Spencer’s Stage 3 High-Risk Multiple Myeloma Story
Interviewed by: Taylor Scheib Edited by: Katrina Villareal
Spencer, diagnosed with stage 3 multiple myeloma in June 2023 at the age of 39, reflects on his experience from the onset of symptoms to treatment. Initially, Spencer experienced discomfort in his rib cage and chest area, which persisted despite normal X-rays. He later developed pneumonia and, during his ER visit, was informed of his anemia. Follow-up blood work confirmed low white and red blood cell counts, prompting a referral to a hematologist. Initially, the hematologist was unconcerned, but after further testing, Spencer was diagnosed with stage 3 multiple myeloma, a term unfamiliar to him at the time.
Following the diagnosis, Spencer began aggressive treatment, including chemotherapy and immunotherapy to prepare for a stem cell transplant. His first chemotherapy cycle was particularly challenging and he suffered significant side effects, including strep throat and severe discomfort from the medications. The side effects lessened in subsequent cycles and after completing chemotherapy, a biopsy and PET scan revealed no signs of myeloma in his bone marrow, allowing him to proceed to the next stage: an autologous stem cell transplant.
The stem cell transplant process was intensive. Spencer first underwent stem cell collection, which was interrupted by a COVID-19 diagnosis. Despite this setback, the procedure was eventually completed successfully and the transplant itself involved high-dose chemotherapy before reinfusing his harvested stem cells. Spencer faced challenges post-transplant, including extreme fatigue and lack of appetite, but was fortunate to avoid the more severe side effects often associated with the procedure.
Following his transplant, Spencer entered a maintenance phase. He focused on improving his physical health through walking and running, even signing up for a marathon. His treatment and recovery allowed him to reconnect with family and friends and adapt to his “new normal” life. He emphasizes the importance of finding hope in difficult situations and now actively participates in a myeloma support group, where he encourages others, especially younger patients like himself.
Spencer is determined to maintain his health and well-being, recognizing the long-term nature of managing multiple myeloma. His experience highlights the significance of choosing the right medical team, staying active, and embracing support networks to navigate the challenges of living with cancer.
CLL 360°: Navigating Side Effects with the Experts
Edited by: Katrina Villareal
The Patient Story Webinar – CLL 360°: Navigating Side Effects with the Experts
Hosted by The Patient Story
You don’t have to endure uncomfortable side effects just because it’s part of cancer treatment. Empower yourself with the latest information to make informed decisions about your health, get effective care, and get the most out of life. Listen in on a crucial discussion on Chronic Lymphocytic Leukemia (CLL) with Dr. Matthew Davids from Dana-Farber Cancer Institute and Dr. Kerry Rogers from Ohio State University Comprehensive Cancer Center. This webinar will equip you with the knowledge to better manage CLL side effects and improve communication with your healthcare team.
Hosted by CLL patient advocate Jeff Folloder, this crucial discussion on chronic lymphocytic leukemia (CLL) with Dr. Matthew Davids from the Dana-Farber Cancer Institute and Dr. Kerry Rogers from The Ohio State University Comprehensive Cancer Center will equip you with the knowledge to manage CLL side effects better and improve communication with your healthcare team.
Understand the latest advancements in CLL therapy and their impact on patients. Gain practical advice on managing the side effects of CLL treatments, including BTK inhibitors. Learn how to balance treatment efficacy with tolerability to improve your daily life. Discover ways to communicate your concerns and treatment preferences with your healthcare providers.
This interview has been edited for clarity and length. This is not medical advice. Please consult with your healthcare provider for treatment decisions.
Thank you to Pharmacyclics, an AbbVie Company and to Johnson & Johnson, for their support of our patient education program. The Patient Story retains full editorial control over all content
Stephanie Chuang: As part of our CLL 360° series, this topic is navigating side effects with the experts, both on the patient side as well as the medical side. The goal is for you to walk away with a better understanding of what side effects are commonly driven by CLL treatments and how to help manage them, including how to have conversations with your medical team about them.
This program is hosted by The Patient Story. Our multi-platform organization aims to help people navigate life before and after diagnosis through in-depth patient stories and educational discussions, which we understand is especially important for a community like CLL where you’re all so engaged and want to empower yourselves continuously.
We want to thank The Leukemia & Lymphoma Society for its partnership. The LLS offers incredible free resources, like their Information Specialists who provide one-on-one support in different areas for blood cancer. We also want to thank The CLL Support Group on Facebook led by our friends, including our moderator Jeff Folloder. They provide virtual support anytime for folks dealing with a CLL diagnosis.
We want to thank our sponsor Pharmacyclics, an AbbVie company, for their support, which helps us to host more of these programs for free to our audience. The Patient Story retains full editorial control over all the content. While we hope this will be very helpful and that you walk away with a better understanding of this topic, this is not meant to be a substitute for your own medical advice.
Now we get to the great discussion ahead and I’ll send it to Jeff.
Jeff Folloder, CLL Patient Advocate
Jeff Folloder: I am a passionate patient care advocate. Why am I an advocate? Because I know that it is truly possible to live a great life with a CLL diagnosis.
I went through a clinical trial many years ago. I experienced side effects, which were quite profound. I know so much has changed. There are still side effects, but we take care of them differently.
We have two fantastic medical experts to join us. Dr. Matthew Davids is an associate professor of medicine at the Division of Lymphoma of the Harvard Medical School. He’s a hematologist-oncologist at the Dana-Farber Cancer Institute where he serves as director of clinical research and associate director of the Center for Chronic Lymphocytic Leukemia. Dr. Davids, why CLL?
Dr. Matthew Davids, Hematologist-Oncologist
Dr. Matthew Davids: I was very fortunate to grow up in the era when we were starting to transition away from chemotherapy to targeted therapies. We could begin to develop drugs with fewer side effects and more effective than chemotherapy. I was inspired by that and the relationships with my patients, and I haven’t looked back.
Dr. Kerry Rogers, Hematologist-Oncologist
Jeff: Also joining us is Dr. Kerry Rogers. She’s the assistant professor in the Division of Hematology at The Ohio State College of Medicine and a hematologist-oncologist and researcher at The Ohio State University. Dr. Rogers, why did you choose CLL?
Dr. Kerry Rogers: When I was doing my fellowship in hematology and medical oncology, I had the privilege of taking care of patients in some of the original ibrutinib clinical trials who had been living with CLL for over a decade or two and had experienced a lot of chemoimmunotherapy treatment. They said ibrutinib was the best that they’d ever had in terms of how it impacted their daily lives and so I thought that was intriguing. Now we know that these drugs are not only generally more tolerable but also more effective than chemoimmunotherapy.
I like the long-term relationships you can have with patients and the variety of experiences with CLL. You have people who are quite ill, who have experienced a lot of treatment, may have had CLL that’s become resistant to some of those treatments, and need to do a lot of things to manage the CLL. You also see people who are living with it who currently do not have symptoms or do not need treatment.
I also like the fact that there are some people you encourage to do what they want with their lives and not focus on having cancer. Mostly, I’d like all my patients to be bothered by CLL as little as possible, but you do get people in a variety of situations and I appreciate that about working in CLL.
Everyone reacts completely differently to the treatments. For most patients, treatment side effects are common and can be impactful. Some of them are temporary, but some of them are more severe.
Impact of Recent Treatment Advancements on Side Effects
Jeff: I participated in a clinical trial where I received intravenous monoclonal antibodies. Like everyone who participates in a clinical trial, I received a stack of informed consent papers that looked to be about a foot and a half thick and described every single side effect that might happen with the administration of that drug.
The first time that I went to get the infusion, I was told to expect that it was going to take four to six hours. Unfortunately, it took 23 1/2 hours for me because I experienced pretty much every single one of its side effects. We got through them all. When the side effects happened, they were treated and we’d hit the reset button and start over again. As I progressed through the clinical trial, the side effects were minimized.
We’re in a different world now. Everyone reacts completely differently to the treatments. For most patients, treatment side effects are common and can be impactful. Some of them are temporary, but some of them are more severe. Sometimes treatment or even hospitalization is required. And then some people, for whatever reason, never have a single side effect.
Dr. Davids, the treatment landscape for CLL patients has truly evolved in the past couple of years. Could you discuss these advancements and how the side effects of CLL therapy, more specifically the BTK inhibitors, have changed over time?
Dr. Davids: To contrast with the older chemotherapy-based approaches, I told patients, “We’re going to start treatment. You’re going to need to take probably 6 to 12 months off from work and go on disability. Your whole life is going to change over this timeframe, but eventually, you’ll get better and get back to what you’re doing.”
When BTK inhibitors like ibrutinib first entered into trials, we quickly realized that these drugs are much better tolerated than the older chemotherapy-based regimens. There may be some side effects that arise, but we’ll deal with them. We’ll use various supportive care measures. We may need to reduce the dose, but you should be able to keep doing the things you want to be doing. You can usually keep working while on treatment.
We have venetoclax, which we usually give in a time-limited fashion for one or two years, and it has a different side effect profile than the BTK inhibitors. The nice thing about that is with a time-limited treatment, when we stop the treatment, usually the side effects go away or are minimized at least. There are lots of different options.
Jeff: When we first started treating CLL, the treatments were pretty drastic and the side effects were pretty intense for the majority of people. Now, with the new treatments, specifically the BTK inhibitors and drugs like venetoclax, the side effects aren’t as intense and are usually easier to treat. Is that a fair statement?
Dr. Davids: Yeah, that’s a fair statement. There are rare situations where you can have more severe side effects, but for the most part, most of our patients are doing very well with these new targeted therapies.
Relationship Between Side Effects and Quality of Life for CLL Patients
Jeff: For me as a patient, quality of life is at the top of my list. If I can’t do the things that I want, we’re going to have a conversation and figure out how to get there. Quality of life is a complex and personal concept, but it’s directly intertwined with cancer treatment side effects. Dr. Rogers, how do you define the quality of life for a CLL patient and how do the side effects of treatment impact this in your experience?
Understanding what matters most to the patient or what’s going to have the least impact on their enjoyment of their life is what we do.
Dr. Rogers: If we’re trying to decide between two targeted agents that are both good options with different side effects, understanding what matters most to the patient or what’s going to have the least impact on their enjoyment of their life is what we do and it can be different for different people.
With a BTK inhibitor, some of the common side effects are bleeding, bruising, joint pains, and abnormal heart rhythms like atrial fibrillation. I have a few patients where atrial fibrillation has been much harder for them than the CLL was at any point. If you’ve had someone who’s experienced atrial fibrillation before and it was bothersome to them, they’re going to try to avoid that treatment.
Treatment Side Effects vs. Treatment Schedule
Dr. Rogers: Meanwhile, for venetoclax, quality of life isn’t treatment side effects but also treatment schedule. When given as a first treatment, venetoclax is given for a year with obinutuzumab, which is a monoclonal antibody, and that requires an eight-week start-up. It was very striking what Dr. Davids said that we try hard now to make it clear to patients that we don’t want them to be disabled by the treatment they’re going through, even temporarily.
We mostly want you to be able to live your life and do what you want.
People have told me they worry about their lives being over and not being able to do anything when they do treatment. I usually tell them that some people get severe side effects that have to be dealt with, but we mostly want you to be able to live your life and do what you want. That’s how I define quality of life as a physician. I want my patients to be living their lives and doing the things they enjoy with minimal impact from their treatment.
These days, one of the biggest impacts can be the treatment schedule. You can continue to work or play golf, but you still have to come for visits and that’s the biggest thing that changes with treatment. The venetoclax and obinutuzumab regimen requires an eight-week start-up, which can be intense. I’ve had patients who choose not to do that because they don’t want to take time off work to go to infusion visits.
I’ve also had patients take online meetings during infusion visits, which I’m fine with as long as they’re still engaged in their medical care. I don’t tell anyone they have to work during treatment but some people love their job and don’t want to step away from it.
Quality of life is defined by the person and I try to minimize the impact of what we’re doing for CLL on people’s enjoyment of their life, whatever that is. Something that isn’t talked about a lot is trying to minimize the psychological impact of starting treatment. We make sure people know that treatment doesn’t mean that they won’t get to do the things they like, even though scheduling is something they’re going to have to deal with for the most part.
Dr. Davids: If they do want to do the venetoclax-based regimen, it’s a bit disruptive to their schedule. When patients aren’t on a clinical trial, I try to have some flexibility so that if they have an important event, we can delay by a day or two, but I try to keep them on schedule. It can be a little more challenging when patients are on a clinical trial because we need to rigorously adhere to a specific schedule.
For some patients, it doesn’t make sense to invest eight weeks or so in coming in for frequent visits. A lot of our patients are used to being seen every three to six months. If we tell them to come in once or twice a week for a couple of months, that can be challenging. Some of our older patients don’t drive anymore and need to get rides or live far from the center.
One of the beauties of the CLL treatment setting right now is that we have other options. Patients like that can go on BTK inhibitors. They require minimal monitoring and for some patients, that’s nice for their quality of life. They come in a couple of times early on to make sure everything’s going well and they can get back to their three- to six-month interval schedule.
Having different options for different patients is great. We’re on the precipice of having a new regimen approved in the US, which is a combination of acalabrutinib, one of the BTK inhibitors, with venetoclax. That’s going to be a nice advance because it will offer the opportunity for an all-oral, time-limited regimen.
One of the beauties of the CLL treatment setting right now is that we have other options… Having different options for different patients is great.
Right now, if you want all-oral therapy, you have to go with a BTK inhibitor and take it long-term for many years or you can go with obinutuzumab and venetoclax and have that opportunity for the one-year, time-limited therapy.
If approved, which we think it likely will be, acalabrutinib with venetoclax will be two pills. It’s a time-limited treatment of about 14 months or so and then you’re done. You don’t need to go through obinutuzumab infusions and that’s going to be a nice option to have for patients as well.
Dealing with Side Effects
Jeff: Whether you’re being treated, waiting for treatment, relapsed, or in remission, all CLL patients are dealing with some kind of symptom. It’s a fact of life, whether we call it fatigue, low platelets that make people bleed easily, joint pain, or AFib. Dr. Davids, what kind of practical advice do you give patients for dealing with those symptoms?
Dr. Davids: The disease itself can cause a lot of symptoms. There’s an interesting study where they took CLL patients who didn’t need treatment and randomized them to an early intervention approach with ibrutinib versus a placebo. It’s a rare opportunity in CLL to see what happens in a population being treated with a placebo.
As they stay on therapy longer and the CLL gets into a better remission, they tend to start to feel better overall.
In that study, there was a 90% rate of side effects in the patients on ibrutinib and a 90% rate of side effects in the patients on placebo. It was the same in both arms. That speaks to how the disease itself can cause a lot of issues, like fatigue, unintentional weight loss, sweats, and joint aches.
I counsel my patients that as they stay on therapy longer and the CLL gets into a better remission, they tend to start to feel better overall. They’ll develop better energy, their appetite may come back, they may gain some weight back if they’ve lost weight, and sweats can go away.
