My Portal Hypertension Journey

This blog was created for those seemingly uncommon few (like myself) who are living with this often discouraging condition called portal hypertension. Through this “work in progress” I hope to encourage, inform, and provide a comprehensive forum for fellow sufferer’s, their family members, or their friends. In my personal quest to find others struggling through the side effects of this disease, I came to realize that there really wasn’t a one-stop site that provided all the things one might be looking for when dealing with a disease of this nature. All this being said, I want to extend a heartfelt welcome to you and most of all I trust you find information and solace from within. You are not alone in this journey!

Who are you?

My name is Clint Steenson, and my personal journey with Portal Hypertension began in the summer of 2002. Within months after starting a new business, the loss of my father, and 4 trips to the emergency ward of 2 local hospitals, I was shocked to learn that I had Portal Vein Thrombosis (PVT). After a week in the hospital I spent the next several weeks recuperating. Providentially for me, I was re-hired by my former employer, who I had worked for prior to starting my business to be their national sales representative. After a little over 2 year’s of employment and virtually no symptoms, I was re-admitted to the hospital in the early part of December of 2004 with severe pain and bleeding. It was then discovered that I had a clot to my superior mesenteric artery which explained the pain, and the bleeding at that time was from varices in my stomach. Again I spent another week in hospital, was released and took the next few week’s off work. I returned to my employment in January of 2005. After only 5 weeks back at work, I took ill again and was re-admitted to hospital with pulmonary embolisms (clots to both lungs).

As one may expect I take a boatload of medications which is necessary to stave off further clotting, and relieve pressure to areas in danger of bleeding due to the existing clots. It is suspected that my condition was started by an underlying genetic defect and at the time there were only 7 known defects, however all tests were returned negative. The most common and yet deadliest side effect to portal hypertension is a condition called esophageal varices. Varices are formed when arteries on the wall of the esophagus protrude inside because of pressure from the clot. Varices are graded on a scale of 1 to 3, with 3 being the most likely to bleed. Gastroentrologists can discover and grade the varices while doing a gastroscopy. In my case, my specialist discovered that I had 2 columns of grade 3 varices – grade 3 being the most deadliest. At the time of my last scope, it was determined that it was too dangerous to even check the condition and all future scopes were discontinued.

In October of 2007 my doctor’s strongly advised that I stop working as that would take much stress off my life. I heeded their recommendations, and fortunately was able to obtain long term disability benefits from my former employer, plus a government medical pension from which I understand is most difficult to get unless one has a condition such as I. As one may imagine my medications are very expensive so I am extremely grateful that the costs are covered by a plan, otherwise I do not know where I would have been without it? Of course there are the daily challenges of raising a family on a limited income, but I am still grateful for what has been provided and I carry on despite all this.

In summation, my life is filled with seemingly endless blood tests, iron infusions, doctor’s appointments, and all too frequent bathroom trips – not to mention some associated pain! Despite my condition I remain very optimistic and for the most part don’t dwell much on the negative aspects of this disease. I believe God has spared my life, and I have peace about the future. What is your story?



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{ 9 comments… read them below or add one }

Dominic Benson May 2, 2017 at 10:48 am

Hi Clint,
My daughter was diagnosed with PVT on 15 Feb 2017. The Doctors tell me this was secondary to appendicitis infection. My daughter was put on warfarin for 3-6 months with no other treatment. In April 2017 my daughter went for a scan and we are told the main portal vein is clear now . Also the left vein is clear , main portal and both right and left veins were clotted to begin with. The right vein is still blocked after 6 weeks on warfarin. I am told the liver and spleen are normal. My daughter has had no pain at all because of the clot but some small varices have appeared around the blocked right portal vein.Doctors advise me she may have a touch of PH but not severe because she’s got a good flow to her liver. Sometimes I am at a lost when talking to Doctors about this. Can you give me a run down on the situation, thanks Dom.

