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. My name is Lara and I live in Chicago. I am not putting my phone number or email on the Web site, but you can find me on Facebook or by asking at FPWR or ask Dr. Jennifer Miller. All of them can put us in contact if you think I can be of help to you.
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.
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.
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.
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.
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.
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.
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.
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!
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.
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.
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!
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.
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.
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.
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.
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
Jarrow carnitine fumarate http://www.bodybuilding.com/store/jarrow/lcarnitine500.html
Creapure creatine monohydrate http://www.bodybuilding.com/store/un/creapure.html
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.
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...
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.
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.
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
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
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.
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
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.
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
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.
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.
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 some were even 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.
The parent-teacher conference was today. The teacher has seen great improvements in Kian over the past three months. The last conference was right before we started B12. Now Kian can work on the floor more easily. He still talks with his friends, but he is better at finding appropriate places and times to do so. His handwriting is improving, and his hand strength and control (as evidenced by other Montessori works) has dramatically improved. She observed that Kian has more energy, stamina, and confidence.
Kian seems to be breaking away from one of his old friends, but the teacher seems to think that is because Kian is maturing more quickly than the other boy and enjoys more complex conversations and activities.
After our conversation in January, she is more careful to provide Kian support and give him more lessons if he is having trouble extrapolating from one work to another. That seems to be helping a great deal. She finds that with just a little bit more support, he can go much farther with the works.
He is where he should be with the curriculum and will be a lunch kids (full day kindergarten) next year!
I mentioned before that his thoughts are suddenly more complex. Here is another example. This morning we were running late and driving into school. Kian asked me (out of the blue): “What sound does a tiger make when he is hurt?” I responded something like: “I have no idea. What tiger?” Remember, that I was distracted and not doing my best parenting.
Kian thought for a second and said very carefully: “What sound WOULD a tiger make if he were hurt?”
Kian has the PWS deletion.
He gets Cyanocobalamin USP for injection, 1000mcg/ml. Right now he gets 750 mcg every 2-3 weeks. For a long time now he has also gotten 2500 to 5000 mcg or oral cobalamin (Solgar megasorb). These are all forms of B12.
Last week I switched to the “good” cobalamin – methylcobalamin. We noticed a dramatic decrease in his speech. Yesterday he was stuttering a lot. The disfluency was irritating and undermined his confidence. He had stopped expressing ”big” ideas. Yesterday was especially sad as he tried to stammer his way through telling me basic things about his day.
Last night it dawned on me about the methylcobalamin. I did not give him any B12 today. Today was the washout and tomorrow I plan to start him again on the plain cobalamin. This morning was bad. He really couldn’t express much in the car on the way to school. Lunch time was bad and he just didn’t want to talk. He took a nap and met his father at the door. After a big hug he said clear as could be: “I have to get back to my computer game now.”
This reminds me of years ago when I had Kian on the Nutrivene formula which also has lots of methyl donors. I ran out one time and was surprised by his speech improvement. So, I stopped using the Nutrivene…
I do not know if this info will apply to kids with PWS by UPD. My suspicion is that somehow the methyl groups are methylating his precious maternal copy and turning off whatever function we may have coaxed out of those genes.
Who knows? Whatever the reason, I feel horrible that my poor kid lost his ability to speak and am now much happier that he seems to have regained it.
One other possibility is that the methylcobalamin is more bioavailable and I was simply overdosing.
Kian turned five last week. He wanted a Dora the Explorer party. Well, we dote on him, so he got what he wanted complete with Swiper and an adventure to find a coconut (one of his favorite treats). He had 4 friends over and had a lovely party. I gave each kid a piece of dark chocolate and home made coconut and mango “ice cream.” Kian was so excited about the party and the presents that he didn’t eat either. He ended up circling back for the chocolate an hour later and I tucked the ice cream in the freezer so that he could have it the next day (for breakfast!).
He is doing very well, beyond what I would have ever dreamed possible 5 years ago.
He is in a mainstream Montessori school and will be a lunch kid next year. This means he will go all day to kindergarten. He is pretty much with the other kids in terms of the Montessori curriculum. He knows his letters and is starting to read. He can write his name and most of his letters and numbers. His handwriting still isn’t very good, though, and we are working hard on it.
He does mainstream gymnastics and keeps up with his peers. He can run down the tumble track and do a diving somersault. He can alternate tuck jumps and straddle jumps on a trampoline. He can walk down a balance beam. He can skip. He can push himself up on a bar and then roll over the ball and come down on his feet. (I forget what that is called.)
His language is good, but he still has articulation problems. At the last assessment he had a 25% delay in articulation. I think that may be a bit better now. I will have him reassessed next month.
He wears glasses because he has intermittent V-pattern esotropia with vertical incomitance. He still has binocular vision and his strabismus seems to be corrected by the glasses (bifocals). The glasses are cute and all, but I think that the eye problem has a lot of implications for his life. He really can’t catch balls (although he can throw). His depth perception seems to be off when climbing on new playground/gym equipment. He has trouble writing in boxes (large grids). All of these things may sounds subtle and I guess they are in the great scheme of things. I am just puzzling a lot about eyes right now and the role that perfect binocular vision plays in navigating the world. We are working with the OT on this and may start some sort of eye/vision therapy soon if I can figure it out… Oddly enough, his eyes seem to have been getting better over the past couple of years.
Kian is in all day Montessori school. I know the teacher very well as she has been teaching one or another of my kids for 8 years now!
She has been great with Kian and I have a lot of confidence in her. That said, Kian has gained 3 pounds since school started! He has gone from 47 to 50 pounds. Even Kian’s doctor was concerned by the gain. I was puzzled and frustrated and I asked Kian what he was eating at school.
He answered cake. Which didn’t seem right, but got me on the phone with the teacher.
Well, Kian has always had access to food work at school. Montessori is big on self-care and kids learn to cut and serve fruit and share it and they get their own snacks, etc. All of this has been fine with Kian, although there was always a rule of only one food work a day. Which is a reasonable rule for any kid, I think.
Montessori has multi-year classrooms and Kian is now a big kid and a lunch kid. He and his gang rule the class and show all of the little kids the ropes. He is also not supervised quite as much. And, as it turns out the teacher puts out banana work at the beginning of the year because bananas are really easy for little three-year-olds to cut and it makes them happy to be able to cut and eat their own banana.
