PWS Therapies

Ribose

Wed, 31 Mar 2010 20:18:36 -0700

Well after learning that another mom was trying ribose with her daughter and seeing amazing results, I decided to jump on the bandwagon. I did a little research first, of course, by checking in HolisticPWS archives. This is what I found.

D-ribose seems to work alongside carnitine, creatine, B12 and CoQ10. In fact, when I was checking the ingredients on my NOW CoQ10, I noticed that along with the 100mg CoQ10, there was 100mcg cyanocobalamin b12 and 10mg d-ribose!

Thank you to Oneida (PWS Dots) who reported the following (I have summarized her work so please refer to her post in the archive Jan ‘09 for exact verbage and detail).
Ribose helps replenish ATP in all cells (e.g. neurons, heart muscle, ect)
A combination of carnitine, creatine monoydrate, coQ10 and ribose can help with post-exercise recovery
B12 supplementation MAY increase the bodies need for ribose

Supplement manufacturers such as NOW foods, Carlson Labs and Jarrows usually list the adult dose as 5g/day. Doses in clinical trials for congestive heart failure, chronic fatigue syndrome, fibromyalgia and post-exercise recovery have typically ranged from 10-20 g/day. Oneida suggests beginning supplementation at about 1/4 to 1/2 of the adult dose. Increased dosage may be warranted based on clinical response

Given that those with PWS seem to be particularly prone to hypoglycemia and that ribose can lower blood sugar, ribose should always be taken with food. It is important to watch for signs of headache, stomach ache or getting excessively hungry as these are signals that blood sugar is low. If any of these signs are present, back off the dose a bit.

Observations:

3/31/10. I did not observe any changes the first two days of beginning ribose supplementation. If anything, Jayden has been taking longer naps, but that can easily be attributed to a number of factors. Today, day 3, Jayden became “superman” practically “running” across the room while crawling and babbling more than I have ever heard. While I cannot necessarily attribute this to the ribose, it is an interesting coincidence. I will continue to take note of any notable changes.

I decided to run a small experiment....After 4 days of being on ribose, I removed ribose from Jayden’s diet to see if there would be any changes. After discontinuing ribose for 3.5 days, I included the ribose with his supper on day four. The next morning, after taking him out of his crib, he "ran" as soon as I put him on the floor. I have continued giving Jayden ribose twice a day (morning and night) with the rest of his supplements.

I want to believe I saw the following when ribose was included in his supplements:

He is faster. He will often "run" while crawling vs. shuffling along
He babbles more frequently and loudly
He engages in activity more quickly. For example, we went to the park and immediately upon being set down, he began crawling off. (Usually, especially when away from home, he has to think for awhile before moving.)

In the video below Jayden crawling at our home 3 days after ribose was removed from his diet. The second half of the video is of day 3 with ribose. I don’t think you can see much of a difference. Even though he is slipping a bit with his pants on the tile, he may be a bit more stable crawling in the video with ribose.

I also have some interesting footage of Jayden at therapy. When he was not on ribose, he seemed unsure of what to do. When on ribose, he would take off without as much “thinking” time. I don’t have the on ribose comparison footage but I will try to post it once I have taken it.

Unfortunately, there were too many environmental variables present during my mini-study. My parents were in town, he missed a nap one day, I shared much of my care giving responsibility relying on my parents for their take on his behavior when I was not present.

I think to truly trust my observations, I will have to try the experiment again. Maybe even going for a week without the ribose. For now, however, he is getting 1/4th tsp/day.

Sensory Integration

Thu, 18 Feb 2010 13:17:22 -0800

Sensory integration is at the forefront of my mind these days. I am just beginning to research sensory integration and understand what it means to those who struggle with it. From my reading, it seems many children with PWS struggle with sensory integration.

COQ10

Thu, 18 Feb 2010 08:29:24 -0800

CoQ10 is reported to increase energy levels in children with PWS. Dr. Judy (Florida), recommends CoQ10 for individuals with PWS and has several examples of successful individuals with PWS who have taken CoQ10.

SPEECH THERAPY

Wed, 30 Dec 2009 08:18:32 -0800

Background. I have been concerned about speech development since Jayden was 5 months old at which time I had his first speech consultation. At 10 months old, I have begun doing more research into his speech needs. I understand little of the complexities of speech development so I have heavily relied on other parents of children with PWS, speech experts and PWS professionals. This entry is to record my findings and make available the resources I found helpful. Thank you to the many parents on the PWS 0-5 message board that helped provide the information posted here.

When to begin therapy? PW California Foundation “encourages beginning oral-motor therapy in infancy to assist with feeding and the acquisition of the oral-motor skills necessary for babbling and speech.” The general consensus from the PWS message boards is to begin therapy as soon as possible. Many families have started within the first month, although this is more to address feeding issues than actual speech. The information presented on the message boards has conflicted with the opinions of my pediatrician and speech consultant.

