ACUTE PANCREATITIS | CHRONIC PANCREATITIS | FAQ | INHERITED DISORDERS | PATIENT STORY | RESOURCES

Overview

Pancreatitis is an inflammation of the pancreas, an organ located behind the stomach. Although relatively rare, pancreatitis in children is more common than was previously thought, and it can progress in a surprisingly short timeframe.

The pancreas performs many important functions, including the secretion of insulin and other key hormones, as well as production of a fluid containing precursor forms of enzymes and bicarbonate that flows through ducts into the intestine, where the enzymes become activated and aid digestion of food. In pancreatitis, the digestive enzymes become activated too early—while still inside the pancreas. This causes inflammation and damage to the organ, leading to the symptoms of pancreatitis— the main one being abdominal pain that is often severe, as well as nausea and vomiting.

The disease can occur in three forms: acute, acute recurrent (two or more acute episodes), and chronic. Acute pancreatitis can progress to the chronic form, which carries with it an increased risk of pancreatic cancer. Once considered an uncommon disease in children, the incidence of pediatric acute pancreatitis has increased over the last 10 to 20 years and currently affects approximately 1 in 10,000 children. Chronic pediatric pancreatitis, in which children have diagnostic or functional evidence of irreversible pancreatic damage, is estimated to have an incidence of approximately 2 per 100,000 children per year.

Both acute recurrent and chronic forms of pediatric pancreatitis place a significant burden on children and their caregivers. Risk factors for pancreatitis differ for children compared to adults. In children, the main risk factors are inherited genetic variants, followed next by obstructed ducts caused by congenital abnormalities or gallstones. In adults, risk factors include genetics and gallstones, but environmental factors such as alcohol and tobacco use are the most predominant. Although diabetes is another risk factor for pancreatitis, it is not as common in children as adults. An insufficient production of pancreatic enzymes is found in both children and adults with pancreatitis.

Emergency room visits and hospitalizations are common in children and adults with the disease, as are missed days of school or work. Pain, whether it comes in discrete episodes or is constant, is frequently difficult to treat, leading to lost school time for children and increased utilization of health care and high medical costs. There are currently no drugs that effectively halt the progression of this potentially debilitating disease or that reverse the disease process.
Treatment options to manage the severe, often unremitting pain typically accompanying chronic pancreatitis include opioids, which carry the risk of addiction. If traditional pain management fails, a child with pancreatitis may need a surgical palled a total pancreatectomy-islet autotransplantation (TP-IAT), in which the pancreas is surgically removed and its insulin-producing islet cells, which regulate blood glucose (sugar), are collected and infused into the liver, where the cells implant and function.

Acute Pancreatitis in Children

Acute pancreatitis is irritation of the pancreas. Your child’s pancreas is an organ located in the upper abdomen that is important for digestion and blood sugar regulation.

Pancreatitis occurs when the pancreas sustains an injury and becomes inflamed. There are a variety of different reasons that the pancreas can become injured. This initial event triggers inflammation. As the inflammation proceeds, further injury to the pancreas occurs. The extent of this damage determines the severity of acute pancreatitis. The damage and irritation to the pancreas typically resolves over time. Thus, acute pancreatitis is a reversible inflammation of the pancreas.

Helping Kids and Teens Understand Acute Pancreatitis

Chronic Pancreatitis in Children

Chronic pancreatitis describes a condition in which your child’s pancreas has been progressively irritated and becomes irreversibly damaged. This damage causes scarring in the pancreas and leads to the loss of a portion of digestive function. It may cause diabetes, as well.

Helping Kids and Teens Understand Chronic Pancreatitis

Pediatric Pancreatitis FAQ

Common symptoms include abdominal pain, nausea, and vomiting. However, not every patient with pancreatitis will have all of these symptoms. Pancreatitis symptoms are nonspecific and can easily be confused with signs of another disease. They also vary depending on your child’s age and developmental level; for instance, non-verbal infants may present with increased crying.

There is no single test to diagnose pancreatitis. The diagnosis is clinical and depends on the presence of symptoms consistent with acute pancreatitis, abnormal blood tests, or radiographic images showing inflammation in the pancreas. A diagnosis of acute pancreatitis can be made if two or more of these criteria are fulfilled.

