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My Story

My baby was born spring 2004 and what I was most struck by is that my child's PWS experience is not reflected by the medical or new parent literature currently available. Partially, this is because my child appears to be mildly impacted. And partially this is because my child belongs to the new generation of PWS children with multiple early interventions.

Both my husband and I are medical scientists by training. In addition to the extreme emotional pain of having a child with a disability, we are engaged in the problem of PWS. We have no choice about our boy's genetics and therefore we choose to throw everything we have into modifying his environment. We choose to believe completely in the redundancy of the human genome and the incredible plasticity of the nervous system, especially in the early years.

I am grateful to my new brother-in-law who has overwhelmed me with his kindness and care about my son and about PWS. He suggested this wiki as a means of gathering together all of the things that we parents have learned about our children. He spent many hours over Thanksgiving 2004 setting this up for us.

Basics about my son

  • Two older sisters: 6 1/2 and 3 years older
  • PWS by deletion
  • Half Chinese and half white
  • Moved a great deal in utero
  • Born term at 7 pounds, 11 ounces
  • Apgar of 9 and 9
  • Placed in intensive care at 24 hours for low tone and inability to feed
  • Put on ng tube
  • In intensive care for 2 1/2 weeks
  • Diagnosed at 2 1/2 weeks
  • CoQ10 (Cyto-Med) at 4 weeks
  • Weekly physical therapy at 8 weeks
  • Weekly occupational therapy at 8 weeks
  • Monthly speech therapy at 8 weeks
  • EFA's at 8 weeks
  • HGH at 12 weeks
  • Breast milk for first 4 months and then Enfamil formula
  • Vojta therapy at 5 months
  • Started solids at 6 months
  • Nutrivene customized vitamin supplement at 6 months

My son's milestones

  • Moved head back and forth at 4 weeks
  • ng tube removed at 6 weeks
  • Social smile at 2 months
  • At 4 months 25% for weight and 75% for height
  • Laughed at 4 months
  • At 4 months he had met his age-appropriate gross and fine motor milestones (low points for style, however)
  • At 4 1/2 months could eat all food from regular store bought nipple
  • At 5 months feet in mouth
  • At 5 months can roll over and over to get objects
  • At 5 months discriminates between rougher and gentler sisters when he sees them coming
  • At 5 1/2 months he was seen by a physiatrist and determined to no longer be hypotonic
  • At 6 months can prop sit indefinitely - until he reaches for something
  • At 6 months infrequent crying, lots of cooing, poor babbling
  • At 6 months moves frequently
  • At 6 months can creep and pivot while on belly (but moves in wrong direction)
  • At 6 months drops objects and belly laughs when we pick them up
  • At 6 months sleeps about 12 hours at night and takes 2-3 45 minute naps during the day
  • At 6 months 10% for weight and 50% for length
  • At 6 months difficulty eating

Developmental Pediatrician - 6 Months

We saw Dr. Alan Rosenblatt. Telephone 773-481-1818. He has a private practice on the corner of Peterson and Cicero in Chicago.

I really liked him. He is new to Chicago. He had a flourishing practice next to NIH but moved her 1 1/2 years ago for quality of life reasons. He is a Johns Hopkins guy. he was nice and friendly and a team player.

My boy is now 6 months old. Briefly, Dr. Rosenblatt had him at: 3 months for trunk and neck tone 5 months for arm and leg tone 5+ months for gross motor skills 5+ months for fine motor skills 5 months for "catching himself while falling" - I forget the official word 3 months for language. He is just starting to say Bah. Also, he noted the bell but didn't turn to teh bell. This surprised the OT because she has seen him regularly turn to sound as have I. So, it may not be an accurate read. The Bah part, though, is accurate.

So, not too bad. Pretty close on his milestones. Tone is still weak but coming along. Actually there has already been some dramatic improvement since the assessment. Today he was able to keep his arms flexed and his head even with his body while being pulled up by his forearms. He could only do this twice in a row, though, before becoming fatigued.

7 months

Developmental therapist assessed him as having zero delay in cognitive skills and 5% delay in social emotional skills. I think that there was some concern because Kian is only barely displaying stranger anxiety.

8 months

He is now 8 months. Last week he was assessed as developmentally 6 1/2 months. Practically, this means that he should be sitting up and pivoting and getting in and out of the sitting position. Instead, he is "just" sitting and playing with toys. His efforts to get from sitting to belly are awkward at best.

He bears weight and bounces up and down and dances when we hold his waist (and maybe his arms). He can almost stand when holding onto something. He can stay on his hands and knees for about a minute before collapsing.

He waves and claps and smiles and giggles and watches and hugs. He sleeps well and is otherwise alert. He isn't really on a schedule, but if I were to imagine a schedule it is: Wake up at 7:00. Nap from 9:30 to 10:30. Nap from 4:00 to 5:30. Go to sleep at 8:30. We wake him up once during the night to get extra calories into him.

He is a leisurely eater and seems to have no hunger. He is 50th percentile for height and 10th percentile for weight. He has pretty much stayed on a decent growth curve since birth.

12 months

We wanted to get him to eat healthy foods and we wanted every bite to count. We shifted to whole milk, but blended it with banana. I think we did 20 ounces of milk plus 1-2 bananas and put it in a straw sippy cup. We would make it in the morning and have three cups in the fridge for the day. He was pretty good at drinking it. We also fed him an avocado a day and an egg a day. I would take a hard boiled omega-3 egg and give him teh white (at age one) and take teh yolk (iron?) and mix it with a bit of milk and flax meal to make a paste. Believe it or not, he would eat it. We also fed him lots of whole milk yogurt and cottage cheese and bits of cheese. These foods were his staple diet. We would also give him bits and pieces of what we were eating to teh extend that he was capable of eating/chewing them. We didn't count calories at this phase, just made sure he got the egg and the banana milk in him and always had a high calorie food (avocado or cheese) on his tray.