Hmmmmm… Kian took it upon himself to help teach the little kids the banana work. And the apple work. And the end result is that he was eating a banana and half of an apple a day.
Typically we limit him to only one piece of fruit a day.
The teacher wasn’t really on top of it, because we never said much about fruit. We just told her no grains and no sugar.
Now she realizes that Kian shouldn’t get much fruit either in class, so she is going to limit him to only the carrot work. One carrot work a day and after that he has to do big kid work (like long addition!).
The bottom line, as I see it, is that Kian’s behavior was very age appropriate. What wasn’t appropriate is his crazy body’s response to the fruit.
We have had a long weekend (Friday off of school) and he has already dropped one of the three pounds. This is despite eating a pound of ground beef one day for lunch! Hopefully he will drop the rest of the weight quickly.
We relied heavily on bananas to keep Kian gaining weight when he was 6-24 months. I wish I had not done so because I think that they just put on fat and don’t actually give him real energy. I would suggest, instead that folks consider using avocados and egg yolks. At one point we were feeding Kian a lot of bacon (organic and no nitrates, but still bacon). People would roll their eyes at us as if it was the worst thing in the world. I know bacon isn’t great, but it filled an important need at the time. Kian would eat it and we could guarantee that we were getting protein and fat in him. There were days and meals where that was very important, especially when he was little.
Now he has one of the widest palates of anyone little person I know. If I could only get him to eat more greens…
Kian astounds us. He had another language burst and has very complex ideas and his articulation has dramatically improved. He blows me away at gymnastics. Today he walked up and down the balance beam on his tip toes!!!!
He is fearless on a horse. He muscles that pony around the ring, going over poles and trotting independently. (And when I say independently, I mean independently -crazy little kid on a pony by himself thumping his legs on the side of the pony and steering like a madman!) He posts and rides on two point and waves and turns like it is the most natural thing in the world. His handwriting is getting much better. He is starting to recognize words on signs as we drive past.
We have chosen to manage Kian’s PWS by assuming that his bodily has a higher need for nutrients than most children’s bodies and that he needs to eat nutritionally dense foods often. In my opinion, he had a toddler’s need to eat every couple of hours up until he was five. He is just now losing it. I always send him to playdates with almonds and tell the moms and nannies that if he says he is hungry, he probably is and, moreover, that he should be stopped to eat a handful of nuts every couple of hours or so – sort of like you would do for a toddler.
Now he is five and I don’t notice the toddler-like need to eat so much. I would say, though, that after he works out hard, he is not hungry, but I really should make him eat a little something, because if he gets swept up in activities without eating, his mood becomes more labile and he yells more. We are really working on yelling in our house.
So, to sum up, we sort of manage PWS the opposite of what is frequently recommended. We do not limit calories. We feed often, even stopping activities for a bite of food, we focus on nutrient-dense food even though those foods may be very high calorie. We rarely if ever allow nutrient poor food – no cake or bread or candy, for example.
I know that there are a handful of other parents who are managing PWS this way and they also seem to be having success. By success I mean: normal cognitive development, high energy/activity, no food seeking, and stable mood/normal emotional development.
I would love to see this studied further to learn if Kian is an outlier or if many kids with PWS would respond in this way to a nutrient-rich diet.
Kian weighs 50 pounds and gets:
Nordic Naturals Ultimate Omega http://www.vitacost.com/Nordic-Naturals-Ultimate-Omega - 2 a day
NOW B-100 http://www.bodybuilding.com/store/now/b100.html - 1/4 capsule a day
Jarrow carnitine fumarate http://www.bodybuilding.com/store/jarrow/lcarnitine500.html - 1 a day
Creapure creatine monohydrate http://www.bodybuilding.com/store/un/creapure.html - 1/2 scoop a day
500 mg of cyanocobalamin injected im once a month
For the last 6 months we have been battling (off and on) diarrhea in Kian. What this means is that he would have a fecal accident a few times a week. His stool was always loose, and he would run to the bathroom and have 5-6 bowel movements a day. The poor kid. What this also meant was that he more or less stayed in Pull Ups. Note that this was getting to be a bummer because he is a mainstream Kindergarten kid with an active social life.
I have no idea why he had this problem and I was unsuccessful in engaging his endo in the issue. Basically most kids with PWS have constipation and they didn’t know what to do with Kian.
Of course, there is always Oneida, who seems to have never met a problem that she won’t tackle (and beat!). So she listened to me whine and helped me…
I started out wondering if he had a GI infection. I gave him probiotics (Threelac and Florastor kids). No difference. Then I wondered about supplements. I took him off of Vitamin A and oral B12 . Still no difference. I looked closely at his diet. No pattern emerged. I stopped giving him the limited amount of homemade yogurt that I give him. No difference. I took him for acupuncture. No difference. I massaged his belly. No real help.
Finally I gave him half a capsule of Ferrochel (iron). Logic: Oneida thinks he should probably be getting iron; we both think that iron is constipating.
It worked! Now he just has a bowel movement a couple of times a day and he actually has to sit on the toilet for few minutes in order to go! This is a huge improvement for us.
I am not sure that this discussion is relevant for most (any?). But I thought I should document our solution here. I still don’t know why Kian has loose stools. I feel that I am masking some underlying problem. That said, I feel that I am masking it in a low toxicity “natural” way, so I am satisfied.
Well, Kian is in an interesting place right now. He is in a phase where he gains weight very easily – even more easily than before. He has really bulked up. He doesn’t stick out in a crowd, but he is no longer a slim kid. He is also growing taller and his own GH seems to have kicked in because his IGF levels are high and the endo is having us dial back his GH levels. He now gets 0.5 (50 pounds).
The good news is that the weight doesn’t seem to be compromising his movement and exercise. He can now swim across a large swimming pool by himself and pull himself out of the water and jump back in over and over by himself. And, he can do 150 seat drops in a row on the trampoline and he is training to do a back flip. And, he can ice skate (but not very well). And, he can do a great plank in yoga.
Also, he doesn’t seem to have tremendous hunger.
So, now we are really focusing on snacks versus meals. A snack is small and you are supposed to go to the dinner table hungry. This irritates him a bit, but no more than it irritates my girls.
