BILL ANALYSIS                                                                                                                                                                                                    �



                                                                            



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                                    THIRD READING


          Bill No:  SCR 126
          Author:   Fuller (R)
          Amended:  As introduced
          Vote:     21


           SUBJECT  :    Adrenoleukodystrophy Awareness Month

           SOURCE  :     Author


           DIGEST  :    This resolution memorializes the month of November  
          2014 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. 

          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. 

          4.The most common symptoms are behavioral changes, including  
            abnormal withdrawal or aggression, poor memory, and poor  
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            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.Newborn screening is effective in catching and preventing the  
            negative effects of X-ALD and is relatively inexpensive. 

          6.The prognosis for patients with childhood cerebral X-ALD is  
            generally poor due to progressive neurological deterioration,  
            unless bone marrow transplantation is performed early. 

          7.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. 

          8.Death usually occurs between one and 10 years after the onset  
            of symptoms of childhood cerebral X-ALD. 

          9.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. 

          This resolution proclaims the month of November 2014 as  
          Adrenoleukodystrophy Awareness Month. 

           FISCAL EFFECT  :    Fiscal Com.:  No


          JL:nl  6/11/14   Senate Floor Analyses 

                         SUPPORT/OPPOSITION:  NONE RECEIVED

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