15 months

He continues to astound us. He is not yet walking, but he crawls and crawls up stairs and pulls up and cruises and can stand for a second or two. His language skills seem quite good. He uses quite a few words (30-40??) and regularly uses two word combinations. He has used a 4 word combination (play bath bubble Jerica - his big sister). Fine motor seems fine. He signs about five words and feeds himself - sometimes even with a spoon!

His weight continues to be at the 25th percentile while his height is between 50th and 75th percentile. He is off the bottle. We make him a smoothie of banana in whole milk which he takes through a straw sippy cup. We also give him a lot of avocado and cheese. He now feeds himself completely and we do not worry about calories (other than being sure to offer at least one calorie -dense food per meal). Our family has always been a big whole grain, olive oil, fruits and vegetables sort of family and so we continue that. We also have never eaten a lot of sweets. He now wants everything we have and so he has had a couple of meals of macaroni and cheese and a few bites of ice cream over the past month or so. Mostly, however, his meals have been very healthy.

We took him to Singapore for 2 weeks and he did wonderfully. He ate giant clam and curry and white radish cake. He was doted on by grandparents and none of the extended family knew or suspected his diagnosis (this is at the request of his paternal grandmother).

His hair has turned dark brown, but he still has dark blue eyes and pale skin. His features look very much like my girls. The tone has improved in his face and so he now makes more expressions. He is not a loud baby, but he is more than capable of shrieking and crying and repeating words to make his point understood.

He no longer qualifies for early intervention based upon his actual milestone progression, instead he gets it based upon his genetic diagnosis. We work proactively on all of the PWS problem areas. He receives physical therapy weekly, occupational therapy weekly, and speech therapy weekly. He continues to receive monthly Vojta therapy. We have also started him on weekly hippotherapy. Last week, on a fluke, he received four days in a row of hippotherapy. On the third day he started standing and he climbed the stairs.

He is on growth hormone, essential fatty acids, coenzyme Q10, and a multivitamin supplement. We sign to him and read to him multiple times a day. He has a puppy and a dog. He sees our family being very active and he strives to keep up.

18 months

Kian shrieks with delight as he is chased from room to room by his sisters. His joy is infectious and the dogs join in the stampede. Kian is not yet able to walk, but he crawls with enthusiasm and grace. He loves to demonstrate his “tricks.” He entertains us by standing at the edge of his crib and letting go to hold his hands up high and balance on his legs. His face is marked with concentration as he holds his balance before bringing his arms down and giving in to laughter. He applauds himself as we all applaud and cheer. He breaks the standing ovation by speaking and signing “more tricks.” Then he does it again. A typical toddler, he practices over and over and over. We cheer and applaud; he grins and applauds; and he does it again. He cannot yet toddle. He gets what he wants through smiles and talking and gestures. He knows that words are powerful and that when reinforced with signs, they are magic. With his words, he can make us laugh and make us chase him and make us hand him almost anything he wants. With his words, he can make his sister read to him, his mama lift him up, his dog drop the sock, his other sister cover him with a blanket, and his daddy give him water.

And did I mention the horse? He loves to ride, especially over the poles set out in the ring. “More poles, more poles,” he cries and he directs Sonny “up” as the horse lifts his legs extra high to clear the pole. Kian anticipates the extra effort and responds with his words. Sonny responds to the encouragement and turns around for more poles.''

The above was submitted as a description of Kian for a fundraiser for an early intervention program.

At 18 months Kian was assessed by a developmental pediatrician and found to be age appropriate for everything, except for the fact that he is not yet walking. Specifically, Kian scored at 16-18 months for visual-motor problem solving and 18-21 months for language. He is in the 10th percentile for weight and 50th-75th percentile for height.

He receives weekly ST, OT, PT and therapeutic riding. He receives monthly Vojta therapy. He is on a customized multivitamin, EFA's, and 0.4 mg GH daily. We read to him daily and make an effort to get him out and about exploring the world. We have an active household. Currently his eating is entirely self-regulated.

20 months

Kian can walk about 5 steps unassisted and does this a couple of times a day. He can stand independently indefinitely. He is 10th percentile for weight and 50th percentile for height. He has an ear infection and a cold. We note that he has been remarkably healthy. He has two older sisters (4 and 8) and we live in Chicago. So, someone always has the snuffles in our house and Kian is no exception. But, he really has never been truly sick.

Now Kian is 20 months and his physique really surprises me. I am not sure if it is the GH or diet or exercise or luck or a combination, but he is wiry like my girls were at that age. He also has very decided opinions about what he wants to eat. I don't push the high calorie foods as much as I did before. He loves pasta and will eat anything remotely like pasta. He won't eat my healthy egg yolk paste, but he loves it when I scramble his (omega-3) egg with a liberal dose of canola oil (omega-3). He doesn't really drink milk (or banana milk), but he drinks a lot of water and eats a fair amount of whole milk yogurt and cottage cheese. He can eat most of what we eat as a family and will try most things, but always requests pasta. We give him vegetables at lunch and dinner, but he doesn't seem to be too keen on them. So, far, no sign of hyperphagia - except when it comes to pasta! :) He has even gone so far as to request pasta when he sees me poking around the kitchen getting ready to make dinner.