His reading is really coming along. In fact, it surprised his teacher how well he could already read when she first started introducing reading to him. His handwriting is also really coming along now.
I guess our main concerns now are weight and still some speech. Although, his speech really isn’t that bad. He sold raffle tickets at the boys gymnastics meet. We weren’t there because we weren’t invited. The coaches just invited Kian to help. Well, turns out that Kian broke all records for selling raffle tickets. And, the boys meet was about a quarter the size of a girls meet. My husband went to pick up Kian and one father went up to my husband and said something like: I run a sales team and your little boy is the best salesman I have ever seen. He has no fear and he is charming and persuasive. I wish I could bottle what he has and use it in my sales force.
My little guy is also at a small private school where he is cherished. In our case, we are at a Montessori school. He is my third child and so I know this school very well and they know me very well. I sent through two bright, hard working, athletic girls, and then I show up with Kian. They pledged to love him and care for him.
Sounds perfect, right? But, I have seen over the years that they have loved and cherished children who went on to be identified as having special needs and learning disabilities. In many cases, the parents had to make the painful decision to pull the child because the teachers weren’t aggressively flagging problems.
I believe this is because the teachers are kind and nurturing and hope that the problems go away. I also think that the teachers have close relationships with the parents and know the struggles that the parents face and don’t want to add to the burden. I also think that loving schools that believe in personalized education loathe to use labels. Finally, at least in the case of our school, I don’t think that the teachers are well-trained to identify developmental delays that are best suited to therapy.
So, when Kian started school I told his teacher that we knew that she loved him and that he was wonderful. We wanted to know every time he deviated from the norm and we wanted to work with her to solve every problem. This was painful. We had to hear that he couldn’t roll a mat and that he couldn’t work on the floor because his legs weren’t strong enough to do a high kneel. We had to hear that he was eating too many bananas and that he couldn’t generalize between works. But, we didn’t shoot the messenger. Instead we strategized. If he couldn’t do floor work, then could the work be modified for a table? At the same time we did exercises at home to strengthen his legs.
There are an infinite number of problems that Kian had at school. All of them were small, but all of them could have big repercussions. Conferences were horrible. Instead of hearing (like my daughters) how wonderful my kid was, the teacher nitpicked every hand movement and choice of work. We had monthly calls about whether he was fitting in socially – and we strategized solutions for home and school.
Anyway, I don’t know the ending to my story. I can just give you my thoughts, for what they are worth…
Kian is slimming down. I am not sure how or why, but he now weighs less than he did last August. We went to the endo and his IGF-1 levels are still high. He has been dialed back to 0.4 mg/day (50 pounds). Gymnastics is going very well.
School is going well. We found out that Kian will be graduating from kindergarten and moving up to first grade. He is doing well with the Montessori curriculum and is well on the way to reading and doing math. The teacher is not able to identify any learning disabilities.
I have bad eyes, Kian’s daddy has bad eyes and Kian has really bad eyes. He has v-patterned accommodative esotropia (strabismus) with mild myopia and astigmatism. He has just now developed ambylopia (one eye is starting to shut off) and needs to wear an eye patch. Yikes! This means he is always going to the eye doctor and getting new glasses and he has trouble catching balls.
On the plus side, his reading and writing are fine and he is good at using his eyes to navigate the environment.
We just started him on vision therapy. I looked around a bit and found a vision therapist who would work with our main eye doctor (a strabismus expert). I have insisted that all vision therapies done with Kian be complimentary and not alternative. And, I have to say, I have been pleased. It has just been a few weeks, but I feel that he is using his eyes better. He seeks out up close work to do and does it more easily. Also, he had started complaining about headaches and those complaints stopped.
Kian turned 6 on May 21.
I have two other kids and we always have small birthday parties at home. Kian, however, wanted a large party at the gym and we gave in. There were about 20 kids – mostly his classmates. Pretty much every child came and many of them cancelled their activities to come. The parents commented on how eager all of the kids were to go to Kian’s party! It was a multi-age group (ages 3-6), but you really couldn’t tell the difference between Kian and the other kids. Kian walks a balance beam, does diving somersaults, does back flips with the help of a mat, does seat drops and straddle jumps on the trampoline. He climbs 10 feet up a rope with knots and he swings along a bar to drop into a foam pit. Really, it is extraordinary and I would never have believed it possible.
He talked a friend of the family into baking shortcakes out of almond flour and we piled them high with fresh strawberries. Most of the kids really liked them. He gave away pretty tins of mints (Newman’s Own).
Kian will graduate from kindergarten (Montessori) in a week. Since it is a Montessori classroom and they have midyear graduations, some of his classmates have been invited to stay in kindergarten for another semester to master some more of the basic skills. Evidently, Kian’s academic work is pretty good and he was encouraged to move up to first grade. He writes and writes cursive and is a beginning reader and he does addition and subtraction.
He is a spirited boy and manages to get into trouble a fair amount. I wish this weren’t the case, but as I understand it, his getting in trouble falls within the range of a bad boy, not in the range of pathology. He is a very independent boy and he is full of many plans. He is quite good at getting people to follow him with his plans.
He has weak eyes and wears glasses and we are working on vision therapy. He still has problems with articulation. This is a bummer because he has a lot to say. He gets speech therapy at school. He can pretty much be understood by everyone, but I can tell that they may have to replay it in their head to understand him. He doesn’t get any other traditional therapies. He has private gymnastics lessons twice a week and private yoga once a week. Every week or so he receives acupuncture from a pediatric acupuncturist.
We worried about rapid weight gain in the fall, but that has now stopped. We are blessed that there is no food seeking. He is at the 60th percentile for height and 75th percentile for weight, which is heavy for our family, but he doesn’t stick out as being heavy in the classroom. He eats primarily protein and vegetables. We allow very few grains, sugar, or processed food. He also gets many metabolic supplements.
I am realizing that Kian is atypical for PWS and that what I notice about him may or may not be relevant to other kids. I do not know if it is because we are lucky or because of things that we have done (probably both). Here are my observations of Kian and interpretations of my observations.
I believe that Kian has regular growth spurts just like other kids. He goes through periods where he is barely interested in foods and periods when he wants to eat a lot. When he wants to eat a lot (as happened last fall) it as if his body doesn’t know how to use the extra calories. My girls have always eaten a lot, gotten taller, and then slowly filled out. You know how some kids fill out and then get taller? Well, Kian seems to be sort of an extreme version of filling out and then getting taller.
