Explanation of Fiona's Problems Right Now

This is very complicated. I will try to explain what I understand. Liver disease can cause 2 types of problems in the lungs: hepatopulmonary syndrome and pulmonary hypertension. If you have to have one you want hepatopulmonary syndrome as it gives you huge exception points on MELD. What hepatopulmonary syndrome is that the lungs start forming malformations between the arteries and the veins, so the pressure gets really high and the likelihood of bleeds become more of a concern. (Fiona has these) With hepatopulmonary syndrome, the key to the definitions of this syndrome is that even on 100% oxygen the blood oxygen levels are always reduced. (Fiona's are normal) So by definition she does not have hepatopulmonary syndrome. Now she has the AVMS (the malformations so something is going on but her oxygen levels are slightly lower on room air but totally normal on 100% oxygen). They did a right heart cath and the pressures in the heart the normal. Definition of pulmonary hypertension is that the pressures in the heart get really high. (Fiona's are normal) Also with pulmonary hypertension the arteries and veins, because of the high pressures start to scar so they don't work as well. With this you see an inbalance in oxygen/carbon dioxide ratios in the blood. (Fiona has this inbalance on exercise) She has shunting because of the AVMS which means because of the malformations in her lungs the blood is returning to the heart too quickly, thus not getting oxygenated well enough. (Could cause fatigue, dizziness, trouble breathing and chest pains -- Fiona has all of these symptoms.) But pulmonary hypertension can cause the same symptoms. Hepatopulmonary syndrome is directly liver disease related and with a transplant, if transplanted soon enough, will 100% resolve itself. Pulmonary hypertension will not improve with transplantation and is permanent damage to the lungs which causes permanent damage to the heart. They don't transplant these patients (some experimental transplants have been done but not at our center) because they don't feel the patient will survive the transplant and the lung/heart damage will continue. So here is where we stand. There is something really wrong with Fiona. She is experiencing disabling problems which we have figure out what it is. We are PRAYING for some weird form of hepatopulmonary syndrome! This next test she will have an angiogram (right heart cath) and will have to exercise to see if the pressures in the heart go up. If they go up she has exercise induced pulmonary hypertension, which means no transplant. If the pressures are ok, then it is probably some weird form of hepatopulmonary syndrome but now that has to be specifically proven--not an easy job. So where we stand is totally reliant on this next right heart cath. WE need to rule out pulmonary hypertension. That is a must. Then if that is ok, they will have to do a left heart cath to rule out a hole in her heart. If those are both normal, then we work on proving hepatopulmonary syndrome. If that can be "proven" then we can go back to the tx center and try to open her case again. Otherwise, with a MELD of 8, which she has right now, she is just between every rock and every hard place. She doesn't qualify for living relating or donor liver. She will just have to get sicker and this is truly a game a Russian roulette because she is getting sicker in her lungs and this could ultimately be the complete determining factor of her life. Now on a positive note, the liver doctor started her on a new med for high ammonia and WOW, we see a difference already. She has been awake all day today.....have my fingers crossed this continues, because if it does it may mean this is much more liver related and the symptoms we are seeing shouldn't be pulmonary hypertension....got my fingers crossed and talking to God all the time.