Ayden Jane is coming out of her shell. I mean WAY OUT. It is absolutely amazing. Tonight we went to a soccer game Gary was coaching. It was an away game at a nearby high school. I only mention that to make the point that Ayden Jane had never been there before. She was hilarious. She hung out on the bench with Dad, had an orange slice or two and talked with the boys. Then she climbed her way up to me and danced and climbed on me. That's not too strange, but when other moms spoke to her she answered them and smiled and performed... Then, all on her own, she went to a couple of them and leaned on them and fished for thier attention. Of course they obliged and Ayden Jane was thrilled. As I was saying goodbye to some of the boys parents, Ayden Jane piped in, "see ya later." It was as if I brought a different child with me.
After the game Ayden Jane was chatting up a storm. She informed Gary, "Come on Dad, let's go to Hubaway (Subway)." All the while dancing and jumping around. She easily walked the 1/4 mile back to the car swinging her arms and bouncing as usual. She trotted toward every puddle she could find and tried to get her sister to play tag.
It is so hard to put into words how dramatically different she was. I mean, that is the goofy kid we hang out with at home with our family, but outside of home she is normally... in a shell? or a daze? or just 'taking it all in'? overwhelmed? You know the comments we get as parents of a child with PWS: she looks sleepy, she is so quiet, she is so well behaved, it must have been a long day for her... Not tonight. Tonight she was playful and mischievous and adorable and social. What will tomorrow hold? I hope it is a new trend.
Quite a while back there was some talk about D-ribose and I believe the amazing William. Around that time I purchased some as I was ordering the other supplements. I ended up working on other things and was also trying to keep bouts of what seemed to be hypoglycemia type tendencies under control so I just put it in the cabinet. Well, I discovered it maybe a month ago.
I figured it was worth a shot with Ayden Jane because, if I remember right, it works somehow with or maybe it is needed more because of B-12 and she is a big responder to that. I did not really expect any grand improvements as Ayden Jane is really doing great so I was trying it more because I knew I bought it for a reason...
Here is what I think I see. Well, I definitely see it, I just don't know if it is from the ribose. Sort of the last significant 'oddities' about AJ have been how different she is out and about compared to home and some sensory seeking issues. At home, or even out and about but with her family members she is loud and animated and active. Outside of home or with non family members she is silent, hardly animated and active only in her own little games or running off to investigate. It is sort of like her brain is so busy taking stuff in and processing it when dealing with unfamiliar people, that she cannot possibly respond to them. (Not sure if that makes any sense to any of you)
After a bit less than a week, I started to notice that AJ actually spoke to some of my friends. They had never heard her talk before. She began to have personality no matter where we were and with people she had not met before. Most amazing to me of all is that at the playground this week, she truly played with another little girl about a year older. She has been at that playground many times and has never even acknowledged other kids accept to watch what they were doing. This time she followed the little girl as they repeatedly climbed up to slide and waited for each other at the bottom. Then AJ asked her to go swing with her! I had to translate, (after I snapped out of my state of shock) but off we went happily to the swings. It is hard to put into words other than to say that her little personality shines through now.
So, could it be the ribose? I know that she was quicker and more responsive after we started the B-12, so are they somehow working together with the other supplements? Or is this just the age these things happen?
After my baby sister was born, I began to spend more time with my family. It’s amazing how a tragedy to one member of the family, whether it is a newer or older member, brings the family together. It is sad that a tragedy must happen for loved ones to rediscover their love for each other, but in my family, it did indeed happen that way.
It’s not like we didn’t love each other- we just were all too busy. I always have soccer, my sister has other sports of her own, and my mom has somewhat of her own taxi service to perform. That consists of driving me to soccer three times a week and my eight year old sister Mckenna to her Karate or soccer. Ayden Jane Gulley was born October 1st, 2007. She was motionless for forty-eight hours. Although born at Waccamaw hospital, she was sent to MUSC as soon as possible. For the first six months of her life, she was very lacking in muscle and she was still not diagnosed. Finally around six months of age, she was diagnosed with Prader-Wili Syndrome. Finally diagnosed, Ayden was able to start a growth hormone that would help her eventually build muscle on her own. Her growth hormone is an injection every night. It is a small needle but it still is a shot Ayden will have to deal with the rest of her life.
