BILL ANALYSIS �
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|SENATE RULES COMMITTEE | SB 224|
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THIRD READING
Bill No: SB 224
Author: Walters (R)
Amended: 5/28/13
Vote: 21
SENATE HEALTH COMMITTEE : 9-0, 4/3/13
AYES: Hernandez, Anderson, Beall, De Le�n, DeSaulnier, Monning,
Nielsen, Pavley, Wolk
SENATE APPROPRIATIONS COMMITTEE : 7-0, 5/23/13
AYES: De Le�n, Walters, Gaines, Hill, Lara, Padilla, Steinberg
SUBJECT : Newborn screening program
SOURCE : Hunter's Hope Foundation
DIGEST : This bill requires the Department of Public Health
(DPH), until January 1, 2019, to expand the screening of
newborns in Orange County to include screening for Krabbe
disease, and exempts the amendment of contracts for this purpose
from existing provisions that establish standards for contracts,
as specified.
ANALYSIS :
Existing law:
1.Requires DPH to develop a genetic disease testing program.
Requires newborn screening for preventable heritable or
congenital disorders leading to physical defects or
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intellectual disabilities. Requires newborn screening for
sickle cell anemia, phenylketonuria, and severe combined
immunodeficiency (SCID). Also requires tandem mass
spectrometry (TMS) screening in newborns, as defined.
2.Requires DPH to establish a genetic disease unit to coordinate
programs in the area of genetic disease and evaluate and
prepare recommendations on the implementation of tests for the
detection of certain hereditary and congenital diseases.
Requires DPH to provide genetic screening and follow-up
services.
3.Requires DPH to charge a fee for newborn screening and
follow-up services, and requires the amount of the fee to be
established pursuant to regulation and periodically adjusted.
4.Requires that any fee charged for screening and follow-up
services provided to Medi-Cal- eligible persons, health care
service plan enrollees, or persons covered by disability
insurance policies are to be paid directly to the Genetic
Disease Testing Fund, and are subject to the terms and
conditions of the health care service plan or insurance
coverage.
5.Requires, under regulation, testing for hereditary
hemoglobinopathies, primary congenital hypothyroidism, and
galactosemia.
This bill:
1.Declares that this act shall be known, and may be cited, as
Jacquelyn's Law.
2.Requires DPH to establish a pilot program to expand in Orange
County the screening program established in existing law to
include the screening of newborns in that county for Krabbe
disease.
3.Requires the expenditure of funds from the Genetic Disease
Testing Fund to be authorized for implementation of this act
through the amendment of the Genetic Disease Branch Screening
Information System contracts, as defined. Exempts the
contracts from existing policies, procedures, regulations or
manuals, as specified.
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4.Requires Jacquelyn's Law to remain in effect only until
January 1, 2019, unless a later enacted statute, that is
enacted before January 1, 2019, deletes or extends that date.
Background
Currently, all babies in California are screened for over 75
diseases at birth through newborn screening. These diseases are
not otherwise clinically recognized at birth but if untreated
can cause irreversible physical and mental damage to children,
with many resulting in death For these diseases, early
detection is essential to avoid such devastating outcomes.
Likewise, early detection through newborn screening for Krabbe
disease is crucial to give these children a chance at a healthy
life. While treatment options are available, these treatments
are only effective when given before symptoms are present. Most
often children are not diagnosed in time and, therefore, are not
able to receive these life-saving treatments.
Newborn Screening Program . Newborn screening is recognized
nationally as an essential preventive health measure and an
important state-based public health program. All states in the
nation and the District of Columbia mandate newborn screening of
all infants born within their jurisdiction for certain treatable
disorders that may not otherwise be detected before
developmental disability or death occurs. Newborns with these
genetically screened disorders typically appear normal at birth.
The testing and follow-up services of newborn screening
programs are designed to provide early diagnosis and treatment
before significant, irreversible damage occurs.
According to DPH, positive test results are immediately
telephoned to a follow-up coordinator at one of the Newborn
Screening Area Service Centers throughout the state. The
coordinator contacts the newborn's physician to arrange for
repeat testing. If repeat testing determines that the baby has
a disorder, the coordinator will supply the latest clinical
information on diagnosis and treatment and assist with referrals
to special care. In 2011-12, approximately 492,000 newborns
were screened for 80 genetic disorders. Approximately 9,250, or
under 2%, were classified as positive or questionable and were
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referred for follow-up testing or services.
Krabbe Disease . According to the National Institute of
Neurological Disorders and Stroke, Krabbe disease is a rare,
inherited degenerative disorder of the central and peripheral
nervous systems. Krabbe disease occurs in about 1 out of
100,000 individuals in the United States. The symptoms of
Krabbe disease usually begin before the age of one year (the
infantile form). As the disease progresses, muscles continue to
weaken, affecting the infant's ability to move, chew, swallow,
and breathe. Less commonly, onset of Krabbe disease can occur
in childhood, adolescence, or adulthood (late-onset forms).
