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Feeding
Issues,
Nutrition,
donated by Stephanie Price
Over the years, I
have had many parents ask me about the diet I use for Hannah. I will state over
and over that I learned this diet from other moms who have had great success
with it. I hesitate a bit to write
this diet information. All of the children with SMA are unique and different
individuals just as we are all as individuals unique and different.
There are some basic fundamental principles that should be followed if
you decide to use this diet for your child in my opinion. ……
Elemental
amino acid formula
Double
dilution or more of the formula
Vitamins/Minerals
No
meats
No
dairy
No
junk food (candy, soda, ice cream etc. etc)
Acidophilous
Because the
children are so different in where their strengths and weaknesses lie, their
formula recipe needs to be adjusted for accordingly.
I emphatically do
not believe you must have a medical background or a college degree to feed a 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 have inborn errors of fatty acid and protein
metabolism. Most SMA children I
know of who are not on the diet present with symptoms of malabsorption and
Kwashiorkor . The children look
bloated. Some children have fatty livers (as noted upon autopsy). One common
mistake is for SMA children to be put on high caloric-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. Allergens
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, pneumonia 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.
I have been 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, Scandi Shake etc. were not doing as well and becoming
sick and / or dying.
Tolerex and/or Pediatric Vivonex
does not give all the necessary vitamins, minerals or necessary fat 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 Q 10, etc to Hannah’s diet. I try to give most of the vitamins
and minerals in amounts 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 the market. This book in
particular is easy to read and readily available in most bookstores. The FSMA
website has a few articles you can refer to under nutrition.
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 to 1 ½
grams per kilogram are 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 nowhere
to stay.
To begin
Use 1 to 1 ½ 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 to thick
to place through 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 or added in over a 24-hour period of time.
I have used both methods and both ways work well.
The danger of most 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 these children as if they are "
normal". Another danger is that physicians and dieticians prescribe way to
many calories. A normal healthy active moving child needs more calories.
Typically 70-80 kcal/kg. In SMA, a good range is 60-70 kcal/kg. As long as the
child grows and is gaining weight then they are doing well. Children do grow
when on this diet and do gain weight. 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. (Do not mix breastmilk together in a blender).
If a mom were still nursing I would encourage this. I recently 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 a Milkbank in North Carolina.
(www.hmbana.org) There are six milkbanks in the USA. A 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 with 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 - 300
mg/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 heartrate and vomiting. These are classic signs
of protein toxicity (to many amino acids). I
have not ever had this problem. Begin slowly and work up the amounts of amino
acids. You should 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. Read
the 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 ounces a day of liquids in her diet consisting
of juice, water and / or breastmilk. On days where she is hot and sweats, I give
her more water.
It has been proven that children on this diet do not get scoliosis like other
SMA children. In some instances, there has been a correction of the degree of
curvature of the spine. I know of one example where a little girl with type 1
went from a 33-degree curve to a 21-degree curve with early detection and proper
bracing! Some SMA patients have begun this non-dairy elemental amino acid diet
when having been on a dairy or whey or soy formula diet and actually had
dramatic improvements in curvature of the spine as well.
Currently I add the following to Hannah’s diet in addition to the Tolerex……
L-glutamine
L-valine
N-acetyl-cysteine
L-isoleucine
L-leucine
L-phenylalanine
L-arginine
L-carnitine
Co
enzyme Q10
Creatine
B12
(oral and b-12 sublingual drops)
Folic
acid
Copper
Folinic
acid
Methylcobalamin
Vitamin E
Vitamin
C
Phosphorous
Calcium
Magnesium
Potassium
Evening
Primrose oil
Multivitamin
Other things I have tried…….
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 recommend you have labwork drawn
regularly (fasting quantitative plasma amino acids, basic metabolic panels, mg,
zinc, phos, creatine ). When fasting the children get their morning meal and
then fast 6 hours. The lab results always come back for Hannah saying within
normal limits. However, if a biochemist reviews the results it is noted
otherwise. The biggest problem most often seen is metabolic acidosis!
I send the lab results to a biochemist in Canada at Nutrichem (www.nutrichem.com).
