Help! Carb levels

[Guest guest]

I feel like I am close to my breaking point with the doctors. Hannah is now 7

months old and has been officially on the ketogenic diet for 12 days. She is

currently at a 4:1 ratio and only spilling trace ketones. Sometimes, in the

evening, she may go up to small/moderate, but this is rare. I say " officially "

because since birth, Hannah has basically been on a 2:1 ratio due to her

inability to metabolize carbohydrates.

Hannah was born Jan 9th and stayed in NICU until February 11. They originally

thought she had PDh but the skin biopsy came back negative and they are now

thinking of ruling that out, but of course they are unsure. She has been

hospitalized a number of times and we are currently waiting for additional

results from her muscle biopsy. This is what we know so far from the muscle

biopsy:

Here's what we have so far: no mutations in the mitochondrial DNA were found.

This does not rule out a mitochondrial disorder because they can also be caused

by nuclear DNA mutations. The assay of mitochondrial enzymes (oxidative

phosphorylation) did not point to a specific disorder either. We are still

awaiting the immunohistochemistry and electron microscopy, where the structure

of the mitochondria and some of its proteins can be examined

When Hannah had her muscle biopsy, she had an adverse reaction to the

anesthesia. In post op, she had to have an emergency intubation tube and was

hospitalized overnight in ICU. They said her body's reaction to the anesthesia

was indicative of a mito disease.

I have been patient waiting for answers, but my patience is wearing thin. I

understand we may never get an answer, but today, that just isn't good enough

for me. I spoke with Hannah's dietician this morning and suggested her ratio be

changed to 4.25-4.5:1 instead of 4:1. My rational is that Hannah's body is used

to metabolizing a 2:1 ratio and may need a higher ratio in order to go into

ketosis. My dietician is hesitant. The dietician says Hannah's brain needs

carbohydrates in order to grow. Since Hannah's body reacts to carbs, this is a

problem. It appears Hannah's lactic acid levels increase to a point of dangerous

acidosis when she has carbs. The dietician thinks Hannah is too young to go into

ketosis.

I know there are some kiddos out there on a higher ratio, what have your

doctor's said about the carb issues? Hannah is currently at 4:1, 10.95g protein,

2.19g carbohydrates. She takes 4cc L-Carnitine, a multivitamin supplement, 30mg

CQ10 and 700mg thiamine.

Please help,