Senate Concurrent Resolution No. 8

Introduced by Senator Fuller

January 13, 2015

Senate Concurrent Resolution No. 8—Relative to X-linked adrenoleukodystrophy.

LEGISLATIVE COUNSEL’S DIGEST

SCR 8, as introduced, Fuller. Adrenoleukodystrophy Awareness Month.

This measure would memorialize the month of November 2015 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 2015 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.