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Prophylactic therapy in order to avoid esophageal varices in case of portal hypertension?

Dear expert team!

First of all I would like to articulate my cordial thanks and respect for everybody of the team! I am very glad, that you have such a great human commitment and answer anonymous patient questions!

A short word about me:
I suffer from CF, I am 26 years old and have a very good general condition: I do a lot of sports, weigh 82 kg with 180cm height and I cannot be distinguished outwardly from a healthy human being. In the main focus of my CF is my liver: since I can remember I have a liver fibrosis (mainly at the bile ducts) and since 2001 also an enlargement of the spleen. 2012 the diagnosis "beginning liver cirrhosis" was made, that pulled the rug out from under my feet. However, in spite of this, my blood values are without any exception in the normal range (bilirubin: 0.3-0.7, pTPZ 27 sec.), borderline are only however the alkaline phosphatase as well as the number of thrombocytes (about 150 - 160). My CF-center is of the opinion that my blood values are excellent, therefore I am allowed to eat what I like (no alcohol is taken for granted).

Now to my concrete matter:
As until 2012 I did not engage myself deeply in the progress of my CF, as I was doing always fine, I caught up on this until today very intensively due to the "new" diagnosis of a beginning liver cirrhosis. Thereby questions came up my mind that concern mainly my portal hypertension (thrombosis can be excluded according to the ultrasound finding) and the possible development of esophageal varices. The latter could fortunately be totally excluded in the ultrasound finding from august 2012. However I am afraid, that the portal hypertension increases and collateral circulations are going to appear.

As I am a very active person and the wish for doing something is namely in my nature, concerning my liver and its portal hypertension, I would like not only to watch how it is doing collateral damage, but I would like to avoid the development of varices in the esophagus!

Is it advisable in my actual, shortly presented course of illness, to lower the portal hypertension with drugs, in order to aviod the development of esophageal varices and other collateral circulations??? Is that possible at all? I would very much like to do everything for this and get prophylactically active.
Are there any options and drugs for prohpylaxis of varices via reduction of the portal hypertension that I could propose concretely to my CF-center at my next visit?

For your efforts, your human commitment for all of us questioners cordial thanks!
Berst regards,
Dear Mr. M.,
please excuse the delay of the answer.
You have been diagnosed to suffer from a CF-related liver disease. As an indication for an increased portal hypertension until now an enlargement of the spleen/splenomegalie has been found. Esophageal varices have not been detected via ultrasound.
The laboratory testings showed thromboytes in the lower borderline area, the alkaline phophatase was silgthly increased.

The course of the CF-related liver disease is individually very variable and often only slowly progressing. The ultrasound picture looks often more dramatically as an investigation of the liver tissue would show. More helpful is the fibroelastography of the liver, with this, the amount of connective tissue is measured. Aim of the therapy is the prevention of possible complications of the portal hypertension.
In case esophageal varices as a manifestiation of an increased portal tension are already present, it can come to bleedings. This risk can be decreased. Via a gastroscopy not only the grade of the esophageal varices respectively the situation of the vessel stasis can be evaluated, with this, there is also the possiblity to treat possible esophageal varices outright with a so-called banding. This is the only sensible measure in order to avoid an acute danger of bleeding. In case there are no esophageal varices detectable in the gastroscopy, this investigation should be repeated every 2-3 years. The therapy with drugs in order to lower the portal hypertension is not recommended in case of CF anymore; there are no other prophylactic measures available. Some patients get a medication with ursodeoxy cholic acid. Unfortunately, the development of a portal hypertension can not be avoided, just as little as the development of esophageal varices.

In your case there is at the moment no hint for the existance of esophageal varices, furthermore, you have a very good nutritional status (BMI), both signs of a not very progressed CF-related liver disease. The progresseion of the illness over the years seems to be slow.

We do not at all restrict patients with a CF-related liver disease, we support them in doing their sporting activities, as this is i.a. sensible for the maintenance of the lung function. Patients with a CF-related liver disease should at least once a year be seen by a gastroenterologist/hepatologist. A gastroscopy should be performed at all patients as an initial medical finding. Optimizing of the nutrition with enrichment of polyunsaturated fatty acids, as well as a sufficient enzyme- and vitamin-substitution is recommended.

Best regards,
Dr. Helmut Ellemunter

On the topic, if the intake of ursodeoxycholic acid (UDCA) should be recommended to all CF patients with CFLD (Cystic fibrosis-associated liver disease), there is controversial discussion.
On the one hand, there are guidelines by Debray et al. 2011 in the Journal of Cystic Fibrosis (10 Suppl.2; S29-S36): "Best practice guidance for the diagnosis and Management of cystic fibrosis-associated liver disease", that can be cited the following: "...the objective of UDCA treatment is to delay the progression of the disease and so the treatment should be started as soon as the diagnosis of CFLD is made, altough there are no data on long-term outcomes such as death or need for liver transplantation..."
On the other hand, there is a letter to the editor from Nightingale, Durie und Freedmann in the same edition of the Journal of Cystic Fibrosis as the above mentioned guidelines, that can be cited the following: "The role and use of UDCA is controversial and the evidence for and against its use requires critical appraisal, especially in best practise guidance/guidelines written by expert hepatologists.
Simply put, at present, there are no convincing data to demonstrate that UDCA is efficacious or harmful in CFLD.
Future studies addressing the aforementioned issues are urgently needed before firm recommendations for or against the use of UDCA can be made."

In the clinical practice in Germany, for example, nearly all patients with symptoms of CFLD are treated with UDCA. The dosage can differ a lot, the individual response on this treatment should be controlled in any case (laboratory findings).

Dr. H. Ellemunter and Dr. D. d'Alquen