I have been helping a mother in the NICU whose baby was born in the 35th
week  with Netherton Syndrome,  a erythematous, cornification skin disorder
( see picture in web site below). ( I was the one who made the diagnosis.
Every expert they brought in made a different diagnosis. I remembered a baby
with this syndrome from when I worked with  pediatric dermatologist, and
offered the younger docs material on this syndrome.  The next time I spoke
to the mom and asked what the final diagnosis was, sure enough it was
Netherton!)
I had originally been helping her with pumping, and assisted in the first
breastfeed . Milk transfer was unusually exceptional in this baby and mom's
motivation was very definite.
Yesterday I met the mom  in tears in the hallway.  The baby is FTT ( a
common feature of this disease) had had a second NEC episode, had been NPO
and was now off breast milk and on special formula.  I was speechless!!  All
I could do was hug her.
Luckily mom is still pumping a few times a day gradually supressing
lactation.
( I am already imaging bathing the baby in the milk she pumped!!)
Has anyone worked with a baby with Netherton?
I am dying to find something in the literature citing importance of breast
milk for these abies, but of course the most extensive search came up with
zilch.  My heart is broken for this mom.
I didn't get a chance to speak to the head of the NICU yesterday, but I
would prefer to come with material, even if it is anecdotal.
http://matweb.hcuge
ch/Selected_images/Developmental_genetic_diseases/Netherton_disease.htm

Esther Grunis,IBCLC
Lis Maternity Hospital
Tel Aviv, Israel

             ***********************************************
The LACTNET mailing list is powered by L-Soft's renowned
LISTSERV(R) list management software together with L-Soft's LSMTP(TM)
mailer for lightning fast mail delivery. For more information, go to:
http://www.lsoft.com/LISTSERV-powered.html