As we discussed in a previous blog, pancreatitis is a health condition where the pancreas becomes inflamed. Hereditary pancreatitis, as the name implies, is a genetic condition characterized by recurrent bouts of pancreatitis. Pancreatitis can be brought upon by a poor diet or heavy alcohol consumption, but individuals with hereditary pancreatitis develop the condition because of a gene mutation. Today, we take a closer look at how hereditary pancreatitis develops, and how it is treated compared to other forms of pancreatitis.

Hereditary Pancreatitis – It’s In Your Genes

Hereditary pancreatitis is a serious condition, but thankfully it’s a pretty rare condition. Estimates suggest that only about 4-10 people in a million will develop the genetic condition. Additionally, while it can be passed down from one parent, because the condition is categorized by a genetic mutation, you can develop the condition even if your parents don’t have the same mutation so long as gene mutation occurs during early stages of development.

Scientists have narrowed the condition down to the mutation of a specific gene, but it can also be caused by other non-specified genetic issues. According to researchers, about 65 to 80 percent of people who develop hereditary pancreatitis have a mutation of their PRSS1 gene, which is the gene responsible for the production of trypsinogen, an enzyme that helps digest food. If this enzyme is not effectively controlled by the body, it can damage pancreatic tissue and cause inflammation and pancreatitis. Lastly, medical experts noted that about 1 in 5 people with the PRSS1 mutation will never have an episode of pancreatitis, but it’s unclear why symptoms don’t appear.

Symptoms and Treatment

Hereditary pancreatitis typically begins to show symptoms in late childhood in the form of acute pancreatitis. Acute pancreatitis is characterized by episodic bouts of abdominal pain, fever and nausea, typically lasting 1-3 days. As the child ages, episodes become more frequent, and acute pancreatitis can develop into chronic pancreatitis. When this occurs, the pancreas remains in a persistently inflamed state. If left untreated, a person with chronic pancreatitis can develop calcium deposits in their pancreas, scar tissue and loss of pancreatic functions. Insulin production is also affected, meaning roughly 1 in 4 people with hereditary pancreatitis will develop type 1 diabetes by mid-adulthood.

Unfortunately, there is no cure for genetic disease, but there are treatment options to help control symptoms and prevent further damage. Some treatment options for hereditary pancreatitis include:

• Healthy Diet Habits – Making smart food and beverage choices, as well as limiting alcohol and tobacco intake, is key.
• Enzyme Treatment – Medications can help ensure certain enzymes don’t harm the pancreas.
• Surgery – A pancreatectomy, also known as the removal of the pancreas, can eliminate the risk of pancreatic cancer, but you’ll need additional care to ensure your body can function properly without a pancreas.

For more information about the condition, or to discuss your symptoms with a specialist, contact Dr. Koeplin for a consultation.