BILL ANALYSIS                                                                                                                                                                                                    Ó






                                 SENATE HEALTH
                               COMMITTEE ANALYSIS
                       Senator Ed Hernandez, O.D., Chair


          BILL NO:       AB 395                                      
          A
          AUTHOR:        Pan                                         
          B
          AMENDED:       May 27, 2011                                
          HEARING DATE:  July 6, 2011                                
          3
          CONSULTANT:                                                
          9
          Trueworthy                                                 
          5                                                          
                                        
                                     SUBJECT
                                         
                           Newborn screening program
                                         

                                    SUMMARY  

          Requires the California Department of Public Health (DPH) 
          to expand statewide screening of newborns to include 
          screening for severe combined immunodeficiency (SCID) and 
          other T-cell lymphopenias detectable as a result of SCID.


                             CHANGES TO EXISTING LAW  

          Existing law:
          Declares the intent of the Legislature that the state's 
          hereditary disorders program activities are to be fully 
          supported by fees collected for services provided by the 
          program, unless otherwise provided. 

          Requires DPH to establish a genetic disease unit to 
          coordinate programs in the area of genetic disease and 
          evaluate and prepare recommendations on the implementation 
          of tests for the detection of certain hereditary and 
          congenital diseases. 

          Requires DPH to charge a fee for newborn screening and 
          follow-up services, and requires the amount of the fee to 
                                                         Continued---



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          be established pursuant to regulation and periodically 
          adjusted. 

          Requires that any fee charged for screening and follow-up 
          services provided to Medi-Cal eligible persons, health care 
          service plan enrollees, or persons covered by disability 
          insurance policies are to be paid directly to the Genetic 
          Disease Testing Fund, and are subject to the terms and 
          conditions of the health care service plan or insurance 
          coverage. 

          This bill:
          Requires DPH to expand statewide screening of newborns to 
          include screening for severe SCID and other T-cell 
          lymphopenias detectable as a result of SCID.

                                  FISCAL IMPACT  

          According to the Assembly Appropriations committee 
          analysis, AB 395 has the following potential costs:
             1.   Annual estimated screening costs to the DPH Newborn 
               Screening (NBS) Program statewide of $5 million 
               (special fund).  
             2.   Annual estimated screening costs to the Medi-Cal 
               program of $2.2 million (50 percent General Fund 
               (GF)). Medi-Cal pays for approximately 45 percent of 
               the births in the state, and reimburses the NBS 
               program for screening costs associated with Medi-Cal 
               births.  
             3.   Annual treatment costs to the Medi-Cal program for 
               babies diagnosed with SCID are estimated to be in the 
               range of $250,000 annually (50 percent GF). 
             4.   Annual treatment cost savings to the Medi-Cal 
               program from early detection of SCID, potentially in 
               the range of $1-2 million annually (50 percent GF).  
               In the absence of early detection, treatment costs for 
               newborns in the Medi-Cal program with SCID annually 
               are highly variable and can be significant, depending 
               on the time of diagnosis, whether they are diagnosed 
               before succumbing to lethal opportunistic infections, 
               and whether and when treatment is attempted.  


                            BACKGROUND AND DISCUSSION  





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          According to the author, AB 395 expands the California NBS 
          Program to adopt SCID as the 30th "core" disorder screened 
          for in the program. The author argues that this bill is 
          needed to implement the recommendations of the federal 
          Health and Human Services (HHS) Secretary's Advisory 
          Committee on Heritable Disorders in Newborns and Children 
          (SACHDNC) and bring the NBS Program into alignment with the 
          most up-to-date research, technology, laboratory, and 
          public health standards and practices.  The author states 
          that SCID is the most serious primary immunodeficiency 
          disorder and leads to extreme susceptibility to serious 
          illness.  The author argues that unless these defects are 
          corrected early on in the child's life the child will die 
          of opportunistic infections before their first or second 
          birthday.

          SCID 
          The defining characteristic of SCID is the absence of 
          T-cells and, as a result, the lack of B-cell functions.  
          B-cells are specialized white blood cells made in the bone 
          marrow that fight infection.  These genetic defects lead to 
          extreme susceptibility to serious illness.  Unless these 
          defects are corrected, children are vulnerable to 
          opportunistic infections and will likely die before their 
          first or second birthday.  In the past, children with this 
          disorder were kept in strict isolation, sometime in a 
          plastic isolator or "bubble."  Now treatments are available 
          to significantly enhance the health outcomes of infants 
          with SCID who are presyptomatic or early symptomatic.  

          Based on national studies, stem cells from either umbilical 
          cord blood or bone marrow appears to be effective in 
          significantly decreasing the morbidity and mortality for 
          children with a type of SCID caused by a mutated gene on 
          the X chromosome.  According to the genetic disease 
          screening program, if the treatment is provided within 3.5 
          months of life, the long-term survival rate is 95 percent 
          and after 3.5 months it is 60 to 70 percent.  When left 
          untreated, children rarely survive past the age of two.