One of the more challenging periods is getting started on treatment because that’s when you have some side effects from the drugs and there’s still a lot of CLL around, so they may not be feeling great. As they get a few months into treatment, the CLL starts to get better. It involves counseling and coaching them through that. Hold out and be optimistic. It’s going to get better with time. For most patients, they may start feeling better pretty quickly, even within a few weeks of starting on therapy, because of disease control.
We have a very good multidisciplinary team. We have a great nurse practitioner who’s seeing the patients often more frequently than I do. We have pharmacists who help and look for issues with the drugs and side effects. The caregivers for the patient are a key part as well. It’s a team effort, but we do a pretty good job of getting patients through that more difficult period and to a better place where they’re feeling better.
How Can Patients Actively Participate in Their Care?
Jeff: Dr. Rogers, it’s hard for a doctor and the care team to know how to guide a patient through their side effects if they don’t know they exist. How do we do this? What does a patient have to do to get the best possible side-effect care from you and your team?
Dr. Rogers: One thing Dr. Davids said that’s important is I’m not the only person. Different members of the team are asking about side effects. Nurses, nurse practitioners, and physician assistants help too.
The nurse practitioner I primarily work with is a man and sometimes, patients tell him things that they won’t tell me or vice-versa. Sometimes the gender of the care provider or the care team member can make a difference in people disclosing side effects.
Asking about specific side effects that we know are common can help because I find people disclose more. If there’s one that they perceive is ruining their quality of life, we ask about it so we can get the spectrum.
Sometimes there are side effects that are bothersome to one person that aren’t to somebody else, like joint pain. We ask about it, learn about it, and suggest pain medication. But if the person’s happier living with it than trying any sort of intervention, I’m okay with that.
Effective Patient-Physician Communication
Jeff: Dr. Davids, it sounds like in the current landscape, most side effects are fairly easily managed for CLL patients. Let’s talk about how you deal with these side effects.
Dr. Davids: Every drug is different. Every side effect is different. Every patient is different. Sometimes we see more significant side effects with BTK inhibitors where we run into issues with bleeding and that can be serious.
Being very proactive and communicating with your doctor about what’s going on is important.
One thing that’s very important for patients on BTK inhibitors is to know that if they need any kind of surgical procedure, they need to stop taking their BTK inhibitor before and after the procedure. It’s helpful for patients to check in with us because every procedure is different and may require a different length of hold.
I have seen some patients run into issues where they forgot to call and ask us. They were taking their BTK inhibitor and had pretty serious bleeding issues after surgery. It’s about communication and anticipation. We’ve had situations where we’ve had to postpone surgeries. Being very proactive and communicating with your doctor about what’s going on is important.
Another common one that I see with venetoclax and BTK inhibitors is diarrhea or loose stools when patients are first getting started on these drugs. There’s a period when your body needs to adapt to the drug.
If it’s pretty significant diarrhea, we usually want to do some testing first before we do interventions. We’ll look for certain infections, like C. diff, which can be a more serious infection. It’s usually not there, but we like to make sure that’s not there before we recommend things like loperamide, which slows down the bowels.
There are little tricks as well. Sometimes taking the pill at a different time of day or with a different type of food can be helpful.With venetoclax, I’ve had luck with patients taking it with a fatty meal at night and when they wake up, they feel better and not having diarrhea during the day.
Sometimes we need to reduce the dose of the drug. I do find that it settles within the first couple of months. Most patients can then back off on loperamide and get back up to the full dose of venetoclax.
Overcoming the Fear of Dose Reduction
Jeff: Dr. Rogers, for whatever reason, a lot of patients are uncertain and fearful regarding dose reduction because it introduces a cloud of uncertainty to what’s going on.
Dr. Rogers: This is true across other cancers as well. People worry about dose reduction largely because they worry that the treatment will stop being effective if the dose is reduced.
With venetoclax, for example, the target dose for most people who don’t have drug interactions with it is 400 mg a day. However, when people have been on it for a while, there’s an efficacy at 300 mg or even 200 mg. If you’re taking 100 mg twice a week, you start to worry that it’s not going to work. There’s a point where you get to a dose where most people think that’s not going to be effective or with BTK inhibitors, you’re not going to have occupancy or the inhibitory action.
There’s a lot of room for dose reduction before you get to a point where the drug isn’t going to work. For people with side effects, maybe the target dose isn’t the right dose for them. When we do studies to design dosing of drugs, they look at drug levels in the blood, biological outcomes, and how much of that BTK protein is occupied with different dosing schedules. But that’s in a group of people and individuals can have different experiences.
There’s a lot of room for dose reduction before you get to a point where the drug isn’t going to work.
There’s some thought and some data to support this that people with side effects might naturally have different drug metabolism and maybe what’s needed is a different dose. With BTK inhibitors, there’s some scientific evidence that early in the disease, the drug is cleared by the amount of BTK in the body, which is a lot when there’s a lot of CLL, and further in is not a lot and the protein production is downregulated.
It’s quite possible that a lower dose is necessary to maintain that response after beginning treatment. Some of these side effects improve over time. People have a lot of CLL in their bodies. They see things improve every time they come for a visit. But a year in, when you’re looking at joint pain that’s interfering with your pickleball game when your blood counts look good, maybe it’s time to think about dose reduction. It’s also a time when the CLL is in a different spot and dose reduction might be more reasonable.
It’s quite possible that a lower dose is necessary to maintain that response after beginning treatment.
There are even some studies looking at databases of people using these drugs through insurance claims showing that reducing the dose did not result in a sooner time being prescribed a new treatment. It looked like it might even be going better for people probably because they were able to tolerate and stay on this drug.
It’s always worth asking your physician anything you’re worried about. If dose reduction is suggested, talk to them about whether they expect this will change your treatment outcome, but there are plenty of reasons to think that in most cases it will not. There’s a lot of room for dose reduction in a way that we know is not going to compromise how well the treatment works.
Fostering Effective Provider Communication
Jeff: We’ve talked about side effects. We’ve talked about the difference between male and female patients and how they communicate these side effects to their care team. Is there a way that you prefer patients to communicate with you?
Dr. Davids: I encourage patients to make a list of their issues ahead of time before coming to the visit. When you get to a visit, sometimes you’re like a deer caught in the headlights. Sometimes people don’t think of the things that have been occurring to them.
Keep a little notebook over the months in between the visits and jot stuff down. I find it most efficient to go through all those things in person together with the patient and counsel them through each one. I don’t mind if it’s a long visit.
A challenging mechanism that has developed is using a patient gateway to communicate, which has its pros and cons. It’s good for patients to be able to access their care team. But sometimes, over three months, patients send a message every time they think of a question, so they may be sending 15 to 20 messages in between their three-month visits.
It takes a lot of time on our end. We try to be as responsive as we can. We’ve hired additional staff to manage the volume of messages that come in because as you can imagine, if you have 10 or 15 messages from 200 or 300 of your patients, that’s a lot. I’m not saying that’s a bad thing, but we need to rethink how we do that.
If patients are having more acute side effects, I encourage them to page us directly. We’re available to call back and have teams to help support us as well. If you’re sitting on a more serious side effect, that’s not good. Communication is key, but be smart about how to communicate.
A challenging mechanism that has developed is using a patient gateway to communicate, which has its pros and cons.
Dr. Rogers: If there are things you want to discuss, make a list. I’ve had a couple of people who send a list of questions a week before their appointment through the patient portal. We have a list now so that they know what it is and I can read what it is. Some people take physical notes, some people have them on their phones. If there’s something that they want to discuss, decide ahead and use your visit time to do it.
The patient portal is an excellent communication tool, but you can’t be doing that every day, asking something new. We do those during business hours. I tell my patients that they’re welcome to write a note at 2 a.m., but we will not be replying until the next business day. Sometimes, you get people who send a bunch of concerns and I tell them that we can’t answer all of these on the phone and that we’re going to schedule a visit. Occasionally, we’ll provide education. This is a concern. This is something that should be handled by the phone, so we can call and get it sorted out.
I have some patients who don’t use MyChart and they have a different way to communicate with us. It’s good to have multiple approaches. In terms of making lists of questions and sending them ahead, whatever helps you stay organized so that you get the answers you need.
Something that has been very helpful for us is open notes. We have been required to provide all test results to patients and provide notes. Some people like reading them but some people don’t. Some patients couldn’t remember something I said so they went back and looked at what I wrote and that helped. They didn’t have to call because they saw my notes. Some people know how to look at those. Some people don’t. They’re not written in patient-friendly language.
Patients will get their blood test results before they even come to see me, so they can think of questions about their results and I can address that during the visit.
Think through if you want to open your test results on a weekend and do what’s right for you.
Overall, it’s good to allow people access to their health information immediately. But I’ve had a few patients who call us up very angry that we released imaging results on a weekend. We didn’t do that; radiology did. Then they thought they had no way to reach out to us about something they were terrified about, which is also not true.
We have a 24-hour answering service if people are extremely worried about a test result that was released to them by the system on a weekend. Understand the process and the value in it but also think through if you want to open your test results on a weekend and do what’s right for you.
Dr. Davids: I feel very similarly about test results. It’s great to have access to all your data, but you have to know yourself. If you’re the type of person who’s going to freak out if you see something strange in a report and it’s a Friday evening and no one’s going to be able to get back to you for a few days, don’t open the report.
I see our job as looking at those reports, filtering out the things that aren’t worrisome, and telling you what’s important. Patients will be very worried about a tiny little lung nodule that wasn’t described before. If it’s 2 mm, I’ll say that they don’t have to worry about it, but if you see that result first and I’m not immediately available, you may be worried for days. It doesn’t bother some people and if you’re one of them, look right away. You have to know yourself and what you’re comfortable with.
If you’re the type of person who’s going to freak out if you see something strange in a report… don’t open the report.
Dr. Rogers: We have a system where if it’s a result that needs emergent action, which rarely happens in my clinic, the radiologist will call me so I can contact the patient. We don’t release test results with emergent findings without anyone on the healthcare team being notified, which I think some people don’t know.
Jeff: For the past couple of years, as I sat in the waiting room after having my blood work done, I have a very distinctive alert from my phone as my test results start coming in. I’m one of those people who are face down on the phone almost instantly because I want to know what questions to ask my doctor when I go in to review those results.
I’m coming up on 15 years of living with CLL and I’ve learned that the most important thing is not one test result; it’s the trend of the test results and how things are going over time. Not everyone is there yet and this is part of how we communicate with our care providers. This is how we work with our team.
Clinical Trials in CLL
Jeff: We’ve been talking about BTK inhibitors and it seems like the drugs keep on coming, one after the other after the other. When I started, it was pretty much FCR (fludarabine, cyclophosphamide, and rituximab) or nothing.
Let’s talk about how we got here. We’ve been doing all this research. We have CLL patients participating in clinical trials. We have the data coming back. How do you anticipate these advancements and how they’re going to impact treatment decisions? Are people going to put off starting treatment because something new is right around the corner?
Dr. Davids: We wouldn’t have all these great new drugs if patients didn’t go on clinical trials. The patients who came before helped bring these drugs to the patients of today. Patients of today also should consider clinical trials because you can help to develop the next wave of drugs for the next wave of patients. Patients deserve a ton of credit for making all this possible.
We wouldn’t have all these great new drugs if patients didn’t go on clinical trials.
Ibrutinib was a huge advancement. When I heard about a new BTK inhibitor coming along, acalabrutinib, I was skeptical at first. We already have a great drug with ibrutinib. Why do we need a second BTK inhibitor? Acalabrutinib turned out to be as equally effective as ibrutinib but with fewer side effects. It was a big innovation that acalabrutinib came along.
Then zanubrutinib came along and I said the same thing. Why do we need another, more selective BTK inhibitor? It turned out that in one study, zanubrutinib is better than ibrutinib in terms of how well it controls the CLL and is better tolerated.
Pirtobrutinib works very differently from the three BTK inhibitors and it can work even after all three of those other drugs stop working, so that’s a big advance. I’ve been proven wrong at each step along the way.
It’s helpful to have four different BTK inhibitors. I’m trying not to make the same mistake with a new class of drugs called BTK degraders, which are in early-phase clinical trials. This is another way to target BTK and they’re showing some significant promise.
It’s possible and very likely that eventually, we’ll have at least a fifth BTK inhibitor approved for this disease. It’s not what we’d call me-too drugs where it’s the same drug with a different label. These are all different drugs. They all have pros and cons and there’s a role for each of them in the treatment of CLL. It’s exciting times and continues to be exciting for patients to have all these options continuing to evolve.
It takes some courage to try new combinations or schemes for giving them.
Dr. Rogers: I completely agree. The only reason that we have these drugs is because people were willing to participate in clinical trials. I also see people who are afraid of participating in a clinical trial for fear of getting a placebo. Some of the lack of understanding of what research participation is a barrier to people doing it. It takes a lot of courage to do something where we don’t have all the information about how this particular treatment scheme is going to work.
A study we have right now for people going through their first treatment is a combination of currently approved drugs. Mostly, when people hear what it is, they say, “Okay, neat. These are drugs that you’re talking about as a standard of care.” It takes some courage to try new combinations or schemes for giving them. I always try to remember to cover that people will not be getting a placebo because we don’t have that in any of our current CLL trials.
Someone said once that participating in a clinical trial is like getting tomorrow’s treatment today. For people who are worried or don’t want to take an investigational drug, I remind them that these currently approved drugs were drugs that people got as research participants before they were on the market. They were able to benefit from what is a highly effective therapy before it was commercially available. This can directly improve the type of treatment people are getting, even though there’s some element of unknown because it’s a research study and we’re trying to learn more about these drugs.
For anyone considering research participation, definitely asking what’s known about the drug, where it is in development, and what the goal of this study is can help people understand better. When people are excited about approved drugs, we were able to treat people with those same drugs before they were commercially available because they participated in research.
These currently approved drugs were drugs that people got as research participants before they were on the market.
I would never suggest a clinical trial that I didn’t think was going to be highly beneficial to the person who was going to take that treatment. We’ve talked about choices for taking a first targeted agent, but there are people who have had CLL that’s now become resistant to several targeted agents. That’s where drugs we have under investigation are much more likely to be effective for their CLL than drugs that are currently on the market and a huge reason for people to consider research participation. At that point, you’re going to get something that we know is very likely to be better than commercially available drugs.
Shared Decision-Making in Your Care
Jeff: What you were saying got me thinking about this process as a collaborative process. It used to be like it or not. The paradigm was a patient presented with an illness, they saw the doctor, the doctor did an evaluation, the doctor prescribed a course of treatment, and the patient took the treatment. Things have changed now.
We have a lot of options. There are four BTK inhibitors. There’s venetoclax. There are monoclonal antibodies. There’s no treatment. I would like to think that we’re moving closer to a collaborative environment where the care team does the decision matrix with the patient. Dr. Rogers, how do you foster that environment with the patient?
