BILL ANALYSIS �
AB 1559
Page 1
ASSEMBLY THIRD READING
AB 1559 (Pan)
As Amended May 23, 2014
Majority vote
HEALTH 19-0 APPROPRIATIONS 17-0
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|Ayes:|Pan, Maienschein, |Ayes:|Gatto, Bigelow, |
| |Ammiano, Atkins, Bonilla, | |Bocanegra, Bradford, Ian |
| |Rendon, Ch�vez, Chesbro, | |Calderon, Campos, |
| |Gomez, Gonzalez, Roger | |Donnelly, Eggman, Gomez, |
| |Hern�ndez, Mansoor, | |Holden, Jones, Linder, |
| |Nazarian, Nestande, | |Pan, Quirk, |
| |Patterson, Ridley-Thomas, | |Ridley-Thomas, Wagner, |
| |Wagner, Wieckowski, | |Weber |
| |Bocanegra | | |
|-----+--------------------------+-----+--------------------------|
| | | | |
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SUMMARY : Requires the Department of Public Health (DPH) to
expand statewide screening of newborns to include screening for
adrenoleukodystrophy (ALD).
EXISTING LAW : Requires DPH to establish a genetic disease unit
to coordinate all DPH programs in the area of genetic disease
that will promote a statewide program of information, testing,
and counseling services and to have the responsibility of
designating tests and regulations to be used in executing this
program and to have the responsibility of designating tests and
regulations to be used in executing the California Newborn
Screening Program (CNSP).
FISCAL EFFECT : According to the Assembly Appropriations
Committee:
1)Annual estimated screening costs to the DPH Newborn Screening
(NBS) Program statewide of $4.5 million (Genetic Disease
Testing Fund). This test is estimated to cost $9 per child.
2)Annual estimated costs to the Medi-Cal program of $2.3 million
(45% General Fund (GF), remainder federal funds). Medi-Cal
pays for approximately 50% of the births in the state, and
reimburses the NBS Program for screening costs associated with
AB 1559
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Medi-Cal births.
3)It appears likely that with the imposition of the screening
test, monitoring of ALD-diagnosed children who otherwise would
not have been monitored will increase costs by a small amount
in the California Children's Services (CCS) program, likely
less than $100,000 (GF/ federal) annually. Treatment costs in
CCS associated with ALD would likely be similar to, or
possibly somewhat less than, what CCS pays currently, with
treatment being provided earlier, to slightly more children,
with lower costs, fewer risks, and better outcomes.
To the extent successful treatment avoided permanent
disability and reduce the number of children receiving
services the state would otherwise pay for, such as regional
center or long-term care services, unknown but potentially
significant annual GF cost savings are possible.
COMMENTS : According to the author, ALD is an X-chromosome
genetic disorder that is passed down from mother to son and once
symptoms show, it progresses quickly and it is usually too late
to do anything meaningful to save the boy. The author further
states that our knowledge about and ability to combat the
disease has increased greatly in the last couple decades,
including treatments that can cure patients if caught early
enough and this makes it imperative, medically and morally, that
we catch the disease as early as humanly possible - which makes
ALD the perfect candidate for newborn screening.
In 1966, California began its CNSP with the testing of
phenylketonuria. Since its creation, the CNSP has been expanded
several times as new discoveries are made and tests developed
and now screens for more than 70 disorders. Diseases have been
added through regulation and legislation.
Currently only New York and Connecticut screen newborns for ALD.
However, on January 16, 2014, the federal Discretionary
Advisory Committee on Heritable Disorders in Newborns and
Children, which advises the Secretary of the United States
Department of Health and Human Services on the most appropriate
application of universal newborn screening tests, technologies,
policies, guidelines, and standards, approved ALD for review to
add the disease as a core condition for recommended screening of
all newborns in the United States The review process should
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take approximately two years before a final decision is made.
According to the California Department of Health Care Services,
between 2004 and 2013, the cost of treating 22 children with the
disease in the California Children's Services program was
slightly more than $100,000 per child, during that nine-year
period.
ALD is a rare X-chromosome genetic disorder (which affects
mostly boys) that can cause injury to the brain, nerves, and
adrenal glands. People with ALD do not generate enough of the
protein that breaks down very long chain fatty acids (VLCFA).
The myelin sheath, which acts as insulation around the nerves,
is made up of these fatty acids; buildup of the VLCFA prevents
the brain from communicating with the body. The first signs of
ALD are behavioral and it is often misdiagnosed as attention
deficit disorder, but it rapidly leads to a vegetative state and
ultimately death.
The childhood form is the most severe and affects boys between
the ages of four and eight years old. The slightly milder adult
version affects men in their 20s and 30s. Unless treated before
symptoms show, children affected with ALD will die within a few
months to a few years. Early detection and treatment provides
dramatically better quality of life for the affected individuals
and their families. Cord blood and bone marrow transplants
performed at a very early stage in the disease have proven to
treat and heal the patient, enabling a healthy and long life.
The article "Long-term effect of bone-marrow transplantation for
childhood-onset cerebral X-linked adrenoleukodystrophy," in
medical journal the Lancet, August 26, 2000; Volume 356, found
the long-term beneficial effect of bone marrow transplantation
when the procedure is done at an early stage of the disease.
Analysis Prepared by : Lara Flynn / HEALTH / (916) 319-2097
FN: 0003730