Life with The Leivas: Good News, Bad News Day +122

Sunday, November 22, 2009

Good News, Bad News Day +122

Always one to start on a positive note, Olivia is feeling great. Aside from the occassional "roid rage" temper tantrum Olivia is feeling great. She's eating, drinking, and gaining weight. Today at clinic everyone was so impressed with her tolerance and patience. She's such a good girl. Mike and I are both so proud of her.

The bad news is that we are all still unable to draw blood from Olivia's port. Today we headed back to the clinic so that they could try and draw some blood from her port. Unfortunately we didn't have any luck. They put some more "TPA" into her port and then they proceeded to try every hour to draw some blood. We waited a long 4 hours at the clinic today with no luck at all, not even the faintest tint of blood. It looks like tomorrow we will have to do a "dye study" where they infuse a small amount of dye while looking at an x-ray of her chest to see what happens when something is infused into her port. Mike and I are both really apprehensive about this port situation. On one hand we believe that Olivia's port is technically working. The purpose of the port is to allow medications/fluids/etc to be infused into Olivia. Drawing blood for labs is a bonus! Of course we don't want Olivia to have to experience any more discomfort than necessary but you can draw blood from other areas in order to obtain a sample to do various labs. On the other hand we know that we still have a long way ahead of us, Olivia will most likely have her port for at least a year. It is important that we are able to easily draw blood from Olivia for various tests. At this point both Mike and I are sure that if Olivia's port is infusing correctly we do not want her to have undergo another surgery unless it's ABSOLUTELY necessary. If there are any medical professionals out there please feel free to weigh in. We keep going back and forth!

Since I want to end on a positive note I'll share that Olivia and Peter both went to bed easily and I'm enjoying my dinner of wine, cheese and crackers. I hope they sleep well tonight!

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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.