BILL ANALYSIS                                                                                                                                                                                                    �



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          Date of Hearing:   August 30, 2013

                             ASSEMBLY COMMITTEE ON RULES
                              Richard S. Gordon, Chair
               SCR 65 (Fuller/Vidak) - As Introduced:  August 12, 2013

           SENATE VOTE  :   37-0
           
          SUBJECT  :   Adrenoleukodystrophy Awareness Month.

           SUMMARY  :   Memorializes the month of November 2013 as  
          Adrenoleukodystrophy Awareness Month.  Specifically,  this  
          resolution  makes the following legislative findings:

          1)X-linked adrenoleudodystrophy (X-ALD) is one of a group of  
            genetic disorders called the leukodystrohpies that cause  
            damage to the myelin sheath. An insulating membrane that  
            surrounds the nerve cells in the brain; and, X-ALD affects an  
            estimated one in 20,000 boys, which is roughly 13,600 people  
            in the United States, including boys like Jeremy Hill Jr. of  
            Bakersfield.

          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 the  
            extra X chromosome.

          3)Nearly all patients with X-ALD suffer from adrenal  
            insufficiency, also known as Addison's Disease, the  
            neurological symptoms can begin in childhood or adulthood.   
            The childhood cerebral form is the most severe, with onset  
            between ages four and ten.

          4)Common symptoms are behavioral changes, including abnormal  
            withdrawal or aggression, poor memory, and poor school  
            performance; and, additional symptoms include visual loss,  
            learning disabilities, seizures, poorly articulated speech,  
            difficulty swallowing, deafness, disturbances in gait and  
            coordination, fatigue, intermittent vomiting, increased skin  
            pigmentation, and progressive dementia.

          5)Newborn screening is effective in catching and preventing the  
            negative effects of X-ALD and is relatively inexpensive; and,  
            treatment with adrenal hormones can be lifesaving, and  
            symptomatic and supportive treatments for X-ALD include  








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            physical therapy, psychological support, and special  
            education.

          6)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.

          7)The prognosis for patients with childhood cerebral X-ALD is  
            generally poor due to progressive neurological deterioration,  
            unless a bone marrow transplantation is performed early; and,  
            death usually occurs between one and 10 years after the onset  
            of symptoms.

           FISCAL EFFECT  :   None

           REGISTERED SUPPORT / OPPOSITION  :

           Support 
           
          None on file
           
            Opposition 
           
          None on file

           Analysis Prepared by  :    Nicole Willis / RLS. / (916) 319-2800