Relapsing - What’s Next?

When you have an incurable disease that has treatments available, like amyloidosis, unfortunately it does not necessarily mean there is a cure. Treatments can be effective in getting the disease under control and reducing side effects, but patients are continually monitored to ensure the disease doesn’t relapse. And if at some point the disease reactivates, emotions can come alive. In today’s episode we’ll be talking about relapsing and what patients may go through when that happens. For an overview of amyloidosis, please see episode 2 (for clinicians) or episode 3 (for patients).

Transcript

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SPEAKER_00 0:00

Hi everyone. Welcome to the All Things Amyloid Podcast, where we feature patients, caregivers, and clinicians who discuss amyloidosis, a rare and incurable disease. Amyloidosis occurs when the body builds up abnormal amounts of misfolded amyloid protein, which can lead to serious and life-threatening outcomes. I'm your host, McKenzie, an amyloidosis patient and founder of Mackenzie's Mission. Our goal for this podcast is to raise awareness and share firsthand what it's like to live with this disease, to care for those affected by it, and to treat it. We hope this leads to earlier diagnosis and start of treatment, which will improve patient lives. When you have an incurable disease that has treatments available, like amyloidosis, unfortunately, it doesn't necessarily mean that there's a cure. Treatments can be effective in getting the disease under control and reducing side effects, but patients are continually monitored to ensure that the disease does not relapse. And if at some point the disease reactivates, emotions can come alive. In today's episode, we'll be talking about relapsing and what patients may go through when that happens in their journey. I'm happy to have Linda with me today to talk about relapsing in a patient's journey with amyloidosis. Welcome, Linda. It's so great to have you on the podcast again.

SPEAKER_01 1:24

Happy to be here.

SPEAKER_00 1:26

So I'm sure our discussion is probably one that every amyloidosis patient has thought about, particularly those with AL. And perhaps a good place for us to start today is with your diagnosis and what the initial treatment for amyloidosis looked like for you.

SPEAKER_01 1:42

Sure. So I was diagnosed at age 64 with AL amyloidosis, and I had cardiac, lung, GI, autonomic, and carpal tunnel involvement. The diagnosis came after a couple of years of unexplained symptoms, which were robbing me of my ability to lead the life I had become accustomed to. So I started after the diagnosis on a treatment protocol, Cyber D, and that was in June of 2018. That treatment, along with stem cell transplant, were really the only two treatments available at the time, but I didn't qualify for the transplant because of the severity of my cardiac. I tolerated the cyber-D treatments really well, and it was successful in reducing my elevated capolite change by about 80%. But about six months in, thinking things were going along nice and smoothly, it just all plateaued. So in I would say like November or December of 2018, uh no progress was being made. And that went for a few months. At that point, I was switched to deritumamab or in early 2019. Um, and I got my light chain numbers to normal range really in just a couple of months. But the derotumamab was really potent and pretty terrific at managing my disease state. Um, and at the same time, I was experiencing organ recovery that seemed to give me my old life back. I mean, I was in that very lucky cohort with both a very good hematological response and an equally impressive organ response.

SPEAKER_00 3:20

Yeah, and I'm I'm sure that had to give you a good feeling knowing that your light genes were coming under control, your organs were starting to respond to some of these treatments, and right, that sense of normalcy was returning to your life. So I'm curious to know how how long did that last?

SPEAKER_01 3:38

Well, you know, it it gave me every reason to be hopeful at first, and and it lasted for about three years, you know, during which time I had moments where I forgot I even had this disease. My kappa's were in normal range, and I had few, if any, organ issues uh during that time frame.

SPEAKER_00 3:58

Yeah. Were you on any maintenance therapy at that time?

SPEAKER_01 4:02

Uh yeah, they kept me on the Derotuma map. I I never stopped taking Dara. I started it in, I think, February of 2019, and I'm still on it today.

SPEAKER_00 4:10

And when you were going through that, were you ever thinking about the possibility of relapse? I mean, knowing that it always could be a possibility, or were you kind of trying to keep that in the back of your mind and and avoid those those thoughts?

