The Proof it works!!

The proof blended diet works!  

We started blended diet around age 2.  At the time he has been on formula since birth.  At 18 months he weighted 24 pounds (50%)  and was 32" (50%)  tall and his EB was still very severe; about 60% of his body had open wounds.  We had blood work on in the fall of 2012 and here were the results

Vit D 26 (range 30-80)
Creatinine 0.52 (range 0.26-0.42)
Protein 6.4 (range 6.5-8)
Sed Rate 91 (range 0-15)
Iron 38 (range 45-182)
Hemoglobin 9.3 (range 9.4-14.3
Hemaoticrit 28.3 (range 34-40)
Calcium 8.9 (range 8.5-10.5)
Ferritin 25 (range 36-84)
Zinc 54 (range 60-120)
Selenium 80 (range 23-190)

(items in bold are abnormal results) 


My request for yearly blood work went ignored because no lab was willing to draw blood due to his EB.  Finally last month we were able to get a lab to draw some labs.  At age 4 1/2 he is 39 pounds (75%) and 43" (95%) tall.  His skin looks amazing and if he wasn't so rough when he played, we could stop wrapping all together but he still needs the protection!   After 2 1/2 years on blended diet this is the the blood work results.

Vit D 30 (range 30-80)
Creatinine 0.30 (range 0.26-0.42)
Protein 7.5 (range 6.5-8)
Sed Rate 7 (range 0-15)
Iron 42 (range 45-182)
Hemoglobin 11.9 (range 9.4-14.3)
Hemaoticrit 34.3 (range 34-40)
Calcium 9.8 (range 8.5-10.5)
Ferritin 30 (range 36-84)
Zinc 62 (range 60-120)
Selenium 134 (range 23-190)

(items in bold are abnormal results) 

Low Iron and Ferritin are pretty normal in EB.  It may also be because GI took him off his multivitamin back in March when he turned 4 and they said he was too old for the infant version but offered no alternative.  So we have started a new multi-vitamin with iron and added more iron rich foods to his blended diet and will recheck the results in December. 


In addition to blood work, you can also see the difference in his skin!!

Joey's back before blended diet,

 

a couple months after starting the blended diet,

a year after 

 

and his back today.   

 

In addition to improved blood work and wound healing, blended diet also has eliminated his constipation, reduced his over all inflammation levels and a decrease in his dosage of acid-reflux medication.


I do understand that blended diet my not be the answer for everyone with a G-tube but I think the results speak for themselves.  I can only imagine how bad his skin would look and how bad his results would be if we was only getting sugar supplements all day long.  He hasn't had a skin infection in years and the number is colds/viruses he gets yearly have decreased each year as well.   His doctors are very pleased with his overall heath.  There is no plan to remove his G-Tube anytime soon.  He can and does eat by mouth but not enough to survived without his tube and that is OK!  If he goes to college with it, I don't care.  As long as it isn't causing him any issues, the benefits we are seeing certainly out weigh the risks.



*Disclaimer:  I am not a medical professional.   This is not medical advice.  i am just sharing what I have learned along the way with the hope it makes things easier for others. 

©Garrett's House 2021

Administering Blended Diets and Recipes!

Administering blended diets is a little different than feeding formula.  

Most blended have to be pushed by hand quickly vs using a feeding pump over the course of an hour. 

We use the wider extension (blous) to attach to the mic-key button.  And we use a 60ml syringe to push the blends into the stomach.  We push it thru very quickly because that is what my son prefers.  But some kids need it pushed much slower.  It will also depend on your child and what they prefer and can tolerate.  Once the feeding is finished, we flush the line with 20cc of water.  Water is very important for kids with eb to keep the kidneys healthy.  The amount of water each kid needs depends on their age, weight, and health.  Your child's doctor can tell you how much free what they need daily.  

We feed at normal meal/snack times, to mimic when a child without a gtube would eat.  We don't do overnight feeds, which is common in kids with gtubes.  The human body isn't designed to be feed continuously overnight. The gut needs time to rest and when one is feed overnight it really never has time to rest.  

Blended diet is time consuming and not as easy as using formula.  But the health benefit it SO worth all the extra work. 

