The joy of Coombs negativity

Been busy here. Kitchen remodel + Ikea = eating too many Swedish meatballs after tramping around a football-field-sized maze of furniture where everything has names like Akurum, Famtid, and Wurm. Me, Marilyn, and half of Phoenix were there on Sunday. Soylent Green is people!

The good news is that I am healthy enough to endure the experience, which is not unlike a Bataan Death March through a pressboard jungle where gibberish is the lingua franca. My hemoglobin is 14.7, more than double what it was a year ago when I was in the throes of autoimmune hemolytic anemia (AIHA). My RBC is 4.72, hematocrit 41.2. All are well within the normal reference range.

The big cherry on this red blood sundae is that my last two Coombs tests have come up negative, which means the AIHA beast is in its cave, unlikely to show up in the dramatic fashion by which it attacked me last year. Converting to Coombs negativity was my not-so-secret hope when I underwent R+C(V)P therapy for AIHA and CLL, which commenced on October 22, 2007. Conversion took so long to happen that I thought it wasn’t going to. And then on August 19 it did. And on October 7 it did again. (I am a believer in the “make sure you get two tests to confirm a trend” school of blood draw divination.)

According to a study by Dr. Kanti Rai, et al of CLLers with AIHA treated with a similar protocol to mine, conversion to Coombs negativity led to a mean response of 23 months, as opposed to 8.8 months for those who remained Coombs positive. “This finding that Coombs conversion portends a longer duration of response suggests treatment goals for AIHA should be a conversion to Coombs negative, and not stopped with recovery of HGB,” the authors wrote.

Well, I’ll be damned, pass the lingonberries and pour some Absolut in my Ljuvlig, I am one of the lucky ones. I am also lucky in that I achieved this following just three rounds of chemo (not the five or so that most of Rai’s patients had). And that my absolute lymphocyte count is 8.9 a year later, much lower than I ever thought it would stay.

The imperfect part of the picture is that the lymph nodes began to return, slowly, around June and July. This follows the typical pattern of 11q-deleted disease, where the CLL collects in the nodes. They aren’t as bad as they used to be, but they’re there, a definite reminder that CLL relapse after chemotherapy is, like getting a Florvag for your Tolga, inevitable.

Nonetheless, I am for the moment enjoying a satisfying, quiet victory over the most acute medical crisis I have ever faced. My saga is a reminder of how easy it is to take the fundamentals of health for granted, and what a long and difficult slog it can be to return to a simple, functional state.

There are two lessons I am taking away. One is that things don’t always go by the book, that statistics are general but results are individual. By all counts, my response to therapy shouldn’t have been this good. Rai’s patients were Rituxan-naive; I certainly am not, and I had fewer rounds of chemo than they did. Two is that I believe controlling my AIHA is directly proportional to controlling my CLL; when time comes again for treatment I am going to make sure there is at least as much punch to it as there was before. The days of single-agent Rituxan as a means of controlling my CLL are almost assuredly over.

The nice thing about a remission is that I don’t think about these things that much right now. Instead, I ponder what to do with the three extra inches on the wall over the sink, what color granite goes with Adel medium brown, and whether to put a Luftig over the Mumsig.

Had I tried to make it through Ikea this time last year, when my HGB was 6.8, they would have had to carry me out on an Ektorp.

Ibuprofen and AIHA

A few years ago, I read on the ACOR CLL List about a patient who took ibuprofen when his swollen lymph nodes began to bother him. His doctor said it was OK to take 1600 mg daily for a week, which is half the maximum allowable daily dose.

The lure of NSAIDs (non-steroidal-anti-inflammatory drugs) like ibuprofen is their easy availability, low cost, and ability to alleviate symptoms. But like all drugs they come with side effects and toxicities, which means that their role in combating CLL is necessarily limited.

When I read about ibuprofen controlling the nodes, I decided to give it a try. I didn't need a lot of convincing, as I had always rather enjoyed the effects of ibuprofen on those rare occasions when I took it for a headache or muscle pain. It would leave me with a pleasant feeling, not exactly tranquilized, but just that more at peace with the world.

I found that it worked on the nodes to a small and very temporary degree, just enough that I began to take it when my ever-growing nodes got painful. Even then, I never went up to the line and used it as much as I could. I am rather conservative about drug use. The most I did was take it once or twice a day for two or three days.

Later, by the way, when I used steroids, the nodes reduced dramatically, so there really is no contest when it comes to NSAIDs vs. SAIDs. But steroids are much worse in terms of side effects; being conservative about drugs, I used single-agent Rituxan for as long as I could before circumstances, in the form of my AIHA diagnosis last March, forced me to throw steroids into the mix. AIHA, of course, is short for autoimmune hemolytic anemia, about which I have written a great deal, and which I would not wish on my worst enemy.

After I came down with AIHA, I stumbled across an interesting bit of information in the Merck Manual online. Ibuprofen was listed in a table as one of several drugs "that can cause warm antibody [autoimmune] hemolytic anemia."

It was hard to find any more information on the subject, but I immediately quit using it, even on the rarest of occasions. I am convinced, based on my reading and the conventional wisdom of doctors, that my AIHA came about as a result of the CLL having gotten out of control and gumming up the immunity works. But the thought that ibuprofen might have had even a tiny role in helping me reach the tipping point into hemolysis -- the vicious cycle in which the body destroys its own red blood cells -- was enough to make me quit using it.

Some time later, after my R-C(V)P therapy at the end of last year, I wrote to Dr. Clive Zent of the Mayo Clinic. Dr. Zent is a CLL and autoimmune disease expert who has generously allowed me to share his thoughts with you. I asked, quite simply, whether it w as safe to use ibuprofen, or whether it could contribute to a possible relapse of AIHA. Here's his reply:

Ibuprofen -- this is certainly a drug that has been associated with autoimmune complications including AIHA. It (and all the other drugs in its class -- NSAID) have many other potential toxicities which are generally rare. We rarely determine the triggers (if any) of AIHA in an individual. This is all a prelude to saying that unless ibuprofen was the cause of your AIHA, I don't believe that you are at high risk of causing a relapse of your AIHA if you use the drug, but it (and all other drugs) should be used only when indicated. If acetaminophen is effective for your pain, it could be a safer option. 

The bottom line is "better safe than sorry." How to handle CLL and its myriad effects is something that keeps even the experts guessing. Those of you out there who are Coombs positive, or have been diagnosed with AIHA, and who are also scarfing down the ibuprofen, may want to think twice about it.