SPEAKER_01 4:26

Yeah, it's it's it's a great question. Uh, I mean, at the beginning, when the Dara kicked in and everything was going my way, uh it almost seemed too good to be true. I mean, even my doctor was amazed at the at the progress I was making. Um, but you know, nonetheless, you know, when you go in for your labs once a month and then the lab report comes up and you open the patient portal, I I there was some trepidation. Um, but I have to say they were consistently so good for so many months that I actually let myself think that somehow Deborah was going to be this miracle medicine. I was gonna be scot-free for like 20 years on this. Um, and as you'll find out, you know, it didn't quite work that way. So what happened was um in June of 2022, so now I'm about three years in, um my capa started rising again. The doctors didn't panic. Um they took a what they call a wait and see approach for about six months, and we just kept watching the daras, the cappas tick, tick up a little bit at a time each month. Um, and then at a certain point, they decided they were going to put me on Revlimid in addition to my monthly uh derotumath injections. Um this combo, it really seemed to move my life chains down a bit, but really they're still not back to normal. And it's been over a year, going on really a few years now.

SPEAKER_03 5:54

Yeah.

SPEAKER_01 5:55

Uh in June of this year, we switched from Revolomid to Pomelist as my additional medication. Um, the capas have come down just a little bit more, but you know, we're not back in normal range just yet.

SPEAKER_00 6:09

That is so incredibly frustrating, you know, and just to see it you're so close, but you're not where you want to be, and that's hard. And but it seems like at this point and and in this period of your treatment journey, so to speak, your your kappas are were rising and then switching to the pommel span about two years. And and throughout this this period, how did these events affect you both emotionally and physically? I mean, I I know it has to be difficult.

SPEAKER_01 6:43

Yeah, I have to say back in June of 2022 when the kappa started rising after three really great years, um, it was like, you know, a punch in the gut. I remember um I was sitting in the exam room waiting to see my hematologist, and the patient portal lit up. Yeah, and I had no idea that the bad news was coming, right? Because I was just used to every month seeing some great numbers and being on my way, right? So I opened the portal and when the uh the labs came up, my kappas were out of range. And it felt like the blood just drained to my feet, and I thought, you know what, here we go. Here we go, it's happening already. I'm relapsing and now moving forward, I don't know what my life's gonna be. So it was it was it was a hard hit, uh, mainly because I'd done so well on DARA and like I said, I thought it was my my miracle drug. So mentally I would say I was unprepared to face the idea of a relapse. Um, I had really let myself feel optimistic about the longevity of DARA and I had almost taken for granted that I had my own life back. And now all of that just seemed so threatened, with you know, the future being the big unknown at this point. Um physically, I would say it was equally challenging. Um, I've had a hard time with some of the cardiac side effects of revelmet. I've had some tachycardia, occasional episodes of AFib, and now we're finding some extra heartbeats, which may or may not be premature ventricular contractions. So there's right now I'm I'm seeing a cardiologist fairly regularly. And in the past it would be six months to a year between visits, and now I'm seeing him every few weeks because we're just really struggling to stay a little bit ahead on these developments, um, tweaking my medication and and just seeing what's going on. You know, I just have to continue to work very closely with with my cardiologist, and I have to try to just stay optimistic. You know, sometimes I wake up and I'm feeling very strong and energetic, and at about 10 in the morning I start feeling the heart stuff happening. And you it's it's hard not to be discouraged discouraged by that.

SPEAKER_00 9:00

Of course. It truly is a roller coaster of emotions, right? Things appear to be stabilizing, right? Life is kind of returning to a semblance of what was normal. And just as you, I think, beautifully described it, you get a punch in the gut. And that hurts even more when you're on the heels of such a good experience like you'd had with the Derotumum MAM. And I I I certainly sympathize with you. I've had my own relapse over these last seven and a half years since I was diagnosed. And as I'd mentioned in a previous episode, I I had a stem cell transplant in 2017, and that immediately followed my diagnosis. That was the first treatment that we we we sought out. And at the time, there were no FDA-proof drugs like we have now, and transplant was kind of the gold standard at the time. You mentioned the cyborg D and the transplant, and we elected to go with transplant, and it worked beautifully. I mean, but before we had even gone home, we knew that I'd had a had a a complete response, and we'd hoped it was a one and done. Like you said, um you just feel so optimistic about the longevity of it. And we thought we hoped, rather not thought, we hoped this would really buy years, if not decades, in the complete response. And it was only two short years later, almost to the month, that my light chains first crept outside of normal range. And to say I was devastated was an absolute understatement. I mean, I I I couldn't bel believe that it was already back. I mean, I had just finished the revaccination process that you go through after having a transplant, and boom, you relapse again. I think you said it beautifully that you almost take for granted that you have your old life back. And that punch in the gut hurts all the more. And I remember the first thing I did, I found out I was actually on vacation in the Middle East, and I called my parents, and we were crushed. I mean, everyone had invested so much time and so much energy in just getting through the transplant process. And I think we were just starting to get over the hump and and start looking forward when it turns out we actually were looking back again. And we handled it well, without a doubt. I think we focused on taking the approach of let's not put the cart in front of the horse. Let's hear what the hematologist has to say before we ourselves get kind of too worked up about what we might be facing. And we always found great comfort in in having a solid plan. And that's exactly what we did. Like you, I ultimately ended up on rebel knit as a result. And it was effective for about a year or so, but you know, my light chains plateaued outside of normal range, higher than we'd like. And we made the decision to add Dara, and that seemed to be the magic ticket and um knock on wood. My labs have been normal since we stopped both of those drugs back in early 2022. So for me, leaning on my family was incredibly important for me to be able to navigate the emotions that not only come with diagnosis and the treatment of this disease, but also the relapse. And they were always there to listen, to serve as a sounding board and really just be there, be present to help in whatever way I needed, whether that was physically, emotionally, mentally, they were there. And I'm forever grateful for them. And you know, I'd like to talk more about how you navigated your relapse.

