There are quite a few families who use this same diet and see the same miraculous results It has been published that there is protein metabolism problems with those who have SMA. I have found that this is true with our daughter as well. For this reason, we began using a free-form amino acid diet with Hannah. Our particular choice at the moment is Tolerex. Free-form amino acids are the simplest forms of protein you can acquire. All proteins are made up of amino acids. Free-form amino acids do not come from food sources. The amino acid formula eliminates the bodies need to breakdown the protein because it is already in its most simplistic form. Because the body does not have to work so hard to break down the protein this saved" energy can be used to prevent burning muscle for energy. I believe the children with SMA do not use the protein from foods properly and do not make muscle or maintain an anabolic state. They are always burning muscle for energy. If you look closely and research protein malabsorption or protein energy malnutrition or Kwashiorkor (starvation) our children exhibit these same signs and symptoms. Bloating, fat protruding bellies, sluggish digestion, low body temperature etc etc ..they exhibit these signs in spite of getting high protein diets. (Typically doctors in their ignorance put the children on Pediasure. This is a very high protein drink but these children still exhibit the aforementioned symptoms, why because they do not use the protein; DAIRY) I do not find these symptoms to be nearly as noticeable and in most cases nonexistent when using the elemental amino acid formula. Added pluses are the constipation goes away, the sweating goes away for the most part and so does the puffy feet, eyelids and hands. Temperatures regulate to a more normalrange. Allergies and poor diet indirectly effect the bowel. If you have excess proteins to eliminate or to digest (because your body is not using them properly) you will become constipated, edematous and bloated..
For whatever reason, it seems that children with SMA do not digest proteins well, especially dairy proteins. This can be seen a lot when children are on such dairy formula as Similac, Enfamil, Prosobee, Ensure, Pediasure, Boost, breastmilk fortifier etc etc. All these products have dairy or dairy based proteins in them. Keywords to watch out for are whey, casein, and caseinate. I am not talking about lactose free formulas. Lactose is milk sugar and is a problem as well. Typically when children are on dairy they have these common symptoms, weakness, tiredness, unexplained coughs, unexplained fevers, reflux and bronchitis. Young babies with SMA who are fed dairy might exhibit these signs: crying fits after or during feeds, reflux and gassiness or colic type symptoms, major constipation, unexplained wakefulness in the middle of the night, and crying for no apparent reason. If your child has a feeding tube you might even notice that they do not empty their stomachs very well. Food can be present in the stomach hours after a feeding. I do not know if there is an allergy to the dairy or if the children with SMA just can not properly eliminate the dairy because of protein malabsorption problems or both. I do know that when protein is not absorbed and filtered from the body properly it cannot be eliminated properly. I believe this is why I see almost no edema in Hannah. She utilizes her elemental proteins effectively and can urinate the excess out of her system.
(YOU MAY OR MAY NOT NOTICE THE BLOATING IN THE STOMACH HANDS AND FEET IF YOU DO NOT know what you are looking for. You might also not notice the difference until you do things to make a difference. Sometimes seeing is believing. If you decide to try an elemental amino acid formula I highly recommend you speak to moms/caregivers who have been there done that. After all, there is no real knowledgeable healthcare practitioner who lives this disease day in and day out like we do. It is important to remember to start out slow and work up to desired levels of amino acids. Since our children do not have a lot of muscle mass and can not store vitamins, minerals etc. etc you do not want to overload them all at once and cause serious health problems).
I used to give Hannah only chicken fruits and vegetables plus the Tolerex. I noticed after awhile that she seemed to be weaker on the days when I gave her chicken. Over a period of time, I quit using chicken. Now the only protein Hannah gets is from breastmilk and the Tolerex plus the additional amino acids I add to her diet. Some families use soy products and see no negative side effects from soy protein. Other families report the same dairy sensitivities when feeding soy, wheat products and corn products. I will tell you that Tolerex does have maltodextrin. Maltodextrin is a type of cornstarch. My daughter does have flushing and what I believe to be allergy to dairy, corn, barley, strawberries, and papaya. I avoid these foods as well as soy and eggs. (I do give our daughter the split virion flu vaccine which is made from chick egg embryo. So far, I have never noticed a reaction so I guess we have been lucky.)
Other families who have children on free-form amino acid diets report improvement in spinal curvature. One girl with type 2 sma went from a 24 degree curve to a 12 degree curve (on x-ray) during the first year of amino acid formula. In other cases, the progression of the curvature has been halted.
If you are going to give your child extra amino acids the first premise is of course to do no harm. It is important to try to treat someone who has a basic dietary problem and give them the minimal nutritional amounts necessary. I feel that I am doing this with Hannah.