Our whole family has taken part in helping with Ayden’s condition. My mom has basically taken medical research as a side job. Her first job is being a full time mom to all four kids including Ayden. My mom, Janet Gulley, has devoted herself full time to helping Ayden Jane in any way. When Ayden was diagnosed my mom was told the worst situation possible. As my mom started researching, she found a whole organization and people that were fighting with Prader-Wili Syndrome as well. All these people have been dealing with it longer and they helped my mom. Some of the people that are helping my mom are actually medical researchers. My mom was told of the supplements that she can use to help Ayden function as normal as possible. Ayden takes multiple supplements a day. One of them is B-12. B-12 helps with the normal functioning of the brain and nervous system. Once my mom got all these supplements for Ayden we all started seeing miraculous changes. We even started calling Ayden “B-12 Baby” as a joke because of the amount of energy she had after she started taking her supplements.
First my mom got into the research, but soon enough she got us all hooked. Kayla my older sister is very into the medical field, so she understands more of what my mom is talking about than I do. Our mom shows us articles and other sources at which we can read about Prader-Wili Syndrome. All of the information is way over our heads and always confuses me, but it gets us that much closer to understanding Ayden than I already was. Our family has contributed even in small ways such as putting up with the balance beam in the middle of our living room.
Physical therapy is a big part of Ayden’s life. She has physical therapy every Wednesday with Mrs. Jennifer Jennings. My whole family loves Mrs. Jen. She has so much energy and works so well with Ayden Jane. We joke that she knows where certain buttons are on Ayden that make her do things we never thought she could do. Mrs. Jen has a wonderful family of her own, and our families have become quite good friends. Ayden’s “best friend” is Mrs. Jen’s youngest daughter Lilah. Ayden also goes to Hippotherapy. Hippotherapy is physical therapy, just it is horse back riding. She goes to Hippotherapy once a week on Tuesdays. Hippotherapy is for her core strength, because kids with Prader-Wili Syndrome tend to be weak in their core. Also it is to help prevent scoliosis. Ayden is also at high risk for scoliosis. Ayden, at first, did not talk at all. She was extremely quiet. Ayden has speech therapy every other week on Fridays.
Ayden has many struggles in life. The main struggle is that with Prader-Wili Syndrome, she will always be hungry. She eats plenty, but her brain tells her that she is still hungry even with a full stomach. That is why obesity is very common with kids with Prader-Wili Syndrome. Ayden cannot eat grains or very many carbohydrates. That is basically taking away all childhood junk food, such as chips, “Goldfish,” and “Cheez-its.” People may think that she is deprived, but in the long run she will benefit from it. Ayden eats extremely healthy. She eats lots of eggs, fruit, vegetables, and meats. She eats lots of protein and health food. Ayden’s favorite snack is almonds. She eats almonds straight out of the bag. For a special treat, we give Ayden an almond covered in dark chocolate. She loves these treats.
I make it sound as if Ayden’s life is horrible, but really she is as normal as ever. When people find out that she has a genetic disorder, they can’t believe it. Ayden seems as normal as possible. Ayden is a little ball of energy. She is always being goofy and making our whole family laugh. Ayden is more entertaining than anyone I know. She is always giggling or doing something goofy just to make us laugh.
Ayden has completely changed my and my family’s lives. Ayden has changed my whole look on my life. I love Ayden Jane and I don’t regret having Ayden Jane in my life. Sometimes think to myself that I would take Ayden’s disorder from her if I could, but then I realize that we almost have. With all the effort we put in to make Ayden as healthy as possible has made it so that people can’t even notice she has a disorder. Ayden Jane is the best baby sister I could ever ask for, and we all love her.
Yep. Just 2 1/2 and it was he second trip to the dentist. I was very slack with my older children with them making their first trips closer to 5 or 6, but with all that I have heard about dental issues in kids with PWS off we went. Ayden Jane was great. I guess by now she is just used to strangers poking and prodding... She let them polish her teeth and everything. The great news is that so far so good.
Sometimes it feels like a sort of lottery. We have this giant list of things we are "at higher risk" for and only time will tell which adventures we get to live and which ones we avoid. Constantly on the watch for things we hope we will never experience. Oh, and don't forget to stop worrying about it all and enjoy the ride!
