Energy Levels & Growth Hormone

It's not uncommon to notice changes in the energy level of your child with Prader-Willi Syndrome. There are multiple reasons for this, and each child is different, hence this syndrome being spectrum. In a previous entry I talked about supplements. It's important to remember that manufacturers (of vitamins & supplements) do not need to register their product with the FDA, nor get the FDA's approval, before producing & selling to the public. When choosing a supplement for energy maintenance, consider the source. If your doctor can give you some recommendations, as did ours, please take their advice. Doing your own research into your child's supplements can make a huge difference.

By now you are probably aware of the different phases of Prader-Willi Syndrome. Some of these phases present at different times, child to child, due to their course of treatment. A big factor is the use of Human Growth Hormone. GH has now been used for about 13 years, getting approval from the FDA in 2000,to treat patients with Prader-Willi Syndrome. Some children may show a deficiency of GH, but even with no such deficiency, GH is prescribed to help with over-all body composition & growth. It has been documented that the use of GH in individuals with PWS improves lean body mass, decreases body fat, increases bone mineral density & normalizes height. Growth hormone also influences the body's conversion of food into energy.

A physical characteristic of a child or adult with PWS is short stature, obesity & some level of mental disability. There is also a reduction in muscle mass that is associated with diminished strength, physical function & energy expenditure. This is now somewhat avoidable with the use of growth hormone injections. Studies suggest it has positive effects on behavior and development. It has been noted that children who start GH therapy before the insatiable appetite begins (between ages 2 & 3), that hunger is delayed until age 7, 8 or 9.

Supplements, diet & growth hormone all play a major roll in the over-all energy of an individual with PWS. We keep a fairly strict diet with Claire, keeping to this daily ratio: 25% protein, 35% fat & no more than 40% carbohydrates. It's not easy - it's actually REALLY difficult. Before our first appointment with Dr. Miller, I got the idea to make a menu board for our family. I spent hours upon hours finding, creating, re-doing favorite recipes that met the calorie restrictions I was under the assumption we needed to stick to. I organized it by color, laminated it all, included nutritional content per serving. Here's a picture...

I was so excited to show off my pretty menu board to Dr. Miller. However, she said to me "Oh, we don't count calories, we just balance." My bubble was burst.
Nonetheless, when I returned home I went through the recipes, tossed some out, and started searching for more or tweaking the ones we love. I also started doing a bunch of research on 'healthy' foods.

Since adopting the 'Balancing Diet', we all have noticed that it's not so bad! There is a noticable increase energy, and I have lost 15 lbs in 6 months. Food is essential to the proper functioning of your body, but those with PWS need a little more help. Each time Claire's Growth Hormone is increased, we see an increase in her energy, sometimes more than others. A few weeks ago I asked the doctor if we could add B12 to Claire's regimine- she said yes, told us which kind to get & the proper dosage. There was no difference in her energy, which was quite poor at the time. After a blood test, we learned that Claire actually had a pretty severe case of anemia. We added gentle iron (non-constipating) & after about 5 days, she is back to her silly, funny, energetic self! We took away the B12 & will add it in the future if we feel it's necessary.

Because of the plethora of information on the internet, it's easy to fall prey to fad diets, pills, supplements, juices, etc. Our doctor just put out a very informative PSA that I think everyone should read - and remember.  PSA: Warning

There is no cure for Prader-Willi Syndrome. It is mostly about treating the symptoms of the disorder as they arise, and each person is very different. We consider ourselves extremely lucky thus far that Claire has been so healthy. She is in 4 therapy sessions a week, is in horseback riding therapy (hippotherapy), plays with children her age, has the regular childhood illnesses, and is the JOY of our lives. We trust our doctor, and that is a big deal. You know your child, and you may know best in some situations, but I urge you to consult with the professionals when making decisions on the course of care for your child. What works for one child, may actually hurt another. Diets included.

Oxytocin

Most of you moms recognize the word Pitocin. Pitocin is a drug used to induce labor, or create stronger contractions during labor & is also sometimes used to control bleeding after birth. Pitocin is the chemical brand name of the naturally occuring, and quite powerful, hormone Oxytocin (not to be confused with the pain killer Oxycontin, or Oxy). It is often referred to as the "trust hormone" & is released when we hug or kiss a loved one and plays a huge role in bonding.  

There have been several studies conducted in France on the use of Oxytocin in adults on the autism spectum. They found that the adults socialized more often after taking Oxytocin, they understand the behaviors of others & can respond appropriately. They have also found that this hormone, even in a synthetic form, enhanced autitic adults' abilty to understand emotions in speech & can lessen the common symptom of repetitive behaviors. Some behaviors of children & adults with Prader-Willi Syndrome mimic those of autistic tendencies, and therefore, some of these studies have been very useful in the treatment of Prader-Willi. Autism is more common in the most rare form of PWS (imprinting defect), but a lot of the symptomatic issues are common in all three types. 

