The problem is is that the dieticians working with doctors (even the good doctors) calculate diet to the 10th percentile using the CDC growth charts for regular children. Doing this is NUTS!!! if you take into accounte the physical makeup of an SMA patient based on Dr. Kelly's findings i.e. that "that sma type 1 patients have muscle mass as low as 5-10% that of a regular child and that muscle mass makes up around 40% of your body mass" if you calculate to the 10th percentile the patient would be obese as far as fat to muscle ratio. Dr. Kelly also states that only the Age/Length charts should be used to insure the child is growing properly. When a child stops growing properly is anyone's guess when it comes to SMA Type 1 there is no data at all let alone even clinical or anecdotal there is none. The main thing to remember is that if a dietitian recommends you do something without doing skin fold tests and other tests to properly estimate the bodies physical makeup they are only guessing. And because they have guidelines set out by the government they have to follow they have to calculate to the 10th percentile to avoid liability problems. This is why the Kennedy Krieger CP Quadrilgia chart is so important becasue at least its a population with closer physical traits than regular children and if you are going to use a chart it just makes more sense. Here is an explanation below from Dr. Kelly to Jeanna Huette years ago. This information is basically what the AA Diet is based on as far as calculating the diets. I advise getting skin fold tests done on your child by a qualified dietitian that does it a lot and is good at it. Shira has muscle mass around 15% that of a regular child. it's important to note the information below should be applied to what ever diet you deliver to your child and that the diet should be calculated optimally and re calculated often. The goal is to deliver the optimal nutrition without excessive volume to prevent reflux, and retain as much movement as possible. Our children are sedentary and have very low muscle mass so there needs are far different than regular children. The trick is to find the balance and deliver exactly what they need and like the rest of us they need a healthy diet not just processed food which I see all the time building diets for people. The type of tube and placement are also important when calculating diets but in my opinion the crap most dietitians suggest these children to eat is nothing short of ridiculous with very little thought put into it.
, “For such an "average" child, muscle constitutes 40% of body weight, whereas for the average SMA-I child, muscle is at most only about 10% of body weight, and often only 5%. Similarly, because about 40 to 50% of caloric expenditure is from muscle metabolism, a child with SMA needs far fewer calories, often only 60% of that recommended for age. For nutrition recommendations, physicians are taught to go by the book. However, unfortunately, there is no nutrition book written for SMA, and dietary recommendations made using standard scales are just not appropriate. For example, when a child is very small for age (below the third percentile), as some SMA children are, physicians are taught to use the "weight-for-height" chart to specify an ideal weight for a child's size rather than age. However, again, the weight-for height charts were developed for children whose body composition is normal or at least potentially normal when better nourished, which never is the case for SMA. Thus, the published weight-for-height charts are not valid for SMA and should never be used.
Although what I have written here explains the basic principles behind the special weight and nutrition goals for SMA, in practice I usually look only at the length chart to make sure a child's linear growth has been steady. If so, then the rest of my recommendations are based on what a child looks and feels like, not a number that I calculate. However, for physicians who are not familiar with SMA and muscle disorders of similar severity, the calculations I have presented usually help them approach the problem correctly and avoid the almost universal problem of overfeeding in SMA.”
I also want to point out that just because an SMA Type 1 child is alive passed the age of 2 doesn't mean they are healthy for an SMA Type 1 child it just means they are alive. Many dietitians and parents can't look passed this fact and overload the children and fall back on the knowledge just that the child is alive so in the SMA Type 1 world must mean they are doing well. our children have a different baseline normal than regular children and most doctors and dietitians do not want to believe this. Food has many psycho social dynamics attached to it. Some parents have serious eating challenges themselves which directly affects their children and the same goes for doctors and dietitians and their own problems directly affect what they see before them instead of using science, logic, observation within our community etc. basically science. It's pathetic!