BILL ANALYSIS Ó
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THIRD READING
Bill No: SCR 65
Author: Fuller (R)
Amended: As introduced
Vote: 21
SUBJECT : Adrenoleukodystrophy Awareness Month
SOURCE : Author
DIGEST : This resolution memorializes the month of November
2013 as Adrenoleukodystrophy Awareness Month.
ANALYSIS : This resolution makes the following legislative
findings:
1. X-linked adrenoleukodystrophy (X-ALD) is one of a group of
genetic disorders called the leukodystrophies that cause
damage to the myelin sheath, an insulating membrane that
surrounds nerve cells in the brain.
2. Women have two X chromosomes and are the carriers of the
disease, but men are most severely affected because men only
have one X chromosome and lack the protection provided by an
extra X chromosome. The loss of myelin and the progressive
dysfunction of the adrenal gland are the primary
characteristics of X-ALD.
3. X-ALD affects an estimated one in every 20,000 boys, which is
roughly 13,600 people in the United States, including boys
like Jeremy Hill Jr. of Bakersfield. The childhood cerebral
form is the most severe, with onset between ages four and 10.
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4. The most common symptoms are behavioral changes, including
abnormal withdrawal or aggression, poor memory, and poor
school performance. Other symptoms include visual loss,
learning disabilities, seizures, poorly articulated speech,
difficulty swallowing, deafness, disturbances of gait and
coordination, fatigue, intermittent vomiting, increased skin
pigmentation, and progressive dementia.
5. Treatment with adrenal hormones can be lifesaving, and recent
evidence suggests that a mixture of oleic acid and erucic
acid, known as "Lorenzo's Oil," administered to boys with
X-ALD prior to the onset of symptoms can prevent or delay,
but not stop, the appearance of the childhood cerebral form
of X-ALD.
6. Newborn screening is effective in catching and preventing the
negative effects of X-ALD and is relatively inexpensive.
7. The prognosis for patients with childhood cerebral X-ALD is
generally poor due to progressive neurological deterioration,
unless bone marrow transplantation is performed early. Death
usually occurs between one and 10 years after the onset of
symptoms of childhood cerebral X-ALD, and in adult-onset AMN
deterioration will progress over decades.
This resolution memorializes the month of November 2013 as
Adrenoleukodystrophy Awareness Month. November, the month of
Jeremy Hill Jr.'s birthday, was chosen to acknowledge the
struggles of Jeremy Hill Jr., his parents, Jeremy and Debra
Hill, and his sister, Meagan Hill, in fighting X-ALD.
FISCAL EFFECT : Fiscal Com.: No
JL:k 8/21/13 Senate Floor Analyses
SUPPORT/OPPOSITION: NONE RECEIVED
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