          Pilot project
          Beginning in August of 2010, DPH has been participating in 
          a pilot project to test for SCID sponsored by the Jeffrey 
          Modell Foundation and the National Institutes of Health.  
          According to the March of Dimes, all babies born in 




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          California since the pilot began have been screened.  Out 
          of 235,686 babies screened, seven have been identified as 
          SCID babies.  The pilot has also lead to the identification 
          of four other babies with T-cell lymphopenia.  Current data 
          as a result of the pilot has shown the rate of SCID to be 1 
          in 35,000 in California while it was previously estimated 
          to be 1 in 100,000.
          
          Methodology for adding new conditions
          According to DPH, there is no national standard process to 
          add a disorder to a state's screening program.  Some states 
          will add a disorder once it is recommended by SACHDNC but 
          prior to official acceptance by the HHS Secretary, while 
          other states wait for the official acceptance by the HHS 
          Secretary.  In California disorders have been added through 
          the budget process or through legislation directing the 
          department to add a specific disorder to the screening 
          program.  

          In May 2010, Secretary Sebelius of the HHS adopted the 
          SACHDNC recommendation of the Uniform Screening Panel to 
          adopt the recommendation to add SCID as a core condition 
          and the related T-cell lymphocyte deficiencies to the list 
          of secondary targets.  

          Prior legislation
          SB 142 (Alpert), Chapter 687, Statutes of 2004, extends the 
          date by which DPH would be required to obtain screening 
          from laboratories by competitive bid from July 1, 2005 to 
          August 1, 2005.

          SB 1103 (Senate Budget and Fiscal Review), Chapter 228, 
          Statutes of 2004, expands statewide screening of newborns 
          to include mass spectrometry screening (TMS) screening for 
          fatty acid oxidation, amino acid, organic acid disorders, 
          and congenital adrenal hyperplasia and provides that if DPH 
          is unable to provide this screening by July 1, 2005, 
          requires screening for these disorders to be obtained from 
          one or more laboratories.  Provides specified flexibility 
          to amend contracts for implementation of the TMS screening.

          AB 442 (Committee on Budget), Chapter 1161, Statutes of 
          2002, requires hospitals to collect fees associated with 
          any tests conducted under the state's NBS Program, and 
          makes an outreach and community awareness process for this 




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          program voluntary, versus mandatory.

          AB 2427 (Kuehl), Chapter 803, Statutes of 2000, makes 
          various changes to existing law relating to the genetic 
          disease testing program, as specified, and states 
          legislative intent that unless otherwise specified, the 
          program carried out is to be fully supported from fees 
          collected for services provided by the program.

          SB 148 (Alpert), Chapter 541, Statutes of 1999, requires 
          health care service plans and specified disability insurers 
          to provide coverage for the testing and treatment of 
          phenylketonuria (PKU), a genetic disorder.
          
          SB 537 (Greene), Chapter 1011, Statutes of 1998, requires 
          DPH to establish a program to provide extended newborn 
          genetic screening services for persons who elect to have, 
          and pay for, the additional screening, and would require 
          the department to charge a fee not to exceed the costs of 
          these additional screenings, that would be deposited into 
          the Genetic Disease Testing Fund.
          
          Arguments in support
          The March of Dimes, the sponsor of the bill, writes that, 
          any disease affecting newborns that can be detected and 
          treated should be, and it is critical that SCID be added to 
          the California NBS Program.  The March of Dimes argues that 
          the health benefits of screening newborns for SCID are 
          apparent, but screening would also likely lead to economic 
          relief in California as well.  The Children's Advocacy 
          Institute writes that AB 395 would make possible the 
          identification of SCID shortly after birth, allowing for 
          timely, cost-effective treatment and could save the lives 
          of children who are born with this condition.  

          The American Academy of Pediatrics writes that early 
          screening for SCID is critically important as data shows 
          that SCID infants who receive a related donor bone marrow 
          transplant within the first 14 weeks of life are 
          significantly more likely to survive and have fewer 
          problems over time than those who receive transplants later 
          in infancy or who have already developed an infection.


                                  PRIOR ACTIONS




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           Assembly Health:    15- 1
          Assembly Appropriations:12- 5
          Assembly Floor:     71- 4


                                    POSITIONS  
          
          Support:  March of Dimes (sponsor)
                    American Academy of Pediatrics, California
                    California Medical Association
                    Children's Advocacy Institute
                    Talecris Biotherapeutics
                    The American College of Obstetricians and 
               Gynecologists, California

          Oppose:   None on file.

                                        
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