This month we have been so busy. I will start first with how Nicholas is doing. He really has continued to decline this month both with his eye sight and mobility. It has been very difficult for us to watch this rapid decline. Here is a picture of Nicholas with a home 24 hour EEG. Electrodes are glued to his head and his brain activity is monitored over a 24 hour period.
He is wearing fanny pack which records all activity. The good results were that he is not suffering any silent seizures (even when he has some myclonic jerks through out the day) We had never done this test at home but it was much easier for everyone. William joined in by have his own fun “hat”.
Just looking back a year ago Nicholas was running around with no problem at all. In fact last August 2008 I took Nicholas for a 2 hour easy hike in the woods (at that time I did not know he was blind or had batten disease). Now he has a lot of difficulty keeping his balance on his two feet. He gets around our house fair but has a lot of difficulty if we go outside our house. He does become agitated/hyper and then exhausts himself trying. I can clearly see his frustration in this decline but he does not understand. He continues to want to go go go but his body does not agree. We do have a walker for him but he does not yet need that. Although some days we feel like it would be easier for him if we brought it out, emotionally, we are not ready for that.
As far as seizures, they continue to be fair and we have had to continue to adjust the Ativan dose to prevent more seizures. All seizures and medicine changes are noted on our web site calander. The medications will always be difficult to manage but we still do not want to turn to the anti-convulsant medications at this time. Nicholas is still himself and his personality is not affected by with current medications. The decline that we are seeing seems to be disease progression. He continues to take 3.3 ml of levocarnatinve three times a day, multivitamin and calcium.
I continue to feed Nicholas most meals. He now pools food in his mouth when eating and needs to be cued to chew and swallow. Feeding is slower now and usually takes about 45minutes to an hour to get a good amount of food into him. I also try to give him high calorie snacks through out the day. His weight remains stable, he rarely verbalizes he is hungry but becomes aggitated when he is hungry and if I don’t catch his cues…he will end up in a non stop crying episode and refuse to eat all together. His feeding and eating is time intensive and I am working with his nurses to help in his feeding. Nicholas is also not always willing to let everyone feed him but myself.
We now have nursing care through a local agency. They have been with us for about 2 weeks now and I did not realize how much I needed it. We have been in contact with some real terrific nurses to take a turn caring for him. Nicholas still has his good sence of humor and is very witty. He fights this battle everyday and still maitains the joy and wonder of a child. As I have said before I will never give up on Nicholas and I am doing what any mother would do for their child. I would give my life for him.
We have also been busy taking many trips up to Vermont. Nicholas loves spending time at this lake house. He especially appreciates the sounds. This most recient trip he pointed out he could hear “that bird all night” which were the loons. He also loves the sound of the brook which trickles water down from the mountain, boats speeding by and the sound of my dad’s old antique car. He had a rough time fighting seizures and drop attacks (which may have been triggered by the heat) but once he managed to get through that episode he enjoyed swimming with his cousins, fishing (touching the fishes), eating smores and watching a lot of movies. It is always worth the trip.
We took another trip to the beach. As last month the boys ran in and out of the water for about an hour. Our intention was to build a sand castle but the water was just too much fun. We found another seafood shack and again Nicholas devoured a stuffed lobster drenched in butter as well as part of my and Chris’s meal. Geez this boy can eat if you just take the time to feed him. Check out the picture… It was a fun day!
Most weekends we have been busy with fundraisers this month. First was the bounce U event “Bounce for Nicholas” on Friday August 7 at the Bounce U in Bedford NH. This was a great event and see our additional pictures on the side panel of this web site. Nicholas had a fantastic time but his excitement and the heat got the best of him.
Unfortunately he did have a small seizure at this event, ended up sleeping it off in the owners office for 1 1/2 hours. When he woke, he took his medications, ate some food and was rearing to get out and bounce again. We could not help but let him. A huge amount of baked goods was donated by the Bedford Mom’s group and friends for our bake sale (thank you!). We were not able to sell all the goods at this event. Chris and I decided it would be best to then donate yummy left overs to New Horizons food shelter in Manchester NH. They were thrilled to get the treats (“everyone comes here hungry”) In this event we made roughly $4,600.00 which was a big success.
Our next event we had this weekend was at the Bake Sale at the Stow Vermont Antique Car show on Friday-Saturday August 7-8th. At this event family raised a lot of awarness toward our foundation and Nicholas. Giving out Our Promise To Nicholas pamplets and wrist bands was a big success. The bake sale sold out.