Finally, he is walking. This happened at 20 1/2 months. What surprised me is that it took him several weeks to move from the first steps stage to the truly walking stage. He still crawls quite a bit, but can also walk from one room to another. He also practices walking quite a bit on his own.

24 Months

He ran into the developmental pediatrician's office yelling "doctor, doctor." After about half an hour of playing and talking with him, the doctor stated that he was not going to give him a formal assessment. "This is a perfectly normal two year old," he said. He pointed out that Kian is still at risk for learning disabilities, but, so far, so good!


27 Months

He has now been in early intervention for 2 years. He had his assessments and is normal for: gross motor, fine motor, speech, social development, and cognitive development.

The bad news is that when barefoot and playing in the sand, he shows slight pronation. We will be putting him in Sure Step orthotics.

He starts a normal toddler Montessori program (2 mornings a week) in 2 weeks!

Kian eats whatever he wants when he wants. The whole family is now on skim milk and no fat yogurt. We eat whole grain bread and pasta. We eat a lot of fruit. No cheese. We really don't have any treats in our house. We will occasionally go out for ice cream. Kian isn't really that interested and he shares with his Daddy. This means he maybe gets 5 small bites of a sorbet. On weekends we go out for hot chocolate (and latte's!) and we pour some of the hot chocolate in Kian's milk. He loves that! Right now it is just very easy.

He does take food and it drives me crazy, but his version of taking food is reaching into the fruit bowl to grab a pear, taking a bite out of the pear and then putting it back!!!! Arghhh!!!!

He loves pasta and bananas. I think that if his weight continues to go up, we would cut those two things from his diet. He is now 50th percentile for height and 50th percentile for weight and so I think his metabolism is slower than his sisters'.

We are big on protein. He eats two scrambled omega eggs for breakfast and we pretty much always offer him fat free yogurt at lunch and dinner.

We are also really quick to clear his plate and excuse him from the table when he says he is done, even if the rest of us are still eating.

29 Months

LJ and I decided that we would take advantage of a gloomy day and trick the kids into eating an early dinner and going to bed early. It worked, except Kian woke up at 3 AM wanting to eat. I said no, and he went back to sleep. An hour late he woke up and wanted to eat and said his tummy hurt. I gave him 1/3 of a banana. Told him to eat it in the chair and crawl into bed with mommy and daddy "just this one night." End of story. Nothing more has come of it. (Although I pretended to keep my cool, I was actually pretty freaked out about it.) Could it have just been normal????

And now, my funny story: I took Kian to the mall today where he saw some women getting their makeup done. He ran into "line" and told the salesperson, "me next." We had to break it to him that they only did adults. She did give him some nice smelling hand lotion, though.

30 Months

Please visit Healing Thresholds. It is a Web site dedicated to parents who of children with autism. Some of us fall into this category as our children are at risk for autism and Asperger's. It is my attempt to take PWSNotes and formalize it into a Web site that can help parents make a difference early in their children's life. If you are reading PWSNotes, then you, like me, understand the profound power of early intervention. Please help get the word out and visit Healing Thresholds and tell others about it.

32 Months

Two weeks ago we started Kian on 250 mg/day of [carnitine fumarate] spread out in three doses throughout the day.

Today Kian had his EI Peabody gross motor assessment.

Just as background, Kian is doing quite well (even without carnitine fumarate). Six months ago, at 26 months, he scored an 83. The way the PT described it to me, 90-110 is normal and 80-120 falls within 2 standard deviations of normal. In other words, a score of 83 puts him within normal ranges, but on the low end of normal for gross motor.

We started carnitine fumarate 2 weeks ago. Today Kian was given the same test and compared to other 32 month olds. We all felt that Kian is on the verge of doing many things. For example, his jumping is good, but he still does not have the confidence to jump off of something unless he is holding someone's hands. We did not give him anything. We judged him entirely on how he is today. He scored 91! Completely normal. Gross motor is his greatest deficit, so this was astounding to me.

The PT was shocked. She had never known a two year old to close the gap so quickly. She is a very evidence-based lady who was slow to jump on the carnitine fumarate bandwagon and wanted to explain his burst of newfound skills on many things (maturation, time with grandparents, time with sisters, etc.). I believe this assessment really converted her, however, especially since he is still acquiring skills at a very rapid pace and many of the things that we failed him on he could just almost do.

In the interest of full disclosure, Kian also took the fine motor portion of the test and I think he must have failed it. He kept throwing the blocks around the room. His aim was good (which got him points earlier on the gross motor portion), but it was not helpful in building a choo choo train. So on I go teaching him to use his carnitine fumarate superpowers for good and not evil. Perhaps these kinks will be worked out in time for the three year assessment.

32 1/2 months

Well, he didn't fail his fine motor portion of the assessment. For fine motor he got a 94 and for gross motor he got a 91. (This is measured like IQ where 90-110 is normal.) For verbal he scored 2 month delay in expressive language, 1 month delay in articulation and 1 month ahead on comprehension. His height and weight are fine (75th% for both) and he has no food seeking behavior whatsoever. He is also quite healthy. We feel incredibly lucky!

36 months

Today Kian turns 3. He has no delays. He scored comfortably normal for: gross motor, fine motor, speech, social skills, and cognitive skills. He no longer qualifies for intervention, although we do intend to have him followed monthly by his therapists.

His language scores: 110 for auditory comprehension, 95 for expressive communication, and 103 for total language. He scored 85 on the Goldman-Fristoe Test of Articulation. In all cases, 90-110 is normal. 85-115 represent the outer ends of normal.

