Stephen French: My Twenty-Year Journey with WM

I have had a long journey with Waldenstrom’s macroglobulinemia.  I have had good news and bad news during this period, but the good news has been with me for over sixteen years.  I hope it lasts a whole lot longer.  I was diagnosed with WM in August, 1998 and started chemotherapy in September, 1998.  I was 63 years old when diagnosed, and began to wonder how long I had to live.  Now I ask in hindsight:  how did I get to where I am today?

The story began in 1996.  My bone joints were getting very painful and I started seeing a rheumatologist.  In 1997, my physician diagnosed my condition as polymyalgia rheumatica and prescribed high dose prednisone, a powerful steroid.  It was a pleasant treatment because prednisone made me feel energetic.  Eventually, however, after a year of this therapy and with the joint pain having substantially decreased, in mid-1998 I had to stop taking the drug.  The good news was that the joint pain did not recur.

Many blood tests were conducted during this time to measure the effect of the treatments.  One day, near the end of my arthritis treatment, while the doctor was checking the results of the most recent blood tests, she suddenly excused herself to go to her office.  When she returned to the examination room, she asked me to immediately see my internist, whose office was located in the same building.  I did so, but did not get to have a discussion with my internist, as she directed me to go to the x-ray laboratory without delay.

The x-ray technician took many pictures of my bones with large marrow chambers.  After the x-ray results were studied by a radiologist and by my primary doctor, I was sent to see a hematologist/oncologist.  At that point, I got the shock of my life – the oncologist told me that the tests were for multiple myeloma.  But thankfully it was determined I did not have multiple myeloma.  Then my oncologist told me that he needed a bone marrow sample for further diagnosis.  I wanted to watch the procedure, so the oncologist gave me a mirror to view what he was doing.  He could not aspirate a sample from one hip because the marrow fluids had become very viscous.  He finally got some marrow from the other hip.  The sample was sent for analysis – electrophoresis indicated the presence of an unusual monoclonal protein, and the IgM level was over 2500 mg/deciliter.  In August 1998, as a result of all these tests, it was determined that I had Waldenstrom’s macroglobulinemia, a rare non-Hodgkin lymphoma, which I had never heard of.  I remember that I couldn’t pronounce the name very well and had to say it very carefully.  I learned later that most everyone who has the same lymphoma calls it WM.

Shortly thereafter, I began also to experience nose bleeds, unexplained bruising, and considerable fatigue, all characteristic (I later learned) of WM.  Most importantly, apart from the uncertainty of discovering I had a new illness, I became apprehensive about how these symptoms and whatever therapy I would require might affect my ability to continue working, about the burden it might place on my family, and more generally how it would affect my lifestyle.

My oncologist had not yet treated a patient with WM, so he called colleagues in Boston, MA, Rochester, MN and Houston, TX.  He told me that he believed in a conservative approach when designing a treatment regimen.  He started with Leukeran (chlorambucil) therapy.  The first treatment called for taking ten small pills at breakfast and then a repeat dose the next day.  I felt no unpleasant effect from the drug.  After what seemed a long wait, my blood serum was tested for the IgM level.  There was no reduction.  In fact, the IgM level had increased.  The treatment was repeated again. The result was the same – no decrease in IgM level.  I began to wonder if the drug that was being used was powerful enough.  My oncologist told me that dosage and timing were difficult to anticipate for best results, so he changed the timing of the drug dosage.  This time all twenty pills were taken at one sitting.  My oncologist paused treatment again and my blood serum was tested after a few weeks.  The IgM level had not decreased – in fact, it had increased!  I was starting to worry, but my oncologist counseled me to be patient.  Not everyone has a positive reaction to the first drug used during chemotherapy.

It was time to try another drug.  Fludarabine was chosen.  The care plan was four infusions per treatment cycle, each administered one week apart.  We again waited for several weeks to allow the drug to work.  As before, the test for the IgM level indicated little response.  The fludarabine treatment then was repeated, and the result was as before.  It was now the end of 1999 and I was getting physically weaker and more discouraged.  I could not do outside work on our property, and it was getting tougher teaching computer skills at the private school where I worked.  I spent most of my free time just lying around and resting.  My wife, however, was the stronger partner.  She went to all of my treatment sessions, listened to my grousing about how I felt, and kept a positive outlook that a treatment would be found.  She maintained the house and did my outside chores.  I do not know how she kept up with everything that was happening.  My son and two daughters understood what I was going through and helped me out as much as possible while raising their own families.  This was a period when my family got me through some tough times, and I can never thank them enough.

My oncologist had been reading positive reports about treating various lymphomas with a new chemotherapeutic agent called Rituxan (rituximab).  In June 1999, he decided to try that agent next.  The drug was still being evaluated and was very expensive.  My four infusion treatments cost my insurance company several thousand dollars.  The first infusion of that drug is usually quite difficult on the patient.  Many patients have moderate to severe reactions.  I remember feeling cold, so cold that the nurses brought me a blanket to wear during each infusion.  The oncology nurses belong to a special group of nurses, and they took very good care of me.

