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- The ARC - California Edition -

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Newborn Screening Gets Upgraded

As the 1997-98 two-year session of the legislature came to a close this year, we were pleased to see a piece of prevention legislation (Senate Bill 537) get approved by the legislature and signed by the Governor. After a year of being held in “suspense” by the Assembly Appropriations committee, Senate Bill SB537 – Genetic Diseases: Expanded Newborn Screening Program cleared committee in the final week of the session and was approved by both houses of the legislature and forwarded for the Governor’s signature.

This measure had been authored by Senator Leroy Greene in order to upgrade and expand the existing newborn screening program. The changes stipulate the use of new technology called Tandem Mass Spectography and it is expected that improvements in accuracy, repeatability, and cost will be achieved by applying this new technique.

A bill to mandate a newborn screening program was originally authored many years ago by then rookie Assemblyman Leroy Greene. Now Greene is a senior Senator and has just completed serving his final term. The current screening program conducts tests on all babies born in the state of California in order to determine if they may have been born with a birth disorder. The current test methods are considered to be quite good, but, during the last 30 or 40 years test technology has been greatly improved.

Now that the Governor has signed the SB537 measure, the newborn screening program will be upgraded and at least 16 additional disorders and conditions could potentially be added to the program. These disorders all into five categories: Cystic fibrosis; Biotinidase defect; Urea cycle defect; Organic acid defect; and Fatty acid oxidation defects. All of the disorders and conditions being considered for addition to the screening program are conditions which are genetically inherited.

Cystic Fibrosis — Early detection of Cystic Fibrosis can prevent hospitalization. With delayed detection, the disease often leads to prolonged periods of hospitalization for the person involved. Currently, there is no known cure for Cystic Fibrosis. The incidence is one case out of every 2,000 live births — This means that there are about 300 new cases of Cystic Fibrosis occurring in California every year.

Biotinidase Defects — All detected cases are treatable and curable. Detection is key. Without screening, the infant usually has brain damage before the disorder is discovered, and before treatment can be started. Screening of newborns for the lack of Biotin (required for brain growth) can be very meaningful. The incidence of this disorder is one case per 15,000 live births — 40 new cases in California per year.

Urea Cycle Defects — There are three defects of the urea cycle that are understood enough for consideration. Two are treatable, one is fatal. Detection and treatment of these two treatable disorders can minimize progressive brain damage.

Organic Acid Defects — These are serious metabolic disorders which can be treated. These diseases are usually fatal without treatment.

Fatty Acid Oxidation Disorders — This type of disorder is treatable, life-saving, and curable. It is an inherited disorder. After the initial diagnosis is made within a family, one can anticipate a recurrence risk of 25%. Once again, newborn testing is obviously of great benefit because it identifies the problem before brain damage occurs.

The Arc-California wishes to express its appreciation to
those who took the time to communicate to the author
(LeRoy Greene), their own legislator, and/or the governor
regarding this bill. Without this input of support, it
is doubtful that the bill would have been approved.
Thank You!!!


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