The next event was our Mud Hog Race at Gunstock NH on Sunday August 9th. This event was run by our dear friends Jannette and Kim. They made fancy “Our Promise to Nicholas” t-shirts for this event and raised great awareness toward our foundation. See the pictures below and additional on our side panel.
On Monday August 10 was our Golf Tournament at The Farms in Connecticut. It turned out also to be a great success. On this day which was nearly 95 degrees and muggy there were approximatly 50 players and we raised nearly $11,000.00. There were a lot of great prizes donated and won. Because of the heat Nicholas could only ride out in the golf carts for about 30 minutes, but he wanted to hit a few golf balls too.
Breaking a sweat for Batten Disease turned out to be another fundraiser which was on Saturday August 15. This event was put on by Chrisann Dalton and crew. This fundraiser had a fun kids work out, medals and balloons for the kids to kick around the gym and the second part of this event was another free work out for adults. This event raised a good awareness and donations toward our foundation. Thank you Chrisann!!!
The 6th fundraiser event for this month was also on Saturday August 15th at the Liquid Planet in Candia NH. It was Lily’s 5th birthday fundraiser for Nicholas. Lily is a dear little friend of ours that has taken Nicholas under her wing. This terrific little girl, who takes on the world, understands that Nicholas is blind, can not communicate well, and does not walk very well. She told her mom she wanted Nicholas to get better, so in lieu of her birthday gifts she wanted all money to be donated to our foundation. (What kids does that!) It was a very special day for both the Mooney family and ours. We are lucky to have met such a kind hearted little girl with such wonderful parents.
Lastly, we were on the front page of the Bedford Bulletin this month. It was a bit overwhelming for me to see a picture of myself on the front page. Batten disease has over-ruled my quiet and shy past to now speak out on Nicholas behalf….my promise to him is to find a cure. I will do what ever it takes to save my son.
We have been very busy in the past month with all these fundraisers. We have had a lot of family, friend and community support to get all these events off the ground. Chris and I want to thank each one of you personally for helping us raise this money for our son and others with Batten disease. Time continues to move very quickly for Nicholas as the disease progresses. The phase II gene therapy study is ready in NYC please continue to help us get these kids into this study to stop this disease. We are looking for a white knight to come through to help finance the millions needed. We know somone is out there. In the meantime every donation brings us closer to our promise.
UP COMING EVENTS:
Friday October 2, 2009 Our Promise To Nicholas Gala Manchester Country Club, Bedford NH. Tickets are on sale now. Please join us for an incredible event to benefit this foundation. A performance by The Boston Symphony Orchestra cellist, Luis Leguia. A rocking performance by The Jimmy Lehoux Band. Gourmet meal by Manchester Country Club, hundrends of scrumptious desserts donated by local bakerys. Silent Auction and raffle prizes to be won! https://promisetonicholasgala.weebly.com/ for more information. Contact: Heather @ heatherdainiak@hotmail.com or 603-617-2023 or Kim at knaioti@comcast.net
October Not so Scary Halloween Party and Movie Night in Bedford– This event will be a halloween party. Kids wear costumes. Movies: Great Pumpkin Charlie Brown Movie and ET. Pizza, popcorn, cotton Candy, donuts, hot chocolate. We are still working out the logistics for this event but check out our web site for updates on this event.
IGive– Please continue to purchase on-line items from this web site and a percentage of your purchase will go toward our foundation.
www.itspersonalgifts.vpweb.com. a local artist, Mindy Shoals plans to donate 2.5 % of purchases toward our foundation. She sells personalized hand- made gifts. Beautiful work Mindy! Thank you!
Facebook Cause -Cure Genetic Brain Disease-Our Promise to Nicholas foundation. This cause is up and running thanks to my brother and sister in law Dan and Amy. So far it has 1,451 members and has raised $1,125.00 Please continue to send our cause to your friends and family to raise awareness to our foundation and batten disease. Together we can get closer to reaching our goal to start the gene therapy trial in NYC to save 5 children with Batten disease.
Old Town Day Saturday September 19 from 10AM-3PM. Riley Field Complex, Nashua Road. We will have a booth at this event. Please come visit us.
Thank you again for all your donations and support. We have now raised roughly $100,000.00 in five months and we need $2,400,000.00 to get the human phase II gene therapy study at Cornell in NYC started. It is ready, just waiting for money and these children don’t have any more time. You can make a difference in Nicholas life and children like him. Please donanate. There is not a day that goes by that we are not fundraising. We will never give up!
