Over the course of the last year or so, we have pushed for autoimmune testing. Which Dylan completed and his markers all came back within “normal range”. But yet again, Dylan had bad labs, we tested again and his bilirubin was up (both his direct and indirect but it’s his total bilirubin that is alarming), we tested yet again and it was even higher…this is completely abnormal for Dylan, all through his life (ever since his post first surgery at 9 weeks old) he has had rock solid bilirubin; even during Cirrhosis/End Stage Liver Disease. For those that don’t know what bilirubin is, “bilirubin is formed by the breakdown of red blood cells in the body”. High levels are an indicator of liver damage and higher than normal direct bilirubin indicates the liver isn’t clearing the bilirubin properly. Although Dylan’s direct bilirubin is elevated, it’s the total bilirubin that has us concerned – as this can indicate additional issues going on.
We contacted Dr. Edward Rich, Dylan’s GI doc since 6 weeks old. He is not only responsive to us but early on he gave us his personal cell phone (which we DO NOT abuse) but he is someone we regard as family to us – he cares deeply about Dylan and is always there for us in helping trying to figure out something that doesn’t have a clear cut path. After Amber did a bunch of research and reading medical journals after medical journals, she requested Dr. Rich order some more tests. This time he ordered a Haptoglobin test – which is a protein marker to test for Hemolytic Anemia. This test has a range of 30-220 and Dylan’s came back at 15: this result indicates he has abnormal Hemolysis taking place. Hemolysis is the breakdown of red blood cells and although this is normal for everybody, his is happening faster than normal – which is destroying red blood cells faster than his bone marrow can produce. Hemolytic Anemia can take place in tissue/organ or in the bloodstream. This answers why his total bilirubin is high and his direct isn’t concerning - excess broken down red blood cells bind with bilirubin, hence more red blood cells = higher bilirubin.
Now that we know he has Hemolytic Anemia, this may answer why he has constant stomach pains, cold urticaria, headaches/dizziness, and as of recently chest pains too. We need to figure out if this is coming from his tissues/organs or from his bloodstream. In addition, it’s sadly possible that we could be dealing with an even scarier possibility, that this is PTLD (Posttransplant Lymphoproliferative Disorder, which is cancer), which is presenting as Hemolytic Anemia. Although we don’t know where this is happening, we suspect it's happening in his liver and its drug induced, which would attribute to his fluctuating liver labs. You see, being that Dylan is on an immunosuppressive medicine (Tacrolimus aka Prograf), it’s possible albeit extremely rare that anti-rejection drug(s) can cause Hemolytic Anemia. The fix, if this is the reason, is to switch his anti-rejection med. The idea of switching is a terrifying thought as we attempted to switch Dylan off Prograf to Rapamune - literally 6 years ago to the month. That process ended badly and put him in a downward spiral for nearly 2 years of chaos. After speaking to Dr. Rich about this, he said although it’s possible for drug induced Hemolytic Anemia in transplant patients, he has personally never seen it. This is nerve racking and hopefully not wishful thinking on our end, as its the best case scenario we could ask for.
We contacted Stanford and they agreed that something is amiss and are putting in a referral to Stanford Hematology/Oncology Dept. Our next weeks/months are going to be incredibly stressful and we’re doing the best we can to be as positive as possible. This is made even more complicated as we now care for my 96 year old grandmother, who is requiring more and more care as days go on. Although at times it’s hard to not be flustered and/or jaded - it just seems like never ending hurdles - and it makes us incredibly sad for Dylan and how hard this is for him.
Thank you to all whom still follow Dylan and our lives, we truly appreciate it.
-Noah and Amber