We’ll discuss the treatments and by the time we’re done talking, it’ll be clear what to do and we can decide together.
Dr. Rogers: I strive to make my clinic a collaborative environment. When the venetoclax and obinutuzumab regimen was approved, you have BTK inhibitors versus venetoclax and obinutuzumab. I had a lot of consultations with people considering their options for the first treatment and asking me what they should do. Until I get to know you, I can’t help you. I can’t tell you what to do.
Usually, I say, “Why don’t we talk? I have some questions for you. You probably have some questions for me. We’ll discuss the treatments and by the time we’re done talking, it’ll be clear what to do and we can decide together.” That’s the best approach.
Some people don’t want to get options. They’re very overwhelmed by the options, so they want a recommendation. After a discussion, they say, “I don’t want to think about this. Ask me what you need to know to make the best recommendation for me, but I don’t want options. I want you to tell me what I should do.”
I find it difficult to pick for someone. I have to ask enough questions so that I can make the recommendation for what I think will be best. I respect the fact that some people get overwhelmed by the choices, don’t want to have options, and want to be told what to do. That’s not the way I prefer to do things, but if that’s what the person who I’m taking care of wants, then I try to do that.
I try to narrow it down. Often, there will be 1 or 2 options that I would prefer for the patient.
Dr. Davids: I have a very similar approach, I would say. Some patients prefer to be told what to do and it’s important to ask what their preference is upfront about how they want to approach it.
Another scenario is completely leaving it up to the patient. Present the options as all being equal and let the patient choose. I tend not to do that so much either, so I try to narrow it down. Often, there will be 1 or 2 options that I would prefer for the patient.
Sometimes you get the dreaded question: what would you do if this was your sister or your mother? I would recommend to a patient anything that I would for a family member because I believe in it no matter who I’m recommending it for.
There are scenarios where we’ve gotten down to maybe two or three options and the patient will say, “I would lean more toward this one based on what you’ve told me, but these other choices are reasonable too, so think it over.” Ultimately, it’s the patient’s choice. I see my role as guiding them to that choice and sometimes it does help if I express what my thought is for a particular patient. A lot of patients find that helpful.
Final Takeaways
Jeff: If a brand new CLL patient comes into your clinic and you’ve got 60 seconds to give them one message, what do you tell them?
Dr. Rogers: I usually tell people that we expect them to live with this for a very long time. The goal is to help them live as well as possible and be the least bothered as possible by CLL.
Dr. Davids: Depending on their age, for patients diagnosed with CLL in their 70s and 80s, I always say to them that our goal is to have them live whatever their normal life expectancy would be otherwise. We have very effective treatments and it’s unlikely that CLL is going to shorten your lifespan.
For younger patients, I don’t feel as confident saying that yet because if you’re diagnosed with CLL in your 50s, we don’t know what happens with these targeted therapies 30 years from now. I tell those patients that we already have a lot of good treatments, but over the next few years, we expect even more good treatments to be developed. The longer you live with CLL, the longer you’re going to live with CLL because we’ll have more and more treatments available. I also try to be optimistic.
Jeff: I was diagnosed with CLL at 46 and I’m now 60. Currently, I’m knocking out between 40 and 55 miles every week speed walking. I’m eating well and drinking well. I’m being a good father and husband. I’m doing all the things that I would want to do if I didn’t have CLL. I’m living a good life. It’s my mission as a patient advocate to make sure that it is possible to live a very good life, even with a CLL diagnosis.
I take an active role in my care. I listen to my care team when they say I need to do this, this, and this. I do what I need to do to present my body to my care team in the best possible shape so that when it is time to treat, we get to pick the right treatment plan so that I can live my best life. That is literally what I want for every single patient and caregiver. You can live a very long, very good life with CLL.
Conclusion
Stephanie: Thank you so much, Jeff, for your guidance in the conversation and for your perspective. We always want to make sure that we are emphasizing what that patient and care partner voice is. It is critical obviously beyond the education here.
Thank you so much, Dr. Rogers and Dr. Davids, for not only being incredible as CLL specialists but also for providing so much more time and extra time to help the community beyond the people and the patients you see in the clinic.
We also want to say thanks again to our sponsor Pharmacyclics for its support of our independent patient program and special thanks also to our partners, The Leukemia & Lymphoma Society and The CLL Support Group.
Thank you so much for joining us. I hope that this was helpful in one way or another. We hope to see you in a future program. Take good care.
Thank you to Pharmacyclics, an AbbVie Company and to Johnson & Johnson, for their support of our patient education program. The Patient Story retains full editorial control over all content
The Latest Treatment Options for Relapsed/Refractory Follicular Lymphoma
Edited by: Katrina Villareal
Follicular Lymphoma 360: The Latest Treatment Options For Me
Hosted by The Patient Story
Discover the latest in treatment options for relapsed/refractory follicular lymphoma from leading experts. This insightful webinar covers everything from the nature of the disease to innovative therapies and patient support. Don’t miss this opportunity to learn and connect with those at the forefront of lymphoma care.
Discover the latest in treatment options for relapsed/refractory follicular lymphoma from leading experts to help you or your loved one navigate this journey with confidence and hope. Dr. Joshua Brody from The Mount Sinai Hospital and Dr. Celeste Bello from the Moffitt Cancer Center discuss bispecific antibodies and CAR T-cell therapy, including their benefits and side effects.
Understand the role of watchful waiting and active surveillance. Gain valuable advice from lymphoma specialists on navigating treatment options and clinical trials. Discover the importance of support groups and resources for patients.
Stephanie Chuang: I’m the founder of The Patient Storyand a blood cancer survivor myself. I went through hundreds of hours of chemotherapy for my diffuse large B-cell lymphoma diagnosis. During that time, I realized how lonely it was and how hard it could be to get good information, and that was the genesis of The Patient Story.
Our goal is to help patients and care partners navigate life after a diagnosis and what it means in human terms. We do this primarily through in-depth patient stories and educational programs. This is especially important in spaces like follicular lymphoma. Patients and loved ones are being told by doctors that it’s highly treatable but not curable, so it’s great to know the latest therapy options and clinical research. We stand behind this message of self-advocacy, especially after diagnosis.
We want to thank Genmab for supporting our independent educational program. Their support helps us host more of these programs for free. The Patient Story retains full editorial control of all content.
We’re bringing this to you in collaboration with The Leukemia & Lymphoma Society, which has raised and invested over $1.5 billion in blood cancer research and provides free educational resources and support. Their information specialists provide one-on-one support on questions from treatment to social and financial challenges.
While we hope that you will walk away with more knowledge, we want to stress that this is not a substitute for your medical advice.
Our discussion focuses on the latest treatment options for relapsed/refractory follicular lymphoma. My co-moderator Dylan Lepak, another non-Hodgkin lymphoma survivor who became a patient advocate, will help lead the discussion.
Dylan Lepak, Patient Advocate
Dylan Lepak: I’m a diffuse large B-cell lymphoma survivor like Stephanie. I was diagnosed in 2021. I went to the hospital with stomach pain and came out with a horrible diagnosis, but luckily, I had a very standard treatment approach. I went through six cycles of R-CHOP and since then, I’ve been good. Since that time, I’ve done a ton of different charity work and moderated online discussion boards.
Dr. Bello, when you first see a patient with lymphoma, what message do you want them to take away after that first meeting?
Dr. Celeste Bello: I try to convey to them that they can relax and take a deep breath. In most cases, we can manage this quite well. Most of the meeting is trying to get everybody’s anxiety down a bit.
Dr. Josh Brody, Hematologist-Oncologist
Stephanie: Another amazing doctor and leader in this space is Dr. Josh Brody, a hematologist-oncologist, director of the Lymphoma Immunotherapy Program at Tisch Cancer Institute at Mount Sinai in New York, and faculty member of the Icahn Genomics Institute. Since 2011, he has developed The Brody Lab, a translational cancer immunotherapy lab that investigates the development of novel therapies, particularly for lymphomas, and specializes in how to use immunotherapy against treatment-resistant lymphoma.
Dr. Brody, I know that you’re very invested in patients beyond the clinic. When you see patients and family members in your office, what do you hope they walk away with from that first meeting?
Dr. Josh Brody: Aggressive lymphomas are a sprint and we need to get things going right away, but follicular lymphoma is a marathon. If you see someone starting a marathon by sprinting, they’re not doing it correctly. We don’t want to overwhelm people with information the first time we meet them. We give them the big picture that hopefully this is going to go very well.
There are a lot of great medicines for front-line therapy and second-line therapy. Some patients might need Plan C, Plan D, and Plan E, so how you develop Plan A and Plan B might impact those. Even though there’s no emergency for the vast majority of follicular lymphoma patients, Plan A and Plan B might affect Plan C, so you want to plan out the marathon thoughtfully and as best you can.
We have to be flexible because we’re going to have to change the plan depending on how things go. There are unique options available at some places that may not be available to others. We might want to do the smartest plan A so that we don’t burn bridges down the road.
What is Follicular Lymphoma?
Stephanie: Dr. Bello, could you summarize the nature of follicular lymphoma?
Dr. Bello: Follicular lymphoma is an interesting disease that’s broken down into different subtypes. We have low-grade indolent ones, which are slow-growing, and one subtype that’s a little more aggressive than the others. Most of the time, when we’re talking about follicular lymphoma, we’re talking about the low-grade, slow-growing one.
A lot of times, they’re picked up by accident on another diagnostic test, like a mammogram, which is a common one I see. Somebody had a screening mammogram and there’s no evidence of breast cancer, but there’s an enlarged lymph node in the underarm or axillary area.
Some can behave aggressively, so we have to keep an eye on these people and treat them differently, but as a whole, it’s a long-term disease that’s managed like a chronic illness. We try to keep it under control and if there’s a flare, we can manage that in most cases. It’s a cancer of lymphocytes, but one that sometimes doesn’t even need treatment.
Most Common Symptoms of Follicular Lymphoma
Dylan: Dr. Brody,if patients do have symptoms, what symptoms do you usually see with follicular lymphoma?
Dr. Brody: My grandma died at the age of 101. She didn’t have follicular lymphoma, but she had the next cousin over that we treated in a very similar way as other low-grade B-cell non-Hodgkin’s lymphomas. We did watchful waiting for 37 years and never needed any therapy.
Most people are asymptomatic, but some people can have symptoms. The most common symptom of follicular lymphoma is a painless lymph node. The B symptoms, which are fevers, night sweats, and weight loss of more than 10% of your body weight, are pretty rare for follicular lymphoma and happen in less than 10% of patients.
Terminology: Watchful Waiting vs. Active Surveillance
Stephanie: Some people don’t like “watch and wait” as a term. It can be tough for so many people to be told they’ve got cancer but they don’t need to be treated right away. How do you describe that period to your patients? What term do you use? How often are people told that they’re on watchful waiting or active surveillance? What is the plan that accompanies that?
Dr. Brody: I grew up saying watchful waiting. Dr. Mike Schuster, a lymphoma doctor at Stony Brook, told me he patented the phrase “active surveillance.” I’m not sure if he patented the phrase, but it sounds a lot better. We’re going to actively surveil you as opposed to watchful waiting, which sounds like we’re going to sit around and do nothing. It’s all about branding.
The reality is we’re going to keep a close eye on you and how close depends on what’s been going on with them, how well you’ve known the patient, and for how long. This concept is super freaky to patients and their families when they first hear it. Some patients don’t get surveilled for that long, but every patient who is still on active surveillance a year later loves the concept. But on day zero, “Wait, what? Why aren’t you treating me? Cancer and no treatment sound super freaky.”
I come in with my grandma’s story, so they can hear that sometimes this can go for decades without needing therapy. The only test that tells you if you’re going to go down the same course is the test of time. There’s no test as good as that, but we’re going to watch you closely as we give you that test.
Dr. Bello: I usually use observation or keep an eye. Some people say that’s what they wanted to hear. Others say they’re getting a second and third opinion. They can’t take it and I feel for them. You have people whose lymphoma was picked up incidentally and you tell them, “We’re just going to watch you.”
Since these are picked up by accident, I always wonder if it would’ve been better if we picked this up 20 years later. Are we doing a disservice that we diagnosed this little lymph node under their arm? Should we have left it alone to begin with? They were living life peacefully and blissfully, and now they’re going to have 20 years of anxiety instead. The anxiety can sometimes be the hardest part of the diagnosis.
Standard Treatment for the First Two Lines of Therapy
Dylan: We’re going to dive into the treatment options, but before we go into the novel treatments, can you give us a breakdown of the standard treatment for the first two lines?
Dr. Bello: The standard is there’s no standard. That’s always the first problem, but it’s a good problem because we have a lot of different options. You like to base it on the patient you’re treating and your goal with treatment.
Some people may have one large lymph node that’s causing them discomfort, so radiation to that spot may be the best option. Some people may have generalized symptoms, like severe fatigue that’s interfering with some of their daily activities, but don’t have a lot of disease. In that case, you may want to give them a less intensive treatment, like a monoclonal antibody like rituximab, put the disease into remission, and see if it corrects their symptoms.
Other people are very symptomatic. They come to you with rapidly growing lymph nodes and have fluid build-up in certain body cavities like in the abdomen. You want to treat them with something that’s going to work quite quickly and that’s usually a chemotherapy plus immunotherapy regimen.
There are a few options available. One of the more popular ones is bendamustine plus rituximab or bendamustine plus obinutuzumab, which is another monoclonal antibody. There are also milder or non-chemo approaches, like oral therapy with lenalidomide and rituximab. The treatment has to be geared toward the patient you’re treating, fitness level, and treatment goal.
What are Bispecific Antibodies?
Dylan: Dr. Brody, what are bispecific antibodies? How are they different than CAR T-cell therapy? What’s their mechanism of action?
Dr. Brody: Dr. Bello mentioned that one of our go-to therapies over the last 24 years is monoclonal antibodies, anti-CD20 antibodies, especially rituximab and obinutuzumab. Rituximab is certainly the most famous. It’s an anti-lymphoma antibody. CD20 is one protein marker on the surface of lymphoma cells. CD3 is a marker on T cells. Bispecific antibodies are two of those antibodies.
In Lady and the Tramp, when the puppies were sucking from the dish at the same time and ended up accidentally sucking on the same noodle, it brought the two of them together. Now instead of that, it’s the kiss of death where the lymphoma cell and the immune cell are brought together by the bispecific antibody and it activates the immune cell at the same moment so the T cell kills the lymphoma cell. I think it was Dr. Matthew Lunning at Nebraska who made up the Lady and the Tramp reference.
T cells are incredibly good at killing lymphoma cells, better than probably any other cancer. It’s the same idea as CAR T-cell therapy. The main difference is that we have to take the T cells out of the person, ship them somewhere, get the CAR T cell made, and have it shipped back. A bispecific antibody is in some ways an off-the-shelf version of that, so you can use it today.
