With great optimism I share this news with you today. Dr. Riad Salem at Northwestern University in Chicago Illinois, and a team of over a dozen doctors have recently developed and tested a new non-surgical procedure for the treatment of portal vein thrombosis (PVT).
A few days ago Dr. Salem reached out to me via this blog with the results of the clinical study. His words below encapsulate this new technique.
“At Northwestern, we have pioneered a new, non-surgical approach for the treatment of portal vein thrombosis with or without cavernomatous transformation. It involves creating a new channel in the clotted portal vein, and placing a transjugular intrahepatic portosystemic shunt (TIPS) stent. We do this using ultrasound and X-ray guidance. Our first series of 61 patients has already been published with excellent success, and many of these patients have gone on to liver transplantation since they had cirrhosis of the liver. We are now performing this on adults without cirrhosis with cavernoma. Some of these patients have varices that have bled. This is a procedure you should be evaluated for if you are considering a Rex shunt, since this is a non-surgical alternative”.
Riad Salem MD MBA Professor of Radiology, Medicine and Surgery Chief, Section of Vascular and Interventional Radiology Vice-Chairman, Image-Guided Therapy Department of Radiology Northwestern University 676 N St Clair, Suite 800 Chicago, IL USA 60611 1-312-695-6371 office
It’s been a few months now since I was admitted to the hospital (08.26.12) with yet another new blood clot, and this last time it was discovered in the superior mesenteric vein. I am happy to report I am doing quite well now, with no pain in the immediate area so it would seem the TPA (thrombolytic therapy) I was administered in the hospital worked to dissolve the clot. To say I am grateful would be an understatement as the complications from the latest episode could have been quite disastrous from what I learned.
On reading through the discharge summary I received from my family doctor several weeks ago, I have had to go back and research some of the terminology within that report.
Since the original diagnosis of my condition in 2002, I was told that the homocysteine levels in my blood were high. At the same time I was tested for all known causes as to why these levels were high, but all the results came back negative and the real cause has never been pinpointed in what one could call “the genetic realm of things”. The report I received from my doctor stated that I have a history of hyperhomocysteinemia with an elevated factor VIII level. This has caused hypercoagulability and as a result I have clotting, in the adjoining portal, splenic, and mesenteric veins respectively. This new clot settled below the existing clot in the superior mesenteric vein (see diagram below) causing restricted blood flow to my abdominal cavity, which in turn caused the arteries in the bowel to distend explaining the pain I had for 2 weeks prior to being admitted. To better understand hypercoagulability here is a really good site which I believe explains it well: http://labtestsonline.org/understanding/conditions/hypercoagulable-disorders/
Prior to this latest episode I had been prescribed a drug called Pradax (dabigatran etexilate), which is used to prevent harmful blood clots from forming and normally is prescribed to patients with atrial fibrillation. New research has shown the overall effectiveness of the drug for other clotting issue to be quite positive. In my case, its not clear if the drug allowed the new clot to form, because as you know one person can react much differently to medications than another. I don’t lay blame on the doctor who prescribed it nor the drug itself although for sure it could have played a role in causing the clot, but I was very happy to be on the medication because it was taken orally as opposed to the 7+ years of poking, prodding, bruising, not to mention scarring from all the injections over this period of time. So while in the hospital, I was put back on Fragmin (low molecular weight heparin) as being the safest way in treating my clotting condition, and despite the unpleasant side effects of these needles mentioned above, I am back to injecting myself twice daily. I have to admit though that I am much more willing now to accept the fact that I have to use Fragmin than ever before as I have virtually been free of any new clotting since being prescribed way back in December of 2004. Because my homocysteine levels have always been high, I have also been taking Vitamin B75 to help combat this issue from another angle.
When I was discharged I was given the actual report from the CT scan while in the emergency department which revealed the clot, but also accentuated the fact that the varices in my esophagus were even more engorged than a previous scan I had back in 2009. The scan also showed that the gallbladder wall was irregular and thickened due to portal hypertension. While the pain was present for about almost 2 weeks before I went into emergency, it was thought all along by my doctors that it was caused by the gallbladder itself. In February of this year, I had been having some discomfort just under my right rib cage, and at that time my doctor ordered an ultrasound of the abdomen. That ultrasound did show gall stones and thickening. In fact I am still being treated with a drug called Ursodiol which was prescribed last February as a ultrasound I had then revealed gall stones and thickening around the gall bladder itself. I saw a liver specialist at the end of October for follow-up, this being the same doctor who in 2006 recommended that surgery was a very dangerous option for me then let alone now. In 2006 I was being considered for a rare operation called a Multivisceral Transplantation (cluster transplant) that would have included the liver, pancreas, stomach, and small bowel. The thinking of course is that it would have eliminated the clot in the portal, spleenic, and the original clot to my superior messenteric vein. At that same time the team of doctors reviewing my case were considering a TIPS procedure, which was ruled out because I was doing so well. Conclusively any operation would be highly dangerous because of the risk of bleeding, and the possibility to clot is definitely another risk factor to be concerned about.