Speech delays are very common, this is why early interevention with ALL types of therapy are necessary, even before there are any true signs there may be a specific issue. This may sound silly, but it is very likely there will be some sort of delay, and it's better to approach the care of your child's PWS as preventative, rather than the "catch-up" type, as I like to refer to it. This will cause you to really stand up to coordinators, or case managers, for your child's first 3 years of therapy if you choose to do it through the state. Most states do not work in a manner of preventative therapy - it's always the "catch-up" aprroach, and personally I think it's negligent. 

We spent countless hours fighting for the proper care & the correct amount of therapy sessions for our daughter. There were also set-backs when we needed to ask for a new therapist because we knew the sessions were virtually useless. When I couldn't make any more calls or argue with another case manager, because my emotions were getting the best of me, AJ would take over. Luckily, he is calmer than me, and has a way with words. I found that they tend to listen to the man of the house with a little more respect... so if you have that in your back pocket, don't be afraid to use it. 

The reason I'm highlighting speech delays in this post is because the use of Oxytocin in kids with Prader-Willi Syndrome is showing improvement in speech & socialization. It's also believed to help with anxiety, irritability, social communication, behaviors & possibly even some food issues.  Individuals with PWS have been found to have a deficit of oxytocin-producing neurons and decreased oxytocin receptor gene function. Oxytocin is produced by the hypothalamus, where most of the issues with PWS begin, so it makes sense to try to bring it back to it's normal level.

Our speech therapist, who we really respect & trust, has told us that Claire has social anxiety & this is why, in part, she is practically non-verbal 3 months out from her 3rd birthday. We know she will talk, and she CAN talk, she just needs to form some new pathways in her brain that allow her to feel comfortable in social settings, enough to speak. It's definitely baby steps, but I see improvements weekly. 

Oxytocin helps with creating that trust within herself to want to have conversations with others. She used to remove herself from play groups and go hide in the closet, or just go into a different room, away from other little kids her age. Now, she's trying to grab everyone's hand and dance with them, or lead them to a toy. She may not be saying anything, but her non-verbal communication has improved significantly. Do not be discouraged if progress seems to be slow - there is no timeline for which this all needs to happen. Afterall, Einstein didn't speak until he was 4.  

Supplements

Supplements are a good way to fill in what your body isn't receiving directly from food. Prader-Willi Syndrome is a spectrum disorder, and what works for one person may not always work for the next. Each person (diagnoses aside) is unique in their reactions, tolerations & processing, so it is possible that the following supplements may not work for your child. Not the end of the world. I'm sure there are other supplements that are being used by those with Prader-Willi, but these are ones with which I have had some experience. It is likely this blog entry will be updated in the future with supplements added &/or taken away from Claire's daily routine. It's important to remember that manufacturers (of vitamins & supplements) do not need to register their product with the FDA, nor get the FDA's approval before producing & selling to the public. If your doctor can recommend a certain brand, as did ours, I highly recommend going with the physicians advice. I read somewhere once that CoQ10 can sometimes be derived from tobacco plants... gross! Know what you are putting into your, and your child's, body. 

L-Carnitine
The first supplement a lot of children with PWS are put on is called L-Carnitine. This is a naturally occurring substance in the body, and sometimes kids with PWS have a deficiency. Carnitine helps the body use certain chemicals (long-chain fatty acids) for energy & to keep you in good health. The most common symptoms in people who are unable to use Carnitine from the food they ingest (mostly in proteins such as meat and dairy) are low energy, weakness & low glucose.

L-Carnitine is used for Prader-Willi patients (sometimes when they don't have a deficiency to begin with) to boost their energy level. It does take a little bit of time to show a difference in energy & each child is different. Also, keep in mind that this doesn't work for everyone.
Your pediatrician should be the one who prescribes this (Rx is called Carnitor/Levocarnitine) - and note that it should never be purchased over-the-counter or online as those are not in a pure form. 
 In Claire's case, we took her off Carnitine after only being on it for about a month. At first we thought we saw a difference in her alertness, but after a few more weeks, it didn't seem to be working. It was disappointing, but we were on to the next!

CoQ10
CoQ10 is produced by the human body and is essential for the basic functioning of cells, especially those found in muscle. It generates energy in each cell, specifically the mitochondria, in the form of ATP (Adenosine Triphosphate), which in return transports the chemical energy in each cell for metabolism. CoQ10 also acts as an antioxidant, which protects the body from harmful molecules, especially the heart and skeletal muscles. 

A blood test can determine if a person with PWS has low CoQ10 in their system. Whether or not there is a deficiency, it is sometimes prescribed in hopes it will have the same effect as L-Carnitine. There is also a question of whether or not those with PWS are inadequately utilizing the body's supply of CoQ10. Supplementing will also aid in muscle function & metabolism. 

Our doctor recommends ordering our CoQ10 from a company called CytoMed because it is in it's purest form. If you do not want to order it, here are a few tips for choosing a CoQ10 supplement: 1)  If you see a CoQ10 supplement with “ubiquinone,” walk away. Your body has to work extra hard to recognize and convert this form into one it can use.  2) Ubiquinol is identical to 95% of the CoQ10 your body is designed to naturally produce. Make sure this is in your CoQ10 supplement.
You can find CytoMed here: CytoMed
A dose of 50mg a day (1 capsule)is what we started Claire on after removing the carnitine from her regimen. We squeeze the capsule into her Greek yogurt each morning. She is 2 years old (by just over a week & weighs 22lbs. This has made a HUGE difference in her overall energy level & alertness. She quickly went from two down to one nap per day (age appropriate) & she can handle all her therapy sessions from start to finish, no problem. 

