I received this e-mail from my wonderful friend Moreena this morning. Thank you, again, Moreena, for taking the time to write this. As you read this, you may wonder what exception points are. When you have a secondary condition develop (HPS - Hepatopulmonary Syndrome) from your primary liver disease, its time to look at transplant, and, most likely, a person will get extra "exception" points that will put them higher on the transplant waiting list.

Here's what I remember:

In order for exception points for HPS, you need to have three things:
1--liver disease (check!)
2--positive bubble echo (check!)
3--low oxygenation

#3 is tricky, because a regular pulse oximeter reading doesn't do it. You have to have a low arterial blood gas. In order to check that, there has to be an arterial blood gas drawn, which is super-duper, extra-yucky painful (Grandparents: warm up those credit cards!). They might not want to put Shelby through an arterial blood gas draw until they start seeing a drop in the regular thumb or index finger pulse oximeter readings, but maybe they'll check it just to be sure. Annika's was always low, but never low enough to qualify for the diagnosis of HPS. And those draws were pretty much the worst, like 50 I.V.s being put in at once. Awful, awful, awful.

Chicago also had Annika do a nuclear test, the tagged albumin thingamabobby. This test is super-accurate for diagnosing moderate to severe HPS, and it's super easy. I don't even think she has to have an I.V. for that one (my memory is that they just use an injection, but maybe I'm misremembering). I bet they'll have Shelby do that one, too, since it's so sensitive to HPS and not at all painful.
Here's the lowdown, as I remember it, on a positive bubble echo. A positive bubble echo tells you that, for sure, something is going on. Shelby's blood isn't all taking the correct path through her heart and lungs. That is, blood comes into your heart on the right side, gets pumped into your lungs to pick up oxygen, and is then sent back to the left side of your heart, and then is pumped out of your heart to carry oxygen out to be used by your body.
A positive bubble echo tells you that, somewhere along the way, some amount of blood is going through some alternative pathway. However, it doesn't tell you where or what kind of alternative pathways are being taken and it doesn't tell you why the alternative pathways formed and it doesn't tell you how severe the problem is. When the problem is severe, though, you'd definitely know it because you'd have a lot of blood not getting properly oxygenated by the lungs, which would lead to Shelby being out of breath a lot (like when just sitting down or walking a few steps), and it would show up as lower numbers on the pulse oximeter. And she'd be needing oxygen all the time.
With HPS the problem is that the liver disease causes some of the vessels in the lungs to go wonky. The reason that they look at the heart (with the bubble echo) is that when you have problems in the lung vessels, you'll start seeing effects back at the heart, too (that is, you'll start seeing blood taking alternate routes to get around the problems in the lungs). I'm assuming that it's easier to spot these problems in the heart, rather than looking directly at the lungs themselves (the blood vessels that are going wonky could be really small). But, again, I'm just assuming that's why they look at the heart to diagnose a problem in the lungs.
In the setting of liver disease, without any underlying cardiac or pulmonary issues, it's pretty well assumed that a positive bubble echo means that the liver disease is beginning to affect the lungs. This is a big deal because compromised lung function (while usually reversible after transplant), makes the transplant that much more risky. Problems with the lungs would definitely be a reason to consider going ahead with the transplant, even if the liver is still cranking along doing its thing, despite the liver disease, because you don't want to wait until the lungs are a mess and then do a big major surgery.
For the purpose of exception points, though, HPS isn't diagnosed on the basis of a positive bubble echo alone, because of the non-specificity of a positive result.
I really don't know what Chicago will do with those results. I know with Annika, they still held off with transplant even after the positive bubble echo, and even after an abnormal lung CT, and even after the lung biopsy showed fibrosis in her lungs. She never fulfilled the criteria for HPS because her oxygenation, while somewhat lower than normal, never got low enough to fit the criteria. Her tagged albumin scan was also negative. They knew something was going on with her lungs, but they had no idea what, exactly.
With Shelby, though, they might take this as a sign that it's time to transplant. Shelby, after all, doesn't have a mess of surgical adhesions inside her abdomen making the surgeons push to hold off on surgery. For her, transplant right now might be the best option, especially after all those weird infections she was getting last winter."