Pancreatic insufficiency is more common than you think, and many patients aren’t getting relief. Here’s how to make sure enzyme replacement therapy works

By the time the average patient with pancreatic exocrine insufficiency sees Advanced Accredited Practicing Dietitian Lauren Atkins of Oncore Nutrition, they’re in a fairly desperate state.

“It’s universally underdiagnosed and undertreated,” Atkins says.

Pancreatic exocrine insufficiency occurs when the enzymes secreted in response to a meal are insufficient for maintaining normal digestive function.

“It will only be clinically evident when 90% of pancreatic function is lost,” she says. “This means it can present months post a Whipple or partial pancreatectomy, or even years post a pancreatitis.”

Pancreatic insufficiency is a given in total pancreatectomy or in most patients with cystic fibrosis, but Atkins says cases presenting some time after pancreatic insult often fly under the radar.

By the time symptoms present, the patient is usually no longer engaged with the treating team, so the GP will most likely be the first professional they tell, Atkins says.

Conditions that predispose to Pancreatic Exocrine Insufficiency

• Total Pancreatectomy
• Pancreatic Cancer (both unresectable and post-surgery)
• Cystic fibrosis
• Chronic pancreatitis
• Gastric surgery include Whipple procedure and bariatric surgeries
• Acute pancreatitis

Subclinical pancreatic exocrine insufficiency is characterised by a range of malabsorptive gastrointestinal systems that will often be brushed off by both clinicians and patients as due to something else: side effects of chemotherapy or radiotherapy, or “just” irritable bowel syndrome, for example.

Allowed to continue, malabsorption will lead to malnourishment. Patients will lose weight, have a variety of micronutrient deficiencies, and even present with bone fractures.

Atkins recommends considering this as a differential diagnosis in patients with gastrointestinal symptoms (pain, flatulence, bloating, diarrhoea) with any history of pancreatitis or pancreatic surgery.

Signs & Symptoms of Pancreatic Exocrine Insufficiency

• Steatorrhoea
• Abdominal pain/discomfort
• Bloating/increased flatulence
• Weight loss
• Fat-soluble vitamin deficiencies (A, E, D, K)
• Other nutritional deficiencies (iron, folic acid, magnesium, calcium, zinc)
• Osteoporosis/fractures
• Sarcopaenia
• Cardiovascular events
• Fatigue and weakness

“The big clue on history is steatorrhoea, as opposed to just diarrhoea,” she says. Due to the malabsorption of fats, bowel motions will be pale, frothy and buoyant.

Choice of diagnostic tests will depend on the individual patient, Atkins says. “There are gold standard tests, but these tend to be expensive, invasive and hard to access.” These include MRI and endoscopy, along with pancreatic stimulation tests and 3-day faecal fat analysis.

The Australian guidelines recommend at least a CT scan to look at pancreatic structure.

A simple diagnostic strategy used by Atkins and her colleagues is to order Vitamin E and faecal elastase. Faecal elastase alone has a high rate of false positive results, but Atkins says that if both results are decreased, this proves malabsorption is occurring. “Don’t rely on serum lipase or amylase,” she says. “They are not always abnormal.”

If clinical suspicion is high, Atkins recommends a trial of treatment even whilst awaiting test results. If dosed appropriately, she says patients will notice the difference almost immediately.

Pancreatic enzyme replacement therapy is quite simply capsules of enzyme, which need to be dosed immediately before all oral intake in order to work.

“Therapy often seems to fail because it is underdosed, or taken too early or too late with respect to the meal,” Atkins says. She recommends referral to a specialised dietitian for patient education.

Tips for Pancreatic Enzyme Replacement Therapy

• First mouthful, first creon – capsules must be taken at exactly the start of the meal, snack or drink.
• Do not restrict fat intake – just increase the dose for higher fat content foods
• Second dose needed if the meal lasts longer than 20 minutes
• Needed for ALL food and drink that contains fat or protein
• Not needed for fruit, juices, jelly, lollies, cordial, black tea/coffee, herbal tea and soft drinks
• Enteric-coated – do not chew
• If ineffective or patient has gastroparesis, trial acid suppression with a PPI

Therapy is very well tolerated with almost no side effects, says Atkins, although the enzyme is porcine-derived. “There’s not a good alternative for people with true pork allergy” or with religious or cultural inability to use it, she says. In these rare cases, patients will require semi-elemental formula under dietitian supervision in order to prevent malnourishment and its consequences.

It will be clinically obvious that therapy is working because the patient will notice a symptomatic change. Steatorrhoea, stool frequency and consistency, and abdominal discomfort will improve significantly, if not normalise altogether, Atkins says. She nevertheless recommends annual blood tests for biochemistry and fat-soluble vitamins, and bone mineral density every two years, to demonstrate that nutritional needs are indeed being met.

For more information:

• Australasian guidelines for the management of pancreatic exocrine insufficiency | Australasian Pancreatic Club
• Pancreatic enzyme replacement therapy (PERT) | QLD Health

 
Recommended resources:

• Pancreatic Disease and Insufficiency Explained