Emma Rilee Daniels
July 20, 2009 – March 24, 2012

Ryan has asked me to extend a personal invitation from him to each of you who have held Emma in their heart to join him in Emma's Life Celebration.  He also asks that folks don their brightest attire in honor of Emma.

We are humbled by the greatness of Emma.  Emma was a prophet in this world, lighting the way for a better life for others, showing us how to positively affect others' lives, and giving us the example of what sacrifice and courage really mean.

Assemblyman David Rible, author of Emma’s Law, will deliver the eulogy.  

 

In lieu of flower, donations may be made to help with Emma’s medical expenses by either:  paypal at Emmadaniels.org; or sending a check to Ron Daniels, 125 Ridgway Dr, Bordentown, NJ 08505.

Again I thank each and every one of you for holding us up along Emma's journey.

 

With Hope

Sherri and Kate

Assemblyman Dave Rible / 732-974-1719
Assembly Republican Press Office / 609-847-3400 

January 9, 2012

Legislation known as “Emma’s Law,” sponsored by Assembly Republican Whip Dave Rible, R-Monmouth, requiring infants in New Jersey to be screened for lysosomal storage disorders (LSDs) was signed into law by Governor Christie today.

Rible is a primary sponsor 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 new law 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 law is named for Emma Daniels, who was born in 2009 and was eventually diagnosed with Krabbe Disease.

“One of the most devastating things a parent can face is a serious health problem with their child, like that of Emma Daniels,” 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 and can save lives,” 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. Emma is a brave young girl whose courage is an example to everyone. The new law will give parents and newborns that are diagnosed with lysosomal storage disorders more than hope. It will hopefully give them the cure.”

The new law 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.

Emma Rilee Daniels was born July 20th 2009 to a loving family in New Jersey.  A week before Christmas of that same year, she was diagnosed with Krabbe disease (pronounced krah-BAY).  If Emma had been screened for it at birth, she could have received treatment to prevent the onset of the disease.  Even sadder, if Emma had been born just a few miles north over the border in New York State, she would have been tested for this disease at birth.

Krabbe disease (also called globoid cell leukodystrophy) is a degenerative disorder that affects the nervous system. It is caused by the shortage (deficiency) of an enzyme called galactosylceramidase. This enzyme deficiency impairs the growth and maintenance of myelin, the protective covering around certain nerve cells that ensures the rapid transmission of nerve impulses. Krabbe disease is part of a group of disorders known as leukodystrophies, which result from the loss of myelin (demyelination). This disorder is also characterized by the abnormal presence of globoid cells, which are globe-shaped cells that usually have more than one nucleus.