Naglazyme Concentrated Solution for Intravenous Infusion 1mg1ml

Land: Malaysia

Språk: engelska

Källa: NPRA (National Pharmaceutical Regulatory Agency, Bahagian Regulatori Farmasi Negara)

Aktiva substanser:

GALSULFASE

Tillgänglig från:

PHARM-D SDN. BHD.

INN (International namn):

GALSULFASE

Enheter i paketet:

5ml mL

Tillverkad av:

BioMarin International Limited

Bipacksedel

NAGLAZYME
®
_ _
Concentrated Solution for Intravenous Infusion
Galsulfase (1mg/1 ml)
1
_Consumer Medication Information Leaflet (RiMUP)_
WHAT IS IN THIS LEAFLET
1.
What Naglazyme is used for
2.
How Naglazyme works
3.
Before you use Naglazyme
4.
How to use Naglazyme
5.
While you are using
Naglazyme
6.
Side effects
7.
Storage and disposal of
Naglazyme
8.
Product description
9.
Manufacturer and Product
Registration Holder
10.
Date of revision
WHAT NAGLAZYME IS USED FOR
Naglazyme is used to treat
patients with
Mucopolysaccharidosis VI (MPS
VI, Maroteaux-Lamy syndrome),
a rare lysosomal storage disease
(LSD).
People with MPS VI disease have
either a low level or no level of
activity of an enzyme called
_N-acetylgalactosamine 4-_
_sulfatase_, which breaks down
specific substances
(glycosaminoglycans) in the body.
As a result, these substances do
not get broken down and
processed by the body as they
should. They accumulate in many
tissues in the body, leading to a
progressive disorder with multiple
organ and tissue involvement.
HOW NAGLAZYME WORKS
This medicine contains a
recombinant enzyme called
galsulfase. This can replace the
natural enzyme which is lacking
in MPS VI patients. Treatment
has been shown to improve
walking and stair-climbing ability,
and to reduce the levels of
glycosaminoglycans in the body.
BEFORE YOU USE NAGLAZYME
-
_When you must not use it_
Your doctor will tell you if you
should not be treated with
Naglazyme.
_ _
-
_Before you start to use it _
Tell your doctor if you have or
have had any medical conditions,
especially if you:

Have ever had an allergic
reaction to galsulfase or any
other ingredients of
Naglazyme.

Have sleep apnea (a condition
in which breathing stops for a
short time or becomes
shallow during sleep)

Have heart disease

Have lung disease or breathing
problems

Have a fever, cough, or cold

Are pregnant or planning to
become pregnant.

Are breast-feeding or planning
to breast-feed
-
_Taking other medicines_
Tell your doctor or nurse if you or
your child are

Produktens egenskaper

FULL PRESCRIBING INFORMATION
INDICATIONS AND USAGE
NAGLAZYME®
(galsulfase)
is
indicated
for
patients
with
Mucopolysaccharidosis
VI
(MPS
VI,
Maroteaux-Lamy
syndrome).
NAGLAZYME
has
been
shown
to
improve
walking
and
stair-climbing
capacity.
DOSAGE AND ADMINISTRATION
RECOMMENDED DOSE
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 _ [see
_
_Warnings and Precautions]._
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 [_see Warnings and Precautions and Adverse Reactions_]. 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.
INSTRU