They’ve been incredibly effective at melting away lymphoma cells, even when other therapies like chemotherapy, lenalidomide, and a few others have not been working, especially when rituximab has not been working. Still, these bispecific antibodies have been awesomely effective in inducing remissions in the great majority of follicular lymphoma patients. It’s been an absolute revolution. That is not an overstatement because we didn’t have anything nearly as effective and safe as this until bispecific antibodies came along.
Approved Bispecific Antibodies for Follicular Lymphoma
Stephanie: We have two FDA-approved options in follicular lymphoma so far. Dr. Bello, for what kinds of patients would you recommend bispecific antibodies? Is there anything practice-shifting for you?
Dr. Bello: For follicular lymphoma, we have mosunetuzumab and epcoritamab. Thankfully, the majority of these lymphomas are pretty indolent and respond very well to front-line treatment, whatever you use. You can use chemoimmunotherapy, like bendamustine and rituximab or obinutuzumab, both of which are monoclonal antibodies. We have the luxury of having a lot of patients who don’t end up needing to get a bispecific antibody.
But for patients who progress, we have these immunotherapies: bispecific antibodies and CAR T-cell therapy. I use bispecific antibodies in people who have progressed after two lines or more of treatment. I always try the other lines first, unless we have a clinical trial because they’re proven and have a great track record. I don’t skip straight to a bispecific.
Bispecific antibodies have some side effects that are very unique to them and may not be the best for everybody. They also require a little bit more skill in monitoring them. They are administered in the hospital at the beginning and in the office for the subsequent doses.
Stephanie: Dr. Brody, what about you? How do you approach bispecific antibodies and how do you decide to use one over the other?
Dr. Brody: We’re very lucky to have two FDA-approved options. Their roles are evolving very rapidly and will be evolving very rapidly over the next few years. I would expect that five years from now, bispecific antibodies will be an option for front-line therapy. They may be given as a monotherapy, but honestly, I don’t think that’s their future. I think the future is like rituximab, which is to become part of combination therapy.
Dr. Bello was doing some comparison between CAR T-cell therapy and bispecific antibodies, and I agree very much with what she was saying. But in the future, it may be an irrelevant comparison because it won’t be what’s better between CAR T-cell therapy or bispecific antibodies. It’ll be an apple and orange comparison. Who cares what’s better?
In the future, we probably won’t be giving bispecific antibodies by themselves. Today, they’re only FDA-approved to be given by themselves, so the only way to get them as part of a combination is through a clinical trial. The preliminary data from some trials of bispecific antibodies plus standard therapies have been awesome with the huge majority of people getting complete remissions in some cases, even when prior chemotherapy and such weren’t working.
Medically, mosunetuzumab and epcoritamab are pretty similar. They have pretty similar efficacy and pretty similar safety profiles. Epcoritamab is given subcutaneously and mosunetuzumab is intravenously. For many patients, it doesn’t make a difference. For some patients, especially those who have had multiple prior lines of therapy and whose veins have gotten beat up, it does make a difference to them. Some patients have a port that they don’t want to have anymore or they don’t want to get a port.
Another difference is that mosunetuzumab is a finite course of therapy, about nine months, while epcoritamab can be given until it stops working or until you have a bad side effect. We have a lot of patients who have been on it for a year or two and say they want to pause for now, and we’re okay with that. It’s not exactly the recipe as written, but we don’t feel like we’re burning any bridges because if the lymphoma were to grow back, we might be able to treat them again. Some patients prefer to keep going because they don’t like the notion of doing nothing.
Because it’s more about the total doses of epcoritamab, for me, it’s simple. If I have a patient who lives across the street and they don’t want any IV therapy, that patient gets epcoritamab. If the patient lives four hours away and feels strongly about not making too many trips and doesn’t mind getting IV therapy, that patient gets mosunetuzumab. Patients have a big role in that decision.
Dr. Bello: I still prefer CAR T-cell therapy over bispecific antibodies because I see more durable responses, but not everybody can wait to do the whole process. As Dr. Brody mentioned, you have to collect the T cells, send them to be manufactured, and wait for them to be sent back. By then, four to six weeks have gone by and if they’re having rapid progression, they can’t wait.
CAR T-cell therapy can have a little more toxicities with them. Patients are hospitalized a bit longer, so not everybody has a performance status that’s robust enough. That’s when I would use the bispecific antibodies.
Common Side Effects of Bispecific Antibodies
Dylan: Dr. Bello, you touched on the side effect profile of bispecific antibodies. If I was a caregiver or a patient, what should I be on the lookout for and what would my experience be like?
Dr. Bello: A lot of people are now familiar with cytokine release syndrome (CRS). It mimics the most severe infection you’ve ever had with symptoms including fevers and chills. Sometimes, it can be more severe. Patients can have a drop in blood pressure. There can be fluids leaking into the tissue and lungs.
Bispecific antibodies don’t cause much of CRS in most people, so it’s nice that we err on the side of caution. We do it in step-up dosing where we start with a small dose, have the patient come back a week later and give them an increase, then a week later, another increase. Sometimes we hospitalize them to monitor them for 24 hours, but the majority of the time, they do very well.
Neurologic toxicity or immune effector cell-associated neurotoxicity syndrome (ICANS) is more serious. It can present as confusion, word-finding difficulties, or as severe as being obtunded or in a coma. Again, we don’t see much of it with bispecific antibodies so fortunately, it’s not something we need to look for. We let patients know about the possibility, but we don’t usually see that.
Dr. Brody: Dr. Bello was very thoughtful in reminding people that this can be severe, so the patient and, even more importantly, the caregiver needs to know it can be severe. But then we also like to tell people about the reality and the statistics. If they read too much on Google, they’ll unfortunately also read about aggressive lymphoma bispecific antibodies and mantle cell lymphoma bispecific antibodies where the numbers are a little bit worse. But with the follicular lymphoma bispecific antibodies, almost all of these CRS incidences are low-grade.
Similarly for ICANS, in the biggest studies for follicular lymphoma patients, it was the high-grade version of that where people were confused. There was 0% in all of the recent studies, so we do have to tell people about that. At the same time, we want to tell them not just what might happen but what likely will happen. What likely will happen is about half of patients get a fever and they get it either on the first dose or the first few doses.
If a person is getting many more doses in the future, they rarely happen. Most side effects of cancer therapies get worse as you go, like with chemotherapy. Bispecific antibodies are the opposite. It’s the first couple of weeks that have the risk of fevers and then that risk goes down to about zero. I don’t want to say there are no risks because there still are some risks. The risk of infection from common viruses like the flu, RSV, and COVID are real, but they’re true for almost every therapy we give and even for some lymphoma patients who are not even on therapy. Those are the common risks that most of us are facing.
The main thing is cytokine release syndrome and neurological side effects mostly happen in the first few weeks or never happen at all. In that way, it’s a bit of a comfort to a patient because once they get through the first few weeks, I don’t want to say they’re in the clear, but they’re definitely in the easy running part.
Access to Treatment
Stephanie: When CAR T-cell therapy first came out, there were some issues with access in that it has to be done at very specific places. What are your thoughts about bispecific antibodies and access to them?
Dr. Brody: That’s supposed to be the main transformation and revolution about bispecific antibodies. They’re supposed to be accessible and they are or definitely will be. Let me make a little distinction.
For aggressive lymphoma bispecific antibodies, there’s one little speed bump: the ones that are approved now require a one-day hospitalization. One day in the hospital isn’t that bad, but that becomes a real pain for the doctors who have to coordinate that. Even though it’s not a big deal medically , it’s a logistical pain in the butt. Still a lot easier than CAR T-cell therapy, where the patient can spend weeks in the hospital sometimes, but it’s still not nothing.
By contrast, no hospitalization is required for the follicular lymphoma bispecific antibodies (epcoritamab and mosunetuzumab), based on the FDA label and the way that we do it. We occasionally will have an elderly patient we are worried about, so we’ll admit them to be safe, but for most patients, no hospitalization is required. That makes the accessibility hugely more doable than CAR T-cell therapy or even bispecific antibodies for aggressive lymphoma.
The main obstacle to accessibility is community oncologists getting comfortable with these. They’re recently FDA-approved, so they haven’t used them a lot before. Doctors like Dr. Bello and I have been using these for years as parts of clinical trials, so we’re pretty comfortable with them. Like Dylan said, rituximab sounded scary when it was first released and people having infusion reactions was a big deal. Now, no one’s surprised by infusion reactions and everyone’s comfortable with it.
I think it’ll be similar with bispecific antibodies. The hurdle to accessibility is community oncologists getting comfortable with them. There are different ways to facilitate that. One version is the academic doc treats the patient for the first few weeks until they get over that little hump of the risk and then we send them back to their community oncologist. They don’t have to drive back and forth so much. In a couple of years, community oncologists will be very comfortable with these, so it will be less of an obstacle.
Dr. Bello: I agree. Like all drugs, once they’re out for a few years and people have become more comfortable with them, they’ll become more readily available at multiple locations.
Differences Between Bispecific Antibodies and CAR T-cell Therapy
Dylan: CAR T-cell therapy is still a major player. We have three FDA-approved options—liso-cel, axi-cel, and tisa-cel—and tons in the pipeline targeting anything you can find. We talked about bispecific antibodies. What’s the difference between them and CAR T-cell therapy, and the mechanism of action for CAR T-cell therapy?
Dr. Bello: As Dr. Brody mentioned earlier, they’re similar, but the mechanism of action is obtained differently. CAR T-cell therapy is a much more complicated process. Patients come in, they donate blood, and a machine filters out their lymphocytes and gives them their whole blood back. Their lymphocytes are sent to a facility that will engineer the T cells to recognize a marker on the lymphoma cells. In the three products that you mentioned, the marker is an antigen called CD19, which is pretty much common in almost all B-cell lymphomas.
In addition to the T cell being able to recognize that antigen, the T cells are also programmed to be turned on. By nature, when T cells are in your body, they’re not attacking your body randomly. They need a trigger to turn them on. These T cells are engineered to recognize a certain antigen in the lymphoma cells and they’re activated already.
When they’re ready, they ship them back. Usually, it takes a couple of weeks for them to do this. The patient comes in and we give them a moderate dose of chemotherapy over a couple of days to suppress their immune system and then we give their T cells through a transfusion.
Once they get into the body, they immediately start to attack the lymphoma. A lot of times, we hospitalize people for this portion because now is when they start getting cytokine release syndrome or neurological toxicities. We monitor them in the hospital because there are tests we can do to see if they’re going to start getting more severe symptoms. We also have some medicines we can use to attenuate some of these if they start happening.
Patients are in the hospital anywhere from seven days to three weeks, depending on how long the inflammatory process lasts. It can start as soon as the cells are put in or it can start a couple of days later, so we don’t let them go home within 24 or 48 hours. There’s a peak in the symptoms and then you’ll start to see the inflammation lessen, the patients start to get back to their baseline or their normal status, and then we eventually let them leave the hospital.
Obviously, their work is not done because, at that point, their blood counts will be low. Even though it’s an immune therapy and we think that it’s attacking the lymphoma cells, it does cause quite a bit of a drop in the white cells, red cells, and platelets. Some of these patients require transfusion support during the first few weeks after and antibiotics because we see infections happen because of the low white cell count.
What I always found weird with CAR T-cell therapy is sometimes people get very sick for a few days and then it’s like a light switch is turned off. All of a sudden, the inflammation stops. Once the inflammation lessens, the patients start to get back to normal. I always tell them they’re going to have a week of hell and then they’re going to feel all right afterwards.
Dylan: I’m glad you said that because these neurological symptoms can be terrifying. For the patient, it’s going to be a blur most of the time, but for the caregivers, it’s the most terrifying thing in the world.
Dr. Bello: It’s good to let them know because there’s some misconception that CAR T-cell therapy and immunotherapies are benign. They come into the office saying, “I don’t want chemotherapy. I want CAR T-cell therapy.” No, we’re not going straight to that. There’s a misconception that because it’s not chemo, it must be easy. It’s an intense treatment. It’s not going to be a walk in the park.
The Role of Inhibitors
Dylan: Probably the most confusing for patients are the inhibitors. There’s BCL2, BTK, and SYK, and some of these are FDA-approved. Where do those fit into this new paradigm with CAR T-cell therapy and bispecific antibodies? Dr. Bello, how do you use them in your practice?
Dr. Bello: Unfortunately, the data with BTK inhibitors in follicular lymphoma has not been the greatest. BTK inhibitor stands for Bruton’s tyrosine kinase inhibitor. We use them a lot for other types of indolent lymphomas, like small lymphocytic lymphoma (SLL), chronic lymphocytic leukemia (CLL), and Waldenstrom’s, but with follicular, it hasn’t worked that well. There are some studies in development looking at BTK inhibitors in combination.
The other one is the BCL2 inhibitor venetoclax. That one is still a shocker to me. BCL2 is what’s mutated in 99% of follicular lymphoma and this drug that blocks the BCL2 pathway doesn’t work in follicular lymphoma.
The one that had the best results, PI3K inhibitors, was pulled from the market. Thankfully, we have these other options that I don’t miss them too much.
Cure vs. Longer Periods of Survival
Stephanie: Dr. Brody, there’s so much development happening in the immunotherapy space, like CAR T-cell therapy and bispecific antibodies. I know doctors don’t like using the word “cure,” but is there a discussion about that? And if not cured, what’s the consensus on the impact on longer periods of survival?
Dr. Brody: Doctors, especially academic doctors, are extremely diplomatic. We say to patients all the time that follicular lymphoma is incurable. Nothing’s incurable. We just have not found the cure yet. It’s not the disease’s fault. It’s us. There’s nothing magical about the disease. We haven’t been smart enough over the past 40 years, but we’re getting close. When my grandpa’s sister had Hodgkin’s lymphoma in 1949, it was 100% incurable. Now it’s 85% curable and that wasn’t that long ago.
We refer to follicular lymphoma as incurable. That’s how we write it in all of the papers. That sounds scary. It’s a terrible word. Whenever I say that, I have to say it’s incurable, like high blood pressure or diabetes. We don’t have a cure for high blood pressure, but we’ve got pretty good pills that can keep your blood pressure good for the next 50 years. We can’t give you one pill to cure it and that’s what we say for follicular lymphoma so far.
Bispecific antibodies have been the highest bang for the buck in follicular lymphoma in terms of efficacy versus toxicity. By themselves, they have some limitations. They’re good but not perfect. But in combination with standard therapies, like rituximab and lenalidomide, the complete remission rates have been huge in the first early studies. The big studies are getting done now.
Quite honestly, I wouldn’t be surprised if we look back in 20 years and find out that those who got bispecific antibodies plus combination standard therapies never relapsed. It will not hold for 100% of the patients, but it will be for a fraction. I don’t know that that will happen, but I absolutely would not be surprised because so many people are getting such deep remissions. We may be handing out curative therapies to people on those studies today. What’s the likelihood? Ask me again in 20 years, but it’s a real likelihood.
