As told to Alice Riginos, IWMF Torch Editor
Published in the Torch 17.3 (September 2016), pages 8-11
This story translated into Spanish by Graciela Molina MD, Buenos Aires, Argentina and can be found here.
An oncologist of 2016 informing a new patient of a diagnosis of Waldenstrom’s macroglobulinemia will likely add the following phrases in rapid succession: “indolent cancer,” “incurable but treatable,” “watch and wait status possible for several, or even many, years,” “many new drugs in preparation and trial,” “patients may survive for decades.” The newly diagnosed patient will likely not hear from his or her oncologist the words “transformation” or “Bing-Neel,” referring to developments seen rarely in WM patients and, when seen, often following years of treatment. In this very personal account, written for publication in the Torch, WM patient Julie Davidson furnishes information in a frank and open manner about her experience as a patient diagnosed with Bing-Neel, the form of WM in which WM cells move into the patient’s central nervous system.
About Bing-Neel we patients hear very little, in part because it is so rare, in part because it manifests itself in ways that are almost unique in each patient, and in part because most Bing-Neel patients of record do not live for more than 2 years following this second diagnosis related to WM.
Julie Davidson’s experience as a Bing-Neel patient is here discussed in detail. This is a topic not found in the general literature and one that is referred to only infrequently in the personal reports we read in IWMF-Talk. Julie’s brave voice is that of a survivor. She has been diagnosed with Bing-Neel, treated with powerful chemotherapeutic drugs, and at her most recent evaluation by her Dana-Farber team in May 2016 she received a report of results that were truly exciting because they showed a significant response to treatment in the reduction of involvement of her spinal nerves. Three and a half years after learning of her Bing-Neel status, Julie is receiving hopeful signs of continuing survival.
Julie’s personal message to others with a Bing-Neel diagnosis is one of encouragement and hope. In speaking openly, however, she acknowledges the feelings of loneliness and isolation that come with every diagnosis of cancer, especially when the cancer is a type that is so poorly understood. She is very clear that support from her family and a close WM friend (see the post script to this article) have enabled her to keep moving forward on her journey.
Loneliness and isolation are the words that Julie Davidson says best express the emotions she has often felt during her journey as a cancer patient. The same sentiment she hears repeated over and over when members new to IWMF-Talk express their relief in finding other Wallies who understand the emotions they are experiencing as cancer patients. Loneliness and isolation are frequently cited, especially by the newly diagnosed, when persons intending to offer help and support make comments that are not so helpful and are often hurtful, or when the friends who initially offered a huge swell of support later begin to fade away.
From the outset, Julie’s experience of the past 5 years was unusual, beginning as it did at age 55 with simultaneous diagnoses of both Stage III A breast cancer and the orphan blood disease we know as Waldenstrom’s macroglobulinemia. Overnight she found herself to be a cancer patient with two very serious and very different forms of cancer. One can appreciate that a sense of isolation and loneliness enveloped her as she faced the decision of not only “how or when to treat” but “which to treat.”
Aggressive treatment for the breast cancer followed for the next 10 months, treatment that seemed to also hold the WM in check, and then Julie found herself in new and lonely territory when she learned that, within 6 months of completing treatment for breast cancer, her blood disease had progressed from Waldenstrom’s to Bing-Neel Syndrome (BN). In other words, already coping with WM, a rare disease, she was suddenly confronting that extremely rare form of WM occurring when the lymphoplasmacytic cells advance into the central nervous system (CNS).
Julie today speaks of her life before cancer diagnosis as a satisfying time marked by many accomplishments. Julie and her husband of 38 years, Dr. Wade Davidson, met at the University of Tennessee Center for the Health Sciences in Memphis when Julie was enrolled at the School of Nursing and Wade was in training for his future career as an obstetrician-gynecologist. Today he remains a busy OB/GYN in the Nashville area where they reside. At the time of Julie’s diagnosis in February of 2011, the Davidsons’ two adult children were living independently, and Julie had moved on from a career in nursing to employment as a commercial property manager supervising properties in five states, a responsibility that required frequent travel for onsite visits. She had a busy life. She was up to its challenges.
