Maddi B. (Abernethy Malformation Patient)

Prompted by a comment I received a few weeks ago from a great-grandmother in North Carolina concerning her 2 1/2 year old great-granddaughter, I want to bring attention to a rare condition called Abernethy Malformation.

Congenital absence of the portal vein called a portosystemic shunt (aka Abernethy Malformation or liver shunt) causes blood to drain away from the portal system, and in turn the blood bypasses the liver.  In more recent findings however, there is new evidence which seems to point to the portal vein as being so tiny it does not present itself in regular testing (ultrasounds, cat scans and MRI’s), but shows up through radiologists entering into the jugular vein and making the discovery through that method.  Any blood that does bypass the liver and does not get filtered through it can cause toxins to enter the blood stream which may affect the normal function of the brain.  One such effect, common to portal hypertension patients is called hepatic encephalopathy.

I was first made aware of abernethy malformation when I began this blog a few years ago by Chris B., a mom from Minnesota whose daughter Maddi had it.  At that time I had promised Chris I would write about the condition for my blog, but to be perfectly honest I did not know enough about it nor had I come across anyone else with it, until just recently and so like many other things I unfortunately put it in the back of my mind.  Prior to that Chris started her own blog on the condition and because of  that has been able to help several families (numbering 16 in total) whose children also have been faced with the challenges of the disease.  Coincidentally Dr. Superina who is mentioned several times in this blog for his work with portal hypertension children, contacted Chris personally to say that her information was not correct and that he could help in the situation.    Through extensive testing Dr. Superina and his team of radiologists are credited with discovering that Maddi’s portal vein was indeed there but very tiny, and she did have a chance of being cured!  (Maddi is now 9 years old now, and is doing great.  She will be in Chicago soon for a follow-up appointment with Dr. Superina).  So the question prevails … are there others out there who have been diagnosed with abernethy malformation and the portal vein is so small it does not show up on regular scans, or maybe their portal vein really is missing?

Here is the link to Chris’ blog where you can read Maddi’s story and obtain valuable insight into Abernethy Malformation:  http://abernethymalformation.blogspot.ca/

Maddi and children such as her with this condition and of course portal hypertension need our prayers.  I am thankful and I know of many other parents who are also thankful for the dedicated work of Dr.Superina in Chicago in the area of portal hypertension and also Abernethy Malformation.  If you have just received the disconcerting news that your child has either one of these conditions I encourage you to seek his help (see below) if you have exhausted all means for finding a solution in your part of the world.

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

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 and was restarted on coumadin a year ago to help with the liver pain associated with this condition. The feeling was that thinner blood would flow more easily through the collateral veins that had developed and it did help with the discomfort. Of course it was pretty unnerving to know I had very thin blood with a history of bleeding varices but I was willing to try anything to try and get a normal life back. It was also found that my mesenteric and splenic vein had thrombosed but fortunately, my intestines never became ischemic. Today I went to my specialist for my annual checkup and Doppler Ultrasound showed normal flow through the portal vein – main, right and left! Had no idea after all these years that this condition would reverse itself but can only thank God and my doctor that it did. I can stop the coumadin and the nadolol and hopefully get the stamina back to start exercising again and, along with good nutrition, back to my old self. I tell this story to let you all know that it can happen, and not only in the acute stages. The clot was attributed to 9 months of hormone replacement therapy so my liver was healthy to start and luckily remained healthy throughout this whole process which I’m sure helped in the overall picture. My heart is with all of you, I know what you’re going through and hope and pray that my story becomes your story. Godspeed”.

So there you have it.  Up until about a month ago I had not heard of anyone with portal hypertension (PH) being completely healed of the clots and its accompanying symptoms except for another gal whose condition I recently learned had cleared up, albeit in the supposed “acute” stage of portal hypertension.  When I asked my specialist about this recently she confirmed that PH can in rare cases can clear up so the possibility is there, and beyond that my understanding was it was very unlikely.  With a fantastic outcome such as Janet’s I can only hope and pray that this would eventually be the case for many of you, your loved one, or possibly for myself.  Recent imaging I had done showed extensive congestion of arteries due to the clotting, and with that I have bowel ischemia so I don’t feel too hopeful sometimes, but anything is possible I guess so I’ll just keep praying and trusting.  

Keep the stories coming!

Clint

 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!   

 Pictured above:  (1st)Sonya the Bunny, (2nd) Alex Stang (Rex Shunt 2011) and Sonya – 2 little miracles (picture taken Nov. 2011), and (3rd) Sonya “Bunny” (Christmas in Chicago 2011).

To read more about Sonya’s story click the following links: 

http://www.portal-hypertension.com/2011/04/14/little-sonyas-plight-update/

http://www.portal-hypertension.com/2011/08/28/rex-shunt-for-sonya-update-08-28-11/

http://www.portal-hypertension.com/2011/08/31/rex-shunt-sonya/

http://www.portal-hypertension.com/2011/09/04/post_op_6_days/

http://www.portal-hypertension.com/2011/09/05/sonya-post-op-one-week-later/

http://www.portal-hypertension.com/2011/09/09/follow-up-help-for-sonya-post-op-2-weeks/

http://www.portal-hypertension.com/2011/10/31/little-sonya-2-month-post-op-update/

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!

Until next time,

Clint

Tatyana and Sonya in Chicago October 29/11

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: 

http://www.portal-hypertension.com/2011/04/14/little-sonyas-plight-update/ 

http://www.portal-hypertension.com/2011/08/31/rex-shunt-sonya/ 

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. 

I’ll keep you posted! 

Until next time, 

Clint

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.

 September 5, 2011

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 

September 4, 2011

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.

August 31, 2011 Update Sonya

“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,

Clint

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.

Until next time,

Clint

Sonya’s history: http://www.portal-hypertension.com/2011/04/14/little-sonyas-plight-update/ 

                                   http://www.portal-hypertension.com/2011/08/28/rex-shunt-for-sonya-update-08-28-11/

 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.

Here are a few articles which may be of interest:

http://blogs.wsj.com/health/2010/11/15/rocket-science-stacking-jjs-rivaroxaban-against-warfarin-for-atrial-fibrillation/

 http://clotconnect.wordpress.com/2011/07/01/xarelto-rivaroxaban-fda-approved-good-news/

The battle rages on!

Until next time,

Clint