Yearly evaluations are always scheduled for Mon-Wed, with Monday being the longest and most intense day. Vials upon vials of bloodwork, every bathroom test you can think of, vitals and EKG, echocardiogram, chest x-ray, consultations, and the ever-so-popular bone marrow biopsy. I even have a special technique for that oh-so-special procedure; 2 extra strength Tylenol about 1 hour before, and an Ativan under the tongue about 20 minutes prior. Doesn't make it any more enjoyable, but certainly takes the edge off!
The next couple of days include consultations with nephrology, hematology, and a review of the initial test results, as well as an amyloidosis support group. The one benefit of all this sitting around in between appointments is we get to meet and chat with the nicest people; including those who've already been treated as well as people who are recently diagnosed being evaluated for treatment. Having been through the entire process twice and knowing all of the doctors and staff allows both Steve and I to offer some support and guidance to those just starting on this journey, and I consider that the benefit of having had to go through this experience. Being able to connect with other patients makes me feel like something good came out of something pretty crappy.
I received my test results at the end of the week, which was perfect timing, right before Father's Day. In order to really appreciate the improvement, I'll give you a brief description on how amyloidosis affected me. The disease is a malfunction of the bone marrow, in which plasma cells start "folding" incorrectly. When this happens, they cannot be broken down and metabolized properly, and they start building up in the blood. All these wacky plasma cells start sticking together and attaching to different organs like chewing gum. Amyloid fibrils can attack any organ, but for me, they were clogging up my kidneys and damaging them. Left unchecked, the disease eventually leads to organ failure.
As a result of the amyloid damage, I was losing protein in my urine. Protein is supposed to stay in your blood, and acts like a sponge, holding on to fluid, which keeps your blood thin and loose. Instead, as I lost protein, the levels of protein in my blood went down, causing a few problems. First, because there was a build up of extra fluid, I had terrible edema, or swelling in my belly, legs and feet (this was the first sign that something was wrong which sent me to the doctor). Second, as extra fluid leaked out of my blood into the tissues, my blood was less "watery" and I was at higher risk of blood clots. Third, as my blood protein levels went down, my body tried to make more by churning out protein from my liver. The only problem with that is with every protein molecule your liver makes, there are 2 cholesterol molecules attached to it. So my cholesterol was extremely high, starting around 350, getting as high as over 500! I had the cholesterol of a stick of butter, and was put on a statin.
So every year, we look for certain numbers. First, I always want to see the protein in my urine going down as much as possible (I have no more swelling or edema, so it's improving and shows that my kidneys are no longer being damaged and are actually healing). Second, I'm always interested in my cholesterol, both the total number (which should be under 200), as well as HDL and LDL. And the most important test is the levels of plasma cells in the bone marrow.
I'm happy to report that there's less than 240 mg of protein in the urine (normal is 150 or less; at diagnosis I had between 8000 and 16,000 mg of protein!), that is awesome! Because that number is coming down, my total cholesterol is about 205, which is pretty healthy. The bone marrow biopsy showed normal amounts of plasma cells, between 5-10%, so that is awesome. But here's the extra good news...there are also two immunofixation tests that look for abnormal proteins, blood (serum) and urine. After 2 stem cell transplants, both of these tests showed a "barely detectable trace" of paraprotein, literally described as a "smudge" (that's actually what it is, a shadow, a visible marker). They saw this "smudge" in tests done in December 2008, June 2009, and June 2010, no changes, it was just there. Since my test results have continued to improve steadily, they assume that it's leftover crap floating around, but they can't guarantee that it's not new amyloid being produced. However, tests done this week show the same smudge in the serum tests...BUT NOT IN THE URINE TESTS. It has *disappeared* from the urine immunofixation tests! This gives us hope that perhaps in the next year or so, it will disappear completely from the serum tests as well.
In the meantime, this is awesome news, and we are all so happy. Every year around this time the fleeting thought of a relapse does cross my mind, how could it not?! I'm sure this is what every patient experiences. But all I can do is choose not to dwell on that, using my affirmations as a tool to help me stay positive.