Omega 3
Omega 3 fish oil is something we should all incorporate into our daily diet, whether or not we take it in vitamin form or directly from diet. Omega 3 consist of three fatty acids: ALA (found in plant oils) EPA & DHA (both found in fish oils). DHA is crucial for the proper development of brain, eyes & heart in children, and has been helpful with attention disorders. EPA is proven to lower inflammation in the body & have positive affects in mental health. There is more information on Omega 3 in this blog Part V. 

We were turned onto Coromega for our Omega 3 by fellow Prader-Willi moms. It is a very good form of the vitamin & u can order directly from them, or on Amazon.com
She gets one of the orange flavored squeeze packets each morning & LOVES IT!! (We found that the regular orange squeeze packets are the same as the kids shown below, so we obviously look at the cost difference before buying.)
To visit the website for Coromega, go here: Coromega
 We're led to believe Claire suffers from asthma, although she hasn't been technically diagnosed. She is on a breathing treatment twice a day as maintenance with back-up meds to add if needed. It is believed that a diet high in Omega 3 can help respiratory health by reducing inflammation, which increases the effects of asthma.

Vitamin D
This vitamin is essential for maintaining proper calcium levels in the body & without it, the body is unable to absorb & use calcium efficiently. The bones, nervous system & immune system depend on calcium for proper functioning. There are two types of Vitamin D  > D2 (synthetic) & D3 (natural). Vitamin D can be found in small amounts in a few foods, including fatty fish such as herring, mackerel, sardines and tuna. You may see products that say "fortified with Vitamin D" like dairy products, juice & cereal, but these are also smaller amounts. 80% to 90% of the Vitamin D our body receives is through exposure to sunlight.

If using a D supplement, make sure that it is D3, since this is the kind that is naturally produced by the human body. The synthetic D2 has been known to be derived from plant matter & made by irradiating fungus (exposing it to radiation... I mean, seriously???) If you want an easy way to get your Omega 3 & Vitamin D3 in one little easy dose, visit the webpage for Coromega: Coromega Omega 3 & Vitamin D3

Sunlight is the best & easiest way to get your daily dose of Vitamin D, but there is also the fear of UV rays & skin cancer. Unfortunately, the use of even a level 8 SPF sunblock decreases the absorption of Vitamin D by 95%. Using anything higher virtually prevents the body from using those rays to get your crucial dose of D. It is recommended that everyone be exposed to direct sunlight (without sunblock) for about 10-15 minutes per day, as that is all the body needs. 
A few months ago, Claire's quarterly blood tests came back good & our doctor mentioned how her Vitamin D level was the best she had ever seen in a kid her age. Still not sure what I was thinking, but the very next time we ordered her Coromega Omega 3 Orange Squeeze packets, I ordered the Omega 3 + D. The next blood test came back showing Claire had an elevated level of calcium. There are a few reasons for this, one being too much Vitamin D can raise your safe level of calcium. Have your doc checks Vitamin D levels & adjust accordingly, but there is such a thing as TOO MUCH SUPPLEMENTING!!! 

The Basics: Part V

Hello! In this entry I'm going to educate you on FAT. The good, the bad & the ugly. Good news is that fat is an essential part of our daily diet & should most definitely be consumed in moderation. For our Prader-Willi kids we need to make sure the fats we include in the balanced percentage of their daily intake consists of only qualifying fats. Fat carries vitamins throughout the body & also supplies Essential Fatty Acids. Fat is essential in eye & brain development and for cardiovascular health. It's also necessary for balancing hormones.

Here we go... Fat brings vitamins A, D, E & K to all parts of the body. It is advised not to take mega-doses of these vitamins as they can be toxic to your body in large quantities. This is because the body naturally stores these vitamins in fat tissue & your liver, hence being called fat-soluble vitamins. Water-soluble vitamins include vitamins B & C, which need to be replenished on a regular basis. 

Vitamin A plays a major role in eyesight, bone growth, tooth development, reproduction, cell division & regulation of the immune system.
Vitamin D is responsible for the body's use of calcium by increasing the amount absorbed from the small intestine, helping to form and maintain bones.Vitamin D also plays an essential role in the body's immune system.
Vitamin E** acts as an antioxidant. It also shields vitamins A and C, red blood cells, and essential fatty acids from being destroyed.
Vitamin K is essential for proper blood clotting & bone health. This vitamin is also naturally produced in the intestines & produces proteins for blood, bones, and kidneys. 

Fat also supplies Essential Fatty Acids (EFAs). These are the most important of fats, and play the most important roles.We must ingest EFAs since the body does not produce them on it's own. You know EFAs by their more popular names: Omega 3 & Omega 6.

Seafood contains Omega 3, the type the body prefers, and are linked to lowering the body's level of triglycerides (fat). When the blood is rich with triglycerides there is an increased risk of clots that block the flow of blood to the heart & brain, and an irregular heartbeat.

