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Interviewer: Amyloidosis is a rare but not uncommon disease that is a lot of times difficult to diagnose and can be very tricky to treat. If you've been told that you have this condition or know somebody who has, this is the podcast that's going to help explain it and help you understand it better. That's coming up next on The Scope.

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Dr. Jose Nativi is a Cardiovascular Director of the Amyloidosis Program at University of Utah and Huntsman Cancer Institute. He's an expert in this stuff and we're going to explore with him this condition. What you need to know and what kind of treatment options are available. So let's talk about the condition first. You're a cardiovascular expert, but from what I understand this condition can affect a lot of different organs in the body and that's part of what makes it complex. Is that a fair statement?

Dr. Nativi: That's right. Amyloidosis mainly is a condition when there's proteins in your body that they misfold and then deposit on several tissues. So depending on what tissue is affected, symptoms are going to appear and also depending on the type of protein that is going into the several tissues, it will have an impact in your system.

Interviewer: Two different variables here.

Dr. Nativi: Right.

Interviewer: That's kind of unusual for any sort of illness or disease. It makes it very complicated.

Dr. Nativi: Right. These patients tend to show with multiple symptoms, as you mentioned. If the deposition is in the heart, it will come with heart failure symptoms, like shortness of breath, cough, fatigue. But at the same time, they might have the deposition in the kidneys and they will come with swelling legs, weight gain. And sometimes, unfortunately, it takes time for these patients to underwent the full workup and finally get into the diagnosis of amyloidosis.

Interviewer: Depending on what organ it gets into it sounds like it starts with the symptoms of that organ failing.

Dr. Nativi: Exactly. So it's hard to recognize at the beginning. Amyloidosis the majority of the times, get a different diagnosis. The most common example is the patients with carpal tunnel syndrome. We have patients that they go into carpal tunnel surgery because of numbness of the hands but later, with time and with the progression of the disease, we figure out that this carpal tunnel syndrome was amyloid deposition in the nervous system. So, yes, it will depend heavily in what organ is affected and you need a big multidisciplinary team to make a comprehensive evaluation and make sure that you evaluate the majority of the organs that could be impacted by amyloidosis.

Interviewer: What are the organs that could be impacted?

Dr. Nativi: Heart is one of the most commons. It's affected in all types of amyloidosis.

Interviewer: So regardless of my other symptoms, likely my heart's going to be affected if I have this?

Dr. Nativi: In the majority of the cases, yes.

Interviewer: And then other things on top of that?

Dr. Nativi: Correct. Kidneys is very common. Kidney patients will have abnormal proteins and then they go into a routine check-up to their primary care providers and they have an abnormal protein in their urine, it's very common. A lot of neuropathies, numbness and tingling in the hands or the lower extremities is very common. If there is deposition in the skin, you can have changes in your skin. You can have purpuric changes around your eyes or in your arms or fingers. Also, there could be problems in your blood. If you check a routine blood test, some types of amyloidosis, the problem come from the bone marrow so you can see abnormalities in routine blood tests too.

Interviewer: And that's where it all originates, in the bone marrow.

Dr. Nativi: For the most common type of amyloidosis . . . let's talk about the most common types of amyloidosis. The most common is called AL amyloidosis. A is for amyloidosis. L is for light chains. That means that the light chains are the proteins that are going into the different tissues. In this type of amyloidosis, the problem is in the bone marrow. You have an abnormal production of this protein that usually is used for your body to fight infections, but it's an overproduction of this protein and then the misfolding of the protein. And because of the bone marrow that is in contact with the blood, the AL amyloidosis will tend to affect several organs because the proteins are just spread out in your body.

The other type of amyloidosis is called transthyretin amyloidosis. A Transthyretin is a protein that is normally produced in your liver, however, some people will have genetic mutations of this normal protein, and the genetic mutation will produce the misfolding and the deposition. We called that familial amyloidosis.

That is the second one, and then the third type of amyloidosis, there are several types, but these are the ones we are going to focus today. The third type is called senile amyloidosis. It's called senile amyloidosis because it's more prevalent in older adults. And it's a normal transthyretin protein produced in the liver. There's no genetic mutation, but with time and with aging, the protein misfolds and go into deposition to several organs, mainly the heart, lungs, and nervous systems.

So, amyloidosis, there are several types, several causes, and several symptoms, so you need a comprehensive team to get together and put this puzzle together.

Interviewer: Yeah, and we have a clinic here at University of Utah Health Care which we'll talk about more in-depth in another podcast that brings all those experts together. Which brings the question, do most people have it diagnosed by their physician or are you finding that a lot of people are self-diagnosing it because it is somewhat of a rare but not uncommon disease? I mean, how are people finding out they have it?

