Spring is here! I always have to start our first line as “we continue to be so busy with Nicholas!” Every day Nicholas’s day is filled with an adventure would be the best way to describe it!
First, an update on Nicholas. We had our 6 month evaluation in NYC for the gene therapy surgery that Nicholas had in August 2010. Being a study, the doctors and scientists are unable to share with us their results in the evaluation of Nicholas. I reflect back in the past 6-7 months and we grateful for having the opportunity of enrolling Nicholas into this study. Even if it will only give us a few more months or weeks with him it was worth it. Nicholas is the oldest child in the study so it is hard to compare him to the children ages 2-4 going through the study now. My hope is that they are able to preserve more function when treated earlier in the disease. Looking at Nicholas subjectively we know he has significantly declined in 6 months. His speech continues to deteriorate to the point where is can say only a few words such at “mom, help, home, ouch”. Sometimes he can spontaneously blurt out a word we have not heard for a while. He is still able to use some sign language and able to use his “talker devise” with buttons to communicate his needs. By Nicholas’s expressions, head nodding, few words and sign language we know that Nicholas is using all he has to try to communicate with us and we know cognitively our Nicolas is still there. He understands us and luckily I don’t see that he is getting too frustrated. His mobility has continued to decline but he is still walking with assistance. He tires easily but there is no change in motivation to play with toys, walk up stairs (very wobbly and with full assistance) and play video games. But there are bad days when he does not walk much at all, but is comforted by listening to his favorite movies and hearing familiar voices around him. The doctors have increased Nicholas’s night tme medications as he has been waking up at 4-5AM with lots of myoclonic jerks, crying and irritable, unable to get back to sleep. We hope this will do the trick. I have had to put bed rails up in his bed because I am afraid he will fall out of bed when he wakes up. We are considering an oxygen monitor alarm to put on him at night while he is sleeping. I am up checking on him about 4 times a night, when I a not seeping right next to him. His is growing like a weed so this again might be the reason we needed to increase meds.
He is still eating well and maintaining his weight. He is 58 pounds and I am still lifting him but struggling! We met with a swallowing expert in the last month who was very impressed at how we handle Nicholas. She watched me feed Nicholas his meal which now consists of food finely cut in to very small pieces, lots of butter, sour creams (and or other sauces). The expert reported to me that it does not look like he is really chewing his food buthe is not aspirating at all. She gave me some great tips on feeding him, keeping his chin tucked, sitting up as straight as possible, change foods when he starts pooling food in his mouth (which will wake his mouth up), keep the enviornment calm around him (minimal talking around him, besides his movies) so he can concentrate on swallowing. I have ordered a “feeding chair” which will also help keep him in one spot while myself or his nurses feed him. We have started thickening his liquids with a gel thickener which makes it easier to swallow. Nicholas had a swallow study done this week, which confirms that he swallows well, without aspiration. A PEG tube (stomach feeding tube) may be in the future, but clearly not right now. However, knowing the inevitability of a feeding tube breaks my heart.
Nicholas had a dental appointment this month and had a excellent report. No cavities. Chris is very conscientious about brushing his teeth, flossing and using the fluoride gel. I find it very difficult to brush his teeth without Chris. I have contacted Nicholas’s pediatrician about obtaining a suction machine to help with the fluids as he is coughing with swishing thin liquids.
Nicholas continues to go to the Easter seals for occupational therapy (OT) twice a week. They do
such a fantastic job with Nicholas. He loves it as he plays with the therapists Sara and Erica. Swing, horns, shaving cream, painting are to name a few things he does. The therapists are so kind and gentle with Nicholas as well as very motivating him as he plays with their toys. It is wonderful! My only regret is that I wished we had started this program when he was diagnosed 2 years ago.