I decided not to limit his food during this growth phase. Instead, I just was very careful to give him lots of protein and good fat. I made sure that he could satisfy his hunger with that so he was not tempted to eat carbs. This odd-looking growth phase lasted for about 4 months and it was scary. I was afraid it was the beginning of the end. One night he ate a pound of ground beef. But, I stayed the course and did not restrict calories. I did limit snacks and implemented the family rule that a snack is a snack and a meal is a meal. This helped me to focus him on sitting down and eating meals as opposed to loading calories mindlessly while running around. Kian gained weight. He was 5 years old and he went from 48 pounds to 53 pounds in the course of 4 months. It was scary, but I just hung on. I put a lid on carbohydrates, kept him active, and limited snacks to celery, a hard boiled egg, or a few almonds. Meals were virtually unlimited affairs of protein and vegetables.
Then, after 4 months, he stopped gaining weight. And we measured him and he had grown half an inch. And, he lost interest in food. And, he started losing weight. Eight months after the beginning of the weight gain, he was back down to 48 pounds and had grown another half inch. And, he was stronger, and smarter, and more muscular.
So, in retrospect, he looked like my daughter when she went through a growth spurt. The slip of a girl could eat a whole pound of steak in one sitting. Or a whole can of salmon with mayonnaise or 4 hard boiled eggs or ¼ cup of peanut butter and she would circle back around an hour later for fruit, a smoothie, something sweet, or all three. We don’t have excess carbs around the house and so she turned to protein and ate and ate. She was about 6 years old at the time. And, she shot up. She stayed skinny and is only now (at 9) starting to fill out a bit, but she had a huge feeding frenzy. Now she is filling out a bit and she hardly eats anything.
I think that the metabolism of growing kids is mysterious. If your kids are healthy and thin and you provide them with unlimited healthy food options then there is rarely a need to think about it at all.
But, heaven forbid you have a kid with PWS, then you second guess every bite and every pound and every meal of apparent gluttony. I work hard to be peaceful and be in the moment with my little boy and not project a horrible future on his every action and choice. This is a big lesson for me. I have made some progress and he provides me with many opportunities every day to perfect my understanding.
I would add one other thing and that is that when Kian has a higher carb day, the next day he is hungrier than usual and craves carbs. While I notice the same thing for myself and my girls, it is quite pronounced with Kian. When I look at it through my fearful PWS eyes, it looks like THE HUNGER. It goes away, though, if I focus him on protein and vegetables. I point out to him that it can’t be hunger since he can have all of the protein and vegetables that he wants to eat.
Letter to Kian's teacher as he starts first grade in a Montessori school:
Please feel free to share this with anyone who is interested. Kian’s syndrome is something I willingly talk about. Keystone is our home and members of the Keystone community have nurtured Kian and supported our family since his birth. Kian has a genetic syndrome (Prader-Willi Syndrome). This means that he has identified and well-characterized challenges. I tell you about these challenges so that you can join us as we help him to overcome them. We do not believe that Kian’s genetics represent his destiny. Our goal for him is an independent and happy life. We understand that he (and we) will have to work hard to achieve this goal. We intend to work hard and appreciate all of the tips and suggestions that you can give us as we travel this journey with him. Kian is missing a small piece of his paternal chromosome 15. It only amounts to a few genes, but these genes have many ramifications throughout his body. PWS is recognized as the most common genetic cause of life-threatening childhood obesity. I have attached a fact sheet about PWS. When Kian was born he looked like a classic infant with PWS. He could not move, could not cry, could not open his eyes, and could not eat. Today, Kian does not look or act like a typical child with PWS. We attribute this to incredible good fortune, his hard work, and the support of a large and loving team of therapists and teachers and caregivers. Right now, for Kian, PWS means: 1) Food – Kian is on a very low carbohydrate diet. He does not eat grains (bread, pasta, crackers) or sugar (cookies, cake, candies) and he eats very little fruit (1/2 piece per day). When he accidently gets carbohydrates his brain becomes foggy, his behavior becomes irrational, he becomes uncoordinated, he begins to complain of hunger, and he may begin actively seeking (even stealing) food. He also gains weight very quickly. We will pack lunches and snacks for him that are primarily protein (almonds, meat, eggs) and vegetables (celery, red peppers, carrots). We can send in dark chocolate for you to give him as an alternative when kids have cupcakes or cookies. If you choose to have junk food/game days, can you give me a heads up and I will figure something out when that time comes?
2) Eyes – Kian’s eyes are weak and he has crossed eyes. This is controlled by his bifocal glasses. He needs to do up close work by looking through the bottom of his bifocals. This means that he cannot do up close work on the floor – the bifocals don’t work properly from that angle. Sitting on the floor during lessons or class reading is fine. We ask that, whenever possible, you modify work so that he can be at a table. Also, if you think about it, can you remind him to push his glasses up his nose?! Kian is currently in vision therapy which means that every night he comes home and does half an hour of exercise to strengthen the muscles in his eyes.
3) Exercise – Kian is at risk for obesity and he naturally has weak muscles. In order to compensate for this he is active in gymnastics, yoga, and horseback riding. He gets private gymnastics lessons twice a week. Last year we did this on Tuesday mornings and he came to school late on those days (about 9:45). Do you think such a plan would work in first grade?
Please call me with any questions (XXX-XXX-XXXX) and please flag any and all potential problems that you see with Kian in your classroom. Of course I don’t enjoy hearing bad news, but anything that you see now can be woven into a plan to help make Kian stronger and give him an independent and happy future. Kian is so excited to start first grade!!!!
Kian is working out so hard right now it is unbelievable. He is crying from the muscle soreness sometimes, but then he just stretches out and then goes right back to it. He even does the work himself in the mornings (push ups, etc.).
He learned how to run! His gymnastics coach taught him to lift his knees and throw his chest up. He looks beautiful and he flies! He loves the feeling. Now he is running everywhere (and his legs are sore). His coach has extended his privates to 1 1/2 hours and Kian wants to do 2 hours! And, he works that entire time.