SPEAKER_01 12:58

Well, first of all, Kenzie, I have to say I'm not sure that I would have handled the relapse as well as you did. I know that stem cell transplant can be extraordinarily challenging and taxing, both physically and mentally. You know, when I was first diagnosed, I was evaluated for a transplant, but it was ruled out, you know, because my cardiac situation made it risky that I would not survive the procedure. I have to say I was almost relieved that they would not approve the transplant for me because I knew what an ordeal it can be. I was terrified of it. And then, you know, now I think here you were two years after enduring that ordeal, facing the awful specter of a relapse. It just it's overwhelming. And I have to say, I think you are amazing getting through it with such grace. Um, and I know from my own experience just how helpful and supportive um your family had to be for you and how important, you know, that was. Um, I too derived a great deal of strength from my immediate family and from my many friends. You know, I'm a sharer. When I got diagnosed and when I hit some bumps in the road, everybody knows. I I keep no secrets. Um, so everybody in my orbit really knew what was going on throughout. And they were always there for me when I needed them. And I like that phrase, just be there. Yeah. And sometimes you don't even have to do anything, just be there for me. And I know you're there.

SPEAKER_00 14:24

Yeah, absolutely. Because you're gonna tell them what you need.

SPEAKER_01 14:27

Exactly.

SPEAKER_00 14:28

Exactly. And you know, I think that's wonderful that you have the support that you lean on because this is this journey is not for the faint of heart. It really is filled with ups and downs. So to have people that you can surround yourself with who are there for you is is really just a priceless feeling. So let's talk about your outlook today, right? You've been through the relapse.

SPEAKER_01 14:51

So I would say it's a bit of a roller coaster. You know, generally I'm back to my old self physically and mentally. Um, you know, but then I have those days, especially when the cardiac issues surface, and you know, it sets me back because it holds me back physically those days. And and then mentally. You know, that's the sort of next step. Once you you start losing your old physical self, you you go to kind of a scary and and and disappointed place mentally. Um I think the hardest part about relapse is that it reminds you that this disease is not at all linear. You know, and we have to be reminded of that time after time. By its very nature, there are going to be setbacks, treatment changes. And you just you have to be patient through that process and try to remain as, you know, as hopeful as as you can. Um, and we all know now, compared to when you and I were first diagnosed, there are so many more treatments available out there and others coming down the pipeline. So I think it's a little bit easier for us today to remain hopeful than it might have been three, four, five years ago. My hematologist is optimistic about my process and thinks that that even if my capas stay just above normal range and I do not have serious organ involvement, this just might be my new norm. And I have to kind of get my head around that because I was always the kid that wanted the A plus in school. So I want my cat was in normal range. And he's like, that may not be necessary.

SPEAKER_02 16:25

Yeah.

SPEAKER_01 16:25

So he's kind of, I mean, I I bow to him because he has seen so many patients and he's got patients who have cathas just above normal for 10, 15 years, and they're doing just fine.

SPEAKER_02 16:36

Yeah.

SPEAKER_01 16:36

So I have to give up hopes for the A plus and just kind of get on board with where I am and just view this as my new normal and try to keep myself optimistic and really grateful for being where I am right now.

