damaged liver can be repaired

Question

I have a boy aged 7 years, diagnosed with CF at the age of 2 months .One year ago he was diagnosed with multifocal biliary cirrhosis, I was told that is the consequence of disease.
To understand that all those with CF sooner or later will get cirrhosis, diabetes ...?
I saw the final stage of cirrhosis is chronic hepatitis, cirrhosis now can lead to liver cancer?
For several months appeared in our country NEXAVAR for liver cancer, is useful for cirrhosis?
It is true that in patients with cirrhosis after liver transplantation chances of survival is only 1 year?
I recently read an article that ill liver may be regenerated (“rebuild”):
... quote the article: The damage caused to the liver due to excessive alcohol consumption or hepatitis can be "fixed", argue researchers from the University of California. Researchers argue that development of “scar" around the body can be stopped by blocking a protein who help their growing.
Excessive alcohol consumption and hepatitis lead to a process called fibrosis.
This process involves the formation of an excessive collagen tissue around the liver.
When this tissue is very abundant cirrhosis occurs. In the present available treatments are not only to stop spreading "damages" and not helping restoring the body.
For research have been used guinea pigs with severe liver fibrosis. They were divided into two groups. Some have received drugs that blocked the action of proteins that lead to fibrosis, while others received treatment classic.
Those who received drug blocking the protein have been cured of fibrosis and liver was totally rebuilt.
Those who received classic treatment only improved clinical condition
Researchers said that the treatment can be used to treat pulmonary fibrosis.
........... End quote.

You know more about these studies? These drugs have been developed or are just in the stage of research?
And I have a question.
Recently appeared in a pharmacy ESSENTIAL LIV-100 with protective effect an regenerating:
- Remove fat accumulated in the liver
-liver cell-activating
-regenerated liver cirrhosis
Ingredients: conjugated linoleic acid (CLA), L-ornitin cetoglutarat, adenozil S-methionine (SAME), methionine, dried extract of Schizandra chinesis, silimarina, vit. B1, B2, B3, B6, B12, folic acid, alpha-lipoic acid, chromium, selenium.
It may be administered to children over 6 years 2 / 1 capsule daily. Treatment should be given for minimum 60 days, 2 times per year.
Can I give my son this medicine?
Now he is taking: Liv 52 syrup, and Essential, Ursofalk .

Answer

Dear questioner,
You asked quite a few questions and we will try to answer the aspects as far as possible.
First of all you wanted to know if all CF patients get cirrhosis. Even if an involvement of the liver and the biliary tract is a common complication in CF, not all CF patients develop a cirrhosis. A relevant multifocal cirrhosis occurs in about 1% of babies and small children. The prevalence rises up to 24% in adulthood. A severe biliary cirrhosis with portal hypertension and an enlargement of the spleen occurs in about 3 – 5 % of CF patients.
Does cirrhosis lead to liver cancer? As liver cirrhosis presents a risk factor for developing liver cancer, it can not be excluded, that cancer may develop in a cirrhotic or damaged liver of a CF patient. There is no exact data on this available, reviewing the literature, there are fortunately only rare single case reports of CF patients having developed liver cancer so it seems a very rare complication.
The mentioned drug by you (NEXAVAR) contains the substance Sorafenib, which blocks a protein that leads to damage of tumor cells and vessels. It is a drug used in liver cancer and therefore not useful for CF liver cirrhosis.
In case a liver transplant is a therapeutic option, chances of survival after liver transplantation depend very much on the pulmonary status, too, and on the fact if the patient receives only a liver or a triple transplant (lung/heart/liver). Recent data from the UK report about 18 patients with CF who underwent liver transplant alone; 1- and 5-year actuarial survival rates were 100% and 69%.
The medical management of liver disease in CF includes optimising of the nutritional status, correcting deficiencies of essential fatty acids and avoiding antioxidant deficiencies that are common in CF, such as alpha-tocopherol (vitamin E) and beta-carotene. Ursodeoxycholic acid (URSO or UDCA) is accepted as a treatment for biochemical liver disease and has beneficial effect on liver enzyme levels. Studies that show long-term effects on the prevention of fibrosis are lacking.URSO may enhance biliary bicarbonate secretion and bile flow. Some studies have provided indications for positive effects of URSO on biochemical parameters indicating liver disease, the bioavailibility of dietary lipids and lipid soluble vitamins, and on weight gain which deserver further investigation.
You mention the drug Essential LIV 100: This seem to be a drug containing a variety of substances, like trace elements, vitamins, essential fatty acids and some extracts from herbs/plants. CF patients often tend to have essential fatty acid deficiency or an antioxidant deficiency, like vitamin E or beta-carotene, then it could make sense to replenish this deficiency. The mentioned drug however, contains a variety of substances, in which side effects are yet unclear and no studies are available showing a positive effect and no risk for CF patients. Therefore we can not recommend such not well documented kind of therapy.
You state an article about some drugs with a potential of curing cirrhosis. On the one hand it is hard to comment on the specific drug, as you did not provide the name of the substance they talk about in this article. What we can conclude from the article and from the substances we know that are available on the market is, that those substances are still in the phase of testing in the animal model, which is far away from being available to patients and market authorization. Therefore this would at the moment and the near future not be an option for liver disease in CF patients.
We hope we could have helped with this information.
Thank you for asking us questions and best regards
ECORN Romanian team

28.05.2009