Seafood's Omega 3, an unsaturated fat, is called DHA (same kind found in mother's breast milk) & EPA. DHA is crucial for the proper development of brain, eyes & heart in children, and has been helpful with attention disorders. EPA is proven to lower inflammation in the body & have positive affects in mental health.

The best source for Omega 3 fats is fatty, cold water fish such as salmon, sardines & tuna. Not only those with Prader-Willi benefit from regular intake of Omega 3. Claire eats her Omega 3 mostly in fish & also takes a supplement each day, which is recommended by her doctor. I add ground flax to things like meatballs & quinoa bites, too!  
For the supplement we use Coromega in the orange flavored packets with vitamin D added. To check this out click here: Omega 3 + D  
Flax has long been considered a good source of Omega 3, but they are from the essential fat ALA (alpha-linolenic acid) and experts say that less than 10% are actually converted to Omega 3.
Update 9/2015 - We have changed Claire's Omega 3 to Nordic Naturals. Because she has been formally diagnosed with Apraxia of speech, we follow a formula that was created after lots of research specifically with children with significant speech delays. This blog follows this formula as well, and has it nicely written here: https://apraxiastory.wordpress.com/top-3-must-dos/fish-oils/brand-and-dosage-information/

Saturated fat is one of the fats we should restrict the most from our diet, the other is Trans fats. There are no dietary guidelines for saturated fat because the body already produces all it needs, but there is no need to totally avoid saturated fat to maintain good health, just limit it. Cheese, milk & meat are some popular forms of saturated fat & also provide nutrients like protein, vitamins & minerals. 
Cheese is a big part of Claire's diet. Right now we rotate cottage cheese, 2% reduced-fat cheese & the part-skim mozzarella, usually in the form of string cheese. We did take her off drinking cow's milk due to the carb content & our own feelings that it isn't a necessary part of her diet, but she eats Greek yogurt all the time. Also, we limit the amount of red meat she consumes and mostly stick to chicken & turkey. We don't seem to have an issue with protein in her diet, it's definitely a challenge with qualifying carbs & fats.
There are highly saturated vegetable fats such as coconut oil, palm & palm kernel oil & cocoa butter and these are considered unhealthy. They are used in packaged goods such as chocolate, cookies, crackers & chips. I know coconut oil is trendy right now, but before you use it on a regular basis please read this:  Coconut Oil Update
I understand that it is REALLY difficult to not purchase pre-packaged foods from the grocery store. After these "basics" entries are done, I promise to give you some easy ways to avoid buying some of the popular packaged items & replace them with homemade items. 

 

Eating less saturated fat doesn't necessarily help your weight, either. In a study in The New England Journal of Medicine, they found that a low-carb diet resulted in faster weight loss & better cholesterol levels than those on a low-fat diet, even though the low-carb group was eating more saturated fat. This may be because eating less carbs causes less insulin to be released, which may reduce fat storage, control hunger, and influence metabolism. (I can't help but think of this being a huge reason that the diet of someone with PWS is a carbohydrate-restricted diet). Also, when you replace saturated fats (bacon) with refined carbs (bagel), your triglycerides can go up and your good cholesterol can go down.

Unsaturated fats - monounsaturated & polyunsaturated - should be the primary dietary fats in a diet. Polyunsaturated fats include Omega 3 & can help reduce blood cholesterol. Monounsaturated fats also help reduce blood cholesterol levels, and are typically high in vitamin E. They also provide nutrients to help develop and maintain your body’s cells.

Sources of polyunsaturated fats include: 

• corn & safflower oils 
• sunflower seeds & sunflower oil 
• flaxseed & flaxseed oil 
• soybeans & soybean oil 
• Fresh fish (Salmon, Herring & Trout)

Sources of monounsaturated fats include: 

• olive, canola & sesame oils 
• avocado 
• almonds, cashews, pistachios 
• peanuts & peanut butter

And here comes that nasty 5-lettered word... TRANS

Trace amounts of trans fats are found naturally in some beef & dairy. Most of them are MAN-MADE fats made when hydrogen GAS reacts with oil. Gross. This is where the name hydrogenated comes from. It converts the oil into a firmer, tastier product & increases it's shelf life.

Trans fats are bad, bad, bad for you. Ditch them! Here's what they will do for you:

• Clog your arteries that feed your heart & brain
• Cause cancer (including breast & colorectal)
• Cause heart disease (reports say this doubles in women)
• Cause heart attack or stroke

Trans fats are found in A LOT of processed foods. Because of it's serious health threat, trans fats were required by the FDA to be listed on (US) food labels starting in 2006. Some food manufacturers have greatly reduced or limited the amount of trans fats used in packaged foods when this rule was enforced. More bad news - any amount at, or under, .5 grams in a serving size is not required to be accounted for on the food label. So if you eat 3 servings of something that has .5 grams of trans fat, 4 times a day, you're really consuming 6 grams of trans fat. YUCK!