Amylase and lipase are the most commonly measured blood tests. Both are enzymes which are made by the pancreas to aid the digestion of foods. When the pancreas is injured or inflamed, the blood levels of both amylase and lipase can rise above normal. Since other conditions can also raise these enzyme levels, they are not specific to pancreatitis. Thus, in the absence of concerning symptoms or radiologic signs of pancreatitis, an elevation in either the amylase or the lipase level does not necessarily lead to a diagnosis of pancreatitis.

Ultrasound and CT scans are the most common imaging modalities that can be used to look for pancreatic irritation. Importantly, the pancreas can sometimes appear normal on these scans during an episode of pancreatitis.

The treatment of pancreatitis is supportive care. There is no single medication or treatment that will help the pancreas to recover. Patients who have abdominal pain can be treated with pain medications. Some patients do well with acetaminophen (Tylenol), while others may require stronger, narcotic pain medicines. Nausea and vomiting are treated with anti-nausea medications.

If a patient can’t tolerate eating by mouth because of pain, nausea, or vomiting, he or she will not be allowed to eat and will be hydrated with intravenous (IV)fluids.

Your child can start eating when he or she feels hungry and is ready to eat. Any number of initial diets may be offered, ranging from clear liquids to regular food. The choice depends on how ill your child is and the preference of the doctors caring for your child. Most children feel well enough to start eating within the first day or two after an episode of acute pancreatitis. Occasionally, their symptoms may be more severe or persist for a longer period of time. If this is the case, your child may be given nutrition through a feeding tube or an IV to prevent malnutrition and improve healing.

Not necessarily. If your child can tolerate eating enough food or drinking enough liquids to stay hydrated, and pain can be controlled with oral medications, he or she might not need to stay in a hospital for treatment. In the past, diagnosing pancreatitis in children was a lot harder. Many children who were actually having acute pancreatitis were likely diagnosed with a stomach virus and kept home without a problem.

On average, symptoms of pancreatitis will last about a week. This does not mean your child will be in the hospital for that whole time period since symptoms do improve over time. A few patients have more severe diseases and may end up staying in the hospital for a month or more.

The complications of acute pancreatitis depend on the severity of the pancreatic irritation. The most common complication is the collection of fluid around the pancreas. These collections can be small or large enough to cause abdominal distension. Generally, the fluid will go away with time. If the pockets of fluid cause symptoms, doctors may recommend draining the fluid. The symptoms of fluid collections include vomiting from blockage of the stomach or part of the small intestine and fever due to infection. Rarely, the fluid collection causes bleeding into the abdomen or intestines.

Death from acute pancreatitis is quite rare in children–but it can happen. Most deaths associated with pancreatitis occur in children who have a significant illness that damages multiple organs.

Yes, it can. About 10% of children will experience another bout of acute pancreatitis. Even fewer will have multiple episodes. If your child has another episode, his or her doctor will do additional testing to search for known causes of recurrent acute pancreatitis.

There are some treatable causes of acute pancreatitis. These include gallstone disease, high blood calcium, high blood triglycerides, and abnormalities of the bile ducts that come from the liver, or the ducts within the pancreas. Treatment of these disorders can help prevent future episodes. Unfortunately, for most patients, there is no way to prevent pancreatitis.

Frequent or chronic abdominal pain is the most common symptom of pancreatitis. The pain can be constant or can come and go unpredictably. Other symptoms include nausea, vomiting, weight loss, diarrhea and oily bowel movements. Some patients have trouble digesting food and may experience poor growth, especially if they are quite young when their first episode of pancreatitis occurs. Diabetes generally takes many years to appear, but this, too, is highly variable; some patients with chronic pancreatitis will develop diabetes in adolescence.

Those at risk for chronic pancreatitis are children who have genetic, metabolic or anatomic abnormalities that predispose them to having multiple episodes of acute pancreatitis. Not all patients with a genetic predisposition or anatomic abnormalities will develop chronic pancreatitis. The reason for the variability is that modifying factors act in concert with the primary predisposition to increase an individual’s risk of developing chronic pancreatitis. Examples of modifying factors include other genes or environmental variables, which is a term that scientists use to describe things that your child is exposed to in his or her daily life or certain behaviours. Researchers are still working on achieving a better understanding of the way in which these factors interact to cause the disease.

