SENATE HEALTH, HUMAN SERVICES AND SENIOR CITIZENS COMMITTEE

STATEMENT TO

SENATE, No. 974

STATE OF NEW JERSEY

DATED:  JUNE 11, 2018

      The Senate Health, Human Services and Senior Citizens Committee reports favorably Senate Bill No. 974.

      This bill requires all infants born in this State to be tested for the genetic markers associated with spinal muscular atrophy (SMA), which is a progressive neurodegenerative disease that is caused by abnormally functioning motor neurons that control voluntary movement, such as walking, talking, and swallowing.  SMA leads to progressive muscle weakness and atrophy, particularly in the muscles of the torso, upper legs, and upper arms.  The symptoms of SMA typically appear in the first six months of life, although they may manifest later in childhood or, in rare cases, during adulthood.  Individuals with the most common form of SMA typically have a lifespan of less than two years.  Approximately one in 50 Americans is a carrier of the disease, and it is estimated that, in New Jersey, 165,889 people are SMA carriers, 304 are currently living with the disease, and approximately nine babies are born with SMA each year.

      In December 2016, the federal Food and Drug Administration approved Spinraza (nusinersen) as the first drug approved to treat SMA.  Early testing has suggested that diagnosing and treating infants with SMA before they become symptomatic may be the key to successful treatment outcomes, underscoring the importance of early detection of the disease.