He scored 34 months on the Hawaii Early Learning Profile (both cognition and social emotional).

He is at the 75th percentile for height and weight. He does not have any food seeking behavior, but he does have a whacked out metabolism and appears to do better on high protein foods. We do not count calories or really limit portions, but we do control the food in the house. For example, we do not eat pasta and we only eat a bit of whole grain bread. Mostly, we eat brown rice as our starch/grain. We do not eat cheese or butter and we don't give Kian milk. He does get a lot of skim yogurt. We don't really have any sweets in the house (except for dark chocolate - yum!). Snacks are a hard boiled egg or walnuts or bananas. He gets 2 omega 3 eggs per day.

He spent last year mainstreamed at a Montessori toddler program. He went 2 mornings a week. Next year he will be going 5 mornings a week to the Montessori primary program. His teachers have not identified any areas of weakness or concern.

He gets GH (0.6 mg/day), carnitine fumarate/coenzyme Q10 (250 mg/day), Nordic Naturals Ultimate Omega fish oil (1 gram per day), and the Nutrivene multivitamin supplement.

40 months

Kian really does have a slow metabolism and we had to get rid of the bananas.

We now have Kian on a healthy Atkins diet. For us that means minimal starchy carbs. He doesn’t eat bread or crackers or cereal or cakes or cookies. Every once in a while he may have some rice. I can’t say, though that we are truly gluten-free. Most foods he eats are from scratch and I do have the gluten-free soy sauce, so he doesn’t get any gluten from processed foods. Every once in a while he may get a bite or two from the girls’ bagel, but we are trying to minimize that.

He does eat some fruit and whatever vegetables we can get into him. Those would be his source of carbs. And whatever carbs are in plain yogurt and the bit of maple syrup we use to sweeten the yogurt.

So, Kian mostly eats yogurt, eggs, almonds, and walnuts. When I make dinner, I serve a protein (bison, fish, chicken, etc.), a dark green salad, a vegetable, fruit, and yogurt. I let him eat his fill and he always leaves food on the plate. He does not seem to have any cravings or hunger issues.

What I have noticed is that he is quicker. His brain is quicker and his body is quicker. Also, his body has changed. He is lean and long and more muscular.

What I don’t know is if these changes are because he is low-gluten, or low carb, or just plain getting older.

We have stopped the Nutrivene multivitamin. When I ran out, I noticed that he had a burst in language. So, we have decided to stop it. Instead we use the Schiff liquid multivitamin. It is a pretty basic multivitamin that's claim to fame is its iron, zinc, and the fact that it is palatable.

He is in a mainstream Montessori school for 3 hours per day and is doing a great job there. He does do the "food work" there and has limited access to bananas, apples, oranges, pickles, and eggs. For now, this is ok with me. We have sent in almonds so that at snack time Kian helps himself to three almonds (instead of crackers). I have sent in bars of wonderful dark chocolate and he will get a square of chocolate instead of cakes and cookies. The teacher is working under the premise that the most important thing to do is to avoid wheat products. It seems like a good rule of thumb.

Today he had his first playdate with a beautiful little girl. I explained to the mother that Kian is on an Atkins diet because he is missing some genes and can't process carbs. I asked what was her daughter's favorite source of protein. Turns out it is salami! So, I served salami and yogurt (plain nonfat sweetened with maple syrup) for lunch. It seemed to work really well!

Kian does gymnastics 5 days a week. Three days a week he does a one hour class at My Gym. One day a week he does a one how class a our local gymanstics club and one hour is family free time on the gymnastics equipment. He also does horseback riding once a week. Plus, he is a pretty active kid. I really don't think that you could pick him out on the playground as being behind. He does lack motor confidence, though. Whereas my daughters would just assume that they can climb something or jump something, Kian is a bit slower to embrace new gross motor activities. Also, Kian is slower to jump. Although, this, too, is changing. Yesterday he surprised me by voluntarily jumping off of the last step in a staircase.

Cognitively, he seems to be ahead. His recent PT assessment showed him to be low normal for gross motor. His biggest weakness is in ball activities, but the PT pointed out that we aren't a ball family. (I think this was confirmed by the fact that we had to look in the dog's lair in order to find a ball.) I think he is still fine for ST and OT, although I am in the process of scheduling another assessment. I know he talks constantly in sentences and most people can understand him. He has lots of big ideas and big plans and he is very eager to share them.

41 months

We visitied Kian's endo last week. Dr. Carrel also noted a lot of changes in Kian since he last saw him six months ago. Note, that it was after our last endo visit, that Dr. Carrel said that we had to do something about Kian's weight gain. So, we put him on a high protein diet and tried to remove all starchy carbs. We do not limit the quantity of food, but rather only allow him to eat foods that don't contain starchy carbs. The whole family eats this way, not just Kian.

There were many subjective changes, but there were also objective ones. Over the last 6 months, Kian lost 1 pound. He also grew over an inch. Dr. Carrel did a DEXA scan to measure his body fat. Kian had 20% body fat. To put that in perspective, 45-50% body fat is typcal for untreated PWS> Treatment with growth hormone brings body fat down to about 40%. SO, Kian's percent body fat is quite remarkable.

Briefly,we strive to keep all starchy carbs from Kian’s diet: no bread, no pasta, no potatoes, no rice. I did allow a bit of fresh corn when it was in season. I also allow SmartPop popcorn and occasional wild rice. We do eat root vegetables such as carrots and beets. We drink water or tea (fresh brewed, no sweetener) or sometimes sparkling water with a splash of apple cider. I would be very curious to know if anyone else has tried this sort of diet and what you all have seen. For us it has been remarkable.

