I just saw how my reply looks. Sorry. I'll try to be more helpful. 

These kids have a several defects and vary greatly on their presentation. This is what I usually see cause the most problems:
Heart defects - causing the usual very high needs of CHD and poor intake. The MD may be trying to just increase the calories. There are obviously better way of doing this than stopping breastfeeding all together.
Liver defects causing fat malabsorption and very high needs (again). Depending on how bad this is, the baby may need MCT oil or Pregestimil (sorry, formula may be needed for this baby) ADDED to EBM. 

The patient's need vary a lot, and what we do with them has to match that. If no fat malabsorption and great volume, do hind milk. If the MD is very strict he/she may want a creamatocrit (if he/she has ever heard of it) to know exactly how many calories the baby is getting. Depending on the liver size/growth/albumin levels, wt gain may not be terribly reliable. 
Honestly, they usually need EBM + Pregestimil to make milk that is ~27 kcal/oz or more. We also give them MCT boluses if we have to. 

Hopefully not too generic or confusing. 

Daniela Torres Marco MS, RD. 
Pediatric Clinical Dietitian 
Texas Children's Hospital 
6221 Fannin St MC-2-3521 
Houston, TX 77030 
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This information is privileged and confidential pursuant to Texas Health & Safety Code sections 161.031 - 161.033 and Texas Occupations Code section 160.007 and or T.R.C.P. 192.5.



-----Original Message-----
From: Lactation Information and Discussion
[mailto:[log in to unmask]]On Behalf Of tibs
Sent: Friday, June 09, 2006 10:35 AM
To: [log in to unmask]
Subject: [LACTNET] Alagille Syndrome


I am in touch with a mother whose baby has just been diagnosed with this syndrome and she has been advised to stop breastfeeding- has anyone come across this?
The statistics are 1 in 100,000 !!

Congratulations also to the ladies who started and manage Lactnet on receiving the Innovation Award.

Marilyn Stollmeyer
Trinidad-the smallest island to play in the World Cup
West Indies

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