The Rituxan infusions were followed by several weeks of waiting, and then more blood tests.  The IgM level once again showed no change.  It was a very discouraging time.  My oncologist wanted to repeat the Rituxan treatment, but my insurance company balked at covering the high cost.  It took several high-level phone calls by him to get insurance coverage for one more infusion series.  The insurance company finally agreed, and on this occasion, while the therapy did not cause a cold allergic response, neither did it lead to any lowering of the IgM level.

Throughout this period I continued working full time as a computer skills instructor.  But it was getting difficult to teach all-day classes.  I needed the income and the good health insurance that my job provided.  I had now been on chemotherapy for over two years without much effect.  My nosebleeds continued, as did fatigue and energy loss.  One new symptom now occurred – peripheral neuropathy.  I never experienced pain in my feet as many other WM patients have, but the lack of feeling in my feet caused walking and balance problems.  Since I live in an area where it can get very cold in the winter, I had to be very careful that my peripheral circulation was not compromised.   Another effect of the chemotherapy and WM was a weakening of my immune system.  I had more colds and I was getting anemic.  My dentist was aware of my lymphoma and wanted me to take an antibiotic medication before cleaning or treating my teeth and gums.

Clearly, it had become time for another approach.  I was in my third year of treatment.  This time my oncologist chose Cytoxan (cyclophosphamide}.  It is an older drug, having been introduced in the U.S. in 1959.  It can be administered by mouth, by injection, or by infusion.  I received infusions at the rate of one each week.  It is toxic enough that the nurses had to administer anti-nausea treatment before I received my infusions.  Then I had to take more anti-nausea medication in the evening after each treatment and still more anti-nausea medication the next day.  I forgot my medication one day after treatment and was nauseous for an entire morning while trying to teach a computer class.  I do not remember how many Cytoxan treatments I received but my treatment lasted for several months.

My oncologist followed my treatment very carefully during this period.  One day, late in 2001, he met me in the infusion room and happily presented me with results of my most recent blood tests.  My IgM level had DECREASED, HOORAY! and so had my paraprotein!  At that point I felt a confidence that I had not had in a long time.  The infusions of Cytoxan continued.  My blood tests showed that this treatment was working, and my IgM level continued to decrease.  Finally, in early 2002, my oncologist said he was going to stop the Cytoxan therapy and see if the IgM level continued to decrease.  The IgM level did continue to decrease, finally falling to a level of less than 100 mg/deciliter.  My final infusion of Cytoxan was administered in January, 2002.  My treatments had lasted for three-and-a-half years.  I have been on watch and wait ever since.

When I look back to the time I was diagnosed with WM and all of the treatments that followed, I realize how blessed I was to have the love and support of my family.  I had excellent and expert care from a concerned oncologist.  The nurses who cared for me were loving and tough at the same time.  They would not let me shirk my treatment for any reason.  Not even when Rochester, New York, was having one of those blizzards it is so famous for.  I stayed at home that day and a nurse called me and asked where I was.  She had walked to the treatment center and was wondering, was I planning to come in for treatment?  I told her that roads out my way were a mess, and I would use my Emergency Medical Technician training to extract the IV port and make sure the area was clean.  My caregivers were so kind and so well trained that I did not think about consulting with another treatment center.

I did a lot of reading about the drugs I was taking, and had complete confidence in my treatment regimen.  I think that I had confidence in my treatment plan because I am a chemist and an EMT.  Looking back, I think that my treatments went smoothly.  I was physically uncomfortable some of the time and worried about the future, but my oncologist and those beautiful infusion nurses gave me confidence that things would work out.  The support of my family was critical.  I could not travel far much of the time.  I could not enjoy my hobby of gardening, and I could barely work in the classroom.  But here I am today – retired, living in an apartment complex, and looking forward to trips and gardening this spring and summer.

The end of the story has yet to be written.  My IgM has slowly increased to about 600 mg/deciliter, but no symptoms of WM have recurred.  I also am taking seven 2.5 mg tablets of methotrexate once a week to treat rheumatoid arthritis.  My oncologist had to approve this treatment, and said that the methotrexate might also keep my WM in check.

During this long period of good times and some bad times, one thing remained constant – the loving care by the oncology nurses never wavered.  They have always been there.  They were concerned about my comfort during infusions.  They checked my health before, during and after infusions.  And when infusion treatment ceased, they continue to be concerned about my health.  I love each and every one of those angels.

So, it was a long procedure, using drugs not prescribed very often now.  The treatments consisted of administering one drug at a time, but eventually the final treatment worked.  Am I in remission?  I hope so, but I might need to be treated again sometime in the future.  If and when that happens, the drugs will be newer and more specific for WM.  But one thing will remain without change:  the skilled and caring medical personnel who never give up on their patients, and who always give their all.

Stephen French
Rochester, NY
January 2019