45 months

Kian is almost 4 and we saw the developmental pediatrician yesterday. Kian's only delay is a slight and subtle OT delay. Basically, this means that Kian is still a bit clumsy. He can do all of the fine motor things that he should be doing, but the quality of his movement is not all that we hope for.

Also, since it was morning and Kian was well-rested, the doctor did not notice that Kian also has articulation issues. When Kian is tired, he has mouth muscle fatigue and his words slur and become garbled. He received weekly speech therapy in order to strengthen his mouth muscles.

Kian does not eat grains and potatoes. He eats eggs and walnuts and almonds and bacon and fish and beef and chicken and fruit and some vegetables (and other things I can't think of right now). Each day we give him 2 ultimate omega gel caps, 1 teaspon of liquid Schiff's multivitamin, one quarter of a B NOW B vitamin supplement, and one capsule of carnitine fumarate with CoQ10. He does gymanstics 5 days a week and yoga twice a week. He attends normal Montessori every morning. His Montessori teacher declares him to be the same as or ahead of the other kids his age.

47 months

Kian broke/mangled his thumb 2 weeks ago by slamming it in the bedroom door. All I can say is thank goodness that PWS dampens pain sensations, because my little boy was in a lot of pain and screamed a great deal. :( He needed surgery because the bone was sticking through tthe fingernail.

3 medical appointments for Kian this week:

1) Eye doctor; eyes seem to be the same (strabsimus), but his face has grown and so that changes the prescription a bit. Plus he has broken his glasses by pulling them off with just one hand.

2) Endo says all is good. He is now 60% for weight and 70% for height. I think he has gained a bit of weight lately because of inactivity. (We had to cancel gymnastics because of his thumb.) We have also started drifting towards allowing more dry fruit in his diet. We will now be eliminating raisins and various other dry fruits. I think we will keep the occasional goji berry.

3) Thumb doctor says that thumb looks fine. The bandage is off and the thumb is pink and moves. I think it looks pretty good, but Kian's sister and friend proclaimed it as gross. He has some skin rot between his fingers and along his palm where the sprint rubbed him. He is now enjoying his first bath in 2 weeks.

And, teacher conference last week went well. No delays. The only thing that she could think of is that when he is on the floor working he tends to be reluctant to do the works that require getting up and down and running around (such as the go get them cards.) I just ordered creatine and we are going to start that in the hopes of developing more muscle mass/strength. It has worked very well for some other kids with PWS.

OT has been canceled because of the broken thumb.

The ST is very impressed with his progress. He is now much better at saying syllables and his tongue moves much better.

Whew! It has been a busy couple of weeks.

47 1/2 months

Wow. The creatine has been impressive. Kian is jumping all over the place. He is in a gymnastics class with typical kids and now his coaches describe him as being in the top half of the class! His current supplements:

Nordic Naturals Ultimate Omega http://www.vitacost.com/Nordic-Naturals-Ultimate-Omega

Schiff Liquid multivitamin http://www.vitacost.com/Schiff-Childrens-Multi-Vitamin-Liquid

NOW B-100 http://www.bodybuilding.com/store/now/b100.html

Jarrow carnitine fumarate http://www.bodybuilding.com/store/jarrow/lcarnitine500.html

Creapure creatine monohydrate http://www.bodybuilding.com/store/un/creapure.html

49 months

Kian just had a speech assessment. For this test 100 is average, with 85-115 being normal range. He tested 96 for language! He also had an articulation test. He is 4 years old and he tested as articulating like a 3 year old. This puts him at the 25th percentile for boys his age. We are going to spend the summer hitting articulation hard. There are just a handful of sounds that he should be making that he is not making.

52 months

Kian had his check-up today with his pediatrician. 38 pounds (50-75%), 42 ½ inches (75%), and BMI of 14.9 (25%).

She did the scoliosis check and thought his back looked straight. I pointed out his shoulders and she did acknowledge that one shoulder looked slightly higher than the other. She counseled me not to worry about it. Hmmmm…

She said that his fine motor skills sounded great and that I should part ways with Kian’s OT. The OT and I have a troubled relationship and I guess that I will just use Kian’s doctor as an excuse to get out of OT for now.

Don't get me wrong. We are working really hard on Kian's fine motor skills at home, but we are making progress. He can draw a circle and a square and a triangle. He can write something that maybe looks like his name. At school he is practicing writing the number 2. He can cut out shapes. He can scribble within the lines. Basically, all of the simple things you would want a 4-year-old to do.

He is in Montessori in the mornings and has 4 hours per week of gymnastics and 2 hours per week of yoga and 1/2 hour per week of horseback riding.

We do not limit the amount of food he eats, but we do limit the type of food he eats. He is on a high protein diet and is grain-free. Today he ate a lot and this is what he ate:

6 AM 6 almonds

7:30 AM 2 fried eggs, 1/4 cup whole milk yogurt with vitamins, 1 date, 1 turkey meatball

10 AM 1 square dark chocolate

1 PM 1/4 cup walnuts

2 PM 2 turkey meatballs

3:30 PM 1 egg white

6 PM 9 shrimp and 1 cup of cantelope

I wish I could get him to eat vegetables... I keep trying...

53 months

You know we work hard to achieve a low carb/high protein diet for Kian. Kian and I don’t eat grains or sugar or potatoes. I limit Kian to just one serving of fruit a day. He eats lots of almonds and walnuts and eggs and a variety of meat and shellfish. He eats the occasional vegetable. His diet is a work in progress and it definitely seems to agree with him.