Clinical Trials in Follicular Lymphoma
Stephanie: When it comes to clinical trials, are there ones that are particularly exciting in the follicular lymphoma space?
Dr. Brody: Clinical trials are not always great. However, clinical trials are a time machine into the future. We have been giving people some of these bispecific antibodies in combination for about four or five years now, and some of them are just getting FDA-approved now. Some of these combination therapies are not FDA-approved yet, but they will be FDA-approved in the next year or in some cases, the next five years.
It’s a bit of a tragedy that Americans don’t get access to these mostly for lack of awareness. They’re available at many dozens or sometimes hundreds of hospitals around America, but maybe they didn’t think to ask or the person they asked wasn’t aware of them. That’s where resources like The Patient Story, The Leukemia & Lymphoma Society, and the Lymphoma Research Foundation can be helpful in guiding patients. There’s no absolute commitment to be part of a trial, but hear about it and get the information.
We were talking about front-line therapies for follicular lymphoma before and then hinting at later lines. The NCCN Guidelines are clear. They recommend finding out about clinical trials. Old therapies are considered non-curative, but it doesn’t mean we can’t get a curative therapy with some of these promising trials.
The first thing that patients need at the beginning of their journey is the awareness that there may be some self-advocacy involved. No doctor in America should ever be annoyed if a patient goes and gets a second opinion. My patients get second and third opinions beyond me and I always ask because I’m always interested to hear what they say.
People think of trials as a last-ditch effort and it’s such a bad branding. It shouldn’t be.We have trials for front-line therapy and second-line therapy. The trials for first-line therapy are what we’re super excited about. One example is getting immunotherapy instead of chemotherapy. We have trials of front-line therapy of bispecific antibodies where half the patients get that and half the patients get standard therapy.
Patients have concerns that they’re guinea pigs and don’t know what they’re getting or they’re getting a placebo. There’s no such thing as a placebo in cancer clinical trials. They may get the standard treatment plus a placebo. In most trials, people are aware of what they’re getting. There’s no mystery.
Some of the most exciting trials give patients the chance to skip chemotherapy, to get front-line bispecific antibodies, and combinations of the standard with or without the bispecific on top of it. Because bispecific antibodies have been pretty gentle and mostly safe, we can safely combine them with standard therapies.
The results from some of the early versions of those trials that have been presented at ASCO and ASH showed unparalleled remission rates. They’ve been awesome. Some of those front-line and even second-line trials of similar combinations are, for me, some of the most exciting trials out there right now.
Dr. Bello: I agree. For bispecific antibodies or immunotherapy treatments, the trials that are moving them up now to the second line and even to the front line are the most interesting. If you could get a non-chemotherapy approach, that would be great.
Again, we always have to be careful because when we find out years later that this drug caused this kind of malignancy or this kind of immunologic problem, maybe we should have stuck with the original treatment that we had. Immunotherapies are the wave of the future and the sooner we can move them up, the better, and those are the trials that I’m looking for the results in.
Dr. Brody: In many ways, it’s a rehashing of 20 years ago. Rituximab was approved as a monotherapy and now, we say that rituximab is like chocolate chips: add it on top of everything. There’s not a single B-cell lymphoma where you don’t add rituximab on top of whatever the standard was before and made it better.
CHOP was the standard of care then, but no one talks about CHOP anymore. R-CHOP is the only thing now except for some newer versions of R-CHOP as well. I do think that that will be the standard five years from now. Wait five years for the better stuff or get access to it now through clinical trials.
Recommended Online Resources
Stephanie: A follicular lymphoma patient, Donna P., said, “One of my first questions was how long will I live. Until I found a support group, I was so depressed and fanatical about my disease. Then I started seeing all the comments and how longevity was in my diagnosis, so many others then gave me hope.”
In the initial conversations with the patients when they’re dealing with so much, do you have any guidance for them, whether it’s support groups or resources?
Dr. Brody: My first recommendation always is The Patient Story and as Dr. Bello said, The Leukemia & Lymphoma Society (LLS) or Lymphoma Research Foundation (LRF). There are a few others as well, but those are the best for lymphoma patients.
We also sometimes have in-person groups. The stress of this can be a significant factor, especially for someone who already had stress as a problem before or anxiety as a diagnosis before, so we try to get them personalized counseling. At cancer centers, we’re very lucky to have counselors who have experience with cancer patients specifically or, in our case, with lymphoma specifically.
The LLS Clinical Trial Support Center
Stephanie: The Leukemia & Lymphoma Society has a Clinical Trial Support Center that offers free, one-on-one support. You get connected to an LLS Clinical Trial Nurse Navigator to help you throughout the clinical trial process from finding the right trial all the way through when you’re on a trial, making sure that you have all your questions answered.
Clinical trials aren’t a last resort. The terminology itself can be quite daunting. Patients need to understand more about what clinical trials are so that they can decide for themselves with their healthcare team. ClinicalTrials.gov isn’t necessarily the easiest site to navigate, but the Clinical Trial Support Center can help you navigate a resource like that.
The Right Time to Seek Lymphoma Specialists
Dylan: People often ask, “When’s the right time to get a second opinion?” If you or your loved one has follicular lymphoma, when is the time to get an opinion from a lymphoma specialist? Can you explain the difference between a lymphoma specialist and a hematologist-oncologist?
Dr. Brody: Let me first say that being a community oncologist is tough. Most of these folks are taking care of lung cancer, breast cancer, kidney cancer, bladder cancer, etc. It goes on literally all day. I’m buddies with dozens of them and I hear about what they do and how they stay on top of everything. It’s a very tough job.
Academic oncologists and community oncologists have a pretty good way of working together, which hopefully means they have my cell phone, and for any little thing, they’ll give me a buzz or give me a text. For most of those, the patient doesn’t need to come see us.
Compared to breast cancer, follicular lymphoma is rare. Your regular community oncologist knows about follicular lymphoma and has had a few patients with it. Every month, they see dozens and dozens of breast cancer and lung cancer patients, but probably zero follicular lymphoma. They’ll see maybe a few per year, so it would be impossible for them to stay on top of every little bit of data. There’s no way they could do that for every single type of cancer. Dr. Bello and I can barely do it for all the types of lymphoma, so you can imagine that plus other cancers.
The answer for when to see a specialist is always. However, most patients don’t need to keep seeing a specialist. They can have a back-and-forth where we share the patients. The most common intervention I make when there’s a difference in opinion between me and the community oncologist is to downgrade the therapy. They’ll come in and say, “My community oncologist said I need R-CHOP,” and I’ll say, “You don’t. You definitely do not.” They can get either nothing and undergo active surveillance or get something gentle. We make them aware of clinical trials where they may get access to something that won’t be available for years sometimes.
I don’t fault them for saying that. Even a hematology-focused oncologist sees acute myeloid leukemia (AML), myelodysplastic syndrome (MDS), myelofibrosis, and myeloma most of the day, and then a little bit of follicular lymphoma. As a lymphoma specialist, all we see is lymphoma, so we have to have a little more experience.
Final Takeaways
Stephanie: We’ve had an incredible discussion. Hopefully, many things have resonated, but if there’s one thing that you hope people would walk with, what would you like that to be?
Dr. Bello: One of the most important things is that it’s okay to not get treatment. There are a lot of times when people don’t need to have their lymphoma treated because a lot of low-grade follicular lymphomas can be observed. If that’s your doctor’s recommendation, come to terms with that and know that you’re being monitored. If something changes or happens, we have great options.
Lymphomas are very different than solid tumor cancers in that they all need to be treated and the sooner the better. You have to change your whole mindset with these kinds of conditions.
Dr. Brody: Dr. Bello said it beautifully and I agree with how she said it. If you get diagnosed with follicular lymphoma, thank God you got follicular lymphoma and not some horrible lymphoma because it could have been something else. Thank God you got lymphoma in the 2020s and not in the 1990s because the advances have been unparalleled. There’s no cancer where the advances have been as incredible as in lymphomas and B-cell lymphomas, like follicular lymphoma especially. I guarantee that the 2030s medicines will be better than the 2020s medicines, so some patients will be savvy enough to get access to those even now.
Conclusion
Stephanie: Thank you, Dr. Brody, Dr. Bello, and Dylan for this discussion. I really appreciate your time.
Thank you to Genmab, our sponsor for this program. Their support allows us to do more of these programs and keep them free for our audience. I hope that you enjoyed the discussion and we hope to see you in a future program. Take good care.
Interviewed by: Taylor Scheib Edited by: Katrina Villareal
Ashley’s symptoms began with mild back pain in October 2022, which escalated to severe discomfort by January 2023. After a series of tests, a CT scan revealed an 11 cm tumor on her adrenal gland and she was eventually diagnosed with stage 4 adrenocortical carcinoma (ACC), a rare cancer affecting about 300 to 400 people annually in the U.S. Despite facing numerous challenges, including a delayed diagnosis due to lack of insurance, she educated herself on her condition, sought out specialists, and joined support groups.
Her treatment journey began with EDP (etoposide, doxorubicin, and cisplatin) chemotherapy and mitotane, which caused severe side effects, including hypothyroidism. Despite the challenges, the tumor showed signs of shrinkage and she underwent surgery to remove it. When chemotherapy became ineffective, she switched to targeted therapy with lenvatinib, which showed promising results.
Throughout her journey, Ashley also struggled with mental health issues and adjusting to the need for help, but she found support through therapy and counseling. Ashley emphasizes the importance of self-advocacy in medical treatment, seeking second opinions, and the value of support groups in navigating her rare diagnosis.
Name: Ashley P.
Diagnosis:
Adrenocortical carcinoma (adrenal cancer)
Staging:
4
Symptom:
Mild back pain on her left side that escalated in severity
Treatment:
Chemotherapy: EDP (etoposide, doxorubicin, and cisplatin) & mitotane
Surgery
TKI inhibitor: lenvatinib
This interview has been edited for clarity. This is not medical advice. Please consult with your healthcare provider for treatment decisions.
I was having some mild back pain on the left side, which was eventually where the tumor was discovered.
Introduction
I live in Beaumont, Texas, about an hour and a half away from MD Anderson.
In October 2022, I first started showing symptoms of my cancer, but I wasn’t diagnosed until March 2023 and I’ve been living with stage 4 cancer since then. It’s extremely rare. It affects 300 to 400 people in the US every year, which is about one in a million people.
Pre-diagnosis
Initial Symptoms
When I first started having symptoms, they were very mild and pain-related. I was having some mild back pain on the left side, which was eventually where the tumor was discovered. I would rate the pain at a 3 out of 10. It would always happen after long car rides, so I chalked it up to bad posture.
The pain was on and off throughout the next several months until about January. Then I had severe back pain that manifested at a friend’s house. I was sitting on the floor and it overtook my whole body. It was probably a 7 or 8 out of 10. Within a couple of days, it eventually settled down and then went away. I still thought it was my posture.
Come February, I started to feel a pinching pain around my rib cage. I had never had that. It was persistent throughout the day in a specific spot and that’s what got me worried. I couldn’t figure out what it could be, so it scared me a little bit. Some family members told me it could be this or that, it might be something serious, and that I should get it checked out.
I went to our local hospital where they ran a bunch of tests, including blood work and X-ray. I came in with high blood pressure of around 190/105, if I remember correctly. I wasn’t aware that I had high blood pressure, but I think it has been ongoing and I hadn’t been checking it. I was also anxious, so I’m sure that was playing a part.
CT Scan Revealed Tumor
They saw something in my X-ray, which they said could be an infection in one of my lungs but I had no respiratory symptoms. They gave me a COVID test and it came back negative. Everything they checked came back negative, so they sent me in for a CT scan because they couldn’t figure out what was going on. It was a good thing they did that because that’s when they discovered the tumor coming off of my adrenal gland, which I believe was around 11 cm at the time.
She gave me the news of this enormous tumor coming off of my adrenal gland and the very last thing she said was, ‘It has metastasized to your lung.’
Getting the Scan Results
The doctor comes in with a look on her face and I knew she was about to give me bad news. My stomach dropped when I saw the way she looked at me. The whole mood in the room changed. My fiance came with me to the hospital, but because it had gotten so late and the chairs were so uncomfortable, he was sleeping in the car in the parking lot when she told me.
She gave me the news of this enormous tumor coming off of my adrenal gland and the very last thing she said was, “It has metastasized to your lung.” I could tell she didn’t want to say that part because that meant stage 4. At that point, I didn’t quite understand anything about what she was saying. She said it was 95% sure I had cancer because they can’t tell you 100% until they biopsy.
She ended with, “We don’t think we can handle this here, so we’re going to have to transfer you to an ICU.” They treated my blood pressure because that was all they could do for me.
I called my fiance, told him where he needed to go, and gave him all the information I could. We didn’t know much, so I was relaying what little information I had. He was sitting there crying and I was comforting him more than I was comforting myself. I was completely disassociated. I went into shock a little bit. It was a surreal experience.
Transferring to Another Hospital
I had to be transported to another hospital’s ICU about an hour and a half away, but I had to wait until the next morning for an ambulance to transfer me.
The oncologist came in and said, “I don’t think it’s ethical to treat you without insurance.” They sent in a social worker who handed me his phone to talk to an insurance agent. They sent me home after three days. I had to wait a full month for the insurance to kick in.
When I went home, I hadn’t had a biopsy yet, so I didn’t even know my diagnosis. I couldn’t look anything up because I didn’t know what it was. All I knew was there was a tumor and something in my lungs.
I had something serious going on and all I was doing was waiting. I had no idea how fast things needed to be moving.
Waiting for Insurance
I didn’t have any coping skills because medical issues weren’t something I had ever dealt with, so it all hit me like a train. I went into a state of almost paralysis. All of a sudden, it was almost as if I could feel the tumor and it made me afraid to move and get out of my bed. I thought, Am I going to make it worse? Is it going to rupture? These types of tumors are very, very fragile. They’re like an egg. At the time, I didn’t know, but that was still a thought because I knew how big it was.
I was at my heaviest, but I started losing weight because I wasn’t eating well. I was so paralyzed by fear, worry, and not knowing anything. While at the hospital, I even asked the nurse if I was going to die soon and she changed her demeanor a little bit. She said, “Oh no, no, but this is very, very serious,” and that stuck with me.
I had something serious going on and all I was doing was waiting. I had no idea how fast things needed to be moving. Each day, I would try to get through the day and after another day’s done, move on to the next day. I was looking at my calendar every day, compulsively checking the days off until I could get my insurance.
Diagnosis
Getting the Biopsy Results
Once I got the biopsy results and knew what I had for sure, I started digging. I put all of my anxious energy into finding out more about my type of cancer. I joined adrenal cancer Facebook support groups and started talking to people and watching YouTube videos. There are a few conferences that have a few of the experts that exist in the United States. There’s only a handful of them and they put together this conference with all of this educational information.