Nichoals also is still riding his horse Collin with UpReach Therapeutic Horseback Riding. He always loves it but I don’t think his body always feels the same way. At the beginning of the session he is able to hold his body up and by the end he is still smiling but his body is haunched over. I think he uses every bit of his energy reserved for the ride. He is able to get the words out “walk or gooo!” Which is terrific. It is a very positive
Nicholas continues to see his physical therapist Venetia who has really preserved his body and muscle strength even as he declines. Nicholas remains very strong. His shoulders are starting to hunch forward and he has clonus in his feet, legs and hands at times (which is uncontrollable tremors). We try to minimize this by keeping him as active as possible. He is using a stander at school for an hour each day and we have ordered one or home as well. This stander is imperative for his health to keep weight on his muscles and bones, otherwise he is at higher risk for osteoporosis and fractures. He is starting to have reddened areas on his ankle bones from his AFO’s so we think they need to be re-fit to prevent any skin break down. He receives a massage from Laura P. three times a week and we believe this helps him immensely to keep his body loose. His feet still give him difficulty and are very tight so she concentrates on this area the most.
The best part of this last month was having Nicholas participate in NEHSA which stands for New England Handicapped Sports Association in which he skied Mt. Sunapee in NH. Nicholas was lucky to be guided by Tom Kersey who is a board member and executive director of NEHSA. We were honored to be skiing with him. This is a day brought tears to my eyes because it brought so much joy to Nicholas. He squealed in excitement as he went down the mountain. “again again again” that is what he was saying to us.
So our journey with Nicholas continues. William has been asking me about angels lately. I start to walk into any church, I bow my head and my eyes fill with tears. I don’t feel sorry for myself but I do feel sorry that Nicholas has to go through the pain of this disease. I would do anything to take his place! How do I explain this to William as he asks why am I crying and he tries to comfort me. But over this two years I have slowly stopped being angry and realized that I need to find answers for my younger son as he watches Nicholas decline. At the age of 4 he has started to ask why. I am not sure how many months or years we will have with Nicholas as I take one day at a time. I put my head up now and look around me. Wow! I am lucky to be part of this unbelievable foundation making a difference for children like Nicholas. I am lucky to have a strong husband who I know loves me and is not only so busy with his own job but puts any extra time he has toward our foundation and family. I have a terrific younger son William that keeps me smiling and laughing. I am lucky to have my mom and sister who are very involved in helping us run the foundation and they both put in many hours behind the scenes to keep foundation running. Thank you to the rest of our families that are a big support system for us and help in every fund raiser. I feel lucky for the new friends that reach out to me and are not afraid to reach out to Nicholas, my family and myself. They have helped us stay positive. We are lucky to be surrounded by a community that continues to support every fundraiser, reach out to volunteer for us, or even lend us a hand personally.
Someone said to me recently “I am sorry you miss out on so much in life” My thoughts were first I don’t feel I miss out. My life is so full and I am lucky it has a lot of meaning. I am the lucky one. It always comes back to we are lucky to be able to make a difference in the children after Nicholas. There will be a cure in our lifetime; it continues to be My Promise To Nicholas! But for now I like to enjoy every minute of him.
Saturday April 16, 2011Bedford Mom’s Club Easter Egg Hunt was a huge success raising over $3,500. Thank you everyone for all your efforts. We have come to look forward to this event every year, just for the fun of it. I think by the turnout, the community feels the same way!
Saturday June 25, 2011 3rd Annual Fore Nicholas Golf Tourament Location: Stonebridge Country Club,161 Gorham Pond Road, Goffstown, NH Time: 2pm Start, Cost: $125.00 per player includes dinner, $50 dinner option available. 18 holes of golf scramble, team prizes, hole prizes, cocktail reception, music and dancing, silent and live auction. Dinner is your choice of Clam & Lobster, Prime Rib or Grilled Chicken. Contact: email@example.com for questions.
Click here to sign up for Fore Nicholas Golf Event
Do you want to help us? We do need your help!!!!
We need volunteers on the day of the golf tournament. Please call firstname.lastname@example.org
heather i am a nurse at the basc whom would very much like to volunteer the day of the golf tournament if you can use the help. i spoke with dr,dainaik and he said to contact you . please let me know where i can help . thank you . annie
Nicholas and Heather, I miss you guy's! Still thinking and praying for you and the entire family! God Bless!