Here's a tip to purchase foods without, or with very little, amounts of trans fat. If the nutrition label states 0 Trans Fat, don't stop there. Look at the list of ingredients. Again - you should be reading ALL of your food labels. Look at serving sizes first of all - you'd be surprised at how little some packages consider a serving size. This is what you should do if on that Nutrition Facts label you see the words "hydrogenated" or "partially hydrogenated":  Take a mental note of that food item, put the package back on the shelf quickly, back away from the shelf, grab your kids, your cart, your purse, and all things holy to you & don't ever pick up that package ever again (thanks to the mental note you took first).

Biggest categories containing trans fats:

• Fried foods: chicken, French fries, biscuits, fried fish
• Donuts & muffins
• Crackers
• Cookies
• Cake mix, icing & pie
• Microwave popcorn
• Canned biscuits
• International/instant lattes & coffees

Let's play a game. What is WRONG with this food label of these cookies? 

I guess this might only be a difficult game if you were color blind...

WAIT! There is good news in all this! A study released in February of 2012 from the Centers of Disease Control & Prevention finds that Americans' blood-levels of trans fats have dropped 58% over the past decade - possibly in part of the new labeling law. 

Qualifying fat in a diet of someone with Prader-Willi can prove to be very challenging. Everyone should live by the guidelines on this information on fat. NO TRANS FAT (including no hydrogenated oils), very limited SATURATED fat intake, and regular intake of Omega 3-rich foods. Fat is essential, but we have to be smart about what kinds we consume.

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**Research from decades ago suggested that taking antioxidant supplements, vitamin E in particular, might help prevent heart disease and cancer. However, newer findings indicate that people who take antioxidant and vitamin E supplements are not better protected against heart disease and cancer than non-supplement users. Many studies show a link between regularly eating an antioxidant rich diet full of fruits and vegetables, and a lower risk for heart disease, cancer, and several other diseases. Essentially, recent research indicates that to receive the full benefits of antioxidants and phytonutrients in the diet, one should consume these compounds in the form of fruits and vegetables, not as supplements. (www.ext.colostate.edu/pubs/foodnut/09315.html)

The Basics: Part IV

In this entry on the Prader-Willi Foodie blog, I will be covering proteins, and why they are essential to one's health. To re-cap, we are on a balancing system when it comes to Claire's meals. The total amount each day that she should consume is made up of 40% carbohydrates, 35% fat & 25% protein. I admit that each day is not perfect for multiple reasons. 

As a lot of you know, at this stage in a toddler's life, a lot of food tends to be ejected off their plate. Claire will pick up a fist full of food, look me dead in the eye while she hangs her little hand over the floor, and as soon as I say "no Claire, that's not nice, we don't..." food is thrown onto the floor. And then she does this exaggerated lean over the side of her high chair like, how did that get there? Stinker. I swear if she wasn't so cute...

This picture is hilarious! Those ARE NOT HER TEETH! It was one of those round puffs & it ended up in her mouth like that so perfectly. She had no teeth at this time - as you can see she was still being fed via NG tube.  






We do the best we can do, but no, it's not perfect each day. I try to get each meal & snack balanced so there is no spike in her blood sugar that will cause hunger sooner than a few hours from that particular meal. Getting Claire in the right habits at a young age is going be essential in her ability to make the proper food choices for herself in the future. I don't expect her at age 10 to be able to whip up a cauliflower crust pizza with 3 oz of part-skim mozzarella cheese & 2 tablespoons of sauce. It is my job as her mother to make sure the ratios are what they are. When she goes to get ice cream with her friends is when I need to be comfortable in knowing that she'll get one scoop of frozen yogurt. Plain. And wash it down with a bunch of water. :)

I cannot predict the future, but I want to be able to take away any anxiety there is about food. Food is for energy. Yes, we can & should enjoy our meals, but that's it. Our family has decided that in the next few years, when Claire is able to take part in more physical activities, that we are going to start creating different traditions on those holidays that surround food. Thanksgiving for example, we have decided to volunteer at a soup kitchen or retirement home. This is good for all of us on many different levels. Maybe we'll go paddle boarding in the morning (if I can peel AJ away from football - thank the good Lord for DVR) or go for a long bike ride. Holidays are about family, not gorging yourself to the brink of sickness on an annual meal. (Average Thanksgiving Meal for One Person: 3,000 calories)

Protein: A molecule composed of polymers of amino acids joined together by peptide bongs. A protein has several functions. It may serve as a structural material (keratin/hair), as enzymes , as transporters (hemoglobin), as antibodies, or as regulators of gene expression.
A protein may be classified based on its form and main functions: it can be a globular protein like most enzymes, fibrous protein which are for structural role; and membrane proteins that serve as receptors or channels for a molecule to pass through the cell membrane.

• Each gram of protein equals 4 calories
• There are complete & incomplete proteins (complete proteins contain all the essential amino acids)
• Complete proteins include meat, poultry, fish, dairy, eggs, and soy
• Incomplete proteins include nuts, grains, fruits, and vegetables
• Too much protein in the diet can be harmful to your kidneys & liver
• Proteins are used mostly for energy, metabolism & muscle mass 

Most people in the United States/UK do not have issues with meeting the recommended daily intake of protein. Too much protein, or the wrong kind, in your diet can definitely cause issues. For example, consuming too much meat can cause gout. 

