What we know – Part 2

I’ve tried to start this post about twenty times so far and am still struggling to find the words.  They’re difficult to find when I try to explain it in person, yet alone in writing.  Hopefully just putting it in writing will help me process it and prepare us for what is to come.   We have mentioned in passing over the last few months that we are watching Keegan’s heart closely.  This is why.  We are going to be very real and realistic here.  Please remember that there are a lot of “ifs” and unknowns.  This is going to be a very long post, so grab some coffee & put your thinking cap on now…

Keegan is in the early stages leading to an irreversible type of rejection, called antibody mediated or humoral rejection (AMR).  We can try different treatments to slow down the process, but right now, the medical community does not know how to stop it.  This is a less common type of rejection and frankly, the type that we know the least about – how it works or how to treat it.  This is also a type of rejection that almost never “plays by the rules.”  It can progress quickly and be fatal without ever being confirmed.  Many times they treat patients for it, knowing that’s what is happening, without ever being able to find it.  Gray and I have asked a lot of questions and done a lot of research, and Keegan’s transplant team has been extremely helpful in trying to answer and educate us.  Because there is so much uncertainty surrounding it in general, the transplant team doesn’t always have answers to our questions.  I will do my best to explain what we do know.

Theoretically, AMR progresses like this: (1) body develops antibodies to the heart; (2) as antibodies attack the graft, (3) the heart becomes stiff and pressures increase due to narrowing of the vessels, (4) leading to failure of the graft/heart.  Some patients have the antibodies for years and years without ever progressing further.  Some patients are already to graft failure before anyone ever realizes what’s happening.  That is because this type of rejection, as opposed to more common cellular rejection, is generally asymptomatic, meaning there are no outward symptoms.  Often it’s never caught at all until it’s too late.  It can happen in as little as 3-6 months or over the course of a year or more. 

In an ideal world, we would have blood tests and imaging to see this entire process happening and be able to intervene before you get to the end stages.  Unfortunately, we don’t.  Echos don’t even pick up on the pressure differences most of the time.  And most of the time, the biopsies don’t ever capture the antibodies attached to the tissue, nor do the angiograms pick up on the vessel narrowing.  Sometimes, as in the case of one of our dear friends, you treat what you can’t find until you’re blue in the face, and a fatal heart attack sneaks up before you know what’s happened.  As I’ve mentioned before, heart attacks are the silent nemesis of the heart transplant world.  The nerves of the heart are severed during the transplant, meaning you don’t feel the tell-tale pain that generally precedes a heart attack.  It’s over before you know it’s happening.

So how did we pick up on it in Keegan?  When the blood tests to confirm what we thought was intestinal graft-vs-host were run, they discovered that Keegan had formed two different types of antibodies to his heart (see this post).  Luckily, he was already taking the immunosuppressant drug that he would have been switched to once the antibodies were discovered (rapamune), so we planned to watch the antibodies in his bloodwork and repeat echocardiograms more often.  After the bad port was removed and his fevers continued, the team wanted to go ahead with a heart catheterization, biopsy, and angiogram to make sure the port hadn’t caused any damage and get a good map of his heart before placing a new port.  We mentioned after the cath that they noted his heart was very stiff and his diastolic (relaxed) pressures were very high.  Now, we have to assume it is related to the AMR.  We are assuming the antibodies have attached enough to cause the pressure increase, but the biopsy did not confirm that.  As I mentioned above though, this is not uncommon.  There are many cases where the biopsies do not detect the antibodies in the tissue, while clinically we can see the results of it.

The great thing for Keegan is that, unlike many patients in his situation, the echo we did the day before his last cath actually DID pick up on the pressure increase.  So, we are hopeful that we will be able to see any further progression via an echocardiogram.  That is why we are repeating them every two weeks.  There is no guarantee, but we are hopeful to stay ahead of it this way.  As our transplant cardiologist said, the minute we see any change whatsoever in the wrong direction on one of those echos, we “go immediately to the cath lab and come out with guns blazing.”

The first ways to treat AMR are to switch to rapamune as the primary immunosuppressant (check!) and do a steroid pulse (check!).  Unfortunately, Keegan’s case has progressed while on the rapamune, and the steroid pulse has so far not changed his heart function for the better.  We will repeat the echo on Thursday of this week and start weaning the steroids.  We will continue to watch his heart closely, as well as repeat his colonoscopy once he is back to the maintenance level of steroids.  If we feel he needs further treatment for the AMR, the options are a high-dose round of IVIG, followed by a drug called rituximab.  Unfortunately (have you sensed that word being used a lot?), the gold-star treatment is plasmapheresis, which Keegan is too small to have due to the size of the catheters involved in the treatment.

The goal of all of this is to keep Keegan stable enough for him to be relisted for another heart transplant, and the plan right now is to list him for a kidney at the same time.  There are very strict rules surrounding when you can list, and in this instance, we would need to be able to visualize the coronary artery disease via a heart cath and angiogram.  Here comes that word again…unfortunately, very often patients fall victim to this disease faster than that can happen.  He is not a candidate for retransplant yet because of that.  We don’t know when it will happen.  Not tomorrow and likely not within the next few months.  All we do know is that it will happen.  Again, the team will not waste any time listing him as soon as he qualifies for it.  Even if we get to that point, we will have to consider his GI condition at the time.  It also appears from the recent GI testing that many of his problems point to a propensity to develop antibodies to himself or any transplanted organ.  That may weigh heavily in a decision to give him another organ – would the same thing just happen again.  We may never know that for sure.

That’s about all there is left to say at this point.  Obviously, this has been weighing heavily on us the last month.  We have enjoyed the last week or so of overall stability at home.  However, it appears a bigger fight is looming in our future.  No one can tell us or predict for us how this will go.  We may have years or only a few months.  Anyone who tried to convince us otherwise would be lying.  The only one who knows that now is God.  We are trying, perhaps not hard enough, to place all our trust in Him.  We will not give up the fight.  We will not let go of Keegan.  He is our love, our Bug, our son, and he is worth every minute.  We will continue walking this road every day…even if some days it feels more like a crawl.  We know that we must and that we can because He is with us and will carry us.  No matter what may come.