Yesterday he got in trouble for trotting his pony independently over the poles. He was supposed to walk, but he had seen me trotting and decided trotting would be more fun. (Note that his behavior is a bit challenging at the moment. He is quite independent and head strong.) Well, he did it successfully, but then Ms. Denise put him on a lunge line for a little more control. He pretty much trotted (and posted) for half an hour. (This was after an hour private gymnastics lesson in teh morning.) She said his core and shoulder strength was dramatically improved from 3 weeks ago. He showed no fatigue. Then she let him trot independently. He wanted to trot around the ring, but she had him trot across the ring to a "catcher." Trot, trot, trot, and he slowly slipped off. The good pony stopped. No harm.
Kian dusted himself off, got back on and kept trotting the pony independently!!!! Now, I think that is a sign that the kid has low levels of anxiety. Most kids would not be able to get back on a pony and keep trotting. Ms. Denise (and I) were pleased about the fall. We felt that it was an inexpensive and much-needed lesson in humility. The boy is getting big for his britches. It drives him crazy that he can't do everything that his sisters do.
His body is changing again. I think it is the muscle mass. He is still a bit on the heavy side, but he carries himself differently. His shoulders are back and square and he appears more trim. He moves like an assertive and strong boy.
I had a conversation with another mom over the weekend that I thought was interesting. The mom referred to Linda Gourash’s (sp?) work and her discussion of how the pleasure centers of the brain of people with PWS may be highly/overly developed. This is typically seen by the eyes trolling back, immersed, blissed out state that they sometimes demonstrate when eating.
Well, Kian doesn’t do this. But, I have to say that he has the most sophisticated palate of most anyone. His appreciation of a good piece of fish or a good soup or a perfect piece of fruit is really unbelievable. When he saw the opening scene of Lion King, he had his hands to his heart and tears were streaming down his eyes. He absolutely loves his favorite friends and favorite teachers and would do anything for them. I could go on and on, but it is true that he takes an amazing amount of pleasure out of many aspects of life.
If I paint my kids in broad strokes, I would say that one is quite serious and cognitive; one is sheer joy in motion; Kian is very appreciative of the pleasures (big and small) that the world has to offer. He takes intense pleasure in moving his body and mastering new skills and completing a book. He is not a total hedonist, but I believe that he could be if he didn’t also have an amazing work ethic and a take intense pleasure from a job well done.
This was a new realization for me and I thought I would share. I don’t know if it is just a Kian thing or if it is an odd manifestation of a PWS-enhanced pleasure center.
Briefly, Kian is in a mainstream Montessori class – 1st grade. He receives weekly speech therapy for moderate articulation issues. Otherwise, he doesn’t receive any special services. He does find academically (probably average). So far the teacher has not flagged any learning disabilities. He has visual-motor problems that manifest as poor ball skills and poor drawing skills. His handwriting is fine, though, since we have drilled that.
I know this sounds crazy, but he is athletic. We are a gymnastics family and all of my kids (including Kian) are gym rats. He does what advanced 6 year olds do at the gym (diving somersaults, pull overs, handstands against the wall). At practice he has done 600 seat drops in a row! He was invited to join the boys team this summer. He is very young and he doesn’t have all of the skills, but the coach is impressed with his enthusiasm and work ethic. The coaches recognize that he is a little more uncoordinated and slower to build muscle than other kids, but he works harder than pretty much any other kid and so he excels. He also works to keep up with his sisters who are quite athletic.
His behavior is fine – for a first grade boy. I am always diagnosing him with something or other, but his teachers just remind me that he is a boy and that sometimes he is a bad boy and that is all. He mixes it up with kids: has friends, gets in fights, has crushes on girls, girls have crushes on him. He is extraordinarily charismatic.
He eats protein and vegetables and has pretty much learned to manage his own diet. He eats very high quality food. He is an expensive kid to feed and he eats like a prince. We do not count calories or limit calories or servings, other than I would do for a regular little kid who I know is eating too much and will get a tummy ache. Don’t we all have trouble holding back with a plate of yummy food?! He has been actively/annually followed by a nutritionist (Melanie Silverman) since birth. She calculates that he eats the same amount of calories as any other kid his age (although I know this is a wide range). He eats 60% fat, 25% protein, and 15% carbs. He is very sensitive to carbohydrates. Eating a handful of crackers or a cookie brings on disorientation, food cravings, behavioral outbursts, slurred speech, fatigue – basically the PWS phenotype. He hates the feeling and so is pretty good at avoiding carbs. We are all still learning, though. We have found, for example, that he can do ok with a bit of a very rich dessert after a meal – chocolate mousse or caramel flan. In contrast, if you give him a piece of bread on an empty stomach, it is disastrous.
He is well-travelled. My husband is from Singapore and so we have travelled there many times. Kian has also been to France and Thailand and Malaysia and Spain and Costa Rica and Japan and the Bahamas. I think it speaks to his flexibility and behavior that he can comfortably travel in all of those different environments. He can always find something to eat – meat and vegetables! And, he always makes friends!
He is not finicky and enjoys trying new foods. He also enjoys helping me in the kitchen. We take food very seriously. The Omnivore’s Dilemma is one of our bibles. That and World’s Healthiest Food (www.whfood.org) and Alice Waters cookbook. We enjoy eating, eat locally as much as possible, and set aside a lot of time for enjoying food (and each other).
Kian gets a lot of supplements.
For energy: carnitine fumarate (helps to burn fat for energy) CoQ10 B50 B vitamin supplement B12 shot every month or two
For smarts and mood and to reduce inflammation: 6 Nordic Natural omega-3 fatty acids generous doses of coconut oil in his food throughout the day
For muscles: Creatine monohydrate
For lots of things: iron (ferrous bisglycinate) vitamin D (4,000 units/day)
For water balance: Natural Calm magnesium vitamin C with bioflavinoids selenium once a week zinc once a week 2X a day shotglass
He is also on a modified Atkins diet. He eats less than 20 grams of carbs a day. WHen we test his blood ketones, they are at 2. When we use the urine ketone stick, he is at the maximum level. He eats less than 10% of his calories from carbs, 60+% from fat and 25+% from protein. We use coconut oil liberally. When we monitored his calories, we found he was eating 1350 calories a day. In an effort to get him to drop weight we have cut his calories to 1250 pounds. He doesn't seem to notice the reduction and remains full as long as he is in the ketozone. He is 7 (almost 8) and weighs 65 pounds. We would love to pull 5 pound soff of him.