I speak to Kent MacLeod. He mixes more amino acids to add to the tolerex food
mixture I give Hannah based upon HANNAHS bloodwork. 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. Most of the families who use the amino acid diet with their children
just guess and add more amino acids and supplements. I do not feel comfortable
doing things this way. Again, my medical background is a strong factor in this
area. I do it this way. I am the only mom I know who does this. All the other
moms just guess. This is ok for them. They add glutamine, arginine, creatine,
some branch chain amino acids (leucine, isoleucine and valine) All this
"stuff" is what I give Hannah extra of from my mix made in Canada.
What can I say? It still is a guessing game however. Noone 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. To much protein? Over time Hannahs body adjusts 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, casienate 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. There
are families who would give their left arm to know all this information I have
just told you. 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. In my opinion why not try the diet? You have everything to gain 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!
If you would like I can refer you to parents who use this diet and will tell you
the dramatic differences they have seen in their children since beginning this
diet.
There is a lot to this diet as you can see. If you have
further questions please e-mail me.
Respiratory
Issues, donated by Monica English
As the mother of a child with type 1
SMA, I wanted to take the opportunity to share my perspective on respiratory
care. SMA is a very misunderstood
disease and often in trying to do the right things we are counseled by well-meaning
care providers to do the exact opposite of what will truly help. I take the
responsibility to educate myself so that I can actively participate in all of
the decisions - medical, emotional, social - that affect my child. I know that
it is through this education that my child lives and thrives today.
Far and away the leading cause of death for children
with SMA is respiratory complications.
Because SMA affects all voluntary muscles controlled by the motor
neurons in the spinal column, the muscles that aid in breathing are affected.
These children breathe very in very shallow breaths and coughing
effectively on their own is almost impossible.
The first thing I wanted to address is the incorrect
use of oxygen for our children.
If children with SMA can fill their lungs with air they have no problem
using the oxygen available.
Their problem is filling their lungs with air and clearing their
secretions.
Giving continuous oxygen will not solve either of these problems.
Oxygen can actually be detrimental to our children.
Delivering continuous oxygen will bring the oxygen saturations in their
blood up and make the numbers on the monitors look good but will not solve the
underlying problem which is a need for better lung expansion and to clear
mucus from the airways.
There is a sensor in the brain that controls rate and depth of
breathing.
If there is not enough oxygen in the blood the brain sends a signal to
the body to breathe deeper.
Our children breathe shallowly in the first place; if they are given
continuous oxygen, the drive to breathe deeper is satisfied and the child’s
muscles don’t have the opportunity to continue to work hard and maintain
their strength.
It becomes a downward spiral - shallow breathing leads to less oxygen
in the blood so more oxygen is administered which leads to breaths becoming
more shallow and on and on.
On top of all of this the mucus that probably caused the lower oxygen
saturations in the first place is still sitting in the lungs blocking air from
being delivered to that particular area of the lungs.
The first thing we use to help our child with lung
expansion is a bipap machine.
We use this to aid our child’s breathing when she is asleep or in the
case of a chest infection.
The bipap has a mask that is secured over the nose, the mask attaches
to a tube which attaches to a machine that gives an extra “push” of air
when the child breathes in.
This way air is delivered deeply to the lungs during the time the bipap
is used. Each morning we take it off and she is free and clear for all of her
waking hours.
The bipap helps to fully expand the lungs keeping all portions of the
lung healthy.
If a child is not able to breathe and unable to use portions of the
lung it does not take a long time for that unused portion of the lung to die.
By helping her fill her lungs with air properly the lungs stay
healthier.
Another thing that deserves
mentioning here is the settings for the bipap.
Often when a bipap is prescribed it is prescribed with settings such as
8/4 or 12/6. The first number
refers to the pressure delivered when the child is breathing in and the second
to the pressures delivered when the child is breathing out. Settings such as
those mentioned above are too low to truly expand a child’s lungs and give
them the benefit they need from the bipap. My daughter has used settings around
18/4 since she was seven months old. We
look for pressures high enough that her chest expands well when breathing.
The pressures delivered when she breathes out can be left as low as the
machine will allow. These children don’t have problems breathing out - just in.
The second machine we use for lung expansion is the
In/Exsufflator also called the Cofflator or Cough Machine.
