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Dr. Michael J. Smith: 22 Years and Counting - My WM Journey

The following is an account of a life journey with Waldenstrom’s Macroglobulinemia (WM) that later turned into WM/Lymphoma (WM/L). My story is aimed at beginning patients, so I will cover the WM episode more thoroughly than the subsequent WM/L. Full disclosure: I am only a novice in medicine, so I may have occasionally included errors in my treatment. Consequently, I have omitted my doctors’ names to avoid having any of these errors attributed to them.

As with many WM’ers, there was a telltale indicator that my WM began well before it was formally diagnosed. Buried in my internist’s blood work report in June of 1994 was the fact that my Erythrocyte Sedimentation Rate (ESR) was 83.3 mm/hr – way too high – when it should have been in the range 0 - 15 mm/hr. My internist didn’t have a reason for it, and I swiftly buried it within the cobwebs of my mind as I had another more pressing problem. I had a cancer – more specifically Acinic Cell Carcinoma – in my left parotid gland (a salivary gland), and I needed to take care of it immediately. This was operated on in July, followed by radiation treatments a month later. The problem has not recurred since, although some symmetry has been lost in my facial features. Unbeknownst to me, it was the beginning of interweaving threads of curable and incurable cancers in my life.

As I neared retirement, which occurred on May 3, 1997, I began suffering the rare nosebleed. One particularly embarrassing episode occurred right on the job at a meeting, requiring a quick visit to the restroom. I was in the habit of walking at noon for exercise and felt myself to be in pretty good shape. But I noticed that I tired more quickly than usual if I walked with a heavy weight. After retirement, in October 1998, while doing my usual exercise walking in the neighborhood, I found myself severely short of breath and had to sit down. This alarmed my wife, and it was off to the doctor’s office for tests. My heart was ok, but blood tests returned hemoglobin and hematocrit values of 5.6 and 17.4 g/dl respectively. This required a trip to Providence Hospital (PH) for more tests, which showed an ESR of 102 mm/hr, a high blood viscosity (13.3 centipoise), and an IgM concentration that was an astounding 9760 mg/dL. A bone marrow test was also done. It was then, 18 years ago, that a diagnosis (WM) was finally made, and the source of a litany of medical symptoms was finally explained. In addition to shortness of breath and fatigue, those symptoms included near constant nosebleeds, hoarseness that showed up in phone conversations, and the “box car” or “sausage” appearance of blood vessels in my eye. That last condition was first noticed in a routine exam for new eyeglasses.

Needless to say, I was perplexed as to what to do next. Thanks to my daughter and the internet, we found Arnold Smokler (IWMF founder) who sent us a package full of information on where to go, what specialist to see, and what regimen to get on. I reported to Georgetown University Hospital (GUH) where I underwent plasmapheresis, and received six units of red blood cells and Cladribine treatment. I later followed my doctor to the University of Maryland Medical Center (UMMC) for follow-up treatment. It was at UMMC that my situation greatly improved.

But, back to those intertwining threads. In July 2000, I was diagnosed with prostate cancer. Shortly after this I came down with pneumonia (due to my weakened immune system) while on a day visit to Philadelphia. It was diagnosed and successfully treated when I got back home. The prostate cancer, which initially received hormone treatment to control it, was finally subjected to radiation treatment, completed in February 2001.

By late summer, there were signs that WM was recurring and I was being given shots to boost my white blood cell (WBC) count. On July 27, I reported Hgb/Hct values of 9.5 and 28.5 g/dl. I had anemia and received four shots of Procrit over the next few weeks.  My temperature started to go up. I reported to the hospital on August 24 with a 101.8 F temperature that was diagnosed as neutropenic fever. I was given two units of red blood cells and five units of antibiotics by IV. The hospital team started me with Neupogen shots, which were continued by my wife after I was released. I appeared to improve. Months prior, we had planned an autumn trip to London, Paris, and Rome. I did not want to miss it, WM notwithstanding. So, our doctor sent us off with a list of whom to call in the case of an emergency.

We arrived in Europe on September 11, 2001 and received a call from our daughters back in the States to turn on the TV. We stared in horror at images of the attacks on the World Trade Center and Pentagon. Our trip continued, and I enjoyed the sights while soldiering through the experience with an elevated temperature, and night sweats and not much appetite for the food. Miracle of miracles, we made it back to the U.S. safely. I immediately sought treatment, which required two units of platelets, two units of Cladribine and four units of Rituximab. I ended up staying in the hospital for close to a month because of a blood infection that caused hallucinations, and I was also subject to great chills at the outset of receiving Rituximab for the first time.

