Symptoms, Screening, Management of Exocrine Pancreatic Insufficiency
In this podcast, Elissa Downs, MD, MPH, speaks about exocrine pancreatic insufficiency (EPI), including the symptoms, screening tools used to diagnose patients with EPI, how patients with EPI should be managed, and gaps in the research of EPI.
Elissa Downs, MD, MPH, is a pediatric gastroenterologist at the University of Minnesota Masonic Children’s Hospital.
For more information on exocrine pancreatic insufficiency, visit the Resource Center.
TRANSCRIPTION:
Jessica Bard: Hello everyone and welcome to another installment of Podcast 360, your go-to resource for medical news and clinical updates. I'm your moderator, Jessica Bard, with Consultant 360, a multidisciplinary medical information network. Chronic pancreatitis is the main cause of exocrine pancreatic insufficiency, or EPI, in adults. As many as 8 in 10 adults with chronic pancreatitis will develop EPI. As for children and infants, cystic fibrosis is the main cause of EPI and nearly 9 and 10 infants with cystic fibrosis will develop EPI in the first year according to the Cleveland Clinic.
Dr Elissa Downs is here to speak with us today about the symptoms, screening tools used to diagnose patients with EPI, how patients with EPI should be managed, and the gaps in the research of EPI. Dr Downs is a pediatric gastroenterologist at the University of Minnesota Masonic Children's Hospital. Thank you for joining us today, Dr Downs. EPI is caused when the pancreas becomes damaged and can't produce the proper enzymes to digest food. Can you elaborate for us on the causes of EPI?
Dr Elissa Downs: Yeah, I think it's a great question to sort of think about, And it's one that we're certainly learning more and more about all the time. To take a step back, we know that the pancreas itself has two main functions, including kind of your endocrine management or management of blood sugars, and then your exocrine management or your management of digestion. And when that gets impaired, either through processes in adults like from chronic pancreatitis or in children, the top cause is actually cystic fibrosis, and we don't put out enough digestive enzymes to help us with absorption, that's when we give people a diagnosis of EPI.
Jessica Bard: So talk to us a little bit about the symptoms of EPI.
Dr Elissa Downs: Symptoms can range from more on the mild side, where you can just sort of see bloating, abdominal distension, and gassiness that can be confused for many other disorders. Versus symptoms that are more severe where you're now having more severe abdominal pain, where you're having nutritional deficiencies and the complications of those nutritional deficiencies. And then ultimately steatorrhea, or kind of greasy, bulky, foul, smelling poops that are hard to flush down the toilet.
Jessica Bard: Let's talk about screening tools now. What are screening tools that are used to diagnose patients with EPI and what's the gold standard, limitations, that sort of thing?
Dr Elissa Downs: We kind of break our screening tools into two major categories. We think of ones that are directly testing the pancreas and then ones that are more indirectly or measuring the pancreas. So sort of proxy measurements of the pancreas. Now we love our direct tools if we can use them, but most of them involve doing some kind of stimulation of the pancreas with IV hormones to put out some pancreatic product. And then kind of look at levels of both fluid output of pancreas and kind of bicarbonate amounts in the fluids. They're a little bit more time-consuming. Even the newer methods where we can do this endoscopically, they still require general anesthesia, and they still require probably at least an hour of collection of the fluid of the pancreas and kind of measuring it for it's bicarbonate levels. So those direct tests are not performed all that often, only when we're maybe a little bit questioning of the diagnosis. Because we don't always necessarily want to do general anesthesia and it's not always available at any medical centers.
So sometimes more specialized medical centers are the ones that are going to be able to do this. So we rely a lot on kind of the indirect testing of the pancreas. And that can be through different blood tests, but more often actually different poop tests. So one of the kind of main gold standard of poop tests that we can do is actually a 72-hour fecal fat collection, which sounds as obnoxious as it could be. But 72 hours of collecting all of your fat content in those stools and then contrasting that against the fat content of your diet over those three days. So you can kind of hear that it's sort of onerous and not something that we usually have people do. So the kind of other best assessments of stool tests is one called a fecal elastase, which measures elastase, which is an enzyme that we can find in the poop. And we use that as sort of a proxy measurement of how well the pancreas is doing with its function. It is an easy test to do relatively and it's a little more cost-effective than some of our other options, but it still has some ranges in terms of how sensitive and specific it can be. So it's a test that you can sometimes see be falsely negative, especially if the stool is diluted with diarrhea for other reasons. But it tends to be more sensitive and specific once people are probably pretty far into their EPI. So for more severe EPI it is probably the most common test though that does get used.
Jessica Bard: You mentioned newer methods of screening tools. Could you kind of talk to us about the newer versus the older methods?
Dr Elissa Downs: Yeah. So some of the older methods, or kind of more dated procedures, involve different collection tubes for pancreatic fluid after simulation. And so now some of the newer methods are kind of what we call endoscopic pancreatic function tests, or EPFTs, that are just using, one, your endoscope tube down into the duodenum and then collecting the pancreatic fluid over about an hour. There are some possibly kind of newer methods that people are using to assess pancreatic function, including some sort of stimulated MRI scans. Again, that's probably something that you're only going to really get at specialized centers and things that we'll probably need to develop protocols and guidelines for to develop those.
Jessica Bard: Got it. Moving on to management now, how should a patient with EPI be managed?
