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?
Connect with Clint
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Hi Clint,
One of the things I have learned the hard way is to take responsibility for as much of my health as possible. I will never not get my lab records again, and I research doctors, diagnoses, and meds for myself. I have also learned to ask pharmacists questions. But one side effect of PBC (in later stages) can be Portal Hypertension. That is what led me to your site. I am trying to educate myself in all aspects of this disease. By the way–for anyone who doesn’t know–Primary Biliry Cirrhosis is an auto immune disease that mostly inflicts women. It has nothing to do with alcohol. Thanks for again for sharing, and I will keep you in my prayers.
Helene
Thanks Helene,
Appreciate your comments and especially the clarification on Primary Biliry Cirrhosis. There seems to be some what I would call ‘main causes’ of Portal Hypertension, and cirrhosis is one of them, but specifically this type of cirrhosis as a cause it not familiar to me. I too am learning though, and when others comment on her it causes me to dig deeper. Hopefully someone else will see this post and possibly respond to it – shedding more light on the subject from a personal perspective and bringing our level of understanding on the subject even higher.
Thanks also for your prayers. I believe in prayer and as you may have detected in earlier posts it has really gotten me through 10 plus years of the unknown regarding PH. Like most who come on her, I will pray for you also!
Take care and come back often,
Clint
Hi Clint,
You said “In my particular case, portal hypertension was caused by thrombosis or clotting of the portal vein due to an apparently unknown genetic defect, which was thought to be some kind of protein deficiency.”
I am intrigued by this. Do you know any more about the genetic defect?
I have made a few extra posts today. I hope they will help someone.
Tony
Hi Tony,
As always I am grateful for your insight and appreciate the personal dimension you add by relating your journey on this blog. I know it has been a blessing in my life, and I always hope others are reading and learning in order to gain a better understanding into portal hypertension and it’s most often disconcerting side effects. Instead of replying to each of your last posts separately, I am going to wrap this up in one reply.
Firstly I want to address your question of a genetic defect as the cause of my condition. Your comments prompted me to follow-up with my hematologist yesterday regarding some results that were just made available a few days ago over blood work that was done while I was in the hospital the last week of August. When I called in I never expected to speak to my doctor directly, but fortunately he was there and took the call. Conclusively they still do not know the reason for my clotting. They ran a test looking for the Jak 2 mutation, but that showed negative and he told me that it is more common to find this in older adults (I am 52 years young). The same test was performed over 10 years ago when I was first diagnosed. Maybe from your medical background you could shed more light on this phenomenon?
The test also showed my ferritin levels at 385, which is high. Iron saturation was on the lower range of normal, but I had my quarterly iron infusion early yesterday so that would bring my numbers up, and that is where they want them to be. Bilirubin was high at 28 and normally should be under 20. For years prior to the discovery of my portal hypertension, my numbers have always been on the high side and there was some mention of the condition called Gilbert’s disease. So conclusively for me to say my cause is some kind of protein deficiency, I still can’t say and this is something I should have asked but forgot as I had just pulled off the road to take the call from a message I had left earlier.
Thank you for shedding more light on the cause of esophageal varices. I was not aware that acid reflux or hiatus hernias may also be subjected to varices from erosion. Very interesting indeed. This poses a question in my mind now, and I am wondering how many people actually get to this stage and have a bleed from these causes? I was also reminded of how important the use of non-selective beta blockers are vs. ones that keep the heart rate low. I wonder how many doctors around the world would actually know this, let alone patients themselves?
What a beautiful and historic place you live in (Cambridge). I visited the website you copied for Cambridge University – would love to have a tour of that someday. I have always wanted to tour the UK, and also Ireland as that is where my grandparents on my fathers side were from. Right now it is very cost prohibitive for me/us to go there, but only a dream at this point.
So Tony, I do believe that God has extended our lives for a purpose. Like you I hope my knowledge is passed on to my son, but he too has to learn from his own experiences in life. Through this blog, I hope that others (even after I have gone) will continue to benefit from the knowledge and experiences we both have written about here and not lost when we both die. For the life that has been granted I am grateful, and even today I was thinking about all the blessings I have where I live. As most people in the developed world know, people in other places around the world do not have the access to health care, and doctors there do not have the means or possibly the knowledge to treat their patients in the same manner as we have been treated. It would give me great satisfaction to know that others have benefited from this information and hopefully feel the empathy by the words we have shared. I believe part of the purpose my life has been extended has been to extol the virtues of a God who loves me (yourself included), and to testify to the fact our very lives are in His hands. The only thing I we can do is to share and pray for others who are on the same road as we. Make sense?
I was reminded yesterday of a familiar passage of scripture that I hope will be of benefit to you. It is taken from John 14:27 and says, “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.”