The regiment of medications and injections have remained a constant for me for several years now as are the frequent required blood testing, and doctors appointments. Pain, when it comes is quite intense, and thankfully only intermittent at best. Pain usually is accompanied by an irritable bowel, which can be most inconvenient, especially if you have to be out anywhere. As with most people with the condition I am on high dosage of nadolol (beta blocker) which is used to keep my heart rate low and hopefully prevent bleeding from the varices. The current dose I am on causes me to be quite fatigued at times, and in the afternoons I struggle sometimes to stay awake . As with any chronic health conditions there are challenges, however I feel very blessed that I am still alive, but also that I have access to good health care in the area where I live. That being said, I know in many places around the world access to good health care is a real issue, and I am conscious of that as I continue to write this blog. In the end I always hope (and pray) things will improve for those of us with portal hypertension, and that a cure or new treatment will one day be available to everyone, no matter where you live.
In your corner,
To read a prior update on my condition click here.
It’s been a few days since my last update, and I wanted to let you all know that I am now at home “safe and sound” after my week long ordeal at the hospital. I arrived home here early Friday evening after being directly released from ICU at the University Hospital here in Edmonton.
In my last post I spoke about being treated for the latest clot through a method called TPA which is short for Tissue plasminogen activator. The treatment is primarily used for stroke or heart patients in the very early stages (up to 4.5 hours) of discovery, and it is often effective, however in my case my hematologist did not even know of another person in the world who had the treatment for the type of clot I developed called mesenteric venous thrombosis. My hematologist had recommended this procedure to an entire team of specialists at the time, because it was the only thing he thought they could do that would be less evasive then actually cutting me open! When I asked him if this had ever been used before for this type of thing, he only said that a young fellow they had admitted a few weeks prior had the procedure done for a clot that had developed on the entrance to his kidneys and that it was successful. Initially my doctor had wanted a radiologist to insert a catheter directly over the clot location and target the actual clot from there, but that was ruled out as it again entailed opening me up and the risks of course are enormous.
At this point we are not sure if the TPA did what is was supposed to do, because it was administered almost 14 days since the onset of the pain I started having. I am still dealing with some pain, but I will not know for sure if the treatment has worked until I do follow-up with my hematologist in a few weeks time, and of course if the pain has totally diminished. At the time I was having pain it was thought that I was dealing with that from the thickening of my gall bladder due to the congestion of arteries from the existing clots, but that was not to be. This latest clot caused swelling and distention of the bowel, and I was in considerable pain which increased in intensity since its onset a few weeks earlier. After being admitted to the hospital early last Sunday morning, a CT scan revealed that a new clot had formed just below an existing clot in the mesenteric vein. Click here for a diagram (black felt marks shows the existing clots I now have).
The danger for anyone having a TPA is that they could develop a clot in the brain, or bleeding, but in my case severe bleeding can occur as TPA is considered a clot buster with strong anti-coagulant properties. Since my original diagnosis 10 years ago, I have always have been at risk to bleed, but more so now because I have esophogeal varices.
Thanks to many of you who prayed for me while I had the TPA, otherwise who knows what could have happened? For those who sent emails, posted on my Facebook page, called me on the phone, stopped in to see me personally, brought food to the house, prayed diligently for me, or encouraged me through their words or hugs – I am feel so humbled by all your kindness! Knowing that so many people from around the world were in the background cheering me on, made me feel special, and I cannot begin to tell you what that means to me. You are a loved bunch! Bless you for your faithfulness. Not only was I blessed by all your love, but it was encouraging to my family that people were cheering me on in the background!
For the last several days I have had some thoughts I feel I need to share with you. Many of you already know that I am a person of faith, but I have only touched the surface of that reality in prior posts of what that faith actually means to me. In the past I have shared with you concerning the peace I have, and that has always been evident, except for the occasional moments (being human) when I did have some pervading fear – even as recently as early last week when I was not sure what would happen to me upon learning about this TPA treatment and how it could affect me. Last Tuesday, 2 of members of our pastoral team, plus a good friend and former pastor came up to the hospital to pray for me. I had called them because of the scripture that says, “Is anyone among you sick? Let them call the elders of the church to pray over them and anoint them with oil in the name of the Lord”. (James 5:14) After they prayed for me and I myself prayed to have the fear removed that I had within me concerning the unknown, I overwhelmingly sensed “peace” and I can testify to that fact as it was real – all the way through the procedure until this very moment. Whether or not I was healed at that moment is not known, but I carry on in faith that God goes before me and gives me the peace that I need to endure all obstacles.
