Today was overwhelming for us. So much information to process and such tremendous decisions to make for Sam. It is hard enough to make decisions that only impact your own life; but when you have someone else depending on you to wade through the mounds of information, listen to several opinions, and then make a decision that will ultimately affect the quality and length of their life; where do you begin?
Sam needs his TPN (total parenteral nutrition) in order to survive. Without it he would not get the necessary nutrients that his body needs to live and grow. The problem, which is not uncommon for TPN dependent individuals, is that his lifesaving TPN is also damaging his liver. They believe that this may be caused by the Intralipids that are added to his TPN. This is where his fatty acids and a good portion of his calories come from each day. Sam's Direct bilirubin (a product that results from the breakdown of hemoglobin) has been slowly creeping up. On top of this, his liver enzyme reading have been increasing as well. This all means that it appears that Sam has begun to experience some level of damage to his liver, which could be very minor at this point, but it will most likely continue to get worse.
We basically have three options available to us right now:
1) Do nothing. See what happens. Maybe the changes in his levels will stabilize and maybe his liver damage will never become severe. If it does and his liver begins to fail, put him on a transplant list. In defense of the person who presented this option, they were just trying to present all of the options and even they do not think we should go this route.
2) Adjust the makeup of his TPN. They can reduce the amount of intralipids in his TPN and replace the calories he would be missing with extra glucose. This sounds simple. Why don't they just do this with all TPN patients and lessen the risk of liver damage? The problem is that excessive amounts of glucose being pumped directly into a persons blood stream can cause hyperglycemia. Let's fix one problem and create another whole set of problems while we are at it. (No anger there, just pointing out what we thought was an obvious flaw in that plan).
3) Adjust the makeup of his TPN. Reduce the amount of intralipids that he receives and replace the calories with a amino acid derived from fish oil called Omegaven. WHile this method is not FDA approved, it has been widely used in Europe, and there are patients receiving Omegaven in the US. So far there have been no reports of any sever adverse reactions to this treatment and in some cases it has corrected some of the decreased liver function that was caused by the Intralipids.
Can we get Omegaven here? The answer was - shockingly - yes. They can have it here and the protocol set up and get FDA approval to administer it to Sam in Milwaukee in 6 to 8 weeks. This was amazing news, we thought we were going to have to go all the way to Boston. Oh wait, there is a catch. Children's Hospital of Wisconsin will only bring in Omegaven if our health insurance will cover this non FDA approved treatment that is considered investigational. They will not be able to fund this for us because if they did it for him, they would have to do it for everyone, and that would be too expensive. Although disappointing, we can certainly understand this, this treatment is not cheap. You will be shocked, I am sure, to learn that our health insurance company will not, in fact, cover a treatment that is not currently approved by the FDA and is considered investigational.
So it appears that will be be making that trip to Boston after all. Dr. Puder's group at Children's Hospital of Boston has the ability to use this treatment and cover it's cost if that is needed. Unfortunately we were also informed by our insurance company that they also will not cover his hospital stay, doctor visits, lab work, etc... if we choose to go to Boston. Apparently it would be more beneficial for them to pay for the liver transplant in a few years and all of the necessary treatment that would entail - even if that cost would be much greater than what we are asking them to do.
It is time for all of us to get some rest after a busy day. I hope we sleep soundly tonight, knowing that we are making the best choices that we possibly can each and every day for Sam.