Dr Elissa Downs: In general, I feel like the management is pretty straightforward. So once we've established the diagnosis of EPI, we really want to make sure that people are getting pancreatic enzyme replacement therapy, or PERT. And so this is going to be something that's prescribed by a physician. And we don't necessarily want people using enzymes from health food stores because these are not FDA regulated and they could certainly come with a variety of other things in there that we're not intending for patients to have. So we don't have great evidence that those are effective and they're not going to necessarily be helpful or certainly sometimes strong enough for these types of conditions. So there are a few FDA-approved formulations of these products. It'll be very important to take them with every meal and every snack lifelong, which is crucial. So if you do miss a dose of enzymes and you had a meal or you had a snack, you'll have those same symptoms, again, of gassiness and bloating or pain or diarrhea.
So taking the enzymes are incredibly important. Those enzymes contain amylase, lipase and protease to help us break down fats, carbohydrates, and proteins in the foods that we're eating. But we also want to make sure that people are taking specially formulated pancreatic multivitamins, which are going to contain fat-soluble vitamins, our vitamins A, D, E, and K because those will be at the most risk of being mal absorbed if we're not taking our enzymes or if we're not in a high enough dose of enzymes. The other things we just kind of recommend are kind of what we lump into that lifestyle changes category. So making sure people are eating healthy, making sure they're not smoking if that's what they've been doing, because smoking is one of those main factors in pancreatitis. Making sure they're not drinking, or heavy drinking. Because again, that's been implicated as a co-factor in chronic pancreatitis. And then staying active if they can, staying hydrated if they can, those types of things. And then the last piece I guess I'll comment on for that question is just kind of management of long-term complications. So some of them are just going to be screening for nutritional deficiencies at regular intervals. Supplementing with extra vitamins if we have deficiencies to help prevent bone disease and coagulopathies and eye problems, things like that. But then also monitoring for potential other issues of the pancreas itself.
So with both endocrine and exocrine function of the pancreas, they can decline separately. And so it's not that automatically if one declines, the other will decline as well. So long-term monitoring for the development of diabetes is going to be really important. But then also the management of whatever underlying condition caused your EPI in the first place. So if it is something like cystic fibrosis, doing our comprehensive multidisciplinary care for the management of CF. If it's chronic pancreatitis, are we enrolled in a good pain program? Are we doing physical therapy? Are we seeing psychology? Do we need to be thinking about genetic testing, that type of thing, just to really make sure we understand what's causing the EPI in the first place and how to lifelong monitor complications of that.
Jessica Bard: And that's a good segue. You mentioned both chronic pancreatitis and cystic fibrosis. How would the management, or would the management of EPI be different from the management of those other two?
Dr Elissa Downs: Yeah, it's a great question. We probably don't necessarily differentiate between the EPI caused by CF or the EPI caused by chronic pancreatitis. We're going to highlight management in the same sorts of ways in terms of making sure people are on enzymes and vitamins and eating a healthy diet. The differences probably come in the management of those diseases from a multisystem-based perspective.
Jessica Bard: What would you say are the gaps in the research of EPI and what's next for research?
Dr Elissa Downs: I feel like some of the gaps in the research are just our knowledge of how common this is. As I was preparing for our talk together and doing some literature review, there were some very wide estimates of the prevalence of EPI. And maybe it's not something we'll really ever know because of the myriad of factors that can contribute to it. But that, I think, is something important to think of down the road. Other gaps kind of include how we're treating EPI. Our enzyme products right now that we have, they're all capsule-based formulations, but they're all porcine-based. The one difference would be an enzyme cartridge that we have that we can hook up in line with enteral feeds, which only contains a lipase product in it. So you're missing amylase and protease enzymes in that product.
But despite that, it seems to work quite well. But you know, not the whole population of people with either a CF or with chronic pancreatitis are going to be on tube feeds and could potentially use that product. And it's also only applicable for people that are getting more continuous tube feeds, not that are getting kind of a bolus of feeds here and there throughout the day. So those are some of the things I'd like to see the pancreas community work on down the road, can we highlight an enzyme product that's not porcine-based? Can we get a better sense of who suffers from EPI and how to help them the best?
Jessica Bard: What would you say are the overall take-home messages from our conversation today?
Dr Elissa Downs: Yeah, I think one is that EPI is probably more common than we think, especially as we start to think of the factors beyond chronic pancreatitis or beyond CF that can cause EPI. So if that's people with diabetes, people with inflammatory bowel disease, people that have had gastrointestinal surgeries or other surgeries on their pancreas. And then too, also sort of thinking that the symptoms of EPI can be kind of nonspecific. So there are certainly other people that have gassiness, bloating and diarrhea after eating because they are lactose intolerance or maybe they have celiac disease. These same types of symptoms can be present in bacterial overgrowth. Or people with inflammatory bowel disease can have chronic diarrhea, nutritional deficiencies that can overlap with those in EPI. So how do we as physicians keep a broad differential and include EPI in that differential when we're seeing people with either nutritional deficiency or poor weight gain or chronic diarrhea, and then think about the available testing methods that we have.
Well, thank you so much Dr Downs. Is there anything else that you'd like to add today?
Dr Elissa Downs: No, but thank you so much for allowing me to speak with you today.
Jessica Bard: Thank you for being on the podcast