Patrick's fever finally broke overnight, and he hasn't has any fever at all today. He also finally got his system running, so they were able to put him back on feeds. Unfortunately, his x-ray this morning showed more atelectasis on the left side, so they decided not to extubate him just yet. Although they did chest PT quite frequently today, his 4:15 p.m. x-ray was much worse, so they increased the peeps on the ventilator and started him on pulmozyme to try to thin his secretions. On the plus side, his heart rate has been amazing today--120s, 130s, and even extended periods in the 110s, with sats in the upper 80s, lower 90s.

We met with Patrick's surgeon for about 1.5 hours today.  We looked at images in the cath lab showing the growth they had been able to achieve and where the problems were. Apparently, it isn't just the small size of his vasculature, but it's architecture--which is "tortuous." The surgeon indicated that although the PAH specialist doesn't think the PAH meds will achieve much, if anything, and he is also concerned about long-term quality-of-life issues with PAH meds that require IV access, the surgeon believes that, since Patrick already needs the IV access for the milrinone, there's no reason not to at least try them and answer that question rather than leave it on the table.

We are all in agreement that the short-term goals are to get Patrick off the vent, figure out what is happening with the fever, and seeing what benefits, if any, we got from the angioplasty which may be currently masked by his fever/vent discomfort, etc. He reiterated that, for him, the goal is to see Patrick thrive, without the need for milrinone. He defined thriving as Patrick growing, eating, developing and "being himself," irrespective of what any tests or readings show.

The surgeon admitted that he has struggled with "next steps," but that he would prefer we not take the transplant route with Patrick. He advised that, even if we get a donor, the mortality rate is something like 50%, and then there are still rejection issues and, even if there's none of that, there's so much medication, etc., that quality of life may be quite diminished for the whole family.

He explained that, from a surgical standpoint, there's really nothing left to do with the PAs.  He had hoped stenting would be available, but given Patrick's "tortuous architecture," there just aren't any good options. He does think he could do something with the conduit, however, because it is extremely dilated and the valve is very insufficient.  He believes (although he notes not everyone agrees with him) that part of the difficulty with the RV pressures and function is that the bovine patch is so dilated from the pressure that it's hard to see the actual RV. He noted that they might want to do a cath to check out Patrick's coronary arteries and make sure nothing is happening with them and that he would want a chest CT (or MRI--we don't quite remember) to check out the placement of the patch he put under the sternum before sending Patrick back in for surgery.  Phil and I have differing memories over whether he wants a cardiac MRI to try to determine what is bovine patch and what is RV. Either way, depending on what this preliminary testing showed, his preference would be to replace the conduit with a porcine valve conduit because it would not dilate from the pressures. He admitted, however, that he would normally never use a porcine valve in someone Patrick's age. He thought he might be able to get a 16mm conduit in there, which would translate to about 12mm for flow, which would be similar to the conduit he has now in that it was 13mm when they put it in and has dilated to 16, so there is likely enough room in his chest to fit the 16, and the 12 is similar enough to the 13 for flow size. He acknowledged that the conduit would not last as long as the one Patrick currently has, but the benefit would be that it wouldn't dilate from Patrick's high pressures. Thus, to the extent that Patrick is having problems caused by the dilated patch pressing on the RV, which in turn is pressing on the LV, it could relieve those issues. The downside is that all of the pressure would fall on the RV itself, which could cause the conduit to tear away and cause an aneurysm. Even with this risk, the surgeon believes this route is superior to the transplant route and he would not feel he failed Patrick, even in the event of catastrophic failure of the conduit.  

He noted that transplants are not good on an emergency basis, so that we would really need to pick one path or the other. That being said, he is willing to send Patrick's information to the heart/lung specialist.  It appears that he knows the other doctor and seemed more comfortable about our seeking another opinion when he realized to whom we were talking. He called us this evening to let us know he was getting Patrick's pertinent records copied and he had dictated a first draft of a letter to send all of the stuff to the specialist which he hopes to get out sometime next week. He said he would be overwhelmed if he got Patrick's records given how complicated Patrick is, and wanted to make certain that his letter helps convey a good sense of who Patrick is. He said he would give us a copy for our records once he sends it.

We know this is a lot to digest, as we are still working at it ourselves. Fortunately, nothing has to be decided right away.  As the saying goes: How do you eat an elephant? One bite at a time.  We are going to take our time to eat and digest this huge decision a little at a time. We certainly welcome thoughts and questions that are intended to help us move through this process, but please be aware that we do not believe that there is "a right answer," but rather, "an answer that is right for us."