Management

Frequent Feedings of a Low Fat Diet When Adam's body runs out of metabolized food he can use for energy he gets very sick because his body starts trying to break down his muscles for energy. His muscles can't give him energy and decompose, going out of his body in his urine in the form of blood. The first sign of this muscle breakdown for him is 'hurty legs'. Usually starting with his lower legs then moving up and eventually all over his body his muscles ache and become weak. He says that sometimes it hurts to talk and to breathe.

To keep Adam from getting in to this state, he has frequent meals and lots of fluids during the day and 3T of Cornstarch in his late night beverage. Cornstarch takes longer to break down than most carbohydrates and allows him to go all night without a meal. He can metabolize Medium Chain fat, which unfortunately isn't in our regular foods. So as a supplement he has MCT Oil, medium chain fat added to his fat free beverages. If Adam were to eat too much fat all the time, gradually he would have fat stuck in his muscle cells infiltrating and weakening them. Gradually this would clog up even his heart muscle and he would die of heart failure. So besides the high carbohydrate diet supplemented with MCT Oil, he is on a very low fat diet ­ 20 grams of fat a day.

 

Diet

 

Adam's favorite foods are rice, particularly Sushi, pasta, and breads. He went from a very sick baby of 9 pounds at 5 months to a rather chubby very healthy-looking 9-year-old off the growrth chart in weight. Adam drinks three 6oz bottles of fat free milk a day supplemented with 3T of MCT Oil in each bottle and 3T of cornstarch in his late night bottle.

Unlike many LCHAD children Adam doesn't have a G Tube so when he doesn't want to eat, we must try to force feed him or make feeding (actually drinking) as easy as possible so he still is used to drinking out of a baby bottle even when he's asleep.

A big recommendation!!! That your child has a G Tube so you can push in the fluids when they are feeling sick or vomiting. We would have been able to prevent MANY trips to the hospital if we could have fed Adam by way of a G Tube.

Food Ideas :
  • Bagels
  • Crackers
  • Grahm Crackers
  • Toast
  • Bread Sticks
  • Pretzels
  • Granola bars
  • Rice
  • Pasta:sauce on the side
  • Marshmellows
Low fat or fat free foods:
  • Healthy Choice Foods:
    • cookies
    • frozen dinners
    • lunchmeats
    • cheese
  • Fat free cheese slices
  • Cereals
  • frozen waffles
  • Pop Tarts
  • Snackwell snacks
We always try a new low fat or fat free food when we see one but most of the time Adam doesn't like it. Of course all fruits and vegetables if you can get your child to eat them

.

From Fast Food Places: Burger King and McDonalds -Grilled Chicken sandwiches without mayonnaise. Pizza, we let him have a little but he takes off the cheese. When they still want a 'Happy Meal' or a 'Kid's Meal' get them a Hamburger without the meat.

Drinks :
  • Fat free milk
  • Capri Suns
  • Kerns
  • Any fruit juices
  • Soft drinks sometimes without caffeine but with sugar
  • Smoothies- especially Chocolate ones from Jamba Juice

We always try to limit Adam's snacks to no more than 6 grams of fat