An Expert Offers Advice and Resources for Navigating Rare Lymphoma

Dr. Neha Mehta-Shah discusses resources, challenges and credible information to help patients better understand and manage rare types of lymphoma.

Although being diagnosed with a rare cancer, like lymphoma, can often create feelings of isolation in patients, Dr. Neha Mehta-Shah emphasizes the importance of remembering that you are not alone; millions of patients every year are diagnosed with a rare disease, she says.

In an interview with CURE, Mehta-Shah sat down to further provide patients with lymphoma messages of encouragement, as well as provide different resources for them. In the interview, she discussed how patients can better understand their specific subtype of rare cancer, how to sift through the abundance of information that can be found online (and how to verify it), as well as expands on the challenges patients with rare lymphoma subtypes face throughout treatment.

Mehta-Shah is an associate professor in the Division of Oncology at Washington University School of Medicine in Saint Louis, Missouri.

CURE: What initial steps do you recommend to help patients better understand their specific subtype of rare lymphoma, and feel more grounded during those early, uncertain days?

Mehta-Shah: Lymphomas are relatively uncommon types of cancers to begin with, and the term lymphoma actually encompasses over 100 different diseases. Each of those diseases is quite rare overall compared to some of the more common types of cancer that we encounter, like breast cancer, prostate cancer, or lung cancer. Consequently, I think most patients who have lymphoma feel emotionally that they are dealing with a rare diagnosis; some patients are truly dealing with ultra-rare diagnoses, which can make it really hard to figure out how to navigate their experience, how to arrive at their diagnosis, how to identify doctors who are familiar with their diseases, and then how to get credible resources to ensure they are advocating for themselves.

For example, patients who have T-cell lymphomas, those constitute only about 10% of all non-Hodgkin's lymphomas. However, that term represents almost 20 different diseases. Each of those diseases can be particularly rare. I think some of the things that patients can do to help ground and orient themselves is, first, the Lymphoma Research Foundation does a fantastic job of providing resources for patients who have rare diseases.

They have handouts written at a level that patients should be able to understand about the general overview of some of these diseases, how we approach treatment, and all of that information is really vetted and written by experts across the country. That information is highly credible and updated at least annually. Similarly, there are certain other patient education resources available from other nonprofit organizations. Some of these include the Cutaneous Lymphoma Foundation, the Waldenstrom's Macroglobulinemia Foundation, and the Leukemia & Lymphoma Society, which present certain similar patient education or patient-facing material, as does the NCCN, the National Comprehensive Cancer Network.

This organization sets the guidelines for how to treat and diagnose these diseases, but they also have a patient-facing education section. All of these resources are really meant to help orient patients as to how to approach their diagnosis, what to expect during the course of their workup for the disease, and then what treatment options are available, as well as resources to figure out when to consider clinical trials as part of their treatment.

Could you elaborate on some of the key challenges these rare lymphoma subtypes pose for both clinicians and patients when it comes to diagnosis and treatment planning?

I think there are a lot of challenges faced by both patients and their families, as well as the physicians. Some of these diseases are quite rare, in that a doctor you might see in the community may have never encountered the disease, or may have encountered it only years ago, or maybe sees one case per year. Consequently, they often feel like they lack the full clinical experience to guide the patient effectively. That is certainly a challenge. However, we know that most people don't live within walking or driving distance of a large academic medical center, and so sometimes getting care and advice from people who have dealt with this before or who can help guide patients requires traveling to some of these centers.

The NCCN publishes which centers are involved in writing their national guidelines for these diseases, and the NCI has something called NCI-designated comprehensive cancer centers. Usually, there's someone at these major cancer centers who can guide patients, and there usually is one in most major cities, so they tend to be within drivable distance for most patients. It's not uncommon for us to see patients for a one-time consultation or only at key time points in their disease journey.

