ATTR-CM Nurse Navigation Podcast

Episode 3

A conversation about the needs of patients with ATTR-CM from the time of disease diagnosis, including the need for primary care for ongoing management.


00:00:05 Moderator: Kim Newlin

Welcome. You’re listening to Nurse Navigation and ATTR-CM and this is episode 3 titled “The ATTR-CM Arc: Helping Patients Navigate Their New Normal.”

00:00:17 Moderator: Kim Newlin

I’m your moderator, Kim Newlin, and I’m joined today by Janell Grazzini Frantz and Karen McLean, two nurses working in different roles but making a difference every day for our patients with transthyretin amyloid cardiomyopathy.

00:00:31 Moderator: Kim Newlin

We’ll use the acronym ATTR during today’s podcast. The disease of protein misfolding leading to amyloid fibril deposition and organs and tissues, once considered rare, it is increasingly recognized as a cause of heart failure.

00:00:46 Moderator: Kim Newlin

 Today we’re going to discuss ATTR: what it is, working as part of a team to ensure coordinated care and effect changes for these patients, and how to help patients and family members successfully navigate health care to reduce symptom progression and improve outcomes.

00:01:01 Moderator: Kim Newlin

We encourage you to take advantage of the related resources at the following website,, to help you in your clinical practice.

00:01:14 Moderator: Kim Newlin

Welcome Janell and Karen. First question for you: Like other diseases, there is a progression in ATTR-CM. Can you tell us the types of patients you see in practice and the focus for these patients and families? Janell, why don’t you start.

00:01:29 Speaker 1: Janell Grazzini Frantz

Thank you, Kim. I’m delighted to be here. While we diagnose our own Mayo Clinic patients with amyloid, which is always kind of interesting, the majority are referred by their local cardiologists or they’ll self- refer. Many are just coming for a second opinion, or sometimes they’re coming specifically for research opportunities.

00:01:48 Speaker 1: Janell Grazzini Frantz

We see them in all stages of the disease–from incidentally picked up on a biopsy or very early to late and/or end stage.

00:01:56 Speaker 1: Janell Grazzini Frantz

The first thing we assure is that the diagnosis is correct. They’re set up with an echocardiogram; pyrophosphate, or PYP, scan; and a fat biopsy; light chains; serum electrophoresis; and urine electrophoresis.

00:02:10 Speaker 1: Janell Grazzini Frantz

We also make sure that they’ve had DNA typing to rule out mutation. We discuss the treatment options for TTR amyloid and how to manage their symptoms from amyloid.

00:02:21 Speaker 1: Janell Grazzini Frantz

Often, we’ll need to refer them to the appropriate other specialties, such as heart rhythm, hematology, or neurology. Some patients will go home to be managed by their local cardiologists or primaries, but some prefer to have me specifically manage their amyloid and heart failure.

00:02:37 Speaker 1: Janell Grazzini Frantz

Given many of them are end stage, they’re exceedingly high touch, which we achieve through phone calls, videos or frequent—sometimes every one to two weeks—visits in an attempt to improve their symptoms.

00:02:49 Speaker 1: Janell Grazzini Frantz

Because of our high volume of amyloid patients, it’s 60% of my practice right now. Patients will often contact us for our opinion on recommendations made by the local team.

00:03:01 Speaker 1: Janell Grazzini Frantz

The vast majority of these are appropriate, but occasionally we will disagree with the recommendations, such as adding beta blockade to manage minor tachycardia in a patient with low cardiac index.

00:03:13 Moderator: Kim Newlin

Thank you, Janell. You talked a little bit about some things in episode one, which was the teams and the patients that you see. Karen, would you like to add some about your patients?

00:03:22 Speaker 2: Karen McClean

Sure, thanks for having me. If we’re seeing you for the first time to confirm a diagnosis or you are already diagnosed incidentally, we initially start by confirming that diagnosis, and we do that by ordering a variety of tests which are mostly imaging and labs that Janell mentioned just now, which is the echocardiogram, a cardiac MRI, and a PYP.

00:03:42 Speaker 2: Karen McClean

And then some of the labs, the SPEP [serum protein electrophoresis] and UPEP [Urine protein electrophoresis] to name a few. We also will set you up to meet our genetic counselor and this is usually a coordinated visit [at] the same time you see the cardiologist, but occasionally can occur outside of your cardiology appointment and they take a more deep dive into the patient’s family tree and discuss the necessity of genetic testing and all that’s involved in the process.

