Life with The Leivas: Polishing the Pearly Whites

Friday, July 1, 2011

Polishing the Pearly Whites

Have I mentioned how much I love our dentist here? I especially appreciate their evening and Saturday hours! Mike and I took the kids for their 6 month check ups a couple weekends ago. Olivia was up first and she was such a champ! Olivia has a "happy, healthy mouth" according to the dentist. I'm relieved we were able to get through the entire exam--brushing, flossing, cleaning, polishing, and fluoride treatment without any biting! As Olivia gets older it is likely that she'll have to be sedated for dental work so I am thrilled that we're able to get through cleanings without issues at this point.

Peter was AWESOME! The hygienist and the dentist both said that Peter was the most well behaved 2 year old patient they'd ever had. Proud Mama! I'm sure it helped that Peter got to choose a cartoon episode (The Backyardigans), he got to wear earphones, and he got to pick out a cool pair of shades.

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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.