So, coming to one of these centers doesn't mean you're going to have to get all your care there. You certainly could opt to, but you don't have to, and most of the doctors at these centers are used to partnering with community doctors to help deliver care closer to home. Sometimes getting information about a more personalized treatment plan does involve traveling, which is certainly a big challenge for patients, but also for their family members who are often accompanying them.

I think for doctors, some of the challenges involve knowing what the first, second, and third steps are in terms of treatment, because in some of these rare diseases, there aren't large studies of thousands of patients where half got treatment X and half got treatment X plus Y. The types of trials available for diseases like breast, lung and prostate cancer are different. Some of the experience comes from expert panel discussions and consensus statements, and some of the data comes from looking at retrospective studies, like looking back at patients who have had that disease and determining which treatments seemed to work better by looking at their treatment course over time. Those studies are just not as robust as prospective randomized studies, where people are enrolled and half get treatment X, and half get treatment X plus Y.

Sometimes you have to extrapolate information from those types of retrospective or smaller studies, and it can be hard for a clinician to interpret them because you're kind of grasping at trying to make conclusions based on small numbers and limited experiences and understanding how those studies compare to others. It can be a little bit tricky because it's sometimes hard to find all that information, especially if it's not an area where you're well-versed, which I would say is true for most people. So, I think doctors can also rely on some of these national guidelines for advice.

The NCCN tries to provide providers with some basic principles of how to approach treating these rare diseases, how to go about the diagnosis, and what the options are for treatment, either FDA-approved or treatments commonly used by doctors who are more experienced in those diseases. We call that Compendia-listed. So, I think that can really help physicians take care of patients with rare diseases—these guidelines, and then certainly physicians also have resources through these nonprofit organizations like the Lymphoma Research Foundation, in reaching out to people at the Lymphoma Research Foundation to identify where they can advise patients to go for next steps or who they can reach out to for other treatment options.

How can patients with rare lymphoma navigate the abundance of online content to find credible resources specific to their disease?

Yeah, I think there are so many resources available online that it can become totally overwhelming, and it's hard to tell who is offering or vetting some of that information. I think nowadays, there's so much information embedded in social media, and some of that information is really, really great, while some of it is not as well vetted or validated, making it hard to know what to trust.

However, I think the information from nonprofit organizations is very useful because all those organizations have scientific advisory boards, and the material they put out there, whether it's from the Lymphoma Research Foundation, the Leukemia & Lymphoma Society, or the NCCN, all gets reviewed thoroughly by physicians who are experts in the field. They also have a mission and a plan to update it very regularly, so you know that information is pretty up-to-date, readily available, free, and validated by experts in the field as well. That's super, super information.

Similarly, some of those organizations have patient education events, whether they're via Zoom, video-recorded and streamed, or on YouTube, where you can attend a little seminar about your disease and how to navigate different issues from the comfort of your couch or bedroom. Certainly, many of these nonprofit organizations do such work, and similar work is being done through many other patient education and outreach organizations, and most of those are on YouTube. If you look and recognize that the person giving the discussion is someone known in the field, then I think that's also pretty valid information and tends to be very well-balanced.

The things to be a little bit careful about are some of the patient support groups online, which I've found can be a little bit difficult for some patients. Some patients really find a lot of gratification in being part of those, along with reassurance, a sense of connection, and a sense of community, and that's terrific if that's you.

However, there are other patients who realize that these groups are predominantly filled by people who have had a more difficult time with their treatment or are more vocal about their difficulties. Some of my patients have felt like they've become friends with these people on social media, and then when something sad or unfortunate happens to them, they've realized they carry a bit of survivor's guilt about that situation. If that's you, it's okay not to be part of those groups if it's not helping you on your journey.

Also, know that patients who tend to be doing well tend not to post a lot of information, so those forums tend to be skewed towards patients who are looking for more guidance about their next steps or what to do next, and not necessarily telling the stories of people who are not really thinking about their cancer and are going out and living their lives. So, just be aware of that and have an internal dialogue about what helps you go through your own journey.

Transcript has been edited for clarity and conciseness.

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