An actual case report regarding the Rex Shunt Procedure (adults)
In the last decade, the Rex Shunt procedure has been successfully performed on children with portal vein thrombosis, however to the best of my knowledge has not been performed on adults with the exception of one person which I found had the operation in Niigata, Japan. The following article (click link below) was posted by a group of Japanese doctor’s who had successfully performed the Rex Shunt Procedure on an adult male a few year’s ago. Although the article is technical in nature, nonetheless I hope you are able to glean some good information from it?
It is interesting to note that this group of Japanese doctor’s are recommending the Rex shunt procedure be performed on a larger scale for adults with portal vein thrombosis.
http://www.springerlink.com/content/e53875128l97v008/fulltext.html
This article begs the question of whether or not there is general consensus amongst gastroentrologists and other specialists that this procedure is even a viable option for patients with PVT. Cost factor’s and risks versus gains would likely weigh into the equation also? This being said I would really appreciate input from anyone, especially doctor’s as the report above and a recent statement (see below) from Dr. Riccardo Superina (Children’s Memorial Hospital Chicago, IL) support the procedure be performed on adults.
“There is no reason that it cannot be applied to adults, and we would be happy …to hear their cases if they wished to send case summaries along and recommend further investigation or action.”
{ 21 comments… read them below or add one }
This is great news! So, I think I might make a few enquiries with Dr Superina and find out if he would be willing to operate on me!
It may be worth a try to contact Dr. Superina as you have youth on your side? Don’t know what funding is available for you but you may want to investigate that also. Wouldn’t be great if this was to be?
Hey Clint,
E-mailed doc and hospital the other day offering to be an adult ginea pig for the adult option of rex shunt proceedure! Fingers crossed, will figure out the funds later if they say yes but regardless of the funds, anything they can suggest I would LOVE to have considered! Lots of prayers that they will say “yes”, and “come on over!”
Claire,
Wow Claire you are one brave girl! You have endured much in your life, and I think if I was in your position I believe I would be doing the same thing!
Please keep me in the loop as soon as you hear of anything? Praying for sure!
HI CLint
I have spoken with Dr. Jean Emond in NewYork he does both Children and Adult Rex Shunts. He is so kind.
His number is 212-305-9691
Thanks Jen
Good to talk to you today in person! Thanks for the information on Dr. Emond – I will try to contact him when I get a free moment.
Hi!
my name is Kristi, i was diagnosed with portal hypertension when I was two years old ( this was back in 1991) … I was approved for the surgery for a Rex Shunt when I was fifteen, but was not able to have the procedure due to the fact that my portal vein had atrophied. However, I did have a spenal-renal shunt, which worked just as well. So if you aren’t able to get the Rex Shunt, ask about the Spenal Renal shunt,( either way I believe the blood is diverted from your hepatic portal vein and the pressure is relieved,
O I also had portal vein thrombosis. And the procedure was done by Dr. Surperina, who I found to be a really nice guy.
Hi Kristi,
Thank you for taking the time to post your experience on this blog. You also have had quite a journey and this is valuable information to others who may be experiencing the same things. From what I have learned lately there seems to be a consensus among doctors that the best candidates for the Rex Shunt are children under the age of 5 as they have not experienced vascular atrophy to a large degree. Yes, the Splenel Renal shunt is another good option that possibly may work, however unfortunately it is not one for me as doctor’s do not want to “open me up” for any reason due to the fact I have to be so highly anti-coagulated. There are other complications that can play into whether or not any kind of operation can be performed for portal hypertension, but I always encourage others not to give up the fight. Thanks again for your insight!
I was diagnosed with having a large spleen when I was born. It wasn’t until 5 years ago, when I was 19 that I learned I had esophageal varicies and portal hypertension. I’ve been fortunate enough to never experience any major complications. I was seen at the Mayo Clinic a few years ago, but they said there isn’t really anything they can do for me at this time, that it is best to wait to do any procedures until I have complications.
I’ve seen Dr. Superina’s named mentioned quite a few times on here. He actually contacted my doctor to ask if I would be willing to do the Rex shunt operation as an experiment on an adult. Unlike the other lady, I denied the surgery. Everything was so new to me at the time I wasn’t ready for any other risks.