Melanie Silverman, the pediatric nutritionists (www.feedingphilosophies.com), just analyzed his diet. He is at 60% fat, 15% carb, and 25% protein. She described it as Atkins-like with very high fat and very low carb. His protein is elevated, but not much higher than the 15-20% that is traditionally considered desirable.

The fat that he gets tends to be from nuts and farm-raised eggs and olive oil. We eat pretty much only pastured meat, so the meat tends to be on the lean side. Milk doesn’t agree with him, so he doesn’t get butter or cheese or milk, so no fat there. He does get about a ¼ cup of whole fat homemade yogurt (from grass-fed cows) per day, though. If you want to read more about what pasturing (versus corn-feeding) does to the fat profile of animals you can read up on it here (www.eatwild.com).

I don’t want to put words in Melanie’s mouth, so take this as only my notes from the conversation. Kian’s nutritional needs are being met, although she is concerned that he does not get enough calcium and has suggested that we add a calcium supplement.

Melanie calculated Kian as getting 1300 calories a day. She said an average 4 year old eats 1900 calories a day.

Kian is active (gymnastics, yoga, horseback riding) and often requests food and often stops eating and leaves food on his plate. We let him control how much food he eats and only limit him to the extent that we would limit any kid. For example, no snack right before dinner.

I find it interesting that he chooses to eat fewer calories than average. He receives 0.6 mg GH per day (38 pounds).

I know that Melanie is quite busy right now and has limited availability, but I want to remind folks that she is out there and has been following Kian since he was 5 months old. I appreciate her role in acting as a safety net as we reject the Goldfish crackers and work on this rather unconventional diet.

54 months

We started Kian on 1 teaspoon of cod liver oil in the evening 2 evenings ago. We decided to keep him on one gel cap of ultimate omega in the mornings. He had really bad diarrhea through the night and early morning on both days after the cod liver oil. I am going to stop the cod liver oil tonight, give it a few days and try again. (He had no trouble tolerating the B12).

We had parent-teacher conference yesterday. Kian is 4 and in an all morning Montessori preschool. No special aids or anything. We observed him last week and he seemed quite comfortable in the classroom. While observing him, we wrote questions to the teachers. We both wrote a lot of questions scrutinizing his fine motor and social interactions and every other possible thing that could be a deficit.

Well, not surprisingly, the teacher gave us what we asked for: a run through of precisely every way our boy deviated from the norm. It wasn’t the most fun conference that I have ever had, but I have to keep it in context of what we asked for. On the plus side, he is good at cutting and has mastered the basic works in the classroom and knows most/all of his letters. The basic Montessori curriculum is very cool and I won’t describe it here, but he has worked his way through practical life stuff and all of the basic counting work and the basic manipulatives and sensorial work. She identified 3 main problems.

1) He is very social. He has many friends and he is always stopping his work to help someone else or entertain someone else. He comes to school to party. He is the ring leader of a group of four boys, but is indiscriminate and will distract anybody given a chance. The teacher deals with this by sitting him (and whoever else) in tables by themselves and asking them every few minutes if they are now ready to work. Yesterday, Kian kept telling the teacher no and thought it was hilarious to sit there and watch everyone else work for half an hour. Hmmmmm… On one hand I like the fact that he is integrated in the classroom and has friends. Also, he is just 4 and I think it is a bit hard core of Dr. Montessori to expect all 4 year olds to buckle down and work. Perhaps this is just a passing thing. On the other hand I don’t like that I have the screw-off boy in the class when I am used to being the parent of the hard-working girls. Solution: try to let it go and let the teacher handle it. Also, we brainstormed in the car this morning (with the girls) how to balance the desire to socialize with the need to work. Plus, Kian just walked in the door and reported proudly that he worked hard all day today. I am not sure, however, if this is true…

2) He cannot write any letters. His handwriting is very light and his letters are barely recognizable. His hand tends to get fatigued easily. His peers can write letters. What to do? We are really going to hit hand exercises and writing exercises hard over the next month at home. If we don’t see any progress then I will have to find an OT and we will have to get more specific direction. Also, see if acupuncture can address??

3) He has trouble kneeling on the ground and going up to a high kneel and reaching out and getting out the map puzzle pieces and arranging them. He knows where to place them, but the physical work of kneeling on the floor is too hard for him. The teacher has noticed this with other works and, where possible, she adjusts by allowing him to do manipulative works while sitting at tables. She thinks that the maps are too big to make such an arrangement, however. Solution: yoga? Acupuncture? Practice the position at home? Also, his big sister wants to go in with him after school hours one day and work on a map with him. She wants to show him how to do it and see where he is having troubles. Hmmmm… Maybe buy a map for home?

That’s pretty much it. No food problems. Snacks (almonds) go well. The teacher only allows him to do one food work per day. This seems reasonable and is a rule that is made for many kids.

We also had his endo appt last week and that was uneventful.

So, I guess all the news is good. I have just got to get used to being the mother of the class clown.