Before this latest clotting episode, I have had the following passage swirling around in my head for many weeks which reads, “Therefore we do not lose heart. Though outwardly we are wasting away, yet inwardly we are being renewed day by day. For our light and momentary troubles are achieving for us an eternal glory that far outweighs them all. So we fix our eyes not on what is seen, but on what is unseen, since what is seen is temporary, but what is unseen is eternal”. (2 Corinthians 4:16-18). Allow me to paraphrase this for you from my own personal point of view … The first part of this verse tells us not to lose heart, because we are wasting away (boy do I know this), yet on the inside (not physically but spiritually) I am being renewed day by day. For our “minute” and momentary (fleeting) troubles are achieving for us an eternal glory (heaven) that outweighs anything on this earth (I love this). Eternity is forever, the here and now is just temporary. If I/we fix our eyes on only what is seen, we lose sight of all that God wants us to be here on earth, and if we dwell (by God’s grace try to stay true or obedient to His word), we can have live on this earth with an eternal perspective which out does anything the world has to offer. I know in my own life this order often gets turned around because I don’t always put God before everything I do, therefore I fail and it keeps me from the beauty of the relationship and the peace that usually prevails which can happen when we trust Him for everything. One of the main reasons I think about this passage so often is that I do not have perfect health, of which I have never blamed God for, but have always thought it was His way of showing me that I needed to trust Him even through the most difficult times in life.
Lastly, there is one more passage I would like to share with each of you that is one of the most comforting passages I know, and one of many favorites at that. It is taken from John 14:1-4, and it is where Jesus is comforting his own disciples through these words, “Do not let your hearts be troubled. You believe in God; believe also in me. My Father’s house has many rooms; if that were not so, would I have told you that I am going there to prepare a place for you? And if I go and prepare a place for you, I will come back and take you to be with me that you also may be where I am. You know the way to the place where I am going.” When you live your life with an illness such as portal hypertension or any other sickness of which the unknown is uncertain, it is comforting to know that when the end comes, full healing will take place because of a decision I made as a young child, to believe in all that Christ Jesus, God’s only son did for me by dying on a cross for my sin. When I think of this and the sacrifice that was made on my behalf, and the gift of eternal life that was given, it is a tremendous comfort – and in reality it makes life worth living all the more! As I look to the future, it may not be bright in many respects for me or anyone one of us, however I press on despite the odds!
Some of you may or may not know that I had been admitted to the University Hospital early Sunday morning with a new clot. As you know any clot can be dangerous, but this new one is located below an existing clot in my superior mesenteric Vein is most troublesome. This clot has restricted blood flow to the bowel region, causing it to enlarge and of course pain comes with that. If collateral arteries do not begin to feed this area of the bowel, it would die and they would have no option but remove this section – and that would be dangerous for me also. They have me on morphine and they are giving me antibiotics intravenously to stave off any potential infection. The actual terminology of this particular condition is referred to as mesenteric venous thrombosis. To be more precise the severe pain I was having is caused by swelling and distention of the bowels.
I was informed last night by my hematologist that he was going to speak with a team about a procedure commonly used for stroke patients in the early stages. The procedure is called TPA and is performed in the early stages (usually 1 hour) to break down clots in the brain and also the heart. For TPA to be used in my case, it is almost unheard of, but the alternatives for me not to go ahead do not look good at all.
The other downside is, even if it works, is that the clot could reappear and I would have to be reassessed for eligibility. The doctor said I have age on my side (I liked hearing this) and I am relatively doing okay with my current treatment protocol so he feels confident this will work.
Earlier today I really struggled with all of this as I haven’t had much time to digest it all. Right now I feel much more at peace because of prayers lifted on my behalf. As I face this procedure sometime today, I am asking you for your prayer support as I believe the more people praying, the better the outcome in the end.
Thank you for your support!
(Note: After composing this message, 3 pastors from our church came and prayed with me. I have a tremendous sense of God’s peace over me now and my very life is in His hands – as it always has been for many years now)
UPDATE: The TPA was postponed by the ICU doctor until 8 am this morning. Apparently he wanted easier access to GI doctors in the event of a bleeding issue, and more of those specialists are available during the day. I was prepped early last evening here in ICU and I am spending the night here.
Post TPA Update: IV is out but I still can’t move around for awhile yet. The pain I have still is milder in the abdomen, and the injection site but they still are giving me morphine to handle it. Dr Ritchie came in an hour ago and said that the clot may or may not still be there as it may have been there too long? The GI was also here earlier and said the truth won’t really be revealed until they take a scan – maybe tomorrow or Saturday. They will monitor me for at least a few more days. Thanks for your prayers!
