Why might you need a transplant?
Indications for the intestinal transplant have been typically short gut kids that get TPN disease. Sometimes those kids get liver-intestine transplant; sometimes, if they have difficult anatomy, we do multiviscerals for those too. As we are getting better at managing short guts, there have been less indications to do multivisceral. This year we have done two, and they’ve both been tumors.
Other indications for the intestinal transplant program would be congenital defects in absorption. Because this is a referral center for many parts of the world, we see rare diseases. We see things like microvillous inclusion disease, which basically causes a functional short gut; the gut is in but it doesn’t function, and they can get repeated episodes of dehydration and neurological sequelae from that.
Basically, most of the indications are short guts or tumor, and the short gut can be a physical short gut or a functional short gut.
Rima Fawaz, MD, Medical Director, Multivisceral Transplant Program
Most of our patients are babies who can’t grow from enteral feeds (formula by G-tube or by mouth), so they need parenteral nutrition. But early in infancy, if you’re on parenteral nutrition, it does damage to your liver. For example, a baby with necrotizing enterocolitis needs parenteral nutrition in order for them to grow, but the parenteral nutrition very early on in life can be damaging to the liver, and that’s why we need to list them for a liver transplant too.
For a multivisceral transplant, they have to have been deemed to have intestinal failure. One of the other indications is if they’ve lost vascular access; they may not actually have met criteria to meet intestinal failure, but their access for central lines has diminished. Our patients need central access for PN, but veins can thrombose [develop a clot], and if you are only left with one vein, that is an indication that we need to go to the next step for transplant, because that’s their last life-saving line.
Another indication is an invasive hepatoblastoma that encases all of the mesenteric arteries and veins. Those are our top three – basically, failing parenteral nutrition with liver disease and growth failure.
Courtney Fratto, RN, MSN, CPNP, Transplant Coordinator, Multivisceral and Liver Transplant Programs
What does a multivisceral transplant involve? What is unique about this patient population?
There’s a multivisceral transplant, which is the stomach, liver, intestine, spleen, and pancreas; there’s a modified multivisceral transplant where you don’t get the stomach, you get everything without the stomach; and then there’s the isolated intestine transplant, which is just a small bowel.
Kids with multivisceral transplants are a little bit more complex in that they have a lot of different services that follow them. Rather than a liver transplant patient who just has the liver team, we usually have five or six other services in the hospital that are also following these patients. The good thing here is that we’re such a small group of transplanted patients that my role has been taking on the different consulting services: they all go through me so that the patient only hears my voice and they’re not getting ten different providers calling them in one day. We’ve tried to condense it down where everything filters through the transplant team and then to the patient.
Courtney Fratto, RN, MSN, CPNP, Transplant Coordinator, Multivisceral and Liver Transplant Programs