There is no cure for Krabbe disease. Infantile Krabbe disease
is generally fatal before age two. Results of a very small
clinical trial of patients with infantile Krabbe disease found
that children who receive cord-blood stem cells from unrelated
donors prior to symptom onset developed with little neurological
impairment. Results also showed that disease progression
stabilized faster in patients who receive cord blood compared to
those who receive adult bone marrow.
Uniform screening panel . The Secretary's Advisory Committee on
Heritable Disorders in Newborns and Children (SACHDNC) was
chartered in February 2003 to advise the Secretary of the U.S.
Department of Health and Human Services (HHS) regarding the most
appropriate application of universal newborn screening tests,
technologies, policies, guidelines and standards for effectively
reducing morbidity and mortality in newborns and children
having, or at risk for, heritable disorders. In 2010, the HHS
Secretary adopted the recommendation of SACHDNC for a uniform
screening panel. Lysosomal storage disorders, such as Krabbe
desease although considered, was not included in these
recommendations.
Methodology for adding new conditions . According to DPH, there
is no national standard process to add a disorder to a state's
screening program. Some states will add a disorder once it is
recommended by SACHDNC but prior to official acceptance by the
HHS Secretary, while other states wait for the official
acceptance by the HHS Secretary. In California, disorders have
been added through the budget process or through legislation
directing DPH to add a specific disorder to the screening
program.
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Prior Legislation
SB 1072 (Strickland, 2012), would have required DPH, until
January 1, 2018, to expand statewide screening of newborns to
include screening for Hurler syndrome and Krabbe disease. SB
1072 died in the Senate Appropriations Committee.
SB 1731 (Block, Chapter 336, Statutes of 2012), establishes the
Newborn Critical Congenital Heart Disease (CCHD) Screening
Program and requires hospitals, beginning July 1, 2013, to offer
a pulse oximetry test for the identification of CCHD to parents
of newborns prior to discharge.
SB 395 (Pan, Chapter 461, Statutes of 2011), expands statewide
screening of newborns to include screening for SCID.
SB 1103 (Senate Budget and Fiscal Review, Chapter 228, Statutes
of 2004), expanded statewide screening of newborns to include
TMS screening for fatty acid oxidation, amino acid, organic acid
disorders, and congenital adrenal hyperplasia.
AB 442 (Committee on Budget, Chapter 1161, Statutes of 2002),
requires hospitals to collect fees associated with any tests
conducted under the state's Newborn Screening Program.
SB 537 (Greene, Chapter 1011, Statutes of 1998), requires DPH to
establish a program to provide extended newborn genetic
screening services for persons who elect to have, and pay for,
the additional screening.
FISCAL EFFECT : Appropriation: Yes Fiscal Com.: Yes
Local: No
According to the Senate Appropriations Committee:
One-time costs to DPH to upgrade information technology
systems and adopt regulations of about $210,000 (Genetic
Disease Testing Fund).
Ongoing costs to expand newborn screening of about
$880,000 per year, covered by fees (Genetic Disease Testing
Fund).
Increased costs to Medi-Cal of $400,000 per year for
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testing fees (50% General Fund, 50% federal funds).
Earlier diagnosis of infants covered by public health
care programs with one of these conditions may result in
increased costs, to the extent that ongoing treatments such
as enzyme replacement therapy begin earlier than they would
without screening for these conditions. The extent to
which this will actually increase costs is unknown.
Whether early screening and diagnosis will improve
medical outcomes and reduce future treatment costs to
public healthcare programs is unknown, given the limited
information available on treatment outcomes.
SUPPORT : (Verified 5/23/13)
Hunter's Hope Foundation (source)
OPPOSITION : (Verified 5/23/13)
California Hospital Association
ARGUMENTS IN SUPPORT : According to the source, Hunter's Hope
Foundation, early detection through newborn screening is crucial
for those afflicted with Krabbe disease to benefit from the
potentially life-saving available treatments. The sponsor
maintains that should California begin screening, significant
reduction in costs can be anticipated due to less costly care of
children with Krabbe disease when they are functional and do not
require continuous nursing care.
ARGUMENTS IN OPPOSITION : The California Hospital Association
(CHA) writes in opposition to this bill that in California there
are more than 500,000 babies born in hospitals each year. For
each of those births, hospitals administer a newborn screening
for more than 75 genetic diseases and congenital disorders that
are preventable or remediable by early intervention testing.
CHA maintains that mandating new screenings will impose new
costs on the newborns parents, the program, the payers
(including Medi-Cal) and the providers. CHA argues that any
screening expansion must be evidence-based and implemented only
once standard protocols have been tested and it is established
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that actual benefits, in terms of improved patient outcomes,
will result. Currently, CHA asserts, there are insufficient
clinical research and evidence-based reviews of these rare
conditions to provide an opinion on the extent to which
lysosomal storage disorders meet the available and efficacy of
treatment thresholds.
JL:ej 5/28/13 Senate Floor Analyses
SUPPORT/OPPOSITION: SEE ABOVE
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