One of the first messages I read this morning was from a young man named Pablo Alvarez who courageously relayed his story on a Facebook site I subscribe to called Portal Vein Thrombosis and Portal Vein Thrombosis in Children and Adults. In reading Pablo’s story this morning I was very moved, and I asked if he would allow me to re-post it on this site for the benefit of others. Pablo, I want to thank you for allowing me to share your story. I pray that God will continue to bless you with good health, and that His favour will rest upon you in every other aspect of your young life.
Without further adieu, here is Pablo’s story in his own words …
Hey guys! My name is Pablo. I was diagnosed with PVT at the early age of 6 months. We lived in Ecuador at the time. I might of not been aware at the moment but I know those had to be some tough times for my family. Doctors in Ecuador were clueless as to what was wrong with me. Some doctors wrongly diagnosed me and ended up doing unnecessary surgeries on me. Money was tight and the many sclerotherapy procedures I had were getting too expensive. Thankfully family pitched in when possible. I remember a sclerotherapy being done one year around my birthday (Feb 14), and in particular not looking forward to that at all, including the many others I had along the way. For all those dreaded occasions my entire family would attend and would take up the whole waiting area – I felt very, very loved and supported. It might of been a tough time for me but having my family there always made me feel better, especially because hospitals in Ecuador in the early 90s looked scary, kind of like wards. As years passed we kept the sclerotherapies going. We saw ourselves in this circle. Yeah the treatment helped me out but where was the solution? They didn’t have any besides taking my spleen out. The gut feeling my Mom had at that time was that this was not the solution for me. Around 1996 my Mom realized she had to do something about this. The internet was the first step to our journey. The first thing she searched for was, “The best hospitals in the United States” and there she found Childrens Hospital in Boston, MA. She knew right away that this was where we had to go. We packed our bags and headed to the states. Leaving my sister behind, we thought we would come back after they had cured me.
We arrived at this Social Workers house. She made the term, “Mi casa es tu casa”, literal! She fed us, gave us beds, transportation, and most importantly made us feel welcomed. We went to Children’s in Boston the next morning. My eyes grew as I walked in. Everything was different about this hospital. The first thing I noticed was the automatic spinning doors! I never saw doors like it before. Next, the “I’m in a cold psycho ward feeling”, didn’t apply to this hospital. It was modern, colorful, and had this feeling of hope that lingered in the air. The doctor in Boston told us that taking my spleen out was absurd! My moms gut was right after all. He had mentioned that at the moment (1997) that the only thing to do is to do more sclerotherapies to keep me stable, until he finds the right surgeon and surgery. The following year we flew in my sister. We new our stay would be a little more lengthy than we had thought. My dad found a job & he started making money, he saved enough so we could get our own apartment. We moved in to an apartment with 2 bedrooms,1 bathroom, a small living room, and a tiny kitchen. It was very small but it was comfortable and it was all we needed at the moment. I started school and had to repeat 1st grade again, seeing my English consisted of “hello” and “where is the bathroom”. I remember being scared the first day, but everyone made me feel welcomed and I made friends in no time. As years passed more sclerotherapies were done and more pills I had to take to keep me stable. I was taking 3pills x2 a day. I became quite the expert at swallowing pills, I got used to it.
The new century was approaching when they had diagnosed me with cataracts. My mom and her grandmother had cataracts, so it was genetic. I got the surgery done at the age of 10. I felt like a pirate going into my 4th grade class after the surgery. Everyone stared but I wasn’t sure what they thought about it. They probably thought I looked like a pirate too. I had it in both eyes so they did each eye individually. After that I was diagnosed with polyps. They scheduled me for surgery to remove them. I remember going in a day before so they could transfer plasma and blood into my system. They didn’t want me to bleed to death when they remove them, seeing my platelet count was very low. It was one of the surgeries we wont ever forget because it almost cost me my life. During the surgery they kept putting more blood and plasma into my system. The blood they kept giving me started filling up my lungs. They took 2 polyps out before they stopped the surgery and took me to intensive care. Where I woke up gasping for air, everything was blurry. I just remember my mom fighting off nurses and doctors to be by my side, like she always has. Her being next to me saying “Breath! Breath!” made me feel better. To be honest, I don’t remember the rest. I remember just going back to sleep. I woke up with my Mom, dad and sister next to me. They didn’t complete the surgery there were 2 more polyps to remove. Couple months passed and they prepped me for surgery again to remove the last 2 polyps. My mom prayed and prayed that those 2 polyps were no longer there. Her faith, that day was unstoppable because the doctor came out and said he couldn’t find them, that they were gone! It was just another sign God was still with us and he was still looking out for my family and I.