Beginning August 2010, a sudden onset of severe headaches and progressively severe fatigue sent her on a round of consultations, first with a neurologist, then a second neurologist, followed by a rheumatologist, and, finally, a hematologist-oncologist for evaluation for possible MGUS on February 14, 2011 (not exactly a Happy Valentine’s Day). The outcome was an overwhelming double diagnosis: Julie was told she had Waldenstrom’s macroglobulinemia with an IgM of 1,600 and, during the course of the initial evaluation, the discovery of a suspicious axillary lymph node led to a diagnosis of two different types of Stage III A cancer in the left breast. Given that she had had a negative mammogram the previous June, Julie and her local medical team agreed to first address the breast cancer in order to try to prevent a recurrence. Her local oncologist remarked: “The WM won’t kill you, but the search for it likely saved your life from a very aggressive breast cancer.”
Chemotherapy began in March 2011 with the drug combination known as AC/TH (doxorubicin and cyclophosphamide, paclitaxel and Herceptin), followed by a double mastectomy in September and then by radiation therapy from November to December. Treatment for Julie’s breast cancer was completed in December of 2011, 10 months after diagnosis. The chemotherapy also had a positive impact on her WM, cutting her IgM by over 50%.
This good news was, unfortunately, of short duration. Julie experienced a slow, but steady, improvement from the after-effects of the rigorous breast cancer treatment until a sudden, major drop in energy 6 months after the end of the radiation therapy. By now she and her husband were members of the IWMF and familiar with information about the disease posted on the IWMF website and on IWMF-Talk. Suspecting that Julie’s situation was more complex than had been recognized by the local oncologist, Julie and Wade determined that the time had come to seek a second opinion and consult with a specialist in WM. They made the first of an on-going series of appointments with Dr. Steven Treon at the Dana-Farber Cancer Institute (DFCI) in Boston. At the time of their first visit, Dr. Treon reviewed Julie’s medical record and shared their suspicion that “something else was happening.” Suspecting the presence of Bing-Neel, he ordered a lumbar puncture and an MRI. The results confirmed that WM cells were in the fluid of Julie’s central nervous system. Julie returned to Boston within a few weeks to follow up with Dr. Mikael Rinne, a neuro-oncologist at DFCI with experience treating Bing-Neel patients.
Julie’s new diagnosis, Bing-Neel Syndrome (BN), indicates the progression of WM into the central nervous system occurring in one of two forms, diffuse BN and tumoral BN. More specifically, Julie was told that she exhibited “Diffuse Leptomeningeal Bing-Neel with nerve root involvement” because WM cells were present in the cerebrospinal fluid within the meninges, the layers of protective tissue that enclose and protect the spinal cord and the brain. Further, the nerve roots in the caudal equina at the base of the spinal column were affected, as seen in the MRI, causing nerve pain and sensory-motor deficiencies in the lower extremities. These were among the symptoms eventually leading her to seek the second opinion.
Only 6 months after completion of the arduous treatment for breast cancer, Julie learned that her new diagnosis was that of a form of WM not merely rare (her local oncologist had never even heard of it) but one occurring in so many different manifestations that each case is virtually unique. No standard treatment has been determined for Bing-Neel. Most Wallies devote time to researching and learning about our very unusual lymphoma. Doing so with Bing-Neel can be a very scary experience. Not only is there very little information available, but also the publications that do provide information indicate a poor prognosis. As will be seen in Julie’s case, however, this is not necessarily true.
Nonetheless, the new diagnosis was a crushing blow. Treatment recommended by the doctors at DFCI was aggressive and harsh: 11 rounds of high-dose methotrexate (MTX) administered intravenously over 6 months, from August 2012 to January 2013. The twelfth round was aborted due to rapidly worsening fatigue.
Describing this treatment Julie has written:
“MTX does involve a hospital stay. Mine were 5 days for each round. I was able to get treatment in Nashville as directed by Drs. Treon and Rinne. Sodium bicarbonate was given by IV for the first day to create a very alkaline blood and urine pH balance. Once a pH of 8.0 was reached, the MTX was given. This was followed 24 hours later by a “rescue” drug called Leucovorin to replace folate in the blood. Urine was monitored every 4 hours during the stay and blood every 12 hours. All of this mainly aimed at minimizing kidney damage. I could go home once the MTX urine levels had dropped to an acceptable level, which usually took 3 days.
