First off I would like to say thank you to FightSma for doing this as its very important to have lots of open dialogue on all of the subjects of SMA.
I do have to say that I shudder when a parent with a toddler with SMA type 1 starts to ask questions and through the questioning we find out they have no equipment and the child is having trouble sleeping among other things. This tells me we haven't come very far with the treatment and care of SMA and still have a mountain to climb to make the medical community understand that there are viable options of treatment and care. There is still a huge disconnect between the worlds of medicine and Quality of Life and Rehabilitation which cares more about the tools to live as well as you can.
Most of what was said was basic and i'm sure an eye opener for the newly diagnosed families. I disagree with one of the comments that there were many choices with the treatment of SMA. From my research which is extensive you only have 3 choices: 1) end-of-life-care. Just let nature take its course 2) NIV Protocol of Dr. John Bach 3) Tracheostomy/and all of the Bach protocol without NIV. I haven't heard of anything else beyond that?
I especially liked the fact that the PT recommends starting PT as early as possible. I would like to add that
Motor Oral therapy should also be started as soon as possible and thedisconnect between the PT's and SLP's have to stop so that we as caregivers can better treat our children from the tops of their heads to the tips of their
toes but otherwise excellent. Another subject on the same topic is speech. It would have been great if someone spoke about the fact that our kids need to speak through a microphone so they can better hear themselves and in turn their speech will develop. I think SLP's with SMA experience have to become more involved and we need more early intervention in the SLP department especially in the areas of jaw contractures, motor oral therapy, and speech development.
Diet: I have to say that Sarah Feasel, MEd, RD, CNSC comments regarding the elemental formulas ws very inaccurate, misleading and not professional. While it is true that elemental formulas were created to deal with allergies the reason why elemental formulas have been adopted by the SMA community is very different. Mary Bodzo created the AA Diet based on the findings of Dr. Kelly of John's Hopkins. Dr. Kelly found that SMA patients better metabolized elemental to medium chain amino acids more efficiently than full chain animal proteins. Below are some exerpts of conversations by parents with Dr. Kelly.
" 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
On breast milk, " I unfortunately have not in recent years worked on the nutritional aspects of SMA or related neuromuscular disorders, so I don't have any more up-to-date thoughts than I have communicated in the past. I
suspect that the benefit of breast milk comes not from any special
"factor" present in the milk but from the lower protein and specific
fat content of breast milk, which better matches the nutritional needs
of someone with greatly reduced muscle mass. The efficiency of
utilization of the lower amount of protein also is enhanced because,
clearly, the amino acid composition of breast milk is better matched
to human amino acid requirements than any type of animal milk or
animal protein. Moreover, compared to animal milks and proteins,
breast milk fat contains a higher proportion of medium-chain fats,which, theoretically, should be more efficiently metabolized by SMA children than regular long-chain fat."
The dietician Sarah Feasel, MEd, RD, CNSC mentioned that the elemental formulas do not contain enough fat to promote proper brain development especially in a childs early and most important developmental stages.
This is true and this is the reason these elemental formulas are used. Because of the low fat content of
both vivonex (which contains more fat than tolerex) we are better able to optimize the diet by adding appropriate amounts of fat while still being able to deliver the correct amount of protein, carbs etc. Almost all other elemental formulas on the market do not allow this flexibility and you always end up with disproportionate amounts of fat to protein levels which works great for active children not sedentary hypotonic children. It is a misrepresentation of the diet to not explain that other foods must be added to enhance calorie intake and that other fats must be added to elevate the fat intake. anyone who is giving their child the amino acid diet and not calculating these other nutritional factors into the diet are not following the AA Diet of Mary Bodzo. The information Sarah Feasel, MEd, RD, CNSC has presented regarding the AA Diet was not science but a biased opinion unsubstantiated opinion. If she actually knew how to calculate the diet, what is added to the diet, why it was created in the first place (the history of the diet) then the AA Diet would have made sense. All that was said by the doctor at the podium and Sarah was innacuarcy and fear. I would hope that we have moved away from this type of discourse especially when it comes to our childrens health but old habits are hard to stop. In a nut shell these people do not truly understand the diet and until they do these types of opinions should not be tabled.
Judging by the conversation on growth charts I suggest the professionals speaking on nutritional needs re visit the web site at the cdc at http://depts.washington.edu/growth/cshcn/text/page1a.htm ; All the way through this 8 page description of the cdc's growth charts the cdc warns of the inaccuracies of using traditional growth charts for children with special needs. While I use them only to insure there is nothing extraordinary going on with the child I am creating a diet for that is pretty much all they are used for. I take more notice of the Kennedy Kreiger Cerebral Palsy Quadriplegia charts as they have much more in common I think with SMA than regular children.
"And that's all I have to say about that!"