It was 2002, my mother had received a call from my GI doctor. He told her he found someone that is performing a new surgery (Rex Shunt Procedure) that had a solution to my PVT. He told her his name was Dr. Riccardo Superina and that he was located in Chicago. We packed our bags and took a road trip to Chicago in Jan of 2003! We stayed in the Ronald McDonald house in Chicago. I remember just thinking that scary clown McDonalds promotes being there while I sleep! The next day we met with Dr. Superina at Children’s Memorial. He explained the surgery and after had a look at me. Made me lay down and feel my spleen and all my insides. It was a regular thing for me; To lie down pull my shirt up and have the doctor apologize in advance if his hands are too cold. He took the measuring tape out and then proceeded to measure my big spleen. We concluded our appointment with Dr. Superina and headed to the city to explore. Of course we were going to take advantage of being in Chicago. We went to the Sears tower and later dined at the Cheesecake Factory for the very first time. My stay at the Ronald McDonald was short seeing I would be spending the rest of the time in the hospital. We met a lovely couple that had a daughter being treated at Memorial as well. Everyone was nice and welcoming there. They even gave us floor seats to a Bulls vs. Clippers game. I never enjoyed watching sports because I could never do any kind of sport. There was always the fear of bleeding to easily. I did in fact enjoy the game though because I was up close and personal to these tall dudes that I had seen in ads on TV. It was January 15, 2003, I woke up to shower at 3am. My nerves were out the roof! We took a Ronald McDonald’s shuttle that took us to Memorial. I felt cold and nervous once I walked into the hospital. My family and I waited in the pre-op for the nurses to take me in. I asked if my mom could be with me until they put me to sleep, and they accepted my request. My mom held my hand while they put me to sleep. She was the last thing the old Pablo saw and felt! I remember waking up, dazed and confused with multiple tubes and wires attached to me. I remember feeling like someone stabbed me multiple times in my stomach. It was a sharp pain that I still remember until this day. I spent 3 days in Intensive Care, those 3 days were vague. My parents would take turns to be with me while one went to get coffee or go back to Ronald McDonald and shower. My sister was there with me the whole time though. She would hold my hand and caress it with her thumb. She was 19 at the time. I remember sitting up for the first time. It felt like all my organs were rearranged they were all moving back to their proper place. Walking was hard the first time, fluid kept dripping from my incision. They moved me to a room, where I met my nurse Erica. She always had a smile on her face. She was my favorite out of all of them. I stayed in the hospital for 8 days. 8 long days for me, it felt like weeks and weeks had gone by. I left the hospital very skinny and pale. Walking the first couple days out was hard, I would get tired quickly. I remember we went to the zoo couple days I got out of the hospital. We were walking to the entrance and an old gypsy looking lady approached us. She said, “You are a sick boy, but I will give you a kiss to make you all better”, She was short so I bent down to her level, her kiss gave me chills. She continued to walk opposite ways from us, couple seconds later I looked back cause I was in disbelieved of what had happened. As I looked back she was not there anymore. No where to be found in fact. It was an empty parking lot and she couldn’t had gone that far. Until this day I don’t know where she went. I just take it God sent an angel to kiss me all better. Our trip to Chicago had come to a conclusion and we felt so blessed and went back home with a new experience to remember forever.
My scars were not looking so good though, They had turned into Keloid. For me it was hard a for a 13 year old to have this ugly scar exposed on my neck. People stared with wonder and some people were brave enough to ask. My response was always, “from surgery”. My scar on my stomach was looking better, though it had a little bit of keloid on the top. As I got into High School it got a lot harder, I had to answer to all these new faces. I got countless stares walking down the halls & I stared at them right back as if they were the one with the flaws. People judged me, yes, but once they got to know it was if the scar was invisible and my personality and qualities over took that role. I have had long term friends say they can’t picture me without the scar and that it gives personality. I took it as a compliment because, I myself felt the same way. My mom contacted a plastic surgeon at Children’s to see what he could do about my keloid. Before I knew it he schedule in me for surgery. He sliced the keloid right off my neck. After he started the steriod treatment on me. It helped to a certain extent until the Keloid came back. It wasnt as bad as before but it was still there.
I kept going back for ultra sounds to see how my shunt was doing! It was all flowing smoothly! My platelet count went up. When I have a cut I don’t bleed as much. It just started to all come together. I got a little break from being in the operating table so many times and started to actually view my life as if I was “normal”. Yes the restrictions were still there, yes the scars were still there, but my view on life was different. We moved to FL going into my junior year of High School. We have family who live here so we decided, why not! We love tropical weather much better anyways.
I until this day I thank God for what he did. How he put all these people in my path at the right time. How he made so many things that would have been impossible, possible. Every little detail in my life I am thankful for. I would not be here without His help and the hard work and sacrifices my family have done for me. I have had one hell of a life and I am only 21 at the moment. I will never get tired of telling this story because people need to know how incredible our God really is.
