It’s been just over a year since Jakob Temple had Rex Shunt surgery in Chicago at Children’s Memorial Hospital under the expertise of Dr.Superina. I know it’s been a long time coming, but I am happy to report that his 1 – year check-up was good and he is leading a normal life now. Jakob’s mom Jen recently posted an update on a Facebook site called Portal Vein Thrombosis and Portal Hypertension in Children and Adults …
“I just wanted to let all of you know that Jakob had his 1 year check up last week and he got a clean bill of health! We are so blessed and grateful! We also got to meet another PVT family! We all go through so much with our kids and this group of people have been so supportive and I am so glad to see new people joining who are looking for support because they are sure to find it here! Jakob was so excited to meet his new best friend who also is a PVT kiddo who touched our hearts and his mom Kelly is amazing! There is something to be said about this group of people when you have never met someone in your entire life and when you finally meet them you start crying and have an instant connection with! I will forever treasure my PVT moms! I hope that one day we can all meet up! Jakob was so amazed to see that someone else has the same scars as him! I almost forgot to mention Dr Superina took Jakob off of his aspirin now! So as of tomorrow – no more aspirin – no meds – no nothing – so happy! Now I will continue to pray for all of your children and hold you all close in our hearts and minds … Hugs from Canada”.
Everyone loves a happy ending when it comes to the health of any loved one and I might add especially children, and Jakob is no exception. Like Jen stated in her post and I agree wholeheartedly, we need to pray for all these children with PVT and hold them close in our hearts and minds. For the happy endings and there are now more than a few, we are thankful for the work of dedicated specialists like Dr. Superina and others around the world, however there are those children (and adults) whose cases are far too complicated or advanced for surgery of this kind.
On the note above I want to segue into Kory Kohler’s current situation. Kory is currently facing a possible rare type of surgery called the Sugiura Procedure and this is due to the fact he was not a suitable candidate for the Rex Shunt. (Kory is pictured above with Jakob Temple). Kory is in good hands with Dr. Superina; however we need to remember him and his family in our thoughts and prayers as they look toward the next step in Kory’s journey. You can read more about Kory’s journey and donate to his cause by clicking here: http://www.caringbridge.org/visit/korykohler21
I’ve talked about Jakob and Kory here, but there is another little guy by the name of Carson Kommer who just had Rex Shunt surgery this past Monday (Feb. 2oth) at Children’s Memorial by Dr. Superina. According to a message I received earlier today from Kory’s mom, Carson is doing relatively well and the flow on the shunt seems to be working fine. He is not totally out of the woods yet – so we can continue to remember him in both our prayers and thoughts.
To date there is not yet an ‘official’ foundation set up to help families whose children are facing surgeries of this nature, but there is a site where people can donate to help families differ costs such as travel, food, or lodging when in Chicago for the Rex Shunt or related PVT surgeries. The site is called PVT/Rex Shunt Family Fund and was set up within the last year by Shelly Hart whose own daughter Aubree had a successful Rex Shunt procedure done a half a decade ago. Your donations to the fund would be appreciated as I know there are families who definitely could benefit from this. Click here to view the site. Thank you in advance for your consideration to this most worthy cause!
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.
As of noon yesterday (Sept. 4th) I received a message from Tatyana stating that Sonya continues to improve! Tatyana said she was eating regular food, she is on oral pain medication (Tylenol), her infusion was disconnected, and she has even begun to walk a little although she tires quickly. They also started her on oral blood thinners.
Tatyana was very elated with the great progress Sonya had made in just only one day! She attributes that progress to prayer and she is thankful to everyone for being faithful to the cause! The picture on the left was taken earlier today and below is a small powerpoint presentation of pictures taken Saturday and Sunday morning. Click to view powerpoint: Sonya’s Recovery
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.
Rivaroxaban (Xarelto) has recently been approved by the FDA and Health Canada for the treatment of Deep Vein Thrombosis (DVT) and according to what I have read it seems to be superior to Warfarin in clot prevention. As and example, the first article below mentions how the drug outperformed Warfarin in preventing strokes. I learned about Rivaroxaban at the appointment I had with my haematologist at the end of June where he prescribed this drug to me in the place of the Fragmin I currently take to combat clots. Needless to say I was very happy to learn that instead of injecting Fragmin like I have done for the past 6 years twice per day, I could now take something orally without the nasty side effects (bruising, bleeding, and scare tissue) of injecting.
