Lurie Children's Hospital nurse contacted us wondering if we still would like Patrick to be evaluated for the Rex Shunt. The doctor in Chicago is the Director of Transplant Surgery and is one of a few pediatric surgeons to perform this surgery. He has performed over 150 surgeries. The rex shunt could restore the blood flow back to Patrick's liver. It, by no means, is an easy fix or easy cure....we would have a really extensive pre-op work-up. We made it past the first round of candidacy. I gave them the latest update with Patrick's last varice bandings and am hoping this will not slow down the process...in some cases they want to have varices that are controlled...not sure they think these are controlled since he has had them banded every 2 months since October.
The preop would be CT of his abdomen, labs, and a venogram. I don't think he will have to have a liver biopsy but that had been done over a year ago. The venogram goes through his jugular vein and takes a good look at his veins. After this is all done they usually schedule the surgery. The surgery lasts about 6-8 hours and they graft a part of the jugular vein and sew to the portal vein (liver) and the vein in his abdomen. Most children stay in ICU for a few days and then transfer to a floor...we would be in the hospital for about a week and would need to remain in the Chicago area for 3-4 weeks after surgery. Patrick would have a 3" scar on his neck and a scar on his stomach that looks like the MercedesBenz logo.
Ideally, the shunt restores the blood flow to the liver. This doesn't necessarily "eliminate the need for a future liver transplant but can postpone it indefinitely."
There are some other surgeries that could divert the blood from his liver rather than restoring the normal blood flow through the liver. Patrick's little miracle body has created these collateral vessels...but the blood flow does not go through the liver...which what I seem to understand could cause some scarring in his liver which in the long term could harm the organ. After our evaluation with the liver doctors at NIH, the doctor there said if we do not work at fixing the problem we are risking his blood clotting (doesn't help with Factor5), growth and cognitive functioning.
We would follow up with office visits, labs, and radiology exams every three months for a year, then every 6 months for two years, then annually.
The surgery risks include: bleeding, infection, organ failure (need to check on this one as just heard this from another parent whose surgeon mentioned to them), failure of shunt, non-reversible. The shunt can sometimes narrow and there is also the possibility of thrombosis (clotting) but this is why we would be monitored quite often.
The risks of not doing surgery: repeated EGDs and bandings, bleeding of varices, internal bleeding, no cure, esophagus issues.
I think the NIH doctor best summed it up for me, "I think you should go up to Chicago to be evaluated. Once he has been evaluated for a clotting disorder, it would be reasonable to proceed with surgical intervention to avoid long term complications. We touched on it when we met, but long term portal hypertension can cause problems with growth and cognitive development. To prevent life threatening bleeding, endoscopy with banding is a good option, but each procedure carries risk, and while the individual risk of endoscopy versus surgery is low, the cumulative risk may exceed that of surgery."
The preop would be CT of his abdomen, labs, and a venogram. I don't think he will have to have a liver biopsy but that had been done over a year ago. The venogram goes through his jugular vein and takes a good look at his veins. After this is all done they usually schedule the surgery. The surgery lasts about 6-8 hours and they graft a part of the jugular vein and sew to the portal vein (liver) and the vein in his abdomen. Most children stay in ICU for a few days and then transfer to a floor...we would be in the hospital for about a week and would need to remain in the Chicago area for 3-4 weeks after surgery. Patrick would have a 3" scar on his neck and a scar on his stomach that looks like the MercedesBenz logo.
Ideally, the shunt restores the blood flow to the liver. This doesn't necessarily "eliminate the need for a future liver transplant but can postpone it indefinitely."
There are some other surgeries that could divert the blood from his liver rather than restoring the normal blood flow through the liver. Patrick's little miracle body has created these collateral vessels...but the blood flow does not go through the liver...which what I seem to understand could cause some scarring in his liver which in the long term could harm the organ. After our evaluation with the liver doctors at NIH, the doctor there said if we do not work at fixing the problem we are risking his blood clotting (doesn't help with Factor5), growth and cognitive functioning.
We would follow up with office visits, labs, and radiology exams every three months for a year, then every 6 months for two years, then annually.
The surgery risks include: bleeding, infection, organ failure (need to check on this one as just heard this from another parent whose surgeon mentioned to them), failure of shunt, non-reversible. The shunt can sometimes narrow and there is also the possibility of thrombosis (clotting) but this is why we would be monitored quite often.
The risks of not doing surgery: repeated EGDs and bandings, bleeding of varices, internal bleeding, no cure, esophagus issues.
I think the NIH doctor best summed it up for me, "I think you should go up to Chicago to be evaluated. Once he has been evaluated for a clotting disorder, it would be reasonable to proceed with surgical intervention to avoid long term complications. We touched on it when we met, but long term portal hypertension can cause problems with growth and cognitive development. To prevent life threatening bleeding, endoscopy with banding is a good option, but each procedure carries risk, and while the individual risk of endoscopy versus surgery is low, the cumulative risk may exceed that of surgery."
I never thought we would get to this point...it seems like so long we have been told to wait and see what happens but now this is becoming a reality. The nurse said it is a big commitment but I feel we owe it to Patrick to get him the best quality of life for a little boy his age.
I am so glad you started this blog to keep track of everything each doctor and specialist has told you. I had no idea Patrick's case was so complicated or rare. I really hope this Rex shunt is the answer to your prayers!
ReplyDeleteThank you Jess!!
Delete