Putting “Risk” in Perspective

I am considered a “high risk” multiple myeloma patient because of a del17 chromosomal abnormality. What exactly does that mean, and how does it impact my future survival with this disease?

“Did your other doctor talk to you about your cytogenetics?” My current doctor, a myeloma specialist, asked me this while giving me a second opinion on my diagnosis.

It was the first time I’d ever heard the term cytogenetics. My doctor ex­plained that I had a “del17” chromosomal abnormality, which made my dis­ease “high risk” and potentially more aggressive than that of the majority of other multiple myeloma patients. Two years later, I under­stand this concept better, but occa­sionally my gut reverts back to that day and I become frightened that being high risk will prevent me from enjoying a longer, healthier remission.

Patients typically undergo a bone marrow biopsy as part of their initial diagnosis. Many labs will, in addition to standard diagnostic tests, perform fluorescence in situ hybridization (FISH) testing looking for any chro­mo­somal abnormalities in the cancer cells. One such abnormality, del(17p), occurs when one of the “p” arms of chromosome 17 is missing. Patients with this abnormality, and others, are con­sidered high-risk patients. While only a small number of patients are con­sidered high risk, when you are one those patients it doesn’t matter how prevalent it is.

Being labeled high risk sounds scary, and in my research I often read that a finding of a del(17p) abnormality is associated with “poor prognosis.” Without understanding what this language really means, it was easy to believe my multiple myeloma was going to win the battle and my time remaining is shorter than most patients. That simply isn’t true.

High-risk multiple myeloma does not mean treatment failures are a certainty, nor is it a guarantee of shorter life. Being a high-risk patient may require extra diligence in evaluating treatment options, and may mean receiving more medication or even con­sidering more experimentation to find a treatment that will keep the multiple myeloma at bay. It doesn’t, however, guarantee a negative outcome.

In terms I can understand, high risk means that I stand a better than average chance of relapsing sooner than most, and a better than average chance that my multiple myeloma will become resistant to treatment quicker than most. Like all statistics surrounding multiple myeloma, these are just statistics regarding how patients in this subcategory generally fair in relationship to patients without these abnormalities.

I found an interview with Dr. Ola Landgren in a 2012 Myeloma Beacon article particularly helpful to put the concept of “high risk” multiple myeloma in context. Dr. Landgren suggests that if all multiple myeloma patients received limited treatment, everyone would be con­sidered high risk. Their initial responses would be low, remissions would be short, and when relapse occurred, there would be no treatments left to battle the disease. On the other hand, if there were a cure for all forms of multiple myeloma, every patient would be low risk. Today, there are many effective treatments for the vast majority of myeloma patients, but not all patients respond the same. As he explains, “‘Risk’ is an interaction between biology and intervention. And intervention is a variable that changes between clinicians and institutions, and it is constantly changing over time.” So, high-risk myeloma is not inherently bad, it is just a type of myeloma for which research has yet to find a con­sis­tently effective treatment.

Multiple myeloma research and treatment is running at a break neck speed. I received three cycles of Revlimid (lenalidomide), Velcade (bortezomib), and dexamethasone (Decadron) before I stopped responding. I immediately started Kyprolis (carfilzomib), Pomalyst (pomalidomide), and dex and saw a fast and deep response. Recent research indicates that both Kyprolis, and Pomalyst have benefits for del(17p) patients in comparison to Velcade and Revlimid. There is research of other treatments targeting high-risk patients. If future treatments prove effective for patients with abnormal chromosomes, we too might be considered standard risk in the near future.

Because I’m del(17p), my medical team and I decided to treat my multiple myeloma aggressively. We knew from the start that I would have an autologous stem cell transplant. While there is a reasonable debate whether a transplant is a necessary front-line treatment for standard-risk patients who respond well to initial treatments, it was not a debate for me. It is also my understanding that most myeloma specialists recommend up-front stem cell transplant for high-risk patients.

I will also follow an aggressive maintenance path. I’m receiving a lower dose of the same medications that I received in my effective induction treatment. I will likely stay on this program at least three years barring relapse or serious side effects. This plan means we will be more vigilant for symptoms of toxicity. I suspect I will always receive some type of maintenance.

Being high risk does not, however, change the way the disease acts in my body or how side effects of the treatments affect my body. I’ve been lucky on both accounts. We attacked the multiple myeloma before it did any serious damage anywhere, and I’m no more at risk of further bone damage or kidney failure, for instance, than any other multiple myeloma patient. So far treatment has been unremarkable, with good responses and mild side effects.

I’m approaching two years post diagnosis, over six months post transplant, and my doctors are happy with my progress and I’m feeling healthy. Nobody can predict how long my remission will last or what the future holds. “High-risk” or “standard-risk” predictions are just based on statistics, and those statistics don’t take into account my personal information, and they don’t predict how new treatments will behave in the future. Haven’t we all been told, “you are a statistic of one?”

Admittedly, the gremlin hiding in the back of my head occa­sionally reminds me that I’m a high-risk patient, and that some of the reports and statistics offering great hope to the majority of multiple myeloma patients may not apply to me. The gremlin tries to put a damper on my hope and get me to forget how good I feel and how lucky I am to still be able to spend quality time doing the things I like and being with the people I love.

When that happens, I lock the gremlin away and remind myself what it really means to be high risk.

In the end, I’m like all multiple myeloma patients. I trust my doctors and the rapid pace of the progress fighting this disease to keep me with my family for many, many more years.

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Photo Credit: (c) 2013 Mark Pouley

My photo “Shells on the Shore” tells a more complete story when you know the context and understand the perspective of the photo. These empty clam shells are on the shore of the Tulalip Bay of Washington’s Puget Sound. They were likely placed there following a family feast, a tradition of the native Tulalip people for millennia, as their sustenance and culture revolves around the natural bounty of the water. The photo was taken by placing the camera low and into the shells themselves, altering the normal perspective of a person standing on the shore.

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