“EMMA’S LAW” LEGISLATION APPROVED BY ASSEMBLY

Legislation sponsored by Assembly Republican Whip Dave Rible, R-Monmouth, and Assemblymen Jason O’Donnell, D-Hudson, and Herb Conaway, D-Burlington, requiring infants in New Jersey to be screened for lysosomal storage disorders (LSDs) was approved by the General Assembly today.

Rible, O’Donnell and Conaway are the three primary sponsors of the bill (S-1999/A-2708), which is aimed at identifying the malfunction of lysosomes which are a cell’s waste disposal system and are critical in the digestive process.  The bill provides for testing once the proper reagents are approved that would enable detection of such diseases such as Krabbe, Pompe, Gaucher, Niemann-Pick and Fabry in newborns.

Lysosomal disorders mainly affect children and can cause developmental delays, movement disorders and pulmonary and cardiac problems. The legislation is named for Emma Daniels, who was born in 2009 and was eventually diagnosed with Krabbe Disease.

“One of the most heartbreaking things a parent can face is a serious health problem for their child,” said Rible. “If an approved and reliable test that can detect this digestive malady in young children is available, it is imperative that we make every effort to implement it and identify the disorder as quickly as possible.

“Early detection is a key in treating many diseases,” commented Rible. “Scientific breakthroughs in treating illnesses that were once thought to be incurable are not uncommon. I believe we owe it to our children and their parents if a disease such as Krabbe can be detected in its infancy and treated. We need to give them more than hope. We need to give them the cure.”

“In 2011, in the greatest nation in the world, our newborns deserve the best medical care we can provide them,” said O’Donnell. “Krabbe is a devastating disease, and I commend Emma Daniels’ family for being tireless advocates for legislation that will prevent other families from experiencing the pain they have suffered.”

“It’s heartbreaking to think Emma would’ve had a better chance of fighting this disease had she been born just a few miles north in New York where this type of screening is required,” said Conaway. “This measure might not help Emma, but it can help other children and families affected by this debilitating, often fatal disease.”

“While there is no cure for lysosomal storage disorders, there is research underway to find reliable cures and treatments. As recently as this past October, new medical methods have been developed that may be applicable to Krabbe’s Disease. It is critical that we detect these disorders as early as possible, to give these children a shot at life,” said O’Donnell.

“Children with Krabbe’s Disease are on borrowed time. Early detection combined with cutting edge stem cell therapies gives these children a chance at cure. The most serious and debilitating symptoms often to do not respond to therapy once they take hold,” said Conaway. “Let’s give these babies a fighting chance at life by making screenings for this disease a requirement in New Jersey.”

The Rible-O’Donnell-Conaway bill provides that lysosomal screening be conducted within six months after the Food and Drug Administration approves a detection test for the disorders; the necessary reagents (substances that enable detection of the disorder) are made available by the Centers for Disease Control and Prevention; that a quality assurance testing method is in place; and the Department of Health and Senior Services acquires the necessary equipment in order to conduct such screenings.