Another brilliant day. Clear blue sky and lots of sunshine, but I have to tell you, there was a biting cold wind blowing right down West Shore Road. Gary Sadowsky, weather man on WCAX-TV called it a northwest wind, and I guess he knows, but as far as I'm concerned it was blowing right out of the icy regions of northern Canada and the north pole.

As we started off on our walk this afternoon, Fred commented on how blue the sky was and took a picture (!) Down the road by Randall's turn there is a marshy area that has opened up, probably from underground springs or water channels, and there was GREEN GRASS (!!) We speculated there is geothermal energy there that we should be harnessing.

Further down the road we met Bob Noyes and his new pal, "Popcorn." We chatted for a while and learned that once again a Joe's Pond family is facing tragedy. Bob's grandson, 6 year old Nicholas, has Batten Disease, described here from the BDSRA web site:

Batten Disease is named after the British pediatrician who first described it in 1903. Also known as Spielmeyer-Vogt-Sjogren-Batten Disease, it is the most common form of a group of disorders called Neuronal Ceroid Lipofuscinoses (or NCLs).

Although Batten Disease is usually regarded as the juvenile form of NCL, it has now become the term to encompass all forms of NCL.

The forms of NCL are classified by age of onset have the same basic cause, progression and outcome but are all genetically different Over time, affected children suffer mental impairment, worsening seizures, and progressive loss of sight and motor skills. Eventually, children with Batten Disease/NCL become blind, bedridden, and unable to communicate and it is presently always fatal. Batten Disease is not contagious or, at this time, preventable.

Nicholas' dad, Dan, has a web site describing what their family is experiencing as they cope with this devastating situation. The web site is www.ourpromisetonicholas.com
Bob has promised to bring more information for us in a day or so. He and Dottie are taking a short break here at their camp on West Shore Road. At present there is no cure for Batten Disease, but the family hopes to raise money for further research that might arrest the progression of the disease for Nicholas and could help others. The disease has taken Nicholas' eyesight and he now has braces on his legs. Please take the time to read Dan's blog entries and look at some of the videos.