55 Months

We started Kian on the B12 shot and it was really amazing. Kian is 36 pounds and he received Cyanocobalamin USP for injection, 1000mcg/ml; he got 0.5 ml or 500mcg IM. He received it in the morning and that evening he was balancing on on eleg and skipping and multitasking and loud. The effects seemed to wear off gradually over a week. It took us a few weeks before we could arrange for another shot. This time we were prepared. Kian had an OT assessment on day 1, shot on day 2, and post-shot assessment on day 3. I will post the entire assessment down below, briefly:

We had just started with this occupational therapist and we came to her with concerns about fine motor skills. She knew Kian had PWS and the university clinic purchased a couple of relevant assessments that she could perform. Kian was 54 months at the time of the assessment. One of the tests she gave him was the Bayley, which maxes out at 42 months. That would have been ok if Kian had a one year or greater (>25%) delay, which is roughly what they look at here when they qualify a kid for services. Before the B12, he did have that much of a delay, at least for his gross motor, testing at 37-39 months. After the B12, he maxed out the test. So, if he does have a delay, it isn't measureable on the Bayley and is <25%.

The BOT is more relevant for Kian's age, but is just gross motor. It showed him below average before the B12 (18th percentile for age) and average after the B12 (about 50th percentile for age).

We also has a pre-yoga assessment on day 1, shot on da 2, and post-shot assessment on day 4. HE could hold all of the yoga poses about twice as long after the B12 shot.

Finally, his teacher noted that his handwriting had improved and he was much more capable of doing work on the floor. His balance was much better and he was stronger.

Kian's OT assessment:

Kian is a sociable and friendly 4 year 6mth old child who had his motor skills assessed using The Bruinicks-Oseretsky Test of Motor Proficiency ( BOT-2: Body coordination section) and the Bayley Scales of Infant and Toddler Development- 3rd edition ( Fine and gross motor subsections) on the following dates: 12/03/08, 12/05/08 and 12/11/08. Below are the findings:

Bruinicks-Oseretsky Test of Motor Proficiency Pre-Test 12/03/08 Chronological age: 4 yrs 6mths 12 days

GROSS MOTOR SUBTESTS Total score point Scale score ( Mean=15, SD=50) Standard score (Mean=50, SD=10) Confidence Interval: 90%

Band/ Interval Percentile Rank Classification Age Equivalent Balance (subtests 2) Subtest Score Profile 8 8 3/5-11 Below average below 4 yrs old Bilateral Coordination (subtests 3) Subtest Score Profile 6 14 4/10-18 Average 4yrs - 4yrs 3mth Body Coordination Composite score profile Sum 22 41 6/35-47 18th Below average

BOT-2 assessment based on the body coordination section at a 90% confidence level found Kian performing below average at the 18th percentile level compared to peers his age level.

In the balance subtest portion for body coordination, he was performing at below average age equivalent to below 4 year age level. In the bilateral coordination subtest portion, he performed at an average level age equivalent to 4yrs - 4yrs 3mth age level.


Bruinicks-Oseretsky Test of Motor Proficiency Post-Test1: 12/05/08 Chronological age: 4 yrs 6mths 14 days GROSS MOTOR SUBTESTS Total score point Scale score ( Mean=15, SD=50) Standard score (Mean=50, SD=10) Confidence Interval: 90%

Band/ Interval Percentile Rank Classification Age Equivalent Balance (subtests 2) Subtest Score Profile 24 16 3/ 13-19 Average 4 yrs 6mth -4 yrs 11mth Bilateral Coordination (subtests 3) Subtest Score Profile 7 15 4/12-20 Average 4yrs6mth - 4yrs 7mth Body Coordination Composite score profile Sum 31 51 6/45-57 54 Average


BOT-2 assessment based on the body coordination section at a 90% confidence level found Kian performing average at the 54th percentile level compared to peers his age level.

In the balance subtest portion for body coordination, he performed at average level at an age equivalency to 4 yrs 6mth -4 yrs 11mth age level. In the bilateral coordination subtest portion, he performed at an average level at an age equivalency to 4yrs6mth - 4yrs 7mth age level.

Bruinicks-Oseretsky Test of Motor Proficiency Post-Test2: 12/11/08 Chronological age: 4 yrs 6mths 20 days GROSS MOTOR SUBTESTS Total score point Scale score ( Mean=15, SD=50) Standard score (Mean=50, SD=10) Confidence Interval: 90%

Band/ Interval Percentile Rank Classification Age Equivalent Balance (subtests 2) Subtest Score Profile 21 14 3/(11-17) Average 4 yrs4mths - 4 yrs 5mths Bilateral Coordination (subtests 3) Subtest Score Profile 7 15 4/(11-19) Average 4 yrs4mths - 4 yrs 5mths

Body Coordination Composite score profile Sum 29 49 6/(43-54) 46 Average


BOT-2 assessment based on the body coordination section at a 90% confidence level found Kian performing average at the 46th percentile level compared to peers his age level. In the balance subtest portion for body coordination, he performed at average level at an age equivalency to 4 yrs 4mth -4 yrs 5mth age level. In the bilateral coordination subtest portion, he performed at an average level at an age equivalency 4 yrs 4mth -4 yrs 5mth age level.

BOT-2 assessment summary: Kian performed better in the body coordination section of BOT-2 in both post tests conducted on 12/05/08 and 12/11/08 compared to pre test results. Although the post-test 2 performance scores were at the 46th percentile level which is lower than post test 1 results at 54 th percentile level it was still higher in comparison to the pre test scores performed at the 18th percentile level.

In the balance subtest portion, in the pretest he performed below average at an age level below than 4 years of age. However, in  the post test 1 (12/05/08) he performed at  average level equivalent to   4 yrs 6mth -4 yrs 11mth  level and in post test 2 (12/11/08) he performed at  average level equivalent to   4 yrs 4mth -4 yrs 5mth.  

