BILL ANALYSIS Ó ----------------------------------------------------------------- |SENATE RULES COMMITTEE | SCR 8| |Office of Senate Floor Analyses | | |1020 N Street, Suite 524 | | |(916) 651-1520 Fax: (916) | | |327-4478 | | ----------------------------------------------------------------- THIRD READING Bill No: SCR 8 Author: Fuller (R) Amended: As introduced Vote: 21 SUBJECT : Adrenoleukodystrophy Awareness Month SOURCE : Author DIGEST : This resolution proclaims the month of November 2015 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. CONTINUED SCR 8 Page 2 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 adrenomyeloneuropathy deterioration will progress over decades. This resolution memorializes the month of November 2015 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. Prior Legislation SCR 126 (Fuller, Resolution Chapter 121, Statutes of 2014) proclaimed November 2014 as Adrenoleukodystrophy Awareness Month. SCR 65 (Fuller, Resolution Chapter 119, Statutes of 2013) proclaimed November 2013 as Adrenoleukodystrophy Awareness Month. SCR 8 Page 3 FISCAL EFFECT : Fiscal Com.: No JA:j 1/27/15 Senate Floor Analyses SUPPORT/OPPOSITION: NONE RECEIVED **** END ****