One time, I stayed up all night because I was so anxious and freaked out because I didn’t know what my future was going to look like. I wanted to know what my options were. I couldn’t get my mind to separate from what I was going through, so that was a difficult time for me. That beginning stage was probably one of the most difficult experiences I’ve dealt with so far, even after everything I’ve been through.
What helped was educating myself and talking to people with the same diagnosis. There aren’t a lot of us because it’s so rare, but there are support groups. I don’t know anyone in my city who has my diagnosis. I’ve been to urgent care centers and some of them have never even heard of what I have.
At a certain point, I couldn’t work anymore. The symptoms had worsened to where I would get very high blood pressure and resting heart rate, so I didn’t feel like it was safe for me to work.
Finding a Specialist
When I joined support groups on Facebook, they were recommending certain doctors who were doing research on my type of cancer, but they’re located in Maryland at the National Institutes of Health. A girl in the support group called me and gave me all this information. She even put me on a three-way call with someone from the NIH about getting set up. Eventually, someone gave me the direct phone number and email address of the specialist, Dr. Jaydira Del Rivero.
I wanted to get recommendations and second opinions, and see what a true expert would say. The support group led me in that direction and I kept seeing her name over and over again in these groups. I emailed my scans to Dr. Del Rivero and they gave their recommendations.
What helped was this was all government-funded, so it’s all covered. For me, it was perfect because, at a certain point, I couldn’t work anymore. The symptoms had worsened to where I would get very high blood pressure and resting heart rate, so I didn’t feel like it was safe for me to work. I was afraid to even get up and use the restroom because my heart would start pounding like crazy.
Treatment
Discussing the Treatment Plan
I had a very skilled expert team collaborating with a local hospital that I knew would work with them. We put together a game plan for the first steps.
At my oncology appointment, we had a full discussion of where I stood and what things were looking like. I knew a lot about it because I read and listened to experts talk about it, so it didn’t come as a shock to me.
I had to get set up with an endocrinologist as well. The day after my appointment, I had a port put in and then the next day, I had chemo. That’s how quickly things started moving.
When I got to the hospital, they told me I was going to be there for a few days. It’s very intensive. It’s four days of infusions.
EDP Chemotherapy
Unfortunately, ACC patients don’t have a lot of FDA-approved treatment options. We only have one line of chemo, EDP (etoposide, doxorubicin, and cisplatin), and that’s done inpatient.
I showed up to my first chemotherapy session thinking I was going to go home after an infusion. But when I got to the hospital, they told me I was going to be there for a few days. It’s very intensive. It’s four days of infusions and the first day is doxorubicin, which is otherwise known as the Red Devil.
Before they start chemo, they have to do all sorts of tests to make sure your heart’s okay and I didn’t have any issues there. On the second day, I had etoposide and then on the third and fourth days, I had a combination of etoposide and cisplatin. Usually, I’d be in the hospital for five or six days.
Side Effects of EDP Chemotherapy
The first and second cycles were the worst by far. My body didn’t know what hit it and by day two, I was hugging the toilet. I remember thinking, This should be temporary. This is what happens. This is what you think of when you think of chemo. I almost felt so bad that I didn’t care. It was strange.
My blood counts were low too and made me feel so out of it. By the end, I needed a blood transfusion because I was right on the cusp of my counts being too low. They wanted to be on the safe side. I had a lot of fatigue for about a week.
There was a lot of vomiting. I’m already a small person, so I didn’t have a lot of weight to lose to begin with. My heaviest before my diagnosis was 108 lbs and I’m only 5 feet tall. I learned how to cope over time. I figured out that I could tolerate mac and cheese and that’s all I would eat. I made it through.
I had to wear a medical bracelet everywhere I went because if I got stressed out, sick, or developed a lot of pain, I could go into an adrenal crisis.
Mitotane
While doing chemo, I was taking mitotane, the only FDA-approved medication for adrenocortical carcinoma that’s been used since the 60s. What it does is shut down adrenal function and supposedly help shrink things, but more often than not, it slows things down. They use it in conjunction with chemo.
By my second round of chemo, I was taking nine pills per day. They would make me gag because they were enormous and very chalky, which would cause more throwing up.
Side Effects of Mitotane
I developed hypothyroidism due to that medication. It does a lot of damage to the body. It shuts down your adrenal glands so they don’t produce cortisol anymore. Then you have to take hydrocortisone, which is like synthetic cortisol. You have to have cortisol or things get very bad and you could end up in an adrenal crisis.
I had to wear a medical bracelet everywhere I went because if I got stressed out, sick, or developed a lot of pain, I could go into an adrenal crisis because my body wasn’t producing cortisol, which is a stress hormone. I didn’t know a lot about it before all of this started.
I have to have emergency injections now. It’s almost the same concept as an EpiPen, but it’s more difficult to administer because you have to draw up the medication. If I’m ever on the verge of an adrenal crisis, I have to inject myself and then go to the ER, but we don’t think that’s going to happen.
I’m off of that medication now, but my adrenal gland is still not producing enough cortisol to not take hydrocortisone, so I have to give myself thyroid hormones and synthetic stress hormones. These are all side effects of the treatment and not because of the cancer.
We saw shrinkage. They recommended for me to do a third round of chemo because we were trying to get to surgery so I could get the tumor removed.
Mid-Treatment Scan
By the time I was going to start chemo, the tumor had grown to 16 cm and it was so painful. The pain went away almost immediately after the first cycle. I was going to get two rounds of EDP and get a scan to see if it was responding. Thankfully, we saw shrinkage. They recommended for me to do a third round of chemo because we were trying to get to surgery so I could get the tumor removed.
I was going to NIH to get that done by one of their amazing surgeons, Dr. Hernandez. They typically keep the tumors and do different treatments to see what might work.
I was getting my third round in the hospital when I got the call saying they had approved my surgery, so that helped me push through. I was so happy. Initially, I didn’t know if we were going to get surgery or not. I’ve seen people not make it through the beginning stages of chemo and even have their tumors removed before things got bad. The ultimate goal is to get the tumor out. The faster you can do it, the better.
Other than my port insertion, I have never had surgery in my life. We started preparing for a trip up north for a massive surgery.
I eventually had to do more chemo after surgery, but it ended up not working anymore during my fourth and fifth rounds. I had my last cycle in February 2024.
Things are trending in the right direction, so I’m hopeful that this new treatment is working.
Targeted Therapy
They pulled me off mitotane and put me on lenvatinib, a TKI inhibitor. I don’t have many side effects from it. They use that in combination. It’s better than Keytruda (pembrolizumab) alone.
I only have to take two pills a day now. Symptom-wise, as far as the cancer goes, I feel way better. There are some indicators, like some tumor markers, that have significantly gone down. My team is seeing that things are trending in the right direction, so I’m hopeful that this new treatment is working.
I know some people in my support group who are on the same combination and they’re having amazing results. They’re stage 4 like me with no evidence of disease.
Mental Health Care
It feels like I’m making two steps forward and one step back, but now I feel like I’m moving forward at a steadier pace. It was difficult in the beginning because I didn’t have many coping mechanisms. I had never gotten therapy in my life. I thought I was so healthy—and I—was until cancer came into my life.
I didn’t expect cancer. If I was going to have any health problems, cancer was not what I thought it could be. It doesn’t run in my family. I’ve done genetic testing and I tested negative for everything they tested me for.
When I was trying to wrap my head around this, I thought, Why me? And why such a rare cancer? I don’t fit in the age bracket for the age that this affects people. It’s usually middle-aged women, young children, and some middle-aged men. More often, it’s women between their 40s and 60s. I’ve met some friends through the support group who are around my age, but it’s much more rare.
I struggled with why. I wanted to know. I felt like I would get some sort of closure if I knew that, but I don’t know if I’m ever going to get the answer.
I’m getting a lot of help in areas I’ve never had help in, so in a way, I’m rebuilding myself and enriching my life.
I struggled with body image because after my surgery, I was down to 82 lbs and that played a part in my mental health. I felt helpless because I realized there were so many things I couldn’t do, mostly due to the side effects of the medications I was on.
I would be wiped out and I wasn’t used to that. It hit me out of nowhere. I went from being a fixer or a helper to somebody who needed help. I didn’t know how to do that. It was painful for me to adjust.
For a long time, it was one of the most difficult parts for me mentally, especially after surgery because I was in horrible pain. It was an open surgery, so I have a scar from right under my rib cage to past my belly button. It’s probably 10 to 11 inches long.
After surgery, I had to get a lot of help with doing basic tasks. I felt awful for the people having to take care of me because this wasn’t who I was and that was a huge adjustment. I didn’t know how to cope.
I wasn’t getting therapy at the time, but I am now through MD Anderson, which is amazing. I see a counselor and I feel like my mental health is the best it has ever been despite my diagnosis. I’m getting a lot of help in areas I’ve never had help in, so in a way, I’m rebuilding myself and enriching my life. It’s amazing how these resources have been helping me.
I’m using the time that I have now to get my mental health where it needs to be, which in my opinion should be better than it was even before I was diagnosed. I’m working on myself. I see it as an opportunity.
I’ll never be happy that any of this happened, but I have the time and resources now and I’m seeing some benefits. I’m learning a lot of coping mechanisms in how to deal with things in the future.
If the treatment’s working and I don’t have that many side effects, that’s a win-win. I’ve seen nothing but improvement since going to MD Anderson.
There’s no telling where I would be right now if I hadn’t made those choices to focus on what I needed to do to get myself where I needed to be.
Words of Advice
Don’t let the doctors dictate what happens to you. If you have a gut feeling, act on it. Don’t be afraid to get second opinions. Seek out experts. There are usually other routes that you can take. There are so many resources out there. It was difficult for me in the beginning, but once I got everything set up and figured out, I told myself I may have saved my life with these changes.
There’s no telling where I would be right now if I hadn’t made those choices to focus on what I needed to do to get myself where I needed to be. One thing that they would always repeat in my support groups is don’t let location dictate your treatment, who you see, or who you get your recommendations from. If that’s a problem, then start looking into things. You’d be surprised at how many resources are available out there.
Don’t idly sit back and let things happen if things don’t feel right. Talk to multiple people. Join support groups. Not only do they provide great advice, especially in my case when there’s not a lot of information, but the sense of community that you get from it is priceless. I have made so many amazing friends in the ACC community.
There are usually support groups for every diagnosis that you can think of. Mine is so rare and there are three that I’m on. If I find someone with my diagnosis, the first thing I always think of is to try to plug them into the support groups. It helps in so many different ways that it might change the whole trajectory.
Symptoms: None; found the cancers during CAT scans for internal bleeding due to ulcers Treatment: Chemotherapy (capecitabine + temozolomide), surgery (distal pancreatectomy, to be scheduled)
...
Tracy was diagnosed with stage 2B colorectal cancer at 41. A former special education teacher turned personal trainer and nutrition coach, Tracy has long been passionate about promoting a whole-food, toxin-free lifestyle. Despite her healthy habits, she began experiencing troubling symptoms related to her digestive system that were dismissed by multiple doctors over the years.
Tracy’s health issues began in her 20s when she was diagnosed with irritable bowel syndrome (IBS), leading to frequent trips to the emergency room. Despite changing her diet and lifestyle, her symptoms persisted, including bloating, inflammation, and intermittent bleeding. During a vacation in 2014, Tracy saw blood in the toilet, prompting her to seek further medical evaluation. Initially attributing her symptoms to a hemorrhoid, her OB/GYN prescribed suppositories, which provided only temporary relief.
Concerned by the recurrent symptoms, Tracy eventually consulted a gastrointestinal specialist who recommended a colonoscopy. The procedure revealed a polyp near the anal verge, and further tests confirmed it was malignant. Although not initially alarmed, Tracy soon realized the gravity of her situation as she was referred to a colorectal surgeon.
The news was tough—her cancer was located so low in the rectum that surgery would likely require a permanent colostomy bag. Tracy sought multiple opinions, hoping for a different outcome, but each doctor confirmed the same treatment plan: neoadjuvant chemotherapy, radiation, and surgery. Her optimism was tested, but a compassionate oncology team helped her navigate the emotional and physical challenges ahead.
Tracy’s treatment began with 28 days of chemotherapy and radiation. She opted for oral chemotherapy (Xeloda), hoping it would feel less invasive, but the side effects quickly took a toll. By the third week of radiation, she experienced severe pain, dehydration, and burning sensations, leading to hospitalization. Despite her resistance to the idea of surgery, the worsening symptoms and the results of her post-radiation colonoscopy convinced her to proceed.
In December 2015, Tracy underwent surgery, resulting in a colostomy. Although she initially struggled to accept her new reality, including the emotional impact of living with a colostomy bag, she found strength through her faith and the support of her husband and doctors. Post-surgery, Tracy faced additional chemotherapy, but her body reacted severely and she was eventually advised to stop treatment due to complications.
Through it all, Tracy wrestled with feelings of shame and guilt, questioning whether her past choices led to her diagnosis. However, she ultimately embraced her situation, using it as an opportunity to advocate for self-awareness and early detection, particularly through colonoscopies. Tracy’s message is clear: listen to your body, advocate for your health, and turn pain into purpose by helping others.
Name: Tracy R.
Age at Diagnosis:
41
Diagnosis:
Colorectal Cancer
Staging:
Stage 2B
Symptoms:
Bloating and inflammation
Heaviness in the rectum
Intermittent rectal bleeding
Fatigue
Treatments:
Chemotherapy
Radiation
Surgery
This interview has been edited for clarity. This is not medical advice. Please consult with your healthcare provider for treatment decisions.
Interviewed by: Taylor Scheib Edited by: Katrina Villareal
McKenzie was diagnosed with stage 3C2 cervical cancer at 26. She initially experienced severe cramping following a miscarriage, which was initially dismissed as normal post-miscarriage pain. As her symptoms worsened, including severe back pain, excessive discharge, and heavy bleeding, she sought various treatments but found no relief.
After switching insurance, McKenzie visited her OB-GYN, who conducted an ultrasound and lab work. The ultrasound showed what her doctor suspected to be a hemorrhagic cyst or twisted ovary. However, during surgery, they discovered a large mass, later confirmed as cervical cancer.
Given the advanced stage of her cancer, surgery was not an option. Instead, McKenzie underwent seven weeks of daily radiation, six rounds of chemotherapy, and four rounds of brachytherapy (internal radiation), and is currently on immunotherapy, Keytruda.
The aggressive treatment took a toll on her, causing extreme nausea, sensitivity to foods, and hair loss, which was particularly difficult for her emotionally. Despite the challenges, she found solace in the support of her husband and medical team, especially her radiation oncologist, who provided cutting-edge treatment locally, negating the need to travel to distant hospitals.
The impact of her diagnosis extended beyond physical symptoms. McKenzie faced the reality that her treatment led to early menopause, eliminating the possibility of having biological children. She struggled with the emotional burden of the diagnosis and treatment, but her husband’s encouragement helped her stay focused on recovery. McKenzie also started sharing her experience online, finding it therapeutic to connect with others.