Unfortunately, chronic pancreatitis is a life-long condition, although the severity of symptoms tends to wax and wane over time.

The diagnosis of chronic pancreatitis requires the demonstration of irreversible damage to the pancreas, loss of digestive function or of diabetes. Currently, the damage is assessed by radiographic studies such as CT or MRI scans of the pancreas, by special endoscopic procedures known as ERCP, or by endoscopic ultrasound.

If there is an anatomic abnormality, then surgery, often performed through an endoscope, can be curative. Metabolic abnormalities such as high serum calcium or serum fat (triglycerides) can also be treated.

Currently, there are no effective medical treatments for patients with a genetic predisposition. Some patients are candidates for surgery. In this procedure, the surgeon removes the pancreas and the hormone-producing cells known as ‘islets’ are isolated and returned to the patient, usually by injecting them into the liver. This surgical procedure is called ‘pancreatectomy with islet cell auto transplant.’

Many physicians will prescribe pills containing pancreatic digestive enzymes to patients who experience chronic pain.

There is no clear evidence that a special diet is required for chronic pancreatitis. Still, many physicians will prescribe a low-fat diet, and advise their patients to eat more frequent, smaller meals, typically with fewer than 10 grams of fat. About 20 potato chips contain 10 grams of fat, so it takes discipline to make sure to stay within this range. Patients who have lost the ability to digest food will be prescribed pills containing pancreatic enzymes to help with digestion. They may also be prescribed fat-soluble vitamins A, D, E, and K, since the difficulty absorbing fat also interferes with patients’ ability to absorb these vitamins, which are crucial for maintaining good health.

If your child has chronic pancreatitis, he or she will be at an increased risk of developing pancreatic cancer compared to the general population. The degree of risk depends on the underlying cause of pancreatitis and should be discussed with your doctor.

Childhood Inherited Pancreatic Disorders

About Pancreatic Disorders

There is an extremely wide spectrum of inherited pancreatic disorders in childhood. Depending on the condition symptoms can develop at any time from birth to adulthood. The pancreas makes more than 25 different digestive enzymes which are secreted into the intestine to break down dietary protein, fat and starches into simpler molecules so that they can be absorbed. In fact, the pancreas has a tremendous reserve capacity – more than 95% of the function of the pancreas must be lost before the pancreas fails and symptoms of bloating and maldigestion develop. Children with digestive problems due to failure of the pancreas have to take enzyme replacement therapy with meals as well as additional fat soluble vitamins. The large pancreatic reserve also means that children can have a severe pancreatic problems without experiencing any problems with digestion.

The exocrine pancreas is not fully developed at birth. In fact, all healthy infants show some degree of maldigestion due to the fact that the pancreas is immature and does not have the same ability to produce enough enzymes. This is particularly true for starch and fat digestion. However, the pancreas matures after birth and by two years of age, it is functioning in the same way as an adult pancreas. The immature pancreas appears to have no adverse effects on healthy children but can have a major impact when children become malnourished or very ill.

Cystic Fibrosis (CF)

Cystic fibrosis (CF) is, by far, the most common inherited pancreatic disease of childhood. It accounts for about 90% of childhood-onset pancreatic disorders. CF affects many other organs as well and the most common cause of poor health and death is due to progressive lung disease. The CF pancreas begins to get damaged when the affected child is still in the mother’s womb. The small tubes inside the pancreas which allow digestive enzymes to reach the intestine get blocked with mucus and protein and the pancreas became badly scarred and shrinks. Many children with CF have evidence of severe pancreatic failure immediately following birth, and by two years of age 90% of CF are diagnosed – usually with severe malnutrition. Approximately 85% of all people with CF have pancreatic insufficiency and need to take pancreatic enzymes with meals. A lot is known about the genetic cause of CF. The CF gene, which is on chromosome 7, was identified in 1989. The most common CF-disease causing genetic mistake in this gene is called DF508, and is identified in approximately 70% of CF chromosomes worldwide. However, there are more than 1000 additional genetic mistakes in the CF gene, many of which are extremely rare. A lot of research is being done to try to correct the genetic disorder with gene therapy and to discover ways of getting around the genetic disorder using special drugs.

