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Isovaleric Acidemia

My pregnancy and delivery with Sam were normal. After he was born in 1993, he started to vomit, and it became progressively more over his first few weeks and was projectile. He had some outpatient tests for GI disorders, and I was advised to try several different formulas. Nothing worked, and at 4 weeks of age he was still at his birth weight, and he was admitted to the hospital, diagnosed with a urinary tract infection.

He was treated for the urinary tract infection, but the doctors quickly realized that there was something else underlying wrong, as he was not getting better, and was termed as “failure to thrive”. He was tested for everything imaginable, and finally after 3 weeks in the hospital, he was diagnosed with IVA. He did not have the classic symptoms, never went into a coma, and did not have the “sweaty feet” smell.

He immediately went on a combination of Prophree formula and Similac, along with glycine and carnitine, and started to grow. He did very well, right away. He only had 2 incidents in the hospital after that time-both flu related from vomiting and dehydration, once at about a year, and the other time about 2.5 years, with one night in the hospital. Both times, after going on an IV, he quickly turned around, and was able to keep fluids down.

At the age of one, he started on I-Valex formula, which he remained on until about 5 years old. We then switched to Xleu Maxamaid. He wasn’t having any problems on the I-Valex, but I couldn’t get him off of the bottle. He knew he was too old for a bottle, so wouldn’t drink it in front of anyone but family, but that was still the only way he would drink his formula. The Xleu is more like a juice (it’s compared to Tang, whereas I-Valex looks more like milk), and with the switch, I told him we were throwing out his bottles, and luckily it worked. We mix it with Gatorade, and his compliance is good with it, which I hope continues as he gets older.

He has serious food issues. There are many foods he can have that he won’t touch. He only eats carbs, and is extremely particular with all aspects of food, including preparation, look, etc… He is very hesitant to try anything new. He won’t touch any fruits or vegetables, and lives primarily on pizza (cheese removed), pasta, French fries, cereal, crackers, bread, mac and cheese (light cheese), and chips. I hope he will expand his diet as he gets older, but he truly has some major food aversions. I worry health wise about his unbalanced diet, and also socially, because all social functions revolve around eating, and I don’t want him to feel that he doesn’t want to participate in things because he doesn’t eat much. As a kid, it really doesn’t affect him, because all kids like pizza, and French fries! He sees me eating a lot of salad and fruit, but unfortunately it doesn’t interest him!

He is currently in the 9th grade, does very well in school, and has a quick wit. He is a good athlete, with a passion for baseball.

Lisa, Mother to Sam 15yrs old, IVA

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