It might be a rare disease but your not alone! There are people who have stories that might not be the same as yours but its surrounded on the same thing PVT! If you have been just diagnosed or haven’t had the surgery yet, be strong! You have to keep fighting this battle in order to succeed. God will always be there for you, even if you don’t believe! He still loves you, even if you don’t believe! YOU WILL GET THROUGH THIS! Just like I did and many of you that are in this group!
If you have any question about my story or have questions of details I did not cover contact my mom! Anita Merchan she will be there for anyone who needs advice or just to simply talk! I am here also if you have any questions or need any advice!
Tonight I want to share a good news story from a comment posted on my blog earlier this evening. The story comes from Janet who suffered from portal hypertension for many years. Here is her story …
“Just found this site today and had been posting on DVT forum previously. Have had PVT for 9 years making it a chronic condition and also had one episode of bleeding esophageal varices 3 years ago. I have had numerous endoscopies with 5 sessions of banding and at last scope had Grade II varices. I have been on nadolol to keep the pressure down Continue reading →
Almost 1 year ago I brought you the story of Sonya Danilenko, a little girl from Zaporozhye, Ukraine who was dangerously ill with portal hypertension and subsequent bleeding esophogeal varices. Sonya’s mom, Tatyana was searching for information on the Rex Shunt procedure and happened upon this blog where she learned of the wonderful work of a specialist at Children’s Memorial Hospital in Chicago Illinois by the name of Dr. Riccardo Superina. Initially, after some very close calls in the Ukraine, Tatyana, who is an emergency physician herself, decided to take Sonya to Germany to see if doctor’s there could assess and perform this rare procedure. As is the case for many countries in the world, Ukraine does not have the resources or specialists that are able to handle such a complex condition as portal hypertension. Unfortunately the German doctors were also unable to perform the operation, so Tatyana made arrangements to send her imaging to Dr. Superina and as they say, “the rest is history”.
After successfully raising the necessary funds to fly to the US (a story in itself), Tatyana arrived in Chicago with Sonya at the end of August 2011, and shortly thereafter Sonya had her surgery. In the 11.5 hours of surgery Dr. Superina attempted to connect the shunt 5 times, but it clotted off almost immediately on every try. In the end he ended up doing a mesocaval shunt which is the least desirable, but nevertheless it is open and flowing. Sonya is scheduled for her follow-up appointment with Dr. Superina this May in Chicago. She will need to be continually monitored, but so far she continues to thrive. In my mind I credit Sonya’s overall improvement to the great care and expertise of Dr. Superina, the staff at Children’s Memorial, her mom Tatyana, and not to mention the prayers of many people around the world!
In conclusion it seems that both portal hypertension and its dangerous side effects outweigh any attempt at surgical intervention if the candidate is suitable. If what I have been told is correct I think that Dr. Superina maybe nearing around the 140 mark for operations on children with the large majority of those being successful. There are other specialists in the world who have also realized success in treating children and that number combined with what has been done at Children’s Memorial is around 300. I stand corrected on this information so if you’ve heard of anything different please let me know!
To read more about Sonya’s story click the following links:
Since first learning about the Sugiura Procedure for oesophageal bleeding, I have wanted to write something about it but had difficulty actually deciphering the material from medical terminology into language that was understandable for most. Besides scouring the internet I also tried to find understandable information in book form at my local library, and surprisingly I came up short. Before trying to explain (albeit briefly below) online medical dictionaries define the procedure in the following manner, “Oesophageal transection with paraoesophageal devascularisation, for oesophageal varices”.
As best as I can describe the Sugiura procedure, surgeons would ligate (tie-off) the penetrating oesophageal veins that originate from the portal system in order to stop the bleeding. To maintain critical venous drainage of the oesophagus and stomach the left gastric and paraesophageal veins are left intact. A splenectomy (removal of the spleen) is performed because it is usually congested and non-functioning. Some articles I have read indicate that there have been some modifications to the procedure and with that there has been some reported success. Although the Japanese (University of Tokyo) have reported favourable long-term results, the technique has not been widely accepted in the Western Hemisphere (except for Mexico) because of high postoperative mortality rates. On a recent visit to my gastroenterologist I inquired about the procedure, and she confirmed what I read and stated that it is only performed as a last resort where all other procedures have failed.
Through personal research, personal interaction I have had with other sufferers, plus the advice of a few of my own specialists I remain committed to the task of posting accurate information on new techniques and medical findings for this most disconcerting condition of portal hypertension. The Sugiura procedure is much more complicated than what I have tried to explain here, so maybe you have more information to share than what I have provided? At any rate your comments or questions are always welcome!
