Over the years, I
have had many parents ask me about the diet I use for Hannah. I will state over
and over that I learned this diet from other moms who have had great success
with it. I hesitate a bit to write
this diet information. All of the children with SMA are unique and different
individuals just as we are all as individuals unique and different.
There are some basic fundamental principles that should be followed if
you decide to use this diet for your child in my opinion. ……
amino acid formula
dilution or more of the formula
junk food (candy, soda, ice cream etc. etc)
Because the children
are so different in where their strengths and weaknesses lie, their formula
recipe needs to be adjusted for accordingly.
I emphatically do
not believe you must have a medical background or a college degree to feed a SMA
child this diet. All that is needed is hope for your child and willingness
to learn. I firmly believe this diet helps slow the progression of muscle loss
and or stops the progression.
Typically children with SMA have inborn errors of fatty acid and protein
metabolism. Most SMA children I
know of who are not on the diet present with symptoms of malabsorption and
Kwashiorkor . The children look
bloated. Some children have fatty livers (as noted upon autopsy). One common
mistake is for SMA children to be put on high caloric-high high-protein diets.
This type of diet makes the children weaker. They can not handle the protein and
tend to become fat from all the calories. They also become sick, get recurrent
infections and usually end up dying. Unfortunately this happens all to
frequently with SMA type one children. This diet is not just for type 1 SMA but
Children with SMA have a lot of allergies. I am not sure why when tested lab
results turn up negative to the many things they seem to show reactions to. The
source of all allergens is protein. Dust, animal dander, plants etc. Allergens
are caused by antigens floating around in the bloodstream. Dairy and milk
proteins seem to be a major allergen for children with SMA. Other allergens are
whey, soy, and oats. Side effects noticed are copious secretions, flushing, high
fevers, bloating, weakness, general malaise, bronchitis, pneumonia and
constipation and ear infections.
The beauty of the elemental amino acid diet is that you are giving protein in a
broken down simplistic form. Whole proteins tend to be the problem. These
proteins are not easily digested or are not utilized somehow in SMA. This is
also why when utilizing the amino acid diet you also must eat only fruits and
vegetables to get the most benefit.
I have been asked who designed the diet for Hannah? I did! I began with the
Tolerex and added one ingredient after another over a period of time to make
sure no adverse side effects were noted. I learned the basics of the diet from
other families of type 1 children who were using the diet and had success. Their
children were living. I noticed the children who were on formula such as Similac,
Pediasure, Progestimel, Scandi Shake etc. were not doing as well and becoming
sick and / or dying.
Tolerex and/or Pediatric Vivonex
does not give all the necessary vitamins, minerals or necessary fat according to
the RDA for all age groups. This is why I add additional vitamins, minerals,
evening primrose oil, safflower oil, vit e, calcium, magnesium, phosphorous,
potassium, co Q 10, etc to Hannah’s diet. I try to give most of the vitamins
and minerals in amounts close to or a bit above the RDA. All one needs is a
nutritional almanac to research this. I usually recommend the one written by
Gayla and John Kirschmann. There are many books on the market. This book in
particular is easy to read and readily available in most bookstores. The FSMA
website has a few articles you can refer to under nutrition.
In SMA you do not need a lot of protein for protein
synthesis. A normal healthy child is recommended to get 2 grams per kilogram of
protein a day. In SMA it has been my experience that less is better. 1 to 1 ˝
grams per kilogram are sufficient. SMA
persons do not have the lean muscle mass that "normal" healthy persons
do. This is why they do so much better when fed less protein. Due to the
lessened muscle mass, this can cause problems. Vitamins and minerals can not be
stored. The stores that they have are not stored long because they have nowhere
Use 1 to 1 ˝ packets of tolerex. Double dilute per package instructions. This
is essential. You can dilute with juice and or water. I add even more water but
you must double dilute otherwise the formula is too strong. Then you feed this
mixture over the course of a day. Not to be given all at once. Add baby food
fruits and veggies at meal times. No whole proteins like chicken or veal. No
dairy. You can add vitamins and minerals. You can add safflower oil. Safflower
oil is an essential fatty acid.
I mix the Tolerex in a blender with baby foods. This consistency is not to thick
to place through a g-tube. It works quite well. The key is dilution. You must
flush the g-tube after every feeding. If a continuous feed is given via pump
then additional water can be bolused or added in over a 24-hour period of time.
I have used both methods and both ways work well.
The danger of most physicians and dieticians who try to prescribe this diet is
that they overload these children on amino acids. Four to five packets daily are
prescribed. This is just way too much and is dangerous! It can kill these
children. SMA patients can not handle this much amino acids and or protein! I do
not know of anyone who uses more then 2 packets a day. This is for children that
I know of up to age 13. These children do not have the muscle mass to handle
this much protein. You can not treat these children as if they are "
normal". Another danger is that physicians and dieticians prescribe way to
many calories. A normal healthy active moving child needs more calories.
Typically 70-80 kcal/kg. In SMA, a good range is 60-70 kcal/kg. As long as the
child grows and is gaining weight then they are doing well. Children do grow
when on this diet and do gain weight. You can not base their growth on the
charts. As you recall, the growth charts are for formula fed children not
breastfed or Tolerex fed children. The main factor is they grow and gain weight.
When they are fed to many calories they get fat and can not breathe or move as
For those who are breastfeeding. Keep it up. I still give Hannah breastmilk. I
am a firm believer this is another reason she does so well. You can mix together
the Tolerex and the breastmilk. (Do not mix breastmilk together in a blender).