Dr. Miller (Pediatric Endocrinologist, University of Florida) likes to begin speech therapy as soon as the kids begin to verbalize sounds. At Jayden’s age (10 months) her speech therapist recommends using a mirror to show lip movements that are associated with different sounds. When the children get a little older (exact age I am still unsure of), they should be assessed for signs of apraxia. If any evidence of apraxia is present, a specific technique called tactile cueing is generally applied.

Speech Delay and PWS. Children with PWS do not have a “simple” speech delay. Low muscle tone impacts the ability of the child to move their tongue, cheeks, and mouth.

Dysarthia. Difficulty moving the muscles to produce the desired sounds. Dysarthia is a specific diagnosis helped with oral motor therapy (therapy that is designed to active the nerves and muscles associated with speech production)

Apraxia. Many people with PWS have global praxis problems that impact their speech and language skills. Apraxia must be specifically diagnosed and must be treated with specific therapeutic interventions. For more information on apraxia, visit www.apraxia-kids.org.

Oral Motor Therapy. In trying to remain neutral, I will mention many research articles have concluded sufficient evidence does NOT exist to support the effectiveness of Non-speech Oral Motor exercises in improving speech. This is also the position of my speech consultant. However, many people have sworn of the benefits so here are exercises that have been mentioned to me by parents whose children have had ST. Some of these exercises are utilized by our OT.

Stretch, pull and circular massage muscles inside and outside the cheek area and around the mouth.
Point tongue up, down, or all around. Manipulate the tongue forward, back and side to side.
Use mesh feeder bags for difficult to chew items and to improve jaw strength and stability.
Simulate chewing with chewy tube and NUK brush (see catalog link to purchase)
Stick cheerios to lips and have child retrieve with tongue only.
Curl and point tongue.
Blow on candles, bubbles, spirometer to improve depth and strength of breath.
Wake up the mouth with cold liquids and spicy or sour foods.
Use repetition like repetitive songs, rereading books ect to improve motor planning.
Picture boards

Jayden’s Speech Development

12 Months 03/10/10
Jayden did not begin babbling until the week of his first birthday. His primary sound combinations at this time are: la, na, ya. Today was our first official speech evaluation. 99% of the evaluation was me answering questions. Of course I wanted to say Jayden understands words and makes consistent sounds but the truth of the matter is Jayden does not say much and does not show much understanding of words either. He scored at 6 months for receptive AND expressive speech. More specifically, he was in the 1st percentile (severely delayed) for receptive language and the 3rd percentile (moderately delayed) for expressive. The evaluator recommended weekly parent coaching for the next two months.

B12 INJECTIONS

Tue, 15 Dec 2009 13:19:42 -0800

At 9 months old, I was giving Jayden 3000 mcg oral methylcobalamin (pure encapsulations). Based on my readings of PWSdots, I don’t believe the oral B12 was being fully absorbed so I decided to try injections. Since beginning injections, I have stopped giving oral B12.

None of our doctors were willing to prescribe B12 (I asked our pediatrician, geneticist and endocrinologist), so I found an osteopath who was. We see Dr. Watson, in Del Mar CA. I asked that we follow Dr. Neubrander’s protocol for B12 injections which, in a nutshell, states:

For most children, give 64.5mcg/kg every 3 days.
Use 3/10 cc insulin syringes with an 8mm, 31-guage needle
Use Methyl B12 concentration 25 mg/ml
Inject into the adipose tissue of the buttocks
** Full reference to Dr. Neubrander’s protocol

When first beginning injections with our osteopath, he ordered what he felt was best: hydroxycobalamin (see below for exact formulation). In addition to administering the shot, he also performed cranial-sacral therapy, We were very happy with the initial results: Jayden showed huge gains physically, vocally and mentally. After the initial injections, we obtained a prescription and ordered a three month supply of pre-filled methylcobalamin (as Dr. Neubrander recommends) from Hopewell Pharmacy. We will continue to see Dr. Watson monthly for cranial sacral therapy as well as for an injection of hydroxycobalamin.

Several compounding pharmacies are available to fill your B12 prescription. Dr. Neubrander recommends Hopewell Pharmacy as their quality is consistent and he has seen the best results with their compound. With a doctors prescription, you can buy a 3 month supply. Some insurance will pay for the injections, ours will not so we are paying $90 + shipping for 30 pre-filled injections.

Hopewell Pharmacy & Compounding Center
http://www.hopewellrx.com/wellness/links.shtml
1 West Broad Street
Hopewell, NJ 08525
(800) 792-6670

Hydroxycobalamin B12 Injections Formulation per 1mL
Dexapanenol 25mg
L Methionine 5mg
Pyridoxine 25mg
Inositol 50 mg
Cyanocobalimin 10 mcg
Hydroxocobalmin 500mcg

DIET

Sat, 26 Sep 2009 15:36:59 -0700

We have decided to follow a low carb diet for Jayden. Even though he is still an infant I am thinking ahead and trying to start off on the right foot. While traditionally, rice cereal and other carbohydrates make up babies first meals, Jayden is beginning with vegetables and proteins. I am even limiting fruits. It is challenging to come up with new foods to try as it seems like so many of the foods are starchy or high carb. For example, I recently tried banana later to realize that bananas are really just sugar. Oops.

