Hello wise ones,

I need some creative thinking here for a bad situation with my granddaughter. She is 11 days old 
and diagnosed with citrullinemia, a rare urea cycle disorder, which means her liver cannot process 
proteins. Too much protein causes excessive buildup of ammonia, which was what happened.   
According to the geneticist, she is the only child In Idaho who has ever had this disorder and lived. 
They think that before the routine screening for it,  most who have had it  just died and it was 
ruled as SIDS. Thank goodness for routine screening now in Idaho.

She had been nursing effectively for the first five days when Kara, my dil, became concerned with 
her lethargy and sleepiness. Almost simultaneously, her doctor received the lab report from 
routine newborn screening. So, she was immediately hospitalized, taken off the breast and given a 
special formula. Her ammonia levels have dropped sufficiently but here's the problem.  They mix 
expressed breast milk with the special formula and have tried to give it to her in a bottle.  The 
mixture, after much trial and error is now working.  They want her to have 2 ounces every 3 hours. 
However she will only take 1 ounce and refuses any more. Then they give the other ounce through 
a tube. Until she will take all the formula from a bottle, they will keep the tube in place. The 
doctors seem to think it's the flavor and they are trying to rework that issue. I think she just gets 
full with the 1 ounce and would take the other ounce in an hour or so. 

The baby roots every time she's around Kara, and Kara wants to nurse so badly, it's pitiful. Has 
anyone here ever worked with this problem or any similar situations? Do you have any ideas? I'm 
so in the middle of it, helping take care of the other 3 children and  3000 miles from home that I 
can't think straight.  Would hooking up an SNS and using it with a nipple shield that has the the 
holes blocked work?   How about finger feeding? Could she use the SNS after pumping since the 
breast would be emptier? Any ideas or suggestions would be so greatly appreciated.

This is pretty cruel irony for our family or breastfeeding fanatics, but as my daughter, Joanna, 
keeps reminding me, THAT'S why formula was invented - to help babies who could not nurse in 
rare circumstances. 

Reply to Lactnet or to me personally at  [log in to unmask] . Thank YOU! 

Pam Wiggins, IBCLC
www.breastfeedingbooks.com

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