I feel that I am fortunate. Yes, this can be a terrible thing to live with. I’m 24, my varices are large, I’ve been told by multiple doctors that they will bleed at some point in my life and more than likely, the outcome won’t be good. But in my eyes, it could be worse. I’m not in any kind of pain, I look healthy and I’m still able to enjoy my life. I pray for everyone out there who is also living with this.
Hi Stephanie,
Thanks for your post.
Like you I have been told I will likely bleed at some point, but I have managed to stave that off for the most part – albeit for a bleed I had in the varices in my stomach back in 2004. Coincidentially, I was contacted by Dr. Superina’s nurse last week regarding a Mom whose little girl may be a candidate for the Rex Shunt. “Amy” Dr.Superina’s nurse said that I should considering sending my imaging down to the hospital for him to review and this would be for another rare procedure called the Sugiura procedure. I am not getting my hopes up too high as I have an underlying clotting condition which requires heavy amounts of anti-coagulants to manage, and my doctor’s do not really want do anything because of the high risk to bleed or clot again. I am 50 and have lived with Portal Hypertension now for 9 year’s now with a few setbacks along the way, but for the most part I remain optimistic.
It looks like you have covered your bases well by consulting at the Mayo clinic. One never wants others to give up finding answers (I won’t), but when you realize like we have that there really is no other option for us except to carry on with the prescriptions we take and to remain upbeat – that is the best thing to do at this point. I feel doctor’s have done all they can for me also – I am relatively healthy and am also able to enjoy my life. “Yes” I do also pray for those who are also living with this, and in particular the “little one’s” whose hope may be greater than ours because of the Rex Shunt and a few other procedures which may help in the long run. Keep pressing on Stephanie!!!
Hi my son is 2 and has PVT we are in Australia and i cant seem to find anyone here who does this procedure. I am very interested in contacting Dr Superina to find out if my son would be a good candidate. If we can have fix this to avoid the complications that can follow (ive seen bleeding varacies in person as im a nurse myself) i would pay any amount of money even if we had to sell our house. If anyone has his contact number please let me know. Thanks Clare
Hi Clare,
Sorry to hear about your little guy! Hope we can help you by offering support at the very least. Click this link for a list of specialists who have experience with the Rex Shunt Procedure: http://www.portal-hypertension.com/2011/08/28/rex-shunt-for-sonya-update-08-28-11/
Dr. Superina just operated on a little girl from the Ukraine yesterday who I have posted information on. I know Dr. Superina will preview your diagnostic tests as he has done for others around the world and he will make recommendations after that information is received. You can call the Childrens Memorial Hospital in Chicago and they will be happy to get you started. As far as I know there is no one in Australia doing the Rex Shunt, but I know the Birmingham Childrens Hospital in the UK has some experience with the procedure also. They are both listed on the link.
Keep me posted,
Clint
Hi Clare,
Sorry I copied the wrong link in my previous message. Here is the list of specialists: http://www.portal-hypertension.com/2011/03/17/rex-shunt-procedure-specialist-list/
Clint
Clare – it sounds like you were able to connect with Dr. Superina and Shelly. Hope things come together for you soon.
Clint
I just found this site and I’m so glad I did. I would try ANYTHING even if the chance of success is 1%. I’m so desperately sick. I need some tiny speck of hope.
Hi Jerry,
I am glad you found this site also, but am very sorry to hear of your illness.
My goal is to offer support to others going through this dreaded condition – even if only to offer a speck. Most people on here are looking for answers and hope, if not for themselves but for their own family members. I have provided some pertinent information on Portal Hypertension and for the last few years I have had this blog running I myself have learned a great deal from the very people who have submitted entries. Please feel free to update us on your condition with any new developments for the treatment, and if you have any questions I will do my best to find an answer for you or possibly put you in touch with someone who might help you.
Keep fighting,
Clint
Hi Clint, I was reading your entire website again with everything that is going on and I wanted to add that I know of a young man who is 21 who had a successful Rex right around the time of my son’s Rex revision in September of 2011. Dr. Superina also performed this Rex and from the few follow up posts from his mother he seems to be doing well. Hopefully adult Rex’s may become more of a possibility in those who are good candidates. If nothing else I hope this gives some hope to others looking for options.