Unfortunately the drug has only been approved for short term usage for potential thrombophilia for those who have hip or knee surgery. In my case and possibly that of others I am trying to get the ruling changed (at the very least on a provincial level) for people with rare clotting conditions who could benefit from this drug for long term clottings disorders such as Portal Hypertension.
I received an email update from Tatyana early yesterday concerning Sonya. Tatyana has granted me permission to post that email message below, and for the benefit of people not familiar with Sonya’s story I want to direct you to the link (see below) to the post on my blog that gives you detailed information on her story. If you scroll to the bottom of the page and work your way up from my original post dated 04-14-2011, you will get more of an idea of what this little girl and her family from the Ukraine have been through since the early part of this year. Sonya is in Chicago right now awaiting a rare operation called the Rex Shunt Procedure for Portal Hypertension. In Sonya’s case, no one can undermine the danger she faces regarding this procedure, or what her prognosis will be if doctor’s do not intervene. She has already had six bleeds to date since the early part of this year.
Due to the high cost of travel and medical expenses Sonya and Tatyana are here alone from the Ukraine, but Shelly Hart (yay Shelly) a Mom whose own daughter Aubree had a Rex Shunt 5 year’s ago will be spending the day with Tatyana while Sonya is in surgery on Monday (August 29th). Shelly heads up the parent advisory at Children’s Memorial. Kelly and Amy are 2 other mom’s who have offered moral support to Tatyana. Both Kelly and Amy’s kids are facing the same surgery and their schedules may possibly allow them to see Tatyana within the next week or so .
Thank you for your prayers. The bleed stopped and Sonya is feeling much better now. Her Hb (hemoglobin) is 9, so Dr. Superina says she will receive a blood transfusion during the surgery. Doctors let us go back to Ronald MacDonald house for the weekend where we now are staying.
Yesterday we spoke with Dr. Superina about her CT-scan results, and he feels like the area near the liver doesn’t look so promising for the Rex Shunt, but anyways he is going to try to proceed with one if he can. If after opening the area near her liver and he doesn’t find a vessel good enough to attach a Rex, then he is going to talk to me about the opportunity to do a Mesocaval Shunt instead. I am hoping that he finds a vessel good enough for a Rex Shunt. We could go to Moscow for a Mesocaval Shunt, but we are here for a Rex! But it doesn’t depend on the doctors desire, it is all dependant on Sonya’s anatomy. Dr. Superina gave me a bit of hope by telling that CT-scan doesn’t give a 100% garanty that a vessel exists or doesn’t, but he can only tell for sure when he opens up her liver and has a look.
I feel so afraid for my little one, even though I trust Dr. Superina, as no other doctor in the world. I can’t imagine Sonya continuing to suffer from pain and it makes me sick and crazy. I pray for heavenly comfort for her and me during that rough time for us.
Shelly is coming on Monday to support me, to be my family at that day. I’m so thankful for her!
Please pray for Sonya as they will start the operation at 8:45 am (6;45 am MST)
August 25, 2011
For those of you who have been following the story of little Sonya Danilenko from the Ukraine – I have both good and some ‘disconcerting’ news to share with you this evening. On the good news front, Sonya and her mom arrived in Chicago from the Ukraine last Friday to begin the process for Sonya to have the Rex Shunt procedure which is to be performed by Dr. Riccardo Superina at Childrens Memorial Hospital early next week. On the bad news front, Sonya was taken into the hospital earlier this morning with a bleed. This is Sonya’s sixth bleeding episode since February when they first learned about her Portal Hypertension.
Please keep Sonya in your prayers and I will update you as soon as I hear of anything new. As you might expect Tatyana is very tired from the long journey, and add this latest ordeal to the mix well I am sure you can only imagine how she might feel. Prior to writing this latest post I had started to write a more detailed update a few days ago which included how they got this far in the journey but I will leave that for another time as this obviously takes precedence!
In the meantime if you are just learning about this little girl, you can view the prior posts by clicking the highlighted words within this sentence. Thanks for caring and praying!