I did experience nausea that was well controlled by medication and the ever-present fatigue, but did pretty well otherwise. The worst problem with MTX was boredom while in the hospital. I also developed a strong aversion to hospital food.”
By October of 2013, Julie’s WM had become quite active as seen in a rise of IgM level to 1,900, a drop in hemoglobin to 9, worsening fatigue, and the appearance of bulky adenopathy. Moreover, a lytic lesion was now recognized in her jaw, as well as a lesion on one kidney, which, thankfully, was not renal cell cancer. As Julie put it, “It is hard to imagine being thrilled that a tumor is ‘only WM’!”
The next treatment proposed by the DFCI doctors, in conjunction with her local oncologist, was a combination of fludarabine and Rituxan (FR) administered intravenously. In addition to treating the WM, intravenous FR had previously shown some success with other Bing-Neel patients in crossing the so-called blood-brain barrier to reach the central nervous system. The FR combination was administered to Julie from the end of 2013 until May of 2014. Once more the results included a drop in IgM (now to 700), as well as a rise in hemoglobin to 12.4, and a significant reduction of the lymphadenopathy. However, there were no improvements in the lytic lesion and the lesion on the kidney. Fludarabine was now discontinued, but Rituxan was administered as maintenance for one more year and then stopped when a low level of IgG became problematic.
By the time of Julie’s return visit to DFCI in the summer of 2015, however, her evaluation showed some welcome effects of the FR treatment in an IgM level of 205 and a slightly improved hemoglobin level of 12.9. Additionally, the IgH PCR level (used as another gauge to measure the Bing-Neel activity in the spinal fluid) became undetectable. This had not previously happened, even after the high-dose MTX eradicated the lymphoma cells in the spinal fluid. Julie did not return to DFCI for evaluation until May 2016.
Her most recent round of tests were a year later in 2016 and showed results that were exciting, yet very unexpected, not only to Julie and her husband, but also to the DFCI doctors who had monitored her care since the first visit. Surprisingly, the MRI showed signs of significant reduction of the spinal nerve root involvement caused by Bing-Neel. The amazed specialists quizzed Julie as to what she had tried “that was different,” and the only suggestion that she could offer was that she had tried acupuncture and “other Chinese medicine techniques.” Julie, it should be noted, tried these techniques at the suggestion of her other doctors in an attempt to counter spinal nerve pain caused by the Bing-Neel. She had at that time reached the very upper limits of the medications used to treat her increasing neuropathic pain. She has been told that there is no way to predict if, (and if so, when) the BN-related nerve pain might begin to dissipate.
And so this lonely journey of Julie Davidson into the unknown terrain of Bing-Neel––more than 3 years after finishing methotrexate, 2 years after finishing a combination of fludarabine and Rituxan, and 1 year after completing maintenance Rituxan––has taken another unexpected turn, this time for the better. Her neurologist at DFCI cautions, however, that it is unlikely that her central nervous system is altogether clear of the cancer cells. None of the consulted experts can chart the next turn in the road for Julie or when it will come.
When she speaks of having faced so much alone over the past 5 plus years, Julie is quick to add that there is a hero in this story, one who has been unflinching in his support. Her hero is her husband, her companion at every critical turn, sharing the disappointment of bad news and the bursts of hope coming with good news. At the beginning of this difficult road, Wade told Julie that her job was to find ways to reduce her stress and use that energy to fight the cancer battle and that his job was to take care of her. Wade not only accompanies her on visits to the experts but also has taken on many household chores, including shopping and even preparing dinner after long and busy days at his “real job” for most of the last 5 years.