There once was a day when Ayden Jane was quiet. I mean eerily quiet. Other PWS moms know the sort of quiet I mean. Surrounded by all the crying babies in the hospital, but not a peep out of Ayden Jane. Blood draws, not a sound. Time to eat, still no crying. Gary jokingly told the special care nursery nurses he would give $100 to the first one to make her cry.
That was 2 1/2 years ago. Flash forward to today. Kayla was trying to watch TV and Ayden Jane was working on a puzzle. She noticed the 'tweet tweet' was missing. She started calling, "tweet, tweet! WHERE ARE YOU? TWEET TWEET..." Of course, I noticed it was driving Kayla crazy so I gave Ayden Jane a play cell phone and told her to call the bird. Let's just say that 20 minutes later she proved she does not understand the concept of a telephone. She considers it a megaphone and called all the animals in the puzzle. Kayla was pleading with me to get her to stop so she could hear herself think. Me? I was very much enjoying watching little sister drive big sister crazy!
I haven't said much about food with Ayden Jane. That is a little odd since that is a large part of what is effected in PWS. There are different ways to approach keeping our kids lean and healthy. Many folks work hard to balance calories using weight gain or not to guide how much to feed their children. We started early on to try a different path. It was mostly because I found the arguments for 'going paleo' compelling, but honestly in part because I found it easier to manage. Basically, we have food rules. Ayden Jane may eat ______ but may not eat grain products. We feed her every 2 1/2 to 3 hours, but do not push her to eat a large quantity just something. At times when we let her play too long without eating or if she takes a nap without a snack first she is really cranky. She usually will fight eating at this point and it takes some coaxing to get her restarted. Once she gets some protein or good fats in her she returns to her old cheery self. When she is done we clear it away and send her off on something else. It works for us. What is interesting is it seems we are not alone.
I had an e-mail conversation with Dr. Miller. I would post it, but I have not asked Dr. Miller about that so I will just describe it. She has seen in her clinic others who have kids close in age to Ayden Jane on the same style diet. One is eating 1600 calories per day and another 1300 and she reports they are both thin. They are eating approximately the same as their siblings. This is not the norm for kids with pws.
Following the 'food rules' in our house is not fun and I am sure that as Ayden Jane gets older it will be more difficult, but it is obviously working for her.
Ayden Jane worked hard during physical therapy today. Jen put ankle weights on her and a band around her abdomen. I am not really sure what the ankle weights were for, but the belly band was to try to get those belly muscles to 'stay turned on.' We worked lots of different things from arms down through belly. Homework this week includes working more on catching balls-catching not directly on mid line, walking on the 'balance beam' in our living room (scrap plank of hard wood flooring elevated a few inches off the ground) and reaching for things in the wheel barrow position. Lucky for me, Mckenna will be more than happy to handle much of the ball and balance beam work. I just need to work in the wheelbarrow.
As for language, I think my favorite thing right now is that Ayden Jane actually has a bit of a southern accent. It comes out in the word can't. I will hear her from her car seat, "Mom, I caain't reach." Or, "I caain't get my hock (sock) on."
Speaking of socks, Ayden Jane is obsessed. She does not like to wear her own socks, likely because they are harder to get on. Or maybe just because she likes to wear everyone elses. Either way, she changes socks all day long and they look more like leg warmers. Today I picked up 17 socks in the livingroom! I cannot tell you how excited I was today when she went barefoot for a little while. She even went outside barefoot. Wooo hooo.
Today was one of those days where Ayden Jane was just on. She was loud and funny and even talkative when out and about. She fought her nap with all she had, but finally caved. We went to horses tonight and she did not wear out in the least. After horses, we went to a soccer game and she just kept on going. She was jumping and dancing and running and yelling and just generally goofy. I will be interested in what Jen thinks tomorrow. If she is 'connecting her hips and shoulder girdle better through her abdomen.' I feel like everything is firing and doing so with much more ease.
Her monkey blanket came today and she loves it. I put her down to sleep tonight with the monkey on top of the weighted lap pad and she was very happy. She fell asleep in no time. I am meeting with a friend who is a seamstress on Thurs do see about putting it all together. I am thinking big score for Ayden Jane.
