Tuesdays update

Babies are about 35.5 weeks adjusted age...

Nora is doing well.  She's up to full feeds.  She drinks about 4 of her 8 bottles totally.  The others, she gets tuckered out after 1/2 of the bottle and they put the rest down her n-g tube.  She is nursing well.  Takes about 2/3 of a feedings-worth by nursing a couple times a day (we weigh her before and after to see what she took) and then, they'll tube the rest.   So, now her goal is to get to taking all 8 bottles each day without needing the n-g tube.  She needs to be able to hold her temp (she can already) and be gaining weight appropriately and she can go home.   The docs won't give us much indication of a timeline for her coming home, but the nurses say, best case scenario is within 1-2 weeks.  She is off all IVs. 

Ryan--back on the TPN as we expected.  He started "dumping" at 10mL/hr of formula (basically putting out too much stool), so they pulled him back to 6mL/hr and restarted his TPN.  Now he's up to 7mL/hr again and will stay here for the week and try to increase back to 9-10mL/hr now a bit more slowly.  His bilirubin is up even further (16.6)--he is still acting OK, but still seems weak.  minimal weight gain, but I guess we should be happy it's a gain.  good news is that he was able to be off the TPN, even if only for 1 day.

Dr T is the attending pediatric surgeon this week and he is also one of the authorities on short bowel syndrome in the country, so we feel blessed to be under his care.  He will be taking care of Ryan in the Intestinal Rehab clinic once he is discharged, so this is the beginning of good continuity of his care.  So, all of Ryan's issues are intertwined, but I'll list them separately in hopes that it makes some sort of sense. 

(Editor's Note (from Daddy): If you don't want to read the technical information below it can be summed up as follows:  Ryan's Billirubin = bad--but maybe not b/c of TPN, his weight gain is a concern, and  Omegaven = maybe, but we need to address other things first)

Bilirubin:  Dr T is very convinced something else is going on. Ryan's rate of bilirubin increase is not typical of TPN-associated cholestasis.  Ryan's bilirubin has gone up very high very quickly compared to what they'd expect if it was due to the TPN.  They did an ultrasound of his gallbladder to see if there's sludge--we didn't get the result, but that could explain the cholestasis and elevated bilirubin.  There are some meds he could get if this was going on.  Alternatively, it could be due to a low grade infection.  They did blood cultures last week which were negative, but he had some other signs of possible infection (acting not quite right, "dumping," elevated white blood cell count) over the past week--so they decided to give him a round of antibiotics for the next 10 days.  we'll see if the bili starts trending down soon.

Weight gain (or lack thereof): background--one of the ways they try to protect kids with SBS's livers is to restrict the amount of TPN they get, specifically the fat component--meanwhile they lose some calories they'd otherwise get--so they are watching this closely.  They are willing to sacrifice some weight gain to spare his liver for the longterm.

Omegaven: long talk with Dr. T today.  he is very gracious and informative.  we are convinced he is concerned about Ryan.  Thankfully, TPN-associated cholestasis is one of his areas of research, and he knows a lot about Omegaven as well as other strategies to protect kids on longterm TPN.  John was a superstar: after reading medical literature for 3 hours last night and watching 2 hours worth of a symposium on SBS where Dr. T was one of the main lecturers, we felt prepared and asked some tough questions about Ryan's options, and John did not back down, finally asking "what would you do if this was your kid?"   Dr. T agreed it was worthy of looking into Omegaven and he'd be willing to fill out a special request to the FDA if we felt strongly about it.  kind of a lot of pressure on us, as Dr. T is concerned about essential fatty acid deficiency if we switch his current lipid/fat infusion to the omegaven (they have different fat components in different concentrations), basically b/c it's not FDA-approved and they don't have a huge base of longterm data at this point.  In our own research, we found a few clinics that use Omegaven and none have reported essential fatty acid deficiency when they publish their data--in the end, Dr. T said we'd revisit it in 2 weeks. (first we need to see Ryan's bili come down if it's truly elevated due to infection or a gall bladder issue)--so, stay tuned.  we are praying for discernment for us and for the physicians.

Sorry for all the technicalities--just trying to give specifics so you know what to pray for.

Otherwise, we're told at least another 4 weeks before Ryan's "reattachment" surgery.

OK, enough for now.  if you made it this far, thanks.