Protein is essential for your body in regards to building and maintaining lean muscle mass, strengthening your immune system, repairing damaged cells and tissues, manufacturing hormones & functions as an energy source.

Research proves that protein is by far the most filling, having a large thermic effect. When your body is digesting food it is naturally burning calories in the process. This is called the Thermic Effect Of Food. Different foods have different thermic effects & protein happens to require the most amount of calories to digest.
By no means do I suggest going over the recommended daily amount that is right for you of your child, but this proves that protein is pretty darn good for you.
More Info on Thermogenesis

As you can see from my example below, Ryan Gosling clearly eats his protein. :)

The Basics: Part III

In The Basics: Part I we went over the glycemic index (GI) & how we should make sure to choose foods that don't cause sudden spikes & drops in blood sugar. In Part II we went over the three different macronutrients: carbs, proteins & fats. Now I'll tell you how each macronutrient affects the glycemic index. Let's start with those pesky carbohydrates. Most of the carbs we eat are made up of starches & in order for all of the starches to be absorbed and enter into our bloodstream, they have to be broken down into glucose (the smallest of the sugar molecules of which starches are composed). Glycemia indicates the rate at which glucose is absorbed (digestability). Carbohydrates are very complex (no pun intended), so I am going to try to outline the important parts & explain why it matters to the GI, and our kids.  

 

This is a picture of Claire's first visit to Manny's Deli in Chicago. 

That is half of my turkey pastrami sandwich, just HALF! She weighed around 6lbs in this picture. Clearly I polished off about 4lbs of sandwich, whoops. 

Those days are long gone!

Food Starch:  Extreme boiling temperatures modify starch structure. When starch is heated, water is absorbed, and the starch granules swell with a sort of solution. When the heating process is prolonged - usually boiling to desired consistency, what happens is something called gelatinization, because the solution formed has a gelatinous, highly viscous consistency.  The greater the degree of gelatinization in starches, the greater its capacity to become glucose and, naturally, the greater its tendency to raise blood sugar levels (GI). 

This does not hold true for ALL vegetables, but mostly for starchy ones such as potatoes, sweet potatoes, yams, plaintains, and carrots. Raw carrots, for example, have a GI of 20. That GI spikes to 50 as soon as they are boiled. My point here is that when eating these items, serve in moderation & try to serve both raw & cooked carrots (not at the same time of course). We were advised to stay completely away from white potatoes, and yams or sweet potatoes should be enjoyed not more than once a month. Personally, I can't stand plantains, so those will be no problem to stay away from. Also, slightly cooking veggies, like steaming them, actually helps the body absorb more of it's nutrients. But to steam them so they lose all structure & are just a wet mess, they have been reduced to kinda useless.  

Recent studies confirm that the body absorbs much more of the beneficial anti-cancer compounds (carotenoids and phytochemicals—especially lutein and lycopene) from cooked vegetables compared with raw. Some vitamins in food are the ones to suffer being that they are reduced by up to 30% once the food is heated. 
Claire loves carrots, and right now she has to eat them somewhat cooked to be able to get her little teeth to break them down. Each time I give them to her, however, I try to not cook them quite as much as the time before. Hopefully she will be able to move onto raw ones in the near future. I am trying to get her to eat more & more raw veggies, and we always eat slightly cooked veggies too.It will always be a combination of the both.
It is very note-worthy to share that it has been found that steaming your veggies, rather than boiling them, causes less gelatinization. My message here is that vegetables (qualifying vegetables) should be enjoyed both steamed, or lightly cooked, and raw to maximize all their health benefits.

Corn has an especially high starch content, meaning that it is quite high on the GI. As you know from reading food labels (and you should be reading food labels), food starch is CORNstarch. It is used as a thickening agent, biding agent, in a lot of bread items & in powdered sugar. Modified food starch, again is cornstarch, is really found in so many prepackaged foods. Popcorn, on the other hand, has a lot of benefits like fiber content & the level of an anti-cancer compound called ferulic acid. 

Another reason cooking from scratch has numerous health benefits!

Fiber contained in starches reduces glucose from being absorbed into the bloodstream, hence preventing blood sugar spikes. Starchy fruit is higher on the GI depending on how ripe it is. This is true for many fruits & vegetables: the longer they sit to ripen, the higher their number on the GI. The skin of most fruit, like an apple, contains a good portion of it's fiber. Peeling an apple does no one any favors in regards to GI & fiber. Fresh fruits & vegetables are necessary for more than one reason!

When starchy foods are ground into finer particles (wheat into flour, oats into cereal, etc) it allows for easier absorption, hence raising it's GI.  Because of the way flour is now milled & super-refined, they have lost fibers, proteins, vitamins, minerals & essential fatty acids, which causes the (unnatural) rise in it's GI. 

To see where your next meal falls on the GI, click here:  Check the GI
Nutritionists advise that anything that falls on the GI at 70 or higher is a HIGH GI, anything 50 or below is is low. 

