Hereditary pancreatitis is a genetic condition characterized by recurrent episodes of inflammation of the pancreas (pancreatitis). The pancreas produces enzymes that help digest food, and it also produces insulin, a hormone that controls blood sugar levels in the body. Episodes of pancreatitis can lead to permanent tissue damage and loss of pancreatic function.
Signs and symptoms of this condition usually begin in late childhood with an episode of acute pancreatitis. A sudden (acute) attack can cause abdominal pain, fever, nausea, or vomiting. An episode typically lasts from one to three days, although some people may experience severe episodes that last longer. Hereditary pancreatitis progresses to recurrent acute pancreatitis with multiple episodes of acute pancreatitis that recur over a period of at least a year; the number of episodes a person experiences varies. Recurrent acute pancreatitis leads to chronic pancreatitis, which occurs when the pancreas is persistently inflamed. Chronic pancreatitis usually develops by early adulthood in affected individuals. Signs and symptoms of chronic pancreatitis include occasional or frequent abdominal pain of varying severity, flatulence, and bloating. Many individuals with hereditary pancreatitis also develop abnormal calcium deposits in the pancreas (pancreatic calcifications) by early adulthood.
Treatment for Hereditary Pancreatitis
Treatment typically includes measures geared at controlling pain and ensuring optimal nutrition. Frequently, pancreatic enzyme replacement is indicated. Our pain medicine specialists can offer a wide range of pain management solutions to curb the pain associated with pancreatitis.
Hereditary pancreatitis has been linked to an increased risk for pancreatic cancer. Therefore, it’s important to talk with your physician about this risk and any screening measures for you or your family that may be appropriate to check for signs of pancreatic cancer.
Hereditary pancreatitis is a genetic disorder, which means that it runs in families. Most people with hereditary pancreatitis have a mutation to the cationic tryspinogen gene, also called PRSS1. There are a few different types of known mutations of this gene that are linked to hereditary pancreatitis. However, because some families with a strong history of pancreatitis may not carry any of the known mutations, scientists believe that other genes and mutations may also be linked to familial pancreatitis.
Not every family member who has the mutation will develop the disease. Some people who have the mutation and pancreatitis are at a higher risk of developing pancreatic cancer and, therefore, should consult a pancreatic specialist for assessment.