Shwachman Diamond Syndrome (SDS)

Shwachman Diamond syndrome (SDS) is the next most common inherited cause of pancreatic failure in childhood. It is much less common than CF and accounts for about 5% of inherited causes of pancreatic disease. This condition also affects other organs including the bone marrow (which makes blood cells), the skeleton and the liver. Children with SDS are very short and are at risk of experiencing severe infections and a particularly severe form of leukemia. Unlike CF, the gene that is responsible for SDS has not yet been identified. However, recent research shows that the SDS gene is also located on chromosome 7. The pancreatic problem is quite different from CF. The cells that make enzymes (acinar cells) don’t develop properly.

Other Inherited Pancreatic Diseases

After CF and SDS, other causes of inherited pancreatic disease are extremely rare. They include Johansson Blizzard syndrome, Pearson’s bone marrow syndrome and hereditary pancreatitis. It is interesting to note that the hereditary pancreas gene is on chromosome 7 as well. This is a coincidence!

In extremely unusual circumstances a child may be born without any pancreas – which includes both the digestive (exocrine) and insulin-producing (endocrine) components of the pancreas.

Patient Story

Ezra’s Story

Ezra was 9 years old when he had his first pancreatitis attack. In this story, his parents recount his journey with pancreatitis, how they learned to advocate for their son and provide advice for caregivers going through the same situation. It happened suddenly. Very early one morning, I received a phone call from Ezra’s friend’s mom. Ezra had been sleeping over at their house and had started throwing up. I hurried to pick up my son. His symptoms had not improved as he continued to hold his stomach and throw up all the way home.

Resources

Online Support

The INSPIRE Pancreatitis Support Community offers a place where pancreatitis patients, their family and caregivers can ask questions and share support and information with each other.

Get support when you need it. ANYTIME. ANYWHERE.

Visit INSPIRE Online Pancreatitis Support to receive support.

Education Events

The National Pancreas Foundation education events are posted on our YouTube channel. Visit our channel to find pediatric pancreatitis talks by industry leading doctors to help you learn more about this disease.

Pediatric TPIAT Education Series

Empowering Families and Young Patients with Pancreatitis. 

In partnership with The National Pancreas Foundation & M Health Fairview University of Minnesota Masonic Children’s Hospital. Each education event is 1 hour in duration.

Session 1: TPIAT for children with pancreatitis: What do you need to know as a parent of or child with pancreatitis?

Session 2: Nutrition, exocrine insufficiency, and diabetes after pediatric TPIAT. 

Session 3: What to know about life after TPIAT for the teenager: Patient perspectives on school, social life and other topics after TPIAT.

Animated Pancreas

Our Animated Pancreas Patient offers easy-to-understand animations with audio narration, expert video explanations, patient interviews, slide shows, and downloadable information.

You will find expert advice to help you discuss key issues with your healthcare provider and make important decisions related to the management and treatment of pediatric pancreatitis.

The Pediatric Pancreatitis Passport and Action Plan 

The Pediatric Pancreatitis Passport and Action Plan is a document designed by the Pancreas Committee of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) in collaboration with patient advocacy groups including the National Pancreas Foundation, Rebecca’s Wish, and Mission Cure.

The Pediatric Pancreatitis School Toolkit 

School creates many unique challenges for a pediatric pancreatitis patient. The Pediatric Pancreatitis School Toolkit aims to provide parents, school administrators, and teachers access to helpful information and suggestions to navigate the daily difficulties and obstacles pediatric pancreatitis patients experience while attending school. The information is helpful to anyone who takes care of a child with pancreatitis including after-school caregivers, camp counsellors, and day-care personnel.

Camp Hope

The National Pancreas Foundation and Rebecca’s Wish created Camp Hope, so children and young people suffering from pancreatitis, or post-TPIAT, can experience all the joys of camp without limitations. Camp Hope is also a place where they can meet to bond/build friendships in a fun and safe setting that includes camp activities such as swimming, archery, canoeing, fishing, crafts, and more.