Here are some example recipes we use:

Breakfast/Snack

Lunch

 

 Dinner

Each one is what I call a "batch".  I then divide each batch into a day's worth of blends.  It is at that point I add other things; oils, the baby oatmeal, Benacalorie, etc...  Then I divide all that into a service size for my son, place in snack size Zip-Loc bag and freeze until we need them.  I typically blend once a week making enough for 7-10 days worth of meals.

You can do any combination of food you'd like.  It all depends on your child's needs, food allergies, etc...  These are just the things we have tried.






*Disclaimer:  I am not a medical professional.   This is not medical advice.  i am just sharing what I have learned along the way with the hope it makes things easier for others. 

©Garrett's House 2021

Blended Diet Ingredients

Once you have read blended diet basics, it is now time to move into creating recipes! 

While those with EB  require more calories each day due to wound healing, you need to look at more than just calories when planning a blended diet. Protein, iron, zinc, calcium vitamin D are just a few things that are also vital for a healthy body. 

With blended diet your goal is to create a well rounded meal.  

First make a list of any foods your child is allergic too.  Or any foods your child needs to avoid.  Kids with EB should avoid foods that are highly acidic to avoid triggering their acid reflux.  They also need diets high in protein, zinc and iron for optimal wound healing. 

Next make lists of the major food groups: 

Fruits

Veggies

Protein

Oils 
Liquids

You can choose whatever foods you want to try.  But as an example I will share the lists of foods we have tried along with the nutritional values of those foods.  I also rank them by calorie count.  I know the pictures are a little fuzzy.  It was hard to turn an Excel spreed sheet document into a picture. Clicking on them might help or you can email me and I'd be happy to send the Excel spreed sheet to you.  sara.denslaw@gmail.com

Making Blends

I choose one or two items from each food group, mixing and matching each time to create a wide variety of blends.  I go off what is available in stores and what is on sale.  I always pick fresh over frozen and frozen over canned.  Avoid canned fruits and veggies (except canned pumpkin) when possible.  Canned fruits and veggies tend to be high in sodium and preservatives.    The oils are to add calories since most fruits and veggies aren't high in calories.  Some oils are very beneficial to the skin and over all health. And with 120 calories per tablespoon is a great way to add calories without adding a ton of volume.  


I create blends in large batches, and then divide them into 30oz portions.  To EACH 30oz portion I add the following:

1)  3 tablespoons Happy Bellies Organic Baby Oatmeal.  )The servicing size is 3 tablespoon and it provides 80% of the RDA of iron.  Plus it had probiotics in it as well.)

2)  1 packet of Carnation Instant Breakfast

3)  1 tray of Benecalorie

Once all that is mixed, I then divide the blend into 6oz serving and freeze them in snack size Zip-Loc bags.  You can store blends in the freezer for months but only in the fridge for 2-3 days.  I take out baggies as we need them and thaw! 

The National Nutrient Database is what I used to determine the nutritional value of each food item.  You can look up ANY item you want to use to determine the break down of the nutritional value.  

This is just a basic idea of the MANY wants to create a blended diet.  Tomorrow I will share some of the recipes we use to give you even more ideas on how to create a blended diet for your child!  



*Disclaimer:  I am not a medical professional.   This is not medical advice.  i am just sharing what I have learned along the way with the hope it makes things easier for others.  

©Garrett's House 2021

Blended Diet Basics

There are many things you need to figure out before you start down the blended diet path.  

First you need to determine how many ounces per day your child needs.  Your doctor or nutritionist is the best person to get that number from.  Next you need to determine how many ounces per serving your child can tolerate at a time.  Then once you figure that out you can come up with a feeding schedule. My son has volume issue.  Meaning he can only tolerate small amounts at a time. So we feed small amounts many times a day.

For him we give five 6-ounce servings of blended diet per day for a total of 30 oz. He gets feed a 7am, 11am, 2pm, 6pm and 9pm  He also does eat and drink by mouth on his own so we mostly use blended diet to supplement what he eats on his own.  But there some days he eats nothing and on those day he is on 100% blended foods.  Every kid is different.  Some will tollerate 8-10 oz three times day.  While others need smaller amounts more often.  We try to stick to the same meal/snack as someone without a g-tube would.   