SPEAKER_00 16:50

And I think you make a really important point. I mean, just in the what, seven, six, seven years since we were diagnosed, I mean, the the treatment landscape is completely different than what it was back then. And I think it's important to kind of keep in the back of our minds that yes, while relapse is a very real possibility and it's not necessarily a one and done thing, that just as that is possible, so is the prospect of new drugs and new medications. And you know, I have to remain hopeful that maybe our miracle, our true miracle drug really is just right around the corner for us. And so I think it makes kind of accepting the potential reality of having another relapse that maybe we're gonna have some new tools to help get through that process that we don't have now. So as we wrap things up, I'd love to hear what words you have for the patients out there who have been through or think about the prospect of relapsing.

SPEAKER_01 17:55

Well, first I have to say you need to keep up with your monitoring machine regimen. Um, it's important because if if things begin to trend in the wrong direction, you you really want time to collaborate with your provider and draw a point. You've got to really stay one step ahead on this kind of stuff. Um when I relapsed and was in that what we call the three-month waiting period to see how my kathas would um respond to the new treatment. My doctor told me that, you know, he put me on RevLomid in addition to the Dara injection. And he said the first month or two, you're not gonna see much difference. It's it's you can't read the labs and and know if the meds are being effective. He said, We we need to give it a solid three months. So, of course, what did I do during those three months? I became like totally preoccupied with worry. You know, I I wouldn't even open the monthly test results for fear I'd see something that, you know, would just indicate things were not going our way. Um so I just decided to wait until my three month follow-up with my doctor. And um with well, you know, I wanted to have all the test results in hand this way, whatever they were, he can explain to me how we should be reacting to it at this point. But I'm I would be lying if I said I wasn't terrified for those three months. I went to kind of a really um kind of a dark place where I I was convincing myself this is not gonna work. I'm facing certain death after that, like I just felt like I was going to not have a path out or a way out at that point. And it was a little too much. And I had a very wise person who said to me after I confessed all of these worries to her, she said, you know, look, you're not there yet. You know, meaning we don't know. We cannot even know right now where this will go. So she said, stay in the moment and try to be hopeful. And I took that to heart. I really did. And I realized that I had to make the decision not to borrow trouble. You know, it really helped me through that stressful beating period. And I have to say, I repeat that little mantra, look, you're not there yet, when I feel the worry creeping back. You know, I just I've convinced myself that I things can change, and I just can't let myself become less than hopeful and optimistic. It's just not good for me. Um, so my takeaway obviously is stay hopeful if you can. Remember that it's not linear, that we might get off track here and there, but there's always a good chance that we can get back to a strong and hopefully lasting response with new medications um that are becoming available by the day for us.

SPEAKER_00 20:43

Yeah, and I just want to echo again some of your comments, Linda, for our listeners, because I think they're so important, right? Diseases like amyloidosis that currently have no cure, but we do have treatments available, often means that relapse is just kind of part of the ride that we're on. And I've heard people say that they have the sword of Damocles hanging over their head. And it's a parable that means that they're in a situation in which they feel like something bad could happen to them at any time. And I, you know, it they're referring to relapsing when they say that. And it's um it's a very real feeling. And while there is truth to that feeling, I'm not quite sure it's worth kind of obsessing and getting worked up over. Like you said, look, we're we're not there yet. And certainly tragedies can happen at any time, but they could be random and unforeseen. So putting that energy into remaining hopeful and having a productive outlook, I think, is essential. And ultimately, every patient needs to decide just how much they want to worry over things that may or may not happen in the future. Because while relapse is possible, it is it's certainly not guaranteed. I think it's also worth remembering that therapies can have a shelf life in terms of effectiveness. And that also means that relapse is a possibility, and there are more treatments for providers to consider than ever before. So that really does offer tremendous hope for patients who do face a relapse. There's a pipeline of clinical trials that evaluate new treatments, which very well may be available to patients in the very near future. So I think it's important to surround yourself, like we discussed, with people who support you and uplift you in really the most challenging times of dealing with this disease. But most importantly, to never give up, right? There continues to be more hope than ever. So trying to find hope in you know the face of an uphill battle, I think, is worth trying to do. But again, I want to thank you, Linda, for joining us today. It's been an absolute pleasure to have you and and share some of your insight into what this experience has been like for you. And I know it's certainly going to be something that's very valuable to our listeners. Thank you for listening to the All Things Amyloid Podcast. If you find our podcast informative, please follow and share it with others. If you want to learn more about the work we're doing at Mackenzie's Mission, please visit mm713.org. We'd also like to thank AstraZeneca Pharmaceuticals, Alexion Pharmaceuticals, Al Nylon Pharmaceuticals, and Bridge Biopharma for their sponsorship to help raise awareness about amyloidosis. We hope you stay tuned for future episode drops. Thanks for listening.