Life with The Leivas: *Programming Note*

Saturday, February 19, 2011

Programming Note

The stepfather of a friend of ours will be on the CBS show, Undercover Boss airing THIS SUNDAY, at 9pm (8 central). The stepfather, Al, is one of the trainers featured on the show and during filiming for the episode he mentions Sanfilippo syndrome quite a bit. Al is the stepfather of our friend Shannon, Shannon's children Waverly and Oliver both have Sanfilippo syndrome. You can learn more about their family from their blog here: http://www.familymctravels.blogspot.com

I found a few previews for this week's Undercover Boss episode. We're so excited that Sanfilippo syndrome is getting such exposure!

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MPS/Sanfilippo Syndrome

In October, 2008 Olivia was disgnosed with MPS III or Sanfilippo Syndrome, type B. MPS stands for: Mucopolysaccharidoses. It is a genetic lysosomal storage disease (LSD) caused by the body's inability to produce specific enzymes. Normally, the body uses enzymes to break down and recycle materials in cells. In individuals with MPS and related diseases, the missing or insufficient enzyme prevents the proper recycling process, resulting in the storage of materials in virtually every cell of the body. As a result, cells do not perform properly and may cause progressive damage throughout the body, including the heart, bones, joints, respiratory system and central nervous system. While the disease may not be apparent at birth, signs and symptoms develop with age as more cells become damaged by the accumulation of cell materials.

Babies and young children with Sanfilippo Syndrome appear normal, but symptoms begin to appear with age as more and GAGs build up in the cells of the body. There are 3 stages to the disease. Stage 1 the child begins to lag behind peers and begins to display difficult behaviors. Stage 2 the child losing his/her language, becomes hyperactive, chews on everything, and has sleeping difficulties. Stage 3 the child slows down, becomes dependant for all mobility and loses the ability to chew/swallow. There is no treatment or cure for Sanfilippo. Life expectancy varies.