This machine simulates a cough by delivering a deep breath of air into
the lungs and then switching to a negative pressure that pulls the air out
quickly and moves airway secretions and mucus up to the point that they can be
suctioned out of the mouth or nose.
We have watched this machine save our child’s life several times.
It is surprising how a small amount of mucus in the wrong area of the
lungs can threaten the life of a child with SMA. This machine is the only thing
we’ve found to effectively move her secretions so that they can be cleared
from her airways. We use the In/Exsufflator as part of respiratory therapies in
the morning and at night when she’s healthy to clear whatever mucus has built
up and really expand her lungs well.
We also use it
frequently when she has a chest infections to clear the extra
secretions created during the chest infection.
We have a pulse oximeter at home.
This is a machine that measures the percentage of oxygen in the blood and
the heart rate.
We use the pulse oximeter to gauge her need for airway clearance.
We do preventative therapies morning and night when she is well and
continually monitor her oxygen levels when we suspect a cold.
When her oxygen saturations drop below a certain level we go through the
therapies needed to clear her airways.
Treatments during times of illness will be addressed in another section
of the website.
Here
is a very detailed write-up of what we do for Taleah on a well day.
1.
Chest Percussion Therapy on the Right Side
a. At a slant so her head is inclined - 5 minutes
b. Flat - 5 minutes
c. At a
slant so her feet are inclined - 5 minutes
2.
Coughing with the In/Exsufflator settings are 40 over 40
a. Five
cycles of 2 seconds inhale, 2 seconds exhale 1 second pause
b. Give a
break for child to catch their breath
c. Five
cycles of 2 seconds inhale, 2 seconds exhale 1 second pause
3.
Suctioning
a. Deep
suction nasally
b. Suction
orally
4.
Repeat steps two and three
5.
Chest Percussion Therapy on the Left Side
a. At a slant so her head is inclined - 5 minutes
b. Flat - 5 minutes
c. At a
slant so her feet are inclined - 5 minutes
6.
Repeat steps two, three & four
Chest
Percussion Therapy
When we do chest percussion therapy we use an
anesthesia mask and put tape over the opening that would normally attach to
tubing. This allows us to get
achieve good percussion. We thump
the upper, middle & lower lobes of the lungs on the front and back of her
body. Some like to attach
tongue depressors to the mask to make a bit of a handle.
I find I like just holding the mask in my hand and using it that way.
We always make sure there is a thin layer of cloth over her body so we
are not percussing on bare skin. We
also make sure there are no buttons, snaps or zippers where we are percussing.
Often people who see us doing this for the first time are concerned
that it will hurt her because the hollow mask makes a loud thwapping sound but
she really loves this part, often she will fall asleep as we are percussing.
If you are concerned try it on someone else and see how firmly you can
percuss on their back without causing any discomfort.
We use pillows to position her at the different inclines.
If you happen to have Versaform Pillows for positioning this works
really well, if not you just have to get a little creative with your pillow
stacking to make a good slant.
In/Exsufflator
or Cough Machine
For the In/Exsufflator the pressure settings are 40
over 40, the timing is 2 seconds inhale, 2 seconds exhale and 1 second pause.
Because the cough machine measures in pressure rather than amount of air
delivered, the settings are the same for children and adults - the machine
will adjust the volume as needed. The
newer models have a inhale flow knob. The
company rep told me that the setting with three arrows is for adults and the
setting with one is a more gradual for babies.
After I spoke with a rep I spoke with a very knowledgeable
pulmonologist who said that even the babies need the quick inhale, so we have
always kept ours set to the three arrows.
Our model also has either a manual or automatically cycling options.
We use choose to use the automatic rather than flipping it from inhale
to exhale to neutral each cycle.
Because the knobs are so easily bumped we check the
pressures and settings before every use; the cough machine can deliver an
awful lot of pressure if the knobs get bumped.
We have a mask, the same kind they use to deliver anesthesia that
attaches to the tubing from the cough machine. We try to make sure we lift a
little on the back of her neck to make sure her airway is open.
I should mention that there are babies who cannot tolerate being on
their back and they use the cough machine while turned to the side with no
problems. We place
the mask over Taleah’s nose and mouth and flip the machine on.
