Asking advice from experts in feeding to manage metabolic problems.
Baby boy born 01 Sep 2007, currently weighs 6.6Kg, exclusive or primarily BFing, was admitted on 07 Feb 2008, for RSV, lethargy, dehydration, some mention of spitting up. Mom is age 15 yrs and a champion breastfeeder.
Don't have all details, but MD ordered a genetics consult and a nutrition consult and stated an additional diagnosis of isovaleric acidemia resulting from leucine catabolism. The feeding order is for I-Valex-1 formula by Ross, 4oz every 3 hours. They are allowing ad lib breastfeeding in addition to the formula. An Internet search showed this formula retails for $85.00 per 400gm can of powder.
Baby bats away bottles, either from distaste for the formula, or for the bottle itself, and is being fed the special formula via NG tube. The RN tried to bottle feed this special formula when mom was gone home to shower and change clothes. It was the RNs first experience with a baby who has no clue what a nursing bottle is, and fights having it pushed into his mouth. By the time mom returned, the NG tube was inserted.
He has recovered from his RSV and other problems. They called me in to see mom today, 5 days after admission. Inintial request was to teach mother how to use breast pump, but that quickly changed to finding a way to get baby to consume the specialized formula. They don't want to send him home with the NG tube.
Tried the #5 french tube in a bottle, but baby didn't pull the formula out. So we tried a Starter SNS. Baby got 10 ml of the formula, but he was constantly popping on and off the breast to look at me. He is an active, chunky "baby linebacker", smiles easily, all whirling arms and legs, and focused more on Bfing when I left the room, or I at least got out of his range of vision. We had to hide the SNS under mom's shirt as baby's grabbing hands made using it difficult. But the fact that he did take some of it was encouraging.
My question to the MD (and now to Lactnet) was related to the volume of the I-Valex-1 the nutritionist calculated for this baby to take every 24 hours. Is that to supply all his nutritional needs, as a substitute for regular formula? Or is it the required amount to manage or control this baby's metabolic disorder? It seems to me that 32oz in 24 hours will totally replace breastfeeding - and he is Bfing marathon-style while in the hospital.
Followup question: is it possible that a lesser amount of I-Valex-1 can manage or control baby's metabolic problem? There is no mention in the medical chart of limiting his intake of breast milk or of limiting breastfeeding. It is his security blanket during this hospitalization.
There are a few emails in the Archives about this disorder. A couple mention a maximum limit of safe leucine intake. The 1999 edition of Lawrence says that mature breast milk has 110mg of leucine per 100ml. I don't know how that compares to safe limits for a baby with "isovaleric acidemia, a disorder of leucine catabolism".
Any other questions I should be asking?
What should I be doing or not doing to help this babe and mom? Breastfeeding is central to their relationship and up to now, baby's only, or primary source of food.
By the way: he is also teething, and has been for 2 mos.
Appreciate your help.
Phyllis
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Phyllis Adamson, IBCLC, RLC
Glendale, AZ.
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