Dr. Nativi: I agree with you, there's a lot of under-diagnosed amyloidosis. The incidence in the United States is around 3,000 cases. However, as a field, we believe that there are thousands of patients that are not diagnosed. So how the patient goes to a diagnosis of amyloidosis, is a very high index of suspicion from both, from the patient and from their providers.

You have this patient that have multiple things going on and usually they go to several providers and each provider will tell them different plans. We're trying to avoid that in our center. And finally, at the end, there is an organ that will really, really be affected and sometimes it's very late and that's where some providers start thinking about doing a biopsy.

The diagnosis of amyloidosis requires tissue. This is critical. This is important. This is a very important message. You need tissue of the patient in order to make this diagnosis. So what type of tissue? Several types. The most simple tissue that we can get is fat from the skin of your abdomen. This is a clinical out-patient procedure. You don't need to be admitted to the hospital. We take a sample of your fat of your skin. We send it to the microscope and then we do special staining that is called Congo Red.

With the microscope and the Congo Red staining, we can identify the Amyloid proteins and then we can make a diagnosis. And after that, we do the whole comprehensive evaluation to see what organs are affected. Sometimes, patients will require other types of tissues: heart biopsies, kidney biopsies, bone marrow biopsies. But there are several ways and for primary care providers, a good start would be a fat skin simple biopsy as a way to start.

Interviewer: As somebody who thinks, "Hmm, I wonder if I have this." Is there something that you could say that would make them go, "Yeah, I think do." Because it sounds like, like you said, you get a lot of frustration with a lot of doctors, you get a lot of different diagnoses, nobody can really figure out what is going on, carpal tunnel operation, how do you finally end up here as a patient as quickly as possible?

Dr. Nativi: Yeah, there is several clinical scenarios. Definitely, a patient that has several organs affected at the same time without a clear diagnosis that will be a red flag.

Interviewer: Okay.

Dr. Nativi: Another red flag that is kind of . . . there are some clinical fissures that are very particular for amyloidosis. An enlarged tongue. This is very particular for amyloidosis. If you notice that your tongue is growing or is increasing or is getting thicker, the majority of the time is related to amyloidosis, the position in the tongue, and is very particular for amyloidosis. So if a patient comes to me with a symptom of tongue enlargement, I will try to rule out amyloidosis first.

Another common symptom, it's not that common, but it is very particular for amyloidosis, is for a purpuric change in the skin below your eyes. If your skin below your eyes becomes purple or blue, this is very particular for amyloidosis and I would go to your doctor the sooner the better.

Interviewer: All right. No cure just treatments from what I understand at this point. Is that accurate?

Dr. Nativi: Well, there has been a lot of progress in amyloidosis. Usually when patients read about amyloidosis, either online or other sources, they get really concerned because the belief is that there is nothing to do for amyloidosis. That's not true, especially in big centers that treat amyloidosis, there's a lot of options.

Key messages are for options is the earliest the diagnosis, the more options we have for you. So the less organ involvement, the more options that we have for you. So, for AL amyloidosis, we can offer chemo therapy and even a bone marrow transplantation with very good outcomes with several years of survival. I have a patient with a diagnosis of AL amyloidosis that is more than 20 years with this diagnosis and she went through chemo and she went through several things and she's doing well.

For other types of amyloidosis, if it is transthyretin amyloidosis we can offer either liver transplantation because the problem is coming from the liver. If the liver and the heart is affected, we can offer you liver and heart transplantation. If the kidney is affected, we can offer kidney transplantation. And one of the interests of our group too is also clinical trials, research clinical trials. So right now we are enrolling clinical trials of a medication that will avoid the misfolding of the protein . . .

Interviewer: The cause.

Dr. Nativi: . . . so that you don't have progression of the disease. So the outcomes of amyloidosis are changing a lot and the literature is changing a lot. So I think this is a good time right now for several options and for a better prognosis for these patients.

Interviewer: Anything that I forget to ask anything you feel compelled to say?

Dr. Nativi: I will say that the earliest that we can make this diagnosis the better. If there is any concerns, if you have several symptoms, several organs affected, and it's unclear the diagnosis, I think it will be reasonable to screen for amyloidosis.

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Interviewer: You've had a surgery now what do you eat afterwards? Does it matter? We're going to find out next on The Scope.

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Interviewer: After you've had a surgery does your diet influence how well you'll heal? We're with Dr. Jeffrey Camden. He's a surgeon at the University of Utah Hospital. Does your diet matter after surgery?

Dr. Jeff Campsen: I think so. Let's start from the beginning, so you have some sort of inter-abdominal surgery, and you come out of the surgery, and at some point you're going to want to eat again. Some people take a couple days to feel hungry, some people want to eat right away, and it depends on what's going on. So we sit down as the managing team and really try to make sure that your intestines for lack of a better word wake back up and can accept food and basically allow you not to get sick after surgery. So the things that we look for are basically everybody hears their stomach rumble, we want to hear those sounds. So after surgery sometimes your intestines can shut down. It's called an ileus and it basically means that the intestines aren't actively moving food forward, and so if that's happening then you can't eat yet.