The pace of life today for Julie is far from the frantic schedule she kept before diagnosis, when her employment kept her on the run and her family said she worked “way too hard.” In fact, she lost her job early on in her personal saga. She now drives, runs small errands, and can cook 3-4 times a week, but she is unable to negotiate large grocery and retail stores. Her level of fatigue can be crippling, and she spends much time resting. Nonetheless she walks the dog roughly 1 mile a day and participates in a cardiac rehab type program 3 days a week where she works under the supervision of an exercise physiologist. However, even minor increases in time, reps, or resistance can cause major energy set backs or muscle weakness. Normally, she will have a few good days followed by a couple of days of debilitating fatigue. For the nerve pain in the left hip and thigh, the lower right side from the hip to the foot, and intercostal neuralgia, she sees a pain physician and her acupuncturist. Another “job” Wade asked her to do from the beginning was to challenge her mind, to help minimize “chemobrain,” by playing computer games. She has mastered those skills!! But, unfortunately, she still has significant chemobrain.
Looking back over the past 5 years, Julie adds the following observations. Each new cancer diagnosis brought on a flood of emotion. Each time the “alone again feeling” surfaced as those around her tried to understand the personal challenge of cancer but really could not. Friends continued to drift away. It seemed each new doctor’s appointment brought more bad news. After a while, she found that she had run out of tears. She became numb. Her response became “OK, we have a problem. What are we going to do about it?” Her doctors have been amazed at Julie’s ability to deal so well with adversity. She has borrowed her motto from the movie Finding Nemo: “Just keep swimming, just keep swimming.” Despite the fatigue, nerve pain, and other challenges, she says that every day she puts her feet on the floor is a good day. Currently, everything is in remission, much to the surprise of her doctors, as noted above.
In spite of the mark that Bing-Neel has left upon her life, it is clear that Julie is not defeated. She has been challenged by her disease and has risen to the challenge of learning all she can about it so that she may live better with it. She is now eager to help others feeling “all alone with Bing-Neel” and has assumed the role of active volunteer for the IWMF. Her name appears on the LIFELINE List as an experienced patient willing and ready to speak about the emotions and challenges of WM and BN and offer some information based on her own experience concerning diagnosis, treatment regimens, details about specific drugs. Her offer comes with “a broad shoulder, an ability to listen and support others,” when she writes:
“I am now listed as the resource person for BN on the IWMF support persons list. I am on the same road with those facing a BN diagnosis and can speak to experience. I am available by phone, Skype, or FaceTime, if you would find it helpful.”
Her email address is [email protected](link sends e-mail) and her cell phone number is 615-429-2017. She is quick to admit that she is sporadic with checking and responding to email. She asks your patience with email responses or welcomes phone calls for a more timely response.
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By way of a post script to this article, Julie Davidson noted that the isolation brought on by the cancer diagnosis can be greatly diminished if one acquires a “cancer friend,” one who shares your diagnosis and with whom you feel free to speak frankly about any and all aspects of the cancer survivor’s life. For Julie such a friendship developed with Grete Cooper through contact initiated via IWMF-Talk. Julie writes, “She and I “found” each other on IWMF-Talk and have supported each other through good and bad for about 5 ½ years. I cherish the friendship and she has become my ‘best friend.’ We can tell each other anything and everything.” Grete’s response when Julie wrote that she is preparing an article for the Torch is an excellent conclusion to this article when she says of her friend Julie Davidson:
“I cannot think of anyone else with your kind of stamina and will to live and thrive. You’ll be an encouragement to every Torch reader! Just a fleeting idea … the article may be an excellent opportunity to say something about how important ‘cancer friends’ can be to each other. I don’t know if you remember, but you once asked me if you could say something about it on IWMF-Talk. It’s a very important topic and the two of us are proof that it’s immensely helpful to have a ‘travel companion’ and Best Friend Forever. We have ‘traveled’ together for 5 ½ years ever since we were both diagnosed with WM.”
Recommended for further information about Bing-Neel syndrome: a recent publication by Dr. Jorge Castillo, Dr. Steven Treon, and 13 other oncologists who have treated patients with Bing-Neel: Central nervous system involvement by Waldenström macroglobulinemia (Bing-Neel syndrome): a multi-institutional retrospective study. British Journal of Haematology 172.5 (March 2016) 709–715. This multicenter study combines the records of 34 patients to describe the characteristics of the disease plus the diagnostic criteria, range of response to therapy, and other data, including survival statistics.