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Linda January 18, 2018 at 10:59 pm

Hallo Clint,

I just found your Website and I’m looking very much forward to go through all the information you’re providing here. Thanks a lot for that! It’s very hard to find other patients with this diagnosis and places to share stories and questions.
I am from Germany and so far I couldn’t find something similar in German so it seems like I’m going to improve my English on this topic…

Well about me, I’m 21 years old and my portal vein thrombosis was discovered very early when I was only 5 years old coincidently.
I always had some problems with my digestion and when my parents went to a doctor to check out what’s wrong they found an unusual low amout of thromocyts and went on looking for reasons. I barely can remember this, my diagnosis just has always been a part of my life.

We went to a clinic specialized on liver diseases simply because at that age this was a very uncommon thing. Back then I was too young to intervene so they waited how I would develop living with it and only checked me once a year.
When I was 13 there was the possiblity of laying a shunt to bypass the thrombosis but my doctor recommended not to do it as I was a very healthy child. I did a lot of sports, was good at school and had almost no limitations resulting out of the PVT.
Since then I regularly need to controll my two oesophageal Varices grad 2 and 3 which never bleeded so far.
I have a quiet big milt causing some slighly painful pressure on my stomach sometimes but except this the only think affecting my life is my knowledge of a possible bleeding and and fear my condition might change one day.

I think my body learned to deal with the circumstances and I hope in a way which is going to work out for the rest of my life. We’ll see.

Best wishes

Linda

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Clint January 22, 2018 at 6:07 am

Hello Linda,

Thank you for joining my blog. I am sorry to hear that at such a young age you are dealing with this condition. That being said, I have lived 16 years since my original diagnosis back in 2002. The treatment protocol seems to be similar the world over, but I know there are places and people who know nothing of this condition or how to treat it. I try to keep in the know about PH as much as I am able, so I do rely on others to tell their stories in hopes we all can benefit from what they have learned.

Keep your head up and please feel free share anytime.

Best wishes for a long an healthy life!

Clint

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Isa January 26, 2018 at 5:21 pm

Hello Linda!

We are also in Germany and we have an 8-year-old kid with portal hypertension. Maybe you would like to write to us?

Best wishes
Isa

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chinthaka March 19, 2018 at 7:21 am

Hi Clint,

As I have something similar to Linda, sharing my story with you might help for all to find a better cure.

About my self, My name is Chinthaka and now I am 31 years old. I experienced my first esophageal bleeding when I was 6 years old (1992), and thereafter once a year up to 1994. Then after that, I received sclerotherapy treatment from 1995 twice a year (sometimes only the endoscopy test) until I became 18 years old. Meantime doctors did not attempt to do any surgery as I was young and they expected I would become better with time.Thanks to them until now I did not experience any bleeding events and was spending a normal life. However, as far as I remember I have an enlarged spleen and low blood platelet count.

Currently, I am doing my postgraduate studies in Japan. As I had low blood platelet count, Japanese doctors also did several tests and identified and informed me that I have an extrahepatic portal obstruction, splenomegaly and splenic aneurysm. They recommend me to do remove both spleen and the aneurysm using surgery. Then I consulted my doctors in Sri Lanka and they said meso-caval shunt surgery as the best option. Since I have not decided anything yet, I am looking for an opinion from an experienced doctor in this area.

I would like to hear any idea from you.

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Sergii June 27, 2018 at 9:06 pm

Hello Linda
You described a common history of PVT due to (likely) intestine infection or even covert version of acute append. Liver function is good in this cases but variceal bleeding is a long life threat. Blood flow restored in left branch is a promising feature but you should monitor trombocytes count and spleen volume every 6 months, US Doppler with flow velocity evaluation of splenic artery and splenic vein also strictly recommended. Sometimes we recommend RI procedure – splenic artery embolization (SAE or PSE) to decrease portal pressure and increase trombocytes count. It is safe and effective so you have one more treatment strategy to discuss with your medical advisors. Best wishes, Dr Sergii

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Brooke July 2, 2018 at 4:58 am