Once again, thanks for this site. It has been a wonderful source of information and support!
Kelly
Thank’s for relaying this story Kelly! This is important information for all looking for answers. In some ways I have been somewhat pessimistic about those who are older as the prospects for a positive outlook through medical procedures seems somewhat remote. With time and the advancement of medical science I know that the possibilities for “good” change become better each day. I am excited over what Dr. Superina has done and this especially is encouraging for those with young children. Not only has the Rex Shunt proven to be a life saver for many, but also other procedures, as rare as they are, such as the Sugiura Procedure seem to be making a difference. I think the key words to consider is what you have said above – “those who are good candidates”. We are blessed to be living in the times we now are in, where breakthroughs seem to be commonplace in medicine, and with that I want to say that many who may not be good candidates may eventually be brought to that place where considerations are made. Your note encourages not only myself but others to not give up, and to keep fighting (all the while to keep praying too).
Be blessed Kelly … I’m still praying … it’s about all I can do from this distance. I can’t even begin to imagine what you have been through this past week or so and “your son” and family have been on mind alot.
Peace,
Clint
Dear Clint,
You are a remarkable person and thank you for giving us all hope.
I am writing to you because I need some hope and a place to start looking for new treatment. My boyfriend Terry is 53 and has PVT. He has suffered one episode of bleeding varacies and is stable now for a couple years. He has liver cirrosis and due to the PVT, his liver he can’t process ammonia. He takes the usual drugs for this which includes lactulose. However, his quality of life is pretty poor due to the fact that he is either sitting on the potty from taking lactulose or suffering from such extreme fatigue due to the high ammonia levels. His stomatch hurts practically every time he eats a meal and he would rather sleep then do most anything else. We live in Atlanta and he sees the Emory docs. He has been told he is not a candidate for TIPS and never really asked about Rex Shunt. I am sure the Emory guys know what they are doing but they never seem to try to think outside the box and suggest anything new to improve his quality of life. There has to be something out there that would help him! We are willing to travel anywhere and talk to anyone. What can I do first? Where do I start? I would love to start with someone who is a pro-active like Dr. Supernia seems to be from reading these posts. How about Barcelona, do you know of anyone who has gone there? Help!
Sincerely,
Kristen
Hi Kristen,
Thank you for your post and kind comments. I am sorry about your boyfriend’s condition – I know it is hard for you to see him suffer like this, but I am sure he appreciates your understanding as it would be hard to go through something such as this without the support you provide.
I never want to give anyone false hope, but only provide for them the support and the possible means to contact someone who may review their case. My best guess is the reason that doctor’s told you your boyfriend is not a good candidate for the TIPS maybe because of the complication he has regarding cirrhosis of the liver. That being said I am not a doctor and sometimes it is good to get a second opinion. To my knowledge the Rex Shunt has only been attempted on a few adults, and the operation failed. The Rex Shunt works better on children because atrophy has not set into the arteries like is does in adults. Dr. Superina is renowned for his expertise regarding the Rex Shunt, but primarily his focus is on children. There is however another procedure that is less well known and that is a method called the Sugiura procedure (see: http://www.ncbi.nlm.nih.gov/pubmed/8042326) which was developed in Japan, and from what I have read is relatively successful in certain patients. It does not seem to be a common procedure and the information I have on it is quite technical, but it doesn’t hurt to ask. You could call Dr.Superina’s office to see if he might review your boyfriends case for this treatment option, or you may also want to contact Dr. Jean Emond in New York as I have had a few people recommend him to me since I started this blog. See contacts below:
Dr. Jean Emond
NewYork-Presbyterian Hospital/Columbia
PH Room 14C
622 West 168th St
New York, NY 10032
Phone 212.305.9691
Dr.Riccardo Superina
2300 Children’s Plaza
(707 W. Fullerton Ave.)
Chicago, IL 60614-3363
Call 1.800.543.7362 (1.800.KIDS DOC)
Phone 773.880.4000
I hope this helps? I wish I could offer you more options that what I have here, but the options seem to be few and far between.
Prayerfully,
Clint