We have significantly reduced carbs and increased fats in an effort to get Kian to burn fat for energy and produce ketones - sort of Atkins style. Sadly, it is not the secret to weight loss for him. It does, however, make his brain run beautifully and suppress his hunger. I borrow heavily from the epilepsy community and ketomoms. I got this from them and call it a peanut butter ketocandy - one part heavy cream + 1 part coconut oil + 1 part peanut butter (sugar free). Blend together until smooth and then put in a candy tray. I use a cute heart Ikea hart ice cube tray. Freeze. Each candy in my tray is 1 tablespoon, so each candy is 100 calories. The candy's do a good job of suppressing Kian's appetite.
The most amazing thing is that he passed his standardized test (mainstream 2nd grade, no aid and no IEP). They don’t test in Montessori school and so the standardized test is pretty much the only encounter that the kids have with tests or worksheets or things like that. Also, the standardized tests are sort-of based on a curriculum, learning strategy that doesn’t quite overlay the Montessori learning plan. So, in general, the standardized test underestimates the knowledge of the kids.
Scores are 1-10, with 4-6 being normal. Kian got pretty much straight 4’s! Maybe for most kids, that isn’t much to holler about, but I am amazed and impressed.
Also, the teacher said that last week (first week after spring break) Kian was like a completely different student. He didn’t fight her. He listened. He applied himself to his work. She said that if he can keep that up he could be one of the smarter kids in the class!
And, perhaps sweetest of all, his social skills are kicking in. He is mixing it up with the other kids, playing chess and tag and whatever else kids do during recess. She said that he has come a long way in 6 months.
So, is this maturity? The ketobrain? Increased GH? Aunt Gayle’s cognitive behavioral therapy?
We are delighted. He continues to amaze us and close the gap.
He is in a very good place right now. His brain is zipping and his behavior makes me proud. He is on modified Atkins/ketogenic diet. His blood ketones hover around 2.
We just (couple of weeks ago) upped Kian's GH from 0.6 to 0.8. He also received his GH shot on Friday. This morning he got up and, on his own volition, climbed 101 flights of stairs! He is looking good. Actually, today his size 8 skinny jeans were loose around his waist and short at his ankles.
All is still going well. He has above average intelligence. He is doing well academically in school. By well, I guess I mean above average for a boy. He is not a phenomenal student, but he also isn't horrible. He seems to be keeping up with his work (mainstreamed 3rd grader).
He is mischievous and getting in trouble at school, but his teachers laugh at me and claim that I have gotten the normal little boy that I have worked so hard for. His behavior is driving me crazy. He is pushing every single boundary. He doesn't have outbursts, but he is testing constantly. I keep working on him to listen to his Jiminy Cricket. I had no idea how real Pinocchio could be.
This is odd. He has binocular vision. He has been in bifocals since age 3. He has had three years of vision therapy. He has 20/20 binocular vision. His eye doctors find him a bit puzzling as he has come a long way from strabisumus and ambylopia to 20/20 binocular vision. He is able to use both eyes together and smoothly shift from one eye to the other with both eyes being equally strong. The doctor was amazed at the versatility of his visual system. The uncertainty lies with how much stamina he has for binocular vision throughout the day. We are going to do another push with vision therapy to build stamina with his eyes. As his academics become more rigorous we want to make sure his eyes can keep up. Kian has acknowledged that when he reads for a while (30+ minutes, his eyes get tired and need a break).
We are weaning him off of fiber and so far we are pleased with what is happening. He was developing a lot of mucuous in his stool and I found it alarming. I wonder if the psyllium was an irritant. We are down from 6 teaspoons of fiber a day to 1 teaspoon of fiber a day. The mucous is gone and so far he is regular and his bowel movements can be controlled. He is also less bloated. We are giving him daily culturelle probiotics. Fingers crossed we can get to the bottom of his gut issues.
I am trying to back off and give him more Daddy time. They go off together and fix the tires and run "manly" errands. :) Today LJ is going to watch the game with him a bit so that he can learn about football!
Tonight he told me, "I can't wait until I go to college." I asked him why. He responded: "Because then you can't kiss me so much!" Arghhhh! That boy!
He is 9 and 70 pounds. I will list his supplements and a brief explanation for why we use each supplement. Note that these supplements have pretty much not been tested in kids with PWS, so I am giving my best guess. I could write a document on each one of these supplements and my thoughts, but I am too tired... So, here is a start.
Every 2 months he gets 0.75 mg of cyanocobalamin as a shot - energy and myelination of the nervous system and maybe??!!) turning on his silenced genes
Every morning he gets: 25 mg Gentle Iron - energy and myelination 1000 mg carnitine fumarate - getting fat into the cells so he can burn it (energy) 1 Kids packet Culturelle - normalize his gut and perhaps his nervous system 1 Cytomed CoQ10 10 (90 mg) - energy 1 B complex 100 - energy 3 gel caps Nordic Natural Ultimate Omega - anti-inflammatory, cognitive function, stabilize mood, and promote speech
Every evening he gets: 1000 mg carnitine fumarate 1 CytoMed CoQ10 3 Ultimate Omega 1 Albion Chelated Magnesium (133 mg) - nervous system and bones and muscles plus maybe he is deficient base don his diet 4,000 IU of vitamin D3 - anti-inflammatory, nervous system
Approximately every other day he gets: 22 mg zinc picolinate - immune system plus maybe he is deficient because of his diet 100 mcg selenium - immune system plus maybe he is deficient because of his diet
He just started 4th grade and today I got a call from his teacher complimenting on how hard he is working (mainstream Montessori school, no aid). He just sat down to read the Black Beauty Classics Illustrated book and has read it for an hour solid while I have worked. He comments periodically (in horror) about how the animals are treated. He is learning two digit multiplication in school. I looked it up and was pleased to discover that this is 4th grade math. He scored 86 on his timed (100 problem in 5 minute) math test on the ipad (so he didn't have to write). So far, so good...
This is what he ate today (9 years old, 70 pounds).