After this episode, I experienced a substantial period of WM remission. However, in 2004, the prostate cancer recurred in a more aggressive form than before. I was offered more radiation, but my gut feeling was that having already undergone radiation, this approach wasn’t going to work, and some other therapy was needed. After an intense internet search and further consultation with several urologists, I finally resolved to go with cryosurgery. My PSA reading was 18 and climbing. I was looking for an excellent professional with considerable experience gained by performing a large number of procedures per year. My daughter found just the right specialist willing to treat me, and before long I was off to Columbia Presbyterian Hospital (CPH) in New York, where cryosurgery was successfully performed.

In 2006, the WM recovery I was then experiencing came to an end. I was treated with two units of RBC, two units of Cytoxan, and eight units of Rituximab in two rounds separated by one month. There were also four shots of another drug included, the name of which I cannot recall. Near the end of the same year, I developed bladder/urea stones that were treated to good effect by lithotripsy.

In 2007, I developed shingles and was treated in the hospital, followed by outpatient care by my wife, to whom I am grateful for being a constant source of support. The affected area remains sensitive to this day, off and on, but not really to any great degree of bother.

My wife was diagnosed with breast cancer in April 2008 and underwent surgery that month, followed by subsequent radiation treatments, which continued through the last months of the year. My sister died of lung cancer in May. I mention all this because I wonder whether stress might have had some role in the progression of my illness that year from WM to WM/Lymphoma. I also mean  to inform the reader that my wife’s illness in no way moderated her impact on my subsequent care, even though her treatment sessions often occurred at the same time as – and many miles away from – my own out- or in-patient medical treatment.

The onset of my third relapse began in summer 2008. Blood work showed that my IgM value had gone up to 687 mg/dl in July from 408 mg/dl in January. My white blood cell count increased such that my acyclovir dosage required an increase from one to two doses a day in August. By the end of the month I reported to my local ENT professional with a temperature of 100 F and began an antibiotic regimen for a sinus bacteria infection. Hgb/Hct reached 10.9/34.7 g/dl in October. My initial treatment came on October 17 when I received a course of Rituxan and Velcade. Also in October, I developed shortness of breath, a cough, and had discomfort sleeping on my right side, which progressively worsened, but no fever.  I reported to the Emergency Room at UMMC on October 24 to get a diagnosis. A CT scan revealed a moderate pleural effusion with right lower-ended lobe collapse. Also noted was an anterior mediastinal mass, which was new. Its size was 6.3 cm X 4.6 cm. A subsequent biopsy indicated that this mass contained a mix of small and large cell B cells. A pleural tap was installed in my chest on October 29 to draw out the fluid. The first draw done in the hospital collected two liters, which was free of any cancer cells. Further draws were done at home, by my wife, on an every-other-day basis. They dwindled from 350 to 50 ml at the final draw. The tap was removed on November 21. IV treatment began again on November 14, Velcade was dropped, and Rituxan and CHOP prescribed (R-CHOP, three sessions with the last on December 15). The R-CHOP treatment got me severely constipated (due to Prednisone, the P in CHOP). I had no appetite during the Christmas season and went to the Emergency Room around New Year’s Day, whereupon my issue was resolved.

As expected during treatment, I lost all my hair (which was mostly grey tinged with some brown). But strangely, when it grew back, it was mostly brown tinged with some grey. Sure, I don’t look like a 40-year-old, but I look a lot younger than before. Go figure!

The mass continued to shrink through the winter and spring of 2009 until it disappeared. The right lung regained its full function. Since this time there have been two further relapses. I will list these (including that just discussed) in tabular form below, with the general course of treatment sketched:

Month

Year

Relapse/Remark

Treatment

October

2008

3rd/anterior

mediastinal mass

(6.3 cm X 4.6 cm)

R- CHOP/Thoracic Drain

 

August

2011

4th

R-Treanda

February

2013

5th

R-Bendamustine

From the abnormal ESR report (1994) until now is nearly 22 years, and from the WM diagnosis approaching 18. This is amazing for an illness that used to have such a short projection for further life. I am now 78. In this experience, I cannot speak too highly of my wife, my two daughters, the nurses, doctors, and people like Arnold Smokler, and the IWMF who got me from there to here.

Michael J. Smith, Ph.D.

Washington, DC metro area

June 2016

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