Despite the hardships, McKenzie emphasizes the importance of advocating for oneself. She advises others to listen to their bodies, seek timely medical advice, and not be swayed by non-medical opinions. Her experience underscores the unpredictable nature of cancer and the critical need for early detection and proactive healthcare.
Name: McKenzie E.
Diagnosis:
Cervical Cancer
Staging:
Stage 3C2
Symptoms:
Severe abdominal & back cramping
Persistent & extreme pain
Heavy discharge & bleeding
Treatments:
Radiation
Chemotherapy: cisplatin
Brachytherapy
Immunotherapy: Keytruda
This interview has been edited for clarity. This is not medical advice. Please consult with your healthcare provider for treatment decisions.
Symptoms: Intermittent spotting during or after sex, unpredictable menstrual cycle, abdominal pain particularly under the rib cage Treatments: Chemotherapy (cisplatin & paclitaxel), immunotherapy (Keytruda), surgery (total abdominal hysterectomy with bilateral salpingo-oophorectomy & omentectomy)
...
Interviewed by: Taylor Scheib Edited by: Katrina Villareal
Michael’s cancer journey began unexpectedly after a routine CT scan of his arthritic neck revealed a significant mass. Despite having no symptoms, he embarked on an 18-month ordeal involving surgery, chemotherapy, and radiation. Initially, Michael hesitated to share the severity of his head and neck cancer diagnosis with his wife, given her health struggles, but eventually confided in her and her sister, ensuring he had a solid support system.
His treatment was intensive: a neck dissection to remove the tumor, affected lymph nodes, and salivary glands, followed by 13 chemotherapy sessions with cisplatin and 35 rounds of radiation. Despite the physical and emotional toll, including concerns about dental issues and feeding tubes, Michael maintained a sense of normalcy, continuing to work throughout much of his treatment.
Michael documented his journey on Twitter, where he built a large following and found comfort in the community he created. His first post-treatment PET scan showed no evidence of disease, and he remains determined to defy the grim prognosis he initially received. Michael credits his resilience to his proactive approach and the unwavering support of his medical team and loved ones.
As he navigated the challenges of chemotherapy, Michael initially felt out of place in the treatment room but quickly became a central, upbeat figure known as “Mr. Sunshine.” He brought positivity to others and found unique support among a predominantly male group, as head and neck cancer is more common in men.
Reflecting on the collateral damage of cancer treatment, Michael acknowledges the physical and emotional challenges, including nerve damage, depression, and financial strain. He emphasizes the critical role of online and offline communities in helping him and others cope with the disease. Particularly passionate about addressing the financial toxicity of cancer, he highlights the efforts of organizations working to alleviate these economic burdens.
In addition to cancer, Michael has faced mental health challenges, including bipolar disorder and severe anxiety. He underscores the importance of not facing illness alone and encourages others to seek support. His advocacy on social media has become a significant part of his life, offering hope and connection to others facing similar struggles.
Looking back on his journey, Michael reflects that while cancer could have destroyed him, it has instead made him a better person—more compassionate, present, and committed to helping others. Despite the hardships, he finds purpose in advocating for those affected by cancer, ensuring no one has to suffer in silence or face financial ruin.
Name: Michael W.
Diagnosis:
Squamous Cell Head and Neck Cancer
Staging:
Stage 4
Symptom:
None; caught at routine neck CT scan
Treatments:
Surgery
Chemotherapy: cisplatin
Radiation
This interview has been edited for clarity. This is not medical advice. Please consult with your healthcare provider for treatment decisions.
Interviewed by: Taylor Scheib Edited by: Katrina Villareal
Paula initially experienced painful gas, irregular bowel movements, and eventually blood in her stool. She was often dismissed by doctors who attributed her symptoms to stomach flu, hemorrhoids, or irritable bowel syndrome. As her condition worsened, she became anemic and experienced severe pain, weight loss, and fainting spells.
After years of being misdiagnosed, Paula was finally diagnosed with colorectal cancer in 2014 after a series of urgent care visits and a failed colonoscopy. The tumor had been encapsulated in her colon and despite initial fears, it had not spread to other organs or lymph nodes.
Paula underwent emergency surgery to remove the tumor. Although doctors anticipated the need for chemotherapy or radiation, they were able to successfully remove the tumor and 34 lymph nodes. Paula’s cancer was found to be stage 3, but it had not spread to her lymph nodes.
Throughout her journey, Paula faced multiple challenges, including medical bias due to her race and sexual orientation. She experienced homophobia, racism, and misogyny, which contributed to delays in her diagnosis and treatment. Despite these challenges, she found strength in her community and in sharing her story.
Paula emphasizes the importance of early screening, particularly for those with a family history of colorectal cancer and for marginalized communities. She advocates for self-advocacy in healthcare, urging others to push for fair and humane treatment. Paula believes in the power of survival and thriving with cancer, encouraging others to know their family history, get screened, and not be afraid of a cancer diagnosis. She stresses the importance of treating everyone with dignity and respect in healthcare and encourages individuals to take control of their health.
Thank you to Johnson & Johnson for its support of our patient education program! The Patient Story retains full editorial control over all content.
This interview has been edited for clarity and length. This is not medical advice. Please consult with your healthcare provider for treatment decisions.
I had all the signs and symptoms that they tell you to look out for when it comes to colorectal cancer, but at the time, I didn’t know what they were.
Introduction
I’m in love with my best friend and partner, Lara. We have been together for 38 years and married for 10 years. I’m a cat mom to a little cat named Trixie.
I enjoy dancing and singing. I’m a Duran Duran fan and that’s how I met my wife. For our 38th anniversary, we went to their 40-year celebration in Birmingham, England.
I’m also a great cook and I have a collection of amazing cookbooks. I’m trying to make my way to some of the best restaurants in the world.
Pre-diagnosis
Initial Symptoms
I had all the signs and symptoms that they tell you to look out for when it comes to colorectal cancer, but at the time, I didn’t know what they were.
In the early days, I always had painful gas and weird poop. When I experienced them, I would automatically blame what I ate and drank, so I would dismiss them a lot of times.
But then I started to have more painful symptoms, especially with the gas. I told my wife that I felt like I had gas going up and it was getting trapped. There were times when it hurt so bad that I wanted to scream.
I started seeing more blood. I went to see a doctor who said I probably had hemorrhoids.
PCP Appointment
I started going to the doctor by the end of 2012. I went to my primary care physician, but he didn’t think much of it at the time. When the pain was bad, I would get told that I probably had stomach flu.
Symptoms Worsen
I started to see signs of blood in late 2013. Sometimes I would be straining to poop. Other times, I would have diarrhea. I didn’t have my regular PCP at the time, so I would see whoever was available and they told me to change my diet. When I told one doctor that I saw some blood, he thought I might have stomach flu, so he gave me antibiotics.
By 2014, I started seeing more blood. I went to see a doctor who said I probably had hemorrhoids, so whenever I saw blood, I assumed it was that. I also started having bad pain and being anemic, but I didn’t know it then. I was cold all the time.
Being Dismissed
They said things that a lot of people under the age of 50 would be told because they were too young. “You probably have IBS.” “It’s probably hemorrhoids.” “You just have gas.” “I’m sure it’s nothing. You’re too young.” I heard a lot of that over the year.
By the time 2014 came around, I had been on antibiotics for nine months from different doctors I had seen since 2013.
There were times when the pain would be worse and I would have to go to urgent care. I went at least six times. The first few times, they would say I had gastritis or something stomach-related and send me home with antibiotics and painkillers. The last few times, I was bent over and freezing because I was anemic. I started to lose weight and ended up losing 45 lbs.
I have experienced a lot of bias and straight-up racism with medical care.
The doctor came into the room and before he even said his name or mine, he said, “I don’t give drugs in this room.” My wife took the doctor out and started yelling at him in the hallway while I sat there, thinking, Don’t yell. They will not help me if you yell.
I have experienced a lot of bias and straight-up racism with medical care. In 2014, I had doctors ask, “Did you tell me you were gay? Listen, it doesn’t matter what’s wrong with you because you’re going to hell.”
Losing Her Job
I got a job that year, but I was having such a hard time because I couldn’t keep up. Then I started to see my hands turn white and thought I was working too hard and planning for our wedding was too much. But the doctors kept telling me that I was okay.
I started getting depressed because I didn’t feel good. I felt that I wasn’t pulling my weight or helping my wife like I should. I was telling people I couldn’t do things, but the doctor said I was okay so it was starting to mess with my head. I didn’t want to tell people when it was bad, so I was hiding it, which made it worse.
I ended up losing my job because I kept calling in sick.
I was so sick and dizzy that I could barely see and stand up.
Diagnosis
Turning Point
The day after we got married, we went to a Broadway play, but while we were there, I felt terrible. My colon was becoming completely blocked and I was starting to have serious complications. By the time the play was over, I was so sick and dizzy that I could barely see and stand up. I was shaking, my body was red, and my skin was hot.
When we got back to my best friend’s house, I ran past the door and barely made it to the bathroom. I had the most humiliating accident. I was lying on his bathroom floor and knew that this was bad. I knew something was wrong.
Around January, I started to have fainting spells. I went to an urgent care on a Thursday and said, “Ma’am, I don’t care what you say. Nothing is coming out of me but blood. Nothing!” The person behind the counter got a nurse, who came out and said, “I’ve seen some of your records. Has anybody given you a FIT test?”
I didn’t know what a FIT test was. She was a bit worried. She said, “You probably ate something red, but I’m going to give you this test. Unfortunately, we don’t have a lab here, so I will not have the results until next week.”
I was in agony and stayed in bed all weekend. I was going back and forth to the bathroom and blacking out. I called my wife and we went back to the ER. This time, I must have looked bad enough because they kept me. I still had a 13-hour wait, but they said, “Get her a room. Get some scans. We’re going to do a colonoscopy.”
I don’t remember much after that. I was so sick that they started to medicate me and put me out. When I woke up the next morning, it was time for rounds and the doctors were going to come and talk to me. They did scans, blood work, EKGs, and all these tests, and they tried to do a colonoscopy.
They tried doing a colonoscopy, but they couldn’t get through.
Getting the Official Diagnosis
Around eight doctors came in. My heart started pounding. One doctor said they tried doing a colonoscopy, but they couldn’t get through. I didn’t know what to say after. It was a devastating way to find out I had colorectal cancer.
Reaction to the Diagnosis
What did that man say to me? What did those people tell me? Why did all those people ignore me? How long does this cancer take to grow? Black people have a higher risk of this? I was pissed and it was messing with me as much as the cancer was messing with me. I was mad and when I’m mad, I keep it in because I don’t want to hurt other people. Doubt, depression, and misery set in, and I needed to find a way to turn it around.
Treatment
Surgery to Remove the Tumor
They said, “We’re going to do emergency surgery. Based on what we see, it looks bad. We believe the tumor has broken outside of the colon wall. It might have some interference with your liverand other organs. We’re not sure what we’re going to find. We’re possibly going to do radiation and chemo because it looks massive. We might have to shrink it first or we might have to go in, do surgery, clean it up, and fix what we can. We need to decide. We’re going to keep you here because you are completely dehydrated.” I was skin and bones, and an absolute mess.
The tumor board said it looked bad, so we weren’t going to do chemotherapy or radiation, but we were going to do surgery. They brought me back to the hospital and prepped me for surgery. I was there for about four days prior and then a week and a half after.
The tumor was encapsulated in the colon and has not broken outside of the colon wall.
I went in early morning to prep and they told us that surgery would take 12 to 14 hours. They said, “We believe you are stage 3. When we go in, we might have to take out your uterus. We will take out whatever the tumor has affected. We will take out part or most of your colon. You will probably have a permanent ostomy. We may have to take out part of your liver. If it has gone deeper into the stomach, we will decide from there. We will do what we must do and that’s all we can tell you right now.”
When I woke up, the nurses came over and said, “Ms. Chambers, your doctor will explain everything.” He came in and said, “When we got in, it wasn’t what we thought.” Lara said the doctors came to her about four hours into surgery. They found that the tumor was encapsulated in the colon and has not broken outside of the colon wall. My body was completely swollen. I was blocked. They took out 34 lymph nodes. The whole surgery took 5 ½ hours. When they got the pathology back, there was no cancer in any of the lymph nodes.
Genetic Testing & Family History
Once my doctors knew that I had colorectal cancer, they did all the right things. They asked all the questions, especially my family history. They asked me repeatedly.
I decided to stay at the county hospital because they saved my life. I found my team, so I continued my care with the oncology and GI staff. In conjunction with UTHealth, they were able to offer genetic testing, which I qualified for and was able to do through my wife’s insurance. They suggested that because they think I have a genetic component. They tested me for Lynch, but it was negative.
That same year, my father-in-law and I did a DNA test and the results said I had another brother. A cousin contacted me and we talked about what was going on and I found out about David. He allowed me to share part of his story. He is an ostomate and was diagnosed with stage 3 colorectal cancer two years before me. He didn’t know about our family because he was adopted when he was a kid. We then found out that there’s a family history of not only colorectal cancer but GI cancers, and it runs deep in the family.
Look at your own biases. Think about when you are at the doctor yourself and how you would like to be treated. Keep it professional but bring in that real spirit of caring.
Experiencing Bias
I experienced homophobia, racial bias, bigotry, and a lot of misogyny, which I think went into some of my diagnoses. A couple of people told me I was hysterical and that it was in my head. It was so freeing to tell my story for the first time. It brought people into my life who had a similar story.
Meeting them and finding that community made me realize that it wasn’t just me and that I wasn’t too young. It wasn’t because I was Black. It wasn’t any one of these things. There’s a way that we look at cancer and at diagnosis, treatment, and preventative medicine that needs to be changed. I had whole conversations about this cancer that has stigmas around it that I did not even know I was supposed to be ashamed of.
I’m a nine-year survivor and in those nine years, I’ve seen things change. Things take time. It’s like pulling a bandage off a wound and having the wound heal. We have to treat it and give it light. We have to uncover some of the issues with which we are dealing. Because we all have our own biases, it’s important for anybody in the healthcare field, especially here in the United States, to look at things within us that we need to look at.
Innately, I think most people who go into healthcare do it because they want to. It’s their calling. It’s also their decision to be in healthcare, a space where they care for people who show up in their rawest moments. It’s important to have grace and a little bit of humility. If someone’s coming to you, they’re putting their trust in you.
Treat people with respect and dignity. Ask them, “Who are you? What happened to you? How did you get here? How can I help you? How did you get to me? Thank you for coming here.” Listen to them and meet people where they are.
The calling in healthcare is not for everyone. We still have laws, rules, and regulations, and we are to treat everyone with dignity and respect. Look at your own biases. Think about when you are at the doctor yourself and how you would like to be treated. Keep it professional but bring in that real spirit of caring.
I want people to not be afraid to go to the doctor. I don’t want what happened to me to happen to anybody.