Thank you again for sharing. I like the style in which you have shared in a few of the paragraphs below, and that in itself makes me want more of what you refer to yourself as a “dreamy eyed romantic”. I pray that as you approach the round of tests you mention, that the Lord Himself will continue to preserve your life for many years to come,and you come back to this site to share your heart.
Bless you my friend!
Hi Clint,
I just stumbled upon this blog today after finally having the courage to research portal hypertension and I’m so glad I did. Thank you very much for putting together this site, it’s great to read other people’s stories and not feel so alone. I was diagnosed with cirrhosis in January this year and then it was later discovered that I had a complete blockage of the portal vein, doctors say I was born with it which apparently is quite unique. I’ve never had any bleeds but am riddled with varices, fortunately none have grown in my stomach however so I’ve been deemed a low-risk for bleeding.
I’m 28 years old, my mother passed away in June from cancer and my husband left me because I was tired all of the time so dealing with the ramifications of this illness has been challenging. I first went to my GP 2 years ago because I was exhausted, confused, nauseas, losing weight, jaundiced, constantly sick, having twitches and always needing the bathroom. They kept sending me for kidney function tests and eventually a year later accidentally caught sight of my liver in a CT scan. Since then I’ve been told the right side of my liver is completely dead but the left side has grown ‘extra muscles’, I have so many varices doing scans is difficult, my veins run back to front (blood goes in when it should flow out and vice versa), my spleen is enlarged and the vein from that is pushing into the wall of my stomach, but all of my liver function tests and blood tests come back completely normal. I’m on beta blockers for the hypertension but that is all and apart from fainting spells most of the time I’m pretty healthy I just get a bit tired, have stomach discomfort (with the occasional extreme pain), and still go to the bathroom a lot but I’ve always been this way so I’m quite accustomed to it. I have been told never to have children though which gets me down, it’s hard to keep up with my friends sometimes because I lack energy and work becomes difficult when my brain decides to be foggy (not ideal when you’re a high school teacher).
I’ve found this site really helpful so thank you once again. It’s really hard to find information out about portal hypertension and when you do it’s not very promising but I figure if I survived 27 years of it without medical treatment surely I can survive many, many more with…
Hi Krystal,
You really have been on a journey and my heart goes out to you for what you have endured in your young life. I am going to refer you to a Facebook site in case you haven’t seen it yet, which is called Portal Vein Thrombosis and Portal Hypertension in Children and Adults. I am a member of this group as are over 100 others, and there you will find other people with the same condition or parents of children with portal hypertension. You will additional information plus many empathetic ears from those on the same journey as ourselves.
Thank you for your bravery, I will pray that your health will stay stable, and that you have a long and healthy life despite what you know.
In your corner,
Clint
Hi Clint,
Thank you for your help and support. I will certainly check out that Facebook page. I hope all goes well for you too.
You’re in my prayers.
Krystal
Clint-
I was recently diagnosed with Portal Hypertension and Esophageal Varices- I have good Drs but they haven’t offered too much encouragement other than to tell me if I have a bleed, contact them immediately-
I read an article about a drug Sorafenib which sounded encouraging but the latest study I could find was in 09 and nothing since then. Do you have info about this drug?
have you had banding done for the varices?
Just kind of depressed about the lack of good knowledge about these disorders.
Any insights would be appreciate-
Sissy
Hello Sissy,
Thanks for posting. I am sorry to hear about your recent diagnosis. Portal Hypertension is a very disconcerting condition, and unfortunately there are no quick fixes to date.
I have heard of Sorafenib, but you are right there has been no further word on this since about the time you stated. The drug is primarily used to treat cancers of certain varieties but even that I am not sure how commonly it is used for that disease.
Since my diagnosis in 2002, I have done quite a bit of research, not to mention picked the brains of many doctors along the way, and the consensus is that the condition can only be controlled with beta blockers and blood thinners. If a patient’s blood pressure is kept low with the beta blockers (nadalol is what I am on) then it lessens the risk of bleeding in the esophagus. When a clot forms such as it does in the portal vein, it is quite rare for that to be eradicated or even dissolve in that area unless in the very acute stage of discovery. Blood thinners such as Coumadin or Warfarin are used to prevent further clotting. For myself Coumadin does not work, that is why I have to take injections of a thinner called Fragmin (dalteparin sodium), however from what I understand from my connections on this blog and from my physicians this is very rare. As for a bleed from my esophagus, I have only bled from the varices in the upper part of my stomach back in 2004. I am at high risk to bleed as the varices I have are at the highest grade to rupture, however I have been spared for over 10 years from this happening, and this I attribute to the medications, healthy living, and prayer.