I added the ribose to Ayden Jane supplements a week or so ago. It is not one that would show big sudden differences so I was not expecting too much. The only thing to watch out for is a sort of blood sugar drop if the dose is too high. I have noticed that the past few days Ayden Jane gets cranky around lunch time, but it is more like a tired cranky. She does not ask for food and has even refused to eat anything a couple of times. Once coaxed into eating a bit something she loves, I can then get her to eat enough of a protein... and she becomes a whole new child. This is very similar to how she responded when she was smaller to feeding spread too far apart. I need to be sure to give her even a tiny snack before the crash in blood sugar (or whatever is crashing). I am not sure if the ribose is the reason for some of the extended energy she has had. I think time will tell, but she sure has had some great days lately. Well, besides the cranky, yelling, refusing to eat 20 min.
Dr. Clark called today with the results of Ayden Jane's lab work. In order to put things in perspective, I have to say that in Nov. when we were in Fl. the lab results were a bit high for her gh (IGF -1) level. That is to say she was above the normal range, but Dr. Miller was okay with that because her bp (binding protein) was also elevated. Basically, there was more gh than 'normal' floating around, but there was enough bp to 'mop it up' so she was okay with it. This time her gh is high normal. That is pretty much just what we want. Ayden Jane has had a pretty big growth spurt the last month or two so I guess the numbers in Nov. were just her little body revving up. We have decided to hold tight with the .4 dosage, but Dr. Clark is willing to consider bumping it up a bit before I return and the testing that dosage to decide whether to stay with it. Sounds reasonable to me. I sent all the numbers off to Dr. Miller in Fl to get her opinion as well. I also asked the question of her, "why does Ayden Jane seem to need less gh than most kids with pws?" (her dose is low if done by weight and she is lean) Now I know the standard answer is just that they are all different... I know that, I am just curious if she has any theories as to why they vary so much in their need for gh. I will let you know if she has any good ideas that I can understand.
So, lets just recap where we are with supplements... at nearly 2 1/2.
27 pounds 35 3/8 inches .4 gh daily B-12 methylcobalamin liquid 2500 mcg/day split into 3 doses cyanocobalamin 1500-2000 mcg/day split into 2 doses (breakfast and lunch) Nordic Naturals fish oil + CoQ10 1 gel cap morning and one at night Carnitine Fumarate 600 mg split into morning and night doses Creatine 1/4 tsp. morning and night. Schiff liquid vitamins 5mls morning and night Ribose 1/8 tsp. per day (this is new and I am working on the dosage)
There is a part of Ayden Jane that creeps in and worries me. Not the panic sort of worry just the what can I do to fix this worry. I don't know if it falls in the 'autisic tendencies' category, the two year old category, the Ayden Jane category or some sort of processing category. Basically, it is the difference in how she acts with unknown people and in unknown places compared to how she is with her family. She is just very quiet and will hold back and watch. She would win any staring contest hands down. She does not talk in theses situations. I have read that many other children with pws are the opposite of this. That they talk to any one willing to listen and would go off with anyone. Ayden Jane will wander off on a mission at times, but there is no stranger around that would influence that. She, for the most part, just does not care for strangers.
Obviously there is a part of that I like as a mom. She is definitely safer not warming up quickly to new people.... There is a part, though, that just seems odd when this happens. I can't tell if she is just taking it all in, or if she is just shut down beyond her control.
Occasionally we also notice the delay when there is too much going on. It is strange, though, that even with the delay, she will then follow through and knows just what we were talking about. For example, Jen asked her to get something and bring it to her. Ayden Jane was sort of frozen, I think just processing the request. 'What did she ask for... what is that.... what does it look like.... where is it....' Ayden Jane just stood there for bit. Jen then told her what they would do next. Ayden Jane stood there... then took off, got what she wanted, brought it to her and got in position for what was next. Just a pause in the action, but obviously took it all in receptively.
Some days I wish I could know what she would have been like without PWS for a moment so I could sort out what things to worry about and know what things are just her being two.
Lately I have been trying to imagine what Ayden Jane will be like as ... well, older. What will school be like? Will she be able to play sports well enough to enjoy them? Will she still love music? Will she be able to handle regular classes in school? Will she go to college? What will be her biggest struggles with PWS? Mostly, I guess, will she be happy?
