
Is Naglazyme safe to take when pregnant or breastfeeding?
- Available data from case reports and postmarketing experience with Naglazyme use in pregnant women are insufficient to evaluate for a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes.
- There are no data on the presence of galsulfase in human milk, the effects on the breastfed infant, or the effects on milk production. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for Naglazyme and any potential adverse effects on the breastfed infant from Naglazyme or from the underlying maternal condition.
Naglazyme (galsulfase) is an enzyme used to treat some of the symptoms of a genetic condition called Maroteaux-Lamy syndrome (mucopolysaccharidosis). Naglazyme improves walking and stair-climbing capacity. Naglazyme is indicated for patients with Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome).
Related Disease Conditions
The definition of a genetic disease is a disorder or condition caused by abnormalities in a person's genome. Some types of genetic inheritance include single inheritance, including cystic fibrosis, sickle cell anemia, Marfan syndrome, and hemochromatosis. Other types of genetic diseases include multifactorial inheritance. Still other types of genetic diseases include chromosome abnormalities (for example, Turner syndrome, and Klinefelter syndrome), and mitochondrial inheritance (for example, epilepsy and dementia).
MPS I (Mucopolysaccharidosis Type I, Hurler Syndrome)
MPS I (also referred to as mucopolysaccharidosis type I or Hurler syndrome) is a genetic, inherited condition that involves chromosome number 4. Symptoms of MPS I include thick lips, eye problems, chronic nasal discharge, enlarged spleen or other abdominal organs, joint stiffness, coarsening of facial features. There is no cure for MPS I, but signs and symptoms may be managed with enzyme replacement therapy and surgery to improve symptoms.
Hunter Syndrome (Mucopolysaccharidosis II, MPS II)
Hunter syndrome (mucopolysaccharidosis II, MPS II) is an inherited or genetic disease. Signs and symptoms of Hunter syndrome include diarrhea, voice changes, facial changes, and distended abdomen. Treatment for Hunter syndrome is geared toward symptoms of the disease with idursulfase or Elaprase (enzyme replacement therapy).
Your health care provider may refer you to a genetic professional. Universities and medical centers also often have affiliated genetic professionals, or can provide referrals to a genetic professional or genetics clinic. Genetic counseling provides patients and family members the tools to make the right choice in regard to test for a disease or condition.
Genetic Testing: Families With Breast Cancer
Breast cancer can be a killer and the decision to get tested to see if a patient is prone to the disease should be discussed with a doctor -- particularly if the woman has a history of breast cancer in her family. Genetic testing can only tell so much about breast cancer risk, however.
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