As per Tatyana I wanted to update those of you following this story on little Sonya.
On June 20th, Sonya was taken to a children’s hospital in Dnepropetrovsk with another life threatening bleed from the varices in her esophogas. A few days later Tatyana updated me again to say that Sonya was still critical and of course very weak. Lastly on the 29th I received much better news that Sonya was better, still very weak, but that her Hb was low.
On a more positive note overall is that they have most of the funds in place now to make the long journey to the US for treatment, but will need to recover enough to survive the long trip. Tatyana is to apply for visa this week, and hopefully will receive a visa without any problems. She is hoping to be in Chicago at the clinic in the middle of July?
Tatyana has asked for continued prayer for Sonya’s condition, and for the visa to be approved. I’ll keep you posted … Clint
NEW UPDATE 06-13-11 (Latest Update on Sonya)
It’s been a little over a month since the last update, and still we play the waiting game for Sonya. They have been back home in the Ukraine for a few week’s now, and have only recently received word from Children’s Memorial Hospital in Chicago as to what to expect regarding a possible Rex Shunt for Sonya. Dr. Superina had reviewed the results from the tests in Germany, and he is of the strong opinion that a Rex Shunt is still the best option for Sonya – contrary to what doctors in Germany told Tatyana. Doctor’s in Germany recommended an arterial embolization on Sonya, of which both Tatyana and apparently Dr. Superina did not favor. (Up to this point I was not familiar with this procedure and after a thorough search I was able to locate the link above which I thought explains the procedure in the easiest terms).
Not only were the expectations regarding the procedure outlined in the letter, but the costs were also highlighted. A recommendation was made by Dr. Superina that Tatyana consider applying for a 5 year visa to the US, as Sonya would need subsequent follow-up visits for awhile after an operation of this kind. As you can imagine the costs for a procedure of this nature is very high, and considering the costs to travel from the Ukraine to the US it adds even further depth to those costs. Despite this Tatyana is not deterred in her mission to get the help she needs for little Sonya.
Financially this is a HUGE undertaking and after learning of the costs via Tatyana last week, my heart sank for them. My natural response to any huge hurdle in life is to think the worst, but after mulling this over in my mind, I return to the faith I have in God who I believe is much bigger than all of this. To that end, I continue to uphold this little girl and her family in my prayers. For those of you who are also praying and thinking of them, I thank you for your faithfulness. Lastly I want to personally thank those who have offered to help Tatyana in practical ways, if she ever was to go to the US with Sonya for a operation of this sort.
I’ll keep you updated on any new developments. Thanks to all of you for caring!
Faith is like radar that sees through the fog-the reality of things at a distance that the human eye cannot see.
– Corrie Ten Boom (Dutch Christian Holocaust Survivor)
Update on Sonya 05-09-11
Earlier today I received a message from Tatyana (Little Sonya’s Mom) regarding the latest on her little girls condition. Due to the generosity of a donor in the Ukraine, Tatyana was able to take Sonya to Germany this past April 27th for testing and possible treatment for her Portal Vein Thrombosis.
Here are the details in Tatyana’s own words …
“As you know, we arrived at the KUNO clinic (part of the University Hospital in Regensberg Germany) on April, 27th. I was so happy and my heart was full of hope. The investigations started with the ultrasound and Dr.Knoppke said that it looks like the left lobe of liver has blood perfusion, so it might be the left portal vein that is unclotted. I was so happy to hear that!
On Tuesday she had MRI and also a gastroscopy. The doctors said that her varices look much better than a month ago and there is no danger of bleeding right now. The MRI was not really so informative so they needed to do CT with contrasting. The CT scan was done last Friday. Sonya had to be intubated twice, and under 3-hours general aesthesia, she received twice the high dose of X-ray radiation. After this entire ordeal doctor’s are still not sure how to proceed! Now they want to do an angiography, which is invasive. In order to do an angiogram doctors will open her femoral artery and use a catheter to proceed into the mesenteric artery. When in the artery they will release contrast to trace a path of where the blood is going. One cannot stress enough the danger involved in this type of procedure!