Once total amount is determined, next the nutritional values need to be determined. How many calories does your child need?  How much iron?  How many grams of fiber, protein, carbs, etc.  If your doctor is unable or unwilling to help with that here is a great interactive calculator based on your child's age, weight, height and activity level.  The only thing it doesn't take into account it having EB.  But our doctor has us add 500 calories to what is recommended based on his age, weight, height and activity level.  For my son he needs between 2300-2500 calories a day to gain, grow and heal.  I know that seems like a lot for a 4 1/2 year old bit it is working for him.  

So your check list so far:

1) quantity

2) number of feedings

3) nutritional values needed per day

Next you need a blender.  If you are just starting out a basic kitchen blender will be just fine.  We have a Vitamix that was donated to us from the company to use since my son has a g-tube.  We contacted the company and provides medical documentation and they sent us a refurbished blender free of charge!  But when we first started out, we used a regular blender with no issues. 

Types of high quality blenders:

Vitamix

Blendtec 

Once you have determined:

1) quantity

2) number of feedings

3) nutritional values needed per day
4) blender

You are now ready to move into preparing the foods!!   

*Disclaimer:  I am not a medical professional.   This is not medical advice.  i am just sharing what I have learned along the way with the hope it makes things easier for others. 

©Garrett's House 2021

Just say no.....to Pedisure

When a child with EB (or even without EB) get a G-tube it is very common for the child to be feed a diet of only (or mostly) Pedisure (or  another commercial high calorie formula).  It is a very convenient way to provide nutrition with a g-tube but how nutritious is it really?

Here are the ingredients in a bottle of Pedisure:


Water, Sugar, Corn Maltodextrin, Milk Protein Concentrate, High Oleic Safflower Oil, Canola Oil, Soy Protein Isolate. Less than 0.5% of: Short-Chain Fructooligosaccharides, Natural & Artificial Flavor, Cellulose Gel, Potassium Chloride, Magnesium Phosphate, Potassium Citrate, Calcium Phosphate, Tuna Oil, Calcium Carbonate, Potassium Phosphate, Salt, Cellulose Gum, Choline Chloride, Ascorbic Acid, Soy Lecithin, Monoglycerides, Potassium Hydroxide, m-Inositol, Carrageenan, Taurine, Ferrous Sulfate, dl-Alpha-Tocopheryl Acetate, L-Carnitine, Zinc Sulfate, Calcium Pantothenate, Niacinamide, Manganese Sulfate, Thiamine Chloride Hydrochloride, Pyridoxine Hydrochloride, Riboflavin, Lutein, Cupric Sulfate, Vitamin A Palmitate, Folic Acid, Chromium Chloride, Biotin, Potassium Iodide, Sodium Selenate, Sodium Molybdate, Phylloquinone, Vitamin D3, and Cyanocobalamin.
Contains milk and soy ingredients.

Calories: 240
Calories from Fat: 80
Fat, g: 9	14
Saturated Fat, g: 1	5
Trans Fat, g: 0
Polyunsaturated Fat, g: 2
Monounsaturated Fat, g: 6
Cholesterol, mg: 5	2
Sodium, mg: 90	4
Potassium, mg: 350	10
Carbohydrate, g: 33	11
Dietary Fiber, g: 1	4
Sugars, g: 18
Protein, g: 7	14

Vitamin A:	10
Vitamin C:	40
Vitamin D:	40
Vitamin E:	20
Vitamin K:	20
Thiamin:	40
Riboflavin:	30
Niacin:	10
Vitamin B6:	30
Folate:	15
Vitamin B12:	25
Biotin:	15
Pantothenic Acid:	25
Choline:	15

Calcium:	25
Iron:	15
Phosphorus:	20
Iodine:	15
Magnesium:	10
Zinc:	10
Selenium:	10
Copper:	10
Manganese:	20
Chromium:	10
Molybdenum:	10
Chloride:	8
* Percent Daily Values (%DV) are based on a 2,000 Calorie diet.