People need to get screened, especially those in the Black and LGBTQIA+ community. You need to feel comfortable and know that there are more and more places with affirming care and who show it. They want you to know that you are welcome. Look for the rainbows.
Let us know that we are welcome. Ask me what my pronoun is. Is that my partner or my wife? Do not assume. Ask us what we need and be in partnership with us.
I want people to not be afraid to go to the doctor. I don’t want what happened to me to happen to anybody. Know your family history. A lot of gay kids are kicked out of the house when they are young, but a lot of them stay in touch with their family members.
Words of Advice
We need to get screened for colorectal cancer. The screening age now is 45, but if you have a family history of colorectal cancer like me, especially if you have beautiful melanated skin, you want to make sure that you’re talking to your doctors early.
Cancer affects the family and not just the individual. If there’s cancer in the family, it needs to be openly discussed. We need to be tested. We need to know our family history.
Nobody is going to care for you the way you do. Always put yourself first.
Cancer is a scary word, but it’s a word. People survive, thrive, and live with cancer. We’re called survivors from the day that we are diagnosed, so from that day, I had a choice. Am I going to live with this or am I going to let it take me? I needed to find a way to live with it and I did.
But I didn’t want to just live—I wanted to thrive. If cancer’s something that you’re afraid of and it’s the reason you’re not getting screened, you’re doing yourself a disservice.
Advocate for yourself. You have the right to advocate for fair treatment and humane treatment. Nobody is going to care for you the way you do. Always put yourself first. Put the mask on first and take care of yourself first. Nothing can be healthy and whole until you are healthy and whole. If you’re not comfortable, say so.
Special thanks again to Johnson & Johnson for its support of our independent patient education content. The Patient Story retains full editorial control.
Krista’s Stage 1A IDC Breast Cancer with ATM Mutation Story
Interviewed by: Taylor Scheib Edited by: Katrina Villareal
Krista’s stage 1A breast cancer journey is deeply connected to her family’s history. Her mother was diagnosed with stage 3 breast cancer at 48 and underwent various treatments like chemotherapy, radiation, and hormone therapy. She tested positive for a mutation in the ATM gene, which raises the risk of breast cancer. This finding led Krista to get genetic testing, revealing she also carried the same mutation, giving her a 69% risk of developing breast cancer.
Krista began following a rigorous screening schedule, alternating between mammograms and breast MRIs every six months. Despite a normal mammogram, her MRI detected an abnormality. Though specialists initially dismissed it as non-cancerous, Krista felt uneasy and insisted on a biopsy. This confirmed her breast cancer diagnosis just two weeks before her scheduled preventative surgery.
She chose to undergo a double mastectomy with DIEP flap reconstruction, using tissue from her abdomen to reconstruct her breasts. The process involved an initial eight-hour surgery followed by a revision surgery. After the procedure, Krista was relieved to avoid chemotherapy due to her low Oncotype DX score. Instead, she began a five-year course of tamoxifen, experiencing minor side effects like sleep disturbances and fatigue.
Her treatment plan also included daily exercise, which helped manage the side effects. Krista’s nutrition strategy focused on a plant-heavy diet, aiming for 8 to 10 servings of fruits and vegetables daily with a balanced intake of high-quality, low-quantity meat.
Mentally, Krista dealt with stress by spending quiet time, running, and leaning on her husband’s support. She emphasizes the importance of making informed, personal treatment decisions and encourages others to consider genetic testing and explore all their options.
Krista’s motivation to share her story comes from a desire to empower others with the knowledge she has gained. She hopes to help others make informed decisions and potentially prevent cancer. She advocates for taking one’s time to navigate the overwhelming journey of cancer, stressing the importance of making decisions that bring peace of mind.
Name: Krista B.
Diagnosis:
Breast Cancer
Invasive ductal carcinoma (IDC)
HR+, HER2-
Staging:
Stage 1A
Mutations:
ATM
Symptoms:
None; abnormality detected in breast MRI
Treatments:
Surgery: double mastectomy with DIEP flap reconstruction
This interview has been edited for clarity. This is not medical advice. Please consult with your healthcare provider for treatment decisions.
Expand to read the AI-generated YouTube Video Transcript
[00:01] Hi, I’m Krista, and I am a nurse and a patient advocate and a breast cancer survivor. My story really begins with my mom’s cancer diagnosis. She was diagnosed at age 48 with stage 3 breast cancer. At that time, she was only tested for two gene mutations linked to breast cancer, which were the BRCA1 and BRCA2 mutations. She was negative.
[00:36] Fast forward, she did every type of treatment—chemo, radiation, hormone therapy, everything. She put up a strong fight for about 12 years. Shortly before she passed away, she was unfortunately offered expanded genetic testing for other genes linked to breast cancer. She did test positive for a mutation in her ATM gene, which was a pathogenic mutation and higher risk than the average ATM gene.
[01:15] She shared that with all of her children because we then had a 50% chance of inheriting that from her, so we had the option to also test for that mutation. A few months after she passed away, I decided to move forward with my own genetic testing and found out I was also a carrier of the same mutation. So, I had a 69% risk of breast cancer, a 5 to 10% risk of pancreatic cancer, and also a 2 to 3% risk of ovarian cancer.
[01:56] Because I was at high risk for these cancers, I started to follow the recommendations for more thorough and frequent screenings, which meant on top of mammograms, I was also doing breast MRI, alternating every six months. I started that process and also began considering different surgical options for preventative surgeries.
[02:20] During this time, my mammogram was normal, but my breast MRI showed an abnormality. We did some follow-up testing—ultrasound and diagnostic mammogram. At that time, they said that it did not look like cancer. I was nervous about that with my risk, so I followed up and had three specialists tell me that it was not cancer. They advised me to take my time, make my decisions, and move forward with the surgical plan that I had in place.
[03:01] So, I did that, but because it started to have a possibility of affecting the process of my surgery, I requested a biopsy. It came back two weeks before my preventative surgery and showed a diagnosis of breast cancer. It was a little bit of a shock. I went into my biopsy thinking, “Oh, I’m good. This is just a routine check to make sure it’s okay to move forward with my surgery in the order we had planned.” So, I was really surprised at the diagnosis, but I was grateful to have that plan in place already and that I wasn’t scrambling to make decisions.
[03:42] I had my first surgery, a double mastectomy with flap reconstruction, on January 30th of this year, followed by a second surgery in April. Luckily, I really believe that I owe this all to my mom and advances in genetics. I’m grateful every day for the fact that she did genetic testing because, to this day, at this point in time, I don’t think I would still have a diagnosis based on my screening schedule. I’m very grateful I was able to avoid chemotherapy and a lot of the other things that I watched her go through. I’m grateful for that every day. It saved my life.
[04:36] If you’re interested in doing genetic testing, the first step would be to talk to your medical provider. This can sometimes be your primary care provider, an OB-GYN, or any specialist in the field of cancer that you may or may not have a family history with. You’re going to want to request a hereditary cancer panel, which screens for somewhere around 79 different genes that are now linked to cancer. The first step would be to request that from your provider, and most of the time, they’ll recommend that you see a genetic counselor, which is a great idea in my opinion. They’re amazing and have the most up-to-date information on the different genes and the risks associated with each. They do a deep dive into your family history and then make recommendations for different testing.
[05:31] From that point, it has really changed my life. I have three little girls, and I just think how different it’s going to be for them and how much they can avoid. But when it comes down to choices for reconstruction, there are typically three main choices that are offered to patients or should be offered to patients. One of them is esthetic flat closure, the second one is breast implants, and the third is flat base reconstruction. Flat base reconstruction is one that’s a little less known. It was my choice, and rather than having an implant, they take tissue from a part of your body and basically transplant it with all the vessels and use that in place of the implant for your reconstruction.
[06:26] It’s pretty amazing the way that they do it, and there are different places that they can take the tissue from. One of the most common is the one that I chose called deep flap reconstruction. They take tissue from your abdomen and use that for the reconstruction. It’s a little bit of a longer surgery upfront, and it was a two-phase surgery for me. That’s very common for patients who choose this reconstruction option. It is around an eight-hour surgery usually, so a little longer.
[07:06] My advice to anyone who is facing these choices is that they’re very hard choices, right? They’re life-changing decisions that you have to make. Sometimes you aren’t given a lot of time, but the thing that I hope everyone understands is that there are different options out there. Regardless of what anyone else thinks—whether it’s your provider, your family members, or someone who has been through it—ultimately, it’s your decision, and it’s what you have to live with. It should be the choice that makes you feel the most at peace moving forward.
[07:50] I have a lot of patients who I talk to who get very frustrated because they were not offered all the options. That’s one of the reasons I like to share my story because even for me as a nurse, in the beginning, I did not have a clue that this was an option. My biggest advice would be to take your time. Even with a cancer diagnosis, you have time to make an informed decision. Consider all of your options and choose the one that makes you feel the most at peace moving forward.
[08:25] The recommendation for me, treatment-wise moving forward, was that I had a very low risk of recurrence. My Oncotype score was one out of 100, so no chemo was recommended. But I was hormone receptor-positive, HER2-negative. The recommendation for me was tamoxifen, and that would be over a five-year period. I am at this point only three months in, but very happy to say that my side effects have been very minimal so far. I know that can change, but so far, not bad—just a little bit of sleep disturbance and fatigue, but nothing that is not manageable.
[09:10] One of the things that my oncologist, who I love, recommended was making sure you exercise every day. That was going to make the biggest difference in my side effects on that medication, so he said, “Don’t stop.” So I increased it, and I’m going to keep doing that and hope for the best moving forward. I know that side effects can be really hard sometimes, and it’s always a hard choice. It was something that I never wanted to do, which is why I chose the preventative surgery. But here we are. Just try to make the best of it and take it day by day.
[09:52] I think I tend to carry stress well somehow, but after everything was finished, I felt this huge weight lifted off my shoulders. I remember saying to my husband, “I didn’t even realize how much I was carrying until I was done with the surgery part.” It’s a huge stressor, but I did try to do a few things during the last year as I was going through all of this that helped a lot.
[10:31] For me, I’m not necessarily a meditation person, but that is very helpful for a lot of people. For me, I have a swing on my back porch, and that’s kind of my space where I spend a lot of time. I guess it could be similar to meditation, but that was very helpful to me. I would just go out and have quiet—turn off the phone, have time to just kind of process things, and swing on my swing. Grounding is also really good.
[11:10] Having somebody to talk to is important. I’m very lucky. My husband is very supportive, and he listened to me. I’m an out-loud processor, so he listened as I made all these hard decisions and changed my mind 500 times. Just the back-and-forth, talking about all the things I’m learning about food—you have to have a person who is willing to listen and not necessarily give advice. That was very helpful.
[11:42] I’m also back to the exercise, but running is a huge stress relief for me. That was one of the things I also tried to focus on—making sure I was getting in running and doing some deep breathing.
[11:55] One of the biggest things that feels overwhelming to a lot of people who have just been diagnosed with cancer or are at high risk is, “What do I eat?” That was one of the first things that I said to my doctor, “What should I eat? Is there a specific diet that I should be on?” I talk to women every day who are asking the same questions. It is one of the most impactful things that we can do, but also one of the most overwhelming, especially if you’re trying to navigate all of these things being thrown at you with a new diagnosis and high-risk genes.
[12:34] I am in a Master’s of Medical Nutrition program right now, which I love. I get to focus a lot on the research with cancer prevention and all of the new studies that are coming out. I love it. I’m very passionate about it, but I will also say that there is no perfect plan. There’s no perfect diet that we can all do to prevent cancer, right? There’s no 100% guarantee with anything when it comes to cancer. It does what it wants.
[13:10] Some of the best recommendations I can give are to eat a lot of plants. One of the best things you can do is eat lots of fruits and vegetables. I think the recommendation is 5 or 6 servings. I try to go for 8 to 10 every day, which sounds like a lot, but once you start incorporating them and finding different ways to do it, there are so many things—fruits, vegetables, nuts, legumes, whole grains—that have so many benefits for cancer and trying to prevent cancer and reduce your risk as much as possible.
[13:47] The reason that I like to share this kind of information is because, for me personally, moving from this place of overwhelm and trying to navigate everything into a space where I felt more empowered was huge for me. I remember thinking, “I’m a nurse, and how much of this did I not know from the start, and how much have I had to learn?” I felt very fortunate to have access to a lot of courses and certifications that not everyone has.
[14:26] I feel like I owe my life to my mom and genetic testing, and I would be in a very different place without that. After I went through all of this, I felt this huge responsibility to share with others because I know there are so many people who could benefit from this information. Even if it makes a difference for one person or helps one person feel more empowered in their decision-making and informed about the options that are available, even genetic testing—if it helps one person or prevents one cancer diagnosis—it’s totally worth it.
[15:12] No matter what phase you’re going through, it’s scary, and it’s overwhelming. Whether you have been diagnosed with cancer already or are a provider who is just starting out on your journey, just know that it’s not always going to feel like it feels right now.
Interviewed by: Taylor Scheib Edited by: Katrina Villareal
Ariel, a fitness professional and single mother to an 18-month-old son, was diagnosed with stage 4 high-grade appendix cancer at age 30. Her symptoms began as sharp pains with gas and bowel movements, which she initially dismissed as menstrual-related. A routine OB-GYN visit revealed a large mass, leading to a diagnosis of mucinous adenocarcinoma, a rare and aggressive appendix cancer that had spread to multiple organs.
Ariel underwent a radical hysterectomy, which was one of the most challenging aspects of her journey, ending her ability to have more children. She found this loss deeply difficult and struggled with grief. However, she found gratitude in her role as a mother and the perspective gained through her experience, and found strength by focusing on the present and embracing her emotions.
Ariel went through 25 rounds of chemotherapy (FOLFOX and FOLFIRI) but unsuccessfully qualified for HIPEC (hyperthermic intraperitoneal chemotherapy). She participated in a clinical trial for PIPAC (pressurized intraperitoneal aerosol chemotherapy) at City of Hope, which led to two negative biopsies. To access specialized care, Ariel and her family relocated to be near the Huntsman Cancer Institute. This move provided her with crucial care and an integrated healthcare system.
Ariel encourages others to take control of their health care, actively participate in treatment decisions, and interview doctors to ensure comfort with their care team. She also stresses the importance of asking for help, staying flexible in beliefs, and giving freely to others as part of the healing process.
Symptoms: Severe bloating, bad stomachache, elevated CA 125 and tumor markers Treatments: Hyperthermic intraperitoneal chemotherapy with mitomycin C (HIPEC) surgery; removal of spleen, gallbladder, appendix, ovaries, uterus, womb, fallopian tubes, belly button
Symptom: Severe abdominal pain Treatments: Surgery (right hemisphere colectomy, appendectomy, HIPEC), chemotherapy (adjuvant chemo in 2014; after recurrence, 6 rounds of oxaliplatin with bevacizumab & capecitabine)