I didn’t have the opportunity to talk to the surgeon personally, but our doctor said that he does not think that Rex-shunt is possible for my daughter, because she has only collateral vessels. I feel so disappointed now, I really don’t know what to do?
I was reading your blog and almost made a decision to look for other surgeon who would say that the Rex-shunt IS POSSIBLE. Only the Rex-shunt is healing operation, other types of porto-systemic anatomises will only reduce the pressure in the portal system which causes bleedings. I do not only want her life to be saved, but I also want to save the quality of her future life.
So now I want to have a discussion regarding this problem with Prof. Shlitt, and at that point I will have to make a decision to either sign or not sign an agreement to have the angiography. Also I want to get a second opinion as well. I have decided to make a short summary of her case and all the recent investigations and send that information to doctors in USA who perform the Rex-shunt operation in children. If even one of them will say ”yes, I’ll do that”, then we will come back home and do everything possible and impossible to receive a new visa, and to collect the needed funds. I’m ready to go to the happy end of the story, to overcome all of the trials on that way. I want to see Sophia happy and healthy, she is worthy of that.
Please continue to pray for my girl and for the correct decision. The best would be to have the Rex-shunt procedure performed over here, but if the surgeon says ”no’, I’ll have to find another place in the world and another surgeon … I pray that would be Dr. Superina himself
Original Post 04-14-11
It’s with a sense of urgency today that I bring you the story of a little girl in Zaporozhye, Ukraine by the name of Sonya Danilenko (see below). Sonya is only 14 month’s old and has been diagnosed with Portal Vein Thrombosis (PVT) and a heart defect called pulmonary stenosis. Her Mom Tatyana located this blog last week and was seeking to contact a doctor outside of the Ukraine who had previously performed the Rex Shunt procedure.
Sonya was adopted in the Ukraine just over 4 months ago by the Danilenko family (see below), and oblivious to them at time of her adoption this condition revealed itself only a few months after (February) when she had serious bleed (see: esophogeal varices). Subsequently she has had 4 bleeds to date losing over half of her blood volume and she almost lost her life on March 31st. Added to the urgency of the situation, she has obstructive bronchitis with a breathing insufficiency. If she coughs it could open up another varice and her condition will decline even further.
Tatyana is an emergency doctor herself, and her understanding of Sonya’s condition is excellent. Unfortunately no one in the Ukraine is able to perform the Rex Shunt procedure, however Tatyana was able to locate a doctor at the University of Regensburg, Germany by the name of Dr. Birgit Knoppke who may be able help her. Dr. Knoppke says that this kind of shunt is of course preferable, but can only be performed if the left branch of the portal vien in the left lobe of liver is not clotted. If they find the thrombosis in that left branch, as well as in the extra-hepatic part of the portal vien, that kind of shunt is impossible to perform and they will then consider a meso-caval or another kind of anastomosis. If those options are not feasible, she will be faced to endure encephalopathy with behavioral or mental problems with heart insufficiency and other future problems.
So as you can see Sonya is a little girl who is very ill. Tatyana told me Sonya has to be stable before she is able to make the trip to Germany. As per an email Tatyana sent yesterday she relayed the following, “My privilege in this situation is first of all my faith in God, then a MD degree in medicine with the understanding of the problem and then no language barrier, I can discuss all of the details of treatment with doctor’s there”. Fortunately they have raised the necessary funds through the gifts of some generous donors, so that is one thing less to be concerned about. They are currently in the process of getting their passports and visas to make ready the trip.
The Danilenko family has 8 children in total, 3 are their own, and 5 are adopted including little Sonya. I promised Tatyana that I would pray for Sonya, and their family at this time. I told her I would also ask others to “stand in the gap” for them by praying for Sonya at this critical time. Although I have never met Tatyana in person, I feel I know them by the few messages she has relayed. They have a tremendous heart for children. In Tatyana’s own words she says, “I believe in the Lord Jesus Christ and serve Him by adopting orphans into family, loving them as my own children and telling them about the love of a Father”.
Will you also commit to pray for this little girl and her family? Specifically pray that Sonya will be stable enough to make the trip, and Dr. Knoppke in Germany will be able to intervene to save Sonya’s life. Pray for this family as they deal with this situation and that all the “hurdles” that may come their way before they leave will be taken away. I know they will be encouraged by your efforts in this regard. Their faith in the Lord Jesus Christ is sustaining them for most part, however one can’t deny the sense of concern they no doubt have at this time for this precious little gift from God.