In the bilateral coordination subtest portion, in the pretest he performed at an average level age equivalent to 4yrs - 4yrs 3mth age level. However, in the post test 1 (12/05/08) he performed at average level equivalent to 4 yrs 6mth -4 yrs 11mth age level and in post test 2 (12/11/08) he performed at average level equivalent to 4yrs6mth - 4yrs 7mth age level.


Bayley Scales of Infant and Toddler Development- 3rd edition

Assessment: 12/03/08

The Bayley Scales of Infant and Toddler Development- 3rd edition was used to evaluate Kian's motor development. The following scores were achieved on 12/03/08:


Raw Score Scaled Score Composite Score Percentile Rank Classification Age Equivalent

Motor Scale Fine Motor Subtest 62 18 >42 mths Gross Motor Subtest 65 10 37 - 39 mth

28 124 95 superior

Assessment Summary

Kian is a 4 year 6mth old child who demonstrated superior skills in motor skills, compared to peers at 42 mths 15 days, according to the BSID-3rd. The overall perception today is that Kian is functioning at more than 42month level in fine motor, and 37 to 39 month level in gross motor, based on the Bayley III.

Assessment: 12/05/08

The Bayley Scales of Infant and Toddler Development- 3rd edition was used to evaluate Kian's motor development. The following scores were achieved on 12/05/08:


Raw Score Scaled Score Composite Score Percentile Rank Classification Age Equivalent

Motor Scale Fine Motor Subtest 64 19 >42 mths Gross Motor Subtest 70 17 >42 mths Sum 36 148 99.9 very superior


Assessment Summary

Kian demonstrated very superior skills in motor skills, compared to peers at 42 mths 15 days, according to the BSID-3rd. The overall perception today is that Kian is functioning at more than 42month level in fine motor, and gross motor, based on the Bayley III.

Assessment: 12/11/08

The Bayley Scales of Infant and Toddler Development- 3rd edition was used to evaluate Kian's motor development. The following scores were achieved on 12/11/08:


Raw Score Scaled Score Composite Score Percentile Rank Classification Age Equivalent

Motor Scale Fine Motor Subtest 64 19 >42 mths Gross Motor Subtest 70 17 >42 mths Sum 36 148 99.9 very superior

Assessment Summary

Kian demonstrated very superior skills in motor skills, compared to children at 42 mths 15 days, according to the BSID-3rd. The overall perception today is that Kian is functioning at more than 42month level in fine motor, and gross motor, based on the Bayley III.


Bayley Scales Summary Overall, Kian performed at the 95th percentile level in the pretest demonstrating superior skills compared to children at 42 mths 15days. In fine motor he performed at more than a 42month old child while in gross motor he performed at the level of a 37-39mth old child. However, post test assessment found him performing at the 99.9 percentile level demonstrating performance at very superior level compared to children at 42 mths 15days. His gross and fine motor skill level was found to be at more than a 42 month15 day old level.


Overall perception

Kian chronological age 4yrs 6mths performed better in post test 1 and post 2 scores in both the BOT-2 and Bayles assessment compared to pre test scores. It was interesting that although his motor performance in post test 2 was slightly lower than post test1 scores for BOT-2, he was still performing better in the balance subtest at 4 yrs 4mth -4 yrs 5mth compared to pre test score at less than 4 years of age; and bilateral coordination at 4yrs6mth - 4yrs 7mth age level compared to pre test scores at 4yrs - 4yrs 3mth. Hence, in post test 2 his body coordination skills was being performed at 46th percentile level that l falls within an average range while in the pre test he was performing at the 18th percentile level that was below average for peers of his age.

February 2009

We give Kian B12 shots every 2 weeks. We gave him one yesterday and we still saw the boost. He is louder – sings at the top of his lungs every chance he gets. He is more assertive and squabbles more with his sisters. He had yoga today and his yoga teacher reported that he was much more focused and disciplined. His speech is a smidgen better and he is a bit less loose (better tone).

These effects are much less impressive than they used to be because he doesn’t seem to be losing the benefit of the B12 as quickly. He saw his OT two days before his shot and was doing so well that she thought he had recently had his shot. She hypothesizes that Kian has now established some neuromuscular connections. (who knows???) She did notice that he was fatigued by the end of the session.

Kian grew ¼ inch in the past month as measured by his pediatrician. Not that right before giving Kian his shot, Kian’s pediatrician commented that Kian was as hyperactive as any other 4 year old boy who comes into his office.

Kian has also had some recent gains. I cannot say for sure if they are related to the B12. We have been working hard on hand strength and handwriting. Kian was able to write his name (granted it is a short name!) on all 26 Valentine’s day cards. He did this by himself over the course of several hours and he needed to look at his name and copy it. But still, he did it and it was (mostly!) legible.

I think that his articulation is getting better. When we first started the B12 he started speaking so much and so fast and he seemed to have lost ground with articulation. I think that may be normalizing now. Strangers seem to understand him now.

He is starting to read. Maybe??!! I am still processing this one. I knew that Kian knew all of the sounds of his letters, but the teacher had commented a month ago that he was having trouble stringing sounds together to form words. Today he made a friend with some man with a scratch off board (sounds strange, but that’s my kid). Anyway, the guy started writing out words to see if Kian could read them and Kian could. Again this seems strange to me. Kian got 2 out of 3 words and I don’t know if this is random or Kian had memorized those words or what. I have to experiment with this one.

Kian went to the developmental pediatrician last week and graduated out. He is a pretty normal kid. I had my list of concerns, but the doctor told me to come back in 3 years if Kian was still having trouble with those things. 