As per Tatyana I want to update all of you who have been following the story of little Sonya. It is hard to believe that it has been over 2 month’s since Sonya’s surgery at Children’s Memorial Hospital in Chicago … time has passed very quickly, but unfortunately Sonya continues to have a few complications.
About a month after Sonya’s surgery she developed a hernia on the left side of her incision. Dr.Superina has looked at it and he believes he can fix it, however Sonya will have taken off Coumadin first before any attempt can be made to intercede. He is looking at doing the surgery at the 6 month post op stage which would be sometime in March of 2012. The Mesocaval Shunt operation that Dr. Superina performed on Sonya seems to be working, however she has fluid in her belly and the hernia is getting larger. It would be risky and dangerous to go back to the Ukraine at this point with the hernia and the fact that she is on Coumadin. According to Tatyana the hospitals in the Ukraine do not have the knowledge to deal with this, especially if Sonya was injured in any manner. It would be hard to stop the bleeding on Coumadin and she could easily die if it was not stopped in time. So for the sake of health and safety they will stay put and when the time comes for them to leave they will have to take the risk and go home. They have a follow-up appointment with Dr. Superina on November 10th and from that point he should have a clearer picture of what needs to be done.
For background information leading up to the surgery on Sonya click the following links:
Tatyana and her family are looking forward to being reunited as they have been apart for over 2 months now. When they finally arrive home she will be doing paper work to apply for a visa to study in the US. Tatyana is a physician and in order to practise medicine in the US she would have to upgrade to US standards. After upgrading she would be expected to complete a one year residency in a US hospital.
Please continue to pray for Sonya’s health and now Tatyana as she and her family make preparation for application in the US.
September 8, 2011 For those of you following the story of little Sonya and her Rex Shunt operation in Chicago last week, her Mom sent me an update this morning.
As you know after over 11 hours of surgery with Dr. Superina at Children’s Memorial he was only able to do a Mesocaval Shunt instead of the Rex Shunt as many attempts to do that were met with clots which disallowed it from working. Now the problem is whether or not the Mesocavel Shunt is actually working and they will not know anything for a day or tw0. Sonya’s INR blood is fluctuating too much so they are trying to understand this more. Dr.Superina has ordered an ultrasound to see if they can figure out what exactly is going on with her system. They are concerned with her levels because she is still bleeding from her incision, and she has also been bruising. Another grave concern is the fact that she still has gastric varices and she could also be at risk for bleeding again.
Sonya also continues to have digestion and reflux problems. She could not eat the regular formula or baby foods, and had trouble keeping anything down. The nutritionist in the hospital gave her a special formula called EleCare formula and it seems like Sonya is able to digest that. They provided Tatyana with 6 cans supply of this formula, which was covered from an emergency fund from the hospital. They also gave here a Warfarin supply for 2 months, which was covered from the same fund. Tatyana will still have to buy liquid Omeprasol for her, and it is expensive at 184$ for 300 ml which is her monthly amount. She still needs to buy formula and Coumadin plus other medications to take home for 2 months after when she leaves and she does not know how she is going to pay for it at this point. They are going to stay in Chicago one-month post-op for follow-up, so they will need funds for food. She also mentioned that they would need to purchase warmer clothing; however Shelly has rallied some people from her Facebook who will provide in that regard. Shelly also will be donating her profits from the sale of an on line women’s speciality store and here is the address for that: http://www.mythirtyone.com/shellyhart/ all orders in the US will be shipped directly however orders to Canada will have to be shipped by Shelly herself.
Also a big thanks to Shelly for setting up another important site just today for donations to families of children with PVT/Rex Shunts – check this out: http://www.everribbon.com/ribbon/view/1399 (I can see this directly helping not only Sonya’s family but many others in the days/years to come).
Please continue pray for Sonya’s needs – first of all for her health problems and secondly for financial issues that have arisen from her recent discharge.
When I first saw this latest picture of Sonya which Tatyana sent me early yesterday morning, I could not help but be struck by the by the hopefulness in her eyes. The physical toll this child has endured has been unimaginable, but she seems to be very resilient and this is cause for joy. The progress she has made is due in part by the skilled hands of Dr. Superina, and all the doctors and staff at Children’s Memorial. I also believe that prayer has been the undergirding force behind her progress, and along with Tatyana I want to thank all of you.
Is Sonya out of the woods yet as far as her condition is concerned? Unfortunately the answer is no for now as there are some complications that will be monitored closely for awhile. Here is Tatyana’s latest message …
We had a rough day on Thursday- I would call it a gastro-intestinal crisis! The NG tube was removed on Wednesday and she could even drink Pedialites and everything seemed to be okay, but on Thursday doctors wanted her to start eating, but that was not to be so she started vomiting again. She was cried all the day long and they decided to put the NG tube back. So we had another bad night beginning Thursday to Friday. She continued to vomit and was feeling very bad. She was very tired also and was not interested in any activity- even music therapy. She was not even reacting when I left her with volunteers as I had to go to where I was staying for half an hour- just to pick some needed items. When I returned, she was crying because she had seen me- it was a reaction, even though negative.