Peace I leave with you; my peace I give you. I do not give to you as the world gives. Do not let your hearts be troubled and do not be afraid. John 14:27
There are several complications of Portal Hypertension(PH), including one of the most precarious side effects called Esophageal Varices (covered in a previous post), and of lesser degrees are the conditions of ascites, and splenomegaly.
Ascites takes place when excess fluid accumulates between the lining of the abdomen and abdominal organs. This area is referred to as the peritoneal cavity.
Low albumin levels in the blood as associated with portal hypertension is also a contributing factor. Albumin is a protein made by the liver and is measured in the clear liquid portion of the blood by a special blood test.
Beyond portal hypertension there are other causes of ascites and they are cirrhosis, congestive heart failure, hepatitis, and cancers such as pancreatic, ovarian, or liver. Kidney dialysis can also cause ascites.
Upon examination the obvious signs of ascites are a swollen belly (abdomen). At that point evaluation tests on the liver may be performed which include urinalysis, and kidney function testing.
Common treatment includes diuretics (water pills), limiting salt in the diet, antibiotics (if infection is present), not to mention the avoidance of alcohol. If the patient does not respond to the common treatments, then the placement of a tube in the abdomen may be used to drain large amounts of fluid from the area. Lastly a transjugular intrahepatic portosystemic shunt (TIPS) is a radiology technique that reduces portal pressure and may be the most effective treatment for patients with ascites that are resistant to diuretics. The procedure is performed with the patient under conscious sedation or general anaesthesia. A radiologist places a stint through the skin from the right jugular vein into the hepatic vein. A connection is made between the portal and systemic circulations. This treatment from what I have been reading seems to be the norm in the treatment of ascites for patients resistant to diuretics.
Now on to the topic of splenomegaly or simply put an enlarged spleen. The spleen is an organ that is part of the lymph system. The spleen’s role is to filter blood and to maintain healthy red and white blood cells and platelets. To clarify, your spleen acts like a spongy filter for your blood. As blood circulates through your body, some of the cells (in the form of platelets and red blood cells) get damaged or worn down. The function of the spleen is to remove these damaged cells from circulation. The spleen also acts as a storage location for extra red blood cells and platelets newly formed by your bone marrow. There are several other causes to splenomegaly which include diseases of the blood, liver, cancers, and infections but in the case of PH sufferers the cause is clotting to the portal and splenic veins.
From a personal standpoint I have been fortunate enough to not have any pain as related to the enlargement of my spleen, however the possibility does exist from what I have read and been told. When first diagnosed with portal vein thrombosis(PVT), the first gastroenterologist I had wanted to remove my spleen (splenectomy). Being only a few months since the discovery of PVT in 2002, I felt uneasy about a possible splenectomy so I consulted with my haematologist who practised at another city hospital. His exact words at that time were, “anyone can remove your spleen, but ‘he’ is not touching it – come and see me”! Looking back I realize this was a good decision, and it may have saved me from added or more serious complications. All being stated, a person can live without their spleen, but from what I have learned recently is that the spleen is very helpful in maintaining one’s immune system especially when the liver and artery function is compromised by a clot. Without the spleen a person’s vulnerability to infection does increase, so it is important to get regular vaccinations to prevent bacteria such as influenza and pneumonia from taking its toll on one’s health. I might also add that even though a person still has their spleen, but has portal hypertension, it is important to be inoculated against influenza and pneumonia as either can cause varices (if present) to rupture and bleed with serious consequences. Like seniors and small children, our immune systems are compromised and health care professionals consider PH sufferers as being “high risk”.
Lastly I want to say that if you or a person you know is experiencing symptoms such as mentioned above, you are more than welcome to comment or post your questions below. There is so much more one can learn, and each of us has a story… maybe you can shed some light on the subject for the benefit of someone else?
The words, “there is nothing further we can do for you” didn’t really hit me until shortly after my appointment with the specialist on the drive home that day. I had hoped for some more positive news, but obviously it was not meant to be.
