A diagnosis of familial (hereditary) pancreatitis is likely if there are two or more family members with pancreatitis in more than one generation. This unusual form of chronic pancreatitis can begin at a comparatively young age – before age 30 – and may present itself by prolonged periods of abdominal pain and diarrhea.
Familial pancreatitis typically involves the development of stones and obstruction of the main pancreatic duct, which can cause the digestive enzymes produced in the pancreas to attack the pancreas itself.
It may be possible to treat familial pancreatitis with procedures that remove stones (EHL) and improve drainage of digestive juices from the pancreas to the small intestine (Puestow and Frey procedures). Yet familial pancreatitis may be best managed over the long term by removal of the pancreas (pancreatectomy) when the patient's own islet cells can be transplanted from the pancreas to the liver to help the body continue to produce hormones regulating blood sugar (islet autotransplantation.)