00:04:06 Speaker 2: Karen McClean

They’re really great resource. Once a diagnosis is made and treatments are started, we would usually follow up with them every three to six months initially and then annually depending on the patient’s condition.

00:04:15 Speaker 2: Karen McClean

There is currently no evidence that suggests repeat testing is necessary after the initial diagnosis, so there’s no need to repeat the tests for amyloid. However, if there’s a need for future testing, like new or worsening symptoms, then we will order to suit.

00:04:30 Moderator: Kim Newlin

Thank you both. Definitely a lot to consider. Another question I’ll have Karen start with: What are the treatment considerations for the patients that you see?

00:04:38 Speaker 2: Karen McClean

So, after the diagnosis has been made and it’s confirmed that the patient does have ATTR, we try to start medications like tafamidis that have been approved to slow the progression. .

00:04:48 Speaker 2: Karen McClean

We often have to work in conjunction with Pharmacy, because these medications are really expensive and often require a prior authorization from the patient’s insurance. If the medication is not affordable, we work with the patient through shared decision-making to make alternative options like clinical trials.

00:05:05 Speaker 2: Karen McClean

The few that we have seen in our clinic have been able to either afford the medication with insurance and help from drug companies, but [for] those who can’t afford the medications, we work closely with the patient at managing their symptoms.

00:05:17 Speaker 2: Karen McClean

Most of the management for ATTR amyloid is managing symptoms which often present like heart failure, so starting patients on a diuretic, usually a loop and then potassium supplementations if required, and sometimes you’ll add an ACE for those with hypertension issues but, basically, it’s symptom management.

00:05:37 Moderator: Kim Newlin

Thank you, Karen. Like you said, lots to consider with these complex patients who’ve often been misdiagnosed or haven’t been receiving the right treatment before coming to your type of program.

00:05:46 Moderator: Kim Newlin

Janell, do you want to add anything in around your treatment considerations?

00:05:50 Speaker 1: Janell Grazzini Frantz

Like Karen said, Tafamidis, but it is really challenging and cost prohibitive for most patients. So we work with our specialty pharmacy try to access some of the foundation funds that are available; certainly work with the companies that might be able to help offset the price to some degree.

00:06:09 Speaker 1: Janell Grazzini Frantz

My nurses are crucial in walking the patients through that maze. Say Tafamidis was covered last year, but this year they couldn’t get it covered. I’ve had patients quite terrified that if they stopped the drug, or run out of the drug, they will die suddenly or soon.

00:06:27 Speaker 1: Janell Grazzini Frantz

Or, the family feels incredible guilt because they’re not willing to bankrupt themselves to pay for the medication to extend their loved one’s life.

00:06:38 Speaker 1: Janell Grazzini Frantz

We’ll consider Diflunisal, but that’s actually not even well covered and can’t be used with many of the patients that have significant volume overload or renal dysfunction.

00:06:47 Speaker 1: Janell Grazzini Frantz

Oldies but goodies: Doxycycline and TUDCA (Tauroursodeoxycholic Acid) — you can buy it on Amazon, believe it or not — can be used as fibril disruptors, but the data is scant on those.

00:07:00 Speaker 1: Janell Grazzini Frantz

And then, of course, for TTR hereditary or variant type with neuropathy, the RNA inhibitors Inotersen and Patisiran, were FDA approved to improve their neuropathy.

00:07:10 Speaker 1: Janell Grazzini Frantz

And then, finally, we’re fortunate at Mayo we have many trials running, but the patient needs to meet the criteria and they must be willing to do the requirements of the trial.

00:07:20 Speaker 1: Janell Grazzini Frantz

We currently have one now that’s once weekly blood tests, so that’s a bit challenging.

00:07:25 Moderator: Kim Newlin

Right, especially for patients who may be coming in from outside the area but still want to participate in these trials.

00:07:31 Moderator: Kim Newlin

So, lots of things to consider, not necessarily a lot of options, and again, highlighting the role of nurses, nurse navigators, and making sure patients realize there are places they can go to try to get coverage for these important medications and treatments.

00:07:46 Moderator: Kim Newlin

Janell, I’ll start with you on this one. What other suggestions would you give to a clinician who sees these patients in practice?

00:07:52 Moderator: Kim Newlin

Whether it’s kind of early on and trying to identify or you know, scratching your head about a patient that may have amyloid, or someone who’s actively seeing patients with current diagnosis of amyloid.