Sonya was moved up to the 5th floor at 7 p.m. on Friday after 5 days spent in ICU. They removed the NG tube, so she started feeling better and even smiled once to me. She slept well this night, and I believe that from now on she will recover sooner, having more rest and feeling less stressed. Please continue to pray for stomach as it seems to be very sensitive still. She vomited a little this morning (Saturday), and started crying again. They crush her pills (blood thinners), but they irritate her empty stomach. I was always try to protect her sensitive tummy by giving her omeprazole which reduces acid production and I keep her on diet. Now they are giving her Aspirin without any protection and she continues to vomit. And – the varices are still there, so they have to think about protection from bleeding. This is a major concern that I wanted to share with you. She is still so very weak and tired, having almost 11-hours of abdominal surgery, being 5 days in ICU where she was bothered every 20 minutes, and never getting any rest which she needed the most.
Hopefully things will improve for us since we are here. Thank you for your concern for us.
Scroll down for earlier posts on Sonya’s operation …
August 31, 2011 Update Sonya
As per email from Shelly earlier today, I am posting a message she received from Tatyana regarding Sonya’s current condition. Sonya has rallied somewhat, but still is being monitored closely…
“I’ve seen your incoming calls but couldn’t answer. We’ve had a very rough day and night. Only now she feels better. She was extubated yesterday at 2:30 p.m. and was having trouble with breathing. She has pneumonia and needs oxygen all of the time, they tryed to reduce it’s volume but she turned violet in a seconds and her saturation failed.
Also yesterday she had problems with pain control. Since she has been extubated and they were not sedating her anymore, she was very restless, and has been moving and crying quite a bit. She became tolerant to morphin really quickly and we’ve seen the side effects such as breathing depression and agitation. Doctors didn’t really want to do anything about that until the new doctors from the pain control team came and observed her crying. They switched her to another drug and as soon as it started working, she felt more comfortable and was even able to sleep. But as you know the nurses are going back and forth all af the time and they kept waking her up all of the time to draw blood, check her IV lines, and to take her temperature etc. It is hard for her to have a long, healing sleep. I have been there beside her most of the time, keeping her from harming herself, wiping off her precious tears, pushing the PCA button every 8 minutes or so, singing songs to her, or trying to do at least to comfort her.
Today they removed the NG tube and PIV. The arterial line started leaking so they had to remove it because arterial bleed is dangerous, and a fast way to loose blood. So she’s got only one PIV,CIV nasal oxygen catheters, and has a pressure measuring manget on her.
She is drinking juice with water now and looks happier then a day before”.
Tatyana is very tired and emotionally drained. With Sonya now being able to drink juice and even looking happier, maybe Tatyana will be able to get some more rest. As we continue to pray for Sonya, let’s also remember Tatyana and her family back at home.
Until next time,
August 30, 2011 Sonya … After Surgery
It’s now shortly after midnight here where I am, and I want to update you on Sonya’s operation which took place early yesterday morning. Shelly Hart was able to spend the day with Tatyana while she was in surgery today and again I and other’s are most grateful for that. In Shelly’s own words I have copied and pasted 2 posts she made on Faceboook a few hours ago.
3 hours ago …
“Headed home. So thankful for the opportunity to meet an amazing woman who went to extraordinary lengths to save a baby that nobody wanted, in a country that viewed her as “damaged goods” and told her to leave her to die. Most of us dealt with medical problems with our birth children, but this mother CHOSE her, knowing the rough road ahead. I am utterly amazed by her compassion and faith”.
4 hours ago …
“Sonya is finally out of surgery after 11 1/2 hours of being under anesthesia. Dr.S tried 5 times to connect the shunt, but it clotted off almost immediately every time. He ended up doing a messocaval shunt which is the least desirable, but …is open and flowing. She is stable and Tatyana is anxiously awaiting to go to ICU to see her. They left her intubated for now to give her little body a break. Please pray that Tatyana will get rest and have strength to care for her and for Sonya’s quick recovery. It has been a day with tears of joy and tears of disappointment, but God is in control regardless if the outcome”.
Thanks again Shelly for your compassion and care for Tatyana yesterday. Also want to thank everyone else for praying and for your words of encouragement for Tatyana through Facebook and otherwise. Please continue to remember Sonya and Tatyana at this time. I hope to speak with Tatyana later this week. I’ll keep you all posted.