Interviewer: And that rumbling is that what's happening?

Dr. Jeff Campsen: That's your intestines basically waking back up. Basically being comfortable that no one's going to go in and touch them again and operate on them again.

It's interesting there's actually two things that help your intestines wake up and they've been proven. One is getting up out of bed and walking because that normalcy and the pumping of blood from the walking through the body helps, and then there is a good study out of the Mayo Clinic that said chewing bubble gum actually helps intestines come back quicker, and it's probably because you're not really creating any food but you're giving that sort of reflex of tasting something in your mouth and stimulating your stomach and your lower intestines to think that they need to be working, and so chewing gum and walking those two things are probably the best advice that you can get to moving along.

Interviewer: Got you, alright so what types of foods do you want to start eating at that point?

Dr. Jeff Campsen: So at that point when we start hearing bowel sounds, and we actually ask our patients, you know, "Are you passing gas?" This is a difficult question for some people because no one wants to admit that, but in this particular case that's a good thing and we want to hear that. What we don't want to hear from you is that you're burping. Burping's bad, that means that stuff's not going through and it's backing up in your stomach's getting distended. The reason that that's concerning is if your stomach gets too distended it can cause you to vomit, and if you vomit too much and you've just had surgery, and you're mildly sedated you can actually breathe that vomit in which is called aspiration, and it can give you a very bad pneumonia.

Interviewer: Does that have any chance at that point of rupturing those internal sutures?

Dr. Jeff Campsen: I think that it can. Your abdominal wall is going to spasm to do those violent vomiting episodes, and you probably can damage the repairs that we've made on you, and so we'd prefer that not to happen.

Interviewer: So at this point are you still under the care of a physician and a team?

Dr. Jeff Campsen: Yes.

Interviewer: Up until this point.

Dr. Jeff Campsen: Right, so you've come out of the operating room, we're in the first sort of 24 hours, 24, 72 hours, are you starting...Are you not burping, is your intestines making the rumbling sounds, and plus or minus are you passing some gas? At that point what we would start you on is basically some clear liquids. If you can see through it it's not thick, not milk which is very thick, we start you on that. If you can tolerate clear liquids then we, what we call it is advanced diet is tolerated, then you may go onto something soft, it's called a soft mechanical diet, that's not very spicy, not hot, not all these kind of things that may upset your stomach, that's very bland. Bread and sort of soup those kind of things and if you handle that then you can move along to what you would want to eat.

Interviewer: Alright and when you get discharged and you go home what should you be thinking about in your diet at that point?

Dr. Jeff Campsen: Well I think at that point you can basically go back and eat whatever you would normally want to eat. I do think it's an opportunity to really reevaluate what you are eating and basically try to eat healthy because if you've had inter-abdominal surgery you need the building blocks for healing, protein, sugars, different things that go into along your abdominal wall to heal, and if you're eating unhealthy during that time you're not going to give your body the kind of tools and building blocks that it's going to need to heal properly. So you want to look at your diet and try to eat as healthy as possible during that time. Now if you can parlay that into lifelong better dietary habits then that's great. You might as well; you've gone through the surgery you might as well benefit from it on that angle too.

Interviewer: Got you, so it sounds like if you ate healthy beforehand just continue your normal diet afterwards. If perhaps you didn't have the best diet beforehand that be a good time to reevaluate and maybe make some lifestyle changes.

Dr. Jeff Campsen: I think so; I think it's extremely important during the first month to eat healthy because again you have to have the right components in your diet to allow your body to then create a new abdominal wall, for lack of a better word. To heal that area that was, where the incision was made, and if you don't give your body that then you're not going to heal well, and then if you get an infection, a complication, a hernia it could be a result of that.

Interviewer: And what does it mean to eat well? I mean do you have to go all superstar diet, super clean foods, or...?

Dr. Jeff Campsen: No I don't think so. I think it comes down to you want to have a moderate amount of calories. So you want to look and see how tall you are and based on that how many calories you should have a day and try to stick within that, and then on top on that it needs to be balanced. You need to have the building blocks of protein and carbohydrates, but all sugars and no protein, and I think based on that and who you are going into the surgery your dietary requirements fluctuate a little bit, but again it's the basic food groups that go into it, and then moderation.

Interviewer: The stuff we've learned as kids.

Dr. Jeff Campsen: That's exactly right.

Interviewer: The stuff we all know but ignore when we go to the, you know, the fast food restaurants.

Dr. Jeff Campsen: Right. No I think the biggest thing is that everybody's intestines wake up at their own speed. It's whoever your body actually is at the time coupled with the type of surgery you have, and everybody's intestines will wake up, but if you move too quickly you can hurt yourself.

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updated: September 25, 2018
originally published: June 12, 2014