Cold Agglutination

Doug had his appointment with Dr. Myers in Salida today. His blood work shows no sign of MM - praise God!

Dr. Myers said that Doug has tested positive for "cold agglutinate". This is a rare disorder where abnormal blood proteins called cold agglutinins cause red blood cells to form clumps and die, thus causing possibly severe anemia. The process is triggered by exposure to cold temperatures. Dr. Myers said that Doug needs to avoid getting chilled, so he should dress warmly in cold weather. Or spend the winters in Texas, which we had already planned to do!

Of course we had never heard of this before, so I looked it up for more detail:
Cold agglutinin disease is an autoimmune disease characterized by the presence of high concentrations of circulating antibodies, usually IgM, directed against red blood cells. It is a form of autoimmune hemolytic anemia, specifically one in which antibodies only bind red blood cells at low body temperatures, typically 28-31°C. (82 – 88 degrees F)



There are two forms of cold agglutinin disease: 1-primary and 2-secondary. (Doug's is secondary to the MM.)


• The primary form is by definition idiopathic, a disease for which no cause is known.


• Secondary cold agglutinin disease is a result of an underlying condition.


 In adults, this is typically due to a lymphoproliferative disease such as lymphoma and chronic lymphoid leukemia, or infection.


At body temperatures of 28-31°C, such as those encountered during winter months, and occasionally at body temperatures of 37°C (normal body temp, 98.6 degrees F), antibodies (generally IgM) bind to the polysaccharide region of glycoproteins on the surface of red blood cells (typically the I antigen, i antigen, and Pr antigens). Binding of antibodies to red blood cells activates the classical pathway of the complement system. If the complement response is sufficient, red blood cells are damaged by the membrane attack complex, an effector of the complement cascade. In the formation of the membrane attack complex, several complement proteins are inserted into the red blood cell membrane, forming pores that lead to membrane instability and intravascular hemolysis (destruction of the red blood cell within the blood vessels).


Individuals with cold agglutinin disease present with signs and symptoms of hemolytic anemia.

Dr. Myers said that this disease does not usually result in any significant clinical impact, so we are not too alarmed at this point. Right now the blood tests show mild anemia but that is not new. Doug's cold agglutinin test showed a dilution of 1:64. Normal is 1:32.

So just one more thing to watch -- meanwhile we continue to lead happy busy lives.

Love to all.

Catching up

Wow, time really does fly. I was reminded that I have not been updating the blog when someone emailed to ask if Doug was okay. Yes, he is! Sorry for the lack of information; I will try to do better.

We went to Denver to RMCC April 13 for Doug's one year checkup with Jeff Matous M.D. Jeff (yes he is very okay with being called by his first name) is an amazing guy. He is always enthusiastic, very well informed and upbeat, ready to answer any questions. All doctors should all be like this, right? Unfortunately they aren't as you all probably know. Jeff told us he was leaving soon for Italy where he would take part in a big bike race.

Doug had had blood taken for various tests the week before, so Dr. Jeff had the results. The titers showed that Doug still has immunity on most diseases, so he doesn't need any re-vaccinations except the yearly flu. I was pleasantly surprised on that. His CBC and Chem was good.

 Bone density shows osteopenia (precursor to osteoporosis). He is still on monthly Zometa, the biophosphonate bone strengthener -- August will be the last infusion of Zometa. Studies show that the efficacy drops sharply after two years, and the risk of necrosis of the jaw (with dental work) means stopping is the best thing. Stopping Zometa will be good for Doug's kidneys too. Right now we are told his kidney function is "adequate". I hope his bone strength holds.

Doug's blood shows no sign of MM -- whew. Jeff told us that new studies show improved disease-free survival after SCT if the patient is put on maintenance therapy immediately after SCT. At the time of Doug's SCT that information was not out yet, so Doug is not on any drugs except the Zometa. Jeff said he does not want to start maintenance chemo now. He did tell us about two new drugs that will be available if/when Doug relapses. Pomatidromide is "the new Revlimid" and Carfilzomib is "the new Velcade" and does not result in the dreaded neuropathy.

So for now Doug continues seeing Dr. Adam Myers in nearby Salida every three months for the "MM panel" blood work.

We have been enjoying our wonderful summer weather here in Buena Vista. When the day is sunny it feels hot (with very low humidity so not 'sticky') but if you go in the shade it feels quite nice. Evidently bugs do not like our high altitude; we have a few flies but almost no mosquitoes. It is very pleasant to sit outside in the evening - we haven't even used bug spray. Most nights we sleep under a light blanket.

I'm posting a photo of us taken a couple of weeks ago. The yellow climbing rose is in our back yard; the green house is our neighbor's. Life is good -- we hope all of you are good also.