6 months
Feeding solid foods has not been easy. In fact, every day and every feed is still a struggle. We wear more than we eat. In three months we have tried: avocado (liked), green beans, egg yolk, turkey (hated), chicken, hamburger, and banana (loved but no longer on the menu). It seems like by now Jayden would be a pro at this eating thing but he has limited interest in the foods I have presented. I wonder if it would be any easier if I was introducing the old fav’s of cereals and potatoes??

I will update this page as new foods are introduced and diet “rules” are modified.

8 months
At the completion of 8 months, Jayden is getting much better at eating solids. He likes yogurt and cottage cheese. I feed him whole milk cow and goat milk yogurts and add a little bit of flax and or almond butter. I try to offer vegetables every day, like broccoli but he seems to prefer the starchier vegetables like pumpkin, peas, and butternut squash. Yesterday I made homemade applesauce for him (he loves apple/pumpkin baby food) but I only plan to give it to him in small quantities since it is a sugary fruit. I thought it might make a good mixer for chicken.... He still loves avocado so I use it to mix in chicken and other meat.

Finding suitable finger foods has been a challenge since cheerios are not on the menu. So far, I have tried cheese balls (that is shredded cheddar cheese gently pressed into a ball the size of a cheerio), small pieces of egg yolk (hard boiled or scrambled) and almond butter balls (almond butter rolled in nut crumbs). These have all seemed to work pretty well so far. In another month or so I will give him egg strips to practice biting off pieces.

So far, not feeding grains has not been a problem!!

10 Months
We are trying to limit Jayden’s consumption of milk to 24 ounces (down from 30). His nutritionist believes he is gaining weight too fast. She is not too concerned with the calories from his solid foods but suggests I use caution with the high calorie foods I offer: egg yolk, flax, tahini, nut butters, ground nuts....

He is getting better with finger foods: his favorite is apple. He can get the food off the tray but it is still challenging to get it successfully into his mouth.

Jayden loves yogurt so I combine it with veggies, nuts, meat and egg that he might not eat otherwise. I only give him plain, unsweetened yogurt. He is eating really well - 3 meals a day. Some of the combinations I give him sound disgusting but he eats readily - how about some yogurt with shredded zucchini and a dab of tahini? or perhaps you would prefer yogurt with scrambled egg and peas?

12 Months
Jayden now takes all his milk from a straw cup. He drinks between 16 and 24 oz a day. He can easily pick up foods with a raking of his palm but we are still working on his pincher grasp. His weight gain has slowed and he looks to be a good weight. We have three meals a day plus two snacks. A typical day looks like this:

8 oz milk at wakening
Breakfast: up to one egg, scrambled. 1/4 cup yogurt with flax. Maybe some fruit or avocado.
Snack: diced veggies or fruit, 3-5 ounces of milk
Lunch: tuna salad or bits of meat and cheese, diced veggies or fruit
Snack: fruit and maybe some almond butter or cottage cheese, 3-5 oz milk
Dinner: 1/4c cup yogurt with veggie puree or finely diced meats
8 oz milk before bedtime

I would like to reduce the amount of fruit he eats but he LOVES fruit and without it there wouldn’t be much variety. I feel like he eats a lot of the same foods over and over and I would like to start offering meals that more closely resemble what I eat.

CREATINE

Mon, 3 Aug 2009 09:55:36 -0700

Creatine improves energy and strength. Muscle mass, strength and endurance should increase over the course of a couple of months. Increased muscle mass and muscle hydration may result in added weight gain.

PWS DOTS suggests a dose between 300-500 mg/kg/day and recommends to begin with 1/4 of this. For further information on creatine and other supplements, visit PWS dots.

ADDITIONAL CALORIES FOR INFANTS

Sun, 12 Jul 2009 09:07:25 -0700

To ease the pressure of getting Jayden to drink enough, we added extra calories to his bottle. At 7 months old, I noticed Jayden was looking chunky and I stopped adding the extra calories. I probably should have stopped a month or so sooner.

ORAL B12

Thu, 11 Jun 2009 19:47:55 -0700

High doses of B12 have been observed to have many benefits including increased sucking ability and improved speech.

CARNITINE

Tue, 9 Jun 2009 20:07:51 -0700

Research has indicated carnitine has a positive effect on children with PWS and may improve alertness, energy level, and ability to suck/swallow.

PWS DOTS suggests dose ranges from 35-100 mg/kg/day split among 2 or 3 doses. For further dosage recommendations, visit PWS dots.