Information I have received from Stephanie Price through email!
Amino Acid Diet:
I believe that they are the very best nutrition you can give a child with SMA types 1,2 or 3. You will not get the information I give you from ANY professional Dr., Dietician etc. The tried and true test is however the fact that you can ask any of the moms who have tried the elemental amino acids and have happy living children. The amino acids are not a cure for sma but are very helpful in my opinion in increasing quality and life expectancy for what can be an otherwise dreadful disease.
It has been shown in literature that children with SMA have a protein metabolism problem. Amino acids make up proteins. When you give you child amino acids you are giving them the end product of protein metabolism. Their bodies do not have to work to break down the proteins and this is why I think amino acids are so beneficial. You will most likely see improved health, less illness, more energy, less weakness etc. Each individual is just that individual. You said that your daughter became weaker when taking creatine. I think there is just a fine line. some creatine is probably good for Crystal. I have seen myself with Hannah that if I give her 1 teaspoon of her custom amino acid mixture she is weak but if I give her 3/4 teaspoon amino acid mixture she does great. Again, too much is not good. How do you know how much is too much? You just have to experiment and watch your child. I wish there was a book that could tell us exactly what to do but there is not. I recommend you talk to me and other moms I will tell you of if you are interested and how they do things.
We spoke of proteins. Kinder cal is chalk full of dairy. It is again my opinion that dairy proteins make our children weaker. Dairy and all dairy products... here are some not so obvious forms of dairy allergens: Casein, sodium caseinate, milk protein, whey. Soy is an ok alternative but it has shown to make the SMA children weaker as well. The amino acid diets are simply the best. you can research more about dairy at "the not milkman" website.
I also believe that the best diet is a low protein diet. Most of us moms give our children 1 to 1.5 grams per kilogram protein a day. A normal healthy child should get 2 grams per kilogram a day so you are not giving them that much less. I also believe a diet of only fruits and vegetables is the best because again you want low protein. Hannah and others have become weaker on chicken. I have never tried any other meat. Peas seem to give Hannah secretions that are more copious. I no longer give her peas.
You do not want to overload on amino acids. The two elemental amino acid formulas are Tolerex (the most popular) and Pediatric Vivonex. Tolerex has more carbohydrates and less fat. Vivonex has less carbohydrates and more fat. Remember SMA persons also have a fatty acid metabolism problem as well. Some of the children take a little of both types of formula but again most use the Tolerex.
It is not enough to just give the amino acids. you must also give the rest of the needed vitamins and minerals that are not supplied in the elemental formulas in sufficient quantities. You should give a good multivitamin and glutamine at the very least. I recommend a nutritional almanac and a book that gives you the RDA values for the vitamins and minerals. Most of us moms give over the RDA in some things. The reason is that in SMA you are dealing with less muscle mass (10 percent of normal). Muscle is where these vitamins and minerals are stored. Since we do not have the muscle mass in SMA the vitamins and minerals cannot be stored.
If you use the amino acids you must over dilute more then the recommend amount on the box. I recommend you over dilute. Hannah if four and a half. On a daily basis she gets a minimum of 53 ounces of liquid in the form of water, juice and or breast milk: mostly water.
I can tell you what I give Hannah in a nutshell: 1 1/2 packets of Tolerex, amino 75, glutamine, vitamin c, vit e, calcium citrate with Vitamin D, magnesium, magnesium, multivitamin, salt, evening primrose oil, safflower oil, potassium.
I have a special custom amino acid mixture I give her as well based upon her blood values from fasting quantitative plasma amino acids: valine, leucine, isoleucine, phenylalanine, b-12, cyanocobalamin, folic acid, folinic acid, coenzyme q 10, arginine copper creatine.
I have just recently begun giving Hannah b-12 oral drops. I don not think she absorbs the b-12 in her gut. Recent announcements made by Gideon Dreyfuss show that SMA persons lack the b-12 and folic acid and might show improvement from doses of each.
I can only tell you what works for Hannah. I do believe you will see improvement in Crystal if you do try the amino acids. You must get her off all dairies and be very religious about not giving dairy to truly see the best results. I make no guarantees however. God borbid I tell you something and it backfires. I have not seen this yet only good things. I just want you to know it is all guess work and we do the best we can. The other alternative in my opinion is to listen to the professional. This has gotten us nowhere. In fact, I usually do the exact opposite of what they recommend.
Oh yeah, Hannah has a g-tube. The mixture of Tolerex or Vivonex might taste awful to Crystal. I assume she still eats by mouth since she is a type 2. Brenda adds: "I have had a lot of success mixing the formula with juicy juice and adding jello powder to disguise the taste somewhat. At least to take it to a more tolerable level."
Breast milk?
I would store all the pumped breast milk you can. I still give mine to Hannah but not a lot. Only 2-3 ounces now. I used to give her 8 ounces when she was 6 months. If I could do over again I would mix at 6 months the Tolerex, breast milk and glutamine. The breast milk is not dairy. I do not watch my dairy intake that much and do not see a problem in Hannah. I do not know if it causes a problem or not. I used to get my milk from a milk bank before I had Eliza. So who knows what they ate.
I believe in a nutshell that the breast milk is good for SMA since Dr. Kelly told me that when those moms quit nursing the children go down hill fast. Do you know who Dr. Kelly is? It is my opinion because the breast milk is already broken down in to amino acids and has all kinds of living cells in it that you can not replicate in formula or any food. Be careful when listening to the dieticians since they know nothing of SMA even if they say they do. They do not and can be harmful and get you to overload your child on amino acids. The number one sign will be increased pulse on the saturation monitor. If severely overloaded can cause vomiting which will lead to metabolic problems which you definitely do not want. It can happen quickly. No more then 2 packs and this is for an older child. I recommend you begin with one pack and over dilute, add the glutamine and then slowly go from there. See how it goes. Perhaps add a jar of baby food.