Sample day of Kian's menu (age 9, 70 pounds)
Breakfast: Ketochocolate 4 small strawberries omelet made with 2 eggs, clove garlic, tablespoon butter, 1 oz goat cheese
Morning snack: 15 grams shaved coconut
Lunch: 3.5 ounces duck breast 1 cup cream of mushroom soup
Afternoon snack: 15 grams shaved coconut ketocandy (1 teaspoon coconut oil with 1 teaspoon almond butter)
Dinner: small pork chop, generously covered in butter sauce raspberry lemon ricotta parfait
Top off: Ketocandy
He is 9 and 75 pounds. He had a huge growth spurt this past fall. I think in response to the MCT oil.
We just returned from 2+ weeks in Bavaria and Austria. He is a fabulous traveler. He carries his own backpack and pulls his own roller bag and has stamina and is up for anything and everything. He loves historical walking tours and trying new foods and meeting new people.
He lost a pound on our vacation. He looks good. We were staying at an Austrian resort. Every night was a five course meal which he ate all courses - keto, of course. He also took three days of ski lessons in the Alps (first time on skis) and mastered pizza/French fry. Plus he swam tons and we walked and walked and walked. What a trip!
He is in 4th grade in a Montessori school. In May, there will be a student "literature fair." He is reading The Lion, the Witch, and the Wardrobe (on his own, his choice) and has to write a report and make a project. He finished the book in two weeks in school. He is now in the other room researching the British Blitz and taking notes and printing out photos. He decided that his school project will be a report on the British Blitz and a cardboard wardrobe (from UHaul) covered on the outside with photos from the Blitz and covered on the inside with images from Narnia. Cool, huh? His little brain is working hard. He made the connection between the World War II tours we did in Germany and a creepy Dr. Who episode that he watched (probably a poor parenting choice), and the children in the book.
We just got the word. Two more days of closed school because of the cold. I am glad we bought the wardrobe box. That is a robust two day project! Over those two days, he got a ton of notes from his research and many, many pictures. He was eager to write it and his first draft was so disorganized, that we decided that he actually had three reports: London Blitz, A Child in the Blitz, A Child Sent Away. He rewrote his report into three (more organized) reports. They actually ended up being longer because when they were well-organized, he realized that he had more to say. He has two plus months to revise and pick away at the reports. (I am going to let the teacher take over from here!) All of the rest of his classmates are still reading their book.
One of the things that is so impressive is that it isn’t due until May! A week later, we walked through Narnia-like snow to get the good glue. We assembled the wardrobe box and he started gluing the photos on the outside. It looks pretty cool. He now realizes that the box is large and so he needs more photos. Of course, the more photos he gets, the more he learns.
I am so proud of his curiosity and work ethic and attention to detail. It seemed a long time coming, but right now he is quite impressive.
It is interesting because this is the whole Montessori thing. They feel that the most important thing to cultivate in a child is curiosity and work ethic and self-motivation. I have also been told many times by others that Montessori is inappropriate for a child with PWS because they are not capable of self-motivation and work ethic. In my mind, if a kid is weak in those areas, he needs more work to achieve them. I hate the idea of writing of self-motivation and work ethic. I love that it seems to be blooming in my boy!
Feb 28, 8pm, ketones = 0.4
This was right before bed. He had dinner around 6:30. Dinner was a turkey burger with cheese and buttery mushrooms. He asked for a top off and we gave him a ketocandy after doing the ketotest.
March 1, 8am fasting, ketones = 1.2
March 1, 3pm, ketones = 0.9
Lunch was at 1:00. It was two paleo pumpkin pancakes with a tablespoon of butter and a tablespoon of caramel erythitol syrup.
March 2, 8am fasting, ketones = 1.6
March 2, 2pm, ketones = 1.7
Lunch was at 1:00 and he had decaf coffee with heavy cream, half a burrata, a cup of wilted spinach, and bacon. We walked for about 20 minutes after lunch.
March 2, 3pm, ketones = 3.0
This was 15 minutes after a tablespoon of MCT oil.
Of course, the question is: is this good? I really have no idea. I can say that his brain is working really well right now. He is on a reading frenzy. He is reading at grade level and read three books last week. He is well-organized and advancing nicely in karate. His sleep is good and his mental processing is good. Hunger seems like regular kid hunger. Today at lunch he didn’t finish his bacon and asked us to wrap it to take home.
He is now on three tablespoons of MCT a day. I guess that there is now research out that MCT oil and coconut both suppress ghrelin and therefore suppress appetite. This is consistent with what we are observing. It also suggests that MCT oil may be a great strategy to deal with the hunger of PWS.
He is almost ten. Testosterone, etc are still normal. Fingers crossed he will go through puberty normally.
His IGF was high normal, so Dr. Miller is keeping him on 0.8. We will test again at the end of the summer. Unfortunately, our insurance pulled him off of Genotropin, so he was off of GH for about a week in May. (Generic GH does not seem to work for him.) It was amazing how fast he developed a little spare tire. He is now back on Genotropin and it appears to be going away.
When the blood work was taken, he was on two Gentle Iron's a day. He tested lowish normal for iron. I think he needs more iron. He still looks pale to me. We upped his iron so he now takes three Gentle Iron's a day. His cheeks are not more rosy. (Note for anyone reading, iron can be toxic. If you are playing around with iron, be sure and work with your doctor and get regular blood work to monitor how your kids is doing.)
He is growing well, thinking well, and performing well academically. He had his project/presentation for The Lion, the Witch, and the Wardrobe. It was in a Montessori class of 4th-6th graders. I know this sounds crazy, but his was one of the best. (He is in 4th grade.) He always had a crowd of parents around him. Several parents came up to me after the event and said that his was their favorite.
He had a wardrobe box that he covered on the outside with photos from the Blitz. He covered the inside with photos from Narnia. For me, the funniest was how he "owned" it. At first he just owned his research. As he gained confidence talking with the parents, he "owned" the entire history of the Blitz. He pointed to each photo on the outside and gave detailed histories, making up names, locations, what they were feeling, what happened to them before the picture and what would happen to them afterwards. I thought it was hilarious. The adults kept pushing him by asking him about the different photos and he rose to the occasion, just making stuff up. That said, everything he said was consistent with the history of the Blitz. So, I guess I can't knock him for trying to entertain a crowd and convey some history at the same time, right?