There is much information written on this blog, and I always have one ear to the ground as far as new medications or treatments are concerned. I can understand you feeling the way you feel, but please know that I know of many others besides myself who have lived with this condition for a long time, and they continue to lead relatively normal lives. I would encourage you by saying that I have had moments where I felt complete despair about this, but those times are extremely rare now, because I have lived so long. It may never be the case for you, but in my case doctors do not want to operate on me for anything as the risks are extremely high. I do know of others who have had operations and procedures and they are still thriving today. So in summation, I encourage you to not give up hope, but come back here often, if only to ask or post a question and I will do what I can to help you along the way. You can subscribe to my page or like it on Facebook and you will receive any new postings I make on the subject.
Take care, Clint
Hi Clint
This is all new to me. I was diagnosed with Non Cirrhotic Portal Hypertension caused by a portal vein thrombosis with (subsequent cavernous transformation) a year ago. What does subsequent cavernous transformation mean? I have a specialist I see at the University of Iowa City, Iowa. He said that this may have occurred due to bacteria in the umbilical cord at birth. I do not see him again for two years. I am on a low dosage of Nadolol (20mg) as the higher dosages made me light headed and dizzy. I also see a gastroenterologist once a year. Apparently the varicies in my throat do not meet the criteria for banding. (although I thought they were a grade 3) It is my understanding that a ton of research is being done on this rare disease or condition but still not much known about it. I was diagnosed wrong in the beginning because of the throat varicies. The doctors thought it was cirrhosis of the liver. I had a biopsy done and discovered my liver is healthy. All this was found because I had an ulcer due to H-Pylori bacteria. Does it seem that the doctors I see are on top of this? Any words of encouragement or knowledge would greatly help. I am pleased I found your web page. Thank-you, Evie
Hi Evie,
Thanks for your post. Sorry to hear of your diagnosis, but with that I hope I can offer you some encouragement.
First of all, subsequent cavernous transformation simply put is the re-canalization or re-routing of the blood through secondary arteries around the liver in which the portal vein used to carry. These arteries take on extra blood flow and so become engorged, and in the case of the veins (varices) on the wall of esophagus they are what is the most dangerous aspect of the condition. Fortunate for you they do not need banding right now and you are on a relatively low dose of Nadolol, which is not the case for me as I am on 160mg of Nadalol and I have grade 3 varices. They will not band mine as the fear of further bleeding from the ulceration that occurs after the varices falls off is great because of the high dose of thinners I am on. I have lived 11 years beyond my diagnosis and have had a few close calls with clots to my lungs and a new clot developing a year ago below an existing clot in the superior mesenteric vein. I honestly believe I am still alive because of prayer and actual medications I am on to control any bleeding. Your doctor may be right in saying yours could have developed in the umbilical cord at birth as this is found to be so in some children if caught. Yes there has been much research in this field, but no really great breakthroughs except for the treatment to control potential bleeding. Beta blockers such as Nadolol are pretty much the norm the world over, as far as shunting is concerned it usually works well for children under the age of five if they have no other complications because they do not develop atrophy as older children or adults would. Much of the research on PH has been done at the University of Barcelona in Spain, but there are other researchers who have contributed also. My gastroenterologist had specialized training in Barcelona, and she also trained under Dr. Guadalupe Garcia-Tsao at Yale University who is renowned for her research in the area of portal hypertension. See: http://medicine.yale.edu/intmed/people/guadalupe_garcia-tsao.profile
Well I hope that helps? I know it may not all seem encouraging, but given what I stated above concerning the low dose of Nadolol you are on and the fact they do not deem it necessary to band you, I think you have good prospects to live a relatively normal healthy life. Faith along with prayer has helped me tremendously and for the most part I do not dwell on what could happen, as my life is in God’s hands. PRAYING FOR YOU ALSO!
Clint
Hi, I was just reading through everything and hoping to get in contact with Krystal. My teen daughters symptoms sound so similar to hers. She is exhausted and has mental fogginess and passed out in school last week. The doctors dont think her passing out has anything to do with her abernethy malformation. But from what I read Krystal has feinted several times? any info. would be great. I am also looking to talk to parents with kids that have this problem. My daughter Jadyn is scheduled for surgery with Dr. Superina soon.
Thanks,
Kristin
Hi Kristin,
Thanks for your inquiry. I was going to direct you to a Facebook page called Portal Vein Thrombosis and Portal Hypertension in Children and Adults, but Troy has posted there already. I would also recommend you open the page on my blog called Missing a Portal Vein where I have posted a story on a little girl called Maddi who has dealt specifically with Abernathy Malformation. Her Mom has created a page by the same name discussing the condition. I believe Maddi is doing well now, so that is encouraging. Although I don’t have Abernathy Malformation, I have lived 12+years with portal hypertension and I do know it can be controlled. Please PM me if wish, and I will provide my phone number if you wish to talk further. In the meantime my thoughts and prayers are with you and your daughter concerning this situation. Clint