This past October (2010) I had met with my gastroenterologist after her one year study leave regarding portal hypertension. Due to the high level of anticoagulants I am currently on to prevent further clotting, she and other specialists agreed that in order to do any kind of procedure my life would be compromised greatly. It is important to note that just over 2 year’s ago, I was being considered for a rare cluster transplant which would have entailed the transplantation of my liver, stomach, pancreas, and the large and small intestine. Doctors were also considering the placement of a surgical stint (TIPPS) to relieve the pressure. At that time a panel of 5 specialists – 3 liver specialists, my gastroenterologist, and my haematologist had reveiwed my case.
Beyond the fact that nothing further can be done for me, one small measure would be to titrate me another 20 mg on my Nadolol to keep the pressure down. My blood pressure seems to be relatively stable right now, so my gastroenterologist was hesitant to change the dosage in fear that it would make me even more tired than I get now. In the event of a bleed, of course they would have to use evasive measures (banding) to stop the bleeding, but with the high dosage of anti-coagulants (Fragmin 12,500 IU injections/twice per day) that I am currently on, the fear is I would likely continue to bleed from the varices due to ulceration after the bands had “sloughed” off.
The picture on the left shows an actual diagram I showed my doctor on that day from an article which was posted in The New England Journal of Medicine. The black pen marks represent the extensive clotting to the mesenteric, portal, and splenic veins as noted by my gastroenterologist on that day.
As mentioned above my doctor (Dr.Puneeta Tandon) had been on a study leave this past year to the University of Barcelona in Spain, and she also spent time at Yale School of Medicine in New Haven, Connecticut. She specifically received training in portal hypertension under the mentor-ship of Dr. Guadalupe Garcia-Tsao from Yale. Recalling what she said really solidified what I had been told a few year’s ago and it brought me back to the reality that my life could end abruptly… I am a ticking time bomb! Despite knowing that – I’ve lived 8 year’s beyond my original diagnosis, and that truly is a gift! It’s also important to note that since my diagnosis I have endured countless medical tests, several iron infusions (4 per year), over 4,380 needles (Fragmin 2/day for the last 6 years), almost daily intestinal pain, a serious bleed (2004), not to mention clots pulmonary embolism to both my lungs in early February 2005.
While mulling over the verdict I was given on the drive home, I recalled a text from the Bible which states, “Why, my soul, are you downcast? Why so disturbed within me? Put your hope in God, for I will yet praise him, my Saviour and my God”. The passage is found in Psalm 42:5. At that point I just needed to trust, and quite simply put “my hope in God” for whatever may come my way regarding the future! Until this point in my blog, I had really never expounded about the overriding peace I have concerning my condition, but felt prompted to tell you more in hopes that you may also be encouraged? First of all I need to tell you that only on very rare occasions I do get fearful when I think of the real possibility of a fatal bleed. If a bleed were to happen, I get concerned about where exactly I will be, who will be there when it happens, will I make it to the hospital in time, or will I go into shock like many do when faced with the dire consequences? Thankfully that overriding peace is what the Bible states as, “the peace of God that transends all understanding” (Philippians 4:7). The peace that carries me through stems from a personal relationship I have with Christ, God’s son who ultimately paid the ultimate sacrifice for my sin, and has promised me eternal life where I will be free from pain and suffering in Heaven when I die (John 14:1-4). I know no one really wants to talk about death or dying, but in actuality one’s passing does brings healing and when there is no hope for a cure, then at least the suffering is past.
Since my diagnosis almost 9 year’s ago, I believe prayer has been the key element for the under girding force that has enabled to stay strong, and optimistic despite the obstacles I face. Along with personal prayer, I am extremely grateful to countless others who I know have supported me in this regard. I do believe God can heal, but in my case He has chosen otherwise for reasons I do not understand except to say that I know there is a purpose for everything. In the meantime – I simply carry on and am grateful for life and the very breath I breathe each day.
Corrie Ten Boom, the Dutch Christian Holocaust survivor who helped Jews escape the Nazi’s during World War II penned the following, “Faith sees the invisible, believes the unbelievable, and receives the impossible.” Her words ring true for me today. Although God is invisible to the naked eye, we can only see Him through the eyes of faith (2 Corinthians 4:18).