If a mom were still nursing I would encourage this. I recently had a baby. I am
again pumping my milk for Hannah. When I was no longer able to breastfeed prior
to the birth of my daughter, I got breastmilk from a Milkbank in North Carolina.
(www.hmbana.org) There are six milkbanks in the USA. A doctor who researches SMA
and nutrition has stated to me that when children with SMA who breastfeed stop
nursing they go downhill and rather quickly. My belief is that it is because
they are put on formula with dairy. Also, http://www.hmbana.com/wsnAC28.html
is the website address of the milkbank I used for 4 years.
When beginning I recommend you start with just the Tolerex. The next ingredient
to add would be glutamine. Start with 500 milligrams. Glutamine is the most
abundant amino acid in the body! Typically SMA children are low in glutamine. A
very conservative doctor who studies SMA and nutrition recommends 200 - 300
mg/kg glutamine. I have used additional glutamine with Hannah since she was one
year old and have never had a problem.
Signs to look for are increased heartrate and vomiting. These are classic signs
of protein toxicity (to many amino acids). I have not ever had this problem. Begin slowly and work up the
amounts of amino acids. You should be OK. Now that I have said this, I give no
100 percent guarantee. I just believe you will not have any problems if you do
it this way.
The next ingredients to add would be up to the mom. Usually calcium and the
vitamins are a good place to start.
I also highly recommend you begin giving acidophilus to counteract the yeast
problems so many SMA persons have. I have never ever heard of any problems from
giving acidophilus only great results. I use Natures Way Primadophilous for
children. It is found in the refrigerator at the health food store here. Read
the labels. A lot of the acidophilus has dairy proteins in it, especially, the
In SMA, there is a lot of loss of water via breathing fast, open mouthed, and
sweating. I highly recommend you give lots of water for hydration. Secretions
should be thin not tacky. The recommendation for a 1 - 3 year old is 115-125
cc/kg of water. I give Hannah the amount a younger child should get. Hannah
almost age five gets 53 or more ounces a day of liquids in her diet consisting
of juice, water and / or breastmilk. On days where she is hot and sweats, I give
her more water.
It has been proven that children on this diet do not get scoliosis like other
SMA children. In some instances, there has been a correction of the degree of
curvature of the spine. I know of one example where a little girl with type 1
went from a 33-degree curve to a 21-degree curve with early detection and proper
bracing! Some SMA patients have begun this non-dairy elemental amino acid diet
when having been on a dairy or whey or soy formula diet and actually had
dramatic improvements in curvature of the spine as well.
Currently I add the following to Hannah’s diet in addition to the Tolerex……
(oral and b-12 sublingual drops)
Other things I have tried…….
I have given Hannah flax seed oil. I thought it would be great. She had almost
immediate respiratory problems, became cyanotic (blue lipped) and had secretions
the rest of the day even though she only had one feeding of this. Same thing
happened with fish oil. This was my experience.
If you really are going to use this diet, I recommend you have labwork drawn
regularly (fasting quantitative plasma amino acids, basic metabolic panels, mg,
zinc, phos, creatine ). When fasting the children get their morning meal and
then fast 6 hours. The lab results always come back for Hannah saying within
normal limits. However, if a biochemist reviews the results it is noted
otherwise. The biggest problem most often seen is metabolic acidosis!
I send the lab results to a biochemist in Canada at Nutrichem (www.nutrichem.com).
I speak to Kent MacLeod. He mixes more amino acids to add to the tolerex food
mixture I give Hannah based upon HANNAHS bloodwork. I just feel this works best
for me. I am not good at guessing and want to try to do what I think is best for
Hannah. Most of the families who use the amino acid diet with their children
just guess and add more amino acids and supplements. I do not feel comfortable
doing things this way. Again, my medical background is a strong factor in this
area. I do it this way. I am the only mom I know who does this. All the other
moms just guess. This is ok for them. They add glutamine, arginine, creatine,
some branch chain amino acids (leucine, isoleucine and valine) All this
"stuff" is what I give Hannah extra of from my mix made in Canada.
What can I say? It still is a guessing game however. Noone truly knows all the
ins and outs of SMA. I usually follow the recommendations of the biochemist in
Canada. He however is also no "expert" in regards to SMA. I have found
Hannah gets weaker sometimes with the mix I give her so I slowly work up the
mixture of which I give her until I am at the recommended dosage of the
biochemist. To much protein? Over time Hannahs body adjusts and she is actually stronger
then weaker. All the children are different and their individual diet has to be
made for them. The basics that are important are the elemental amino acids,
essential vitamins and minerals, no dairy, whey, soy or dairy derivatives like
casein, casienate etc. These all cause weakness in SMA.
Over the years I have known many parents from all over the world and have talked
with them regarding this diet. Some have used this diet and some have not. There
are families who would give their left arm to know all this information I have
just told you. Those that do say they wish they had known of it earlier. It is
best to begin this diet when the child is young so that muscle wasting can be
spared. In my opinion why not try the diet? You have everything to gain and
nothing to lose.
Unfortunately, diet alone is not enough when it comes to SMA. Aggressive
respiratory care is needed as well. I again refer you to Dr. John Bach. The
children who follow this diet and his respiratory care protocol tend to live
longer and healthier then any other SMA patients in this country!
If you would like I can refer you to parents who use this diet and will tell you
the dramatic differences they have seen in their children since beginning this
There is a lot to this diet as you can see. If you have
further questions please e-mail me.