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The following excerpts were taken from an article called:

Why PediaSure Is The Worst Thing You Can Do For Your Picky Eater?

 
"After water, the most prominent ingredient is sugar! How is that considered a nutritional supplement?! According to the nutrition label, one cup of serving has 240 calories – 80 calories are derived from fat, 25 from protein, and the remaining 135 are from carbohydrates. Now, here’s where it gets interesting – the carb breakdown is just 1 gram of fiber, and 18 grams of sugar! That’s the equivalent of 4.5 teaspoons of sugar per one bottle of PediaSure."

After sugar, it is followed by Corn Maltodextrin (GMO), which is used as a thickener in soft drinks, salad dressings and soups. The fats in this drink are derived from Safflower oil and Canola oil (which are also GMOs). Can you imagine your child drinking genetically modified oil as a source of their nutrients?

The proteins are derived from milk, soy, and peas as isolates and concentrates. This means that the original, whole food is deconstructed and highly processed, losing its beneficial nutrients."

How on Earth can that be considered a HEALTHY, nutritional meal for a child with constant open wounds?!?!?   Most kids with EB get 4 cans of that over a 24 hour period.  18 grams of sugar per can, times 4 cans, equals 72 grams of SUGAR A DAY!!!!!!  That is about the equivalent of two 12-oz cans of Coke PER DAY.  And that is pretty much the entire daily diet for many kids with EB who have G-tubes.

Sugar is one of the main causes of inflammation within the body.  And EB wounds are often causes by chronic inflammation.  So only eating or being feed nutrition that is is known to cause chronic inflammation just creates a vicious cycle of non-healing wounds and chronic inflammation.  I truly believe that these commercial,  high sugar, inflammatory causing "nutritional" beverages are contributing to the rapid decline of so many kids with EB.

There is however, an alternative that is slowly catching on within the EB community!!  And that alternative is something called The Blended Diet.  It is what is sounds like, normal food that normal people eat but blended into a liquid or puree state that allows it to be pushed thru a g-tube using a syringe.    We have been going the blended diet route for almost 3 years now and the results have been overwhelming positive.  My son is a healthy, happy, very active preschooler who has ZERO chronic wounds, does not have any of the chronic complications that arise in EB such as anemia, constipation, vitamin or mineral deficiencies.

This week I plan to create a multi-part series about all the things one needs to know before start down the blended diet road.  It is not as complicated as it sounds either.  It can be time consuming, but once you get the basics down, it becomes an easy thing to incorporate into your daily routine! 


*Disclaimer:  I am not a medical professional.   This is not medical advice.  i am just sharing what I have learned along the way with the hope it makes things easier for others.  

 

©Garrett's House 2021 

Testing for EB

Why Should Genetic Testing be done?

 

There are actually many reasons to have genetic testing done.  Here are just a few

~ much more accurate than a biopsy

~ will not only give you the type of EB, but subtype too

~aids in family planning; not only for parents, but for the child as well

~ allows for other family members to be testing to see if they are carriers of the same mutation(s)

~ offers insight as to the prognosis of your form of EB

~prepares you for which complications you need to be on the look out for

~allows you to participate in clinical trials and potential be one of the first to receive the cure when it is found.  (otherwise you'd have to have genetic testing done prior to participating any clinical trial/cure)

Once you have received your results, here is a list of question to ask, if the doctor doesn't already answer them.

1) How many mutations were found?

2) Which gene(s) were they found on?

3) Which form(s) of EB are these mutations associated with? 

4) Have the mutations ever been reported before in others with EB?

5) Are the mutations homozygous or heterozygous? 

6) What is the inheritance pattern for the mutation(s)?

7) Should, we have parents be, tested?  Should our other children be tested as well?

 

 

Genetic Testing

Molecular genetic studies (DNA analysis) is a must more accurate way to diagnosis EB.  Molecular studies are done to identify the specific genetic mutation and to determine the mode of inheritance (recessive vs. dominant)  There is a new genetic test that tests for ALL known EB mutations in one sample: It is called the Epidermolysis Bullosa (EB) XomeDx^®Slice  

 

 
Below are two labs in the US  that can perform genetic test for EB.

