Stephanie was kind enough to send this letter of information to Crystal's dietician, since I was having a hard time getting her to understand my words. Nobody can explain it better than Stephanie.
From Stephanie Price to Crystal's dietician:
I am writing to respond to your many questions regarding the diet I use for my daughter Hannah at the request of Brenda Brames. I hope I can do more then "enlighten" you and help you understand as well as see the importance of the elemental amino acid diet for children with SMA. If you could at a minimum help the doctors see the difference the Tolerex diet makes for the SMA children using Crystal as the example, then I will be pleased and feel my time educating you has been well spent.
First of all, I would like to tell you a bit about myself. I have two college degrees. In fact everything I learned in school seems to be the exact opposite in regards to how things work with Hannah and SMA. I am giving you a bit of background so you will know I am educated and not someone who just is doing things the way I do for my daughter without careful thought and consideration. However, I emphatically do not believe y you must have a medical background or a college degree to feed an 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 at times look bloated, have fatty livers (as noted upon autopsy), do not utilize protein due to the fatty acid metabolism and protein metabolism nature of this disease. Most SMA children I know of who are not on the diet present with symptoms of malabsorption and Kwashiokor. One common mistake is for SMA children to be put on high calorie-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 all types.
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 etc. Allergens as you know 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, pneumonias 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.
You 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 etc. were not doing as well and becoming sick and/or dying.
Tolerex and/or the Pediatric Vivonex do not give all the vitamins and minerals or fat necessary 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 1 10, etc. to Hannah's diet. Most of the vitamins and minerals I try to give 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 teh market. This book in particular is easy to read and readily available in most book stores.
Look at he fsma website under articles for nutrition written by Dr. Kelly for other info. Dr. Kelly has also written an article regarding sma and fatty acid metabolism. If you are interested. I also recommend you e-mail Dr. John Bach.
My opinion
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 go 1 1/2 grams per kilogram is 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 no where to stay.
To begin
Use 1 to 1 1/2 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 too thick to place thru 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 in or add in over a 24 hour period of time. I have done things both ways and both work well.
The danger of 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 them as if they are "normal". You have to have an accurate understanding of this disease. The other danger is that physicians and dieticians prescribe way too many calories. A normal healthy active moving child needs more calories. Typically 70-80 kcal/kg. In SMA, the range is 60-70 kcal/kg. As long as the child grows and is gaining weight then they are doing good. Children do grow when on this diet and do gain weight. Again, 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 well.
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. If a mom is still breastfeeding I would encourage this. I recently, as I said, 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 the Milkbank in North Carolina. There are six milkbanks in the USA. The SMA 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 and dairy.
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 200-300mg/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 heart rate and vomiting. These are classic signs of protein toxicity. Too many amino acids. I have not ever had this problem. Begin slowly and work up the amounts of amino acids. you will 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. Again, read labels. A lot of the acidophilus has dairy proteins in it, especially, the liquid ones.
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 oz a day of liquids in her diet consisting of juice, water and/or breastmilk. On days where she is hot and sweats more, I give her more water.
It has also been proven that children on this diet do not get the scoliosis like other sma children do. In some instances, there has been a correction of the degree of curvature of the spine once. Some SMA patients have begun this non dairy elemental amino acid diet when having been on dairy or whey or soy formula diet and actually had dramatic improvements in this area.
Other things I have added in the past.
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 highly recommend you have lab work drawn regularly (fasting quantitative plasma amino acids, basic metabolic panels, mg, zinc, phos, results always come back for Hannah saying within the normal limits. However, if a biochemist reviews the results it is noted otherwise and dramatic problems are noted. The biggest most often is metabolic acidosis!
I send the lab results to a biochemist in Canada at Nutrichem. I speak to Kent Macleod. He mixes up for me more amino acids to add to the Tolerex food mixture I give Hannah based upon HANNAH'S blood work. 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. Again, my medical background is a strong factor in this area. I do it this way. It still is a guessing game however. No one 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. Too much protein??? Over time Hannah's body adjust 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, caseinate 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. 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. It is my belief that after so much time has past and much muscle loss occurs, it is almost too late to get benefit from this diet. Crystal is young and in my opinion has everything to gain by utilizing this diet 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!
All the glory goes to God! He is the great physician and is truly the one in control of who lives and who dies. I am so fortunate to have met the right people in my long journey with Hannah who have taught me these things and helped me. I try to pass on that knowledge and help save other little lives. This diet is not a cure. The diet and respiratory are means to help keep our children with us in the best condition I know possible until the cure is found.