At one time (for a brief moment) I thought a gluten-free diet would probably work beautifully for Claire. Then I started researching what is actually in gluten-free foods and found a lot of them just replace wheat with rice (big no-no for PWS). This is why I urge everyone to read & understand your food labels. Some gluten-free bread is also low-carb, so there are good choices out there, we just have to look for them!  

COMPLEX vs. SIMPLE CARBOHYDRATES:
Complex carbs are usually the more nutritious of the two, found in fruits and vegetables (apples, berries, spinach, broccoli, beans, zucchini, lentils), whole grains (quinoa, barley, wheat, buckwheat)  & cereals (oats, semolina wheat). These carbs are broken down more slowly, giving the feeling of fullness for longer. 
Simple carbs are the bad ones. They are usually found in sweets (table sugar, honey, milk, pop - or soda again for you southerners, juice, etc). These carbs tend to only be a quick fix for hunger, lasting a very short amount of time. Fruit is really the only simple carb considered to be good for you.

 

There are many ways that carbohydrate starches & sugars are modified. They all affect the body's ability to digest & absorb them, leading to glycemic & insulin reactions. (http://www.montignac.com/en/the-factors-that-modify-glycemic-indexes/)
So, basically, carbohydrates are evil. Kidding! Carbohydrates provide fuel you use as energy for physical activity, brain function and organ function. However, carbs that are not used are stored as fat. 

Eating carbohydrates stimulates the production of serotonin, a brain chemical that impacts mood and appetite. Studies indicate that those with PWS have increased neuronal reward activation in response to food, especially high energy foods, both pre- & post- meal (Holsen et al., 2006, 2009: Miller et al., 2007). We believe that is where the craving for these high-energy (high carbohydrate-containing) foods come into play with those with PWS. Getting into good habits with good carbs will help curb those cravings naturally. 

The Basics: Part II

These posts might be a little more technical than you would like, but I feel that in order to WANT to make the appropriate changes for our children in the area of food, we must understand why. In The Basics: Part I we went over the glycemic index & what it means not just for those with Prader-Willi, but everyone. The glycemic index is one of the baselines for which we want to create our meals/snacks around. Personally, I think everyone should use this when choosing what to eat.

In a paper written in part by our favorite doctor, she concluded after a study of 63 children, that a macronutrient balanced diet had much better outcomes than a energy (calorie) restricted diet alone. The kids (ages 2-10) who used the macronutrient ratio of 45% carbs, 30% fat & 25% protein with 20 grams of fiber per day had on average 20% LESS body fat at the end of the study. WOW!

What is a macronutrient? They are the three groups we are trying to balance: Carbohydrates, Fats & Protein. Carbohydrates are mostly made up of starches & sugars. Fat has several different types including saturated, monounsaturated, polyunsaturated & essential fatty acids. Protein is mostly made up of amino acids (long chains of amino acids). A little fun fact: the first amino acid ever isolated was in asparagus!

The typical weight gain in a child with PWS, which usually precedes an increase in appetite starts between 18 - 36 months. This weight gain has been hypothesized to be a result of decreased overall energy of the child & possibly abnormalities in carbohydrate metabolism. There is a difference in hormones with people who have PWS, including high levels of ghrelin** & low levels of insulin**, which are considered to contribute to the appetite abnormalities.

Back to macronutrients! You have probably all seen the new 'My Plate' which took the place of our popular food pyramid. I like the My Plate idea for kids without PWS, but luckily we have our own special plate & it looks like this:

High Quality Proteins:

• Lean cuts of meat
• Lean ground meat
• Nuts & Seeds
• Nut butters
• Eggs
• Seafood
• Soy products (ie: tofu)

High Quality Carbohydrates:

• Green leafy veggies
• Berries
• Vegetables
• Fruit

High Quality Fats:

• Nuts
• Avocados
• Olives
• Fish 
• Some cheese

 Big No No's for Prader-Willi Syndrome:

• Pasta 
• White Potatos
• Rice

Next time I'll talk about more about these macronutrients & what the different types are within each. Also how the body breaks them down & uses them for energy. The balancing we use for these macronutrients is essential in keeping energy levels steady & being able to use diet for optimal overall performance. Claire is in 4 therapy sessions per week, takes one nap & has a typical bed/awake time. We believe that with Claire's balanced macronutrient diet, only using qualifying foods, and the fact she's on a pretty strict routine, is the reason she is doing so incredibly well. She can do an hour and a half of straight physical therapy and not get sleepy. She also does back to back occupational & speech therapy and is still rearing to go after those are over. 
(UPDATE 1/1/2015: Claire is now 3 & has been in preschool since September. She goes 3 half days at a private preschool & LOVES it. She has since been diagnosed with severe apraxia of speech. She has 5 therapy sessions per week plus one session of hippotherapy. She takes a nap on occasion & is still doing very well in the area of energy.)

We are in a Mom's Group here in southwest Florida & we do play dates at least one to two times per week. She is always able to keep up with the kids her age & a few years older with no problem whatsoever. I know at almost 2 years old we have not begun to deal with a lot of the things that come with Prader-Willi Syndrome in behavior & physical attributes, but that is why we are so adamant about getting in front of potential problems before they surface. It's easier to start with a preventative approach rather than fix a problem. 