I think it is helpful that I have older kids. They are quite gifted and things come very easily for them. Why does that make it easier? I am not sure, but I watched Mckenna play in a soccer tournament today and saw in her the joy of playing. She is good and plays at a pretty tough level for an 8 yr old. She had a rough game the first game because it was her turn to play goalie and lets just say it didn't go well. Then the second game came and things went her way. The smile on her face as she came off the field, that is why she plays. I want to see that smile on Ayden Jane. I think it will be there. It may not come from a game with a top level club soccer team, but it will mean soooo much even after a front yard game with mom and dad. I think it will take some thought to figure out what is best for Ayden Jane, but I picture maybe a swimmer. Something where she can be a part and be happy and we can celebrate her accomplishments as though they are Olympic medals. To us her accomplishments will be better than Olympic gold.
I know that in many ways life will be hard for her. She will simply have to work harder at most things. Somewhere in that though is a great satisfaction with the accomplishment of each mountain. With Ayden Jane I have been reminded to stop and celebrate the tiny accomplishments. Not to take things for granted. I think with my big kids I sometimes miss the little accomplishments and treat them with a sort of, 'well of course you got straight A's' attitude. I forget to let them know how proud of them I am for being great kids and developing their talents.
Ayden Jane helps me to not take things so seriously and to stop and enjoy the moment. There is nothing better than watching the joy in Ayden Jane when Mckenna plays with her. Or the laughter of all the kids as Ayden Jane grins and teases them in some way. She just seems to bring out the best in everyone. So no matter how hard my brain tries to pin down what her future will be, happy is what comes to mind as most important. I think that is what I should be imagining for all my kids, but it is easy to get caught up in their accomplishments and abilities. It is easy to forget that gifted, average or challenged in some way, we should all focus on using our talents to honor God, having a joyful spirit and blessing others.
It seems that Ayden Jane has kept to her 2 week seeking pattern. The 2 weeks have past and her seeking is now calmer. Ayden Jane seems to have gained some new skills or at least changes. I think that the physical changes have been core related. Evidence for such is that she can suddenly balance on one foot to step into her croc with the other foot. She has been practicing doing this for a while, but would always take the shoes to a place where she could hold on to something for balance. Now, she just straightens them and puts them on in the middle of the floor. She is also stepping off good sized steps with her arms full (okay not all seeking is gone) without hesitation. We are working on walking on a line and statically standing heel toe. She is doing great with it. The other core based thing is VOLUME. Did you hear how loud I said that? She is having fun yelling for us from the other room and is will make herself heard when she thinks she is being ignored. I would have definitely told my others to talk quietly at her age, but I figure she should just go for it.
As for the upcoming weighted critter, I have the lap pad and cover and I am waiting on the monkey cozy blanket Ayden Jane picked. It is not the zipper variety as Ayden Jane is not a fan of the texture of those. The lap pad cover is fairly thick and has a great Velcro opening. I am going to take the monkey blanket and the pad and cover to a friend who is a seamstress. She does slip covers for sofa's, curtains... serious sewing. She is going to devise the best way to put the pad into the monkey where it will be secure and yet easy to remove for cleaning. I am really excited about it as I have put Ayden Jane to bed a couple times with the pad wrapped in a blanket and she loves it. The monkey cozy looks a bit like Curious George, the only show Ayden Jane will watch. I am hoping for a home run with this one.
Well, I have much more to say, but it will have to wait. Mommyhood calls.
Yesterday I took Ayden Jane to an endocrinology appointment. I used to go to these appointments with questions and concerns and hopes for an increase in gh.... It struck me as a bit odd yesterday when it was sort of just another check-up. Yep, we are low dosed by weight in gh and I am hoping we will go up, but I do not feel like my next breath will depend on that. We talked supplements and concerns, but really nothing has changed in the supplements and my concerns are just things we deal with daily and the sorts of concerns that have no real answers. Like, "what does her future hold...."
Dr. Clark said Ayden Jane looks great and to keep up the good work. We will wait to see the labs come back to decide whether to go up on the gh. At 2 years 5 months Ayden Jane weighs 27 pounds and is 35 3/8 inches tall. She is currently on .4 gh daily. I think she is ready to bump up a bit by the look of her shoulder girdle and the lack of muscle mass there, but I forgot to even show Dr. Clark what I 'see'. Oh well. Eventually I will learn not to stress, but still remember everything.