Hello,
I’m so thankful to find this site! We just found out that my 2 1/2 year old son has PVT with cavernous portal transformation. We live in the Panhandle of Florida (North Florida). He was a normal birth with no NICU issues, and deemed healthy. The only issue we thought he had was reflux as a baby. Long story but we would always notice bruising on his sins when he was younger but right before he was 2 he had an ear and throat infection and while on antibiotics I realized he still didn’t feel well. Went to the doctor and he had pancytopenia, splenomegaly and deemed to have mononucleosis. Given liquid iron for anemia and blood work repeat showed trending labs toward better and end of story…so we thought.
Back in April (2018), I was pouring him to bed when I could feel his spleen. The next night I lifted his shirt and could see it clearly. So of course a visit to the peds revealed what we thought….Peds dr sent us to the ER with Hemo-onc dr waiting for us. Never good when they are shocked at how large his spleen was….diagnosed with massive splenomegaly and pancytopenia…bone marrow biopsy ruled out leukemia and all tests for infectious disease, Doppler flow, hematology came back negative. Dr was unsure and felt that it was a genetic component and possibly lysosomal storage syndrome. So off to UAB in Birmingham, Alabama for genetic testing. All tests were negative so by chance our local Hemo-onc completed CT of chest, abdomen and pelvis to rule out lymphoma…and this is where his PVT diagnosis came in.
They referred us back to UAB GI/liver Dr. He is from Cleveland’s Children hospital originally and he basically said “let’s see and wait….”
He explained the reasons and I somewhat get it but find it hard to believe no endoscopy, and I’m supposed to just let an almost 3 year old run around with a massive spleen and platelets anywhere from the 40’s to 80’s depending on the labs and just wait for something to happen?!!!
Thankfully he has never had any bleeding but he does complain at times of his stomach and throat hurting. Looking for advice and recommendations from anyone for the specialist that has seen/treated the most cases (successfully) in the United States….I’m looking for a second opinion at this time and if they also say let’s wait and see then I can try and live with that…
Any advice, things to ask, look for highly welcomed. This is all completely new and raw to us…
Thank you

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Clint July 2, 2018 at 6:22 am

Thanks for sharing your story. I’m sorry to hear about your little guy’s condition. I suspect that they don’t want to do an endoscopy on him due to the fact he may have varices and they don’t want to cause a bleed from the test itself(?). I’m no expert by any means, but I want to assure you there is help out there for him. From this blog and another one I belong too, many children have received help from Dr. Ricarddo Superina in Chicago. Here is the contact information:

Lurie Children’s Hospital
225 E. Chicago Avenue
Chicago, Illinois 60611
(800)543.7362 (1.800.KIDS DOC)
Phone 312.227.4000

There are also several other specialists you can try and you can see that list under the page on this blog called The Rex Shunt Specialist List. I can even put you in touch with some Moms whose kids have been treated and are doing well today.

For older patients such as myself the options are limited, but I am thankful to still be alive beyond my diagnosis almost 16 years ago. More importantly though is your little one and I believe you will gain reassurance from them and the options available.

Here is the Facebook page where you can find more information from parents or patients themselves regarding this condition. Both Stacey Friedman and Jen Grelish-Temple are the moderators and you can tell them I am referring you or any others reading this post.

Let me know if you have any more questions. Look forward to any updates you may have.

Thoughts and prayers,

Clint

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Jennifer norman July 25, 2018 at 3:03 am

I can’t believe I finally found someone else with PVT history. It is so difficult to find any information on the this with positive outcomes. I had stomach surgery in December 2017 and two weeks later, developed a PVT. Six months working with a hematologist, taking blood thinners and the clot did not clear. The portal vein is toast and won’t reopen. I recently saw a hepatologist and he has scheduled an endoscopy to check the collateral veins (varices?). I was tested for factor Leiden V before my surgery because my mom and sister have it. I tested negative but doctor feels like the surgery in conjunction with something else caused the PVT. I can’t test for any other genetic blood diseases because I’m on warfarin and I guess that interferes with results. I am being tested this week for liver function. He showed some concern during my exam and suggested it might be my liver that caused the problem. I saw pictures of my liver during my surgery and it was gorgeous and red. I hope that the PVT and inflammation has not caused any damage. Needless to say, I am freaked out and in the waiting place now. But now I know other people exist with this and I’m not alone. Thank you for this blog.

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