**Ghrelin is a hunger-stimulating hormone that is found in the lining of the stomach & in certain cells of the pancreas. For further information about ghrelin, click here: Ghrelin Info 
**Insulin is a hormone produced in certain cells of the pancreas & is responsible for the carbohydrate & fat metabolizing of the body. Insulin causes cells in the liver, skeletal muscles, and fat tissue to absorb glucose from the blood. For further information on insulin, click here:Insulin Info

The Basics: Part I

I thought I should run through some of the basics of Prader-Willi before we dive into all the fun stuff like cooking & baking. We use the system recommended by our doctor of balancing. Claire is to eat a mix of carbohydrates, fat & protein each day. Each of the categories has it's qualifying foods, and not ALL food is considered okay for PWS kids regardless of what category it falls into. It's is a lot more difficult to stay away from these foods than we originally thought. No Hostess products, no pop (or soda for you southerners), no fried food, etc. The ratio we use is 40% carbohydrates, 35% fat & 25% protein to make up 100% of her meals per day. We do try to make sure each meal & snack is balanced as well, so there is no spike in blood sugar. 

This is where the glycemic index comes into play. It's important to balance meals (& snacks) because we never want the blood sugar to drop significantly, giving the feeling of hunger. As you can see from this chart, with the right balance, the line stays steady & doesn't raise & drop. Those quick raises are usually caused by sugar spikes (from fruit, bread, or any type of carb) that isn't balanced out with some protein &/or fat. 

The glycemic index is a tool we use to figure out how Claire's body is metabolizing a certain food. For example: I try to keep a snack of an apple paired with some cheese or peanut butter. Apple is the carb & the cheese is a protein & fat. This is where I reiterate saying this doesn't have to be super duper stressy. I have a list of go-to foods, what category they fall under, and what the normal serving size is for her age/size. I pair it up & don't have to worry that an hour after she eats a snack that she'll be hungry again. 

I will start to add some charts & lists of qualifying foods and some ideas of how to balance meals & snacks so the spikes and drops don't happen. 

I'm a mom who wants to help others with the food challenges of kids with Prader-Willi Syndrome.

Hi! I'm going to attempt this blogging phenomenon to help some of the other families out there who have children with Prader-Willi Syndrome. Having a child with special needs is definitely a challenge, and Prader-Willi is one of those that affects what most of us enjoy each day, several times a day: EATING! 

While our thoughts & ideas about food need to switch gears when we have a child diagnosed with this syndrome, life doesn't have to be super duper stressful. "Life happens, and so does sugar" as our nutritionist once said. I want to help make the transitions easier, choices easier & the balancing a no-brainer. 

I have done loads of research, asked tons of questions to doctors, therapists & nutritionists, and have come up with a good system for our family. Things need to change every once in a while, and we do need to eat out sometimes, but it's the routine & good habits we get into, and pass along to our kids that is going to make all the difference in the world. I do not have a medical background, and I do not promote for your child exactly what we do, but I merely want to share what has worked for us & what could possibly work for you & your family. 

A little background: Our daughter, Claire, was diagnosed at 4 weeks old with Prader-Willi Syndrome. She was 6 weeks premature as my water broke early from quick-onset of polyhydramnios (excess fluid - and I mean A LOT of excess... talk about a camel). We have it kind of easy right now because Claire, at almost 2 years old, still depends on us 100% to feed her & isn't aware of all the other food choices out there. Again, what we do with our daughter isn't for everyone, and as broad as PWS is, this may not work for each individual child. 
Claire has been treated with Growth Hormone since about 4 1/2 months old, is taking some supplements, thyroid meds & is on a twice-daily dose of a breathing treatment through cold & flu season. The first year she did the typical sleep studies to rule out apnea, swallow studies to make sure her swallowing was safe & regular appointments at her Endocrinologist. She has had croup, has been hospitalized for dehydration & had had spells of night-terrors. Other than that, she has been quite healthy, and we consider ourselves extremely lucky. 
Claire had an NG tube for the first 12 months of life, eating from a spoon at about 8 months, but still needing the tube to supplement her formula. We went right from the tube to a sippy cup. We find it amazing that even thought she was tube fed for so long & never took a pacifier, that she seems to have no texture issues with food. She crawled at about 14 months & just started walking a few weeks ago at around 22 months. She does have orthotic insoles for her walking shoes because her ankles turn inward. No signs of scoliosis or hip/back issues.
We moved to southwest Florida in November of 2012 from the Chicago area. The ability to be outdoors & as active as we want year round was a huge draw for us. As you probably know, people with PWS are encouraged to stay active & exercise daily. We also wanted to be driving distance & under the direct care of our favorite doctor, Jennifer Miller. She's amazing! 

My idea for this blog is to share pertinent & up-to-date nutritional information directly related to Prader-Willi Syndrome. Since I do a lot of cooking & baking I'll also be sharing recipes, snack ideas & how to handle different food-related blunders.  I will try to keep it strictly food-based as we all have different approaches to discipline, sleep schedules & all things parenting. 

Thanks!