The first full day "on the floor" at 10 East went very well. Blaise had a good night, weight gain continues to be good, and she's even hanging in there at higher levels of enteral feeding (2ml/hr) and oral feeding (7ml every 4 hrs). We've both been pretty happy with the nurses we've met so far and the floor that Blaise is on is where all of the patients with short-bowel syndrome are treated. This means that her nurses are bound to be very familiar with the nature of Blaise's care, probably even more so than the nurses in the NICU.
We also had a visit today from Dr. Mark Puder, who is an extremely important person in the world of short-bowel syndrome. Currently, Dr. Puder is carrying out a randomized clinical trial of a product called Omegaven, which many parents credit with saving their child's life.
The deal with Omegaven is basically this: Blaise still gets almost all of her actual calories and nutrients through an IV. Feeding infants this way is called "Total Parenteral Nutrition" or TPN, for short. It's a way of assuring that a newborn gets all the right stuff delivered into the bloodstream, but there's a catch. If traditional lipids (fats) are being given via TPN they inevitably cause liver damage. Fatty deposits build up in the liver, the lipids also cause an inflammatory response, and liver tissue begins to fail. Ultimately, an infant who has been on long-term TPN will become severely jaundiced due to the excess of a substance called bilirubin, and suffer from advanced liver failure. Bilirubin is a compound associated with the breakdown of hemoglobin in the body and is responsible for the yellow color you see in bruises. A healthy liver can deal with bilirubin. An unhealthy one can't, and so bilirubin levels are monitored very closely in kids that have to stay on TPN. Even just a few years ago, the need to provide nutrients by IV coupled with the knowledge that doing so caused liver damage was a pure Catch-22. The prognosis then for a baby like Blaise would've depended solely on how long we could manage to balance TPN with liver failure while she waited for a double intestine/liver transplant. Needless to say, the overall outlook then was not very good.
So what's different about Omegaven? It's a lipid that can be provided to an infant on TPN in lieu of traditional fats that's made from fish oil rather than plant-based lipids. Most importantly, it appears to not only prevent liver damage from occuring, but even reverse existing damage to the organ. I can't emphasize enough how dramatic the results from Children's Hospital Boston are: While the full study hasn't been completed yet, there are many, many patients who have recovered from severe liver failure after being put on Omegaven. Given the striking success of this treatment, children with short-bowel (and I imagine other kids with TPN-related liver problems) are given Omegaven at Children's Hospital Boston due to conditions of "compassionate use." Basically, this means that once you know that a treatment works, it's unethical to withhold its use from a patient that needs it. The FDA essentially gives approval for individual kids who need it while full approval is pending, and kids like Blaise get the care that will save their lives.
Today, we were told by Dr. Puder that Blaise's bilirubin levels were nearing 2.0 (twice normal). This is their cut-off for administering Omegaven to children on TPN, and he just wanted us to know that he had his eye on her test results and was ready to get her on board as soon as she crossed that threshold. I should point out that 2.0 is actually fairly mild as the numbers go...kids with levels of 14.6, 25, and even 60 have all been treated with Omegaven and thrived. In most cases, those kids only got that sick because it was too difficult to get Omegaven administered. Being at Children's in Boston makes an immense difference in this regard. We're literally in the place where all the work is being done, so Blaise's care is not in any doubt. In this, we're incredibly lucky.
Reading back over what I've written already, I realize it must sound a little nuts. Omegaven sounds like some kind of "miracle drug" or "health tonic" that somehow fixes everything. It's rare in medical science (or any kind of science, for that matter) to come across something that unambiguous. Still, it's hard to argue with the number of children that have been helped. We're both keeping in mind that this is still a treatment, not a magic pill that's guaranteed to work. That said, we're confident that it has the potential to do a lot of good.
OK...enough medicine. There's more pictures in the gallery! We've taken several photos of her new room for everybody to see, and there's some new photos of Blaise with even fewer tubes and lines. She's definitely filling out a bit more these days, which has made the chubby cheeks even more adorable.