In My Shoes: Liver Appointment Today

Wednesday, August 20, 2008

Liver Appointment Today

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Saw the liver doctor today and he, along with us, are just waiting to see what UCLA decides this Friday. We asked him if he had other BA patients like Fiona and he said no. He said, in fact, the problem she is having with her lungs he has only seen 2, one being Fiona. This is really very rare and difficult. He was very pleased to hear that UCLA pulmonologist is going after getting exemption points. That really pleased him.

The test results are difficult to know exactly what they are. He let us read the reports and they were basically pretty darn vague. The reports, but more importantly, the films were all sent to UCLA. The MRA of the brain showed no AVMS, no changes seen since the MRI done 2 years ago. HOWEVER, there are brain changes due to LIVER DISEASE. YES, liver disease causes changes in the brain....Ok this is the first I have heard of this. She has mineralization in the globi pallidi area typical of chronic liver disease.........um.......see pictures below.

The report from the MRA of the lungs stated only seeing the embolized AVM and no new LARGE AVMs visible. There was nothing seen in the left lung. However, her pulmonary artery is larger than the aorta, which was not mentioned before. Don't know still what they feel is birth defect and what is liver disease. Again the films were sent along with the very vague report to UCLA.

Fiona is getting some mild ascites now which we knew but the doctor finally saw. She discussed with him her sensitivity to cold and he said that is part of the liver disease as well. Gosh, there is so much related to liver disease we didn't even know about.

We asked how many young people he had as patients and he said he had a few BAs, a few autoimmune heps, and a few Wilson's disease but none present like Fiona. She is just totally unique and he is learning from her. He is hoping UCLA will accept her and help with her care as it is getting very complicated and having another medical team on our side would help.

The symmetric globus pallidus is typical of carbon monoxide poisoning. Liver disease may cause T1 hyperintensity also. Hypoxic injury could have a similar appearance. (They saw mineralization, along with the T1 hyperintensity in Fiona's brain where the arrows are.)

I think globi pallidi is where the 2 whiter sections are towards the top or front of the brain......not positive and will do more research on this topic. Not sure what the consequences of this is and if it will reverse when and if she has a transplant.

Peggy

PubMed article on this subject:

http://www.ncbi.nlm.nih.gov/pubmed/9228107

Brain MRI changes in chronic liver disease.Skehan S, Norris S, Hegarty J, Owens A, MacErlaine D.
Department of Diagnostic Imaging, St. Vincent's Hospital, Elm Park, Dublin 4, Ireland.

Cirrhotic patients are known to have abnormally high signal principally in the globus pallidus on non-contrast T1-weighted MRI. The purpose of this study was to relate MR changes to clinical and pathological features of chronic liver disease. We confirmed abnormally high signal in the globus pallidus on T1-weighted images in 25 of 28 patients with chronic liver disease, showing that it also occurs in patients who have not yet progressed to cirrhosis. Changes were seen in patients both with and without clinical portosystemic shunting. This abnormality is not responsible for hepatic encephalopathy. Cholestatic disease was more likely to produce marked changes than non-cholestatic disease. No statistically significant correlation was demonstrated between the severity of liver disease and the degree of MR abnormality. However, marked improvement in MR appearances was seen after successful liver transplantation.

PMID: 9228107 [PubMed - indexed for MEDLINE]

Well that gives me hope that once she is transplanted her brain will return to normal.

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Biliary Atresia

Biliary atresia is a condition in which the ducts that carry bile from the liver to the gallbladder and small intestine are not normal. These ducts can be injured, blocked, or missing. When the ducts that carry bile out of the liver are closed, bile backs up and damages liver tissue. In time the liver can develop scarring or cirrhosis. The gallbladder may also be abnormal or missing. The cause of Biliary Atresia is unknown. It occurs in one infant in every 15,000 live births. It is a serious disease which requires immediate surgical and medical treatment.

Arteriovenous Malformations AVMS

I have been diagnosed with AVMS in my lungs do to my liver disease. These have caused me to have 2 lung bleeds which were very scary. I still have more AVMS and they are causing me breathing problems. Below is an explanation of what the AVMs are and a picture of what is happening in my lungs.

Arteries and veins are part of the human cardiovascular system. Normally, the arteries in the vascular system carry oxygen-rich blood at a relatively high pressure. Structurally, arteries divide and sub-divide repeatedly, eventually forming a sponge-like capillary bed. Blood moves through the capillaries, giving up oxygen and taking up waste products from the surrounding cells. Capillaries successively join together, one upon the other, to form the veins that carry blood away at a relatively low pressure. The heart acts to pump blood from the low pressure veins to the high pressure arteries.
If the capillary bed is thought of as a sponge, then an AVM is the rough equivalent of jamming a tangle of flexible soda straws from artery to vein through that sponge. On arteriogram films AVM formation often resemble a tangle of spaghetti noodles. This tangle of blood vessels forms a relatively direct connection between high pressure arteries and low pressure veins.
The result is a collection of blood vessels with abnormal connections and no capillaries. This collection, often called a nidus, can be extremely fragile and prone to bleeding.