1) GeneDx
207 Perry Parkway
Gaithersburg, MD 20877
301-519-2100
www.genedx.com 


2) Prevention Genetics: EB Decode program

3) Invitae genetics; EB Panel

 

 

 

 

Skin Biopsy for EB

Though not the go-to in EB diagnostics anymore, here is the information for those that genetic testing is not a option for.

 

Samples of the skin or a blister may taken by a trained doctor to determine the type of EB one has.  The skin samples must be processed for specific studies, immunofluorescence antigen (IFA) mapping and transmission electron microscopy (EM). 

 
Immunofluorescence antigen mapping is performed to identify exactly where the blister has occurred and which proteins are involved (absent or diminished in amount). This is a specialized study that should be done by a pathologist in a laboratory that specializes in this procedure.

Only two labs in the US can process EB biopsies:

1) Beutner Labs, Inc.
3580 Harlem Road
Buffalo, NY 14215
1-800-288-0549
http://www.beutnerlabs.com/contact/

2) Stanford Dermatopathology Service
Department of Pathology - H2110
Stanford Medical Center
300 Pasteur Drive
Stanford, CA 94305
650-723-6736

 

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©Garrett's House 2021

blended diets

Instead of the pre-made commercial formula, we choose to give Joey a blended diet.  A blended diet is when you take real food and blended up to the consistency of a smoothie.  We then feed like a set volume at every meal. 

Here is a basic guide I came up with that would be easy for someone new to the blended diet world to follow. 

The main ingredient we use is Kefir (it is a yogurt drink)

32oz Kefir  (560 calories 44g protein)

Next I use one 16oz serving of a fruit and one 16oz serving of a veggie.  I mix and match each time depending on what is available at the store and/or on sale.  Here are the fruits and veggies we have tried.

16oz servings:

FRUITS
3 large apples diced (288 calories 16.2g fiber 1.6g protein)
2 mangos diced (214 calories 6g fiber 3% iron)
3 large pears diced (288 calories 15g fiber 6% iron)
3 large peaches diced (114 calories 5.5g fiber 3% iron)
3 large bananas chopped (315 calories 9g fiber 3g protein)
1 pint of Blue Berries (194 calories, 8.2g Fiber 2.52g protein, 5% Iron)

VEGGIES
1 large sweet potato diced (480 calories 6g fiber 3g protein)
1 bag baby carrots chopped (159 calories 6g fiber 3g protein)
2 bunches kale (68 calories 3.9g fiber 6.6g protein)
2 Avocados (454 calories 12.9 fiber 4.2g protein)

Green Beans, Peas, Mixed Veggies, Squash, Pumpkin (canned or fresh) are other options we have used but I don’t have the nutritional value for.

Try to stay away from fruits and veggies that are acidic as they can irritate those with acid reflux.



GRAINS- I recently started experimenting with using a grain...this is what I have tried thus far.  I don't use it every time though

1 1/3 cups cooked minute rice multi grain medley (brown rice, red rice, wild rice, quinoa)
320 calories 4g fiber 8g protein 16% zinc 8% iron


I blend the fruits, veggies and Kefir with 8oz prune juice (180 calories 3g fiber 4% iron 4% zinc)

Once this is all blended it makes about 72 oz of blended food.  I then divide it into five 16oz glass mason jars. I then add the following to EACH mason jar:

3 tablespoon Happy Bellies Organic Baby Oatmeal (60 calories 2g fiber 10% zinc 80% iron.)

1 tablespoon olive oil or coconut oil (120 calories)

1 tray (1.5oz) of Benecalorie (330 calories.)

Each container comes out to around 1300 calories each.  He gets 7-8oz servings 4 times a day depending on how much he each on his own by mouth.  I make his blended food about 3 times a week.  

In addition to the blended diet he also gets 7oz of 30cal/oz Elecare Jr. Formula and 120 cc of free water per day.

The doctor wants him between 1800-2000 calories a day when possible :)  He needs those calories for wound healing, energy (which he had a TON of) and to gain/grow.    

There are many blended diet recipes out there and this is just an example of what we use!  Yes this is a lot of work, but I think Joey is healthier because of it!

©Garrett's House 2021