I had one of the best Mother’s Days ever. We went bike riding – as a family – all of us on bikes! We went to the arboretum and did the 9 mile path around the park. There were hills to climb and hills to coast down. My boy did it!!! He was so incredibly proud of himself. It was really amazing. At the end, of course, he was tired and exhilarated. As he was turning into the parking lot, he wobbled and fell. Poor kid wiped out and has a wicked bloody knee. Of course, he uses it as a conversation opener to describe the awesome bike ride and how tough he is.
He has had a lot of leaps this year. Maybe it is just him maturing. Maybe it is the increase in MCT oil. I don’t know. I will say, though, that, unfortunately, he has a spare tire around his belly (a bike tire, but still). I think that this is partially leftover from the week that insurance kicked us off of growth hormone. Still, though, it is bugging me.
Kian is now in 5th grade in a mainstream Montessori school. I am the volunteer science teacher and I was teaching today. The kids designed an experiment to compare the energy levels of the 4th, 5th, and 6th graders. They had all of the kids jumping until they couldn’t jump anymore and then they sat down. All of the kids started jumping at the same time. All of the 6th graders eventually sat down and half of the 4th graders and half of the 5th graders. Kian and his buddy from 5th grade were still jumping. Two girls (one a competitive figure skater) were jumping from 4th grade. Finally, the buddy sat down, telling Kian he had to win it for fifth grade. Kian kept jumping! Finally I ended the competition with my guy and two girls jumping. Can you believe it???!!!
After that, however, Kian just crashed. I thought I had broken him. I gave him a square of dark chocolate and some almonds and water. It took him about half an hour to recover. An hour later he decided to go off to taekwondo!
New world for us. Kian is 11 and on the ketogenic diet. This is sort of in response to questions about calories and sort of just an updating post on how we are muddling along. I can't say we have figured it out yet, but we are trying. Kian doesn't really have the hunger and he doesn't really have food seeking. He is smart and engaged and independent. That said, he is an 11-year-old boy with a tricky body. I tell everyone he has PWS. I am not hiding it, but it really doesn't register for most folks.
Our goal is for Kian to be independent. Possible? I don't know, but that is what we are working towards. So what does the path to independence look like for a child with PWS? Where are my role models? If you are out there, please step forward! (So, I can learn from your mistakes and make different ones with Kian!) I have had the discussion with Kian that, at this point, the single biggest determinant of whether he will be on a path like his sisters or on a different path is his ability to manage food. I also explained to him that he has 5-10 years to step up and do it and this is now his biggest challenge.
This is too long winded. Briefly, we weigh Kian every day and this provides him feedback on his choices. He has decided to enter a theatre camp without telling all of the counselors to watch him for food. He has told a couple of counselors. I think they think he has a food allergy or something.
It has been one week. He is up 1 pound. He confessed he took one thing (bag of crackers). He also took one bite of pizza at the pizza party today and then excused himself from the group and sat in the auditorium until the pizza party was over. I know this because he told me but also because one of the counselors saw him sitting in the auditorium and joined him to keep him company. She couldn't believe his self-discipline. And, she doesn't even know PWS!
Right now it feels heavy, like I am putting too much on this little kid's shoulders. It seems wrong that, in addition to learning his lines and choreography and his songs and doing improv and making new friends, I am requiring him to learn how to independently carry this very difficult burden. And, I am trying to act like it is no big deal. I have to pretend as if it is doable and that every stumble is just a natural part of the journey. And yet... I don't even know if the journey is possible. No wonder I am exhausted!
Things are going very well for Kian right now. He is 12 and in the 7th grade and just finished a 5K race with a time of 33 minutes. This past year has been really big for us and I am quite excited about the future. I plan to share more details soon.
My son is now on pitolisant. He is 12 1/2 years old, weighs 100 pounds and he has gradually worked his way up to one 18 mg tablet and one 5 mg tablet.
My son is now alert throughout the day. He says that he now feels full. His hunger appears to be normal, even if he eats carbohydrates. We think that we may have just finally normalized his gut.
You can follow our pitolisant efforts at: www.ChionFoundation.org.
I believe that my son is the first child with PWS to go on pitolisant. He has now been on it for years. It has been life-altering for him and for our family. He is now 14-years-old and a freshman in high school. He is in all college preparatory classes and mostly has A's. (He has an excellent and really hard history class that he finds challenging, but he is rising to the challenge and hopefully will get his grade up before the end of the semester.) He still loves theatre, although we have discouraged him from doing the school musical this year. We want to give him a little more time to master the transition from a little Montessori school to a very large urban public high school. He is a Boy Scout and goes on camping trips. This weekend he will be spending the night in a bat cave and exploring caves with the Scouts. He can run a 5 kilometer race in under 30 minutes. He walks the mile to and from high school by himself most days. (He does bum a ride from his sister whenever he can.) His BMI is between 20 and 21. He appears to be progressing through puberty normally and making his own testosterone. This past fall he went to his Homecoming Dance with a beautiful and kind and amusing young lady who also has PWS and who is also on pitolisant. The two kids had so much fun that the tried to stay to the end of the party and we had to make them leave because we were tired waiting at home for them. Before the dance, they went out to dinner at a fancy restaurant. They sat at a table by themselves while we sat at a table in a back room completely out of eye- and ear-shot. They ordered on their own, ate on their own, and shared dessert. It was quite a wonderful evening for all of us.
His current dose is two 18 mg tablets and one 4.5 mg tablet.
He has had to rebuild his brain. I really cannot emphasize this enough. I think it will be different for the little kids, but for the kids who are ten and older who start pitolisant, they will have A LOT of catching up to do. My son's brain remains unstable. His gains continue, after all of these years, to amaze those that are working with him. Right now, he is actively working on his social skills, his reading, his auditory processing, and executive functioning. As his brain speeds up (and it continues to speed up), he has to learn to breathe and remain emotionally stable as he processes a world filled with increasing information and increasing demands. Watching him do all of this has changed me as a human being and changed my understanding of the human condition, the human brain, and the human spirit. It has been and continues to be quite a journey. I likely will no longer post here anymore, although the page remains a wiki (like wikipedia) for any who would like to continue to shape the story.
I suspect as my son's voice grows and becomes more clear, that he will become an advocate for the PWS community, but that will be his choice to make and, to a large extent, the rest of his story will be for him to share.