00:08:03 Speaker 1: Janell Grazzini Frantz

It really is super important to assure you have the diagnosis correct, you have done all the appropriate testing to rule out AL amyloid and then once that’s complete…

00:08:13 Speaker 1: Janell Grazzini Frantz

…assess this stage as that’s really going to drive the treatment recommendations. I mean, for some patients they’re ATTR amyloidosis disease stage three, their New York Heart Association functional class four, their ejection fraction is already down, and the renal function is potentially getting really seriously out of whack.

00:08:33 Speaker 1: Janell Grazzini Frantz

Those patients are really unlikely to benefit from Tafamidis and therefore putting them through the hassle of trying to get it paid for—it really doesn’t make any sense.

00:08:44 Speaker 1: Janell Grazzini Frantz

Other things to remember: they often need a generous pulse and that can get people excited, but they need it to maintain that adequate cardiac output. And so, when you’re looking at the echocardiogram, or your echocardiographer’s, if they could give you the cardiac output that would be incredibly helpful to know.

00:09:03 Speaker 1: Janell Grazzini Frantz

…”Hey, they’re gonna need a higher pulse.” Even consider decreasing or stopping the beta blocker if you can.

00:09:07 Speaker 1: Janell Grazzini Frantz

Also, because they present mostly with a right-sided heart failure picture, furosemide is not such a great choice as they can’t adequately absorb it and, therefore, often I’ll switch them over to Torsemide or Bumex.

00:09:20 Speaker 1: Janell Grazzini Frantz

My preference is Torsemide, just ’cause it lasts about 10 to 12 hours. But last I checked, the VA has a preference for Bumex, but either one has excellent gut bioavailability.

00:09:31 Speaker 1: Janell Grazzini Frantz

And so, if they’re not doing well on furosemide, getting the high doses just flip them over to one or the other–Torsemide or Bumex–and you often get really nice diuresis on those patients.

00:09:41 Moderator: Kim Newlin

Always good to have, kind of, what other tools should we put in our toolbox when handling patients who have these complex conditions. So, thanks for giving us some new tools. 

00:09:49 Moderator: Kim Newlin

Karen, what are your thoughts on other suggestions you can give to clinicians?

00:09:53 Speaker 2: Karen McClean

One of the biggest suggestions that I can give is to encourage patients that have been diagnosed with ATTR to establish care with a primary care, if they don’t already have one.

00:10:05 Speaker 2: Karen McClean

This might sound very basic, but we get a lot of patients that don’t have a need for a primary care, and they think that they’ll just see their cardiologist.

00:10:10 Speaker 2: Karen McClean

But the primary cares pay a really invaluable resource for patients in the system and to manage their day-to-day concerns in concert with specialists. So that is one of the recommendations that we really encourage our patients to seek out. Another one is to have an open relationship with your patient.

00:10:27 Speaker 2: Karen McClean

These are patients that have to be monitored closely, and one thing that we tell our amyloid patients at the University of Virginia, more so than others, is if anyone changes your medications, namely the diuretics and your blood pressure meds, to call us first, because there’s a very fine balance between good and bad for these patients, and having that relationship where the patients feel comfortable that they can contact us to help keep these patients out of hospital is really important.

00:10:54 Moderator: Kim Newlin

You’re definitely highlighting some of the key points we talked about in episode one around you have your team, but making sure you have a primary care which, like you said, you assume people do, but they don’t and that can create a real gap as well as the communication part, and making sure that’s navigated.

00:11:09 Moderator: Kim Newlin

These aren’t patients you can touch every three months. They’re a little bit more delicate, and needing that regular connection.

00:11:15 Moderator: Kim Newlin

Any last thoughts before we wrap up Episode 3?

00:11:18 Speaker 1: Janell Grazzini Frantz

We want to work in concert with your primary team and your local cardiology team. We don’t want to take over. We all have to work together for the very best outcome for the patients.

00:11:29 Moderator: Kim Newlin

Thank you again to our two clinical experts, Janell and Karen, for these great insights and clinical pearls. We’d also like to thank the audience for joining us for “The ATTR-CM ARC: Helping Patients Navigate Their New Normal.” Thank you to Pfizer for support of this ATTR podcast series.

00:11:47 Moderator: Kim Newlin

Remember that you can access the podcast series and the related resources a Thank you.