column logo Alithea Athans

When you have an autoimmune disease such as mine, cold agglutinin disease (CAD), you are always looking for answers and treatment options. There are few choices for treatment, and there is a risk of reaching an end point with the existing options. I am not at that point but I still like to keep myself informed in the event I get there.

For years, there have been a couple of standard treatments for CAD. There was a time when steroids were used as treatment, but I guess successes were few and far between. Now, the first line of treatment is rituximab, and some of the people I have met have been treated successfully often after a combination of rituximab and bendamustine. The results I am familiar with have been mixed; some had full remission, while for others, it didn’t work at all.

It doesn’t make sense why it doesn’t work at all for some. That’s very scary considering the side effects that they had to deal with—it is chemotherapy after all. Then Sanofi came out with a promising drug, Enjaymo®, but again this one has side effects and you need to be on it continuously.

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All of these drugs are infusion therapies that require weekly or biweekly treatment. Rituximab is typically given for a few weeks depending on the need, but Enjaymo, as effective as the response, is requires infusion every 2 weeks for life. That was very disappointing.

Read more about experimental therapies for CAD

At this point, I see my hematologist every 3 months and he just keeps a watchful eye. My hemoglobin has been steady for a long time, in the 10s. I am so thankful for this.

Thankfully as well, there are clinical trials still going on and I am hoping if the time comes, new treatments will be available that have fewer side effects and require fewer treatments.

I recently heard about the use of recombinant erythropoietin (EPO) successfully being used to treat CAD. This piqued my interest and I needed to find out how that was possible. I am familiar with it because of the misuse by athletes as performance enhancement, and I thought it was illegal. It turns out, it is not when used properly to treat disease.

There was a study done in Europe that EPO had a continued response when used in people with CAD. This study had a mix of both primary and secondary patients with autoimmune hemolytic anemia, of different ages and sex. They were treated with EPO and the results were promising for CAD. It effectively increased hemoglobin and reticulocyte counts.

It works best if used within the first year of diagnosis. At first, I thought that was odd because most CAD patients I know have had symptoms that turn out to have been CAD for years prior to diagnosis. What they meant is that EPO is most successful when used within the first year because you probably haven’t had any treatment yet, specifically, immunosuppressive therapies. Another interesting finding was that people with autoimmune hemolytic anemia seem to have lower EPO than those with other anemics.

Read more about CAD diagnosis

They suggest it could be from our bone marrow compensation happening slowly against the fast-paced destruction of red blood cells. It is for this reason that they suggest it may be best to use EPO rather than immunosuppressants. The study does go on to say that those who have primary CAD and have not had any immunosuppressive therapies had the best response. Patients were treated over several months and about one-third of the participants were able to come off it completely. Of course, like all medicines, the EPO too had its side effects. The one that stood out was blood clots. Blood clots are not uncommon among CAD patients prior to any treatment, so it’s a bit concerning.

All in all, this sounds like a promising choice for someone like me. I have primary CAD and I haven’t had any treatment. What amazed me the most is that there were patients that had full remission and were able to stop the treatment altogether. I am so excited and thankful that doctors and researchers are continuing to push the envelope in finding a proper treatment for CAD. I feel like we are getting close to one day finding a therapy that truly works without continuous treatment. One that could be a cure.