STATE OF NEW JERSEY
PRE-FILED FOR INTRODUCTION IN THE 2014 SESSION
Senator NELLIE POU
District 35 (Bergen and Passaic)
"Let Them Be Little Act"; provides for screening newborn infants for Hunter syndrome.
CURRENT VERSION OF TEXT
Introduced Pending Technical Review by Legislative Counsel
An Act concerning screening for Hunter syndrome in newborn infants, designated as the "Let Them Be Little Act," and amending P.L.2011, c.175.
Be It Enacted by the Senate and General Assembly of the State of New Jersey:
1. Section 1 of P.L.2011, c.175 (C.26:2-111.5) is amended to read as follows:
1. a. All infants born in this State shall be tested for the lysosomal storage disorders, known as Krabbe, Pompe, Gaucher, Fabry, [and] Niemann-Pick diseases, and Hunter syndrome, within six months following the occurrence of all of the following:
(1) the registration with the federal Food and Drug Administration of the necessary reagents;
(2) the availability of the necessary reagents from the federal Centers for Disease Control and Prevention;
(3) the availability of quality assurance testing methodology for these processes; and
(4) the acquisition by the Department of Health of the equipment necessary to implement the expanded screening tests.
b. The Department of Health may charge a reasonable fee for the tests performed pursuant to this section. The amount of the fee and the procedures for collecting the fee shall be determined by the Commissioner of Health.
(cf: P.L.2012, c.17, s.120)
2. This act shall take effect immediately.
This bill, designated as the "Let Them Be Little Act," requires infants born in the State to be screened for Hunter syndrome, a lysosomal disorder, under the same conditions as are required for other lysosomal disorders. Hunter syndrome is a rare genetic disorder which causes permanent, progressive damage affecting appearance, mental development, organ function, and physical abilities.
Current law requires newborn
screening of other lysosomal disorders known as Krabbe, Pompe, Gaucher, Fabry,
and Niemann-Pick diseases, within six months of the occurrence of the
following: the registration with the federal Food and Drug Administration of
the necessary reagents; the availability of the necessary reagents
from the federal Centers for Disease Control and Prevention; the availability of quality assurance testing methodology for these processes; and the acquisition by the Department of Health of the equipment necessary to implement the expanded screening tests. Newborn screening of Hunter syndrome would be subject to the same requirements.
Hunter syndrome occurs mainly among males when an enzyme needed to break down certain complex molecules is missing or malfunctioning. There are two subtypes: early- and late- onset Hunter syndrome. Early-onset Hunter syndrome is more common and severe, and appears usually between two and four years of age. By late childhood, a child may suffer from a severe mental disability and may not live beyond the teenage years. Late-onset Hunter syndrome is milder, and is typically diagnosed after 10 years of age but may not be detected until adulthood. A person with late-onset Hunter syndrome may live into their 50s.
It is intended that early screening and diagnosis of Hunter syndrome and appropriate management, through administration of prescription drugs such as ELAPRASE, would enable a child with Hunter Syndrome to live a longer and higher quality of life.
The bill is designated as the "Let Them Be Little Act" to recognize that children with Hunter Syndrome are often unable to enjoy the simple pleasures of youth because so much of their lives are occupied with undergoing medical treatments and tests rather than just being kids.