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SCR-126 Adrenoleukodystrophy Awareness Month. (2013-2014)

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Senate Concurrent Resolution No. 126

Relative to X-linked adrenoleukodystrophy.

[ Filed with Secretary of State  August 20, 2014. ]


SCR 126, Fuller. Adrenoleukodystrophy Awareness Month.
This measure would memorialize the month of November 2014 as Adrenoleukodystrophy Awareness Month.
Fiscal Committee: NO  

WHEREAS, 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; and
WHEREAS, 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; and
WHEREAS, The loss of myelin and the progressive dysfunction of the adrenal gland are the primary characteristics of X-ALD; and
WHEREAS, 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; and
WHEREAS, While nearly all patients with X-ALD suffer from adrenal insufficiency, also known as Addison’s disease, the neurological symptoms can begin either in childhood or adulthood; and
WHEREAS, The childhood cerebral form is the most severe, with onset between ages four and 10; and
WHEREAS, The most common symptoms are behavioral changes, including abnormal withdrawal or aggression, poor memory, and poor school performance; and
WHEREAS, 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; and
WHEREAS, The milder adult-onset form is known as adrenomyeloneuropathy (AMN), which typically begins between 21 and 35 years of age, and has symptoms that include progressive stiffness, weakness or paralysis of the lower limbs, and ataxia; and
WHEREAS, Almost one-half the women who are carriers of X-ALD will develop a milder form of AMN, but almost never will develop symptoms seen in boys with X-ALD; and
WHEREAS, Treatment with adrenal hormones can be lifesaving, and symptomatic and supportive treatments for X-ALD include physical therapy, psychological support, and special education; and
WHEREAS, Newborn screening is effective in catching and preventing the negative effects of X-ALD and is relatively inexpensive; and
WHEREAS, The prognosis for patients with childhood cerebral X-ALD is generally poor due to progressive neurological deterioration, unless bone marrow transplantation is performed early; and
WHEREAS, 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; and
WHEREAS, 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; and
WHEREAS, 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; now, therefore, be it
Resolved by the Senate of the State of California, the Assembly thereof concurring, That the Legislature does hereby proclaim the month of November 2014 as Adrenoleukodystrophy Awareness Month; and be it further
Resolved, That the Secretary of the Senate transmit copies of this resolution to the author for appropriate distribution.