It always starts at the inhale and that solves the problem of trying to
quickly get the mask into place during that one second pause.
It takes firm pressure to get a good seal, we hold it and count out
loud until the five cycles have passed. I
feel it important to count out loud so that she also can know how far through
the therapy we are.
Now I will be honest; this is not Taleah’s favorite
thing in the world but I counted it up one day. If she’s getting a total of 8 sets of five in the morning
and the same at night, that’s a total of six minutes and forty seconds of
something she doesn’t like each day to keep her clear and healthy for the
rest of the day.
Suctioning
You will find that there are dozens and dozens of
different ways to suction, let alone different lubricants, different
catheters, etc. I’ll tell you
my way but this is one thing that can be done effectively several different
ways.
We were originally taught to suction using Bard
Urological catheters attached to a Y shaped valve. The Bards were
very soft and kind to her airways. The
problem we ran into was that Taleah was smart enough to swallow at just the
right time so that rather than guiding the catheter into her lungs it would go
into her stomach. We switched to
a Tri-Flo Suction Cath-N-Glove Kit. This
catheter is a firmer, but that meant that we could wind it around our fingers
(gloved of course) and give it a curve that held as we introduced it into her
nostril. We haven’t had
problems getting into the right place ever since.
Another nice thing is that the kit comes with a basin for sterile water
and sterile gloves. Also with the Tri-Flo catheters they have centimeter
markings so you can watch how far you are going in.
Another point for debate is what is used to lubricate
the catheter. Some will use water
soluble surgical lubricant, but others will tell you that even that can dry
and clog the airways. We just dip
the catheter in water before introducing it into her nostrils.
I should mention that this is not a skill that can be
learned just from reading. We
were taught during Taleah’s hospitalization in New Jersey for her G-tube
placement. Once we got home I
needed some refreshers so Taleah’s pediatrician arranged for us to go back
into a local hospital and learn again I suggest you do the same.
Anyway,
we wash our hands really well and dry with paper towels (cloth towels can harbor
bacteria). We fill the basin with
sterile water. We then we don the
sterile gloves and designate one hand as the clean hand that touches nothing but
the catheter and one hand as the dirty hand that covers the opening in the
catheter and holds the back of suction. We
slowly and carefully introduce the catheter into one nostril and pull at a
moderate pace back while covering the opening in the catheter.
If we get a lot of yucky stuff we go down again and yet again until
things look fairly clear. Then we repeat with the other nostril and if we think she
might have brought some mucus up into her mouth we suction her mouth as well.
When we’re done we suction some water up through the catheter to clean
out the tubing.
Therapy
Issues
ORAL MOTOR EXERCISES
JAW:
1. Chewing in rhythm, beginning slowly and increasing the speed
2. Chewing like a camel, moving the jaw from side to side
3. Stretching the mouth as wide open as possible
4. Move jaw from extreme right as far as it will go and reverse position
to left.
TONGUE:
1. Stick tongue out and in (like a lizard)
2. Touch each tooth with tongue
3. Don some tonghe clicking
4. Touch the nose with the tongue tip
5. Touch the chin with the tongue tip
6. Move the tongue in a circle around the lips (licking something off the
lips)
7. Move the tongue from corner to corner of mouth
8. Push the tongue against the left cheek wall and then over to the right
cheek.
9. Curl the side edges of the tongue up to form a grove down the center
10. Raise the tip of the tongue to the upper gum ridge behind the upper
teeth while the mouth is help wide open.
LIP:
1. Round lips in a pucker (blowing kisses)
2. Stretch the lips into a smile
3. Rotate between #1 and #2
4. Compress and release the lips rapidly (paa, paa, paa)
5. Talking out of first one side of the mouth then the other
6. Hold a cork between the lips and expel it to hit a target
7. "Curl" the upper lip up to try and touch the nose
INHALATION/EXHALATION (RESPIRATORY):
1. Blow bubbles in water
2. Blow soap bubbles
3. Chew and blow bubble gum
4. Blow out candles
5. Blow Pinwheels
6. Exhale vocalizing "ah" and time it to see who can vocalize
the longest
7. Use a straw and cotton ball, inhale and hold the cotton ball against
the straw for as long as possible.
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